Sample records for retroperitoneal sarcoma results

  1. Major vascular resections in retroperitoneal sarcoma.

    PubMed

    Tzanis, Dimitri; Bouhadiba, Toufik; Gaignard, Elodie; Bonvalot, Sylvie

    2018-01-01

    Retroperitoneal sarcomas (RPS) frequently involve major vessels, which either originate from them or secondarily encase or invade them. In this field, major vascular resections result in increased morbidity. However, survival does not seem to be affected by the need for vascular resection or by this higher morbidity. This paper aims to provide descriptions of the surgical strategy and outcomes for retroperitoneal sarcomas involving major vessels. © 2017 Wiley Periodicals, Inc.

  2. The Value of Surgery for Retroperitoneal Sarcoma

    PubMed Central

    Gholami, Sepideh; Jacobs, Charlotte D.; Kapp, Daniel S.; Parast, Layla M.; Norton, Jeffrey A.

    2009-01-01

    Introduction. Retroperitoneal sarcomas are uncommon large malignant tumors. Methods. Forty-one consecutive patients with localized retroperitoneal sarcoma were retrospectively studied. Results. Median age was 58 years (range 20–91 years). Median tumor size was 17.5 cm (range 4–41 cm). Only 2 tumors were <5 cm. Most were liposarcoma (44%) and high-grade (59%). 59% were stage 3 and the rest was stage 1. Median followup was 10 months (range 1–106 months). Thirty-eight patients had an initial complete resection; 15 (37%) developed recurrent sarcoma and 12 (80%) had a second complete resection. Patients with an initial complete resection had a 5-year survival of 46%. For all patients, tumor grade affected overall survival (P = .006). Complete surgical resection improved overall survival for high-grade tumors (P = .03). Conclusions. Tumor grade/stage and complete surgical resection for high-grade tumors are important prognostic variables. Radiation therapy or chemotherapy had no significant impact on overall or recurrence-free survival. Complete surgical resection is the treatment of choice for patients with initial and locally recurrent retroperitoneal sarcoma. PMID:19826633

  3. Carbon Ion Radiotherapy for Unresectable Retroperitoneal Sarcomas

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Serizawa, Itsuko, E-mail: s_itsuko@nirs.go.j; Kagei, Kenji; Kamada, Tadashi

    2009-11-15

    Purpose: To evaluate the applicability of carbon ion radiotherapy (CIRT) for unresectable retroperitoneal sarcomas with regard to normal tissue morbidity and local tumor control. Methods and Materials: From May 1997 to February 2006, 24 patients (17 male and 7 female) with unresectable retroperitoneal sarcoma received CIRT. Age ranged from 16 to 77 years (median, 48.6 years). Of the patients, 16 had primary disease and 8 recurrent disease. Histologic diagnoses were as follows: malignant fibrous histiocytoma in 6, liposarcoma in 3, malignant peripheral nerve sheath tumor in 3, Ewing/primitive neuroectodermal tumor (PNET) in 2, and miscellaneous in 10 patients. The histologicmore » grades were as follows: Grade 3 in 15, Grade 2-3 in 2, Grade 2 in 3, and unknown in 4. Clinical target volumes ranged between 57 cm{sup 3} and 1,194 cm{sup 3} (median 525 cm{sup 3}). The delivered carbon ion dose ranged from 52.8 to 73.6 GyE in 16 fixed fractions over 4 weeks. Results: The median follow-up was 36 months (range, 6-143 months). The overall survival rates at 2 and 5 years were 75% and 50%, respectively. The local control rates at 2 and 5 years were 77% and 69%. No complications of the gastrointestinal tract were encountered. No other toxicity greater than Grade 2 was observed. Conclusions: Use of CIRT is suggested to be effective and safe for retroperitoneal sarcomas. The results obtained with CIRT were a good overall survival rate and local control, notwithstanding the fact that most patients were not eligible for surgical resection and had high-grade sarcomas.« less

  4. Management of metastatic retroperitoneal sarcoma: a consensus approach from the Trans-Atlantic Retroperitoneal Sarcoma Working Group (TARPSWG).

    PubMed

    2018-04-01

    Retroperitoneal sarcoma (RPS) is a rare disease accounting for 0.1%-0.2% of all malignancies. Management of RPS is complex and requires multidisciplinary, tailored treatment strategies at all stages, but especially in the context of metastatic or multifocal recurrent disease. Due to the rarity and heterogeneity of this family of diseases, the literature to guide management is limited. The Trans-Atlantic Retroperitoneal Sarcoma Working Group (TARPSWG) is an international collaboration of sarcoma experts from all disciplines convened in an effort to overcome these limitations. The TARPSWG has compiled the available evidence surrounding metastatic and multifocally recurrent RPS along with expert opinion in an iterative process to generate a consensus document regarding the complex management of this disease. The objective of this document is to guide sarcoma specialists from all disciplines in the diagnosis and treatment of multifocal recurrent or metastatic RPS. All aspects of patient assessment, diagnostic processes, local and systemic treatments, and palliation are reviewed in this document, and consensus recommendations provided accordingly. Recommendations were guided by available evidence, in conjunction with expert opinion where evidence was lacking. This consensus document combines the available literature regarding the management of multifocally recurrent or metastastic RPS with the practical expertise of high-volume sarcoma centers from multiple countries. It is designed as a tool for decision making in the complex multidisciplinary management of this condition and is expected to standardize management across centers, thereby ensuring that patients receive the highest quality care.

  5. Comprehensive Surgical Treatment as the Mainstay of Management in Retroperitoneal Sarcomas: Retrospective Study from Two Non-sarcoma Specialist Centers.

    PubMed

    Petrou, Athanasios; Constantinidou, Anastasia; Kontos, Michael; Papalampros, Alexandros; Moris, Demetrios; Bakoyiannis, Chris; Neofytou, Kyriakos; Kourounis, George; Felekouras, Evangelos

    2017-04-01

    Complete resection, surgical expertise and individualization of patient management in comprehensive oncology centres result in better clinical outcomes in patients presenting with retroperitoneal sarcomas. Clinical outcomes of primary and recurrent retroperitoneal sarcoma resections performed between January 2002 and December 2016 in two large surgical oncology, but non-sarcoma specialist centers, were reviewed to determine the efficacy of complete surgical resection as the principle instrument for treatment. The histological type, tumor size and grade, as well as organ resection, were recorded and subsequently reviewed. Our study included 108 cases of sarcoma resection (60 first-time, 38 second-time and 10 third-time laparotomies) in 60 patients (35 males and 25 females). Most patients had complete resection: 57 had a macroscopically complete (R0/R1) resection and three had R2 resection. The 90-day mortality rate was zero and morbidity was minimal. Five- and 10-year overall survival (OS) rates were 88% and 79%, respectively, whereas the corresponding disease-free survival (DFS) rates were 65% and 59%, respectively. High-grade tumors were associated with decreased DFS (hazard ratio(HR)=3.35; 95% confidence interval(CI)=1.23-9.10; p=0.018) and decreased OS (HR=7.18; 95% CI=1.50-34.22; p=0.013). Complete surgical resection of retroperitoneal sarcomas combined with individualized patient management when offered by experienced surgical oncology teams, adhering to international guidelines, can succeed in providing patients with good long-term outcomes, comparable to those achieved at sarcoma-specialist centers. Copyright© 2017, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.

  6. [Principles of surgery for retroperitoneal sarcoma].

    PubMed

    Hohenberger, P; Dinter, D; Stroebel, P; Kasper, B; Wenz, F

    2014-12-01

    The successful treatment of retroperitoneal soft tissue sarcomas requires an experienced team consisting of not only surgeons but also pathologists and radiologists with a high case load in these tumours. The decisive step in the preoperative work-up of these, often late detected, tumours is their reliable grading as well as, if necessary, recognition of the sarcoma subtype as a basis for determining the direction of treatment. Imaging methods provide essential information with regard to the detection of infiltration of neighbouring structures and organs. Magnetic resonance imaging (MRI) is the most suitable method for this purpose. Punch needle biopsy is to be preferred over fine-needle biopsy in all cases for histological confirmation. The surgical standard procedure for the majority of the patients comprises multivisceral resection as principle, with additional colon resection, nephrectomy, and resection of abdominal wall musculature or, respectively, the psoas muscle in order to achieve an R0 resection of the retroperitoneal compartment. If only small margins of clearance are to be expected, a preoperative (neoadjuvant) treatment with radiation and/or chemotherapy even in combination with deep wave hyperthermia for high grade sarcomas should be strongly considered. Adjuvant postoperative radiation therapy often cannot be adequately applied due to the occupation of the former tumour bed by abdominal organs that were displaced by the mass effect, especially the radiation-sensitive small bowel. The optimal treatment strategy for these patients must be discussed in a multidisciplinary tumour board prior to any diagnostic or therapeutic procedure. Georg Thieme Verlag KG Stuttgart · New York.

  7. Immunostaining for peroxisome proliferator gamma distinguishes dedifferentiated liposarcoma from other retroperitoneal sarcomas.

    PubMed

    Horvai, Andrew E; Schaefer, Jochen T; Nakakura, Eric K; O'Donnell, Richard J

    2008-05-01

    Dedifferentiated liposarcoma can be readily diagnosed by the juxtaposition of a well-differentiated liposarcoma to a nonlipogenic sarcoma. However, if the lipogenic component is not abundant due to surgical sampling or small biopsy, dedifferentiated liposarcoma can be difficult to distinguish from other poorly different sarcomas. Peroxisome proliferator-activated receptor gamma (PPAR-gamma) is a nuclear hormone receptor that plays a critical role in adipocyte differentiation. Prior studies have not only demonstrated PPAR-gamma mRNA in various subtypes of liposarcoma but have also shown that adipocyte differentiation can be induced in some liposarcomas by a PPAR-gamma agonist. In the present study, we investigated whether immunostaining for PPAR-gamma can be used to distinguish dedifferentiated liposarcoma from other retroperitoneal sarcomas. We examined a series of 40 dedifferentiated liposarcoma and compared the staining for PPAR-gamma to a series of 24 retroperitoneal sarcomas that lacked lipogenic differentiation. A monoclonal antibody against PPAR-gamma was used to stain formalin-fixed paraffin-embedded tissue. Specific nuclear immunostaining was present in 37/40 (93%) of the dedifferentiated liposarcoma and 6/24 (25%) of the other sarcomas (two leiomyosarcomas and four undifferentiated sarcomas). Interestingly, immunostaining for CDK4 and/or MDM2 was identified in three of the four PPAR-gamma-positive undifferentiated sarcomas, raising the possibility that these may represent dedifferentiated liposarcoma. This is the first study demonstrating the utility of PPAR-gamma immunohistochemistry in the diagnosis of dedifferentiated liposarcoma in tissue sections. Although not completely specific, the presence of PPAR-gamma staining, in combination with histologic findings and other markers, can aid in the diagnosis of dedifferentiated liposarcoma, particularly on small biopsies that may not sample the well-differentiated component.

  8. Giant retroperitoneal dedifferentiated liposarcoma.

    PubMed

    Beksac, Kemal; Aksel, Bulent; Yukruk, Fisun Ardic; Kandemir, Olcay

    2017-01-16

    Liposarcoma is the most frequent type of retroperitoneal sarcomas. Dedifferentiated liposarcoma is the least common subtype and is an extremely rare tumor. We present the case of a 53-year-old male who was referred with a giant retroperitoneal mass. The patients' mass was deemed unresectable by the previous institution and received chemotherapy with no benefit. We macroscopically removed the 38x32 cm mass with right nefrectomy. Pathological examination revealed dedifferentiated liposarcoma. Surgery is the gold standart in the treatment of retroperitoneal sarcomas. Giant masses present a challenge for the surgeon with possible major vascular injuries and multiorgan resections. Therefore it is important for these patients to be referred for surgery without delay. Dedifferentiated liposarcoma, Liposarcoma, Retroperitoneal sarcoma.

  9. Overall survival after resection of retroperitoneal sarcoma at academic cancer centers versus community cancer centers: An analysis of the National Cancer Data Base.

    PubMed

    Berger, Nicholas G; Silva, Jack P; Mogal, Harveshp; Clarke, Callisia N; Bedi, Manpreet; Charlson, John; Christians, Kathleen K; Tsai, Susan; Gamblin, T Clark

    2018-02-01

    Operative resection remains the definitive curative therapy for retroperitoneal sarcoma. Data published recently show a correlation between improved outcomes for complex oncologic operations and treatment at academic centers. For large retroperitoneal sarcomas, operative resection can be complex and require multidisciplinary care. We hypothesized that survival rates vary between type of treating center for patients undergoing resection for retroperitoneal sarcoma. Patients with stage I to III nonmetastatic retroperitoneal sarcomas who underwent operative resection were identified from the National Cancer Database during the years 2004-2013. Treating centers were categorized as academic cancer centers or community cancer centers. Overall survival was analyzed by log-rank test and graphed using Kaplan-Meier method. A total of 2,762 patients were identified. A majority of patients (59.4%, n = 1,642) underwent resection at an academic cancer centers. Median age at diagnosis was 63 years old. Neoadjuvant radiotherapy was more common at academic cancer centers, while adjuvant radiotherapy was more common at community cancer centers. Improved overall survival was seen at academic cancer centers across all stages compared with community cancer centers (P = .014) but, after multivariable Cox regression analysis, was not a significant independent predictor of survival (hazard ratio = 0.91, 95% confidence interval, 0.79-1.04, P = .171). Academic cancer centers exhibited a greater rate of R0 resection (55.9% vs 47.0%, P < .001) and a lesser odds of positive margins (odds ratio 0.83, 95% confidence interval, 0.69-0.99, P = .044) after multivariable logistic regression. Resection for retroperitoneal sarcoma performed at academic cancer centers was an independent predictor of margin-negative resection but was not a statistically significant factor for survival. This observation suggests that site of care may contribute to some aspect of improved oncologic resection

  10. A Rare Case of Retroperitoneal Follicular Dendritic Cell Sarcoma Identified by 99mTc-HYNIC-TOC SPECT/CT.

    PubMed

    Li, Yi; Xu, Xiaoping; Xu, Junyan; Huang, Dan

    2018-05-31

    Follicular dendritic cell sarcoma is a very rare neoplasm, which is not lymphoma, but originates from a type of immune cells called follicular dendritic cells. We presented a 37-year-old woman who has suffered from obstructive jaundice, weight loss and right upper abdominal pain for 2 months. The contrast CT revealed masses located in the region of pancreatic head and lots of enlarged retroperitoneal lymph nodes, both of which were enhanced on the artery phase of CT images. Meanwhile, Tc-HYNIC-TOC SPECT/CT revealed high activity in the corresponding lesions. After biopsy, the masses were pathologically confirmed as retroperitoneal follicular dendritic cell sarcoma.

  11. Retroperitoneal Sarcoma Target Volume and Organ at Risk Contour Delineation Agreement Among NRG Sarcoma Radiation Oncologists

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Baldini, Elizabeth H., E-mail: ebaldini@partners.org; Abrams, Ross A.; Bosch, Walter

    Purpose: The purpose of this study was to evaluate the variability in target volume and organ at risk (OAR) contour delineation for retroperitoneal sarcoma (RPS) among 12 sarcoma radiation oncologists. Methods and Materials: Radiation planning computed tomography (CT) scans for 2 cases of RPS were distributed among 12 sarcoma radiation oncologists with instructions for contouring gross tumor volume (GTV), clinical target volume (CTV), high-risk CTV (HR CTV: area judged to be at high risk of resulting in positive margins after resection), and OARs: bowel bag, small bowel, colon, stomach, and duodenum. Analysis of contour agreement was performed using the simultaneousmore » truth and performance level estimation (STAPLE) algorithm and kappa statistics. Results: Ten radiation oncologists contoured both RPS cases, 1 contoured only RPS1, and 1 contoured only RPS2 such that each case was contoured by 11 radiation oncologists. The first case (RPS 1) was a patient with a de-differentiated (DD) liposarcoma (LPS) with a predominant well-differentiated (WD) component, and the second case (RPS 2) was a patient with DD LPS made up almost entirely of a DD component. Contouring agreement for GTV and CTV contours was high. However, the agreement for HR CTVs was only moderate. For OARs, agreement for stomach, bowel bag, small bowel, and colon was high, but agreement for duodenum (distorted by tumor in one of these cases) was fair to moderate. Conclusions: For preoperative treatment of RPS, sarcoma radiation oncologists contoured GTV, CTV, and most OARs with a high level of agreement. HR CTV contours were more variable. Further clarification of this volume with the help of sarcoma surgical oncologists is necessary to reach consensus. More attention to delineation of the duodenum is also needed.« less

  12. Retroperitoneal Sarcoma Target Volume and Organ at Risk Contour Delineation Agreement Among NRG Sarcoma Radiation Oncologists

    PubMed Central

    Baldini, Elizabeth H.; Abrams, Ross A.; Bosch, Walter; Roberge, David; Haas, Rick L.M.; Catton, Charles N.; Indelicato, Daniel J.; Olsen, Jeffrey R.; Deville, Curtiland; Chen, Yen-Lin; Finkelstein, Steven E.; DeLaney, Thomas F.; Wang, Dian

    2015-01-01

    Purpose The purpose of this study was to evaluate the variability in target volume and organ at risk (OAR) contour delineation for retroperitoneal sarcoma (RPS) among 12 sarcoma radiation oncologists. Methods and Materials Radiation planning computed tomography (CT) scans for 2 cases of RPS were distributed among 12 sarcoma radiation oncologists with instructions for contouring gross tumor volume (GTV), clinical target volume (CTV), high-risk CTV (HR CTV: area judged to be at high risk of resulting in positive margins after resection), and OARs: bowel bag, small bowel, colon, stomach, and duodenum. Analysis of contour agreement was performed using the simultaneous truth and performance level estimation (STAPLE) algorithm and kappa statistics. Results Ten radiation oncologists contoured both RPS cases, 1 contoured only RPS1, and 1 contoured only RPS2 such that each case was contoured by 11 radiation oncologists. The first case (RPS 1) was a patient with a de-differentiated (DD) liposarcoma (LPS) with a predominant well-differentiated (WD) component, and the second case (RPS 2) was a patient with DD LPS made up almost entirely of a DD component. Contouring agreement for GTV and CTV contours was high. However, the agreement for HR CTVs was only moderate. For OARs, agreement for stomach, bowel bag, small bowel, and colon was high, but agreement for duodenum (distorted by tumor in one of these cases) was fair to moderate. Conclusions For preoperative treatment of RPS, sarcoma radiation oncologists contoured GTV, CTV, and most OARs with a high level of agreement. HR CTV contours were more variable. Further clarification of this volume with the help of sarcoma surgical oncologists is necessary to reach consensus. More attention to delineation of the duodenum is also needed. PMID:26194680

  13. Historical perspectives and future directions in the surgical management of retroperitoneal sarcoma.

    PubMed

    Tseng, William W; Seo, Hyun Jae; Pollock, Raphael E; Gronchi, Alessandro

    2018-01-01

    Retroperitoneal sarcomas (RPS) have fascinated and intrigued physicians both past and present. Operative mortality rates were historically very high and complete resection was not possible for the majority of patients until only the last 2 decades. More recently, changes to the surgical approach and clinical decision-making in RPS have improved patient outcomes. With select integration of nonsurgical therapies, continued RPS-specific research, and ongoing collaborative efforts among major referral centers, the future appears promising. © 2017 Wiley Periodicals, Inc.

  14. Intraoperative radiotherapy using a mobile electron LINAC: a retroperitoneal sarcoma case.

    PubMed

    Beddar, A Sam; Krishnan, Sunil

    2005-01-01

    The advent of mobile LINACs for use in intraoperative radiation therapy (IORT) promises to make IORT more accessible than before and easier to deliver to patients undergoing surgery. Although mobile IORT systems have been available since 1999, few treatment centers currently use them. Here, we present the case of a typical patient undergoing IORT for retroperitoneal sarcoma to show how easy these mobile systems are to use and how adaptable they are within the operating room (OR) environment. We also discuss the roles and coordination of multidisciplinary team members during IORT and the feasibility of using mobile LINACs for IORT.

  15. Treatment Guidelines for Preoperative Radiation Therapy for Retroperitoneal Sarcoma: Preliminary Consensus of an International Expert Panel

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Baldini, Elizabeth H., E-mail: ebaldini@partners.org; Wang, Dian; Haas, Rick L.M.

    Purpose: Evidence for external beam radiation therapy (RT) as part of treatment for retroperitoneal sarcoma (RPS) is limited. Preoperative RT is the subject of a current randomized trial, but the results will not be available for many years. In the meantime, many practitioners use preoperative RT for RPS, and although this approach is used in practice, there are no radiation treatment guidelines. An international expert panel was convened to develop consensus treatment guidelines for preoperative RT for RPS. Methods and Materials: An expert panel of 15 academic radiation oncologists who specialize in the treatment of sarcoma was assembled. A systematicmore » review of reports related to RT for RPS, RT for extremity sarcoma, and RT-related toxicities for organs at risk was performed. Due to the paucity of high-quality published data on the subject of RT for RPS, consensus recommendations were based largely on expert opinion derived from clinical experience and extrapolation of relevant published reports. It is intended that these clinical practice guidelines be updated as pertinent data become available. Results: Treatment guidelines for preoperative RT for RPS are presented. Conclusions: An international panel of radiation oncologists who specialize in sarcoma reached consensus guidelines for preoperative RT for RPS. Many of the recommendations are based on expert opinion because of the absence of higher level evidence and, thus, are best regarded as preliminary. We emphasize that the role of preoperative RT for RPS has not been proven, and we await data from the European Organization for Research and Treatment of Cancer (EORTC) study of preoperative radiotherapy plus surgery versus surgery alone for patients with RPS. Further data are also anticipated pertaining to normal tissue dose constraints, particularly for bowel tolerance. Nonetheless, as we await these data, the guidelines herein can be used to establish treatment uniformity to aid future assessments of

  16. Outcomes in a series of 103 retroperitoneal sarcomas.

    PubMed

    Pierie, J-P E N; Betensky, R A; Choudry, U; Willett, C G; Souba, W W; Ott, M J

    2006-12-01

    To report the effect on outcome of selection in patients receiving intra-operative electron beam radiation (IOERT) and external beam radiation therapy (EBRT). One hundred and three patients treated for primary RS were studied. Median follow-up was 27 months. Clinical presentation, tumor characteristics, and treatment methods were analyzed to determine impact on survival and recurrence and if selection was occurring. Mean age was 55+/-17 years. Mean tumor size was 15+/-6cm and 88 were high-grade. Complete gross tumor resection (CR) occurred in 62 patients and improved survival vs. both debulking (p=0.0005) and biopsy (p<0.0001). The 5- and 10-year survival rates were 62% and 52% for those with CR vs. 29% and 20% after incomplete resection. Among the 62 CR patients, there was selection to receive additional EBRT+/-IOERT in patients with high-grade tumors (p=0.005) and/or microscopically positive margins (p=0.011). In these high-risk patients there was a trend for IOERT to further augment survival vs. EBRT alone and to increase the time to both local and distant recurrences (p=0.036). Complete gross resection is the primary form of curative treatment for retroperitoneal sarcomas. Selection led to patients with high-risk tumors receiving additional radiation therapy. There appears to be a beneficial effect of IOERT plus EBRT in these high-risk patients after complete tumor resection.

  17. [Retroperitoneal liposarcoma as etiology of abdominal pain. Case report and literature review].

    PubMed

    Pérez-Ponce, Yisvanth; Castellanos-Alejandre, Raúl; Guerrero-Romero, J Francisco; Estrada-León, Felipe; Torres-Lobatón, Alfonso

    2008-01-01

    Soft tissue sarcomas are very uncommon types of tumors, with their embryological origin in the mesoderm and in nerve structures of the neuroectodermic layer. They represent only 1.5% of cases in the National Registry of Malignant Tumors in Mexico. They can be encountered anywhere connective soft tissue is found. Because of their specialized localization, retroperitoneal soft tissue sarcomas have a propensity to remain asymptomatic for long periods of time and reach a large size before being diagnosed. The only accepted treatment is wide surgical excision with clear margins, without a clear benefit for adjuvant treatment. The very uncommon nature of these tumors and their varied histopathology, site and behavior classify them as a difficult entity in terms of treatment. We present here the case of a 66-year-old female with a left-side retroperitoneal tumor, complaining only of vague abdominal pain as the presenting symptom. A CT-guided needle biopsy reported a sarcoma and the patient was subjected to laparatomy with complete resection of the tumor (30 x 13 x 10 cm). Histopathological report demonstrated a low-grade retroperitoneal sarcoma and free macroscopic and microscopic borders, without obvious invasion except for left kidney and ureter. The patient refused adjuvant treatment, and she is disease-free 7 years after treatment. Retroperitoneal sarcomas can cause pain and reach very large sizes. The best treatment available is wide surgical resection with clear margins. The most important prognostic factors are free margins, type of resection, age of patient and tumor histology.

  18. Retroperitoneal sarcoma (RPS) high risk gross tumor volume boost (HR GTV boost) contour delineation agreement among NRG sarcoma radiation and surgical oncologists.

    PubMed

    Baldini, Elizabeth H; Bosch, Walter; Kane, John M; Abrams, Ross A; Salerno, Kilian E; Deville, Curtiland; Raut, Chandrajit P; Petersen, Ivy A; Chen, Yen-Lin; Mullen, John T; Millikan, Keith W; Karakousis, Giorgos; Kendrick, Michael L; DeLaney, Thomas F; Wang, Dian

    2015-09-01

    Curative intent management of retroperitoneal sarcoma (RPS) requires gross total resection. Preoperative radiotherapy (RT) often is used as an adjuvant to surgery, but recurrence rates remain high. To enhance RT efficacy with acceptable tolerance, there is interest in delivering "boost doses" of RT to high-risk areas of gross tumor volume (HR GTV) judged to be at risk for positive resection margins. We sought to evaluate variability in HR GTV boost target volume delineation among collaborating sarcoma radiation and surgical oncologist teams. Radiation planning CT scans for three cases of RPS were distributed to seven paired radiation and surgical oncologist teams at six institutions. Teams contoured HR GTV boost volumes for each case. Analysis of contour agreement was performed using the simultaneous truth and performance level estimation (STAPLE) algorithm and kappa statistics. HRGTV boost volume contour agreement between the seven teams was "substantial" or "moderate" for all cases. Agreement was best on the torso wall posteriorly (abutting posterior chest abdominal wall) and medially (abutting ipsilateral para-vertebral space and great vessels). Contours varied more significantly abutting visceral organs due to differing surgical opinions regarding planned partial organ resection. Agreement of RPS HRGTV boost volumes between sarcoma radiation and surgical oncologist teams was substantial to moderate. Differences were most striking in regions abutting visceral organs, highlighting the importance of collaboration between the radiation and surgical oncologist for "individualized" target delineation on the basis of areas deemed at risk and planned resection.

  19. Retroperitoneal tumour radiotherapy: clinical improvements using kilovoltage cone beam computed tomography.

    PubMed

    Juan-Senabre, Xavier J; Ferrer-Albiach, Carlos; Rodríguez-Cordón, Marta; Santos-Serra, Agustín; López-Tarjuelo, Juan; Calzada-Feliu, Salvador

    2009-04-01

    We present a clinical case of a patient diagnosed with a retroperitoneal sarcoma, which received preoperative treatment with daily verification via computed tomography obtained with kilovoltage cone beam. We compare the benefit of this treatment compared to other conventional treatment without image guiding, reporting quantitative results.

  20. Blue Cell Tumour at Unusual Site: Retropritoneal Ewings Sarcoma.

    PubMed

    Javalgi, Anita P; Karigoudar, Mahesh H; Palur, Katyayani

    2016-04-01

    Ewing's sarcoma is a highly malignant tumour of osseous or non-osseous origin, tremed as extra-skeletal Ewings sarcoma if arising from soft tissue. It is rare occurrence tumor most commonly occurring in paravertebral area, chest wall, head & neck and retroperitoneum. Reporting an interesting case of retroperitoneal Ewing's sarcoma in 39 years old female. Patient had complains of abdominal discomfort & vague pain since 2 months, following weakness in lower limb and loss of weight. On detail history and examination she was further referred to detail pathological and radiological investigations. Haematological profile, renal function test and liver function test were in normal limits. USG abdomen was normal, MRI showed a mass in pelvis retroperitoneum measuring 10x10cms, bilateral ovaries and tubes were normal. Because of retroperitoneal nature of tumor and suspicion of uterine sarcoma, laparotomy was performed. The large retroperitoneal mass adherent to posterior of uterus was excised and send for histopathological diagnosis. On gross and microscopy examination the diagnosis of blue cell tumor with PAS positivity, possibility of extraskeletal Ewing's sarcoma/primitive neuro-ectodermal tumor was made which was further confirmed by immunohistochemistry, positive for S100, Vementin and CD99 and negative for desmin and CK. Confirmed diagnosis help in accurate management and improves survival rate.

  1. Retroperitoneal dedifferentiated liposarcoma with osteosarcomatous components: a case report.

    PubMed

    Fujii, Taishi; Arai, Takuma; Sakon, Masahiro; Sawano, Shinji; Momose, Yoshitaka; Ishii, Keiko; Miwa, Shiro

    2013-01-01

    We report a rare case of recurrent retroperitoneal dedifferentiated liposarcoma with osteosarcomatous components. An 82-year-old male diagnosed with recurrent retroperitoneal liposarcoma underwent a tumor resection. Histologically, osseous matrix with osteoid and mature hyaline cartilaginous tissues with high cellularity were observed in a fibrous background through most of the tumor, and scattered MDM2- and CDK4-positive atypical hyperchromatic stromal cells were detected surrounding the dedifferentiated areas. Dedifferentiation occurs in up to 10% of well-differentiated liposarcomas, frequently resembling a malignant fibrous histiocytoma-like pleomorphic sarcoma. In contrast, divergent differentiation with osteosarcomatous components is considered to be extremely rare.

  2. Retroperitoneal dedifferentiated liposarcoma with osteosarcomatous components: a case report

    PubMed Central

    Fujii, Taishi; Arai, Takuma; Sakon, Masahiro; Sawano, Shinji; Momose, Yoshitaka; Ishii, Keiko; Miwa, Shiro

    2013-01-01

    We report a rare case of recurrent retroperitoneal dedifferentiated liposarcoma with osteosarcomatous components. An 82-year-old male diagnosed with recurrent retroperitoneal liposarcoma underwent a tumor resection. Histologically, osseous matrix with osteoid and mature hyaline cartilaginous tissues with high cellularity were observed in a fibrous background through most of the tumor, and scattered MDM2- and CDK4-positive atypical hyperchromatic stromal cells were detected surrounding the dedifferentiated areas. Dedifferentiation occurs in up to 10% of well-differentiated liposarcomas, frequently resembling a malignant fibrous histiocytoma-like pleomorphic sarcoma. In contrast, divergent differentiation with osteosarcomatous components is considered to be extremely rare. PMID:23826426

  3. Rare Aggressive Behavior of MDM2-Amplified Retroperitoneal Dedifferentiated Liposarcoma, with Brain, Lung and Subcutaneous Metastases.

    PubMed

    Ben Salha, Imen; Zaidi, Shane; Noujaim, Jonathan; Miah, Aisha B; Fisher, Cyril; Jones, Robin L; Thway, Khin

    2016-09-05

    Dedifferentiated liposarcoma (DDL) is a histologically pleomorphic sarcoma, traditionally defined as well-differentiated liposarcoma with abrupt transition to high grade, non-lipogenic sarcoma. It can occur as part of recurrent well-differentiated liposarcoma, or may arise de novo . DDL most frequently occurs within the retroperitoneum, and while it is prone to local recurrence, it usually has a lower rate of metastasis than other pleomorphic sarcomas. We describe a case of retroperitoneal dedifferentiated liposarcoma in a 63-year-old male, who showed MDM2 amplification with fluorescence in situ hybridization, which displayed unusually aggressive behavior, with brain, lung and subcutaneous soft tissue metastases. As previous reports of metastatic liposarcoma have largely grouped DDL in with other (genetically and clinically distinct) liposarcoma subtypes, we highlight and discuss the rare occurrence of brain metastasis in MDM2 -amplified retroperitoneal liposarcoma.

  4. Comparison between retroperitoneal leiomyosarcoma and dedifferentiated liposarcoma.

    PubMed

    Ishii, Takeaki; Kohashi, Kenichi; Ootsuka, Hiroshi; Iura, Kunio; Maekawa, Akira; Yamada, Yuichi; Bekki, Hirofumi; Yoshimoto, Masato; Yamamoto, Hidetaka; Iwamoto, Yukihide; Oda, Yoshinao

    2017-06-01

    It is important to distinguish between leiomyosarcoma (LMS) and dedifferentiated liposarcoma (DDLS) in the retroperitoneum. The dedifferentiated component of DDLS shows an LMS-like morphology in some cases; thus, detailed evaluation is necessary to achieve an accurate diagnosis. Immunohistochemically, MDM2 and myogenic markers provide clues for the diagnoses. However, immunoreactivity for MDM2 and myogenic markers has not been well studied in retroperitoneal LMS and DDLS. Here, we compared the clinicopathological data of 20 retroperitoneal tumors initially diagnosed as LMS with that of 36 cases of retroperitoneal DDLS and conducted an immunohistochemical study. Four (20%) of the cases initially diagnosed as LMS were immunoreactive for MDM2. Fifteen cases (41.7%) of DDLS showed positive expression of two or more myogenic markers. The patients with LMS with MDM2 overexpression were older than the patients with LMS without MDM2 overexpression (P=0.0328). LMS with MDM2 overexpression showed a worse prognosis than DDLS (P=0.0408). No significant difference in prognosis was found between LMS without MDM2 overexpression and DDLS with myogenic differentiation. In conclusion, we recommend that systemic MDM2 expression analysis be performed in cases of retroperitoneal sarcoma. Overdependence on the expression of myogenic markers could lead to misdiagnosis in distinguishing LMS from DDLS. Copyright © 2017 Elsevier GmbH. All rights reserved.

  5. Soft-Tissue Sarcomas of the Abdomen and Pelvis: Radiologic-Pathologic Features, Part 2-Uncommon Sarcomas.

    PubMed

    Levy, Angela D; Manning, Maria A; Miettinen, Markku M

    2017-01-01

    Soft-tissue sarcomas occurring in the abdomen and pelvis are an uncommon but important group of malignancies. Recent changes to the World Health Organization classification of soft-tissue tumors include the movement of gastrointestinal stromal tumors (GISTs) into the soft-tissue tumor classification. GIST is the most common intraperitoneal sarcoma. Liposarcoma is the most common retroperitoneal sarcoma, and leiomyosarcoma is the second most common. GIST, liposarcoma, and leiomyosarcoma account for the majority of sarcomas encountered in the abdomen and pelvis and are discussed in part 1 of this article. Undifferentiated pleomorphic sarcoma (previously called malignant fibrous histiocytoma), dermatofibrosarcoma protuberans, solitary fibrous tumor, malignant peripheral nerve sheath tumor, rhabdomyosarcoma, extraskeletal chondro-osseous sarcomas, vascular sarcomas, and sarcomas of uncertain differentiation uncommonly arise in the abdomen and pelvis and the abdominal wall. Although these lesions are rare sarcomas and their imaging features overlap, familiarity with the locations where they occur and their imaging features is important so they can be diagnosed accurately. The anatomic location and clinical history are important factors in the differential diagnosis of these lesions because metastasis, more-common sarcomas, borderline fibroblastic proliferations (such as desmoid tumors), and endometriosis have imaging findings that overlap with those of these uncommon sarcomas. In this article, the clinical, pathologic, and imaging findings of uncommon soft-tissue sarcomas of the abdomen and pelvis and the abdominal wall are reviewed, with an emphasis on their differential diagnosis.

  6. [Surgical management of retroperitoneal soft-tissue sarcomas--an overview].

    PubMed

    Garlipp, B; Schulz, H-U; Zeile, M; Lippert, H; Meyer, F

    2010-12-01

    Retroperitoneal soft-tissue sarcomas (RSTS) represent a rare and heterogeneous class of diseases for which the clinical management is still largely non-standardised. Based on a selective review of recent publications, it was the purpose of the present review article to summarize the current concepts of disease classification, diagnostics and surgical as well as multimodal therapy for these tumors. A clinically based empirical review derived from a literature search focusing on publications from the past 5  years was carried out. Due to the paucity of randomised-controlled trials, therapy for RSTS is largely based on personal experience, retrospectively gathered data and historical controls. Pre-therapeutic planning requires precise information on the localisation, extension, and texture of the tumor through cross-sectional imaging (CT, MRI) as well as histological diagnosis through percutaneous or open biopsy. Complete tumor resection is crucial. Recent studies have confirmed the importance of microscopically negative resection margins which has subsequently led to a trend towards more radical resection. Chemotherapy does not play a role in the adjuvant setting except in clinical trials; however, radiotherapy has been controversely debated in adjuvant RSTS therapy. Efforts to limit radiation toxicity include modern techniques as well as a strategy of using pre-resection radiotherapy instead of postoperative radiation. Surgery is also the treatment of choice for locally recurrent RSTS and pulmonary metastases. The prognosis of RSTS depends on the quality of surgical care and several disease-specific factors (histological type, grading). The clinical management of RSTS is complex and can only partly be considered as evidence-based. Due to the required level of experience in the treatment of these tumor lesions and the involvement of several subspecialties, pre-therapeutic planning, treatment and follow-up should be limited to high-volume surgical centres. In order

  7. Soft-Tissue Sarcomas of the Abdomen and Pelvis: Radiologic-Pathologic Features, Part 2—Uncommon Sarcomas

    PubMed Central

    Manning, Maria A.; Miettinen, Markku M.

    2017-01-01

    Soft-tissue sarcomas occurring in the abdomen and pelvis are an uncommon but important group of malignancies. Recent changes to the World Health Organization classification of soft-tissue tumors include the movement of gastrointestinal stromal tumors (GISTs) into the soft-tissue tumor classification. GIST is the most common intraperitoneal sarcoma. Liposarcoma is the most common retroperitoneal sarcoma, and leiomyosarcoma is the second most common. GIST, liposarcoma, and leiomyosarcoma account for the majority of sarcomas encountered in the abdomen and pelvis and are discussed in part 1 of this article. Undifferentiated pleomorphic sarcoma (previously called malignant fibrous histiocytoma), dermatofibrosarcoma protuberans, solitary fibrous tumor, malignant peripheral nerve sheath tumor, rhabdomyosarcoma, extraskeletal chondro-osseous sarcomas, vascular sarcomas, and sarcomas of uncertain differentiation uncommonly arise in the abdomen and pelvis and the abdominal wall. Although these lesions are rare sarcomas and their imaging features overlap, familiarity with the locations where they occur and their imaging features is important so they can be diagnosed accurately. The anatomic location and clinical history are important factors in the differential diagnosis of these lesions because metastasis, more-common sarcomas, borderline fibroblastic proliferations (such as desmoid tumors), and endometriosis have imaging findings that overlap with those of these uncommon sarcomas. In this article, the clinical, pathologic, and imaging findings of uncommon soft-tissue sarcomas of the abdomen and pelvis and the abdominal wall are reviewed, with an emphasis on their differential diagnosis. PMID:28493803

  8. Combined Modality Management of Retroperitoneal Sarcomas: A Single-Institution Series of 121 Patients

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Bishop, Andrew J.; Zagars, Gunar K.; Torres, Keila E.

    2015-09-01

    Purpose: The purpose of this study was to investigate local control, survival outcomes, and complication rates of patients treated with aggressive surgery and radiation therapy (RT) for retroperitoneal sarcomas (RPS). Methods and Materials: We reviewed the medical records of 121 consecutive patients treated for RPS with surgery and RT between 1965 and 2012. The most common histology was liposarcoma (n=42; 35%). The median follow-up was 100 months (range: 20-467 months). Eighty-six patients (71%) were treated for initial presentation of RPS, and 35 patients (29%) presented with and were treated for RPS recurrence. RT was preoperative in 88 patients (73%; median dose: 50.4 Gy)more » and postoperative in 33 patients (27%; median dose: 55 Gy). Results: Five-year local control and overall survival rates were 56% and 57%, respectively. Two factors were associated with higher risk of any intra-abdominal recurrence at 5 years: positive or uncertain margins (58% vs 30% for negative margins, P<.001; hazard ratio [HR]: 2.7; 95% confidence interval [CI]: 1.6-4.8) and disease recurrence after previous resection (76% vs 31% for de novo RPS, P<.001; HR: 4.4; 95% CI: 2.5-7.5). The 10-year complication rate was 5%, and RT-related complications were associated with postoperative RT (P<.001) and RT dose of ≥60 Gy (P<.001). Conclusions: Intra-abdominal RPS recurrence continues to be a significant challenge despite the use of aggressive surgery and radiation therapy. Given the complications associated with postoperative radiation therapy, we recommend that preoperative radiation therapy is the preferred strategy when combined modality therapy is recommended.« less

  9. Dynamic pion irradiation of unresectable soft tissue sarcomas.

    PubMed

    Greiner, R H; Blattmann, H J; Thum, P; Coray, A; Crawford, J F; Kann, R H; Munkel, G; Pedroni, E; von Essen, C F; Zimmermann, A

    1989-11-01

    Since November 1981, when pion irradiation was introduced for deep seated tumors at the Swiss Institute for Nuclear Research (SIN, now Paul Scherrer Institute, PSI) a dynamic, 3-dimensional spot scan treatment technique has been in use. To exploit this technique a special optimization treatment planning system has been designed. Of a total of 331 patients treated with pions from November 1981-December 1987, 35 were irradiated for unresectable soft tissue sarcomas. In 32/35 patients, tumor sites were retroperitoneal, pelvic or in the groin or thigh. Twenty-nine tumors had a maximum diameter of greater than 10 cm, 18 tumors of greater than 15 cm; 30 tumors had grade 2/3 and 32 Stage III B/IV A/IV B. Eight of 35 patients received a low pion total dose, 7-27 Gy. Twenty-seven patients received a total dose of 30-36 Gy, fraction size 150-170 cGy (90%-isodose), 20 fractions, 4 times per week. Of these 27 patients, severe late reactions appeared in five: 2/8 patients with extremity/groin sarcomas (1/2 caused by biopsy) and 3/19 patients with retroperitoneal/pelvic sarcomas (one a skin reaction after Actinomycin-D, one a small bowel reaction after 36 Gy, a dose no longer used). Seven of 27 patients had metastases at the beginning of irradiation. Three of 27 were treated with excisional biopsy, 9 with incisional biopsy or partial resection and in 15 patients biopsies were performed for histology only. The median follow-up of these 27 patients was 17 months (5-66). There was no progression in eight extremity/groin tumors but in 4 of 19 retroperitoneal/pelvic tumors. Three of these were marginal progressions. The actuarial 5-year rate of local tumor control is 64%; the actuarial 5-year survival rate of patients without metastases at the beginning of treatment is 58%. Dynamic spot scan pion irradiation proves to be a successful treatment technique for unresectable sarcomas with a high rate of tumor control and a very low rate of severe late reactions.

  10. [Retroperitoneal hematoma].

    PubMed

    Dordević, D; Gigić, A; Milev, I; Novaković, B; Sretenović, Z

    1989-01-01

    Diagnosis and management of retroperitoneal haematoma is the problem of controversy in actual moment. It appears most frequently in the range of polytrauma or various traumas of abdomen and retroperitoneal organs. Here we report our experience in management of retroperitoneal haematoma. During the ten year period (from 1979 to 1986) we treated surgically, at the department for surgery, 58 injured patients with retroperitoneal haematoma. In 5 cases explorative laparotomy was done, and in other 53 cases there were injuries of intraabdominal and retroperitoneal organs. The haematomas were caused by the ruptures of spleen, liver, kidneys, pancreas, duodenum, small bowel with mesenterium, large bowel, bladder, retroperitoneal large blood vessels and pelvic fractures. In 17 cases retroperitoneal haematoma was associated with the injury of one organ. In 36 cases there were injuries of two or more organs. Retroperitoneal haematoma was caused by blunt trauma in most cases. During the management there were some diagnostic difficulties. In diagnosis we use: clinical status, of patients, radiography, angiography, ultrasonography, but the most secure was laparotomy. There are two treatment approaches, operative and conservative. Retroperitoneal haematoma was a consequence of ruptured solid organs and retroperitoneal blood vessels, and associated with injuries of intraperitoneal organs. All this, mentioned above, is the reasons for detailed exploration of abdominal cavity.

  11. Predictors of outcomes in patients with primary retroperitoneal dedifferentiated liposarcoma undergoing surgery.

    PubMed

    Keung, Emily Z; Hornick, Jason L; Bertagnolli, Monica M; Baldini, Elizabeth H; Raut, Chandrajit P

    2014-02-01

    Although sarcoma histology is recognized as a prognostic factor, most studies of retroperitoneal sarcomas report results combining multiple histologies and are inadequately powered to identify prognostic factors specific to a particular histology. We reviewed our experience with retroperitoneal dedifferentiated liposarcoma (RP DDLPS) to identify factors predictive of outcomes. All patients with RP DDLPS treated at our institution between 1998 and 2008 were reviewed. Multivariable Cox regression analyses were performed to identify factors predictive of progression-free survival (PFS), local recurrence-free survival (LRFS), distant recurrence-free survival (DRFS), and overall survival (OS). We identified 119 patients with primary DDLPS. Median tumor size was 20.5 cm; 21% were multifocal. French Federation of Cancer Centers Sarcoma Group tumor grades were intermediate in 53% of patients and high in 28% (unknown 19%). Resections were complete (R0/R1) in 80% of patients and incomplete (R2) in 11% (unknown 9%). Tumors were removed intact in 72% of patients and fragmented in 16% (unknown 12%). Median follow-up was 74.1 months. One hundred patients (84%) experienced recurrence or progression, with 92% occurring in the retroperitoneum. Median PFS, LRFS, DRFS, and OS were 21.1, 21.5, 45.8, and 59.0 months, respectively, and were significantly worse with R2 resection. On multivariate analysis, tumor integrity (intact vs fragmented) was predictive of PFS, multifocality predicted LRFS, and extent of resection (R0/R1 vs R2), grade, and tumor integrity predicted OS. In this cohort of primary RP DDLPS, factors under surgeon control (tumor integrity, extent of resection) and reflective of tumor biology (grade, multifocality) impact patient outcomes. Copyright © 2014 American College of Surgeons. Published by Elsevier Inc. All rights reserved.

  12. Neoadjuvant chemotherapy in soft tissue sarcomas: latest evidence and clinical implications

    PubMed Central

    Pasquali, Sandro; Gronchi, Alessandro

    2017-01-01

    Soft tissue sarcomas are a rare and multifaceted group of solid tumours. Neoadjuvant chemotherapy is increasingly used to limit loss of function after wide surgical excision with the ultimate aim of improving patient survival. Recently, advances in the identification of effective treatment strategies and improvements in patient risk stratification have been reached. A randomized trial demonstrated that neoadjuvant epirubicin and ifosfamide improves survival of patients affected by five high-risk soft tissue sarcoma histologies of trunk and extremities, including undifferentiated pleomorphic sarcoma, myxoid liposarcoma, synovial sarcoma, malignant peripheral nerve sheath tumours, and leiomyosarcoma. Selection of patients for these treatments is expected to be improved by the eighth edition of the American Joint Committee on Cancer (AJCC) TNM staging system, as it tailors T-stage categories on primary tumour site and considers a prognostic nomogram for retroperitoneal sarcoma, which also includes soft tissue sarcoma histology and other patient and tumour features not directly included in the TNM staging. Within this framework, this article will present neoadjuvant treatment strategies for high-risk soft tissue sarcoma, emphasizing the most recent advances and discussing the need for further research to improve the effectiveness of neoadjuvant treatments. PMID:28607580

  13. Intra-abdominal primary monophasic synovial sarcoma with hemangiopericytoma-like areas.

    PubMed

    Rao, Lakshmi; Jaiprakash, Padmapriya; Palankar, Nagaraj; Gowda, Vinay

    2013-01-01

    We report a case of retroperitoneal intra-abdominal primary monophasic synovial sarcoma (SS) with hemangiopericytomatous (HPC) pattern in a 25-year-old male arising from the triangular ligament on the superior surface of liver encasing the inferior vena cava (IVC) and masquerading as a hepatic tumor. A large heterogeneously enhancing, well defined, lobulated, exophytic lesion was seen involving segment VIII of the liver with foci of calcification in the periphery. A biopsy, followed by total resection of the tumor, showed a spindle cell sarcoma with HPC pattern, which was consistent with monophasic SS on histology and immunohistochemistry. The unusual clinical presentation, radiology, pathology, and differential diagnosis will be discussed in detail.

  14. Multicystic dedifferentiated retroperitoneal liposarcoma: tumour cyst fluid analysis and implications for management

    PubMed Central

    Khoury, Mitri; Sim, Geok Choo; Harao, Michiko; Radvanyi, Laszlo; Amini, Behrang; Benjamin, Robert S; Pisters, Peter W T; Pollock, Raphael E; Tseng, William W

    2015-01-01

    Liposarcomas are soft tissue sarcomas of adipocyte origin. We describe a case of a dedifferentiated retroperitoneal liposarcoma with an unusual presentation on recurrence as a large, multicystic tumour. The patient was a 72-year-old woman who had undergone multiple treatments including two prior resections. For her most recent locoregional disease recurrence, the patient was offered surgical debulking for symptom palliation. At this operation, performed after two cycles of chemotherapy, the tumour cyst fluid was analysed and found to have a predominance of immune cells with no identifiable malignant cells. This case and the results of our tumour cyst fluid analysis raise several interesting considerations for the management of this unique situation in a rare disease. PMID:26156843

  15. Retroperitoneal liposarcomas: the experience of a tertiary Asian center

    PubMed Central

    2011-01-01

    Background Liposarcoma is the single most common soft tissue sarcoma in the retroperitoneum. Materials and methods A retrospective review of patients with primary retroperitoneal liposarcoma treated between June 1990 and June 2005 were conducted to evaluate the clinical results of resection for retroperitoneal liposarcomas (RPLS) and the prognostic factors for disease recurrence and patient survival in an Asian population. Results Twenty-one patients operated on for curative intent (12 Males, 9 Females; mean age: 52.4 years) were evaluated. Of these, 13 presented with tumors that were well differentiated (61.9%), 4 (19.0%) with myxoid/round cell, 3 (14.3%) with dedifferentiated and 1(4.8%) with pleomorphic morphology. The median tumor burden was 36 cm (9-83). Median follow-up time was 62 months. There was no peri-operative mortality and morbidity occurred in 6(28.6%) patients. Surgical margins were involved in 10(47.6%) patients. Resection of contiguous organs was required in 15(71.4%) to achieve gross surgical margins. Eleven out of the 21(52%) of the patients had recurrence of the tumor. Median disease-free survival was 19 months and the overall 3- and 5-year survival rate was 87% and 49% respectively. Conclusion An aggressive surgical approach in both primary and recurrent RPLS in our institution is associated with 3- and 5-year survival rate of 87% and 49% respectively. Contiguous organ resection is often required to achieve local control. PMID:21284868

  16. Multicystic dedifferentiated retroperitoneal liposarcoma: tumour cyst fluid analysis and implications for management.

    PubMed

    Khoury, Mitri; Sim, Geok Choo; Harao, Michiko; Radvanyi, Laszlo; Amini, Behrang; Benjamin, Robert S; Pisters, Peter W T; Pollock, Raphael E; Tseng, William W

    2015-07-08

    Liposarcomas are soft tissue sarcomas of adipocyte origin. We describe a case of a dedifferentiated retroperitoneal liposarcoma with an unusual presentation on recurrence as a large, multicystic tumour. The patient was a 72-year-old woman who had undergone multiple treatments including two prior resections. For her most recent locoregional disease recurrence, the patient was offered surgical debulking for symptom palliation. At this operation, performed after two cycles of chemotherapy, the tumour cyst fluid was analysed and found to have a predominance of immune cells with no identifiable malignant cells. This case and the results of our tumour cyst fluid analysis raise several interesting considerations for the management of this unique situation in a rare disease. 2015 BMJ Publishing Group Ltd.

  17. Retroperitoneal and transperitoneal robot-assisted pyeloplasty in adults: techniques and results.

    PubMed

    Cestari, Andrea; Buffi, Nicolò Maria; Lista, Giuliana; Sangalli, Mattia; Scapaticci, Emanuele; Fabbri, Fabio; Lazzeri, Massimo; Rigatti, Patrizio; Guazzoni, Giorgio

    2010-11-01

    The surgical management of ureteropelvic junction obstruction (UPJO) has dramatically evolved over the past 20 yr due to the development of new technology. Our aim was to report the feasibility and efficacy of robot-assisted pyeloplasty (RAP) performed by either the retroperitoneal or the transperitoneal approach. A stage 2 investigative study was conducted including development (stage 2a) and exploration (stage 2b) of transperitoneal and retroperitoneal RAP performed in 55 patients at an urban tertiary university department of urology. Retroperitoneal RAP was performed with the patient in full flank position using a 12-mm Hasson-style optical port at the tip of the 12th rib, plus two operative 8-mm robotic trocars and an assistant 5-mm port. The stenotic ureteropelvic junction was excised, the ureter was spatulated, and a dismembered pyeloplasty was performed in all cases. Transperitoneal RAP was performed with the patients in the 60° flank position. The optical port is in the umbilical area, plus two 8-mm operative robotic ports and one 5-mm assistant port. The pyeloplasty technique is similar to the retroperitoneoscopic approach. In both groups, the stent can be positioned in an anterograde or retrograde fashion. Success consisted of no evidence of obstruction on computed tomography urography or mercaptoacetyltriglycine-3 diuretic renal scan, no postoperative symptoms, and no further treatment. Thirty-six patients underwent retroperitoneoscopic RAP and 19 transperitoneal RAP for UPJO. All the procedures were completed with robotic assistance. The overall objective success (measured by diuretic renal scan and/or imaging techniques) was 96% with two cases of recurrence (both in the retroperitoneal group). The main limitation was the short follow-up, although all patients reached at least a 6-mo follow-up. RAP performed either retroperitoneally or transperitoneally was revealed as a feasible and reproducible surgical option for the treatment of UPJO, offering a

  18. Light ion irradiation for unfavorable soft tissue sarcoma

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Linstadt, D.; Castro, J.R.; Phillips, T.L.

    1990-09-01

    Between 1978 and 1989, 32 patients with unfavorable soft tissue sarcoma underwent light ion (helium, neon) irradiation with curative intent at Lawrence Berkeley Laboratory. The tumors were located in the trunk in 22 patients and head and neck in 10. Macroscopic tumor was present in 22 at the time of irradiation. Two patients had tumors apparently induced by previous therapeutic irradiation. Follow-up times for surviving patients ranged from 4 to 121 months (median 27 months). The overall 3-year actuarial local control rate was 62%; the corresponding survival rate was 50%. The 3-year actuarial control rate for patients irradiated with macroscopicmore » tumors was 48%, while none of the patients with microscopic disease developed local recurrence (100%). The corresponding 3-year actuarial survival rates were 40% (macroscopic) and 78% (microscopic). Patients with retroperitoneal sarcoma did notably well; the local control rate and survival rate were 64% and 62%, respectively. Complications were acceptable; there were no radiation related deaths, while two patients (6%) required operations to correct significant radiation-related injuries. These results appear promising compared to those achieved by low -LET irradiation, and suggest that this technique merits further investigation.« less

  19. Soft Tissue Sarcoma, Version 2.2016, NCCN Clinical Practice Guidelines in Oncology.

    PubMed

    von Mehren, Margaret; Randall, R Lor; Benjamin, Robert S; Boles, Sarah; Bui, Marilyn M; Conrad, Ernest U; Ganjoo, Kristen N; George, Suzanne; Gonzalez, Ricardo J; Heslin, Martin J; Kane, John M; Koon, Henry; Mayerson, Joel; McCarter, Martin; McGarry, Sean V; Meyer, Christian; O'Donnell, Richard J; Pappo, Alberto S; Paz, I Benjamin; Petersen, Ivy A; Pfeifer, John D; Riedel, Richard F; Schuetze, Scott; Schupak, Karen D; Schwartz, Herbert S; Tap, William D; Wayne, Jeffrey D; Bergman, Mary Anne; Scavone, Jillian

    2016-06-01

    Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for Soft Tissue Sarcoma (available at NCCN.org) provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumor, desmoid tumors, and rhabdomyosarcoma. This manuscript discusses guiding principles for the diagnosis and staging of STS and evidence for treatment modalities that include surgery, radiation, chemoradiation, chemotherapy, and targeted therapy. Copyright © 2016 by the National Comprehensive Cancer Network.

  20. Retroperitoneal dedifferentiated liposarcoma lacking MDM2 amplification in a patient with a germ line CHEK2 mutation.

    PubMed

    Sadri, Navid; Surrey, Lea F; Fraker, Douglas L; Zhang, Paul J

    2014-04-01

    Germ line mutations in genes that encode proteins involved in the DNA damage response predispose patients to a variety of tumors. Checkpoint kinase 2, encoded by the CHEK2 gene, is important in transducing the DNA damage response. Germ line CHEK2 mutations are seen in a subset of patients with a familial breast cancer and sarcoma phenotype. We report a case of retroperitoneal dedifferentiated liposarcoma in a 61-year-old female with germ line CHEK2 mutation. MDM2 gene amplification normally present and used to aid in the diagnosis of retroperitoneal dedifferentiated liposarcoma was absent in this case. Lack of MDM2 overexpression has similarly been reported in liposarcomas arising in patients with germ line TP53 mutations. We propose this case may highlight a nonamplified MDM2 phenotype in well- and dedifferentiated liposarcomas arising in patients with germ line mutations of genes involved in p53-associated DNA damage response pathways.

  1. Retroperitoneal access for robotic renal surgery.

    PubMed

    Anderson, Barrett G; Wright, Alec J; Potretzke, Aaron M; Figenshau, R Sherburne

    2018-01-01

    Retroperitoneal access for robotic renal surgery is an effective alternative to the commonly used transperitoneal approach. We describe our contemporary experience and technique for attaining retroperitoneal access. We outline our institutional approach to retroperitoneal access for the instruction of urologists at the beginning of the learning curve. The patient is placed in the lateral decubitus position. The first incision is made just inferior to the tip of the twelfth rib as described by Hsu, et al. After the lumbodorsal fascia is traversed, the retroperitoneal space is dilated with a round 10 millimeter AutoSutureTM (Covidien, Mansfield, MA) balloon access device. The following trocars are used: A 130 millimeter KiiR balloon trocar (Applied Medical, Rancho Santa Margarita, CA), three robotic, and one assistant. Key landmarks for the access and dissection are detailed. 177 patients underwent a retroperitoneal robotic procedure from 2007 to 2015. Procedures performed include 158 partial nephrectomies, 16 pyeloplasties, and three radical nephrectomies. The robotic fourth arm was utilized in all cases. When compared with the transperitoneal approach, the retroperitoneal approach was associated with shorter operative times and decreased length of stay (1). Selection bias and surgeon preference accounted for the higher proportion of patients who underwent partial nephrectomy off-camp via the retroperitoneal approach. Retroperitoneal robotic surgery may confer several advantages. In patients with previous abdominal surgery or intra-abdominal conditions, the retroperitoneum can be safely accessed while avoiding intraperitoneal injuries. The retroperitoneum also provides a confined space that may minimize the sequelae of potential complications including urine leak. Moreover, at our institution, retroperitoneal robotic surgery is associated with shorter operative times and a decreased length of stay when compared with the transperitoneal approach (2). In selected

  2. Delayed hydronephrosis due to retroperitoneal hematoma after a seatbelt injury

    PubMed Central

    Yumoto, Tetsuya; Kondo, Yoshitaka; Kumon, Kento; Masaoka, Yoshihisa; Hiraki, Takao; Yamada, Taihei; Naito, Hiromichi; Nakao, Atsunori

    2018-01-01

    Abstract Rationale: Hydronephrosis caused by retroperitoneal hematoma after a seatbelt injury is a unique clinical entity. Patient concerns: A 21-year-old man, who had been wearing a seatbelt, was brought to our hospital after a motor vehicle collision, complaining of abdominal pain. Computed tomography (CT) revealed retroperitoneal hematoma in the upper pelvic region. Since he was hemodynamically stable throughout admission, he was managed conservatively. Seventeen days after initial discharge, the patient revisited our emergency department due to right back pain. Diagnoses: CT scans indicated retroperitoneal hematoma growth resulting in hydronephrosis of the right kidney. Interventions: Laparoscopic drainage of the retroperitoneal hematoma was successfully performed. Outcomes: His symptoms resolved after the surgery. Follow-up CT scans three months later demonstrated complete resolution of the hydronephrosis and retroperitoneal hematoma. Lessons: Our case highlights a patient with delayed hydronephrosis because of retroperitoneal hematoma expansion after a seatbelt injury. PMID:29879068

  3. Primary retroperitoneal mucinous cystadenocarcinoma: report of two cases

    PubMed Central

    de León, David Cantú; Pérez-Montiel, Delia; Chanona-Vilchis, José; Dueñas-González, Alfonso; Villavicencio-Valencia, Verónica; Zavala-Casas, Gladys

    2007-01-01

    Background Retroperitoneal cystadenocarcinomas are rare lesions, the majority of cases presented as one-patient reports. Methods We present two cases of retroperitoneal cystadenocarcinoma, both in women of reproductive age: one with aggressive behavior, and the remaining case, with a more indolent clinical evolution. Results One case presented as pelvic tumor, was treated with surgical resection of the disease, but manifested with recurrent disease a few months later despite use of chemotherapy. The second case involved a patient with diagnosis of abdominal tumor; during laparotomy, a retroperitoneal tumor was found and was totally removed. At follow-up, the patient is disease-free with no other treatment. Conclusion The behavior and treatment of retroperitoneal cystadenocarcinoma are controversial. We suggest aggressive surgery including radical hysterectomy and bilateral salpingoopherectomy with adjuvant chemotherapy in these cases. PMID:17224073

  4. Correlation between CO2 storage at the last minute of gas insufflation and area of retroperitoneal lacuna during retroperitoneal laparoscopic radical nephrectomy.

    PubMed

    Hu, Jian-Jun; Liu, Ya-Hua; Yu, Chan-Juan; Jialielihan, Nuerbolati

    2016-07-22

    Adequate operation interspace is the premise of laparoscopy, and carbon dioxide (CO2) was an ideal gas for forming lacuna. A retroperitoneal space is used to form operation interspace in retroperitoneal laparoscopic radical nephrectomy by making ballooning, and the retroperitoneal space has no relative complete and airtight serous membrane, therefore CO2 absorption may be greater in retroperitoneal than transperitoneal laparoscopic radical nephrectomy. Excess CO2 absorption may induce hypercapnemia and further cause physiopathological change of respiratory and circulatory system. Therefore, exact evaluation of amount of CO2 which is eliminated from body via minute ventilation is important during retroperitoneal laparoscopic radical nephrectomy. The aim of the paper is to study the correlation between CO2 storage at the last minute of gas insufflation and area of retroperitoneal lacuna during retroperitoneal laparoscopic radical nephrectomy. Forty ASA I/II patients undergoing retroperitoneal laparoscopic radical nephrectomy were enrolled. CO2 storage at the last minute of gas insufflation and area of a retroperitoneal lacuna were observed. Linear correlation and regression were performed to determine the correlation between them. There was positive correlation between CO2 storage at the last minute of gas insufflation and area of retroperitoneal lacuna (r = 0.880, P = 0.000), and the equation of linear regression was y = -83.097 + 0.925x (R(2) = 0.780, t = 11.610, P = 0.000). Amount of CO2 which is eliminated from body via mechanical ventilation could be calculated by measuring the area of retroperitoneal lacuna during retroperitoneal laparoscopic radical nephrectomy, and an anesthetist should be aware of the size of lacuna to predict high CO2 storage at the last minute of gas insufflation.

  5. Retroperitoneal fibrosis

    MedlinePlus

    ... prescribed. When medicine does not work, surgery and stents (draining tubes) are needed. Outlook (Prognosis) The outlook ... Names Idiopathic retroperitoneal fibrosis; Ormond's disease Images Male urinary system References Nakada SY, Best SL. Management of ...

  6. Delayed hydronephrosis due to retroperitoneal hematoma after a seatbelt injury: A case report.

    PubMed

    Yumoto, Tetsuya; Kondo, Yoshitaka; Kumon, Kento; Masaoka, Yoshihisa; Hiraki, Takao; Yamada, Taihei; Naito, Hiromichi; Nakao, Atsunori

    2018-06-01

    Hydronephrosis caused by retroperitoneal hematoma after a seatbelt injury is a unique clinical entity. A 21-year-old man, who had been wearing a seatbelt, was brought to our hospital after a motor vehicle collision, complaining of abdominal pain. Computed tomography (CT) revealed retroperitoneal hematoma in the upper pelvic region. Since he was hemodynamically stable throughout admission, he was managed conservatively. Seventeen days after initial discharge, the patient revisited our emergency department due to right back pain. CT scans indicated retroperitoneal hematoma growth resulting in hydronephrosis of the right kidney. Laparoscopic drainage of the retroperitoneal hematoma was successfully performed. His symptoms resolved after the surgery. Follow-up CT scans three months later demonstrated complete resolution of the hydronephrosis and retroperitoneal hematoma. Our case highlights a patient with delayed hydronephrosis because of retroperitoneal hematoma expansion after a seatbelt injury.

  7. [Retroperitoneal bronchogenic cyst treated by laparoscopic surgery].

    PubMed

    Rud, O; May, M; Brookman-Amissah, S; Moersler, J; Greiner, A; Gilfrich, C

    2010-03-01

    The diagnosis of primary retroperitoneal cystic tumors is very infrequent in surgical pathology. We report the case of a 51-year-old woman presenting with an incidental left-sided retroperitoneal mass (32 x 24 mm in diameter) suspected of being an adrenal tumor. Intraoperatively the tumor was identified as a cystic lesion filled with mucous secretion and laparoscopically completely resected. The diagnosis was histopathologically confirmed as a bronchogenic cyst. In this article the laparoscopic removal of such a rare benign congenital aberration resulting from an abnormal budding of the tracheobronchial tree is presented.

  8. Retroperitoneal abscesses in seven dogs.

    PubMed

    Marvel, Sarah J; MacPhail, Catriona M

    2013-01-01

    Retroperitoneal abscesses, although uncommon, are clinically important. Medical records of seven dogs with naturally occurring retroperitoneal infections from 1999 to 2011 were reviewed to document historical, examination, clinicopathologic, imaging, and surgical findings; etiologic agents; and outcome. Middle-aged sporting dogs were most commonly affected. Dogs were febrile with evidence of either abdominal or lumbar pain. Although traditional radiography can aid in diagnosis, ultrasound appeared to be a more sensitive indicator of disease within the retroperitoneal space. Numerous bacteria were isolated from the abscesses, with anaerobic and facultative anaerobic bacteria being the most commonly isolated. Etiology was largely unknown, but migrating plant material was often suspected. Resolution of the infection required surgical intervention as well as prolonged antibiotic therapy. Although recurrence is possible, outcome can be favorable with no long-term sequela.

  9. Endoscopic findings following retroperitoneal pancreas transplantation.

    PubMed

    Pinchuk, Alexey V; Dmitriev, Ilya V; Shmarina, Nonna V; Teterin, Yury S; Balkarov, Aslan G; Storozhev, Roman V; Anisimov, Yuri A; Gasanov, Ali M

    2017-07-01

    An evaluation of the efficacy of endoscopic methods for the diagnosis and correction of surgical and immunological complications after retroperitoneal pancreas transplantation. From October 2011 to March 2015, 27 patients underwent simultaneous retroperitoneal pancreas-kidney transplantation (SPKT). Diagnostic oesophagogastroduodenoscopy (EGD) with protocol biopsy of the donor and recipient duodenal mucosa and endoscopic retrograde pancreatography (ERP) were performed to detect possible complications. Endoscopic stenting of the main pancreatic duct with plastic stents and three-stage endoscopic hemostasis were conducted to correct the identified complications. Endoscopic methods showed high efficiency in the timely diagnosis and adequate correction of complications after retroperitoneal pancreas transplantation. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  10. Retroperitoneal teratoma with somatic malignant transformation: a papillary renal cell carcinoma in a testicular germ cell tumour metastasis following platinum-based chemotherapy.

    PubMed

    Zeh, Nina; Wild, Peter J; Bode, Peter K; Kristiansen, Glen; Moch, Holger; Sulser, Tullio; Hermanns, Thomas

    2013-02-12

    Malignant transformation describes the phenomenon in which a somatic component of a germ cell teratoma undergoes malignant differentiation. A variety of different types of sarcoma and carcinoma, all non-germ cell, have been described as a result of malignant transformation. A 33-year-old man presented with a left testicular mass and elevated tumour markers. Staging investigations revealed retroperitoneal lymphadenopathy with obstruction of the left ureter and distant metastases. Histopathology from the left radical orchiectomy showed a mixed germ cell tumour (Stage III, poor prognosis). The ureter was stented and four cycles of cisplatin, etoposide and bleomycin chemotherapy administered. After initial remission, the patient recurred four years later with a large retroperitoneal mass involving the renal vessels and the left ureter. Left retroperitoneal lymph node dissection with en-bloc resection of the left kidney was performed.Histopathology revealed a germ cell tumour metastasis consisting mainly of mature teratoma. Additionally, within the teratoma a papillary renal cell carcinoma was found. The diagnosis was supported by immunohistochemistry showing positivity for AMACR, CD10 and focal expression of RCC and CK7. There was no radiological or histo-pathological evidence of a primary renal cell cancer. To the best of our knowledge, malignant transformation into a papillary renal cell carcinoma has not been reported in a testicular germ cell tumour metastasis following platinum-based chemotherapy. This histological diagnosis might have implications for potential future therapies. In the case of disease recurrence, renal cell cancer as origin of the recurrent tumour has to be excluded because renal cell carcinoma metastases would not respond well to the classical germ cell tumour chemotherapy regimens.

  11. Special problems encountering surgical management of large retroperitoneal schwannomas.

    PubMed

    Theodosopoulos, Theodosios; Stafyla, Vaia K; Tsiantoula, Paraskevi; Yiallourou, Anneza; Marinis, Athanasios; Kondi-Pafitis, Agathi; Chatziioannou, Achilleas; Boviatsis, Efstathios; Voros, Dionysios

    2008-10-03

    Retroperitoneal schwannomas are rare, usually benign tumors that originate in the neural sheath and account for only a small percentage of retroperitoneal tumors. The aim of this clinical study is to present our experience in managing retroperitoneal schwannomas with a review of the current literature and to point out the surgical technical difficulties we faced, due to the tumor's strange behavior that eroded the vertebra in two cases without causing malignant invasion. We reviewed the medical files of 69 patients treated in our department for retroperitoneal tumors from January 1991 until December 2006. Five patients had retroperitoneal schwannomas according to pathology report. There were two male and three female patients, with a mean age of 56 years (range 44-67 years). All patients were asymptomatic and none suffered from von Recklinghausen disease. Imaging workup included ultrasonography, computed tomography and magnetic resonance imaging. One patient, after having a non-diagnostic computed tomography fine needle aspiration (CT-FNA), underwent exploratory laparotomy and incisional biopsy that established the diagnosis of schwannoma. After complete excision of the tumors, postoperative course was uneventful in all patients. Tumors' maximum diameter was 12.7 cm (range 7-20 cm). No recurrences were detected during the follow up period (6-75 months). Preoperative establishment of diagnosis is difficult in case of retroperitoneal schwannomas, however close relationship of retroperitoneal tumors with adjacent neural structures in imaging studies should raise a suspicion. Complete surgical resection is the treatment of choice. Histology and Immunohistochemistry confirms the diagnosis.

  12. A GIANT RETROPERITONEAL LIPOMA PRESENTING AS A SCIATIC HERNIA: MRI FINDINGS.

    PubMed

    Duran, S; Cavusoglu, M; Elverici, E; Unal, T D

    2015-01-01

    Sciatic hernia is a rare condition and its clinical diagnosis is uneasy. Herniation of pelvic organs as well as of retroperitoneal neoplasm has been reported in the literature. Sciatica occurs as a result of compression of the sciatic nerve by the herniated sac. We present a case of retroperitoneal lipoma in a patient who had lower leg complaint and describe the imaging findings.

  13. Retroperitoneal Laparoscopic Ureterolithotomy for Proximal Ureteral Calculi in Selected Patients

    PubMed Central

    Hu, Qingfeng; Ding, Weihong; Gou, Yuancheng; Ho, Yatfaat; Xu, Ke; Gu, Bin; Sun, Chuanyu; Xia, Guowei; Ding, Qiang

    2014-01-01

    Objectives. To summarize our experience of retroperitoneal laparoscopic ureterolithotomy for ureteral calculi and evaluate the safety and efficiency of this procedure. Methods. We conducted a retrospective analysis of 197 patients with proximal ureteral calculi who accepted retroperitoneal laparoscopic ureterolithotomy from June 2005 to June 2014. Results. All procedures were performed successfully and the mean operating time and estimated blood loss were 87 min and 64 mL. The clearance rate was 98.5% and the rates of urine leak and ureteral stricture were 2.5% and 1.0%. Conclusions. Retroperitoneal laparoscopic ureterolithotomy is a safe and effective procedure for patients with complex stones or anatomic abnormalities, and, with experience of high volume series, it is also a reasonable choice as the primary treatment for such selected patients. PMID:25548791

  14. Locoregional disease patterns in well-differentiated and dedifferentiated retroperitoneal liposarcoma: implications for the extent of resection?

    PubMed

    Tseng, William W; Madewell, John E; Wei, Wei; Somaiah, Neeta; Lazar, Alexander J; Ghadimi, Markus P; Hoffman, Aviad; Pisters, Peter W T; Lev, Dina C; Pollock, Raphael E

    2014-07-01

    Well-differentiated (WD)/dedifferentiated (DD) liposarcoma is the most common soft tissue sarcoma of the retroperitoneum. The frequency of distant metastasis is low and the major burden of disease is locoregional. We sought to define the patterns of locoregional disease to help guide surgical decision making. Data were collected from 247 patients with de novo or recurrent tumors treated at our institution from 1993 to early 2012. The number and location of tumors at both initial presentation and subsequent locoregional recurrence were determined by combined analysis of operative dictations and radiologic imaging. Thirty-four percent of patients had multifocal locoregional disease (two or more tumors) at initial presentation to our institution, including 9 % who had tumors at synchronous remote retroperitoneal sites. The impact of multifocal disease on overall survival was dependent on histologic subtype (WD vs. DD) and disease presentation (de novo vs. recurrence) at the time of resection. Among patients with initial unifocal disease, 57 % progressed to multifocal locoregional disease with subsequent recurrence, including 11 % with new tumors outside of the original resection field. No clinicopathologic or treatment-related variable, including the type or extent of resection, was predictive of either multifocal or 'outside field' progression. Multifocal disease is common in patients with WD/DD retroperitoneal liposarcoma, and tumors can also develop at remote, locoregional sites. Surgical resection remains the primary method of locoregional control in this disease; however, the aggressiveness of resection should be individualized, with consideration of both tumor and patient-related factors.

  15. Results for patients with sarcoma not otherwise specified and other diagnoses than Ewing sarcoma treated according to the Euro-EWING 99 trial.

    PubMed

    Frank, Judith Amalie; Ranft, Andreas; Paulussen, Michael; Juergens, Heribert; Kruseova, Jarmila; Bauer, Sebastian; Niggli, Felix; Reichardt, Peter; Dirksen, Uta

    2017-10-01

    Euro-EWING 99 trial of the European Ewing tumor Working Initiative of National Groups (EE99) was an international phase III study in patients with Ewing sarcoma. The German Society of Pediatric Oncology and Hematology (GPOH) data center registered and followed patients with other diagnoses than Ewing sarcoma who were treated according to the EE99 protocol in an additional non-Ewing database. Data of 27 patients with other diagnoses than Ewing sarcoma treated according to the EE99 protocol were analyzed. Patients had miscellaneous histologic diagnoses, the majority were diagnosed with sarcoma not otherwise specified (NOS) arising in bone and soft tissue (63%). The median age at diagnosis was 16.9 years (range 4.5-41.4). Localized disease was diagnosed in 61.5% of the patients and 38.5% had distant metastases at time of primary diagnosis. The median follow-up time was 3.7 years (range 0.5-9.5). Patients with localized disease showed a 3-year event-free survival (EFS) of 68%, compared to 3-year EFS of 20% in patients with metastases (P = 0.042). Three-year EFS for patients with sarcoma NOS was 52%, patients with localized and metastatic disease showed 3-year EFS of 66 and 20%, respectively. EFS in patients with other diagnoses than Ewing sarcoma treated according to EE99 was significantly higher in patients with localized than metastatic disease. Sarcomas of soft tissue and bone that cannot be classified to current diagnostic categories constitute a therapeutic challenge. © 2017 Wiley Periodicals, Inc.

  16. Retroperitoneal fibrosis as a result of signet ring cell gastric cancer: a case-based review.

    PubMed

    Karbasi, Ashraf; Karbasi-Afshar, Reza; Ahmadi, Javad; Saburi, Amin

    2013-03-01

    Retroperitoneal fibrosis is a rare and obvious condition in the abdominopelvic cavity. Signet ring cell carcinoma of the stomach with gross appearance of linitis plastica is another rare association. We present a rare case of a 49-year-old woman presenting with persistent nausea and vomiting for 20 days. Three months ago, she was admitted with severe flank pain and was referred to the urology service. She was placed with stents for strictures in both ureters. She underwent endoscopy for heartburn, melena, dyspepsia, and weight loss (7 kg/5 month), which revealed congestion of the mucosa of the fundus and body with fine linear erosion and decreased distensibility of the stomach suggesting linitis plastica. A biopsy indicated full-thickness infiltration by signet ring cell gastric cancer. A CT scan showed evidence of retroperitoneal fibrosis with large lymph nodes around the aorta. This paper shows that the gastric linitis plastica can present with many deceptive clinical presentations, raising the risk of postponed diagnosis.

  17. Pelvic confined idiopathic retroperitoneal fibrosis mimicking a large tumor.

    PubMed

    Salemis, N S; Tsiambas, E; Tsohataridis, E

    2009-01-01

    Idiopathic retroperitoneal fibrosis (IRF) entirely confined to the pelvic cavity is an extremely rare clinical entity. Herein, is described the case of a 36- year old male who presented with clinical and imaging manifestations of a large pelvic tumor. Exploratory laparotomy revealed a large mass in the right pelvis originating from the retroperitoneal space, displacing the right iliac vessels, the right ureter and the urinary bladder completely to the left. A laborious resection of the mass measuring 14 x 10cm was performed. Histopathological examination and detailed immunohistochemistry analysis were suggestive of idiopathic retroperitoneal fibrosis with no evidence of malignancy. This is a very rare case regarding localization and clinical presentation of idiopathic retroperitoneal fibrosis. We conclude that IRF should be included in the differential diagnosis of patients presenting with a pelvic mass even if there is no involvement of the typical para aortic area.

  18. Dedifferentiated Liposarcoma With Rhabdomyosarcomatous Differentiation Producing HCG: A Case Report of a Diagnostic Pitfall.

    PubMed

    Maryamchik, Elena; Lyapichev, Kirill A; Halliday, Bradford; Rosenberg, Andrew E

    2018-03-01

    We report a first case of paraneoplastic human chorionic gonadotropin (HCG) production in a dedifferentiated liposarcoma with rhabdosarcomatous differentiation in an 83-year-old man with a retroperitoneal mass, unilateral scrotal enlargement, and a serum HCG level of 843 IU/L. Core biopsy of the retroperitoneal mass revealed rhabdomyosarcoma. Orchiectomy revealed a paratesticular dedifferentiated liposarcoma with rhabdosarcomatous differentiation. Fluorescence in situ hybridization analysis performed on both the retroperitoneal and paratesticular masses revealed amplification of MDM2. The retroperitoneal tumor was interpreted as metastatic dedifferentiated liposarcoma with the dedifferentiated component represented by rhabdomyosarcoma. HCG production is a common feature of testicular germ cell tumors, less common in carcinomas, and extremely rare in sarcomas. Accordingly, sarcomas secreting HCG are a potential diagnostic pitfall, and raise the differential diagnosis of germ cell tumors and a variety of carcinomas. HCG production by carcinomas is a known poor prognostic finding, however the significance of its production in sarcomas is unknown.

  19. Surgical anatomy of the retroperitoneal spaces, Part V: Surgical applications and complications.

    PubMed

    Mirilas, Petros; Skandalakis, John E

    2010-04-01

    Knowledge of the surgical anatomy of the retroperitoneum is crucial for surgery of the retroperitoneal organs. Surgery is essential for treatment of retroperitoneal pathologies. The list of these diseases is extensive and comprises acute and chronic inflammatory processes (abscess, injury, hematoma, idiopathic fibrosis), metastatic neoplasms, and primary neoplasms from fibroadipose tissue, connective tissue, smooth and striated muscle, vascular tissue, somatic and sympathetic nervous tissue, extraadrenal chromaffin tissue, and lymphatic tissue. The retroperitoneum can be approached and explored by several routes, including the transperitoneal route and the extraperitoneal route. The retroperitoneal approach to the iliac fossa is used for ectopic renal transplantation. Safe and reliable primary retroperitoneal access can be performed for laparoscopic exploration. The anatomic complications of retroperitoneal surgery are the complications of the organs located in several compartments of the retroperitoneal space. Complications may arise from incisions to the somatic wall, somatic nerves, blood and lymphatic vessels, lymph nodes, visceral autonomous plexuses, and neighboring splanchna.

  20. Huge retroperitoneal dedifferentiated liposarcoma presented as acute pancreatitis: report of a case.

    PubMed

    Arakawa, Yusuke; Yoshioka, Kazuo; Kamo, Hitomi; Kawano, Koichiro; Yamaguchi, Takeshi; Sumise, Yuko; Okitsu, Natsu; Ikeyama, Shizuo; Morimoto, Kojiro; Nakai, Yoshihiro; Tashiro, Seiki

    2013-01-01

    A 74-year-old male with abdominal pain was admitted to the emergency room in our hospital. The high value of serum amylase was shown in his blood test. The postcontrast computed tomography (CT) showed the huge retroperitoneal tumor with a thin-walled mass occupying most of the part of the right retroperitoneal space. The tumor spread into the soft tissues around the pancreas; as a result, the duodenum was compressed and the pancreas was displaced to the right side. The irregular pancreatic outline, obliterated peripancreatic fatty tissue and fluid in the left anterior pararenal space were revealed, so acute pancreatitis was diagnosed. The diagnostic biopsy of retroperitoneal tumor was done, and the pathological findings of retroperitoneal mass revealed dedifferentiated liposarcoma. The medical treatment against acute pancreatitis was performed firstly. After the patient recovered from that, the surgical resection of the tumor with the right kidney and right adrenal gland was completed successfully. The patient remained well, without any evidence of recurrence three months after surgery. However, the histology showed dedifferentiated liposarcoma; therefore, postoperative regular examination is necessary.

  1. Phase II Study of Neoadjuvant Bevacizumab and Radiotherapy for Resectable Soft Tissue Sarcomas

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Yoon, Sam S., E-mail: syoon@partners.org; Department of Cancer Biology, University of Pennsylvania School of Medicine, Philadelphia, PA; Duda, Dan G.

    Purpose: Numerous preclinical studies have demonstrated that angiogenesis inhibitors can increase the efficacy of radiotherapy (RT). We sought to examine the safety and efficacy of bevacizumab (BV) and RT in soft tissue sarcomas and explore biomarkers to help determine the treatment response. Methods and Materials: Patients with {>=}5 cm, intermediate- or high-grade soft tissue sarcomas at significant risk of local recurrence received neoadjuvant BV alone followed by BV plus RT before surgical resection. Correlative science studies included analysis of the serial blood and tumor samples and serial perfusion computed tomography scans. Results: The 20 patients had a median tumor sizemore » of 8.25 cm, with 13 extremity, 1 trunk, and 6 retroperitoneal/pelvis tumors. The neoadjuvant treatment was well tolerated, with only 4 patients having Grade 3 toxicities (hypertension, liver function test elevation). BV plus RT resulted in {>=}80% pathologic necrosis in 9 (45%) of 20 tumors, more than double the historical rate seen with RT alone. Three patients had a complete pathologic response. The median microvessel density decreased 53% after BV alone (p <.05). After combination therapy, the median tumor cell proliferation decreased by 73%, apoptosis increased 10.4-fold, and the blood flow, blood volume, and permeability surface area decreased by 62-72% (p <.05). Analysis of gene expression microarrays of untreated tumors identified a 24-gene signature for treatment response. The microvessel density and circulating progenitor cells at baseline and the reduction in microvessel density and plasma soluble c-KIT with BV therapy also correlated with a good pathologic response (p <.05). After a median follow-up of 20 months, only 1 patient had developed local recurrence. Conclusions: The results from the present exploratory study indicated that BV increases the efficacy of RT against soft tissue sarcomas and might reduce the incidence of local recurrence. Thus, this regimen

  2. Retroperitoneal Necrotizing Fasciitis Masquerading as Perianal Abscess - Rare and Perilous.

    PubMed

    Amaranathan, Anandhi; Sahoo, Ashok Kumar; Barathi, Deepak; Shankar, Gomathi; Sistla, Sarath Chandra

    2017-01-17

    Necrotizing fasciitis is one of the uncommon presentations of a rapidly spreading subcutaneous tissue infection. Although the actual cause is unclear in many cases, most of them are due to the rapid proliferation of microorganisms. Retroperitoneal necrotizing fasciitis is extremely rare. It is a potentially lethal infection that requires immediate and aggressive surgical care. Early diagnosis is the key to a better prognosis. The possibility of retroperitoneal necrotizing fasciitis should be suspected in patients with symptoms of sepsis that are disproportionate to clinical findings. The rapid deterioration of the patient also gives a clue towards the diagnosis. We report a 35-year-old male with perianal abscess who had been progressed to retroperitoneal necrotizing fasciitis. The patient was managed successfully with aggressive debridement and drainage after laparotomy. Appropriate antibiotics were used to combat the sepsis. The patient recovered well at follow up, three months after discharge. Another patient, a 45-year-old male with a retroperitoneal abscess, progressed to retroperitoneal necrotizing fasciitis, and extra peritoneal drainage and debridement was done. Antibiotics depending upon the culture and sensitivity were used to control sepsis. But the patient succumbed to death 45 days after surgery due to uncontrolled sepsis. Necrotizing fasciitis of any anatomical site needs aggressive surgical care with early intervention. But retroperitoneal necrotizing fasciitis needs an extra effort for diagnosis. After diagnosis, it needs timely surgical intervention and appropriate antibiotic therapy for the recovery of the patients.

  3. First case report of retroperitoneal metastasis of fascioliasis after surgery

    PubMed Central

    Wang, Jun-Ke; Ma, Wen-Jie; Lu, Qiang; Zheng, Er-Liang; Yang, Qin; Hu, Hai-Jie; Liu, Fei; Li, Quan-Sheng; Li, Fu-Yu

    2017-01-01

    Abstract Rationale: Fascioliasis is a rare cause of liver abscesses, and its clinical course consists of hepatic phase and biliary phase. Patient concerns: We describe a 58-year-old female patient who presented with a 2-month history of intermittent fever and abdominal pain. An abdominal computed tomography (CT) revealed confluent low-density lesions in the liver. Complete surgical resection of these abscesses was performed, and postoperative pathological examination and serological tests confirmed a diagnosis of fascioliasis. However, 4 months after the surgery, follow-up CT revealed a lesion in the retroperitoneal area. Meanwhile, ultrasonography-guided percutaneous needle biopsy of the retroperitoneal lesion was performed, and a parasitic infection was suspected. Diagnoses: Retroperitoneal metastasis of hepatic phase fascioliasis. Interventions: The patient received parasitic resistance treatment with triclabendazole at a dose of 10 mg/kg/d for 2 consecutive days. Outcomes: After 2 courses of triclabendazole therapy, the retroperitoneal metastasis regressed to a minor lesion. Lessons: To the best of our knowledge, this is the first case report of retroperitoneal metastasis of fascioliasis, aimed at helping recognize the clinical features and treatment options of this rare disease. PMID:29390366

  4. First case report of retroperitoneal metastasis of fascioliasis after surgery.

    PubMed

    Wang, Jun-Ke; Ma, Wen-Jie; Lu, Qiang; Zheng, Er-Liang; Yang, Qin; Hu, Hai-Jie; Liu, Fei; Li, Quan-Sheng; Li, Fu-Yu

    2017-12-01

    Fascioliasis is a rare cause of liver abscesses, and its clinical course consists of hepatic phase and biliary phase. We describe a 58-year-old female patient who presented with a 2-month history of intermittent fever and abdominal pain. An abdominal computed tomography (CT) revealed confluent low-density lesions in the liver. Complete surgical resection of these abscesses was performed, and postoperative pathological examination and serological tests confirmed a diagnosis of fascioliasis. However, 4 months after the surgery, follow-up CT revealed a lesion in the retroperitoneal area. Meanwhile, ultrasonography-guided percutaneous needle biopsy of the retroperitoneal lesion was performed, and a parasitic infection was suspected. Retroperitoneal metastasis of hepatic phase fascioliasis. The patient received parasitic resistance treatment with triclabendazole at a dose of 10 mg/kg/d for 2 consecutive days. After 2 courses of triclabendazole therapy, the retroperitoneal metastasis regressed to a minor lesion. To the best of our knowledge, this is the first case report of retroperitoneal metastasis of fascioliasis, aimed at helping recognize the clinical features and treatment options of this rare disease. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

  5. Image-guided fine-needle aspiration of retroperitoneal masses: The role of the cytopathologist.

    PubMed

    Mehdi, Ghazala; Maheshwari, Veena; Afzal, Sheerin; Ansari, Hena A; Ahmad, Ibne

    2013-01-01

    Retroperitoneal tumors constitute a difficult diagnostic category as they are not easily accessible. The advent of image-guided fine-needle aspiration (FNA) has resolved this problem significantly. We present a short study based on guided aspiration of retroperitoneal tumors, in which we have tried to assess the role of image-guided fine-needle aspiration cytology as a tool for pre-operative diagnosis. The study was conducted on patients diagnosed with retroperitoneal masses. FNA was performed under image guidance with the help of ultrasonography and/or computed tomography; smears were prepared and meticulously screened according to a fixed protocol. The results were analyzed to determine sensitivity, specificity, and diagnostic efficacy of cytopathological diagnosis using image-guided FNA techniques. We assessed 38 patients with retroperitoneal masses. In all cases, adequate cellular material was obtained. No major complications were encountered. Statistical analysis was carried out in 35 cases; sensitivity, specificity, and diagnostic accuracy were 100% in these cases. FNA under image guidance should be considered a first-line diagnostic approach for retroperitoneal and other abdominal tumors, although caution should be exercised in case selection. In areas where advanced tests are not available, the cytotechnologist and cytopathologist have a very important role to play in ensuring accurate diagnoses.

  6. Apatinib for advanced sarcoma: results from multiple institutions' off-label use in China.

    PubMed

    Xie, Lu; Guo, Wei; Wang, Ye; Yan, Taiqiang; Ji, Tao; Xu, Jie

    2018-04-06

    Anti-angiogenesis Tyrosine kinase inhibitors (TKIs) have been proved to show promising effects on prolonging progression-free survival (PFS) for advanced sarcoma after failure of standard multimodal Therapy. Methylsulfonic apatinib is one of those TKIs which specifically inhibits VEGFR-2. This paper summarizes the experience of three Peking University affiliated hospitals in off-label use of apatinib in the treatment of extensively pre-treated sarcoma. We retrospectively analysed files of patients with advanced sarcoma not amenable to curative treatment, who were receiving an apatinib-containing regimen between June 1, 2015 and December 1, 2016. Fifty-six patients were included: 22 osteosarcoma, 10 Ewing's sarcoma, 3 chondrosarcoma and 21 soft tissue sarcoma. With median follow-up time of 6 months (range, 0.7-18.0 m), thirty-five (62.5%) patients had partial response, and disease was stable in 11 (19.6%). The 4-month and 6-month progression-free survival rates were 46.3 and 36.5%, respectively. The median duration of response was 3.8 months (95% CI 1.9-5.6 m), with much variability among disease subtypes. The median overall survival was 9.9 months (95% CI 7.6-12.2 m). Grade 3 and 4 toxicities were observed in 8 (14.3%) patients, the most common being hypertension, pneumothorax, wound-healing problems, anorexia, and rash or desquamation. Apatinib might be effective, with a high objective response rate, in an off-label study of sarcoma patients with advanced, previously treated disease. The duration of response was consistent with reports in different subtypes of sarcomas. Prospective trials of apatinib in the treatment of selected subtypes of sarcomas are needed. Retrospectively registered in the Medical Ethics Committee of Peking University People's Hospital, Peking University Shougang Hospital and Peking University International Hospital. The trial registration number is 2017PHB176-03 and the date of registration is January 20th 2017.

  7. Type IV Ehlers-Danlos Syndrome: A Surgical Emergency? A Case of Massive Retroperitoneal Hemorrhage

    PubMed Central

    Chun, Stephen G; Pedro, Patrick; Yu, Mihae; Takanishi, Danny M

    2011-01-01

    Retroperitoneal hemorrhagic bleeding is a known manifestation of Type-IV Ehlers-Danlos Syndrome that is caused by loss-of-function mutations of the pro-alpha-1 chains of type III pro-collagen (COL3A1) resulting in vascular fragility. A number of previous reports describe futile surgical intervention for retroperitoneal bleeding in Type-IV Ehlers-Danlos Syndrome with high post-operative mortality, although the rarity of retroperitoneal bleeding associated with Type-IV Ehlers-Danlos Syndrome precludes an evidence-based approach to clinical management. We report a 23-year-old male with history of Type-IV Ehlers-Danlos Syndrome who presented with severe abdominal pain and tachycardia following an episode of vomiting. Further work-up of his abdominal pain revealed massive retroperitoneal bleeding by CT-scan of the abdomen. Given numerous cases of catastrophic injury caused by surgical intervention in Type-IV Ehlers-Danlos Syndrome, the patient was treated non-operatively, and the patient made a full recovery. This case suggests that even in cases of large retroperitoneal hemorrhages associated with Ehlers-Danlos Syndrome, it may not truly represent a surgical emergency. PMID:21966332

  8. Retroperitoneal fibrosis: case series of five patients and review of the literature.

    PubMed

    Shiber, Shacahf; Eliakim-Raz, Noa; Yair, Molad

    2016-01-01

    Chronic periaortitis (CP) is an umbrella term used to describe a group of nosologically allied conditions that include idiopathic retroperitoneal fibrosis (Ormond's disease), inflammatory abdominal aortic aneurysm, and perianeurysmal retroperitoneal fibrosis. Retroperitoneal fibrosis encompasses a range of diseases characterized by the presence of a fibro-inflammatory tissue, which usually surrounds the abdominal aorta and the iliac arteries and extends into the retroperitoneum to envelop neighboring structures-ureters. Retroperitoneal fibrosis is generally idiopathic, but can also be secondary to the use of certain drugs, malignant diseases, infections, and surgery. Here we describe a 5 years follow-up (2006-2011) of 5 patients admitted to our hospital with symptoms, laboratory, imaging and pathologic finding compatible with retroperitoneal fibrosis. We review our clinical course of our patient with respect to the literature. Copyright © 2014 Elsevier Editora Ltda. All rights reserved.

  9. Soft Tissue Sarcoma, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology.

    PubMed

    von Mehren, Margaret; Randall, R Lor; Benjamin, Robert S; Boles, Sarah; Bui, Marilyn M; Ganjoo, Kristen N; George, Suzanne; Gonzalez, Ricardo J; Heslin, Martin J; Kane, John M; Keedy, Vicki; Kim, Edward; Koon, Henry; Mayerson, Joel; McCarter, Martin; McGarry, Sean V; Meyer, Christian; Morris, Zachary S; O'Donnell, Richard J; Pappo, Alberto S; Paz, I Benjamin; Petersen, Ivy A; Pfeifer, John D; Riedel, Richard F; Ruo, Bernice; Schuetze, Scott; Tap, William D; Wayne, Jeffrey D; Bergman, Mary Anne; Scavone, Jillian L

    2018-05-01

    Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for STS provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumors, desmoid tumors, and rhabdomyosarcoma. This portion of the NCCN Guidelines discusses general principles for the diagnosis, staging, and treatment of STS of the extremities, superficial trunk, or head and neck; outlines treatment recommendations by disease stage; and reviews the evidence to support the guidelines recommendations. Copyright © 2018 by the National Comprehensive Cancer Network.

  10. Retroperitoneal abscesses in two western lowland gorillas (Gorilla gorilla gorilla).

    PubMed

    Hahn, Alicia; D'Agostino, Jennifer; Cole, Gretchen A; Raines, Jan

    2014-03-01

    This report describes two cases of retroperitoneal abscesses in female western lowland gorillas (Gorilla gorilla gorilla). Clinical symptoms included perivulvar discharge, lameness, hindlimb paresis, and general malaise. Retroperitoneal abscesses should be considered as part of a complete differential list in female gorillas with similar clinical signs.

  11. Hypercalcaemia associated with a retroperitoneal apocrine gland adenocarcinoma in a dog.

    PubMed

    Bertazzolo, W; Comazzi, S; Roccabianca, P; Caniatti, M

    2003-05-01

    A seven-year-old, entire female golden retriever was presented with a history of polyuria/polydipsia and progressive dysuria. Clinical examination, radiography and ultrasonography demonstrated urinary retention due to a large soft tissue mass in the retroperitoneal space. Laboratory findings revealed paraneoplastic hypercalcaemia. Fine-needle aspiration cytology of the mass suggested an epithelial tumour, resembling an apocrine gland carcinoma of the anal sac. Following euthanasia and necropsy, the histopathological diagnosis of the retroperitoneal mass was apocrine gland adenocarcinoma. Despite ante- and postmortem examination, no perineal or anal sac tumour was found. The retroperitoneal tumour in this case could be a very large lymph node metastasis from an occult primary apocrine carcinoma of the anal sacs, or it could represent the first case of an ectopic apocrine gland carcinoma of the retroperitoneal space in a dog.

  12. Simultaneous peritoneal and retroperitoneal splenosis mimics metastatic right adrenal mass.

    PubMed

    Hashem, Abdelwahab; Elbaset, M A; Zahran, Mohamed H; Osman, Yasser

    2018-06-05

    Right retroperitoneal splenosis is rare with few reported cases. We report, here, the case of simultaneous peritoneal and retroperitoneal splenosis mimics metastatic right adrenal mass. A 28-year-old man who had previously undergone post traumatic splenectomy at childhood and subsequently presented with an large incidental non-functioning right adrenal mass with presence of extra-hepatic peritoneal focal lesion diagnosed as metastasis by magnetic resonance imaging (MRI). Adrenalectomy with metatstectomy was performed, and both masses were identified to be splenosis. Adrenal incidentalomas (AIs) is defined as asymptomatic masses >1 cm. on cross-sectional imaging studies. AIs have significant malignant potential for masses > 6 cm. Splenosis are found most frequently in the left retroperitoneum in cases involving retroperitoneal splenosis. However, right retroperitoneal splenosis have been reported. Traditional imaging techniques cannot differentiate splenosis from malignancy. Large right adrenal incidentalomas present with other abdominal, peritoneal masses could be splenosis in patient following post-traumatic splenectomy. Copyright © 2018 The Author(s). Published by Elsevier Ltd.. All rights reserved.

  13. Primary retroperitoneal seminoma - embryology, histopathology and treatment particularities.

    PubMed

    Gîngu, Constantin Virgil; Mihai, Mihaela; Baston, Cătălin; Crăsneanu, Mugurel Alexandru; Dick, Alexandru Vladimir; Olaru, Vlad; Sinescu, Ioanel

    2016-01-01

    Retroperitoneal seminoma is a very rare form of cancer, with embryological origin represented by primordial germ cells from the urogenital ridges left behind during the fetal development. Extragenital germ cell tumors can also occur in the mediastinum or the pineal gland. The aim of this paper is to outline the particularities and draw embryological, histopatological and treatment conclusions regarding extragonadal germ cell tumors. A 43-year-old patient without any additional pathology was admitted for anemia of unknown etiology. The clinical examination revealed through deep abdominal palpation a mass in the left flank, and normal testes. Thoraco-abdomino-pelvic computed tomography (CT) scan showed a large retroperitoneal tumor adjacent to the great vessels in the left lumbo-iliac region. The blood work revealed just a low hemoglobin and hematocrit. With the established diagnosis of retroperitoneal tumor, radical surgical removal was decided. During the surgery, we were required to dissect a large solid encapsulated tumor mass from the aorta and the common iliac artery, starting at the renal pedicle all the way to the left iliac bifurcation. The surgical access was obtained through a transperitoneal left subcostal incision prolonged pararectally. Histopathological and immunohistochemical studies revealed a seminoma of the usual type. After the histological findings, the patient's tumor markers were investigated (LDH - lactate dehydrogenase, βHCG - beta-human chorionic gonadotropin, αFP - alpha-fetoprotein), all values being within normal ranges. In addition, the left testicle was thoroughly reexamined, clinically, through ultrasound and magnetic resonance imaging (MRI) scans, and no abnormalities were observed. After the surgery, the patient followed three courses of chemotherapy (BEP - Bleomycin, Etoposide and Cisplatin). The CT scan done 24 months after surgery found no signs of local or distant tumor recurrence. The patient entered a follow-up schedule

  14. Mortality risk factor analysis in colonic perforation: would retroperitoneal contamination increase mortality in colonic perforation?

    PubMed

    Yoo, Ri Na; Kye, Bong-Hyeon; Kim, Gun; Kim, Hyung Jin; Cho, Hyeon-Min

    2017-10-01

    Colonic perforation is a lethal condition presenting high morbidity and mortality in spite of urgent surgical treatment. This study investigated the surgical outcome of patients with colonic perforation associated with retroperitoneal contamination. Retrospective analysis was performed for 30 patients diagnosed with colonic perforation caused by either inflammation or ischemia who underwent urgent surgical treatment in our facility from January 2005 to December 2014. Patient characteristics were analyzed to find risk factors correlated with increased postoperative mortality. Using the Physiological and Operative Severity Score for the Enumeration of Mortality and Morbidity (POSSUM) audit system, the mortality and morbidity rates were estimated to verify the surgical outcomes. Patients with retroperitoneal contamination, defined by the presence of retroperitoneal air in the preoperative abdominopelvic CT, were compared to those without retroperitoneal contamination. Eight out of 30 patients (26.7%) with colonic perforation had died after urgent surgical treatment. Factors associated with mortality included age, American Society of Anesthesiologists (ASA) physical status classification, and the ischemic cause of colonic perforation. Three out of 6 patients (50%) who presented retroperitoneal contamination were deceased. Although the patients with retroperitoneal contamination did not show significant increase in the mortality rate, they showed significantly higher ASA physical status classification than those without retroperitoneal contamination. The mortality rate predicted from Portsmouth POSSUM was higher in the patients with retroperitoneal contamination. Patients presenting colonic perforation along with retroperitoneal contamination demonstrated severe comorbidity. However, retroperitoneal contamination was not found to be correlated with the mortality rate.

  15. Percutaneous Radiofrequency Ablation for Treatment of Recurrent Retroperitoneal Liposarcoma

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Keil, Sebastian, E-mail: keil@rad.rwth-aachen.de; Bruners, Philipp; Brehmer, Bernhard

    2008-07-15

    Percutaneous CT-guided radiofrequency ablation (RFA) is becoming more and more established in the treatment of various neoplasms, including retroperitoneal tumors of the kidneys and the adrenal glands. We report the case of RFA in a patient suffering from the third relapse of a retroperitoneal liposarcoma in the left psoas muscle. After repeated surgical resection and supportive radiation therapy of a primary retroperitoneal liposarcoma and two surgically treated recurrences, including replacement of the ureter by a fraction of the ileum, there was no option for further surgery. Thus, we considered RFA as the most suitable treatment option. Monopolar RFA was performedmore » in a single session with a 2-cm umbrella-shaped LeVeen probe. During a 27-month follow-up period the patient remained free of tumor.« less

  16. [Retroperitoneal lymphadenectomy and disorders of ejaculation].

    PubMed

    Deiana, G; Ranieri, A; Micheli, E; Peracchia, G; Canclini, L P; Sironi, D; Levorato, C A; Lembo, A

    1999-09-01

    Retrograde ejaculation is a frequent and permanent complication after bilateral retroperitoneal lymphadenectomy (RPLND). Seminal emission and ejaculation are primarily under sympathetic control. Several studies after RPLND in patients with nonseminomatous testis cancer proved the role of preservation of the efferent fibers originating from the lumbar sympathetic ganglia. Based on the results of anatomical studies, a modified unilateral operative technique and nerve-sparing approach permit to preserve normal anterograde ejaculation without reduction of long-term survival.

  17. Retroperitoneal Pseudoaneurysm Mimicking Ureteral Calculus: Pitfalls in Diagnosis.

    PubMed

    Kalabin, Aleksandr; Mani, Vishnu R; Dinesh, Anant; Landa, Marina; Davis-Joseph, Brian

    2017-10-08

    Arterial aneurysms (AA) can be classified as true aneurysms, characterized by the persistence of all three layers of the arterial wall with progressive dilation and wall thinning; arterial pseudoaneurysms (APAs) are characterized by a tear in the vessel wall and a periarterial hematoma formation. They could occur due to a visceral, retroperitoneal, or peripheral origin. Most AA/APA are usually found incidentally, and it is imperative to be vigilant in order to diagnose and manage them due to their potentially life-threatening complications. We present a case of a 35-year-old woman presenting with right-sided abdominal pain mimicking renal colic with an initial misdiagnosis of ureteral calculus. Post-cystoscopy, a misdiagnosis was confirmed, and subsequently, the patient had a right retroperitoneal mass excision. The histopathology report concluded the calcified retroperitoneal mass to be pseudoaneurysm. Such pitfalls in diagnosis are essential to be shared with the larger medical community for increased vigilance and optimal management of pseudoaneurysms.

  18. Case report: retroperitoneal fibrosis simulating local relapse of sarcomatoid renal cell carcinoma.

    PubMed

    Esquena, Salvador; Abascal, José Maria; Trilla, Enrique; De Torres, Inés; Morote, Juan

    2006-01-01

    Generally, retroperitoneal fibrosis is an idiopathic process that envelopes and displaces ureters, causing hydronefrosis and renal failure. CT scan is the best choice for diagnosis. Other aetiologies described are malignancies, drugs, aorta aneurisms and immunological or rheumatological diseases. A 53-year-old male with hypertension and diabetes was operated on radical nephrectomy for renal mass. Pathological examination showed sarcomatoid renal cell carcinoma, Fürhman 3 grade, pT2 N0. Within 6 months of surgery, control CT scan demonstrated a left retroperitoneal mass, without separation with pancreas queue and spleen hilium, suggesting local relapse. Resection of the mass with splenectomy and partial pancreatectomy en bloc was performed. Microscopic evaluation revealed a dense collagenic tissue with a prominent inflammatory infiltrate, and the immunohistochemical study was negative for cytokeratin AE1-AE3. There was no evidence of malignancy in the histological examination. All these findings aided to diagnose a retroperitoneal fibrosis. Sometimes retroperitoneal fibrosis can simulate or is associated to malignancies. Presentation of a retroperitoneal fibrosis simulating local relapse of sarcomatoid renal cell carcinoma has not been previously reported in the English literature.

  19. Retroperitoneal Angiomatoid Fibrous Histiocytoma Presenting as a Recurrent Spontaneous Retroperitoneal Hemorrhage in a 9-Year-Old Boy.

    PubMed

    Slack, Jonathan C; Sanchez-Glanville, Carlos; Steele, MacGregor; Wong, Andrew L; Bründler, Marie-Anne

    2018-05-01

    Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that typically presents in children and young adults. Occurrence outside of the extremities and the head and neck region is exceedingly rare. We report the case of a 9-year-old boy who presented with recurrent retroperitoneal hemorrhage initially thought to be a manifestation of an underlying bleeding disorder. After comprehensive diagnostic work-up, including multiple negative biopsies, the patient underwent surgical resection of an extensively hemorrhagic intramuscular mass and to date remains well. Pathologic examination confirmed AFH with EWSR1 gene rearrangement. This first documented report of an AFH in a retroperitoneal location in a child highlights the diagnostic difficulties and clinical challenges of AFH arising in an atypical location.

  20. Robotic retroperitoneal partial nephrectomy: a step-by-step guide.

    PubMed

    Ghani, Khurshid R; Porter, James; Menon, Mani; Rogers, Craig

    2014-08-01

    To describe a step-by-step guide for successful implementation of the retroperitoneal approach to robotic partial nephrectomy (RPN) PATIENTS AND METHODS: The patient is placed in the flank position and the table fully flexed to increase the space between the 12th rib and iliac crest. Access to the retroperitoneal space is obtained using a balloon-dilating device. Ports include a 12-mm camera port, two 8-mm robotic ports and a 12-mm assistant port placed in the anterior axillary line cephalad to the anterior superior iliac spine, and 7-8 cm caudal to the ipsilateral robotic port. Positioning and port placement strategies for successful technique include: (i) Docking robot directly over the patient's head parallel to the spine; (ii) incision for camera port ≈1.9 cm (1 fingerbreadth) above the iliac crest, lateral to the triangle of Petit; (iii) Seldinger technique insertion of kidney-shaped balloon dilator into retroperitoneal space; (iv) Maximising distance between all ports; (v) Ensuring camera arm is placed in the outer part of the 'sweet spot'. The retroperitoneal approach to RPN permits direct access to the renal hilum, no need for bowel mobilisation and excellent visualisation of posteriorly located tumours. © 2014 The Authors. BJU International © 2014 BJU International.

  1. The retroperitoneal interfascial planes: current overview and future perspectives.

    PubMed

    Ishikawa, Kazuo; Nakao, Shota; Nakamuro, Makoto; Huang, Tai-Ping; Nakano, Hiroshi

    2016-07-01

    Recently, the concept of interfascial planes has become the prevalent theory among radiologists for understanding the retroperitoneal anatomy, having replaced the classic tricompartmental theory. However, it is a little known fact that the concept remains incomplete and includes embryological errors, which have been revised on the basis of our microscopic study. We believe that the concept not only provides a much clearer understanding of the retroperitoneal anatomy, but it also allows further development for diagnosis and treatment of retroperitoneal injuries and diseases, should it become an accomplished theory. We explain the history and outline of the concept of interfascial planes, correct common misunderstandings about the concept, explain the unconsciously applied therapeutic procedures based on the concept, and present future perspectives of the concept using our published and unpublished data. This knowledge could be essential to acute care physicians and surgeons sometime soon.

  2. Retroperitoneal Pseudoaneurysm Mimicking Ureteral Calculus: Pitfalls in Diagnosis

    PubMed Central

    Kalabin, Aleksandr; Dinesh, Anant; Landa, Marina; Davis-Joseph, Brian

    2017-01-01

    Arterial aneurysms (AA) can be classified as true aneurysms, characterized by the persistence of all three layers of the arterial wall with progressive dilation and wall thinning; arterial pseudoaneurysms (APAs) are characterized by a tear in the vessel wall and a periarterial hematoma formation. They could occur due to a visceral, retroperitoneal, or peripheral origin. Most AA/APA are usually found incidentally, and it is imperative to be vigilant in order to diagnose and manage them due to their potentially life-threatening complications. We present a case of a 35-year-old woman presenting with right-sided abdominal pain mimicking renal colic with an initial misdiagnosis of ureteral calculus. Post-cystoscopy, a misdiagnosis was confirmed, and subsequently, the patient had a right retroperitoneal mass excision. The histopathology report concluded the calcified retroperitoneal mass to be pseudoaneurysm. Such pitfalls in diagnosis are essential to be shared with the larger medical community for increased vigilance and optimal management of pseudoaneurysms. PMID:29226048

  3. Giant retroperitoneal liposarcoma with mixed histological pattern: a rare presentation and literature review.

    PubMed

    Salemis, Nikolaos S; Tsiambas, Evangelos; Karameris, Andreas; Tsohataridis, Efstathios

    2009-01-01

    Retroperitoneal liposarcoma is a rare tumor that may grow to a considerable size before causing clinical symptoms. Mixed-type retroperitoneal liposarcoma is a very rare clinical entity. We herein describe a rare case of a 54-year-old female who was diagnosed with a giant retroperitoneal liposarcoma arising from the right perinephric space. Radical nephrectomy and right salpingo-oophorectomy were necessary to achieve complete tumor excision. Histological examination revealed a mixed-type liposarcoma consisting of well-differentiated and pleomorphic elements and deep invasion into the renal parenchyma, which is quite infrequent. The patient was well and disease-free 12 months after surgery. Mixed-type retroperitoneal liposarcoma is a rare tumor. Aggressive surgical resection of the tumor together with adjacent structures, if necessary, is the mainstay of treatment.

  4. Ewing sarcoma: a chronicle of molecular pathogenesis.

    PubMed

    Kim, Sang Kyum; Park, Yong-Koo

    2016-09-01

    Sarcomas have traditionally been classified according to their chromosomal alterations regardless of whether they accompany simple or complex genetic changes. Ewing sarcoma, a classic small round cell bone tumor, is a well-known mesenchymal malignancy that results from simple sarcoma-specific genetic alterations. The genetic alterations are translocations between genes of the TET/FET family (TLS/FUS, EWSR1, and TAF15) and genes of the E26 transformation-specific (ETS) family. In this review, we intend to summarize a chronicle of molecular findings of Ewing sarcoma including recent advances and explain resultant molecular pathogenesis. Copyright © 2016 Elsevier Inc. All rights reserved.

  5. Retroperitoneal versus direct femoral artery approach for thoracic endovascular aortic repair access: a case-control study.

    PubMed

    Etezadi, Vahid; Katzen, Barry T; Benenati, James F; Alehashemi, Sara; Tsoukas, Athanassios I; Puente, Orlando A

    2011-04-01

    Many individuals who are candidates for thoracic endovascular aortic repair (TEVAR) are found to have iliac artery anatomy and/or disease that preclude transfemoral endovascular access and require retroperitoneal surgical approach through more proximal arteries. This relatively more invasive technique could potentially affect the procedural outcomes. This study compares the retroperitoneal with transfemoral access used for TEVAR in a single center. In this study, 133 consecutive patients (96 men; mean age ± SD: 69.5 ± 14.7 years) who underwent TEVAR between 1994 and 2009 in a single center were retrospectively evaluated. The type of endovascular access was identified in all the patients. The basic demographics, access method, endograft type, 30-day morbidity and mortality rates, as well as procedure recordings including fluoroscopic and procedure duration, estimated blood loss, and duration of hospitalization were compared between the TEVAR procedures performed using a surgical retroperitoneal approach and those using the standard femoral access. Retroperitoneal access was used in 19 (14.3%; 13 women; mean age ± SD: 71 ± 12.2 years) and direct femoral access in 114 (85.7%; 24 women; mean age ± SD: 69 ± 15.4 years) patients. Two of the retroperitoneal accesses were obtained after failure of femoral approach. Techniques that were used included iliac artery conduit (seven patients), aortic artery conduit (eight patients), aortobifemoral artery graft conduit (one patient), and direct sheath introduction through the distal aorta (two patients) or common iliac artery (one patient). Retroperitoneal approach was used more frequently in women (35%) as compared with men (6%) (p = 0.0001). In all, 79% of the retroperitoneal approaches were associated with use of delivery sheath sizes larger than 24F (p = 0.049). TEVAR technical success was 100% with retroperitoneal and 97.3% with femoral access (p > 0.05). Thirty-day mortality rates were 0% and 8.8% and the rates of

  6. Ewing sarcoma

    MedlinePlus

    Bone cancer - Ewing sarcoma; Ewing family of tumors; Primitive neuroectodermal tumors (PNET); Bone neoplasm - Ewing sarcoma ... adulthood. But it usually develops during puberty, when bones are growing rapidly. It is more common in ...

  7. IgG4-related retroperitoneal fibrosis: a newly characterized disease.

    PubMed

    Lian, Linjuan; Wang, Cong; Tian, Jian-Li

    2016-11-01

    Retroperitoneal fibrosis (RPF) is a rare disease characterized by chronic, nonspecific inflammatory and sclerotic or fibrotic tissue in the periaortic or periiliac retroperitoneum that encases adjacent structures. There will be a series of clinical manifestations once the proliferated fibrous tissues encase the abdominal aorta, iliac arteries and urinary duct. RPF is generally divided into two types: idiopathic retroperitoneal fibrosis (IRPF) without identified pathogenesis, making up about two-thirds of cases, and secondary retroperitoneal fibrosis. Recent studies on Immunoglobulin G4-related disease (IgG4-RD) reveal that abundant infiltration of IgG4 positive plasma cells is found in biopsies on the mass of RPF of some IRPF patients, which is identified as one spectrum of IgG4-RD and is named IgG4-related RPF. IgG4-related RPF is often misdiagnosed as retroperitoneal visceral malignancy and is treated with surgery. In addition, because of its good response to glucocorticoid, early detection and treatment is important. We review the definition, epidemiology, clinical features, diagnostic criteria, treatment and prognosis of IgG4-related RPF in this article to raise awareness of this newly characterized disease. © 2016 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.

  8. Pancreas transplantation: Advantages of a retroperitoneal graft position.

    PubMed

    Ferrer, Joana; Molina, Víctor; Rull, Ramón; López-Boado, Miguel Ángel; Sánchez, Santiago; García, Rocío; Ricart, Ma José; Ventura-Aguiar, Pedro; García-Criado, Ángeles; Esmatjes, Enric; Fuster, Josep; Garcia-Valdecasas, Juan Carlos

    2017-11-01

    In the 50 years since the first pancreas transplant performed at the University of Minnesota, the surgical techniques employed have undergone many modifications. Techniques such as retroperitoneal graft placement have further improved the ability to reproduce the physiology of the «native» pancreas. We herein present our experience of a modified technique for pancreatic transplant, with the organ placed into a fully retroperitoneal position with systemic venous and enteric drainage of the graft by duodeno-duodenostomy. All pancreas transplantations performed between May 2016 and January 2017 were prospectively entered into our transplant database and retrospectively analyzed. A total of 10 transplants were performed using the retroperitoneal technique (6 men: median age of 41 years [IQR 36-54]). Median cold ischemia times was 10,30h [IQR 5,30-12,10]. The preservation solution used was Celsior (n=7), IGL-1 (n=2), and UW (n=1). No complications related to the new surgical technique were identified. In one patient, transplantectomy at 12h was performed due to graft thrombosis, probably related to ischemic conditions from a donor with prolonged cardio-respiratory arrest. Another procedure was aborted without completing the graft implant due to an intraoperative immediate arterial thrombosis in a patient with severe iliac atheromatosis. No primary pancreas non-function occurred in the remaining 8patients. The median hospital stay was 13,50 days [IQR 10-27]. Retroperitoneal graft placement appears feasible with easy access for dissection the vascular site; comfortable technical vascular reconstruction; and a decreased risk of intestinal obstruction by separation of the small bowel from the pancreas graft. Copyright © 2017 AEC. Publicado por Elsevier España, S.L.U. All rights reserved.

  9. Surgical anatomy of the retroperitoneal spaces--part I: embryogenesis and anatomy.

    PubMed

    Mirilas, Petros; Skandalakis, John E

    2009-11-01

    Embryologically, the retroperitoneal (extraperitoneal) connective tissue includes three strata, which respectively form the internal fascia lining of the body wall, the renal fascia, and the covering of the gastrointestinal viscera. All organs, vessels, and nerves, that lie on the posterior abdominal wall, along with their tissues and surrounding connective and fascial planes, are collectively referred to as the retroperitoneum. The retroperitoneal space is the area of the posterior abdominal wall that is located between the parietal peritoneum and the fascia. Within the greater retroperitoneal space, there are also several small spaces, or subcompartments. Loose connective tissue and fat surround the anatomic entities, and, to a variable degree, occupy the subcompartments. The multilaminar thoracolumbar (lumbodorsal) fascia begins at the occipital area and terminates at the sacrum.

  10. Postirradiation soft tissue sarcoma occurring in breast cancer patients: report of seven cases and results of combination chemotherapy

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Kuten, A.; Sapir, D.; Cohen, Y.

    1985-03-01

    Seven cases of soft tissue sarcoma developing after primary or postoperative radiotherapy for breast carcinoma are reported. The sarcomas occurred within the irradiated volume, after a latent period of 4-26 years. These cases conform well to established criteria for the diagnosis of radiation-induced sarcoma. Chemotherapy, consisting of the four-drug combination CYVADIC (cyclophosphamide, vincristine, adriamycin, DTIC) was employed in six of the seven patients. Only two of them achieved partial remission, lasting only 2 and 3 months, respectively. The effectiveness of adriamycin-containing chemotherapy regimens in soft tissue sarcomas as well as the remote hazard of radiation-related sarcoma in primary or postoperativemore » breast irradiation are discussed.« less

  11. Two cases of retroperitoneal haematoma caused by interaction between antibiotics and warfarin

    PubMed Central

    Phillips, S; Barr, A; Wilson, E; Rockall, T A; Stebbing, J F

    2006-01-01

    Several commonly prescribed antibiotics are known to interact with warfarin, increasing its anticoagulant effect by different mechanisms. Retroperitoneal bleeding with consequent haematoma is recognised as a complication of over‐anticoagulation. Consequences, which are potentially fatal, include hypovolaemic shock and compression of retroperitoneal structures such as the ureter and inferior vena cava. PMID:16373793

  12. Primary osteogenic sarcoma of the breast

    PubMed Central

    Ogundiran, Temidayo O; Ademola, Samuel A; Oluwatosin, Odunayo M; Akang, Effiong E; Adebamowo, Clement A

    2006-01-01

    Background Primary extra-osseous osteogenic sarcomas have been reported in many tissues of the body but their occurrence in the breast is extremely rare. It can arise as a result of osseous metaplasia in a pre-existing benign or malignant neoplasm of the breast or as non-phylloides sarcoma from the soft tissue of a previously normal breast. Case presentation A 40 year-old Nigerian woman was clinically diagnosed to have carcinoma of the left breast. The histology report of core-needle biopsy of the mass showed a malignant neoplasm comprising islands of chondroblastic and osteoblastic stromal cells. This report changed the diagnosis from carcinoma to osteogenic sarcoma of the breast. She had a left modified radical mastectomy, however there was significant post surgery skin deficit. A latissimus dorsi musculocutaneous flap was used to cover the anterior chest wall defect. Sections from the mastectomy specimen confirmed the diagnosis of osteogenic sarcoma. She died six months after mastectomy. Conclusion A diagnosis of osteogenic sarcoma of the breast was made based on histology report and after excluding an osteogenic sarcoma arising from underlying ribs and sternum. This is the second documented case of primary osteogenic sarcoma of the breast coming from Nigeria PMID:17156481

  13. Mini-open lateral retroperitoneal lumbar spine approach using psoas muscle retraction technique. Technical report and initial results on six patients.

    PubMed

    Aghayev, Kamran; Vrionis, Frank D

    2013-09-01

    The main aim of this paper was to report reproducible method of lumbar spine access via a lateral retroperitoneal route. The authors conducted a retrospective analysis of the technical aspects and clinical outcomes of six patients who underwent lateral multilevel retroperitoneal interbody fusion with psoas muscle retraction technique. The main goal was to develop a simple and reproducible technique to avoid injury to the lumbar plexus. Six patients were operated at 15 levels using psoas muscle retraction technique. All patients reported improvement in back pain and radiculopathy after the surgery. The only procedure-related transient complication was weakness and pain on hip flexion that resolved by the first follow-up visit. Psoas retraction technique is a reliable technique for lateral access to the lumbar spine and may avoid some of the complications related to traditional minimally invasive transpsoas approach.

  14. Sorafenib in Treating Patients With Soft Tissue Sarcomas (Extremity Sarcoma Closed to Entry as of 5/30/07)

    ClinicalTrials.gov

    2014-04-01

    Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Osteosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Osteosarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  15. LAPAROSCOPIC MANAGEMENT OF RETROPERITONEAL INJURIES IN PENETRATING ABDOMINAL INJURIES.

    PubMed

    Mosai, F

    2017-09-01

    Laparoscopy in penetrating abdominal injuries is now accepted and practiced in many modern trauma centres. However its role in evaluating and managing retroperitoneal injuries is not yet well established. The aim of this study was to document our experience in using laparoscopy in a setting of penetrating abdominal injuries with suspected retroperitoneal injury in haemodynamically stable patients. A retrospective descriptive study of prospectively collected data from a trauma unit at Dr George Mukhari Academic Hospital (DGMAH) was done. All haemodynamically stable patients with penetrating abdominal injury who were offered laparoscopy from January 2012 to December 2015 were reviewed and those who met the inclusion criteria were analysed. A total of 284 patients with penetrating abdominal injuries were reviewed and 56 met the inclusion criteria and were analysed. The median age was 30.8 years (15-60 years) and males constituted 87.5% of the study population. The most common mechanism of injury was penetrating stab wounds (62.5%). Forty-five patients (80.3%) were managed laparoscopically, of these n=16 (28.5%) had retroperitoneal injuries that required surgical intervention. The most commonly injured organ was the colon (19.6%). The conversion rate was 19.6% with most common indication for conversion been active bleeding (14%). The complication rate was 7.14% (N=4) and were all Clavien-Dindo grade 3. There were no recorded missed injuries and no mortality. The positive outcomes documented in this study with no missed injuries and absence of mortality suggests that laparoscopy is a feasible option in managing stable patients with suspected retroperitoneal injuries.

  16. Robotic posterior retroperitoneal adrenalectomy.

    PubMed

    Okoh, Alexis Kofi; Yigitbas, Hakan; Berber, Eren

    2015-09-01

    Since its initial description by Mercan et al. laparoscopic posterior retroperitoneal (PR) adrenalectomy has served as an alternaltive to the transabdominal (TL) approach for the treatment of adrenal pathologies. Robotic adrenal surgery has been reported to improve surgeon ergonomics and facilitate dissection. In patients with bilateral adrenal masses, PR adrenalectomy may be the approach of choice. We herein describe the technique, discuss its limitations and present a critical review of the current literature. © 2015 Wiley Periodicals, Inc.

  17. Presentation of idiopathic retroperitoneal fibrosis at a young age: A rare case report.

    PubMed

    Minocha, Priyanka; Setia, Ankur

    2016-11-01

    Abdominal pain is a very common symptom in all age groups but retroperitoneal fibrosis is a rare differential diagnosis suspected in young patients presenting with nonspecific abdominal pain and symptoms of obstructive uropathy. Presented here is a case of a 16-year-old boy who presented with symptoms of persistent abdominal pain and a previous history of swelling in the left leg. A computed tomography (CT) scan suggested retroperitoneal fibrosis and an exploratory laparotomy and histopathological examination were performed for definitive diagnosis. This case report is intended to promote awareness of retroperitoneal fibrosis in young patients among health care providers.

  18. Penile Mondor's disease after anterolateral retroperitoneal approach for lumbar fracture.

    PubMed

    Dobran, Mauro; Benigni, Roberta; Nasi, Davide; Cantoro, Daniele

    2017-11-01

    This is a rare case of thrombosis of the dorsal vein of the penis (Mondor's disease) occurred after an anterior-lateral retroperitoneal approach for a vertebral stabilisation in thoracolumbar vertebral fracture. Potential causes are traumatism, neoplasms, excessive sexual activity or abstinence. Although penile Mondor's disease is a clinical diagnosis, ultrasound imaging is the gold standard to confirm it. In the reported case, 1 week after neurosurgical retroperitoneal procedure of vertebral stabilisation, the patient complained of a painful cord-like mass midshaft of penis. The diagnosis was made by clinical evaluation and ultrasound images. After 2 weeks of therapy with enoxaparin sodium, the patient recovered. The authors report this case evaluating the possible correlation between the anterior-lateral retroperitoneal approach and the development of the rare Mondor's disease. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  19. Retroperitoneal Haematoma in a Patient with Dengue Haemorrhagic Fever: A Rare Case Report.

    PubMed

    Singh, Jasminder; Singh, Harpreet; Sukhija, Gagandeep; Jagota, Ruchi; Bala, Saroj

    2016-11-01

    Dengue Haemorrhagic Fever (DHF) has diverse manifestations ranging from asymptomatic petechial skin haemorrhages to life threatening cerebral, pulmonary, gastrointestinal and genitourinary haemorrhages. However, the association of spontaneous retroperitoneal haematomas with DHF is not well documented in literature. We report a rare case of spontaneous retroperitoneal haematoma complicating DHF.

  20. Enoxaparin-induced spontaneous massive retroperitoneal hematoma with fatal outcome.

    PubMed

    Salemis, Nikolaos S; Oikonomakis, Ioannis; Lagoudianakis, Emanuel; Boubousis, Georgios; Tsakalakis, Christos; Sourlas, Sotirios; Gourgiotis, Stavros

    2014-12-01

    Spontaneous retroperitoneal hematoma (SRH) is a severe and potentially fatal complication of anticoagulation therapy. We describe a case of fatal spontaneous massive retroperitoneal hematoma in a female patient receiving bridging therapy with enoxaparin for atrial fibrillation. Physicians should be cautious when prescribing enoxaparin in elderly patients, in patients with impaired renal function, and in patients receiving concomitant oral anticoagulants. Emergency physicians should always consider SRH in the differential diagnosis in patients under enoxaparin therapy presenting with abdominal pain. Computed tomographic scan is the imaging modality of choice for evaluating SRH. Early diagnosis and aggressive treatment are of paramount importance as SRH is associated with high mortality and morbidity rates.

  1. Soft Tissue Sarcoma

    MedlinePlus

    ... begins in the lining of blood vessels, while liposarcoma arises from fat cells. Some types of soft ... sarcoma Gastrointestinal stromal tumor (GIST) Kaposi's sarcoma Leiomyosarcoma Liposarcoma Malignant peripheral nerve sheath tumor Myxofibrosarcoma Rhabdomyosarcoma Solitary ...

  2. Retroperitoneal duplication cyst with a fistulous tract to the vagina: a case report.

    PubMed

    Filmar, Gilad A; Lotze, Peter M; Fisher, Hilaire W

    2012-01-01

    To describe a rare case of a retroperitoneal duplication cyst that fistulized to the vagina. Case description and discussion of a patient found to have an intestinal duplication cyst. A patient presented for a laparoscopic hysterectomy because of menorrhagia and a fibroid uterus. She also complained of recurrent urinary tract infections (UTIs) and a vaginal discharge. A retroperitoneal intestinal duplication cyst that fistulized to the vagina and caused her recurrent UTIs was identified. Surgical resection of the cyst resolved her complaint of recurrent UTIs. Retroperitoneal intestinal duplication cysts are rare congenital anomalies with vague clinical manifestations. The finding of a fistulous communication to the vagina originating from such a structure can be associated with recurrent UTIs.

  3. Epidemiology and therapies for metastatic sarcoma

    PubMed Central

    Amankwah, Ernest K; Conley, Anthony P; Reed, Damon R

    2013-01-01

    Sarcomas are cancers arising from the mesenchymal layer that affect children, adolescents, young adults, and adults. Although most sarcomas are localized, many display a remarkable predilection for metastasis to the lungs, liver, bones, subcutaneous tissue, and lymph nodes. Additionally, many sarcoma patients presenting initially with localized disease may relapse at metastatic sites. While localized sarcomas can often be cured through surgery and often radiation, controversies exist over optimal management of patients with metastatic sarcoma. Combinations of chemotherapy are the most effective in many settings, and many promising new agents are under active investigation or are being explored in preclinical models. Metastatic sarcomas are excellent candidates for novel approaches with additional agents as they have demonstrated chemosensitivity and affect a portion of the population that is motivated toward curative therapy. In this paper, we provide an overview on the common sarcomas of childhood (rhabdomyosarcoma), adolescence, and young adults (osteosarcoma, Ewing sarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumor) and older adults (leiomyosarcoma, liposarcoma, and undifferentiated high grade sarcoma) in terms of the epidemiology, current therapy, promising therapeutic directions and outcome with a focus on metastatic disease. Potential advances in terms of promising therapy and biologic insights may lead to more effective and safer therapies; however, more clinical trials and research are needed for patients with metastatic sarcoma. PMID:23700373

  4. Examine the patient not the hernia: identification of an asymptomatic giant primary retroperitoneal pseudocyst. A case report and literature review

    PubMed Central

    Karim, Lawen; Larkin, David; Sadat, Mohamed

    2016-01-01

    We present the case of a 70-year-old man with a giant right-sided retroperitoneal pseudocyst, confirmed histologically after resection to be benign with appearances dissimilar to pancreatic and adrenal tissue. The cyst was noted incidentally on table at the time of laparoscopic surgery. Retroperitoneal pseudocysts most commonly arise from the pancreas and adrenal glands occurring as a result of an inflammatory process. Primary retroperitoneal pseudocysts are a rare entity. This case highlights the importance of examining the patient thoroughly and not focusing on the obvious. The mass was not palpated on initial review prior to listing for surgery, and the patient was asymptomatic from the mass. PMID:27190202

  5. AIDS-related Kaposi sarcoma: findings on thallium-201 scintigraphy

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Lee, V.W.; Rosen, M.P.; Baum, A.

    1988-12-01

    No simple, noninvasive method is available for evaluating extracutaneous Kaposi sarcoma in AIDS patients or for following the tumor's response to treatment. We report our preliminary experience with thallium-201 scintigraphy in nine AIDS patients with proved Kaposi sarcoma. Eight of the nine had abnormal uptake of the radionuclide in skin, lymph nodes, oral cavity, vagina, and lungs. Only four of the nine had cutaneous Kaposi sarcoma at the time of scanning. All cutaneous and mucosal lesions were thallium avid. Two of the six patients with thallium-avid nodes underwent nodal biopsy. Both biopsies confirmed the diagnosis of Kaposi sarcoma. Cutaneous Kaposimore » sarcoma developed later in one of these patients, showing the efficacy of thallium scintigraphy for the early detection of extracutaneous lesions. These preliminary results show thallium avidity in Kaposi sarcoma involving the skin and various extracutaneous sites (lymph nodes, lung, mucosa, and vagina). Thallium scintigraphy is a potentially useful procedure for detecting extracutaneous Kaposi sarcoma in AIDS patients.« less

  6. [Demographic Analysis of Patients with Osteosarcoma, Chonddrosarcoma, Ewing's Sarcoma from one Sarcoma Center in Switzerland].

    PubMed

    Hodel, Sandro; Seeli, Franziska; Fuchs, Bruno

    2015-06-17

    Retrospective analysis of presentation, diagnosis and outcome of patients with osteosarcoma, chondrosarcoma and Ewing's sarcoma was performed for a single Sarcoma Center in Zurich at the University Hospital Balgrist. 201 patients were included. Overall survival at five and ten years were 74 ± 6%, 69 ± 7% for osteosarcoma (n = 85, since 2000), 85 ± 7%, 80 ± 9% for Ewing's sarcoma (n = 43, since 1990) and 86 ± 5%, 78 ± 9% for chondrosarcoma (n = 73, since 2000). The here presented overall survival rates from a single Sarcoma Center in Switzerland appear to be equivalent to other large international monocenter studies. The presentation and epidemiology of these patients are in accordance with large multicenter epidemiological studies. A nationwide sarcoma database (SwissSARCOS; www.sarcoma.ch) seems indispensable for more detailed analysis and quality management in such rare diseases.

  7. [KAPOSI'S SARCOMA OF THE VULVA].

    PubMed

    Chokoeva, Aa; Tchernev, G; Wollina, U

    2015-01-01

    Kaposi's sarcoma represents multiple idiopathic hemorrhagic sarcoma--a mesenchymal tumor that affects the blood and lymph vessels. Its performance is associated with an infection with human herpes virus type 8--the so called KSHV (Kaposi's sarcoma -associated virus), and with the human immunodeficiency virus. Kaposi's sarcoma is considered as a typical clinical manifestation in male homosexuals suffering from acquired immune deficiency syndrome (AIDS), while his performance in HIV-positive women is unusual, with a ratio of men to women--10-15: 1. Vulvar localization is much rarer. It is up to 5 times more frequent in HIV- positive patients. It is clinically represented in most of the cases by the clinical picture of nonspecific tumor mass. Biopsy and further virological testing for establishing KSHV in lesional tissue is essential for confirming the diagnosis. Serological testing for HIV/AIDS in affected patients is required. Local treatment includes surgical excision of solitary lesions, cryotherapy as well as radiotherapy. The use of interferon alpha resulted in complete remission in approximately 40% of the affected patients. New trends in treatment tend to be pathogenetically directed as in the process of studies to date are inhibitors of angiogenesis. Due to the rarity of the occurrence, non-specific clinical picture and histological findings, Kaposi's sarcoma should be considered in the differential diagnosis of tumor masses with vulvar localization, especially in HIV-positive patients.

  8. Retroperitoneal extraadrenal paraganglioma report of two cases.

    PubMed

    Becheanu, G; Laky, D

    1997-01-01

    Two cases with retroperitoneal tumours affecting young women who cannot undergo surgery were investigated by biopsy and needle sampling. One of these cases presented a mainly alveolar histologic pattern and was easily diagnosed by immunohistochemical means, as well as a chromaffin paraganglioma. We discussed different diagnoses and approaches in the literature.

  9. Laparoscopic management of retroperitoneal injuries from penetrating abdominal trauma in haemodynamically stable patients.

    PubMed

    Koto, Modise Zacharia; Matsevych, Oleh Y; Mosai, Fusi; Balabyeki, Moses; Aldous, Colleen

    2018-02-27

    Laparoscopy is increasingly utilised in the trauma setting. However, its safety and reliability in evaluating and managing retroperitoneal injuries are not known. The aim of this study was to analyse our experience with laparoscopic management of retroperitoneal injuries due to penetrating abdominal trauma (PAT) and to investigate its feasibility, safety and accuracy in haemodynamically stable patients. Over a 4-year period, patients approached laparoscopically with retroperitoneal injuries were analysed. Mechanism, location and severity of injuries were recorded. Surgical procedures, conversion rate and reasons for conversion and outcomes were described. Of the 284 patients with PAT, 56 patients had involvement of retroperitoneum. Stab wounds accounted 62.5% of patients. The mean Injury Severity Score was 7.4 (4-20). Among retroperitoneal injuries, the colon (27%) was the most commonly involved hollow viscera followed by duodenum (5%). The kidney (5%) and the pancreas (4%) were the injured solid organs. The conversion rate was 19.6% and was mainly due to active bleeding (73%). Significantly more patients with gunshot wound were converted to laparotomy (38% vs. 9%). Therapeutic laparoscopy was performed in 36% of patients. There were no recorded missed injuries or mortality. Five (9%) patients developed the Clavien-Dindo Grade 3 complications, three were managed with reoperation, one with drainage/debridement and one with endovascular technique. Laparoscopic management of retroperitoneal injuries is safe and feasible in haemodynamically stable patients with PAT. However, a high conversion rate indicates difficulties in managing these injuries. The requirements are the dexterity in laparoscopy and readiness to convert in the event of bleeding.

  10. Trial of Dasatinib in Advanced Sarcomas

    ClinicalTrials.gov

    2017-03-20

    Rhabdomyosarcoma; Malignant Peripheral Nerve Sheath Tumors; Chondrosarcoma; Sarcoma, Ewing's; Sarcoma, Alveolar Soft Part; Chordoma; Epithelioid Sarcoma; Giant Cell Tumor of Bone; Hemangiopericytoma; Gastrointestinal Stromal Tumor (GIST)

  11. Ewing's sarcoma of bone tumor cells produces MCSF that stimulates monocyte proliferation in a novel mouse model of Ewing's sarcoma of bone.

    PubMed

    Margulies, B S; DeBoyace, S D; Damron, T A; Allen, M J

    2015-10-01

    Ewing's sarcoma of bone is a primary childhood malignancy of bone that is treated with X-radiation therapy in combination with surgical excision and chemotherapy. To better study Ewing's sarcoma of bone we developed a novel model of primary Ewing's sarcoma of bone and then treated animals with X-radiation therapy. We identified that uncontrolled tumor resulted in lytic bone destruction while X-radiation therapy decreased lytic bone destruction and increased limb-length asymmetry, a common, crippling complication of X-radiation therapy. Osteoclasts were indentified adjacent to the tumor, however, we were unable to detect RANK-ligand in the Ewing's tumor cells in vitro, which lead us to investigate alternate mechanisms for osteoclast formation. Ewing's sarcoma tumor cells and archival Ewing's sarcoma of bone tumor biopsy samples were shown to express MCSF, which could promote osteoclast formation. Increased monocyte numbers were detected in peripheral blood and spleen in animals with untreated Ewing's sarcoma tumor while monocyte number in animals treated with x-radiation had normal numbers of monocytes. Our data suggest that our Ewing's sarcoma of bone model will be useful in the study Ewing's sarcoma tumor progression in parallel with the effects of chemotherapy and X-radiation therapy. Copyright © 2015 Elsevier Inc. All rights reserved.

  12. Ewing's Sarcoma of Bone Tumor Cells Produce MCSF that Stimulates Monocyte Proliferation in a Novel Mouse Model of Ewing's Sarcoma of Bone

    PubMed Central

    Margulies, BS; DeBoyace, SD; Damron, TA; Allen, MJ

    2015-01-01

    Ewing's sarcoma of bone is a primary childhood malignancy of bone that is treated with X-radiation therapy in combination with surgical excision and chemotherapy. To better study Ewing's sarcoma of bone we developed a novel model of primary Ewing's sarcoma of bone and then treated animals with X-radiation therapy. We identified that uncontrolled tumor resulted in lytic bone destruction while X-radiation therapy decreased lytic bone destruction and increased limb-length asymmetry, a common, crippling complication of X-radiation therapy. Osteoclasts were indentified adjacent to the tumor, however, we were unable to detect RANK-ligand in the Ewing's tumor cells in vitro, which lead us to investigate alternate mechanisms for osteoclast formation. Ewing's sarcoma tumor cells and archival Ewing's sarcoma of bone tumor biopsy samples were shown to express MCSF, which could promote osteoclast formation. Increased monocyte numbers were detected in peripheral blood and spleen in animals with untreated Ewing's sarcoma tumor while monocyte number in animals treated with x-radiation had normal numbers of monocytes. Our data suggest that our Ewing's sarcoma of bone model will be useful in the study Ewing's sarcoma tumor progression in parallel with the effects of chemotherapy and X-radiation therapy. PMID:26051470

  13. Molecular classification of soft tissue sarcomas and its clinical applications

    PubMed Central

    Jain, Shilpa; Xu, Ruliang; Prieto, Victor G; Lee, Peng

    2010-01-01

    Sarcomas are a heterogeneous group of tumors that are traditionally classified according to the morphology and type of tissue that they resemble, such as rhabdomyosarcoma, which resembles skeletal muscle. However, the cell of origin is unclear in numerous sarcomas. Molecular genetics analyses have not only assisted in understanding the molecular mechanism in sarcoma pathogenesis but also demonstrated new relationships within different types of sarcomas leading to a more proper classification of sarcomas. Molecular classification based on the genetic alteration divides sarcomas into two main categories: (i) sarcomas with specific genetic alterations; which can further be subclassified based on a) reciprocal translocations resulting in oncogenic fusion transcripts (e.g. EWSR1-FLI1 in Ewing sarcoma) and b) specific oncogenic mutations (e.g. KIT and PDGFRA mutations in gastrointestinal stromal tumors) and (ii) sarcomas displaying multiple, complex karyotypic abnormalities with no specific pattern, including leiomyo-sarcoma, and pleomorphic liposarcoma. These specific genetic alterations are an important adjunct to standard morphological and immunohistochemical diagnoses, and in some cases have a prognostic value, e. g., Ewing family tumors, synovial sarcoma, and alveolar rhabdomyosarcoma. In addition, these studies may also serve as markers to detect minimal residual disease and can aid in staging or monitor the efficacy of therapy. Furthermore, sarcoma-specific fusion genes and other emerging molecular events may also represent potential targets for novel therapeutic approaches such as Gleevec for dermatofibrosarcoma protuberans. Therefore, increased understanding of the molecular biology of sarcomas is leading towards development of newer and more effective treatment regimens. The review focuses on recent advances in molecular genetic alterations having an impact on diagnostics, prognostication and clinical management of selected sarcomas. PMID:20490332

  14. Clinical Trial Enrollment of Adolescents and Young Adults With Sarcoma

    PubMed Central

    Davis, Lara E.; Janeway, Katherine A.; Weiss, Aaron R.; Chen, Yen-Lin E.; Scharschmidt, Thomas J.; Krailo, Mark; Glade Bender, Julia L.; Kopp, Lisa M.; Patel, Shreyaskumar R.; Schwartz, Gary K.; Horvath, L. Elise; Hawkins, Douglas S.; Chuk, Meredith K.; Reinke, Denise K.; Gorlick, Richard G.; Randall, R. Lor

    2017-01-01

    More than half of all sarcomas occur in adolescents and young adults (AYAs) aged 15 to 39 years. After the publication of the AYA series in the April 1, 2016 issue of Cancer, several leaders in the field of sarcoma across disciplines gathered to discuss the status of sarcoma clinical research in AYAs. They determined that a focused effort to include the underrepresented and understudied AYA population in current and future sarcoma clinical trials is overdue. Trial enrichment for AYA-aged sarcoma patients will produce more meaningful results that better represent the disease's biology, epidemiology, and treatment environment. To address the current deficit, this commentary outlines changes believed to be necessary to expediently achieve an increase in the enrollment of AYAs in sarcoma clinical trials. PMID:28493547

  15. Retroperitoneal fibrosis.

    PubMed

    Tzou, Martha; Gazeley, David J; Mason, Peter J

    2014-10-01

    Retroperitoneal fibrosis (RPF) is a rare disease that is marked by systemic inflammation and the development of a periaortic fibroinflammatory mass. The fibroinflammatory infiltration can encase the abdominal aorta, ureters, and other abdominal organs. The clinical presentation often includes constitutional symptoms, abdominal pain, and signs of renal insufficiency or renal failure related to ureteral obstruction. Less frequently, RPF may present with vascular complications, such as venous thrombosis or claudication. The idiopathic form of RPF is most common but secondary forms have been described and are associated with malignancy and a variety of different medications. The pathophysiology is uncertain, but RPF has been linked with periaortitis and IgG4-related disease. Treatment centers on the relief of symptoms and complications associated with mass effects. Corticosteroids and other immunosuppressant therapies can improve constitutional symptoms, reduce infiltrate mass, and achieve disease remission, but a chronic relapsing course is not uncommon. © The Author(s) 2014.

  16. Prognostic factors for survival in patients with Ewing's sarcoma using the surveillance, epidemiology, and end results (SEER) program database.

    PubMed

    Duchman, Kyle R; Gao, Yubo; Miller, Benjamin J

    2015-04-01

    The current study aims to determine cause-specific survival in patients with Ewing's sarcoma while reporting clinical risk factors for survival. The Surveillance, Epidemiology, and End Results (SEER) Program database was used to identify patients with osseous Ewing's sarcoma from 1991 to 2010. Patient, tumor, and socioeconomic variables were analyzed to determine prognostic factors for survival. There were 1163 patients with Ewing's sarcoma identified in the SEER Program database. The 10-year cause-specific survival for patients with non-metastatic disease at diagnosis was 66.8% and 28.1% for patients with metastatic disease. Black patients demonstrated reduced survival at 10 years with an increased frequency of metastatic disease at diagnosis as compared to patients of other race, while Hispanic patients more frequently presented with tumor size>10cm. Univariate analysis revealed that metastatic disease at presentation, tumor size>10cm, axial tumor location, patient age≥20 years, black race, and male sex were associated with decreased cause-specific survival at 10 years. Metastatic disease at presentation, axial tumor location, tumor size>10cm, and age≥20 years remained significant in the multivariate analysis. Patients with Ewing's sarcoma have decreased cause-specific survival at 10 years when metastatic at presentation, axial tumor location, tumor size>10cm, and patient age≥20 years. Copyright © 2015 Elsevier Ltd. All rights reserved.

  17. Preliminary embryological study of the radiological concept of retroperitoneal interfascial planes: what are the interfascial planes?

    PubMed

    Ishikawa, Kazuo; Nakao, Shota; Murakami, Gen; Rodríguez-Vázquez, Jose Francisco; Matsuoka, Tetsuya; Nakamuro, Makoto; Shimazu, Takeshi

    2014-12-01

    Recently, the radiological concept of retroperitoneal interfascial planes has been widely accepted to explain the extension of retroperitoneal pathologies. This study aimed to explore embryologically based corroborative evidence, which remains to be elucidated, for this concept. Using serial or semi-serial transverse sections from 29 human fetuses at the 5th-25th week of fetal age, we microscopically observed the development of the retroperitoneal fasciae and other structures in the retroperitoneal connective tissue. A hypothesis for the formation of the interfascial planes was generated from the developmental study and analysis of retroperitoneal fasciae in computed tomography images from 224 patients. Whereas the loose connective tissue was uniformly distributed in the retroperitoneum by the 9th week, the primitive renal and transversalis fasciae appeared at the 10th-12th week, as previous research has noted. By the 23rd week, the renal fascia, transversalis fascia, and primitive adipose tissue of the flank pad emerged. In addition, the primitive lateroconal fascia, which runs parallel to and close to the posterior renal fascia, emerged between the renal fascia and the adipose tissue of the flank pad. Conversely, pre-existing loose connective tissue was sandwiched between the opposing fasciae and was compressed and narrowed by the developing organs and fatty tissues. Through this developmental study, we provided the hypothesis that the compressed loose connective tissue and both opposed fasciae compose the interfascial planes. Analysis of the thickened retroperitoneal fasciae in computed tomography images supported this hypothesis. Further developmental or histological studies are required to verify our hypothesis.

  18. Stages of Ewing Sarcoma

    MedlinePlus

    ... for Ewing sarcoma have an increased risk of acute myeloid leukemia and myelodysplastic syndrome . There is also an increased risk of sarcoma in the area treated with radiation therapy . Some late effects may be treated or ...

  19. Quantitative morphology in canine cutaneous soft tissue sarcomas.

    PubMed

    Simeonov, R; Ananiev, J; Gulubova, M

    2015-12-01

    Stained cytological specimens from 24 dogs with spontaneous soft tissue sarcomas [fibrosarcoma (n = 8), liposarcoma (n = 8) and haemangiopericytoma (n = 8)], and 24 dogs with reactive connective tissue lesions [granulation tissue (n = 12) and dermal fibrosis (n = 12)] were analysed by computer-assisted nuclear morphometry. The studied morphometric parameters were: mean nuclear area (MNA; µm(2)), mean nuclear perimeter (MNP; µm), mean nuclear diameter (MND mean; µm), minimum nuclear diameter (Dmin; µm) and maximum nuclear diameter (Dmax; µm). The study aimed to evaluate (1) possibility for quantitative differentiation of soft tissue sarcomas from reactive connective tissue lesions and (2) by using cytomorphometry, to differentiate the various histopathological soft tissue sarcomas subtypes in dogs. The mean values of all nuclear cytomorphometric parameters (except for Dmax) were statistically significantly higher in reactive connective tissue processes than in soft tissue sarcomas. At the same time, however, there were no considerable differences among the different sarcoma subtypes. The results demonstrated that the quantitative differentiation of reactive connective tissue processes from soft tissue sarcomas in dogs is possible, but the same was not true for the different canine soft tissue sarcoma subtypes. Further investigations on this topic are necessary for thorough explication of the role of quantitative morphology in the diagnostics of mesenchymal neoplasms and tumour-like fibrous lesions in dogs. © 2014 John Wiley & Sons Ltd.

  20. CIC-DUX4 Induces Small Round Cell Sarcomas Distinct from Ewing Sarcoma.

    PubMed

    Yoshimoto, Toyoki; Tanaka, Miwa; Homme, Mizuki; Yamazaki, Yukari; Takazawa, Yutaka; Antonescu, Cristina R; Nakamura, Takuro

    2017-06-01

    CIC-DUX4 sarcoma (CDS) or CIC-rearranged sarcoma is a subcategory of small round cell sarcoma resembling the morphological phenotypes of Ewing sarcoma (ES). However, recent clinicopathologic and molecular genetic analyses indicate that CDS is an independent disease entity from ES. Few ancillary markers have been used in the differential diagnosis of CDS, and additional CDS-specific biomarkers are needed for more definitive classification. Here, we report the generation of an ex vivo mouse model for CDS by transducing embryonic mesenchymal cells (eMC) with human CIC-DUX4 cDNA. Recipient mice transplanted with eMC-expressing CIC-DUX4 rapidly developed an aggressive, undifferentiated sarcoma composed of small round to short spindle cells. Gene-expression profiles of CDS and eMC revealed upregulation of CIC-DUX4 downstream genes such as PEA3 family genes, Ccnd2, Crh , and Zic1 IHC analyses for both mouse and human tumors showed that CCND2 and MUC5AC are reliable biomarkers to distinguish CDS from ES. Gene silencing of CIC-DUX4 as well as Ccnd2, Ret , and Bcl2 effectively inhibited CDS tumor growth in vitro The CDK4/6 inhibitor palbociclib and the soft tissue sarcoma drug trabectedin also blocked the growth of mouse CDS. In summary, our mouse model provides important biological information about CDS and provides a useful platform to explore biomarkers and therapeutic agents for CDS. Cancer Res; 77(11); 2927-37. ©2017 AACR . ©2017 American Association for Cancer Research.

  1. Clinical trial enrollment of adolescents and young adults with sarcoma.

    PubMed

    Davis, Lara E; Janeway, Katherine A; Weiss, Aaron R; Chen, Yen-Lin E; Scharschmidt, Thomas J; Krailo, Mark; Glade Bender, Julia L; Kopp, Lisa M; Patel, Shreyaskumar R; Schwartz, Gary K; Horvath, L Elise; Hawkins, Douglas S; Chuk, Meredith K; Reinke, Denise K; Gorlick, Richard G; Randall, R Lor

    2017-09-15

    More than half of all sarcomas occur in adolescents and young adults (AYAs) aged 15 to 39 years. After the publication of the AYA series in the April 1, 2016 issue of Cancer, several leaders in the field of sarcoma across disciplines gathered to discuss the status of sarcoma clinical research in AYAs. They determined that a focused effort to include the underrepresented and understudied AYA population in current and future sarcoma clinical trials is overdue. Trial enrichment for AYA-aged sarcoma patients will produce more meaningful results that better represent the disease's biology, epidemiology, and treatment environment. To address the current deficit, this commentary outlines changes believed to be necessary to expediently achieve an increase in the enrollment of AYAs in sarcoma clinical trials. Cancer 2017;123:3434-40. © 2017 American Cancer Society. © 2017 American Cancer Society.

  2. Retroperitoneal haematoma in a postoperative ALIF patient taking rivaroxaban for atrial fibrillation.

    PubMed

    Deekonda, Praveena; Stokes, Oliver M; Chan, Daniel

    2016-11-02

    Novel oral anticoagulants (NOACs) are being increasingly used in the secondary prevention of thromboembolic stroke in patients with atrial fibrillation. Patients taking NOACs are difficult to manage perioperatively, and several unexpected complications have been reported in these patients. We report a case of a rivaroxaban-induced retroperitoneal haematoma in a 72-year-old man who underwent an L5/S1 anterior lumbar interbody fusion (ALIF) for grade 1 spondylolytic spondylolisthesis. The patient suffered from atrial fibrillation and was taking rivaroxaban, a factor Xa inhibitor, for thromboembolic risk reduction. In accordance with perioperative Novel Oral Anticoagulant (NOAC) guidelines, rivaroxaban was stopped 2 days preoperatively and restarted on the third postoperative day. The patient presented on the ninth postoperative day, complaining of severe left iliac fossa pain, nausea, and vomiting, accompanied by swelling and bruising around the surgical site. A computed tomography (CT) scan showed a large expanding retroperitoneal haematoma. The patient was taken back to theatre for an evacuation of the haematoma and subsequently recovered without any further complications. This is the first case of a rivaroxaban-induced retroperitoneal haematoma reported in the literature, secondary to elective spinal surgery. This report adds to the body of evidence on the risk of postoperative bleeding in patients taking NOACs. If patients on NOACs present with abdominal symptoms following anterior approach to the lumbar spine, treating clinicians should have a high index of suspicion for retroperitoneal haematoma.

  3. Promiscuous partnerships in Ewing's sarcoma.

    PubMed

    Sankar, Savita; Lessnick, Stephen L

    2011-07-01

    Ewing's sarcoma is a highly aggressive bone and soft tissue tumor of children and young adults. At the molecular genetic level Ewing's sarcoma is characterized by a balanced reciprocal translocation, t(11;22)(q24;q12), which encodes an oncogenic fusion protein and transcription factor EWS/FLI. This tumor-specific chimeric fusion retains the amino terminus of EWS, a member of the TET (TLS/EWS/TAF15) family of RNA-binding proteins, and the carboxy terminus of FLI, a member of the ETS family of transcription factors. In addition to EWS/FLI, variant translocation fusions belonging to the TET/ETS family have been identified in Ewing's sarcoma. These studies solidified the importance of TET/ETS fusions in the pathogenesis of Ewing's sarcoma and have since been used as diagnostic markers for the disease. EWS fusions with non-ETS transcription factor family members have been described in sarcomas that are clearly distinct from Ewing's sarcoma. However, in recent years there have been reports of rare fusions in "Ewing's-like tumors" that harbor the amino-terminus of EWS fused to the carboxy-terminal DNA or chromatin-interacting domains contributed by non-ETS proteins. This review aims to summarize the growing list of fusion oncogenes that characterize Ewing's sarcoma and Ewing's-like tumors and highlights important questions that need to be answered to further support the existing concept that Ewing's sarcoma is strictly a "TET/ETS" fusion-driven malignancy. Understanding the molecular mechanisms of action of the various different fusion oncogenes will provide better insights into the biology underlying this rare but important solid tumor. Copyright © 2011 Elsevier Inc. All rights reserved.

  4. [Update on soft tissue sarcomas].

    PubMed

    Bui, Binh Nguyen; Tabrizi, Reza; Dagada, Corinne; Trufflandier, Nathalie; St ckle, Eberhard; Coindre, Jean-Michel

    2002-01-01

    Important refinements have taken place in the diagnosis of soft tissue sarcoma with extensive use of immuno-histochemistry. New entities have been described, while malignant histiocytofibroma, the most diagnosed sarcoma type during the last two decades, has been dismembered. As for prognosis, the new UICC classification is effectively more discriminating in the definition of prognostic groups; but the usefullness of new biological or genetic markers remains to be assessed. Several breakthrough have taken place in the last years in the treatment of soft tissue sarcoma. Isolated limb perfusion with TNF, hyperthermia and melphalan have proven its efficacy, and is now an alternative to preoperative chemotherapy and/or radiotherapy for limb sparing treatment of the primary tumor site or to amputation. For systemic treatments, novel cytostatic drugs have been shown to be active in sarcomas, including ecteinascidine (ET743) and Glivec (STI571). This last drug has been shown to be remarkably active in c-kit+ stromal sarcoma of the gastro-intestinal tract. It can hopefully regarded as an example for targeted therapies, which may come with a better understanding of the molecular mechanisms triggered by the fundamental, specific genetic alterations shown in sarcoma.

  5. Retroperitoneal liposarcoma extending into the thigh.

    PubMed

    Salemis, Nikolaos S; Nisotakis, Konstantinos; Patouras, Paraskevas; Karagkiouzis, Grigorios; Gourgiotis, Stavros

    2011-05-01

    The authors describe a case of a giant retroperitoneal liposarcoma that extended into the thigh along the course of the iliopsoas muscle. The tumor was successfully excised en bloc through an abdominal and a proximal thigh incision. Histopathologic examination revealed a well-differentiated, lipoma-like liposarcoma with clear surgical margins. The patient remains well without any evidence of tumor recurrence 18 months after surgery. Copyright © 2011 Elsevier Inc. All rights reserved.

  6. Retroperitoneal and rectus sheath hematomas.

    PubMed

    Kasotakis, George

    2014-02-01

    The retroperitoneum is rich in vascular structures and can harbor large hematomas, traumatic or spontaneous. The management of retroperitoneal hematomas depends on the mechanism of injury and whether they are pulsatile/expanding. Rectus sheath hematomas are uncommon abdominal wall hematomas secondary to trauma to the epigastric arteries of the rectus muscle. The common risk factors include anticoagulation, strenuous exercise, coughing, coagulation disorders, and invasive procedures on/through the abdominal wall. The management is largely supportive, with the reversal of anticoagulation and transfusions; angioembolization may be necessary. Copyright © 2014 Elsevier Inc. All rights reserved.

  7. Proximal-type epithelioid sarcoma - Case report*

    PubMed Central

    dos Santos, Luciana Mendes; Nogueira, Lisiane; Matsuo, Christiane Yuri; Talhari, Carolina; Santos, Mônica

    2013-01-01

    Epithelioid sarcoma, first described by Enzinger in 1970, is a rare soft-tissue sarcoma typically presenting as a subcutaneous or deep dermal mass in distal portions of the extremities of adolescents and young adults. In 1997, Guillou et al. described a different type of epithelioid sarcoma, called proximal-type epithelioid sarcoma, which is found mostly in the pelvic and perineal regions and genital tracts of young to middle-aged adults. It is characterized by a proliferation of epithelioid-like cells with rhabdoid features and the absence of a granuloma-like pattern. In this paper we present a case of proximal-type epithelioid sarcoma with an aggressive clinical course, including distant metastasis and death nine months after diagnosis. PMID:23793215

  8. Retroperitoneal laparoscopic dismembered pyeloplasty with a novel technique of JJ stenting in children.

    PubMed

    Yu, Jianhua; Wu, Zhonghua; Xu, Youming; Li, Zhuo; Wang, Jiansong; Qi, Fan; Chen, Xiang

    2011-09-01

    • To report our experience with retroperitoneal laparoscopic dismembered pyeloplasty for pelvi-ureteric junction (PUJ) obstruction in children. • Between March 2007 and December 2009, 38 children with PUJ obstruction (mean age 8.3 years, range 3-14) underwent retroperitoneal laparoscopic dismembered pyeloplasty. • A ureteric catheter was inserted into the mid-ureter cystoscopically. During pyeloplasty, the proximal end of the ureteric catheter was extracorporeally sutured to the distal end of the JJ stent with silk. • The ureteric catheter was then pulled down and the stent was pulled antegrade into the ureter and bladder. • The approach was retroperitoneal in all patients except one who required open conversion. The overall mean operative time was 162 min (range 145-210 min) and this appeared to decrease with experience. Mean hospital stay was 4 days (range 3-7 days). • Mean follow-up was 20.2 months (range 6-32 months). Satisfactory drainage with decreased hydronephrosis was documented in all patients on ultrasonography and intravenous urography. • Our study shows that retroperitoneal laparoscopic dismembered pyeloplasty is a feasible and effective alternative to open pyeloplasty with a relatively minimal complication rate in children 3 years of age and older, but it should be undertaken by experienced laparoscopic surgeons. © 2011 THE AUTHORS. BJU INTERNATIONAL © 2011 BJU INTERNATIONAL.

  9. Retroperitoneal laparoscopic pyelolithotomy in renal pelvic stone versus open surgery - a comparative study

    PubMed Central

    DHAR, SIDDHARTH

    2018-01-01

    Background The introduction of endourological procedures such as percutaneous nephrolithotomy and ureterorenoscopy have led to a revolution in the the management of urinary stone disease. The indications for open stone surgery have been narrowed significantly, making it a second- or third-line treatment option. Aims and Objectives To study the safety and efficacy of retroperitoneal laparoscopic pyelolithotomy in retroperitoneal renal stone. We compared the results of laparoscopic and open surgery in terms of easy accessibility, operative period, renal injuries, and early recovery. Methods This prospective study was conducted on renal pelvic stone cases from January 2009 to February 2016 in Suchkhand Hospital, Agra, India. The study included a total of 1700 cases with the diagnosis of solitary renal pelvic stones. In group A - 850 cases - retroperitoneal laparoscopic pyelolithotomy was performed, while group B - 850 cases – underwent open pyelolithotomy. Results The mean operative time was less in group B than group A (74.83 min vs. 94.43 min) which was significant (p<0.001). The blood loss was less in the laparoscopic group than in the open group (63 mL vs. 103mL). There were statistically significant differences in the post-operative pain scores, and postoperative complications compared to group B (p<0.001). The mean hospital stay was less in group A (p<0.03), which was significant. Conclusion Laparoscopic surgery reduces analgesic requirements, hospital stay, and blood loss. The disadvantages include the reduced working space, the cost of equipment and the availability of a trained surgeon. PMID:29440956

  10. Experience and learning curve of retroperitoneal laparoscopic ureterolithotomy for upper ureteral calculi.

    PubMed

    Fan, Tianyong; Xian, Peng; Yang, Lu; Liu, Yong; Wei, Qiang; Li, Hong

    2009-11-01

    To summarize our experience and evaluate the learning curve of retroperitoneal laparoscopic ureterolithotomy of the upper ureter. Between May 2004 and May 2007, 40 patients underwent retroperitoneal laparoscopic ureterolithotomy of the upper ureter. We divided the first and last 20 patients into group I and group II. There was no statistical difference in stone size between groups. Operative time and complications were measured as a basis for the assessment of the learning curve. In group I, the complication rate was 15% (3/20), including two patients whose procedure was converted to open surgery because of intraoperative bleeding, and one patient who experienced urine leakage because of a displaced Double-J ureteral stent. In group II, no postoperative complications occurred, while the mean operative time was significantly shorter compared with the earlier operations (65 vs 120 min). Retroperitoneal laparoscopic ureterolithotomy is safe and effective for large or impacted stones of the upper ureter. It is associated with a short learning curve in the setting of an active laparoscopic practice for selected patients.

  11. RUNX3 Facilitates Growth of Ewing Sarcoma Cells

    PubMed Central

    Bledsoe, Krista L.; McGee-Lawrence, Meghan E.; Camilleri, Emily T.; Wang, Xiaoke; van Wijnen, Andre J.; Oliveira, Andre M.; Westendorf, Jennifer J.

    2014-01-01

    Ewing sarcoma is an aggressive pediatric small round cell tumor that predominantly occurs in bone. Approximately 85% of Ewing sarcomas harbor the EWS/FLI fusion protein, which arises from a chromosomal translocation, t(11:22)(q24:q12). EWS/FLI interacts with numerous lineage-essential transcription factors to maintain mesenchymal progenitors in an undifferentiated state. We previously showed that EWS/FLI binds the osteogenic transcription factor RUNX2 and prevents osteoblast differentiation. In this study, we investigated the role of another Runt-domain protein, RUNX3, in Ewing sarcoma. RUNX3 participates in mesenchymal-derived bone formation and is a context dependent tumor suppressor and oncogene. RUNX3 was detected in all Ewing sarcoma cells examined, whereas RUNX2 was detected in only 73% of specimens. Like RUNX2, RUNX3 binds to EWS/FLI via its Runt domain. EWS/FLI prevented RUNX3 from activating the transcription of a RUNX-responsive reporter, p6OSE2. Stable suppression of RUNX3 expression in the Ewing sarcoma cell line A673 delayed colony growth in anchorage independent soft agar assays and reversed expression of EWS/FLI-responsive genes. These results demonstrate an important role for RUNX3 in Ewing sarcoma. PMID:24812032

  12. Kidney fibroxanthoma (malignant fibrous xanthoma): a rare tumor and an unusual cause of retroperitoneal hemorrhage.

    PubMed

    Witz, M; Bernheim, J; Dinbar, A; Griffel, B

    1984-06-01

    A case of kidney fibroxanthoma (malignant fibrous xanthoma, malignant variant of xanthogranuloma), a rare malignant neoplasm of kidney, is described. In addition to the typical histologic features of retroperitoneal xanthogranuloma, this tumor showed obvious pleomorphism and mitotic activity of the histiocytes. We present this case in view of the rarity of this neoplasm and the unusual presentation as massive retroperitoneal hemorrhage.

  13. Management of Breast Sarcoma.

    PubMed

    Hsu, Cary; McCloskey, Susan A; Peddi, Parvin F

    2016-10-01

    Breast sarcomas are exceptionally rare mesenchymal neoplasms composed of many histologic subtypes. Therapy is guided by principles established in the management of extremity sarcomas. The anatomic site does influence treatment decisions, particularly the surgical management. Surgery should be undertaken with the aim of achieving a widely negative margin. Selected patients can be managed with breast-conserving surgery. Breast reconstruction is increasingly being undertaken for selected patients. Radiation therapy and chemotherapy are used selectively for large, high-grade sarcomas for which there is significant concern for local and distant recurrence. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. A Retroperitoneal Neuroendocrine Tumor in Ectopic Pancreatic Tissue

    PubMed Central

    Okasha, Hussein Hassan; Al-Bassiouni, Fahim; El-Ela, Monir Abo; Al-Gemeie, Emad Hamza; Ezzat, Reem

    2013-01-01

    Ectopic pancreas is the relatively uncommon presence of pancreatic tissue outside the normal location of the pancreas. We report a case of abdominal pain due to retroperitoneal neuroendocrine tumor arising from heterotopic pancreatic tissue between the duodenal wall and the head of the pancreas. Patient underwent surgical enucleation of the tumor. PMID:24949389

  15. Soft-Tissue Sarcomas of the Abdomen and Pelvis: Radiologic-Pathologic Features, Part 1-Common Sarcomas: From the Radiologic Pathology Archives.

    PubMed

    Levy, Angela D; Manning, Maria A; Al-Refaie, Waddah B; Miettinen, Markku M

    2017-01-01

    Soft-tissue sarcomas are a diverse group of rare mesenchymal malignancies that can arise at any location in the body and affect all age groups. These sarcomas are most common in the extremities, trunk wall, retroperitoneum, and head and neck. In the adult population, soft-tissue sarcomas arising in the abdomen and pelvis are often large masses at the time of diagnosis because they are usually clinically silent or cause vague or mild symptoms until they invade or compress vital organs. In contrast, soft-tissue sarcomas arising from the abdominal wall come to clinical attention earlier in the course of disease because they cause a palpable mass, abdominal wall deformity, or pain that is more clinically apparent. The imaging features of abdominal and pelvic sarcomas and abdominal wall sarcomas can be nonspecific and overlap with more common pathologic conditions, making diagnosis difficult or, in some cases, delaying diagnosis. Liposarcoma (well-differentiated and dedifferentiated liposarcomas), leiomyosarcoma, and gastrointestinal stromal tumor (GIST) are the most common intra-abdominal primary sarcomas. Any soft-tissue sarcoma can arise in the abdominal wall. Knowledge of the classification and pathologic features of soft-tissue sarcomas, the anatomic locations where they occur, and their cross-sectional imaging features helps the radiologist establish the diagnosis or differential diagnosis so that patients with soft-tissue sarcomas can receive optimal treatment and management. In part 1 of this article, the most common soft-tissue sarcomas (liposarcoma, leiomyosarcoma, and GIST) are reviewed, with a discussion on anatomic locations, classification, clinical considerations, and differential diagnosis. Part 2 will focus on the remainder of the soft-tissue sarcomas occurring in the abdomen and pelvis.

  16. [Indication of chemotherapy according to histological type of musculoskeletal sarcomas].

    PubMed

    Goto, Takahiro; Okuma, Tomotake; Ogura, Koichi; Imanishi, Jungo; Hozumi, Takahiro; Kondo, Taiji

    2009-02-01

    In high-grade musculoskeletal sarcomas, adjuvant chemotherapy is often performed to prevent distant metastases. As the efficacy of chemotherapy varies according to the histological type of sarcoma, its indication is determined according to the histological type and the stage. Prognoses are poor in patients with osteosarcoma, Ewing's sarcoma, or rhabdomyosarcoma, when surgery alone is performed. However, because these sarcomas are chemosensitive, their prognoses are improved with adjuvant chemotherapy, so it is absolutely necessary. Drugs commonly used for osteosarcoma include adriamycin, cisplatin, methotrexate, vincristine, and ifosfamide. For Ewing's sarcoma and rhabdomyosarcoma, vincristine, actinomycin-D, cyclophosphamide, etoposide, and ifosfamide are commonly used. On the other hand, the efficacy of chemotherapy is unclear in most of the non-round cell sarcomas, e. g., malignant fibrous histiocytoma, pleomorphic liposarcoma, and leiomyosarcoma, so adjuvant chemotherapy is relatively indicated and often performed preoperatively. The efficacy is evaluated by reduction of the tumor volume as a surrogate marker. Postoperative chemotherapy is performed when the preoperative chemotherapy is effective. Nowadays, several kinds of antitumor agents are usually used for non-round cell sarcomas, and many authors have reported various kinds of regimens and their clinical results. Among them, the key drugs are adriamycin and ifosfamide. Recently, taxanes and gemcitabine are sometimes used. For chemoresistant sarcomas, e. g., chondrosarcoma, chordoma, alveolar soft part sarcoma, chemotherapy is rarely indicated, even if the tumor is histologically high grade and large. Low-grade musculoskeletal sarcomas, e. g., low-grade chondrosarcoma, central low-grade osteosarcoma, parosteal osteosarcoma, well-differentiated liposarcoma, and dermatofibrosarcoma protuberans, are well cured only by surgical excision, and adjuvant chemotherapy is therefore not indicated. Superficially

  17. Clinical and biological significance of hepatoma-derived growth factor in Ewing's sarcoma.

    PubMed

    Yang, Yang; Li, Hui; Zhang, Fenfen; Shi, Huijuan; Zhen, Tiantian; Dai, Sujuan; Kang, Lili; Liang, Yingjie; Wang, Jin; Han, Anjia

    2013-11-01

    We sought to investigate the clinicopathological significance and biological function of hepatoma-derived growth factor (HDGF) in Ewing's sarcoma. Our results showed that HDGF expression is up-regulated in Ewing's sarcoma. Nuclear HDGF expression is significantly associated with tumour volume (p < 0.001), metastases at diagnosis (p < 0.001), low overall survival rate (p < 0.001) and low disease-free survival rate (p < 0.001). HDGF knock-down results in significant reduction of Ewing's sarcoma cell growth, proliferation and enhances tumourigenesis, both in vitro and in vivo. Meanwhile, HDGF knock-down causes cell cycle arrest and enhanced sensitization to serum starvation-induced apoptosis. Furthermore, recombinant HDGF promotes proliferation and colony formation of Ewing's sarcoma cells. Ninety-eight candidate HDGF downstream genes were identified in Ewing's sarcoma cells using cDNA microarray analysis. In addition, we found that HDGF knock-down inhibited FLI1 expression in Ewing's sarcoma cells at the mRNA and protein levels. Our findings suggest that HDGF exhibits oncogenic properties and may be a novel prognostic factor in Ewing's sarcoma. Targeting HDGF might be a potential therapeutic strategy for Ewing's sarcoma. Copyright © 2013 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

  18. A Clinicopathological Analysis of Soft Tissue Sarcoma with Telangiectatic Changes.

    PubMed

    Kobayashi, Hiroshi; Ae, Keisuke; Tanizawa, Taisuke; Gokita, Tabu; Motoi, Noriko; Matsumoto, Seiichi

    2015-01-01

    Background. Soft tissue sarcoma with a hemorrhagic component that cannot be easily diagnosed by needle biopsy is defined here as soft tissue sarcoma with telangiectatic changes (STST). Methods. We retrospectively reviewed clinicopathological data of STST from 14 out of 784 patients (prevalence: 1.8%) with soft tissue sarcoma. Results. Tumors were found mostly in the lower leg. Histological diagnoses were undifferentiated pleomorphic sarcoma (n = 5), synovial sarcoma (n = 5), epithelioid sarcoma (n = 2), and malignant peripheral nerve sheath tumor and fibrosarcoma (n = 1). No history of trauma to the tumor site was recorded in any patient. Needle aspiration transiently reduced the tumor volume, but subsequent recovery of tumor size was observed in all cases. Out of 14 patients, 9 presented with a painful mass. MRI characteristics included intratumoral nodules (64.3%). The local recurrence rate was 14.3%, and the 2-year event-free survival rate was poorer (50%) than that of most sarcomas. Conclusions. STST is unique in its clinicopathological presentation. Painful hematomas without a trauma history, intratumoral nodules within a large hemorrhagic component, and subsequent recovery of tumor size after aspiration are indicative of the presence of STST.

  19. A Clinicopathological Analysis of Soft Tissue Sarcoma with Telangiectatic Changes

    PubMed Central

    Kobayashi, Hiroshi; Ae, Keisuke; Tanizawa, Taisuke; Gokita, Tabu; Motoi, Noriko; Matsumoto, Seiichi

    2015-01-01

    Background. Soft tissue sarcoma with a hemorrhagic component that cannot be easily diagnosed by needle biopsy is defined here as soft tissue sarcoma with telangiectatic changes (STST). Methods. We retrospectively reviewed clinicopathological data of STST from 14 out of 784 patients (prevalence: 1.8%) with soft tissue sarcoma. Results. Tumors were found mostly in the lower leg. Histological diagnoses were undifferentiated pleomorphic sarcoma (n = 5), synovial sarcoma (n = 5), epithelioid sarcoma (n = 2), and malignant peripheral nerve sheath tumor and fibrosarcoma (n = 1). No history of trauma to the tumor site was recorded in any patient. Needle aspiration transiently reduced the tumor volume, but subsequent recovery of tumor size was observed in all cases. Out of 14 patients, 9 presented with a painful mass. MRI characteristics included intratumoral nodules (64.3%). The local recurrence rate was 14.3%, and the 2-year event-free survival rate was poorer (50%) than that of most sarcomas. Conclusions. STST is unique in its clinicopathological presentation. Painful hematomas without a trauma history, intratumoral nodules within a large hemorrhagic component, and subsequent recovery of tumor size after aspiration are indicative of the presence of STST. PMID:26839509

  20. Laparoscopic retroperitoneal lymph node dissection for testicular cancer

    PubMed Central

    Hillelsohn, Joel H.; Duty, Brian D.; Okhunov, Zhamshid; Kavoussi, Louis R.

    2012-01-01

    Objectives Laparoscopic retroperitoneal lymph node dissection (L-RPLND) was introduced over 20 years ago as a less invasive alternative to open node dissection. In this review we summarise the indications, surgical technique and outcomes of L-RPLND in the treatment of testicular cancer. Methods We searched MEDLINE using the terms ‘laparoscopy’, ‘laparoscopic’, ‘retroperitoneal lymph node dissection’, ‘RPLND’ and ‘testicular neoplasms’. Articles were selected on the basis of their relevance, study design and content, with an emphasis on more recent data. Results We found 14 pertinent studies, which included >1300 patients who received either L-RPLND (515) or open RPLND (788). L-RPLND was associated with longer mean operative times (204 vs. 186 min), but shorter hospital stays (3.3 vs. 6.6 days) and lower complication rates (15.6% vs. 33%). Oncological outcomes were similar between L-RPLND and open RPLND, with local relapse rates of 1.3% and 1.4%, incidence of distal progression of 3.3% and 6.1%, biochemical failure in 0.9% and 1.1% and cure rates of 100% and 99.6%, respectively. Conclusion There are no randomised controlled studies comparing L-RPLND with open RPLND. A review of case and comparative series showed similar perioperative and oncological outcomes. Patients undergoing L-RPLND on average have shorter hospital stays, a quicker return to normal activity and improved cosmesis. PMID:26558006

  1. Retroperitoneal laparoscopic pyelolithotomy in renal pelvic stone versus open surgery - a comparative study.

    PubMed

    Singal, Rikki; Dhar, Siddharth

    2018-01-01

    The introduction of endourological procedures such as percutaneous nephrolithotomy and ureterorenoscopy have led to a revolution in the the management of urinary stone disease. The indications for open stone surgery have been narrowed significantly, making it a second- or third-line treatment option. To study the safety and efficacy of retroperitoneal laparoscopic pyelolithotomy in retroperitoneal renal stone. We compared the results of laparoscopic and open surgery in terms of easy accessibility, operative period, renal injuries, and early recovery. This prospective study was conducted on renal pelvic stone cases from January 2009 to February 2016 in Suchkhand Hospital, Agra, India. The study included a total of 1700 cases with the diagnosis of solitary renal pelvic stones. In group A - 850 cases - retroperitoneal laparoscopic pyelolithotomy was performed, while group B - 850 cases - underwent open pyelolithotomy. The mean operative time was less in group B than group A (74.83 min vs. 94.43 min) which was significant (p<0.001). The blood loss was less in the laparoscopic group than in the open group (63 mL vs. 103mL). There were statistically significant differences in the post-operative pain scores, and postoperative complications compared to group B (p<0.001). The mean hospital stay was less in group A (p<0.03), which was significant. Laparoscopic surgery reduces analgesic requirements, hospital stay, and blood loss. The disadvantages include the reduced working space, the cost of equipment and the availability of a trained surgeon.

  2. Sorafenib in Treating Patients With Metastatic, Locally Advanced, or Recurrent Sarcoma

    ClinicalTrials.gov

    2014-05-07

    Adult Angiosarcoma; Adult Epithelioid Sarcoma; Adult Leiomyosarcoma; Adult Malignant Fibrous Histiocytoma; Adult Neurofibrosarcoma; Adult Synovial Sarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Uterine Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma; Uterine Carcinosarcoma; Uterine Leiomyosarcoma

  3. Risk Factors Related with Retroperitoneal Laparoscopic Converted to Open Nephrectomy for Nonfunctioning Renal Tuberculosis.

    PubMed

    Xu, Bo; Hu, Jinghai; Chen, Anxiang; Hao, Yuanyuan; Liu, GuoHui; Wang, Chunxi; Wang, Xiaoqing

    2017-06-01

    The present study was designed to investigate the risk factors affecting the conversion to open surgery in retroperitoneal laparoscopic nephrectomy of nonfunctioning renal tuberculosis (TB). The records of 144 patients who underwent a retroperitoneal laparoscopic nephrectomy procedure by a single surgeon were retrospectively reviewed. The following factors, including age, sex, body mass index (BMI), diabetes status, hypertension status, side of kidney, size of kidney, degree of calcification, mild perirenal extravasation, contralateral hydronephrosis, the time of anti-TB, and surgeon experience were analyzed. Univariate and multivariate logistic regression analyses were used for statistical assessment. Twenty-three patients were converted to open surgery and the conversion rate was 15.97%. In univariate analysis, BMI ≥35 kg/m 2 (p = 0.023), hypertension (p = 0.011), diabetes (p = 0.003), and kidney size (p = 0.032) were the main factors of conversion to open surgery. Sex, age, side, anti-TB time, calcification, mild extravasation, and surgeon experience were not significantly related. In multivariate regression analysis, BMI ≥35 kg/m 2 , hypertension, diabetes, and enlargement of kidney were the most important factors for conversion to open surgery. Depending on the results achieved by a single surgeon, BMI ≥30 kg/m 2 , diabetes, hypertension, and enlargement of kidney significantly increased the conversion risk in retroperitoneal laparoscopic nephrectomy for nonfunctioning renal TB.

  4. Primary Undifferentiated Pleomorphic Sarcoma of the Penis

    PubMed Central

    Yoo, Hyung Sun; Satti, Suma

    2017-01-01

    Background: Primary penile sarcoma is a rare disease that affects men of all ages. Different subtypes of primary penile sarcoma exist, with the rarest being pleomorphic sarcoma. Delays in presentation and diagnosis of primary penile sarcoma have been reported because of its benign-appearing presenting features and rarity. If penile sarcoma is left untreated, the clinical consequence is metastasis that is fatal in most cases. Case Report: We report an extremely rare case of undifferentiated pleomorphic sarcoma of the penis in a 59-year-old patient who initially presented with a slow-growing penile nodule. The tumor was surgically excised, but the patient experienced local recurrence and, despite receiving chemotherapy and surgery, died of metastatic disease 15 months after initial presentation. Conclusion: Vigilance regarding biopsy and intervention for penile nodules may lead to early diagnosis and improved clinical outcomes. PMID:29230132

  5. Inhibition of CHK1 sensitizes Ewing sarcoma cells to the ribonucleotide reductase inhibitor gemcitabine

    PubMed Central

    Goss, Kelli L; Koppenhafer, Stacia L; Harmoney, Kathryn M; Terry, William W; Gordon, David J

    2017-01-01

    Ewing sarcoma is a bone and soft tissue sarcoma that occurs in children and young adults. The EWS-FLI1 gene fusion is the driver mutation in most Ewing sarcoma tumors and functions, in part, as an aberrant transcription factor. We recently identified that Ewing sarcoma cells are sensitive to inhibition of ribonucleotide reductase (RNR), which catalyzes the formation of deoxyribonucleotides from ribonucleotides. In this report, we show that Ewing sarcoma cells are sensitive to treatment with clofarabine, which is a nucleoside analogue and allosteric inhibitor of RNR. However, clofarabine is a reversible inhibitor of RNR and we found that the effect of clofarabine is limited when using a short (6-hour) drug treatment. Gemcitabine, on the other hand, is an irreversible inhibitor of the RRM1 subunit of RNR and this drug induces apoptosis in Ewing sarcoma cells when used in both 6-hour and longer drug treatments. Treatment of Ewing sarcoma cells with gemcitabine also results in activation of checkpoint kinase 1 (CHK1), which is a critical mediator of cell survival in the setting of impaired DNA replication. Notably, inhibition of CHK1 function in Ewing sarcoma cells using a small-molecule CHK1 inhibitor, or siRNA knockdown, in combination with gemcitabine results in increased toxicity both in vitro and in vivo in a mouse xenograft experiment. Overall, our results provide insight into Ewing sarcoma biology and identify a candidate therapeutic target, and drug combination, in Ewing sarcoma. PMID:29152060

  6. General Information about Ewing Sarcoma

    MedlinePlus

    ... Research Ewing Sarcoma Treatment (PDQ®)–Patient Version General Information About Ewing Sarcoma Go to Health Professional Version ... the PDQ Pediatric Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  7. General Information about Kaposi Sarcoma

    MedlinePlus

    ... Research Kaposi Sarcoma Treatment (PDQ®)–Patient Version General Information About Kaposi Sarcoma Go to Health Professional Version ... the PDQ Adult Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  8. General Information About Uterine Sarcoma

    MedlinePlus

    ... Research Uterine Sarcoma Treatment (PDQ®)–Patient Version General Information About Uterine Sarcoma Go to Health Professional Version ... the PDQ Adult Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  9. The ENCCA-WP7/EuroSarc/EEC/PROVABES/EURAMOS 3rd European Bone Sarcoma Networking Meeting/Joint Workshop of EU Bone Sarcoma Translational Research Networks; Vienna, Austria, September 24-25, 2015. Workshop Report.

    PubMed

    Kager, Leo; Whelan, Jeremy; Dirksen, Uta; Hassan, Bass; Anninga, Jakob; Bennister, Lindsey; Bovée, Judith V M G; Brennan, Bernadette; Broto, Javier M; Brugières, Laurence; Cleton-Jansen, Anne-Marie; Copland, Christopher; Dutour, Aurélie; Fagioli, Franca; Ferrari, Stefano; Fiocco, Marta; Fleuren, Emmy; Gaspar, Nathalie; Gelderblom, Hans; Gerrand, Craig; Gerß, Joachim; Gonzato, Ornella; van der Graaf, Winette; Hecker-Nolting, Stefanie; Herrero-Martín, David; Klco-Brosius, Stephanie; Kovar, Heinrich; Ladenstein, Ruth; Lancia, Carlo; LeDeley, Marie-Cecile; McCabe, Martin G; Metzler, Markus; Myklebost, Ola; Nathrath, Michaela; Picci, Piero; Potratz, Jenny; Redini, Françoise; Richter, Günther H S; Reinke, Denise; Rutkowski, Piotr; Scotlandi, Katia; Strauss, Sandra; Thomas, David; Tirado, Oscar M; Tirode, Franck; Vassal, Gilles; Bielack, Stefan S

    2016-01-01

    This report summarizes the results of the 3rd Joint ENCCA-WP7, EuroSarc, EEC, PROVABES, and EURAMOS European Bone Sarcoma Network Meeting, which was held at the Children's Cancer Research Institute in Vienna, Austria on September 24-25, 2015. The joint bone sarcoma network meetings bring together European bone sarcoma researchers to present and discuss current knowledge on bone sarcoma biology, genetics, immunology, as well as results from preclinical investigations and clinical trials, to generate novel hypotheses for collaborative biological and clinical investigations. The ultimate goal is to further improve therapy and outcome in patients with bone sarcomas.

  10. Treatment Option Overview (Ewing Sarcoma)

    MedlinePlus

    ... for Ewing sarcoma have an increased risk of acute myeloid leukemia and myelodysplastic syndrome . There is also an increased risk of sarcoma in the area treated with radiation therapy . Some late effects may be treated or ...

  11. [Positron emission tomography with fluorine-deoxyglucose in sarcomas and non-sarcoma non-epithelial tumors].

    PubMed

    Massardo, Teresa; Jofré, María Josefina; Sierralta, María Paulina; Canessa, José; Castro, Gabriel; Berrocal, Isabel; Gallegos, Iván

    2012-09-01

    The usefulness of positron emission tomography (PET) with fluorine-deoxyglucose (FDG) in sarcomas and non-sarcoma non-epithelial (NSNE) tumors is not clearly defined. To report a Chilean experience with NSNE tumors evaluated using PET with FDG. Retrospective review of the database of a PET laboratory. Demographic data, indications and metabolic findings were compared with conventional imaging in 88 adults and children with diverse bone and soft tissue sarcomas as well as 24 gastrointestinal stromal tumors (GIST), 6 pleural malignant mesotheliomas in adults, and 9 medulloblastomas in children. FDG showed good concordance with conventional imaging in NSNE tumors. It was helpful for staging, restaging, follow-up after treatment and for the detection of new not previously suspected lesions. PET with FDG could have a prognostic role and help in patient management, mainly in musculoskeletal and high grade or less differentiated sarcomas. In GIST, it was a good tool for immunotherapy control.

  12. Soft-Tissue Sarcomas of the Abdomen and Pelvis: Radiologic-Pathologic Features, Part 1—Common Sarcomas: From the Radiologic Pathology Archives

    PubMed Central

    Manning, Maria A.; Al-Refaie, Waddah B.; Miettinen, Markku M.

    2017-01-01

    Soft-tissue sarcomas are a diverse group of rare mesenchymal malignancies that can arise at any location in the body and affect all age groups. These sarcomas are most common in the extremities, trunk wall, retroperitoneum, and head and neck. In the adult population, soft-tissue sarcomas arising in the abdomen and pelvis are often large masses at the time of diagnosis because they are usually clinically silent or cause vague or mild symptoms until they invade or compress vital organs. In contrast, soft-tissue sarcomas arising from the abdominal wall come to clinical attention earlier in the course of disease because they cause a palpable mass, abdominal wall deformity, or pain that is more clinically apparent. The imaging features of abdominal and pelvic sarcomas and abdominal wall sarcomas can be nonspecific and overlap with more common pathologic conditions, making diagnosis difficult or, in some cases, delaying diagnosis. Liposarcoma (well-differentiated and dedifferentiated liposarcomas), leiomyosarcoma, and gastrointestinal stromal tumor (GIST) are the most common intra-abdominal primary sarcomas. Any soft-tissue sarcoma can arise in the abdominal wall. Knowledge of the classification and pathologic features of soft-tissue sarcomas, the anatomic locations where they occur, and their cross-sectional imaging features helps the radiologist establish the diagnosis or differential diagnosis so that patients with soft-tissue sarcomas can receive optimal treatment and management. In part 1 of this article, the most common soft-tissue sarcomas (liposarcoma, leiomyosarcoma, and GIST) are reviewed, with a discussion on anatomic locations, classification, clinical considerations, and differential diagnosis. Part 2 will focus on the remainder of the soft-tissue sarcomas occurring in the abdomen and pelvis. PMID:28287938

  13. Promiscuous Partnerships in Ewing’s Sarcoma

    PubMed Central

    Sankar, Savita; Lessnick, Stephen L.

    2011-01-01

    Ewing’s sarcoma is a highly aggressive bone and soft tissue tumor of children and young adults. At the molecular genetic level Ewing’s sarcoma is characterized by a balanced reciprocal translocation, t(11;22)(q24;q12), which encodes an oncogenic fusion protein and transcription factor EWS/FLI. This tumor-specific chimeric fusion retains the amino terminus of EWS, a member of the TET (TLS/EWS/TAF15) family of RNA-binding proteins, and the carboxy terminus of FLI, a member of the ETS family of transcription factors. In addition to EWS/FLI, variant translocation fusions belonging to the TET/ETS family have been identified in Ewing’s sarcoma. These studies solidified the importance of TET/ETS fusions in the pathogenesis of Ewing’s sarcoma and have since been used as diagnostic markers for the disease. EWS fusions with non-ETS transcription factor family members have been described in sarcomas that are clearly distinct from Ewing’s sarcoma. However, in recent years there have been reports of rare fusions in “Ewing’s-like tumors” that harbor the amino-terminus of EWS fused to the carboxy-terminal DNA or chromatin-interacting domains contributed by non-ETS proteins. This review aims to summarize the growing list of fusion oncogenes that characterize Ewing’s sarcoma and Ewing’s-like tumors and highlights important questions that need to be answered to further support the existing concept that Ewing’s sarcoma is strictly a “TET/ETS” fusion-driven malignancy. Understanding the molecular mechanisms of action of the various different fusion oncogenes will provide better insights into the biology underlying this rare but important solid tumor. PMID:21872822

  14. Socioeconomic factors and the risk for sarcoma.

    PubMed

    Hampras, Shalaka S; Moysich, Kirsten B; Marimuthu, Sathiya P; Ravi, Vinod; Jayaprakash, Vijayvel

    2014-11-01

    Sarcomas are a heterogeneous group of rare malignancies arising from mesenchymal tissue. Although several occupational exposures have been evaluated in association with sarcoma, little is known about the role of socioeconomic indicators such as education. Socioeconomic status has been found to be associated with risk of development of several types of cancers, primarily lung, gastric, and cervical cancers. We conducted a hospital-based case-control study to evaluate the association of socioeconomic level with the risk for sarcoma. A total of 371 incident cases of sarcoma were matched in terms of age, sex, and year of enrollment in the study with 742 cancer-free controls. Education and income levels were evaluated as the indicators of socioeconomic status. Higher education (college level) was associated with a significantly lower risk for sarcoma [odds ratio (OR)=0.48, 95% confidence interval (CI)=0.29-0.80], even after adjusting for important confounders. After stratifying by sex, significantly lower risk for sarcoma was observed among men who had college level education compared with men with a level of education of eighth grade or lower (OR=0.38, 95% CI=0.19-0.74). A significant association between education and the risk for sarcoma remained after stratifying by income (OR=0.49, 95% CI=0.28-0.86, among the low income group). When analyzed as a composite exposure, individuals with high education and high income status had significantly lower risk for sarcoma compared with those with low income and low education status (OR=0.41, 95% CI=0.23-0.71). Thus, socioeconomic factors may play a significant role in determining the risk for sarcoma and should be explored further to elucidate the underlying factors that may explain these sociodemographic inequalities related to sarcoma.

  15. Advances in sarcoma diagnostics and treatment

    PubMed Central

    Dancsok, Amanda R; Asleh-Aburaya, Karama; Nielsen, Torsten O

    2017-01-01

    The heterogeneity of sarcomas with regard to molecular genesis, histology, clinical characteristics, and response to treatment makes management of these rare yet diverse neoplasms particularly challenging. This review encompasses recent developments in sarcoma diagnostics and treatment, including cytotoxic, targeted, epigenetic, and immune therapy agents. In the past year, groups internationally explored the impact of adding mandatory molecular testing to histological diagnosis, reporting some changes in diagnosis and/or management; however, the impact on outcomes could not be adequately assessed. Transcriptome sequencing techniques have brought forward new diagnostic tools for identifying fusions and/or characterizing unclassified entities. Next-generation sequencing and advanced molecular techniques were also applied to identify potential targets for directed and epigenetic therapy, where preclinical studies reported results for agents active within the receptor tyrosine kinase, mTOR, Notch, Wnt, Hedgehog, Hsp90, and MDM2 signaling networks. At the level of clinical practice, modest developments were seen for some sarcoma subtypes in conventional chemotherapy and in therapies targeting the pathways activated by various receptor tyrosine kinases. In the burgeoning field of immune therapy, sarcoma work is in its infancy; however, elaborate protocols for immune stimulation are being explored, and checkpoint blockade agents advance from preclinical models to clinical studies. PMID:27732970

  16. EWS/FLI1 regulates EYA3 in Ewing's sarcoma via modulation of microRNA-708, resulting in increased cell survival and chemoresistance

    PubMed Central

    Robin, Tyler P; Smith, Anna; McKinsey, Erin; Reaves, Lisa; Jedlicka, Paul; Ford, Heide L.

    2012-01-01

    Ewing's sarcoma is an aggressive pediatric cancer of the bone and soft tissue, in which patients whose tumors have a poor histological response to initial chemotherapy have a poor overall prognosis. Therefore, it is important to identify molecules involved in resistance to chemotherapy. Herein, we demonstrate that the DNA-repair protein and transcriptional cofactor, EYA3, is highly expressed in Ewing's sarcoma tumor samples and cell lines compared with mesenchymal stem cells, the presumed cell of origin of Ewing's sarcoma, and that it is regulated by the EWS/FLI1 fusion protein transcription factor. We further demonstrate that EWS/FLI1 mediates upregulation of EYA3 via repression of miR-708, a microRNA that targets the EYA3 3′UTR, rather than by binding the EYA3 promoter directly. Importantly, we demonstrate that high levels of EYA3 significantly correlate with low levels of miR-708 in Ewing's sarcoma samples, suggesting that this miR-mediated mechanism of EYA3 regulation holds true in human cancers. Because EYA proteins are important for cell survival during development, we examine, and demonstrate, that loss of EYA3 decreases survival of Ewing's sarcoma cells. Most importantly, knockdown of EYA3 in Ewing's sarcoma cells leads to sensitization to DNA-damaging chemotherapeutics used in the treatment of Ewing's sarcoma, and as expected, after chemotherapeutic treatment, EYA3 knockdown cells repair DNA damage less effectively than their control counterparts. These studies identify EYA3 as a novel mediator of chemoresistance in Ewing's sarcoma and define the molecular mechanisms of both EYA3 overexpression and of EYA3-mediated chemoresistance. PMID:22723308

  17. Radiological features of primitive neuroectodermal tumors in intra-abdominal and retroperitoneal regions: A series of 18 cases

    PubMed Central

    Zhang, Youming; Xiao, Desheng; Yin, Hongling; Long, Xueying; Li, Li; Zai, Hongyan; Chen, Minfeng; Li, Wenzheng; Sun, Lunquan

    2017-01-01

    Objectives To characterize the imaging and clinicopathological features of primitive neuroectodermal tumors (PNETs) arising in intra-abdominal and retroperitoneal regions. Methods Eighteen patients with histopathologically proven intra-abdominal and retroperitoneal PNET were enrolled; computed tomography was performed for all cases, and magnetic resonance imaging was performed for a single case. Typical computed tomography and magnetic resonance imaging findings, including morphology, texture and enhancement features, as well as clinicopathological characteristics and prognosis data were retrospectively analyzed. Results Of eighteen PNET patients, fifteen were male and three were female, with a median age of 36 years (range, 2–65 years). The onset of symptoms was most often nonspecific and insidious. The mean tumor diameter was 7.2 cm (range, 3.0–12.1 cm), with necrosis in fifteen cases, cystic changes in eight, partition structure in five, calcification in five, hemorrhage in two, and mural nodules in one. Contrast enhanced computed tomography showed multiple tiny feeding arteries within the masses in six cases, resulting in a crab-like appearance, and mild ring enhancement pattern in five cases. Eleven cases showed surrounding invasion and metastasis. Of the eighteen PNET cases, nine cases showed smooth, well-defined margins, and nine cases had irregular, ill-defined margins. A median survival was 10.0±1.6 months. However, chemotherapy had efficacy on patients even those with advanced disease. Conclusions Primary intra-abdominal and retroperitoneal PNETs are rare, and imaging features documented here may help the diagnosis of this severe disease. Notably, two signs present in retroperitoneal PNET tumors, including a mild ring enhancement pattern and a crab-like appearance of the tiny feeding arteries, may have the potential to help us improve the ability to make a relatively reliable diagnosis. PMID:28319177

  18. Dyspareunia in a Teenager Reveals a Rare Occurrence: Retroperitoneal Cervical Leiomyoma of the Left Pararectal Space.

    PubMed

    Giannella, Luca; Mfuta, Kabala; Tuzio, Antonella; Cerami, Lillo Bruno

    2016-02-01

    Retroperitoneal uterine leiomyoma is a very rare occurrence and to discover it as a cause of female sexual dysfunction in a teen is unusual. An 18-year-old black woman reported deep dyspareunia, resulting in severe distress. Gynecological and instrumental examinations showed a pelvic mass of 7 cm in diameter. The preoperative diagnosis was uterine fibroid, but the exact location of the leiomyoma was uncertain. Laparoscopic examination showed a pedunculated retroperitoneal cervical leiomyoma in the left pararectal space. After surgical excision of the mass, normal sexual activity was restored. When a teen experiences pain with intercourse, pelvic masses should be part of differential diagnosis of dyspareunia. Copyright © 2016 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  19. Complete Resolution of Retroperitoneal Lymphangioma with a Single Trial of OK-432 in an Infant.

    PubMed

    Olivieri, Claudio; Nanni, Lorenzo; De Gaetano, Anna Maria; Manganaro, Lucia; Pintus, Claudio

    2016-06-01

    Retroperitoneal lymphangioma is extremely rare. Although these neoplasms are benign, they can grow progressively with subsequent compression and infiltration of the adjacent structures. Surgical excision is demanding when the lesion surrounds vital structures and it is generally fraught with a high recurrence and morbidity rate. We report the case of a huge retroperitoneal lymphangioma in a newborn treated successfully with intracystic injection of OK-432. Copyright © 2013. Published by Elsevier B.V.

  20. Protective, elective lung irradiation in non-metastatic Ewing's sarcoma.

    PubMed

    Marinova, L; Hristozova, I; Mihaylova, I; Perenovska, P

    2015-07-01

    Ewing's sarcoma in childhood is a disease from family of the peripheral primitive neuroectodermal tumours. For a period of 16 y (1984-2000), 34 children with Ewing's sarcoma were treated and followed in our department. Twenty-seven of these patients were without distant metastases. Complex treatment was applied to all these patients-chemotherapy VACA (vincristine, actinomycin D, cyclophosphamide, adriamycin), local radiotherapy to a total dose of 50-56 Gy +/- surgery. After, a local tumour control was achieved in 11 children with non-metastatic Ewing's sarcoma, elective whole lung irradiation to a total dose of 12-15 Gy was applied. Our experience in these 11 patients with non-metastatic Ewing's sarcoma, in whom elective lung irradiation was applied, showed significant reduction in the lung metastases, improved free of disease survival and overall survival. The achieved good treatment results necessitate extending this treatment approach through defining the risk groups of patients, suitable for elective lung radiotherapy combined with chemotherapy in non-metastatic Ewing's sarcoma. © The Author 2015. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  1. Alisertib in Treating Patients With Advanced or Metastatic Sarcoma

    ClinicalTrials.gov

    2017-11-29

    Myxofibrosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Leiomyosarcoma; Recurrent Liposarcoma; Recurrent Malignant Peripheral Nerve Sheath Tumor; Recurrent Undifferentiated Pleomorphic Sarcoma; Stage III Soft Tissue Sarcoma AJCC v7; Stage IV Soft Tissue Sarcoma AJCC v7

  2. The epidemiology of classic, African, and immunosuppressed Kaposi's sarcoma.

    PubMed

    Wahman, A; Melnick, S L; Rhame, F S; Potter, J D

    1991-01-01

    The etiology of Kaposi's sarcoma remains somewhat obscure. While lesions of classic Kaposi's sarcoma, African Kaposi's sarcoma, and immunosuppressed Kaposi's sarcoma have been found to be indistinguishable from one another, the reasons for the variations in type and severity have not been established. The origin of the spindle cell is yet to be agreed on. Geographic variation does not seem as important as ethnic variation. The very young and the very old, perhaps two ages of weakened immunity, tend to have a higher incidence of Kaposi's sarcoma. Children and AIDS patients tend to develop more virulent disease. Males tend to get Kaposi's sarcoma at higher rates than do females. Jewish and Mediterranean males have the highest incidence of classic Kaposi's sarcoma, and African Bantu have the highest incidence of African Kaposi's sarcoma, classifications which do not apply to the Kaposi's sarcoma population in the United States. Male homosexuals have much higher incidence of Kaposi's sarcoma than do male heterosexuals, but since the early 1980s, its incidence as the presenting manifestation of AIDS has decreased dramatically. There is no unequivocal association with HLA haplotype (though DR5 carriers may be at especially high risk) or evidence of family clustering. There is an impressive but not always consistent association between Kaposi's sarcoma development and immunodeficiency. Environmental factors, such as nitrite use, immunosuppression, and repeated cytomegalovirus infection, are associated with Kaposi's sarcoma, but the exact mechanism is unclear and the associations remain inconsistent. Finally, it is still unclear if there is a causative infectious agent for Kaposi's sarcoma. While cytomegalovirus has been linked to Kaposi's sarcoma, there are weaknesses in its hypothetical role as an etiologic agent as is the case for HIV itself.(ABSTRACT TRUNCATED AT 400 WORDS)

  3. Variations of Surveillance Practice for Patients with Bone Sarcoma: A Survey of Australian Sarcoma Clinicians

    PubMed Central

    Thompson, Kate; Bae, Susie; Desai, Jayesh; Strong, Robyn; Caruso, Denise; Howell, Deborah; Herschtal, Alan; Sullivan, Michael; Orme, Lisa

    2017-01-01

    Introduction. After treatment, bone sarcoma patients carry a high chance of relapse and late effects from multimodal therapy. We hypothesize that significant variation in surveillance practice exists between pediatric medical oncology (PO) and nonpediatric medical oncology (NP) sarcoma disciplines. Methods. Australian sarcoma clinicians were approached to do a web based survey that assessed radiologic surveillance (RS) strategies, late toxicity assessment, and posttreatment psychosocial interventions. Results. In total, 51 clinicians responded. No differences were identified in local disease RS. In metastatic disease response assessment, 100% of POs (23/23) and 93% of NPs (24/26) conducted CT chest. However, this was more likely to occur for NPs in the context of a CT chest/abdomen/pelvis (NP: 10/26; PO: 1/23; p = 0.006). POs were more likely to use CXR for RS (p = 0.006). POs showed more prescriptive intensity in assessment of heart function (p = 0.001), hearing (p < 0.001), and fertility (p = 0.02). POs were more likely to deliver written information for health maintenance/treatment summary (p = 0.04). The majority of respondents described enquiring about psychosocial aspects of health (n = 33/37, 89%), but a routine formal psychosocial screen was only used by 23% (n = 6/26). Conclusion. There is high variability in bone sarcoma surveillance between PO and NP clinicians. Efforts to harmonize approaches would allow early and late effects recognition/intervention and facilitate improved patient care/transition and research. PMID:28348507

  4. Pure retroperitoneal natural orifice translumenal endoscopic surgery (NOTES) transvaginal nephrectomy using standard laparoscopic instruments: a safety and feasibility study in a porcine model.

    PubMed

    Wei, Dechao; Han, Yili; Li, Mingchuan; Wang, Yongxing; Chen, Yatong; Luo, Yong; Jiang, Yongguang

    2016-06-11

    Among the different organs used for NOTES (natural orifice translumenal endoscopic surgery) technique, the transvaginal approach may be the optimal choice because of a simple and secure closure of colpotomy site. Pure and hybrid NOTES transvaginal operations were routinely performed via transperitoneal access. In this study, we investigate the safety and feasibility of pure retroperitoneal natural orifice translumenal endoscopic surgery (NOTES) transvaginal nephrectomy using conventional laparoscopic techniques in a porcine model. Six female pigs, weighing an average of 30 kg, were used in this study. Under general anesthesia, pure retroperitoneal NOTES transvaginal nephrectomy was conducted using standard laparoscopic instruments. Posterolateral colpotomy was performed, and the incision was enlarged laterally using blunt dissection and pneumatic dilation. A single-port device was inserted to construct the operative channel. The retroperitoneal space was created using sharp and blunt dissection under endoscopic guidance up to the level of the kidney. Dissection and removal of the kidney were performed according to standard surgical procedure, and the colpotomy site was closed using interrupted sutures. The survival and complications were observed 1 week postoperatively. Our results showed that two cases failed because of peritoneal rupture. One case was successful, but required the assistance of an extra 5 mm laparoscopic trocar inserted in the flank. Three cases of pure retroperitoneal NOTES transvaginal nephrectomy were completed, and survived 1 week after the operation. In these three cases, no intra- or postoperative complications were observed. All findings confirmed the safety and feasibility of the retroperitoneal pure retroperitoneal NOTES transvaginal nephrectomy using standard laparoscopic instruments, which suggested the possibility of clinical application in human beings in the future.

  5. Primary Occipital Ewing's Sarcoma with Subsequent Spinal Seeding.

    PubMed

    Alqahtani, Ali; Amer, Roaa; Bakhsh, Eman

    2017-01-01

    Ewing's sarcoma is a primary bone cancer that mainly affects the long bones. This malignancy is particularly common in pediatric patients. Primary cranial involvement accounts for 1% of cases, with occipital involvement considered extremely rare. In this case study, primary occipital Ewing's sarcoma with a posterior fossa mass and subsequent relapse resulting in spinal seeding is reported. A 3-year-old patient presented with a 1-year history of left-sided headaches, localized over the occipital bone with progressive torticollis. Computed tomography (CT) imaging showed a mass in the left posterior fossa compressing the brainstem. The patient then underwent surgical excision followed by adjuvant chemoradiation therapy. Two years later, the patient presented with severe lower back pain and urinary incontinence. Whole-spine magnetic resonance imaging (MRI) showed cerebrospinal fluid (CSF) seeding from the L5 to the S4 vertebrae. Primary cranial Ewing's sarcoma is considered in the differential diagnosis of children with extra-axial posterior fossa mass associated with destructive permeative bone lesions. Although primary cranial Ewing's sarcoma typically has good prognosis, our patient developed metastasis in the lower spine. Therefore, with CNS Ewing's sarcoma, screening of the entire neural axis should be taken into consideration for early detection of CSF seeding metastasis in order to decrease the associated morbidity and mortality.

  6. Outcome of video-assisted translumbar retroperitoneal necrosectomy and closed lavage for severe necrotizing pancreatitis.

    PubMed

    Ulagendra Perumal, Srinivasan; Pillai, Sastha Ahanatha; Perumal, Senthilkumar; Sathyanesan, Jeswanth; Palaniappan, Ravichandran

    2014-04-01

    Surgery for necrotizing pancreatitis is associated with a high rate of morbidity and mortality. We present a series of 26 patients who underwent video-assisted translumbar retroperitoneal necrosectomy and analyse their outcomes. Records of 26 patients who underwent video-assisted translumbar retroperitoneal necrosectomy and closed drainage for infected pancreatitic necrosis between January 2008 and March 2012 were reviewed, retrospectively. Twenty-three out of 26 patients were males, with a mean age of 38.6 (±9.9) years. Alcohol was the aetiology in 18 patients, gall stones in 7, and in 1 it was idiopathic. The mean duration of symptoms before patients were taken up for surgery was 47.2 (±34.8) days. The mean computed tomography severity index was 7.7 (±1.2). All patients had undergone video-assisted retroperitoneal necrosectomy through a limited left lumbar incision. Post-operative lavage was given through drains placed in the retroperitoneum. Three patients required re-exploration. Eleven patients developed complications and there were two mortalities. The median intensive care unit (ICU) stay was 4 days (range 2-14 days). The mean post-operative hospital stay was 22.5 (±6.6) days. Video-assisted translumbar retroperitoneal necrosectomy followed by closed lavage of infected pancreatic necrosis in select cases of infected pancreatic necrosis was associated with a low rate of ICU stay, hospital stay and need for re-entry. © 2013 The Authors. ANZ Journal of Surgery © 2013 Royal Australasian College of Surgeons.

  7. Endometrial stromal sarcomas and related high-grade sarcomas: immunohistochemical and molecular genetic study of 31 cases.

    PubMed

    Kurihara, Shuichi; Oda, Yoshinao; Ohishi, Yoshihiro; Iwasa, Atsuko; Takahira, Tomonari; Kaneki, Eisuke; Kobayashi, Hiroaki; Wake, Norio; Tsuneyoshi, Masazumi

    2008-08-01

    Classification and terminology of non-low-grade endometrial sarcomas, which show significant nuclear atypia, have been controversial. Currently, these tumors seem to be classified all together into "undifferentiated endometrial sarcoma (UES)." However, it remains unclear whether these non-low-grade sarcomas are universally "undifferentiated." We divided these sarcomas morphologically into undifferentiated endometrial sarcoma with nuclear uniformity (UES-U) and undifferentiated endometrial sarcoma with nuclear pleomorphism (UES-P), and compared their molecular genetic and immunohistochemical profiles. Eighteen low-grade endometrial stromal sarcomas (ESS-LG), 7 UES-U, and 6 UES-P were examined. All the patients with ESS-LG were still alive, either with or without disease, whereas 4 of the 5 patients with advanced stage UES-U and all 3 of the patients with advanced stage UES-P had died of the disease. JAZF1-JJAZ1 fusion transcript was detected in 6 (50%) out of 12 ESS-LG and in 1 (33%) of 3 UES-U, whereas it was not detected in any of the cases of UES-P. ESS-LG and UES-U frequently showed positive immunoreaction for estrogen receptor (ESS-LG: 94%, UES-U: 57%) and progesterone receptor (ESS-LG: 94%, UES-U: 57%), whereas all the UES-P were negative for these receptors. Nuclear beta-catenin expression was more frequently recognized in ESS-LG (47%) and UES-U (85%), compared with UES-P (33%). Moreover, nuclear accumulation of p53 and TP53 gene missense mutations were limited to 3 UES-P cases. Our data suggest that UES-U shares some molecular genetic and immunohistochemical characteristics with ESS-LG, but UES-P considerably differs from ESS-LG.

  8. Cytokine-induced killer cells eradicate bone and soft-tissue sarcomas.

    PubMed

    Sangiolo, Dario; Mesiano, Giulia; Gammaitoni, Loretta; Leuci, Valeria; Todorovic, Maja; Giraudo, Lidia; Cammarata, Cristina; Dell'Aglio, Carmine; D'Ambrosio, Lorenzo; Pisacane, Alberto; Sarotto, Ivana; Miano, Sara; Ferrero, Ivana; Carnevale-Schianca, Fabrizio; Pignochino, Ymera; Sassi, Francesco; Bertotti, Andrea; Piacibello, Wanda; Fagioli, Franca; Aglietta, Massimo; Grignani, Giovanni

    2014-01-01

    Unresectable metastatic bone sarcoma and soft-tissue sarcomas (STS) are incurable due to the inability to eradicate chemoresistant cancer stem-like cells (sCSC) that are likely responsible for relapses and drug resistance. In this study, we investigated the preclinical activity of patient-derived cytokine-induced killer (CIK) cells against autologous bone sarcoma and STS, including against putative sCSCs. Tumor killing was evaluated both in vitro and within an immunodeficient mouse model of autologous sarcoma. To identify putative sCSCs, autologous bone sarcoma and STS cells were engineered with a CSC detector vector encoding eGFP under the control of the human promoter for OCT4, a stem cell gene activated in putative sCSCs. Using CIK cells expanded from 21 patients, we found that CIK cells efficiently killed allogeneic and autologous sarcoma cells in vitro. Intravenous infusion of CIK cells delayed autologous tumor growth in immunodeficient mice. Further in vivo analyses established that CIK cells could infiltrate tumors and that tumor growth inhibition occurred without an enrichment of sCSCs relative to control-treated animals. These results provide preclinical proof-of-concept for an effective strategy to attack autologous sarcomas, including putative sCSCs, supporting the clinical development of CIK cells as a novel class of immunotherapy for use in settings of untreatable metastatic disease.

  9. Adrenal Ewing's Sarcoma in an Elderly Man.

    PubMed

    Toda, Kazuyoshi; Ishii, Sumiyasu; Yasuoka, Hidetoshi; Nishioka, Masaki; Kobayashi, Takayuki; Horiguchi, Kazuhiko; Tomaru, Takuya; Ozawa, Atsushi; Shibusawa, Nobuyuki; Satoh, Tetsurou; Koshi, Hiromi; Segawa, Atsuki; Shimizu, Shin-Ichi; Oyama, Tetsunari; Yamada, Masanobu

    2018-02-15

    Ewing's sarcoma usually arises in the bones of children and adolescents. We herein report a 74-year-old man with Ewing's sarcoma in the adrenal gland. The diagnosis was confirmed by a genetic test, pathological studies, and several imaging studies. He already had multiple liver metastases when he was transferred to our hospital and died on the 37th day. The diagnosis was further confirmed by autopsy studies. Adrenal Ewing's sarcoma is very rare, and our patient was older than other reported cases. Ewing's sarcoma should be considered even in elderly patients with adrenal tumors.

  10. Castleman's disease imitating adrenal mass in the retroperitoneal area.

    PubMed

    Koç, Gökhan; Turk, Hakan; Un, Sıtkı; Isoglu, Cemal Selcuk; Zorlu, Ferruh

    2015-01-01

    Castleman's disease (CD) is a non-clonal lymph node hyperplasia, mostly seen in the mediastinum. It has various clinical and pathological outcomes. There are different treatments because of its rare occurance and heterogenity. We present 2 cases which were referred to our clinic as retroperitoneal mass and diagnosed as CD after surgical resection.

  11. Adoptive cell therapy for sarcoma

    PubMed Central

    Mata, Melinda; Gottschalk, Stephen

    2015-01-01

    Current therapy for sarcomas, though effective in treating local disease, is often ineffective for patients with recurrent or metastatic disease. To improve outcomes, novel approaches are needed and cell therapy has the potential to meet this need since it does not rely on the cytotoxic mechanisms of conventional therapies. The recent successes of T-cell therapies for hematological malignancies have led to renewed interest in exploring cell therapies for solid tumors such as sarcomas. In this review, we will discuss current cell therapies for sarcoma with special emphasis on genetic approaches to improve the effector function of adoptively transferred cells. PMID:25572477

  12. Current state of pediatric sarcoma biology and opportunities for future discovery: A report from the sarcoma translational research workshop.

    PubMed

    Hingorani, Pooja; Janeway, Katherine; Crompton, Brian D; Kadoch, Cigall; Mackall, Crystal L; Khan, Javed; Shern, Jack F; Schiffman, Joshua; Mirabello, Lisa; Savage, Sharon A; Ladanyi, Marc; Meltzer, Paul; Bult, Carol J; Adamson, Peter C; Lupo, Philip J; Mody, Rajen; DuBois, Steven G; Parsons, D Williams; Khanna, Chand; Lau, Ching; Hawkins, Douglas S; Randall, R Lor; Smith, Malcolm; Sorensen, Poul H; Plon, Sharon E; Skapek, Stephen X; Lessnick, Stephen; Gorlick, Richard; Reed, Damon R

    2016-05-01

    Sarcomas are a rare subgroup of pediatric cancers comprised of a variety of bone and soft-tissue tumors. While significant advances have been made in improving outcomes of patients with localized pediatric sarcomas since the addition of systemic chemotherapy to local control many decades ago, outcomes for patients with metastatic and relapsed sarcoma remain poor with few novel therapeutics identified to date. With the advent of new technologies to study cancer genomes, transcriptomes and epigenomes, our understanding of sarcoma biology has improved tremendously in a relatively short period of time. However, much remains to be accomplished in this arena especially with regard to translating all of this new knowledge to the bedside. To this end, a meeting was convened in Philadelphia, PA, on April 18, 2015 sponsored by the QuadW foundation, Children's Oncology Group and CureSearch for Children's Cancer that brought together sarcoma clinicians and scientists from North America to review the current state of pediatric sarcoma biology and ongoing/planned genomics based clinical trials in an effort to identify and bridge knowledge gaps that continue to exist at present. At the conclusion of the workshop, three key objectives that would significantly further our understanding of sarcoma were identified and a proposal was put forward to develop an all-encompassing pediatric sarcoma biology protocol that would address these specific needs. This review summarizes the proceedings of the workshop. Copyright © 2016. Published by Elsevier Inc.

  13. Sarcoma-The standard-bearer in cancer discovery.

    PubMed

    Potter, Jared W; Jones, Kevin B; Barrott, Jared J

    2018-06-01

    Sarcoma is a rare tumor type that occurs most frequently in connective tissue. Despite its uncommon occurrence, sarcoma research has provided the means for groundbreaking research that has advanced our understanding of general cancer mechanisms. It is through sarcoma research that the pioneering efforts of cancer immunotherapy were explored, that we understand the inherent genetic nature of cancer mutations, and that we appreciate the subclassification of general cancer types to make more accurate prognoses. This review explores the brief history of sarcoma research and what sarcomas can still teach us about the future of cancer research, especially in regard to novel immunotherapy targets, the role of epigenetics in disease progression and chemoresistance, and the benefits of more focused clinical trials. Copyright © 2018 The Authors. Published by Elsevier B.V. All rights reserved.

  14. Epithelioid sarcoma: a diagnostic challenge.

    PubMed

    Pai, Kanthilatha K; Pai, Sathish B; Sripathi, H; Rao, Purnima

    2006-01-01

    Epithelioid sarcoma is an uncommon slow-growing soft tissue malignancy, associated with a high incidence of local recurrence and metastasis. We report a 26-year-old male with epithelioid sarcoma on the right palm with a long history of over seven years, which was initially misdiagnosed as cutaneous tuberculosis and epithelioid hemangioendothelioma, as a result of which the treatment was delayed. No metastasis was found in our patient. The patient was referred to the oncology centre where he underwent wide excision of the lesion followed by radiotherapy. The review of the literature including clinical and histological differential diagnosis is presented as it mimics inflammatory, benign tumors as well as other malignant conditions.

  15. Ewing's sarcoma of the patella.

    PubMed

    Gorelik, Natalia; Dickson, Brendan C; Wunder, Jay S; Bleakney, Robert

    2013-05-01

    Ewing's sarcoma is a relatively rare malignancy, occurring mainly between 4 and 25 years of age. It usually arises from the pelvis, followed by the femur, tibia, and remainder of both the long bones of the extremities and flat bones of the axial skeleton. To the best of our knowledge, Ewing's sarcoma of the patella has never been reported previously. Patellar tumors occur infrequently and represent an uncommon etiology of anterior knee pain. We describe the rare case of a 41-year-old man who presented with a 3-4 month history of escalating right anterior knee pain and swelling. Imaging demonstrated an aggressive patellar tumor with an adjacent soft tissue mass. The diagnosis of Ewing's sarcoma was confirmed by pathology. Physicians should be aware of atypical locations for Ewing's sarcoma and, conversely, of rare tumors arising in the patella and accounting for anterior knee pain. Early recognition of such malignancies allows prompt initiation of treatment, hence improving prognosis.

  16. Primary Ewing's Sarcoma of the temporal bone in an infant.

    PubMed

    Goudarzipour, Kourosh; Shamsian, Shahin; Alavi, Samin; Nourbakhsh, Kazem; Aghakhani, Roxana; Eydian, Zahra; Arzanian, Mohammad Taghi

    2015-04-01

    Introduction : Ewing's sarcoma is the second most common primary malignant tumor of bone found in children after Osteosarcoma. It accounts for 4-9% of primary malignant bone tumors and it affects bones of the skull or face in only 1-4% of cases. Hence it rarely affects the head and neck. Subject and Method : In this case report, we describe a case of primary Ewing's sarcoma occurring in the temporal bone. The tumor was surgically excised, and the patient underwent chemotherapy for ten months. Results : Neither recurrence nor distant metastasis was noted in these 10 months after surgery but about 18 months after surgery our patient was expired. Conclusion : Although the prognosis of Ewing's sarcoma is generally poor because of early metastasis to the lungs and to other bones, a review of the article suggested that Ewing's sarcoma occurring in the skull can often be successfully managed by intensive therapy with radical excision and chemotherapy. This result was supported by the case reported here.

  17. Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.

    PubMed

    Tawbi, Hussein A; Burgess, Melissa; Bolejack, Vanessa; Van Tine, Brian A; Schuetze, Scott M; Hu, James; D'Angelo, Sandra; Attia, Steven; Riedel, Richard F; Priebat, Dennis A; Movva, Sujana; Davis, Lara E; Okuno, Scott H; Reed, Damon R; Crowley, John; Butterfield, Lisa H; Salazar, Ruth; Rodriguez-Canales, Jaime; Lazar, Alexander J; Wistuba, Ignacio I; Baker, Laurence H; Maki, Robert G; Reinke, Denise; Patel, Shreyaskumar

    2017-11-01

    Patients with advanced sarcomas have a poor prognosis and few treatment options that improve overall survival. Chemotherapy and targeted therapies offer short-lived disease control. We assessed pembrolizumab, an anti-PD-1 antibody, for safety and activity in patients with advanced soft-tissue sarcoma or bone sarcoma. In this two-cohort, single-arm, open-label, phase 2 study, we enrolled patients with soft-tissue sarcoma or bone sarcoma from 12 academic centres in the USA that were members of the Sarcoma Alliance for Research through Collaboration (SARC). Patients with soft-tissue sarcoma had to be aged 18 years or older to enrol; patients with bone sarcoma could enrol if they were aged 12 years or older. Patients had histological evidence of metastatic or surgically unresectable locally advanced sarcoma, had received up to three previous lines of systemic anticancer therapy, had at least one measurable lesion according to the Response Evaluation Criteria In Solid Tumors version 1.1, and had at least one lesion accessible for biopsy. All patients were treated with 200 mg intravenous pembrolizumab every 3 weeks. The primary endpoint was investigator-assessed objective response. Patients who received at least one dose of pembrolizumab were included in the safety analysis and patients who progressed or reached at least one scan assessment were included in the activity analysis. Accrual is ongoing in some disease cohorts. This trial is registered with ClinicalTrials.gov, number NCT02301039. Between March 13, 2015, and Feb 18, 2016, we enrolled 86 patients, 84 of whom received pembrolizumab (42 in each disease cohort) and 80 of whom were evaluable for response (40 in each disease cohort). Median follow-up was 17·8 months (IQR 12·3-19·3). Seven (18%) of 40 patients with soft-tissue sarcoma had an objective response, including four (40%) of ten patients with undifferentiated pleomorphic sarcoma, two (20%) of ten patients with liposarcoma, and one (10%) of ten patients

  18. Oncological outcomes of patients with Ewing's sarcoma: is there a difference between skeletal and extra-skeletal Ewing's sarcoma?

    PubMed

    Pradhan, A; Grimer, R J; Spooner, D; Peake, D; Carter, S R; Tillman, R M; Abudu, A; Jeys, L

    2011-04-01

    The aim of this study was to identify whether there was any difference in patient, tumour, treatment or outcome characteristics between patients with skeletal or extra-skeletal Ewing's sarcoma. We identified 300 patients with new primary Ewing's sarcoma diagnosed between 1980 and 2005 from the centres' local database. There were 253 (84%) with skeletal and 47 (16%) with extra-skeletal Ewing's sarcomas. Although patients with skeletal Ewing's were younger (mean age 16.8 years) than those with extra-skeletal Ewing's sarcoma (mean age 27.5 years), there was little difference between the groups in terms of tumour stage or treatment. Nearly all the patients were treated with chemotherapy and most had surgery. There was no difference in the overall survival of patients with skeletal (64%) and extra-skeletal Ewing's sarcoma (61%) (p = 0.85), and this was also the case when both groups were split by whether they had metastases or not. This large series has shown that the oncological outcomes of Ewing's sarcoma are related to tumour characteristics and patient age, and not determined by whether they arise in bone or soft tissue.

  19. Radiation-induced sarcoma of the thyroid

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Griem, K.L.; Robb, P.K.; Caldarelli, D.D.

    1989-08-01

    A 23-year-old white man presented with a thyroid mass 12 years after receiving high-dose radiotherapy for a T2 and N1 lymphoepithelioma of the nasopharynx. Following subtotal thyroidectomy, a histopathologic examination revealed liposarcoma of the thyroid gland. The relationship between sarcomas and irradiation is described and Cahan and colleagues' criteria for radiation-induced sarcomas are reviewed. To our knowledge, we are presenting the first such case of a radiation-induced sarcoma of the thyroid gland.

  20. Molecular Approaches to Sarcoma Therapy

    PubMed Central

    Olsen, R. J.; Tarantolo, S. R.

    2002-01-01

    Soft tissue sarcomas comprise a heterogeneous group of aggressive tumors that have a relatively poor prognosis. Although conventional therapeutic regimens can effectively cytoreduce the overall tumor mass, they fail to consistently achieve a curative outcome. Alternative gene-based approaches that counteract the underlying neoplastic process by eliminating the clonal aberrations that potentiate malignant behavior have been proposed. As compared to the accumulation of gene alterations associated with epithelial carcinomas, sarcomas are frequently characterized by the unique presence of a single chromosomal translocation in each histological subtype. Similar to the Philadelphia chromosome associated with CML, these clonal abnormalities result in the fusion of two independent unrelated genes to generate a unique chimeric protein that displays aberrant activity believed to initiate cellular transformation. Secondary gene mutations may provide an additional growth advantage that further contributes to malignant progression. The recent clinical success of the tyrosine kinase inhibitor, STI571, suggests that therapeutic approaches specifically directed against essential survival factors in sarcoma cells may be effective. This review summarizes published approaches targeting a specific molecular mechanism associated with sarcomagenesis. The strategy and significance of published translational studies in six distinct areas are presented. These include: (1) the disruption of chimeric transcription factor activity; (2) inhibition of growth stimulatory post-translational modifications; (3) restoration of tumor suppressor function; (4) interference with angiogenesis; (5) induction of apoptotic pathways; and (6) introduction of toxic gene products. The potential for improving outcomes in sarcoma patients and the conceptual obstacles to be overcome are discussed. PMID:18521343

  1. Robot-assisted renal transplantation using the retroperitoneal approach (RART) with more than one year follow up: Description of the technique and results.

    PubMed

    Bruyère, F; Pradère, B; Faivre d'Arcier, B; Boutin, J-M; Buchler, M; Brichart, N

    2018-01-01

    To describe the technique and report our first experience of robotic-assisted renal transplantation (RART) with more than one year follow up. In our center the first case of RART was realized in October 2013 with a cadaveric graft. We used the combined extra- and intraperitoneal robot assisted laparoscopic route with extraperitoneal positioning of the graft and intraperitoneal transplantation. The patient was placed in the supine position with arms along the body; the robot came from the right inferior part of the patient. Access to the retroperitoneal space was obtained using an Alexis trocar that permitted the insertion of the kidney with ice without losing the pneumoperitoneum. Ports included a 12-mm camera port (placed under the ombilicus), two 8-mm robotic ports (placed 6cms laterally from the previous port) and a 12-mm assistant port (placed between the upper port and the ombilic). All the pre-, per- and postoperative data were prospectively included in a database. We report the results of the initial experience of RART, performed with more than one year follow-up. This technique is the first described using the retroperitoneal approach that is the routine approach for conventional open renal transplantation. This approach permitted to perform excellent arterial, veinous and ureteral anastomosis. Eight cases of RART were conducted between October 2013 and November 2015 (five men and three women). The average age was 58 years (range 39-75years). The average body mass index was 28 (range 22-38). Five patients had history of abdominal surgery and were dialyzed for 30 months on average (range 3-63months). Three left and five right cadavers kidneys were transplanted in the right iliac fossa. The mean graft size was 109mm (range 90-130). The mean length of the incision for insertion of the graft was 60 mms (40-100mms). Mean warm ischemia time was 63minutes (range 46-84). The total operative time was 200minutes (149-245). No patient was transfused during surgery and

  2. [Extraskeletal Ewing's sarcoma].

    PubMed

    Baram, J; Tichler, T; Nass, D; Brenner, H J

    1992-01-01

    5 patients diagnosed as having extraskeletal Ewing's sarcoma have been referred to our adult oncology unit since 1980. All were men, ranging in age from 18-57 (mean 32 years). The primary tumor was located on the trunk in 4 and in an extremity in 1. Wide tumor excision was feasible in only 2. 3 died within 27 months and 2 are alive, 13 and 67 months, respectively, following diagnosis. This study demonstrates the highly aggressive nature of extraskeletal Ewing's sarcoma and the need for early diagnosis and efficient chemotherapy.

  3. Nonpalpable testicular pure seminoma with elevated serum alpha-fetoprotein presenting with retroperitoneal metastasis: a case report.

    PubMed

    Iwatsuki, Shoichiro; Naiki, Taku; Kawai, Noriyasu; Etani, Toshiki; Iida, Keitaro; Ando, Ryosuke; Nagai, Takashi; Okada, Atsushi; Tozawa, Keiichi; Sugiyama, Yosuke; Yasui, Takahiro

    2016-05-05

    Patients with a primary pure seminoma in the testis who have elevated serum alpha-fetoprotein are rare and should be treated as patients with nonseminomatous germ cell tumors. However, nonpalpable testicular tumors in this condition have never been reported. We describe a case of nonpalpable pure testicular seminoma with elevated serum alpha-fetoprotein presenting retroperitoneal metastasis. A 29-year-old Asian man was referred to our hospital with right flank pain. Computed tomography showed a mass located between his aorta and inferior vena cava, but a testicular tumor was not detected. His serum levels of lactate dehydrogenase, alpha-fetoprotein, and DUPAN-2 were high. Although no tumor or nodule was palpable in his testis, ultrasonography revealed multiple low echoic lesions in his right testicular parenchyma. He was diagnosed with right testicular cancer with retroperitoneal lymph node metastasis and underwent right high orchiectomy. A pathological examination revealed pure seminoma and no nonseminomatous components were found in the specimen. Three courses of induction systemic chemotherapy (cisplatin, etoposide, and bleomycin) normalized his serum alpha-fetoprotein and DUPAN-2 levels. Three additional courses of chemotherapy (etoposide and bleomycin) were performed, and treatment was completed with laparoscopic retroperitoneal lymph node dissection. Pathology of the dissected specimen showed fibrous and necrotic tissue with no viable cells. He is alive without recurrence 54 months after orchiectomy. We report a case of pure testicular seminoma with elevated serum alpha-fetoprotein and DUPAN-2 presenting retroperitoneal metastasis. We recommend an ultrasound examination of bilateral testes when large retroperitoneal tumors are detected in young men, even if a mass is not palpable in the scrotum.

  4. Is There a Predisposition Gene for Ewing's Sarcoma?

    PubMed Central

    Randall, R. L.; Lessnick, S. L.; Jones, K. B.; Gouw, L. G.; Cummings, J. E.; Cannon-Albright, L.; Schiffman, J. D.

    2010-01-01

    Ewing's sarcoma is a highly malignant tumor of children and young adults. The molecular mechanisms that underlie Ewing's Sarcoma development are beginning to be understood. For example, most cases of this disease harbor somatic chromosomal translocations that fuse the EWSR1 gene on chromosome 22 with members of the ETS family. While some cooperative genetic events have been identified, such as mutations in TP53 or deletions of the CDKN2A locus, these appear to be absent in the vast majority of cases. It is therefore uncertain whether EWS/ETS translocations are the only consistently present alteration in this tumor, or whether there are other recurrent abnormalities yet to be discovered. One method to discover such mutations is to identify familial cases of Ewing's sarcoma and to then map the susceptibility locus using traditional genetic mapping techniques. Although cases of sibling pairs with Ewing's sarcoma exist, familial cases of Ewing's sarcoma have not been reported. While Ewing's sarcoma has been reported as a 2nd malignancy after retinoblastoma, significant associations of Ewing's sarcoma with classic tumor susceptibility syndromes have not been identified. We will review the current evidence, or lack thereof, regarding the potential of a heritable condition predisposing to Ewing's sarcoma. PMID:20300555

  5. Perivascular epithelioid cell tumor located retroperitoneally with pulmonary lymphangioleiomyomatosis: report of a case.

    PubMed

    Pata, Giacomo; Tironi, Andrea; Solaini, Leonardo; Tiziano, Travaglia; Ragni, Fulvio

    2014-03-01

    Perivascular epithelioid cell neoplasms, also known as "PEComas", are unusual mesenchymal tumors, exhibiting perivascular epithelioid cell differentiation and characterized by a mixed myogenic and melanocytic phenotype. "PEComas not otherwise specified" (PEComas-NOS) are especially rare; consequently, there are no published large series, but only case reports. These tumors are rarely located retroperitoneally, with only about 15 such cases reported. We report a case of pulmonary diffuse lymphangioleiomyomatosis with large retroperitoneal PEComa-NOS in a 66-year-old woman. Treatment consisted only of tumor resection, without additional adjuvant therapy. We emphasize the importance of correct immunohistochemistry diagnosis, initiation of recommended treatment, and surveillance of this unique family of tumors.

  6. BCOR-CCNB3 Fusions Are Frequent in Undifferentiated Sarcomas of Male Children

    PubMed Central

    Peters, Tricia L.; Kumar, Vijetha; Polikepahad, Sumanth; Lin, Frank Y.; Sarabia, Stephen F.; Liang, Yu; Wang, Wei-Lien; Lazar, Alexander J.; Doddapaneni, Harsha Vardhan; Chao, Hsu; Muzny, Donna M.; Wheeler, David A.; Okcu, M. Fatih; Plon, Sharon E.; Hicks, M. John; López-Terrada, Dolores; Parsons, D. Williams; Roy, Angshumoy

    2014-01-01

    The BCOR-CCNB3 fusion gene, resulting from a chromosome X paracentric inversion, was recently described in translocation-negative ‘Ewing-like’ sarcomas arising in bone and soft tissue. Genetic subclassification of undifferentiated unclassified sarcomas may potentially offer markers for reproducible diagnosis and substrates for therapy. Using whole transcriptome paired end RNA sequencing (RNA-seq) we unexpectedly identified BCOR-CCNB3 fusion transcripts in an undifferentiated spindle cell sarcoma. RNA-seq results were confirmed through direct RT-PCR of tumor RNA and cloning of the genomic breakpoints from tumor DNA. Five additional undifferentiated sarcomas with BCOR-CCNB3 fusions were identified in a series of 42 pediatric and adult unclassified sarcomas. Genomic breakpoint analysis demonstrated unique breakpoint locations in each case at the DNA level even though the resulting fusion mRNA was identical in all cases. All patients with BCOR-CCNB3 sarcoma were males diagnosed in mid-childhood (7-13 years of age). Tumors were equally distributed between axial and extra-axial locations. Five of the six tumors were soft tissue lesions with either predominant spindle cell morphology or spindle cell areas interspersed with ovoid to round cells. CCNB3 immunohistochemistry showed strong nuclear positivity in 5 tumors prior to oncologic therapy, but was patchy to negative in post-treatment tumor samples. An RT-PCR assay developed to detect the fusion transcript in archival formalin-fixed tissue was positive in all 6 cases, with high sensitivity and specificity in both pre- and post-treated samples. This study adds to recent reports on the clinicopathologic spectrum of BCOR-CCNB3 fusion-positive sarcomas, a newly-emerging entity within the undifferentiated unclassified sarcoma category, and describes a simple RT-PCR assay that in conjunction with CCNB3 immunohistochemistry can be useful in diagnosing these tumors. PMID:25360585

  7. Extraskeletal Ewing's Sarcoma: insight into a ten years follow-up.

    PubMed

    Zitelli, A; Manfredelli, S; Brunotti, G; Marcantonio, M; Pontone, S; Angelici, A

    2013-01-01

    Extraskeletal Ewing's sarcoma is a rare malignant soft tissue tumor, classified within the Ewing's Sarcoma Family Tumors. While the classical Ewing's Sarcoma affects mainly the bone during youth, the Extraskeletal histotype differs for age incidence, primary location and prognosis. Peak incidence and typical location are during adolescence and in the extremities respectively. We report a 30 year old woman case with a positive outcome after ten years from first diagnosis of Extraskeletal Ewing's sarcoma. Treatment was achieved through surgical resection plus adjuvant chemoradiotherapy derived from EW93 and IRS III trials. Conclusion. Our report represents an unusual case due to age of presentation, neoplasm location and long survival reached. In last decades several trials results demonstrated that long survival could be achieved by combined surgery and adjuvant multi-drug treatment.

  8. Testicular myeloid sarcoma: case report.

    PubMed

    Zago, Luzia Beatriz Ribeiro; Ladeia, Antônio Alexandre Lisbôa; Etchebehere, Renata Margarida; de Oliveira, Leonardo Rodrigues

    2013-01-01

    Myeloid sarcomas are extramedullary solid tumors composed of immature granulocytic precursor cells. In association with acute myeloid leukemia and other myeloproliferative disorders, they may arise concurrently with compromised bone marrow related to acute myeloid leukemia, as a relapsed presentation, or occur as the first manifestation. The testicles are considered to be an uncommon site for myeloid sarcomas. No therapeutic strategy has been defined as best but may include chemotherapy, radiotherapy and/or hematopoietic stem cell transplantation. This study reports the evolution of a patient with testicular myeloid sarcoma as the first manifestation of acute myeloid leukemia. The patient initially refused medical treatment and died five months after the clinical condition started.

  9. A novel CIC-FOXO4 gene fusion in undifferentiated small round cell sarcoma: a genetically distinct variant of Ewing-like sarcoma.

    PubMed

    Sugita, Shintaro; Arai, Yasuhito; Tonooka, Akiko; Hama, Natsuko; Totoki, Yasushi; Fujii, Tomoki; Aoyama, Tomoyuki; Asanuma, Hiroko; Tsukahara, Tomohide; Kaya, Mitsunori; Shibata, Tatsuhiro; Hasegawa, Tadashi

    2014-11-01

    Differential diagnosis of small round cell sarcomas (SRCSs) grouped under the Ewing sarcoma family of tumors (ESFT) can be a challenging situation for pathologists. Recent studies have revealed that some groups of Ewing-like sarcoma show typical ESFT morphology but lack any EWSR1-ETS gene fusions. Here we identified a novel gene fusion, CIC-FOXO4, in a case of Ewing-like sarcoma with a t(X;19)(q13;q13.3) translocation. The patient was a 63-year-old man who had an asymptomatic, 30-mm, well-demarcated, intramuscular mass in his right posterior neck, and imaging findings suggested a diagnosis of high-grade sarcoma. He was treated with complete resection and subsequent radiotherapy and chemotherapy. He was alive without local recurrence or distant metastasis 6 months after the operation. Histologic examination revealed SRCS with abundant desmoplastic fibrous stroma suggesting a desmoplastic small round cell tumor. Immunohistochemical analysis showed weak to moderate and partial staining for MIC2 (CD99) and WT1, respectively. High-throughput transcriptome sequencing revealed a gene fusion, and the genomic rearrangement between the CIC and FOXO4 genes was identified by fluorescence in situ hybridization. Aside from the desmoplastic stroma, the CIC-FOXO4 fusion sarcoma showed morphologic and immunohistochemical similarity to ESFT and Ewing-like sarcomas, including the recently described CIC-DUX4 fusion sarcoma. Although clinicopathologic analysis with additional cases is necessary, we conclude that CIC-FOXO4 fusion sarcoma is a new type of Ewing-like sarcoma that has a specific genetic signature. These findings have important implications for the differential diagnosis of SRCS.

  10. Medial arcuate ligament: a new anatomic landmark facilitates the location of the renal artery in retroperitoneal laparoscopic renal surgery.

    PubMed

    Cai, Wei; Li, Hong Zhao; Zhang, Xu; Song, Yong; Ma, Xin; Dong, Jun; Chen, Wenzheng; Chen, Guang-Fu; Xu, Yong; Lu, Jin Shan; Wang, Bao-Jun; Shi, Tao-Ping

    2013-01-01

    The purpose of this study was to introduce a new method for locating the renal artery during retroperitoneal laparoscopic renal surgery. The medial arcuate ligament (MAL) is a tendinous arch in the fascia under the diaphragm that arches across the psoas major muscle and is attached medially to the side of the first or the second lumbar vertebra. The renal artery arises at the level of the intervertebral disc between the L1 and L2 vertebrae. We evaluate the role of the MAL that serves as an anatomic landmark for locating the renal artery during retroperitoneal laparoscopic renal surgery. There is a reproducible consistent anatomic relationship between MAL and the renal artery in 210 cases of retroperitoneal laparoscopic renal surgery. Two main types of the MAL, the "narrow arch" and the "fascial band" types, can be observed. MAL can serve as an accurate and reproducible anatomic landmark for the identification of the renal artery during retroperitoneal laparoscopic renal surgery.

  11. Response to pazopanib in two pediatric patients with pretreated relapsing synovial sarcoma.

    PubMed

    Casanova, Michela; Basso, Eleonora; Magni, Chiara; Bergamaschi, Luca; Chiaravalli, Stefano; Carta, Roberto; Tirtei, Elisa; Massimino, Maura; Fagioli, Franca; Ferrari, Andrea

    2017-01-21

    Pazopanib is an oral multikinase inhibitor that has proved effective in adults treated for relapsing soft tissue sarcoma and synovial sarcoma in particular. Two cases are reported here of pediatric patients with pretreated relapsing synovial sarcoma whose tumors showed a prolonged response to pazopanib given on compassionate grounds. These results suggest that new agents found effective in adult patients might achieve similar results in adolescents with the same disease. Facilitating the availability of new drugs for children and adolescents is a major challenge for pediatric oncologists.

  12. Ewing's sarcoma precursors are highly enriched in embryonic osteochondrogenic progenitors.

    PubMed

    Tanaka, Miwa; Yamazaki, Yukari; Kanno, Yohei; Igarashi, Katsuhide; Aisaki, Ken-ichi; Kanno, Jun; Nakamura, Takuro

    2014-07-01

    Ewing's sarcoma is a highly malignant bone tumor found in children and adolescents, and the origin of this malignancy is not well understood. Here, we introduced a Ewing's sarcoma-associated genetic fusion of the genes encoding the RNA-binding protein EWS and the transcription factor ETS (EWS-ETS) into a fraction of cells enriched for osteochondrogenic progenitors derived from the embryonic superficial zone (eSZ) of long bones collected from late gestational murine embryos. EWS-ETS fusions efficiently induced Ewing's sarcoma-like small round cell sarcoma formation by these cells. Analysis of the eSZ revealed a fraction of a precursor cells that express growth/differentiation factor 5 (Gdf5), the transcription factor Erg, and parathyroid hormone-like hormone (Pthlh), and selection of the Pthlh-positive fraction alone further enhanced EWS-ETS-dependent tumor induction. Genes downstream of the EWS-ETS fusion protein were quite transcriptionally active in eSZ cells, especially in regions in which the chromatin structure of the ETS-responsive locus was open. Inhibition of β-catenin, poly (ADP-ribose) polymerase 1 (PARP1), or enhancer of zeste homolog 2 (EZH2) suppressed cell growth in a murine model of Ewing's sarcoma, suggesting the utility of the current system as a preclinical model. These results indicate that eSZ cells are highly enriched in precursors to Ewing's sarcoma and provide clues to the histogenesis of Ewing's sarcoma in bone.

  13. [Pancreatic mucinous cystadenoma doubly complicated by acute pancreatitis and retroperitoneal rupture].

    PubMed

    Maghrebi, Houcine; Makni, Amine

    2017-01-01

    Mucinous cystadenomas are benign tumors with malignant potential. They are often revealed by non-specific abdominal pain, jaundice or an episode of acute pancreatitis. We here report an exceptional case of mucinous cystadenoma doubly complicated by acute pancreatitis and retroperitoneal rupture. The study involved a 30-year old non-weighted female patient, presenting with epigastric pain associated with left hypochondrium evolving over the last three months and which had intensified without fever or jaundice in the last 3 days. Clinical examination showed impingement on palpation of the epigastrium and of the left hypochondrium. There was no palpable mass. Laboratory tests were without abnormalities, except for lipasemia that was 8-times the upper normal. Abdominal CT scan showed bi-loculated cystic mass in the pancreas tail, measuring 111 mm * 73 mm, with a thin wall and a fluid content, associated with an infiltration of the left perirenal fascia. MRI (Panel A) showed mucinous cystadenoma with retroperitoneal rupture. The caudal portion of the main pancreatic duct was slightly dilated and communicated with the pancreatic cyst. The patient underwent surgery via bi-sub-costal approach. A cystic mass in the pancreas tail with retroperitoneal rupture associated with acute pancreatitis (outflow of necrotic content from left anterior prerenal space) was found. Caudal splenopancreatectomy was performed (Panel B). The postoperative course was uneventful. The anatomo-pathological examination of the surgical specimen showed pancreatic mucinous cystadenoma with low-grade dysplasia.

  14. Synovial sarcoma: a rare presentation of parapharyngeal mass.

    PubMed

    Shaariyah, Mohd Mokhtar; Mazita, Ami; Masaany, Mansor; Razif, Mohd Yunus; Isa, Mohamed Rose; Asma, Abdullah

    2010-06-01

    Synovial sarcoma is a rare soft tissue sarcoma of the head and neck region involving the parapharyngeal space. The diagnosis of synovial sarcoma can be very challenging to the pathologists. We present a rare case of parapharyngeal synovial sarcoma in a young female patient who had a two-month history of left cervical intumescent mass at level II. The fine needle aspiration cytology of the mass was proved inconclusive. Transcervical excision of the mass was performed and the first case of parapharyngeal sarcoma was identified in our center by fluorescence in situ hybridization (FISH) technique. Repeat imaging revealed residual tumor. The patient successfully underwent a second excision of the residual tumor and received adjuvant radiotherapy.

  15. Ewing's Sarcoma of the Adrenal Gland.

    PubMed

    Pal, Dilip Kumar; Chandra, Vipin; Ranjan, Kumar Rajiv; Chakrabortty, Debasis; Banerjee, Manju

    2016-01-01

    Ewing's sarcoma (ES) or primitive neuro-ectodermal tumor (PNET) typically occurs in long or flat bones, the chest wall, extra-skeletal soft tissue, and rarely in solid organs. Incidence of adrenal Ewing's sarcoma is very rare. Here we report a case of Ewing's sarcoma of the right adrenal gland in an 8-year-old girl who presented with an abdominal mass. The huge tumor was managed by preoperative neo-adjuvant chemotherapy followed by surgical resection. She died due to metastasis after five months of surgery.

  16. Retroperitoneal laparoscopy management for ureteral fibroepithelial polyps causing hydronephrosis in children: a report of five cases.

    PubMed

    Dai, L N; Chen, C D; Lin, X K; Wang, Y B; Xia, L G; Liu, P; Chen, X M; Li, Z R

    2015-10-01

    Hydronephrosis is a common disease in children and may be caused by ureteral fibroepithelial polyps (UFP). Ureteral fibroepithelial polyps are rare in children and are difficult to precisely diagnose before surgery. Surgical treatment for symptomatic UFP is recommended. At the present institution, retroperitoneal laparoscopy has been used to treat five boys with UFP since 2006. To highlight the significance of UFP as an etiological factor of hydronephrosis in children and evaluate the applicative value of retroperitoneal laparoscopy in the treatment of children with UFP. Between 2006 and 2013 five boys underwent retroperitoneal laparoscopy at the present institution. They were identified with UFP by review of the clinical database. Detailed data were collected, including: radiographic studies, gross anatomical pathology, and pathology and radiology reports. All boys had been followed up at least every 6 months. All of the boys were aged between 7 and 16 years (mean 9.8 years). The main symptoms were flank pain (all five) and hematuria (three). Radiographic examination showed that all of the boys presented with incomplete ureteral obstruction and hydronephrosis. The ureteral fibroepithelial polyps were located near the left UPJ or the left proximal ureter. All of the boys had the UFP removed: three underwent retroperitoneal laparoscopic dismembered Anderson-Hynes pyeloplasty and polypectomy, and two had retroperitoneal laparoscopic ureteral anastomosis. These polyps were all on the left side and between 15 and 35 mm in length (mean 22 mm) (Figure). All of the boys recovered well and were discharged from hospital. The postoperative histological report confirmed that the specimens were UFP. Hydronephrosis was periodically assessed by ultrasonography (using the same method as pre-surgical ultrasonography) after surgery. Mean follow-up was 33 months (range 6-58 months) and no complications were found afterwards. Ureteral fibroepithelial polyps are rare but rather

  17. Biologic Activity of Autologous, Granulocyte-Macrophage Colony Stimulating Factor Secreting Alveolar Soft Parts Sarcoma and Clear Cell Sarcoma Vaccines

    PubMed Central

    Goldberg, John; Fisher, David E.; Demetri, George D.; Neuberg, Donna; Allsop, Stephen A.; Fonseca, Catia; Nakazaki, Yukoh; Nemer, David; Raut, Chandrajit P.; George, Suzanne; Morgan, Jeffrey A.; Wagner, Andrew J.; Freeman, Gordon J.; Ritz, Jerome; Lezcano, Cecilia; Mihm, Martin; Canning, Christine; Hodi, F. Stephen; Dranoff, Glenn

    2015-01-01

    Purpose Alveolar soft parts sarcoma (ASPS) and clear cell sarcoma (CCS) are rare mesenchymal malignancies driven by chromosomal translocations that activate members of the microphthalmia transcription factor (MITF) family. However, in contrast to malignant melanoma, little is known about their immunogenicity. To learn more about the host response to ASPS and CCS, we conducted a phase I clinical trial of vaccination with irradiated, autologous sarcoma cells engineered by adenoviral mediated gene transfer to secrete granulocyte-macrophage colony stimulating factor (GM-CSF). Experimental Design Metastatic tumors from ASPS and CCS patients were resected, processed to single cell suspensions, transduced with a replication defective adenoviral vector encoding GM-CSF, and irradiated. Immunizations were administered subcutaneously and intradermally weekly times three and then every other week. Results Vaccines were successfully manufactured for 11 of the 12 enrolled patients. Eleven subjects received from 3 to 13 immunizations. Toxicities were restricted to grade 1–2 skin reactions at inoculation sites. Vaccination elicited local dendritic cell infiltrates and stimulated T cell mediated delayed type-hypersensitivity reactions to irradiated, autologous tumor cells. Antibody responses to tissue-type plasminogen activator (tTPA) and angiopoietins-1/2 were detected. Tumor biopsies showed programmed death-1 (PD-1) positive CD8+ T cells in association with PD ligand-1 (PD-L1) expressing sarcoma cells. No tumor regressions were observed. Conclusions Vaccination with irradiated, GM-CSF secreting autologous sarcoma cell vaccines is feasible, safe, and biologically active. Concurrent targeting of angiogenic cytokines and antagonism of the PD-1 negative regulatory pathway might intensify immune-mediated tumor destruction. PMID:25805798

  18. [Primitive cutaneous Ewing's sarcoma: a diagnostic and therapeutic dilemma].

    PubMed

    Delaplace, M; Mélard, P; Perrinaud, A; Goré, C; Vergier, B; Machet, L

    2011-05-01

    Ewing's sarcoma (or peripheral neuroectodermal tumour) is generally found in bone tissue, and a primary dermal site is extremely rare. We report a case of primary cutaneous Ewing's sarcoma in a 21-year-old woman. A 21-year-old woman presented with a scapular lesion that had been slowly developing for one year. The 1-cm lesion was removed and histological examination showed proliferation of small round cells in the dermis. Immunostaining revealed cytoplasmic membrane expression of CD99 and a negative immunoprofile for other small round-cell tumors. Ewing's sarcoma fusion gene transcripts were detected using fluorescence in situ hybridization (FISH). A staging examination revealed no other abnormalities. It was decided to treat the lesion as for osseous Ewing's sarcoma with wide resection followed by systemic adjuvant chemotherapy. Cutaneous Ewing's sarcoma raises concerns about diagnosis and treatment. Owing to the non-specificity of its clinical presentation, histology and immunoprofile, diagnosis of superficial Ewing's sarcoma is difficult and numerous differential diagnoses must be considered. When dealing with a surface tumour, the diagnosis of cutaneous Ewing's sarcoma must be considered. CD99 immunostaining and molecular testing for evidence of EWSR1 rearrangement are useful investigations to confirm the diagnosis. Furthermore, modalities of treatment must be carefully discussed. Cutaneous Ewing's sarcoma is currently treated in the same way as osseous Ewing's sarcoma (wide surgical excision, adjuvant radiotherapy when surgical margins are unsatisfactory, systemic adjuvant chemotherapy, and, in some cases, bone marrow transplant). However, some studies show a more favourable prognosis for cutaneous Ewing's sarcoma than for osseous Ewing's sarcoma. We may thus ask whether such aggressive multimodal treatment is needed. Copyright © 2010 Elsevier Masson SAS. All rights reserved.

  19. Metastatic synovial sarcoma of the scalp: Case report.

    PubMed

    Lippert, Dylan C; Britt, Christopher J; Pflum, Zachary E; Rush, Patrick S; Hartig, Gregory K

    2016-02-01

    Synovial sarcoma is a malignant tumor of soft tissue that is rarely found in the head and neck. Even less common are metastasis within the head and neck. We describe a case of a delayed metastatic synovial sarcoma to the scalp. A man who had been diagnosed and treated 16 years previously for monophasic synovial sarcoma of the groin, presented with a new scalp lesion confirmed to be metastatic monophasic synovial sarcoma. Wide local excision and sentinel lymph node biopsy (SLNB) were performed and adjuvant radiation therapy was deferred. A positron emission tomography (PET)/CT was obtained 3 months after surgery and showed no evidence of local recurrence or metastatic disease. This case report describes a rare case of synovial sarcoma metastasizing to the scalp. The genetic, histopathologic, and clinical features of synovial sarcoma are reviewed with a focus on their manifestation and management within the head and neck. © 2015 Wiley Periodicals, Inc.

  20. Depsipeptide (Romidepsin) in Treating Patients With Metastatic or Unresectable Soft Tissue Sarcoma

    ClinicalTrials.gov

    2017-05-18

    Adult Alveolar Soft-part Sarcoma; Adult Angiosarcoma; Adult Epithelioid Sarcoma; Adult Extraskeletal Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Malignant Hemangiopericytoma; Adult Malignant Mesenchymoma; Adult Neurofibrosarcoma; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Gastrointestinal Stromal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Adult Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  1. Gastric myeloid sarcoma without acute myeloblastic leukemia

    PubMed Central

    Huang, Xiao-Li; Tao, Jin; Li, Jian-Zhong; Chen, Xiao-Liang; Chen, Jian-Ning; Shao, Chun-Kui; Wu, Bin

    2015-01-01

    Myeloid sarcomas (MS) involve extramedullary blast proliferation from one or more myeloid lineages that replace the original tissue architecture, and these neoplasias are called granulocytic sarcomas, chloromas or extramedullary myeloid tumors. Such tumors develop in lymphoid organs, bones (e.g., skulls and orbits), skin, soft tissue, various mucosae, organs, and the central nervous system. Gastrointestinal (GI) involvement is rare, while the occurrence of myeloid sarcomas in patients without leukemia is even rare. Here, we report a case of a 38-year-old man who presented with epigastric pain and progressive jaundice. An upper GI endoscopy had shown extensive multifocal hyperemic fold thickening and the spread of nodular lesions in the body of the stomach. Biopsies from the gastric lesions indicated myeloid sarcoma of the stomach. However, concurrent peripheral blood and bone marrow examinations showed no evidence of acute myeloid leukemia. For diagnosis, the immunohistochemical markers must be checked when evaluating a suspected myeloid sarcoma case. Accurate MS diagnosis determines the appropriate therapy and prognosis. PMID:25717265

  2. Uterine sarcoma

    MedlinePlus

    ... Churchill Livingstone; 2014:chap 88. Crum CP, Laury AR, Hirsch MS, Quick CM, Peters WA. Undifferentiated uterine sarcoma. In: Crum CP, Quick CM, Laury AR, Peters WA, Hirsch MS, eds. Gynecologic and Obstetric ...

  3. Comprehensive and Integrated Genomic Characterization of Adult Soft Tissue Sarcomas.

    PubMed

    2017-11-02

    Sarcomas are a broad family of mesenchymal malignancies exhibiting remarkable histologic diversity. We describe the multi-platform molecular landscape of 206 adult soft tissue sarcomas representing 6 major types. Along with novel insights into the biology of individual sarcoma types, we report three overarching findings: (1) unlike most epithelial malignancies, these sarcomas (excepting synovial sarcoma) are characterized predominantly by copy-number changes, with low mutational loads and only a few genes (TP53, ATRX, RB1) highly recurrently mutated across sarcoma types; (2) within sarcoma types, genomic and regulomic diversity of driver pathways defines molecular subtypes associated with patient outcome; and (3) the immune microenvironment, inferred from DNA methylation and mRNA profiles, associates with outcome and may inform clinical trials of immune checkpoint inhibitors. Overall, this large-scale analysis reveals previously unappreciated sarcoma-type-specific changes in copy number, methylation, RNA, and protein, providing insights into refining sarcoma therapy and relationships to other cancer types. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.

  4. 'Telangiectatic' transformation in soft tissue sarcomas. a clinicopathology analysis of an aggressive feature of high-grade sarcomas.

    PubMed

    Sternheim, Amir; Jin, Xiaolong; Shmookler, Barry; Jelinek, James; Malawer, Martin M

    2008-01-01

    'Telangiectatic' change, which contains a large fluid hemorrhagic component, occurs in a variety of high-grade soft tissue sarcomas. In a retrospective database review, we identified 20 consecutive patients (3%) with 'telangiectatic' change in soft tissue sarcomas. Tumors were located in the thigh (55%), shoulder (15%), calf (15%), upper arm (10%), and buttock in one patient. All 20 tumors were high grade. Histological diagnoses were MFH (40%), leiomyosarcoma (15%), synovial sarcoma (10%), and one each of seven other sarcomas (35%). Tumor size was often large-more than 10 cm (35%), between 5 and 10 cm (60%), and less than 5 cm in one case. A history of contusion to the tumor site followed by swelling was recorded in 30% of patients and 80% presented with a painful mass. On MRI imaging, 60% of tumors appeared to contain more than 50% blood, 50% had a hemosiderin-laden rim, and 55% had well-defined tumor nodules within the wall of the hematoma. Limb-sparing surgery was carried out in 90% of patients, the other 10% underwent primary amputation. The 5-year, event-free survival rate was 30%. Of the patients, 15% presented initially with metastatic disease; in 53%, it developed within 2 years of diagnosis. The overall local recurrence rate was 30%. Telangiectatic transformation in soft tissue sarcomas is a rare feature of aggressive high-grade soft tissue sarcomas and is unique in its clinical presentation, MRI characteristics, pathological pattern, and a tendency for a worse-off prognosis.

  5. NUTM2A-CIC fusion small round cell sarcoma: a genetically distinct variant of CIC-rearranged sarcoma.

    PubMed

    Sugita, Shintaro; Arai, Yasuhito; Aoyama, Tomoyuki; Asanuma, Hiroko; Mukai, Wakako; Hama, Natsuko; Emori, Makoto; Shibata, Tatsuhiro; Hasegawa, Tadashi

    2017-07-01

    CIC-rearranged sarcoma is a new entity of undifferentiated small round cell sarcoma characterized by chimeric fusions with CIC rearrangement. We report a NUTM2A-CIC fusion sarcoma in a 43-year-old woman who died of rapidly progressive disease. Histologic analysis revealed multinodular proliferation of small round tumor cells with mild nuclear pleomorphism. The sclerotic fibrous septa separated the tumor into multiple nodules. Immunohistochemistry showed that the tumor cells were diffusely positive for vimentin, focally positive for cytokeratin, and negative for CD99 and NKX2.2. Tumor cells were also negative for ETV4, which was recently identified as a specific marker for CIC-rearranged sarcoma. High-throughput RNA sequencing of a formalin-fixed, paraffin-embedded clinical sample unveiled a novel NUTM2A-CIC fusion between NUTM2A exon 7 and CIC exon 12, and fluorescence in situ hybridization identified CIC and NUTM2A split signals. This case shared several clinicopathological findings with previously reported CIC-rearranged cases. We recognized the tumor as a genetically distinct variant of CIC-rearranged sarcomas with a novel NUTM2A-CIC fusion. Copyright © 2017. Published by Elsevier Inc.

  6. [Clinical and Pathologic Features of Myeloid Sarcoma].

    PubMed

    Jiang, Ya-Jun; Wang, Hong-Xia; Zhuang, Wan-Chuan; Chen, Hao; Zhang, Chang; Li, Xiu-Mei; Zhu, Gui-Hua; He, Yao

    2017-06-01

    To explore the clinicopathologic features, differential diagnosis and therapy of myeloid sarcoma. The clinical data including clinical manifestations, laboratorial tests, histopathologicical examination, immunohistochemistry and clinical prognosis of 10 patients with myeloid sarcoma were analyzed retrospectively. Among 10 patients, 5 male and 5 female, aged 23 to 71 years old (median = 36 years). 2 cases of myeloid sarcoma were secondary from chronic myeloid leukemia, and 1 cases of myeloid sarcoma occurred after the allogeneic hematopoietic stem cell transplantation due to acute myeloid leukemia, and the others lacked the anamnesis of malignancies. The neoplasms occurred at bone, brain, skin, breast, epididymis, uterine cervix, small intestine, ovary and lymph nodes. Microscopically, the tumor cells were round or oval, which infiltrated diffusely or arranged in single-file. The cytoplasm was scarce and immature eosinophils were scattered. The nuclei were round, oval or focally irregular, and the mitosis was visible. The neoplasms were positive for MPO, CD34, CD43, CD45, CD99 and CD117 by immunohistochemical staining. 4 patients progressed into acute myeloid leukemia from 2 to 10 months after the diagnosis of myeloid sarcoma. All of them achieved complete remission after inductive chemotherapy, but 3 patients relapsed from 3 to 12 months after remission and only survived for 14 to 23 months. 4 patients were treated by using chemotherapy before bone marrow abnormality, and with the disease-free survival for 1 to 48 months. Myeloid sarcoma needs to be distinguished from lymphoblastic lymphoma, Burkitt's lymphoma, blastic plasmacytoid dendritic cell neoplasms and so on. The diagnosis and differential diagnosis of myeloid sarcoma are dependent on the pathological and immunohisto-chemical features. The chemotherapy and allogeneic hematopoietic stem cell transplantation of acute myeloid leukemia are the main methods for treatment of myeloid sarcoma.

  7. Like or Dislike? Impact of Facebook on Ewing Sarcoma Treatment

    PubMed Central

    Schippinger, Michael; Liebmann, Paul; Leithner, Andreas; Bernhardt, Gerwin

    2016-01-01

    Background An increasing number of patients are raising their voices in online forums to exchange health-related information. Facebook is the leading social media platform with more than 1 billion international daily users recorded in the summer of 2015. Facebook has a dynamic audience and is utilized in a number of ways, discussing medical issues being one of them. Ewing sarcoma mainly affects teenagers and young adults. Additionally, many individuals within this age group are regular users of Facebook. However, little is known about the impact of this modern way of communication via Web-based platforms on patients with Ewing sarcoma and their social environment. Objective The aim of this study was to analyze and compare Ewing sarcoma patients’ and relatives’ behavior on Facebook to draw conclusions regarding the impact of Facebook on Ewing sarcoma treatment. Methods We examined a Facebook group named “Ewing Sarcoma Awareness” that is used to exchange information for both patients and relatives regarding Ewing sarcoma. A self-designed questionnaire was used to compare patients’ and relatives’ answers. Additionally, we analyzed all processes (posts, likes, threads, links) in the group for 6 consecutive months. A total of 65 members of the Facebook group (26 patients, 39 relatives) out of 2227 international group members participated in our study. Results More than 70% (46/65) of all participants reported that they use the group Ewing Sarcoma Awareness as a source of information about Ewing sarcoma. Of the participants, 89% (58/65) agreed on our scale from a little to a lot that being in contact with other affected people through the group makes it easier to handle the diagnosis. In this study, 20% (13/65) of all participants reported that the group affected their choice of treatment and 15% (10/65) of participants were influenced in the selection of their specialist. Regarding the recommendation of the Facebook group toward other people, significant

  8. Dural metastasis of Ewing's sarcoma.

    PubMed

    Ben Nsir, Atef; Boughamoura, Mohamed; Maatouk, Mezri; Kilani, Mohamed; Hattab, Nejib

    2013-01-01

    Metastatic Ewing's sarcoma to the central nervous system is an uncommon condition and debate concerning the true origin of its metastases is still up to date. To the best of our knowledge, only two cases of dural metastatic Ewing's sarcoma have been published in the English medical literature. We present an additional case in a 24-year-old female and discuss the pathogenesis of these unusual tumors with review of the relevant literature concerning their treatment and outcome. A 24-year-old female with previous history of pelvis Ewing's sarcoma and recently discovered lung metastases, presented with moderate headache for the past 2 weeks and weakness in her left leg for the past 2 days. Computed tomography scan and magnetic resonance imaging revealed an extra-axial right frontoparietal mass invading the superior sagittal sinus but with clear delineation with brain parenchyma. Imaging features were suggestive of a meningioma as no abnormalities in the skull abutting to the tumor were noted. The patient underwent surgical removal of her tumor. Near total resection was achieved and histological examination showed evidence of metastatic Ewing's sarcoma. Postoperative adjuvant radiation and chemotherapy were administered. The patient improved well postoperatively with full recovery of her motor weakness. She is symptom free with no signs of progression, at most recent follow-up, 8 months after surgery. Despite its rarity, metastatic Ewing's sarcoma must be considered in the differential diagnosis of extra-axial dural masses particularly meningiomas.

  9. Cosmetic and functional outcomes following paramedian and anterolateral retroperitoneal access in anterior lumbar spine surgery.

    PubMed

    Jagannathan, Jay; Chankaew, Ekawut; Urban, Peter; Dumont, Aaron S; Sansur, Charles A; Kern, John; Peeler, Benjamin; Elias, W Jeffrey; Shen, Francis; Shaffrey, Mark E; Whitehill, Richard; Arlet, Vincent; Shaffrey, Christopher I

    2008-11-01

    In this paper, the authors review the functional and cosmetic outcomes and complications in 300 patients who underwent treatment for lumbar spine disease via either an anterior paramedian or conventional anterolateral retroperitoneal approach. Seven surgeons performed anterior lumbar surgeries in 300 patients between August 2004 and December 2006. One hundred and eighty patients were treated with an anterior paramedian approach, and 120 patients with an anterolateral retroperitoneal approach. An access surgeon was used in 220 cases (74%). Postoperative evaluation in all patients consisted of clinic visits, assessment with the modified Scoliosis Research Society-30 instrument, as well as a specific questionnaire relating to wound appearance and patient satisfaction with the wound. At a mean follow-up of 31 months (range 12-47 months), the mean Scoliosis Research Society-30 score (out of 25) was 21.2 in the patients who had undergone the anterior paramedian approach and 19.4 in those who had undergone the anterolateral retroperitoneal approach (p = 0.005). The largest differences in quality of life measures were observed in the areas of pain control (p = 0.001), self-image (p = 0.004), and functional activity (p = 0.003), with the anterior paramedian group having higher scores in all 3 categories. Abdominal bulging in the vicinity of the surgical site was the most common wound complication observed and was reported by 22 patients in the anterolateral retroperitoneal group (18%), and 2 patients (1.1%) in the anterior paramedian group. Exposures of >or= 3 levels with the anterolateral approach were associated with abdominal bulging (p = 0.04), while 1- or 2-level exposures were not (p > 0.05). Overall satisfaction with incisional appearance was higher in patients with an anterior paramedian incision (p = 0.001) and with approaches performed by an access surgeon (p = 0.004). Patients who undergo an anterior paramedian approach to the lumbar spine have a higher quality

  10. Primary Adult Renal Ewing's Sarcoma: A Rare Entity

    PubMed Central

    Mukkunda, Ravindra; Venkitaraman, Ramachandran; Thway, Khin; Min, Toon; Fisher, Cyril; Horwich, Alan; Judson, Ian

    2009-01-01

    Background. Ewing's sarcoma of extraskeletal origin is uncommon and that is of primary renal origin in adults are rare. There is no consensus on the optimal management of Ewing's tumors of renal origin. Methods. A retrospective review of the clinical features, treatment, and outcome of adult patients with primary renal extra-skeletal Ewing's sarcoma who were treated at the Royal Marsden hospital from January 1993–December 2007 is reported. Results. Seven adult patients with primary renal Ewing's sarcoma were identified. All four patients with nonmetastatic disease had radical nephrectomy and received adjuvant chemotherapy +/− radiotherapy. Two developed metastatic disease while on adjuvant chemotherapy, and one patient relapsed after 55 months. The three patients with metastatic disease at presentation did not have nephrectomy and were treated with chemotherapy. All three patients had disease progression with a dismal outcome. Only one patient in the whole group is alive and disease free. The median overall survival was 62.8 months, and the median disease-free survival in patients with nonmetastatic disease after combined modality treatment was 30.3 months. Conclusion. Primary adult renal Ewing's sarcoma is an aggressive tumor with a propensity for early metastasis. Radical nephrectomy with adjuvant combination chemotherapy produced the best results but the outlook remained poor with only one patient experiencing long disease-free survival. PMID:19478963

  11. Survivin, a target to modulate the radiosensitivity of Ewing's sarcoma.

    PubMed

    Greve, B; Sheikh-Mounessi, F; Kemper, B; Ernst, I; Götte, M; Eich, H T

    2012-11-01

    Radiotherapy constitutes an essential element in the multimodal therapy of Ewing's sarcoma. Compared to other sarcomas, Ewing tumors normally show a good response to radiotherapy. However, there are consistently tumors with a radioresistant phenotype, and the underlying mechanisms are not known in detail. Here we investigated the association between survivin protein expression and the radiosensitivity of Ewing's sarcoma in vitro. An siRNA-based knockdown approach was used to investigate the influence of survivin expression on cell proliferation, double-strand break (DSB) induction and repair, apoptosis and colony-forming ability in four Ewing's sarcoma cell lines with and without irradiation. Survivin protein and mRNA were upregulated in all cell lines tested in a dose-dependent manner. As a result of survivin knockdown, STA-ET-1 cells showed reduced cell proliferation, an increased number of radiation-induced DSBs, and reduced repair. Apoptosis was increased by knockdown alone and increased further in combination with irradiation. Colony formation was significantly reduced by survivin knockdown in combination with irradiation. Survivin is a radiation-inducible protein in Ewing's sarcoma and its down-regulation sensitizes cells toward irradiation. Survivin knockdown in combination with radiation inhibits cell proliferation, repair, and colony formation significantly and increases apoptosis more than each single treatment alone. This might open new perspectives in the radiation treatment of Ewing's sarcoma.

  12. Multimodality therapy for metastatic sarcomas confined to the lung

    PubMed Central

    GOLLARD, RUSSELL P.; TURNER, J. FRANCIS

    2012-01-01

    Metastectomy or resection of sarcomas which have metastasized to the lung from other sites has a long and established history. At present, there are more than forty different drugs with activity in soft tissue sarcomas. A number of sarcomas demonstrate differential sensitivities to chemotherapy and targeted agents. Intimate knowledge of the biological behavior of each distinct type of sarcoma should predicate what treatment or protocol is most suitable. Certain patients might benefit from either neoadjuvant or adjuvant therapy following the resection of metastatic lesions. Much remains to be learned about the differential sensitivities of various sarcomas to different treatment regimens. PMID:23205068

  13. Primary Intracranial Sarcoma Presenting as Chronic Subdural Fluid Collections in a Child.

    PubMed

    Glenn, Chad A; Fung, Kar-Ming; Tullos, Hurtis J; McNall-Knapp, Rene Y; Gunda, Divya; Mapstone, Timothy B

    2016-02-01

    Chronic subdural hematoma in the pediatric population often results from trauma. Asymptomatic and benign-appearing subdural collections are generally managed conservatively without operative intervention. Primary intracranial sarcomas are uncommon entities. Diagnosis of sarcoma can be difficult because these lesions often manifest as apparent hematoma. Presented is the case of a primary intracranial mucoid spindle cell sarcoma that arose in a child with a history of benign-appearing bilateral subdural fluid collections in the setting of nonaccidental trauma. The patient was initially managed conservatively because her neurological examination result was normal and her subdural collections decreased in size on repeated imaging. The collections did not resolve completely. Years later, she exhibited weakness, seizure, and an increase in the size of her subdural fluid collection. Subdural drainage was attempted without significant effect. Cytologic assessment of fluid was negative for malignant cells. Magnetic resonance imaging revealed multiple enhancing masses along the subdural collection. The patient eventually underwent craniotomy in which a diagnosis of sarcoma was obtained. Pathological and radiographic findings as well as oncological management are reviewed. The authors also review the natural history and treatment of primary intracranial sarcoma in the pediatric population. Early contrasted magnetic resonance imaging should be obtained in patients with subdural fluid collections that appear asymmetric or do not resolve in the expected time course, despite having a normal neurologic examination result. Negative cytologic assessment does not exclude sarcoma diagnosis. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. Effectiveness of Radiotherapy in Myxoid Sarcomas Is Associated With a Dense Vascular Pattern

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Vreeze, Ronald S.A. de; Jong, Daphne de; Haas, Rick L.

    2008-12-01

    Purpose: Surgery and adjuvant radiotherapy (RT) have long been the standard treatment for most deep-seated sarcomas; however, since the randomized trial from the National Cancer Institute of Canada, which described similar local control for pre- vs. postoperative RT, both modalities are now widely accepted. As a group, sarcomas are classified as radiation resistant. The subgroup of myxoid liposarcoma (MLS), a sarcoma with a typical vascular crow's feet pattern, is highly radiosensitive, but a mechanism for this phenomenon is unknown. Here we describe our results with preoperative RT and propose a mechanism explaining the high sensitivity based on the distinctive vascularizationmore » pattern of MLS. Methods and Materials: Between 2002 and 2006, 31 sarcoma patients, including 10 with MLS, underwent preoperative RT at our institute. Resected specimens were histologically evaluated, focusing on classification, grade, and vascularization patterns. Results: Twenty sarcomas showed more than 80% pathologic response after preoperative RT. A pathologic complete response was found in all 'pure' MLS specimens after preoperative RT (n = 8). There were no pathologic complete responses in the remaining sarcoma patients (n = 23), although 12 showed 80% to 90% pathologic response. In contrast to the remaining RT-resistant sarcomas, the highly responding specimens contained branching vasculature, partial thrombus formation and inflammation of medium sized arterioles, similar to the vascular changes in MLS. Conclusions: Both MLS and sarcomas with MLS-like vasculature are highly radiosensitive. Radiation sensitivity may be explained by changes in medium-sized arterioles, obstructing the specific crow's feet vascularization and inducing hypoxia with secondary tumor cell death.« less

  15. Multivariate analysis of prognostic factors in synovial sarcoma.

    PubMed

    Koh, Kyoung Hwan; Cho, Eun Yoon; Kim, Dong Wook; Seo, Sung Wook

    2009-11-01

    Many studies have described the diversity of synovial sarcoma in terms of its biological characteristics and clinical features. Moreover, much effort has been expended on the identification of prognostic factors because of unpredictable behaviors of synovial sarcomas. However, with the exception of tumor size, published results have been inconsistent. We attempted to identify independent risk factors using survival analysis. Forty-one consecutive patients with synovial sarcoma were prospectively followed from January 1997 to March 2008. Overall and progression-free survival for age, sex, tumor size, tumor location, metastasis at presentation, histologic subtype, chemotherapy, radiation therapy, and resection margin were analyzed, and standard multivariate Cox proportional hazard regression analysis was used to evaluate potential prognostic factors. Tumor size (>5 cm), nonlimb-based tumors, metastasis at presentation, and a monophasic subtype were associated with poorer overall survival. Multivariate analysis showed metastasis at presentation and monophasic tumor subtype affected overall survival. For the progression-free survival, monophasic subtype was found to be only 1 prognostic factor. The study confirmed that histologic subtype is the single most important independent prognostic factors of synovial sarcoma regardless of tumor stage.

  16. Retrotracheal Extraskeletal Ewing's Sarcoma: Case Report and Discussion on Airway Management.

    PubMed

    Van Der Meer, Graeme; Linkhorn, Hannah; Gruber, Maayan; Mahadevan, Murali; Barber, Colin

    2017-03-01

    Extraskeletal Ewing's sarcoma is a rare tumor, and the management of airway compromise in case of cervical Ewing's sarcoma has not been established. This report describes the case of a patient with retrotracheal Ewing's sarcoma and discusses a successful approach to airway management. A 12-year-old male presented with a 2-week history of sore throat and sleep-disordered breathing and 48 hours of stridor. Imaging confirmed a retrotracheal soft tissue mass with airway compromise. A planned and controlled approach to his airway management resulted in a secure airway prior to definitive treatment.

  17. Biological characterization of soft tissue sarcomas.

    PubMed

    Hayashi, Takuma; Horiuchi, Akiko; Sano, Kenji; Kanai, Yae; Yaegashi, Nobuo; Aburatani, Hiroyuki; Konishi, Ikuo

    2015-12-01

    Soft tissue sarcomas are neoplastic malignancies that typically arise in tissues of mesenchymal origin. The identification of novel molecular mechanisms leading to mesenchymal transformation and the establishment of new therapies and diagnostic biomarker has been hampered by several critical factors. First, malignant soft tissue sarcomas are rarely observed in the clinic with fewer than 15,000 newly cases diagnosed each year in the United States. Another complicating factor is that soft tissue sarcomas are extremely heterogeneous as they arise in a multitude of tissues from many different cell lineages. The scarcity of clinical materials coupled with its inherent heterogeneity creates a challenging experimental environment for clinicians and scientists. Faced with these challenges, there has been extremely limited advancement in clinical treatment options available to patients as compared to other malignant tumours. In order to glean insight into the pathobiology of soft tissue sarcomas, scientists are now using mouse models whose genomes have been specifically tailored to carry gene deletions, gene amplifications, and somatic mutations commonly observed in human soft tissue sarcomas. The use of these model organisms has been successful in increasing our knowledge and understanding of how alterations in relevant oncogenic and/or tumour suppressive signal cascades, i.e., interferon-γ (IFN-γ), tumour protein 53 (TP53) and/or retinoblastoma (RB) pathway directly impact sarcomagenesis. It is the goal of many in the physiological community that the use of several mouse models will serve as powerful in vivo tools for further understanding of sarcomagenesis and potentially identify new diagnostic biomarker and therapeutic strategies against human soft tissue sarcomas.

  18. Diagnostic Study of Tumor Characteristics in Patients With Ewing's Sarcoma

    ClinicalTrials.gov

    2013-06-20

    Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  19. Monophasic Synovial Sarcoma Presenting as Mitral Valve Obstruction

    PubMed Central

    Chokesuwattanaskul, Warangkana; Terrell, Jason; Jenkins, Leigh Ann

    2010-01-01

    We report the case of a 26-year-old man who experienced progressive left-sided chest pain and 2 episodes of near-syncope. Studies revealed a 15-cm mass in the upper left lung, a 10-cm mass in the medial base of the left lung, and a 5-cm left atrial mass that involved the left lung, infiltrated the left pulmonary vein, and prolapsed into the mitral valve, causing intermittent obstruction. The patient underwent surgical excision of the left atrial tumor. Pathologic evaluation confirmed the diagnosis of monophasic synovial sarcoma. To our knowledge, this is only the 3rd report of left atrial invasion and resultant mitral valve obstruction from a synovial sarcoma that infiltrated the pulmonary vein. We believe that this is the 1st documented case of a metastatic left atrial synovial sarcoma in monophasic form. PMID:20844626

  20. Physiotherapy management of patients with HIV-associated Kaposi's sarcoma.

    PubMed

    Harris-Love, Michael O; Shrader, Joseph A

    2004-01-01

    Kaposi's sarcoma is the most common form of cancer in patients with human immunodeficiency virus (HIV) infection. Although Kaposi sarcoma lesions may contribute to significant physical impairments, there is a lack of scientific literature detailing the role of physiotherapy in the treatment of HIV-associated Kaposi's sarcoma. The present Case Report includes two males, aged 36 and 39 years, seropositive for HIV with invasive Kaposi's sarcoma. Patient A was evaluated for bilateral foot pain caused by plantar surface Kaposi s sarcoma lesions that rendered him unable to walk. He progressed to walking 400feet after a treatment regimen of gait training with the use of custom plastazote sandals. Patient B was evaluated for right lower extremity lymphoedema secondary to invasive Kaposi's sarcoma. He experienced an 18% reduction in limb volume, a 38% reduction in pain and a 20 degrees increase in terminal knee flexion after therapeutic exercise and the use of compressive bandaging and garments. This Case Report suggests that physiotherapy interventions may be valuable in the conservative management of patients with HIV-associated Kaposi s sarcoma.

  1. Epidemiology of the sarcomas of the jaws in a Peruvian population

    PubMed Central

    Sacsaquispe-Contreras, Sonia J.; Morales-Vadillo, Rafael; Sánchez Lihón, Juvenal

    2012-01-01

    Objective: Analysis of the clinical characteristics of patients with Sarcomas of the Jaws treated in the “Instituto Nacional de Enfermedades Neoplasicas. Dr. Eduardo Caceres Graziani” from 1952-2007. Study Design: Review of 155 clinical records of patients with Sarcomas of the Jaws and record of age, gender, size, location, clinical symptoms and signs, histopathological diagnoses and type of treatment. The data obtained were analyzed by means of Student’s statistical t-test, Fisher and Friedman’s test. Results: Analysis of 155 Sarcomas of the Jaws. The average age of patients was 36.8 years old (range: 1-80 years); the female gender was the most frequent (52.9%); the average tumor size was 5.5 cm; in upper jaw 54.84% occurred and 45.16% in the lower jaw; the predominant sign was facial asymmetry (87.74%) and the predominant symptom: pain (63.23%). The most frequent diagnosis was Osteosarcoma 50.3% followed by Chondrosarcoma 18%. Surgery plus radiation therapy was the treatment type of choice with 21.94% of cases. Conclusion: The results of this study demonstrate the delayed diagnosis and facial asymmetry and pain appear as the most important events for the diagnosis of Sarcomas of the Jaws. Key words: Sarcoma, jaw, jaw neoplasms, mouth neoplasms. PMID:22143684

  2. Collecting and Storing Biological Samples From Patients With Ewing Sarcoma

    ClinicalTrials.gov

    2017-12-11

    Askin Tumor; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  3. [Molecular biology for sarcoma: useful or necessary?].

    PubMed

    Neuville, Agnès; Coindre, Jean-Michel; Chibon, Frédéric

    2015-01-01

    Sarcomas are a heterogeneous group of tumors. Their diagnosis is based on morphology and immunohistochemical profile, with categories of tumors according to the type of tissue that they resemble. Nevertheless, for several tumors, cellular origin is unknown. Molecular analysis performed in recent years allowed, combining histophenotype and genomics, better classifying such sarcomas, individualizing new entities and grouping some tumors. Simple and recurrent genetic alterations, such as translocation, mutation, amplification, can be identified in one of two sarcomas and appear as new diagnostic markers. Their identification in specialized laboratories in molecular pathology of sarcomas is often useful and sometimes necessary for a good diagnosis, leading to a heavy and multidisciplinary multi-step treatment. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  4. Spontaneous resolution of apparent radiation associated retroperitoneal fibrosis.

    PubMed

    Khezri, Azadeh; Berman, Howard L; Rosenstein, Elliot D; Kramer, Neil

    2011-12-01

    A 70-year-old man was diagnosed with retroperitoneal fibrosis (RPF) complicated by ureteral obstruction 4 months after finishing radiotherapy for prostate cancer. He was treated conservatively with ureteral stent placement. After 3 months, computed tomography scan of the abdomen revealed resolution of RPF without any medical or surgical interventions. Although an uncommon event, the possibility of spontaneous resolution of RPF, as demonstrated by this and previously reported cases, has led some to encourage conservative management.

  5. Retroperitoneal Liposarcoma: Current Insights in Diagnosis and Treatment

    PubMed Central

    Matthyssens, Lucas E.; Creytens, David; Ceelen, Wim P.

    2015-01-01

    Retroperitoneal liposarcoma (RLS) is a rare, biologically heterogeneous tumor that present considerable challenges due to its size and deep location. As a consequence, the majority of patients with high-grade RLS will develop locally recurrent disease following surgery, and this constitutes the cause of death in most patients. Here, we review current insights and controversies regarding histology, molecular biology, extent of surgery, (neo)adjuvant treatment, and systemic treatment including novel targeted agents in RLS. PMID:25713799

  6. Kaposi Sarcoma

    MedlinePlus

    Kaposi sarcoma (KS) is a cancer that causes patches of abnormal tissue to grow under the skin, in the lining of ... of cancer cells, blood vessels, and blood cells. KS is caused by infection with human herpesvirus-8 ( ...

  7. Retroperitoneal Laparoscopic Partial Nephrectomy Versus Radical Nephrectomy for Clinical T1 Renal Hilar Tumor: Comparison of Perioperative Characteristics and Short-Term Functional and Oncologic Outcomes.

    PubMed

    Yang, Chuance; Wang, Zhenlong; Huang, Shanlong; Xue, Li; Fu, Delai; Chong, Tie

    2018-04-18

    To present our single-center experience with retroperitoneal laparoscopic partial nephrectomy (LPN) and retroperitoneal laparoscopic radical nephrectomy (LRN) for T1 renal hilar tumors and evaluate which one is better. A retrospective review of 63 patients with hilar tumors undergoing retroperitoneal LPN or LRN was performed. The perioperative characteristics, change in estimated glomerular filtration rate (eGFR) from baseline to month 3, and oncologic outcomes were summarized. In total, 25 patients underwent LPN, and 38 patients underwent LRN. The mean tumor size in the LPN and LRN groups was 4.5 and 4.9 cm, respectively. The mean operation time was longer in the LPN group than that in the LRN group (212.5 minutes versus 160.7 minutes, respectively; P < .05). Patients undergoing the LPN had a longer median length of hospital stay after surgery (9 days versus 7 days, P < .05). Four percent of patients in the LPN group experienced postoperative complications compared with 5% of patients in the LRN group, which was not significantly different. Compared with preoperative eGFR, postoperative eGFR at 3 months decreased by 15.2 mL/min/1.73 m 2 and 27.8 mL/min/1.73 m 2 in the LPN and the LRN groups, respectively (P < .05). There was one local recurrence in the LPN group and three local or distant recurrences in the LRN group (P > .05). In experienced hands, although retroperitoneal LRN can result in shorter operation times and shorter lengths of stay, retroperitoneal LPN can preserve renal function better than LRN. Retroperitoneal LPN should be the priority in selected patients with T1 renal hilar tumors, especially for patients with renal insufficiency.

  8. Retroperitoneal versus transperitoneal robotic-assisted laparoscopic partial nephrectomy: a matched-pair, bicenter analysis with cost comparison using time-driven activity-based costing.

    PubMed

    Laviana, Aaron A; Tan, Hung-Jui; Hu, Jim C; Weizer, Alon Z; Chang, Sam S; Barocas, Daniel A

    2018-03-01

    To perform a bicenter, retrospective study of perioperative outcomes of retroperitoneal versus transperitoneal robotic-assisted laparoscopic partial nephrectomy (RALPN) and assess costs using time-driven activity-based costing (TDABC). We identified 355 consecutive patients who underwent RALPN at University of California Los Angeles and the University of Michigan during 2009-2016. We matched according to RENAL nephrometry score, date, and institution for 78 retroperitoneal versus 78 transperitoneal RALPN. Unadjusted analyses were performed using McNemar's Chi-squared or paired t test, and adjusted analyses were performed using multivariable repeated measures regression analysis. From multivariable models, predicted probabilities were derived according to approach. Cost analysis was performed using TDABC. Patients treated with retroperitoneal versus transperitoneal RALPN were similar in age (P = 0.490), sex (P = 0.715), BMI (P = 0.273), and comorbidity (P = 0.393). Most tumors were posterior or lateral in both the retroperitoneal (92.3%) and transperitoneal (85.9%) groups. Retroperitoneal RALPN was associated with shorter operative times (167.0 versus 191.1 min, P = 0.001) and length of stay (LOS) (1.8 versus 2.7 days, P < 0.001). There were no differences in renal function preservation or cancer control. In adjusted analyses, retroperitoneal RALPN was 17.6-min shorter (P < 0.001) and had a 76% lower probability of LOS at least 2 days (P < 0.001). Utilizing TDABC, transperitoneal RALPN added $2337 in cost when factoring in disposable equipment, operative time, LOS, and personnel. In two high-volume, tertiary centers, retroperitoneal RALPN is associated with reduced operative times and shortened LOS in posterior and lateral tumors, whereas sharing similar clinicopathologic outcomes, which may translate into lower healthcare costs. Further investigation into anterior tumors is needed.

  9. Intratumoral oxygen gradients mediate sarcoma cell invasion

    PubMed Central

    Lewis, Daniel M.; Park, Kyung Min; Tang, Vitor; Xu, Yu; Pak, Koreana; Eisinger-Mathason, T. S. Karin; Simon, M. Celeste; Gerecht, Sharon

    2016-01-01

    Hypoxia is a critical factor in the progression and metastasis of many cancers, including soft tissue sarcomas. Frequently, oxygen (O2) gradients develop in tumors as they grow beyond their vascular supply, leading to heterogeneous areas of O2 depletion. Here, we report the impact of hypoxic O2 gradients on sarcoma cell invasion and migration. O2 gradient measurements showed that large sarcoma mouse tumors (>300 mm3) contain a severely hypoxic core [≤0.1% partial pressure of O2 (pO2)] whereas smaller tumors possessed hypoxic gradients throughout the tumor mass (0.1–6% pO2). To analyze tumor invasion, we used O2-controllable hydrogels to recreate the physiopathological O2 levels in vitro. Small tumor grafts encapsulated in the hydrogels revealed increased invasion that was both faster and extended over a longer distance in the hypoxic hydrogels compared with nonhypoxic hydrogels. To model the effect of the O2 gradient accurately, we examined individual sarcoma cells embedded in the O2-controllable hydrogel. We observed that hypoxic gradients guide sarcoma cell motility and matrix remodeling through hypoxia-inducible factor-1α (HIF-1α) activation. We further found that in the hypoxic gradient, individual cells migrate more quickly, across longer distances, and in the direction of increasing O2 tension. Treatment with minoxidil, an inhibitor of hypoxia-induced sarcoma metastasis, abrogated cell migration and matrix remodeling in the hypoxic gradient. Overall, we show that O2 acts as a 3D physicotactic agent during sarcoma tumor invasion and propose the O2-controllable hydrogels as a predictive system to study early stages of the metastatic process and therapeutic targets. PMID:27486245

  10. Intratumoral oxygen gradients mediate sarcoma cell invasion.

    PubMed

    Lewis, Daniel M; Park, Kyung Min; Tang, Vitor; Xu, Yu; Pak, Koreana; Eisinger-Mathason, T S Karin; Simon, M Celeste; Gerecht, Sharon

    2016-08-16

    Hypoxia is a critical factor in the progression and metastasis of many cancers, including soft tissue sarcomas. Frequently, oxygen (O2) gradients develop in tumors as they grow beyond their vascular supply, leading to heterogeneous areas of O2 depletion. Here, we report the impact of hypoxic O2 gradients on sarcoma cell invasion and migration. O2 gradient measurements showed that large sarcoma mouse tumors (>300 mm(3)) contain a severely hypoxic core [≤0.1% partial pressure of O2 (pO2)] whereas smaller tumors possessed hypoxic gradients throughout the tumor mass (0.1-6% pO2). To analyze tumor invasion, we used O2-controllable hydrogels to recreate the physiopathological O2 levels in vitro. Small tumor grafts encapsulated in the hydrogels revealed increased invasion that was both faster and extended over a longer distance in the hypoxic hydrogels compared with nonhypoxic hydrogels. To model the effect of the O2 gradient accurately, we examined individual sarcoma cells embedded in the O2-controllable hydrogel. We observed that hypoxic gradients guide sarcoma cell motility and matrix remodeling through hypoxia-inducible factor-1α (HIF-1α) activation. We further found that in the hypoxic gradient, individual cells migrate more quickly, across longer distances, and in the direction of increasing O2 tension. Treatment with minoxidil, an inhibitor of hypoxia-induced sarcoma metastasis, abrogated cell migration and matrix remodeling in the hypoxic gradient. Overall, we show that O2 acts as a 3D physicotactic agent during sarcoma tumor invasion and propose the O2-controllable hydrogels as a predictive system to study early stages of the metastatic process and therapeutic targets.

  11. Lateral retroperitoneal transpsoas interbody fusion in a patient with achondroplastic dwarfism.

    PubMed

    Staub, Blake N; Holman, Paul J

    2015-02-01

    The authors present the first reported use of the lateral retroperitoneal transpsoas approach for interbody arthrodesis in a patient with achondroplastic dwarfism. The inherent anatomical abnormalities of the spine present in achondroplastic dwarfism predispose these patients to an increased incidence of spinal deformity as well as neurogenic claudication and potential radicular symptoms. The risks associated with prolonged general anesthesia and intolerance of significant blood loss in these patients makes them ideal candidates for minimally invasive spinal surgery. The patient in this case was a 51-year-old man with achondroplastic dwarfism who had a history of progressive claudication and radicular pain despite previous extensive lumbar laminectomies. The lateral retroperitoneal transpsoas approach was used for placement of interbody cages at L1/2, L2/3, L3/4, and L4/5, followed by posterior decompression and pedicle screw instrumentation. The patient tolerated the procedure well with no complications. Postoperatively his claudicatory and radicular symptoms resolved and a CT scan revealed solid arthrodesis with no periimplant lucencies.

  12. Alveolar soft part sarcoma causing perianal abscess.

    PubMed

    Sullivan, Niall; McCulloch, Tom; Leverton, David

    2011-07-01

    A 34-year-old woman presented with a perianal abscess that communicated with the vagina. There was a background of a one-year history of a conservatively treated, traumatic, paravaginal haematoma. Histology of the fistula tract showed alveolar soft part sarcoma and subsequent imaging identified a large soft tissue mass in the pelvis with lung metastases. Alveolar soft part sarcoma is a rare soft tissue sarcoma of unknown cellular origin affecting predominantly young women, often in deep soft tissues and lower extremities.

  13. Castleman’s disease imitating adrenal mass in the retroperitoneal area

    PubMed Central

    Koç, Gökhan; Turk, Hakan; Un, Sıtkı; Isoglu, Cemal Selcuk; Zorlu, Ferruh

    2015-01-01

    Castleman’s disease (CD) is a non-clonal lymph node hyperplasia, mostly seen in the mediastinum. It has various clinical and pathological outcomes. There are different treatments because of its rare occurance and heterogenity. We present 2 cases which were referred to our clinic as retroperitoneal mass and diagnosed as CD after surgical resection. PMID:25624969

  14. Synovial sarcoma of nerve.

    PubMed

    Scheithauer, Bernd W; Amrami, Kimberly K; Folpe, Andrew L; Silva, Ana I; Edgar, Mark A; Woodruff, James M; Levi, Allan D; Spinner, Robert J

    2011-04-01

    Tumors of peripheral nerve are largely neuroectodermal in nature and derived from 2 elements of nerve, Schwann or perineurial cells. In contrast, mesenchymal tumors affecting peripheral nerve are rare and are derived mainly from epineurial connective tissue. The spectrum of the latter is broad and includes lipoma, vascular neoplasms, hematopoietic tumors, and even meningioma. Of malignant peripheral nerve neoplasms, the vast majority are primary peripheral nerve sheath tumors. Malignancies of mesenchymal type are much less common. To date, only 12 cases of synovial sarcoma of nerve have been described. Whereas in the past, parallels were drawn between synovial sarcoma and malignant glandular schwannoma, an uncommon form of malignant peripheral nerve sheath tumor, molecular genetics have since clarified the distinction. Herein, we report 10 additional examples of molecularly confirmed synovial sarcoma, all arising within minor or major nerves. Affecting 7 female and 3 male patients, 4 tumors occurred in pediatric patients. Clinically and radiologically, most lesions were initially thought to be benign nerve sheath tumors. On reinterpretation of imaging, they were considered indeterminate in nature with some features suspicious for malignancy. Synovial sarcoma of nerve, albeit rare, seems to behave in a manner similar to its more common, soft tissue counterpart. Those affecting nerve have a variable prognosis. Definitive recommendations regarding surgery and adjuvant therapies await additional reports and long-term follow-up. The literature is reviewed and a meta-analysis is performed with respect to clinicopathologic features versus outcome. Copyright © 2011. Published by Elsevier Inc.

  15. Efficacy and safety of Apatinib in stage IV sarcomas: experience of a major sarcoma center in China.

    PubMed

    Li, Feng; Liao, Zhichao; Zhao, Jun; Zhao, Gang; Li, Xubin; Du, Xiaoling; Yang, Yun; Yang, Jilong

    2017-09-08

    This study was conducted to review the efficacy and safety of Apatinib in stage IV sarcoma patients who failed previous chemotherapy. The clinical information on 16 patients with stage IV sarcomas who failed in prior chemotherapy and subsequently received Apatinib treatment was collected. Apatinib was given 500mg/daily and 4 weeks as a cycle. All patients had at least one measurable extracranial tumor according to Response Evaluation Criteria In Solid Tumors 1.0 criteria. Progression free survival (PFS), overall survival (OS), objective response rate (ORR), disease control rate (DCR) and treatment-related adverse effects (AEs) were reviewed and evaluated. Patients was administered Apatinib for 0 to 9 cycles with the median of 3.2 cycles. Median follow-up time was 8.4 months (1 to 12 months). Ten of 16 patients received at least 1 complete cycle of Apatinib treatment were eligible for the efficacy analysis. The median PFS was 8.84 months. Two patients achieved partial response (PR) and 6 patients achieved stable disease (SD). Two patients were evaluated as progression disease (PD) and one patient died of disease progression. The ORR was 20.0% (2/10) and the DCR was 80.0% (8/10). The most common grade 3/4 treatment-related AEs were hypertension (18.7%), hand-foot syndrome (12.5%) and proteinuria (6.3%). No drug-related severe AEs occurred. CApatinib treatment in this exploratory study exhibited objective efficacy and manageable toxicity in stage IV sarcoma patients who failed in chemotherapy. This result supports future random controlled trial to further define Apatinib activity in stage IV sarcomas.

  16. Trajectory of the main sensory and motor branches of the lumbar plexus outside the psoas muscle related to the lateral retroperitoneal transpsoas approach.

    PubMed

    Dakwar, Elias; Vale, Fernando L; Uribe, Juan S

    2011-02-01

    The minimally invasive lateral retroperitoneal transpsoas approach is increasingly used to treat various spinal disorders. Accessing the retroperitoneal space and traversing the abdominal wall poses a risk of injury to the major nervous structures and adds significant morbidity to the procedure. Most of the current literature focuses on the anatomy of the lumbar plexus within the substance of the psoas muscle. However, there is sparse knowledge regarding the trajectory of the lumbar plexus nerves that travel along the retroperitoneum and abdominal wall muscles in relation to the lateral approach to the spine. The objective of this study is to define the anatomical trajectories of the major motor and sensory branches of the lumbar plexus that are located outside the psoas muscle. Six adult fresh frozen cadaveric specimens were dissected and studied (12 sides). The relationship between the retroperitoneum, abdominal wall muscles, and the lumbar plexus nerves was analyzed in reference to the minimally invasive lateral retroperitoneal approach. Special attention was given to the lumbar plexus nerves that run outside of psoas muscle in the retroperitoneal cavity and within the abdominal muscle wall. The skin and muscles of the abdominal wall and the retroperitoneal cavity were dissected and analyzed with respect to the major motor and sensory branches of the lumbar plexus. The authors identified 4 nerves at risk during the lateral approach to the spine: subcostal, iliohypogastric, ilioinguinal, and lateral femoral cutaneous nerves. The anatomical trajectory of each of these nerves is described starting from the spinal column until their termination or exit from the pelvic cavity. There is risk of direct injury to the main motor/sensory nerves that supply the anterior abdominal muscles during the early stages of the lateral retroperitoneal transpsoas approach while obtaining access to the retroperitoneum. There is also a risk of injury to the ilioinguinal

  17. General Information about Childhood Soft Tissue Sarcoma

    MedlinePlus

    ... Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version General Information About Childhood Soft Tissue Sarcoma Go to Health ... the PDQ Pediatric Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  18. General Information about Adult Soft Tissue Sarcoma

    MedlinePlus

    ... Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version General Information About Adult Soft Tissue Sarcoma Go to Health ... the PDQ Adult Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  19. An Aggressive Retroperitoneal Fibromatosis

    PubMed Central

    Campara, Zoran; Spasic, Aleksandar; Aleksic, Predrag; Milev, Bosko

    2016-01-01

    Introduction: Aggressive fibromatosis (AF) is a heterogeneous group of mesenchymal tumors that have locally infiltrative growth and a tendency to relapse. The clinical picture is often conditioned by the obstruction of the ureter or small intestine. Diagnosis is based on clinical, radiological and histological parameters. A case report: We report a case of male patient, aged 35 years, with the retroperitoneal fibromatosis. He reported to the physician because of frequent urination with the feeling of pressure and pain. Computed tomography revealed the tumor mass on the front wall of the bladder with diameter of 70mm with signs of infiltration of the musculature of the anterior abdominal wall. Endoscopic transurethral biopsy showed proliferative lesion binders by type of fibromatosis. The tumor was surgically removed in a classical way. The patient feels well and has no recurrence thirty-six months after the operative procedure. Conclusion: The complete tumor resection is the therapeutic choice for the primary tumor as well as for a relapse. PMID:27147794

  20. Retroperitoneal Extraskeletal Osteosarcoma: Imaging Findings and Transarterial Chemoembolization

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Zhang Huojun, E-mail: chyyzhj@hotmail.com; Yang Jijin, E-mail: yangjijin@live.com; Lu Jianping

    2010-04-15

    Extraskeletal osteosarcoma (EOS) is an uncommon and usually highly aggressive mesenchymal tumor. Retroperitoneal extraskeletal osteosarma (REOS) is exceedingly rare. Due to the rare nature of the disease, both the diagnosis and the management of REOS can be challenging. We present the clinical history, CT findings, angiographic manifestations, and use of transarterial chemoembolization for treatment in a case of REOS. To our knowledge, the angiographic features of and attempt at transarterial treatment of REOS have not been reported in the literature.

  1. Clinicopathological and Molecular Factors, Risk Factors, Treatment Outcomes and Risk of Recurrence in Mesenteric and Retroperitoneal Extragastrointestinal Stromal Tumors.

    PubMed

    Apostolou, Konstantinos G; Schizas, Dimitrios; Vavouraki, Eleni; Michalinos, Adamantios; Tsilimigras, Diamantis I; Garmpis, Nikolaos; Damaskos, Christos; Papalampros, Alexandros; Liakakos, Theodore

    2018-04-01

    The objective of the present study was to determine the clinicopathological factors and treatment outcomes of patients suffering from mesenteric or retroperitoneal extragastrointestinal stromal tumors (EGISTs). A detailed search in PubMed, using the key words "extragastrointestinal stromal tumors" and "EGIST", found eight studies fulfilling the criteria of this study. Thirty-six patients with a mesenteric and 24 patients with a retroperitoneal EGIST were analyzed, with a follow-up period ranging from 2 to 192 months. Retroperitoneal tumors presented as larger tumors than mesenteric ones, with 95% and 93% immunohistochemical positivity for CD117 antigen, respectively. Surgical resection was performed in 91% of cases, with 57% of patients with mesenteric and 70% of patients with retroperitoneal EGISTs being alive at the last follow-up. EGISTs most commonly are of considerable size and usually with a high mitotic count, rendering them high-risk tumors. Tumor necrosis, nuclear atypia, tumor histology, and mutations in the tyrosine kinase KIT or platelet-derived growth factor receptor A (PDGFRA) gene, seem to influence tumor behavior. Copyright© 2018, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.

  2. A case report and a literature review of primary retroperitoneal mucinous cystadenoma: the importance of imaging in diagnosis and management.

    PubMed

    Pesapane, Filippo; Renterghem, Sofie Van; Patella, Francesca; Visschere, Pieter De; Villeirs, Geert

    2018-01-29

    Primary retroperitoneal mucinous cystadenoma (PRMC) is an extremely rare tumor: its histogenesis and its biological behavior remain speculative. Since most retroperitoneal tumors are malignant, a preoperative diagnosis of benignity is essential and it can be reached through imaging examinations, allowing a conservative management approach. We describe the case of a 52-year-old woman with abdominal pain and a palpable mass. Computed tomography of the abdomen revealed a retroperitoneal cystic mass, which was resected successfully through laparoscopy and diagnosed as PRMC. Although there are no pathognomonic, clinical or radiological findings for PRMC, it should be included in the list of differential diagnoses and its imaging criteria of benignity should always be sought, with the aim to exclude malignant tumors.

  3. Development and Potential Applications of CRISPR-Cas9 Genome Editing Technology in Sarcoma

    PubMed Central

    Liu, Tang; Shen, Jacson K.; Li, Zhihong; Choy, Edwin; Hornicek, Francis J.; Duan, Zhenfeng

    2016-01-01

    Sarcomas include some of the most aggressive tumors and typically respond poorly to chemotherapy. In recent years, specific gene fusion/mutations and gene over-expression/activation have been shown to drive sarcoma pathogenesis and development. These emerging genomic alterations may provide targets for novel therapeutic strategies and have the potential to transform sarcoma patient care. The RNA-guided nuclease CRISPR-Cas9 (Clustered Regularly Interspaced Short Palindromic Repeats (CRISPR)-associated protein-9 nuclease) is a convenient and versatile platform for site-specific genome editing and epigenome targeted modulation. Given that sarcoma is believed to develop as a result of genetic alterations in mesenchymal progenitor/stem cells, CRISPR-Cas9 genome editing technologies hold extensive application potentials in sarcoma models and therapies. We review the development and mechanisms of the CRISPR-Cas9 system in genome editing and introduce its application in sarcoma research and potential therapy in clinic. Additionally, we propose future directions and discuss the challenges faced with these applications, providing concise and enlightening information for readers interested in this area. PMID:26806808

  4. Development and potential applications of CRISPR-Cas9 genome editing technology in sarcoma.

    PubMed

    Liu, Tang; Shen, Jacson K; Li, Zhihong; Choy, Edwin; Hornicek, Francis J; Duan, Zhenfeng

    2016-04-01

    Sarcomas include some of the most aggressive tumors and typically respond poorly to chemotherapy. In recent years, specific gene fusion/mutations and gene over-expression/activation have been shown to drive sarcoma pathogenesis and development. These emerging genomic alterations may provide targets for novel therapeutic strategies and have the potential to transform sarcoma patient care. The RNA-guided nuclease CRISPR-Cas9 (Clustered Regularly Interspaced Short Palindromic Repeats (CRISPR)-associated protein-9 nuclease) is a convenient and versatile platform for site-specific genome editing and epigenome targeted modulation. Given that sarcoma is believed to develop as a result of genetic alterations in mesenchymal progenitor/stem cells, CRISPR-Cas9 genome editing technologies hold extensive application potentials in sarcoma models and therapies. We review the development and mechanisms of the CRISPR-Cas9 system in genome editing and introduce its application in sarcoma research and potential therapy in clinic. Additionally, we propose future directions and discuss the challenges faced with these applications, providing concise and enlightening information for readers interested in this area. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  5. Cixutumumab and Temsirolimus in Treating Younger Patients With Recurrent or Refractory Sarcoma

    ClinicalTrials.gov

    2018-03-21

    Childhood Alveolar Soft Part Sarcoma; Childhood Angiosarcoma; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Gliosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Peripheral Nerve Sheath Tumor; Childhood Synovial Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Osteosarcoma; Rhabdomyosarcoma

  6. Combination Chemotherapy in Treating Patients With Non-Metastatic Extracranial Ewing Sarcoma

    ClinicalTrials.gov

    2018-02-09

    Adult Supratentorial Primitive Neuroectodermal Tumor (PNET); Childhood Supratentorial Primitive Neuroectodermal Tumor; Ewing Sarcoma of Bone; Extraosseous Ewing Sarcoma; Extraosseous Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Peripheral Primitive Neuroectodermal Tumor of the Kidney; Untreated Childhood Supratentorial Primitive Neuroectodermal Tumor

  7. Hypertensive crisis during wide excision of gastrointestinal stromal cell tumor (GIST): Undiagnosed paraganglioma -A case report-.

    PubMed

    Shinn, Helen Ki; Jung, Jong Kwon; Park, Jay Kim; Kim, Jong Hoon; Jung, In Young; Lee, Hong Sik

    2012-03-01

    Although paraganglioma (PGL), an extra-adrenal retroperitoneal pheochromocytoma (PHEO), is a rare catecholamine-secreting neuroendocrine tumor, it can cause severe hypertensive crisis during anesthesia or surgery if undiagnosed preoperatively. Extraluminal perigastric masses may be presumed to be gastrointestinal stromal tumors (GISTs) or soft tissue sarcomas even when histologic confirmation is not possible. Therefore, without a histologic diagnosis or symptoms of excessive catecholamine secretion, PGL may be mistaken for GIST. We report a case of preoperatively undiagnosed PGL which caused hypertensive crisis during anesthesia for retroperitoneal mass excision.

  8. Uterine sarcoma vs adenocarcinoma: can MRI distinguish between them?

    PubMed

    Hernández Mateo, P; Méndez Fernández, R; Serrano Tamayo, E

    2016-01-01

    To analyze the MRI characteristics of uterine sarcomas (mainly carcinosarcomas) and to compare them with those of adenocarcinomas to define the findings that would be useful for the differential diagnosis. We retrospectively reviewed the MRI studies of 13 patients with histologically diagnosed uterine sarcoma. We analyzed tumor size, signal in T2-weighted, unenhanced and gadolinium-enhanced T1-weighted, and diffusion-weighted sequences. We compared the data obtained with those of another series of 30 consecutive cases of adenocarcinomas studied with MRI. The sarcomas (> 9cm in 77% of cases) were considerably larger than the adenocarcinomas (p<0.001). There were no differences in FIGO staging by MRI or surgery: both tumor types were diagnosed in early stages. The signal intensity in T2-weighted images differed significantly between the two tumor types: all the sarcomas were heterogeneous and predominantly hyperintense with respect to the myometrium in T2-weighted sequences (p<0.001). In postcontrast studies, all the sarcomas showed enhancement greater than or equal to the myometrium; this finding was significantly different from the adenocarcinomas (p<0.001). In diffusion-weighted sequences, we found no significant differences in ADC values in the areas with greatest restriction, but the ADC map was more heterogeneous in the sarcomas. Uterine sarcomas do not have specific characteristics on MRI, but some findings can indicate the diagnosis. In our study, we found significant differences between sarcomas and adenocarcinomas. Sarcomas were larger, had more hyperintense and heterogeneous signal intensity in T2-weighted sequences, and enhanced more than or at least as much as the myometrium. Copyright © 2015 SERAM. Published by Elsevier España, S.L.U. All rights reserved.

  9. Histology and imaging of soft tissue sarcomas.

    PubMed

    Kind, Michèle; Stock, Nathalie; Coindre, Jean Michel

    2009-10-01

    Imaging and histology are two complementary morphological techniques which play a fundamental role in the diagnosis and management of soft tissue sarcomas. Imaging allows to identify some pseudosarcomatous benign lesions such as myositis ossificans, intramuscular hemangioma, angiomyolipoma, intramuscular lipoma, giant cell tumour of tendon sheath, desmoid tumour and elastofibroma. There is no formal criterion for diagnosing a sarcoma on magnetic resonance imaging (MRI) but malignancy is strongly suspected with the presence of necrosis and vascular, bone or joint invasion. Imaging may also suggest some histological types of sarcoma such as well-differentiated liposarcoma, dedifferentiated liposarcoma, synovial sarcoma or extraskeletal osteosarcoma. Imaging is also extremely helpful in determining the appropriate kind of sampling to carry out and in guiding the performance of a microbiopsy. The appearance observed on imaging should always be taken into consideration for the interpretation of the microbiopsy by the pathologist.

  10. Apatinib as targeted therapy for sarcoma

    PubMed Central

    Li, Feng; Liao, Zhichao; Zhang, Chao; Zhao, Jun; Xing, Ruwei; Teng, Sheng; Zhang, Jin; Yang, Yun; Yang, Jilong

    2018-01-01

    Sarcomas are a group of malignant tumors originating from mesenchymal tissue with a variety of cell subtypes. Despite several major treatment breakthroughs, standard treatment using surgery, radiation, and chemotherapy has failed to improve overall survival. Therefore, there is an urgent need to explore new strategies and innovative therapies to further improve the survival rates of patients with sarcomas. Pathological angiogenesis has an important role in the growth and metastasis of tumors. Vascular endothelial growth factor (VEGF) and vascular endothelial growth factor receptors (VEGFRs) play a central role in tumor angiogenesis and represent potential targets for anticancer therapy. As a novel targeted therapy, especially with regard to angiogenesis, apatinib is a new type of small molecule tyrosine kinase inhibitor that selectively targets VEGFR-2 and has shown encouraging anticancer activity in a wide range of malignancies, including gastric cancer, non-small cell lung cancer, breast cancer, hepatocellular carcinoma, and sarcomas. In this review, we summarize the preclinical and clinical data for apatinib, focusing primarily on its use in the treatment of sarcomas. PMID:29849960

  11. [Colonic granulocytic sarcoma: a case report].

    PubMed

    Makni, S; Bahri, I; Ayadi, L; Mseddi, A; Bouaziz, M; Jlidi, R

    2002-06-01

    Granulocytic sarcoma is a rare tumor composed of immature cells of the granulocytic series which usually occurs as a secondary manifestation of acute leukaemia. We report the case of a 60 years old woman without particular previous pathologies who was hospitalised for chronic diarrhea developed in a context of health impairment state. The blood cell count revealed severe leucopenia and thrombopenia; an emergency right colectomy was accomplished. The histologic examination showed granulocytic sarcoma of the ascending colon. The death occurred rapidly as a consequence of a toxic shock. This observation seems to be the sixth case report of the granulocytic large bowel sarcoma in the literature which likely complicated a pre-existant and unknown myeloid leukaemia.

  12. Ewing Sarcoma Treatment (PDQ®)—Patient Version

    Cancer.gov

    Ewing sarcoma treatment usually begins with chemotherapy and/or radiation followed by surgery to remove any remaining cancer. Treatment options for recurrent sarcoma include targeted therapy and high-dose chemotherapy with stem cell transplant. Learn more in this expert-reviewed summary.

  13. Kaposi Sarcoma Treatment (PDQ®)—Patient Version

    Cancer.gov

    Kaposi sarcoma treatment depends upon the type and can include scraping to remove a lesion, radiation therapy, surgery, and sometimes chemotherapy. Learn more about the diagnosis and treatment of newly diagnosed and recurrent Kaposi sarcoma in this expert-reviewed summary.

  14. Extraskeletal Ewing's sarcoma.

    PubMed

    El-Essawy, Manar T

    2009-06-01

    We report 2 rare cases of extraskeletal Ewing's sarcoma, one is arising primarily from the posterior mediastinum in a middle-aged man (patient 1), and the other one is arising from the left kidney in a young male patient (patient 2). The CT in the first case showed a large mass of heterogeneous texture, with areas of cystic changes in the right side of the posterior mediastinum, no underlying bony changes or intra-spinal extension, and this mass was diagnosed as lymphoma. The second case showed almost complete replacement of the left kidney by a mass with extension through the renal vein and inferior vena cava, and it was diagnosed as renal cell carcinoma. The histological analysis of these lesions revealed extraskeletal Ewing's sarcoma.

  15. Sapanisertib or Pazopanib Hydrochloride in Treating Patients With Locally Advanced or Metastatic Sarcoma

    ClinicalTrials.gov

    2018-06-20

    High Grade Sarcoma; Metastatic Leiomyosarcoma; Metastatic Malignant Peripheral Nerve Sheath Tumor; Metastatic Synovial Sarcoma; Metastatic Undifferentiated Pleomorphic Sarcoma; Myxofibrosarcoma; Recurrent Leiomyosarcoma; Recurrent Malignant Peripheral Nerve Sheath Tumor; Recurrent Synovial Sarcoma; Recurrent Undifferentiated Pleomorphic Sarcoma; Uterine Corpus Leiomyosarcoma

  16. Asymptomatic Pulmonary Allograft Kaposi Sarcoma: A Case Report.

    PubMed

    Nannini, Nazarena; Rebusso, Alessandro; Lunardi, Francesca; Loy, Monica; Calabrese, Francesca; Battistella, Lucia; Schiavon, Marco; Rea, Federico; Calabrese, Fiorella

    2017-08-01

    Solid-organ transplant recipients are at high risk of developing malignancies. A greater risk of Kaposi sarcoma has been reported in lung recipients in our country, particularly in those from Southern Italy, probably due to the high prevalence of Human herpes virus 8 infection. Kaposi sarcoma affecting only the lung allograft is extremely rare. We describe a case of a lung recipient who developed Kaposi sarcoma only in the graft, 22 months after transplant. The patient, a 65-year-old man from Southern Italy, underwent bilateral lung transplant for idiopathic pulmonary fibrosis in January 2009. He developed mild/moderate acute cellular rejection (≥A2) in 4 of 6 scheduled transbronchial biopsies thus was treated with increased immunosuppressive therapy, shifting from cyclosporine to tacrolimus and mycophenolate mofetil. In July 2010, a high-resolution computed tomography scan showed small bilateral lung nodules, despite a generally good condition. After 2 months, his condition worsened with a severe weight loss. A positron emission tomography scan showed mild metabolic activity in the lesions with no other localizations. In October 2010, a lung biopsy was performed, with results showing typical histologic and immunohistochemical features of Kaposi sarcoma. Molecular tissue evaluations and serologic analyses were positive for Human herpes virus 8. The patient's immunosuppressive therapy was suspended, and he started liposomal doxorubicin treatment; however, after the first cycle, he developed severe respiratory dysfunction. The patient died 27 months after lung transplant for neoplasm. Our report highlights the importance of considering Kaposi sarcoma in the differential diagnosis for lung nodules in lung transplant recipients, even in the absence of any initial specific symptom or cutaneous lesion.

  17. Hypoxia-Dependent Modification of Collagen Networks Promotes Sarcoma Metastasis

    PubMed Central

    Eisinger-Mathason, T.S. Karin; Zhang, Minsi; Qiu, Qiong; Skuli, Nicolas; Nakazawa, Michael S.; Karakasheva, Tatiana; Mucaj, Vera; Shay, Jessica E.S.; Stangenberg, Lars; Sadri, Navid; Puré, Ellen; Yoon, Sam S.; Kirsch, David G.; Simon, M. Celeste

    2013-01-01

    Intratumoral hypoxia and expression of Hypoxia Inducible Factor 1α (HIF1α) correlate with metastasis and poor survival in sarcoma patients. We demonstrate here that hypoxia controls sarcoma metastasis through a novel mechanism wherein HIF1α enhances expression of the intracellular enzyme procollagen-lysine, 2-oxoglutarate 5-dioxygenase 2 (PLOD2). We show that loss of HIF1α or PLOD2 expression disrupts collagen modification, cell migration and pulmonary metastasis (but not primary tumor growth) in allograft and autochthonous LSLKrasG12D/+; Trp53fl/fl murine sarcoma models. Furthermore, ectopic PLOD2 expression restores migration and metastatic potential in HIF1α-deficient tumors, and analysis of human sarcomas reveal elevated HIF1α and PLOD2 expression in metastatic primary lesions. Pharmacological inhibition of PLOD enzymatic activity suppresses metastases. Collectively, these data indicate that HIF1α controls sarcoma metastasis through PLOD2-dependent collagen modification and organization in primary tumors. We conclude that PLOD2 is a novel therapeutic target in sarcomas and successful inhibition of this enzyme may reduce tumor cell dissemination. PMID:23906982

  18. The role of radiology in paediatric soft tissue sarcomas

    PubMed Central

    van Rijn, R.; McHugh, K.

    2008-01-01

    Abstract Paediatric soft tissue sarcomas (STS) are a group of malignant tumours that originate from primitive mesenchymal tissue and account for 7% of all childhood tumours. Rhabdomyosarcomas (RMS) and undifferentiated sarcomas account for approximately 50% of soft tissue sarcomas in children and non-rhabdomyomatous soft tissue sarcomas (NRSTS) the remainder. The prognosis and biology of STS tumours vary greatly depending on the age of the patient, the primary site, tumour size, tumour invasiveness, histologic grade, depth of invasion, and extent of disease at diagnosis. Over recent years, there has been a marked improvement in survival rates in children and adolescents with soft tissue sarcoma and ongoing international studies continue to aim to improve these survival rates whilst attempting to reduce the morbidity associated with treatment. Radiology plays a crucial role in the initial diagnosis and staging of STS, in the long term follow-up and in the assessment of many treatment related complications. We review the epidemiology, histology, clinical presentation, staging and prognosis of soft tissue sarcomas and discuss the role of radiology in their management. PMID:18442956

  19. Chronic expanding hematoma in the retroperitoneal space: a case report

    PubMed Central

    2013-01-01

    Background Chronic expanding hematoma is a rare condition that develops after surgery, trauma, or injury. It can also develop at any location in the body in the absence of trauma. Clinical findings and various diagnostic imaging modalities can aid in the differential diagnosis of this condition. In general, hematomas are naturally reabsorbed and rarely cause serious problems. However, hematomas that develop slowly without a history of trauma, surgery, or bleeding disorders could be difficult to differentiate from soft tissue neoplasms. In the present case, we describe a patient, without any history or physical evidence of trauma, who exhibited a large chronic expanding hematoma in the retroperitoneal space that resulted in hydronephrosis because of the pressure exerted on the left ureter. Case presentation A 69-year-old man presented to our hospital with a swollen lesion in the left flank. A mass, 19 cm in diameter, was detected in the retroperitoneal space by computed tomography. We suspected the presence of a chronic expanding hematoma, soft tissue tumor, or left renal artery aneurysm. Surgical treatment was performed. However, postoperative histopathological examination indicated that the mass was a nonmalignant chronic expanding hematoma. No recurrence was observed during a 2-year follow-up period. Conclusion In patients without a history of trauma who present slowly growing masses, the differential diagnosis should include chronic expanding hematoma in addition to cysts and soft tissue tumors. Moreover, the use of magnetic resonance imaging and computed tomography is essential to differentiate between chronic expanding hematoma and soft tissue tumors. PMID:24237992

  20. Presence of brown adipocytes in retroperitoneal fat from patients with benign adrenal tumors: relationship with outdoor temperature.

    PubMed

    Betz, Matthias Johannes; Slawik, Marc; Lidell, Martin E; Osswald, Andrea; Heglind, Mikael; Nilsson, Daniel; Lichtenauer, Urs Daniel; Mauracher, Brigitte; Mussack, Thomas; Beuschlein, Felix; Enerbäck, Sven

    2013-10-01

    Brown adipose tissue (BAT) is a metabolically highly active organ with increased thermogenic activity in rodents exposed to cold temperature. Recently its presence in the cervical adipose tissue of human adults and its association with a favorable metabolic phenotype have been reported. The objective of the study was to determine the prevalence of retroperitoneal BAT in human adults. This was an observational cohort study. The study was conducted at a tertiary referral hospital. Fifty-seven patients who underwent surgery for benign adrenal tumors were included in this study. Prevalence of retroperitoneal BAT adjacent to the removed adrenal tumor as determined by uncoupling protein 1 (UCP1) protein and mRNA expression was measured. Using protein and mRNA expression analysis, we detected UCP1 protein in 26 of 57 patients (45.6%) as well as high mRNA expression of genes characteristic for brown adipocytes, independent of the adrenal tumor type. The presence of brown adipocytes within the retroperitoneal fat was associated with a significantly lower outdoor temperature during the month prior to surgery. Importantly, UCP1 expression on both mRNA and protein level was inversely correlated to outdoor temperature, whereas body mass index, sex, age, and diabetes status were not. These findings suggest that human retroperitoneal adipose tissue can acquire a BAT phenotype, thereby adapting to environmental challenges. These adaptive processes might provide a valuable therapeutic target in the treatment of obesity and insulin resistance.

  1. Alveolar Soft Part Sarcoma.

    PubMed

    Jaber, Omar I; Kirby, Patricia A

    2015-11-01

    Alveolar soft part sarcoma is a rare neoplasm usually arising in the soft tissues of the lower limbs in adults and in the head and neck region in children. It presents primarily as a slowly growing mass or as metastatic disease. It is characterized by a specific chromosomal alteration, der(17)t(X:17)(p11:q25), resulting in fusion of the transcription factor E3 (TFE3) with alveolar soft part sarcoma critical region 1 (ASPSCR1) at 17q25. This translocation is diagnostically useful because the tumor nuclei are positive for TFE3 by immunohistochemistry. Real-time polymerase chain reaction to detect the ASPSCR1-TFE3 fusion transcript on paraffin-embedded tissue blocks has been shown to be more sensitive and specific than detection of TFE3 by immunohistochemical stain. Cathepsin K is a relatively recent immunohistochemical stain that can aid in the diagnosis. The recent discovery of the role of the ASPSCR1-TFE3 fusion protein in the MET proto-oncogene signaling pathway promoting angiogenesis and cell proliferation offers a promising targeted molecular therapy.

  2. Novel p53 tumour suppressor mutations in cases of spindle cell sarcoma, pleomorphic sarcoma and fibrosarcoma in cats.

    PubMed

    Mayr, B; Reifinger, M; Alton, K; Schaffner, G

    1998-06-01

    Twenty feline neoplasms were sequenced in the region from exons 5 to 8 for the presence of tumour suppressor gene p53 mutations. In a spindle cell sarcoma of the bladder, a missense mutation (codon 164 AAG-->GAG, lysine-->glutamic acid) in exon 5 was detected. In a pleomorphic sarcoma, a 23 bp deletion involving the splicing junction between intron 5 and exon 6 was observed. In a fibrosarcoma, a 6 bp deletion of p53 covering 2 bp of exon 7 and 4 bp of intron 7, including the splicing junction, was found. The study demonstrates three new p53 mutations in different types of sarcomas in cats.

  3. Retroperitoneal abscess shortly after chemotherapy for lung cancer: A case report.

    PubMed

    Ohara, Gen; Kondo, Tadashi; Kagohashi, Katsunori; Watanabe, Hiroko; Kawaguchi, Mio; Kurishima, Koichi; Satoh, Hiroaki; Hizawa, Nobuyuki

    2014-03-01

    To the best of our knowledge, the formation of a retroperitoneal abscess due to acute appendicitis shortly after administration of chemotherapy for lung cancer has not been previously reported. This is the case report of a 59-year-old male who was admitted to the Mito Medical Center (Mito, Japan) and diagnosed with lung adenocarcinoma with pleuritis carcinomatosis. Although no distant metastasis was identified, combination chemotherapy with cisplatin and pemetrexed was administered. Nine days after initiating chemotherapy, the patient developed right lower quadrant abdominal pain and high fever. Computed tomography (CT) of the abdomen and pelvis revealed the collection of gas and fluid in the retroperitoneum adjacent to the cecum. The abscess was locally drained; however, the infection continued to spread, with subsequent development of a scrotal abscess. Consequently, appendectomy was performed. The patient recovered well and the lung adenocarcinoma was treated with additional courses of chemotherapy following the remission of the local inflammation. Retroperitoneal abscess due to acute appendicitis is an unusual finding; however, this rare complication should be considered during or shortly after chemotherapy in patients with lung cancer.

  4. Treatment of femoral Ewing's sarcoma.

    PubMed

    Terek, R M; Brien, E W; Marcove, R C; Meyers, P A; Lane, J M; Healey, J H

    1996-07-01

    The treatment of Ewing's sarcoma consists of chemotherapy for systemic and local disease. However, the role of radiation therapy, and/or surgical resection for definitive local treatment has yet to be determined. A retrospective review of 32 patients (24 males and 8 females) treated for femoral Ewing's sarcoma between 1970 and 1985 was performed. Patients were divided into 3 treatment groups: chemotherapy and radiotherapy (CR) (10); chemotherapy and surgery (CS) (9); and chemotherapy, surgery, and radiotherapy (CSR) (13). Patients in the CR group received a mean of 5320 centigray (cGy) of radiation and patients in the CSR group received a mean of 3590 cGy. Multiagent cyclophosphamide/doxorubicin based chemotherapy was used in all cases. Surgery consisted of wide resection or amputation. Patients in the CR group had a higher risk of local recurrence than patients in the CS and CSR groups (P=0.02, log rank). The combination of local recurrences and treatment complications necessitated surgery for 7 of 10 CR patients, whereas 1 of 9 and 4 of 13 in the CS and CSR groups required additional surgery. The median survival for the entire group was 39 months. Minimum follow-up for surviving patients was 45 months. Five-year survival consisted of 1 of 10 patients in the CR group, 2 of 9 in the CS group, and 7 of 13 in the CSR group. There were no statistically significant differences among the three survival curves. Tumor location within the femur was a significant prognostic variable. Distal femoral location had a survival advantage compared with proximal and mid-femur locations (P = 0.049, log rank). Femoral Ewing's sarcoma remains a disease with a poor prognosis. Radiation alone for local treatment results in a high rate of local recurrence and complications. Our current local treatment strategy for femoral Ewing's sarcoma includes surgery in all and adjuvant radiotherapy in many of the patients.

  5. Hypertensive crisis during wide excision of gastrointestinal stromal cell tumor (GIST): Undiagnosed paraganglioma -A case report-

    PubMed Central

    Shinn, Helen Ki; Jung, Jong Kwon; Park, Jay Kim; Kim, Jong Hoon; Jung, In Young

    2012-01-01

    Although paraganglioma (PGL), an extra-adrenal retroperitoneal pheochromocytoma (PHEO), is a rare catecholamine-secreting neuroendocrine tumor, it can cause severe hypertensive crisis during anesthesia or surgery if undiagnosed preoperatively. Extraluminal perigastric masses may be presumed to be gastrointestinal stromal tumors (GISTs) or soft tissue sarcomas even when histologic confirmation is not possible. Therefore, without a histologic diagnosis or symptoms of excessive catecholamine secretion, PGL may be mistaken for GIST. We report a case of preoperatively undiagnosed PGL which caused hypertensive crisis during anesthesia for retroperitoneal mass excision. PMID:22474560

  6. Uterine Sarcoma Treatment (PDQ®)—Health Professional Version

    Cancer.gov

    Uterine sarcoma treatment is primarily surgery, with or without radiation or chemotherapy. Get detailed information about the treatment options for newly diagnosed or recurrent uterine sarcoma cancer in this summary for clinicians.

  7. Stereotactic Body Radiotherapy (SBRT) for Pulmonary Metastases in Ewing Sarcoma, Rhabdomyosarcoma, and Wilms Tumors

    ClinicalTrials.gov

    2018-01-31

    Ewing Sarcoma; Rhabdomyosarcoma; Wilms Tumor; Osteosarcoma; Non-Rhabdomyosarcoma Soft Tissue Sarcoma, Nos; Renal Tumor; Rhabdoid Tumor; Clear Cell Renal Cell Carcinoma; Sarcoma; Sarcoma, Ewing; Soft Tissue Sarcoma

  8. Combinatorial Drug Screening Identifies Ewing Sarcoma-specific Sensitivities.

    PubMed

    Radic-Sarikas, Branka; Tsafou, Kalliopi P; Emdal, Kristina B; Papamarkou, Theodore; Huber, Kilian V M; Mutz, Cornelia; Toretsky, Jeffrey A; Bennett, Keiryn L; Olsen, Jesper V; Brunak, Søren; Kovar, Heinrich; Superti-Furga, Giulio

    2017-01-01

    Improvements in survival for Ewing sarcoma pediatric and adolescent patients have been modest over the past 20 years. Combinations of anticancer agents endure as an option to overcome resistance to single treatments caused by compensatory pathways. Moreover, combinations are thought to lessen any associated adverse side effects through reduced dosing, which is particularly important in childhood tumors. Using a parallel phenotypic combinatorial screening approach of cells derived from three pediatric tumor types, we identified Ewing sarcoma-specific interactions of a diverse set of targeted agents including approved drugs. We were able to retrieve highly synergistic drug combinations specific for Ewing sarcoma and identified signaling processes important for Ewing sarcoma cell proliferation determined by EWS-FLI1 We generated a molecular target profile of PKC412, a multikinase inhibitor with strong synergistic propensity in Ewing sarcoma, revealing its targets in critical Ewing sarcoma signaling routes. Using a multilevel experimental approach including quantitative phosphoproteomics, we analyzed the molecular rationale behind the disease-specific synergistic effect of simultaneous application of PKC412 and IGF1R inhibitors. The mechanism of the drug synergy between these inhibitors is different from the sum of the mechanisms of the single agents. The combination effectively inhibited pathway crosstalk and averted feedback loop repression, in EWS-FLI1-dependent manner. Mol Cancer Ther; 16(1); 88-101. ©2016 AACR. ©2016 American Association for Cancer Research.

  9. Molecular pathogenesis and targeted therapeutics in Ewing sarcoma/primitive neuroectodermal tumours

    PubMed Central

    2012-01-01

    Background Ewing sarcoma/PNET is managed with treatment paradigms involving combinations of chemotherapy, surgery, and sometimes radiation. Although the 5-year survival rate of non-metastatic disease approaches 70%, those cases that are metastatic and those that recur have 5-year survival rates of less than 20%. Molecularly targeted treatments offer the potential to further improve treatment outcomes. Methods A PUBMED search was performed from 1997 to 2011. Published literature that included the topic of the Ewing sarcoma/PNET was also referenced. Results Insulin-like growth factor-1 receptor (IGF-1R) antagonists have demonstrated modest single agent efficacy in phase I/II clinical trials in Ewing sarcoma/PNET, but have a strong preclinical rationale. Based on in vitro and animal data, treatment using antisense RNA and cDNA oligonucleotides directed at silencing the EWS-FLI chimera that occurs in most Ewing sarcoma/PNET may have potential therapeutic importance. However drug delivery and degradation problems may limit this therapeutic approach. Protein-protein interactions can be targeted by inhibition of RNA helicase A, which binds to EWS/FLI as part of the transcriptional complex. Tumour necrosis factor related apoptosis inducing ligand induction using interferon has been used in preclinical models. Interferons may be incorporated into future chemotherapeutic treatment paradigms. Histone deacetylase inhibitors can restore TGF-β receptor II allowing TFF-β signalling, which appears to inhibit growth of Ewing sarcoma/PNET cell lines in vitro. Immunotherapy using allogeneic natural killer cells has activity in Ewing sarcoma/PNET cell lines and xenograft models. Finally, cyclin dependent kinase inhibitors such as flavopiridol may be clinically efficacious in relapsed Ewing sarcoma/PNET. Conclusion Preclinical evidence exists that targeted therapeutics may be efficacious in the ESFT. IGF-1R antagonists have demonstrated efficacy in phase I/II clinical trials

  10. Functional genomic screening reveals asparagine dependence as a metabolic vulnerability in sarcoma

    PubMed Central

    Hettmer, Simone; Schinzel, Anna C; Tchessalova, Daria; Schneider, Michaela; Parker, Christina L; Bronson, Roderick T; Richards, Nigel GJ; Hahn, William C; Wagers, Amy J

    2015-01-01

    Current therapies for sarcomas are often inadequate. This study sought to identify actionable gene targets by selective targeting of the molecular networks that support sarcoma cell proliferation. Silencing of asparagine synthetase (ASNS), an amidotransferase that converts aspartate into asparagine, produced the strongest inhibitory effect on sarcoma growth in a functional genomic screen of mouse sarcomas generated by oncogenic Kras and disruption of Cdkn2a. ASNS silencing in mouse and human sarcoma cell lines reduced the percentage of S phase cells and impeded new polypeptide synthesis. These effects of ASNS silencing were reversed by exogenous supplementation with asparagine. Also, asparagine depletion via the ASNS inhibitor amino sulfoximine 5 (AS5) or asparaginase inhibited mouse and human sarcoma growth in vitro, and genetic silencing of ASNS in mouse sarcoma cells combined with depletion of plasma asparagine inhibited tumor growth in vivo. Asparagine reliance of sarcoma cells may represent a metabolic vulnerability with potential anti-sarcoma therapeutic value. DOI: http://dx.doi.org/10.7554/eLife.09436.001 PMID:26499495

  11. Extraskeletal presentation of Ewing's Sarcoma.

    PubMed

    Mangual, Danny; Bisbal-Matos, Luis A; Jiménez-Lee, Ricardo; Vélez, Román; Noy, Miguel

    2018-03-01

    The case of a 27-year-old Hispanic female who presented with an occipito-parietal tumor after suffering trauma to the area. A physical examination revealed no tenderness to palpation and with evidence of healing ulcerations. The biopsy was consistent with a synovial sarcoma. A wide excision of the mass (15cm x 14cm x 6cm) followed by a pericranial flap was performed. A follow-up CT showed recurrence involving the parietal sagittal sinus. After a second biopsy the mass was determined to be a small-cell sarcoma, consistent with Ewing's sarcoma. Chemotherapy included 8 cycles of doxorubicin, vincristine, and cyclophosphamide, with alternating cycles of etoposide and ifosfamide. A year later, a second wide excision of the mass was performed, followed by bilaminate skin substitute and skin graft placement for reconstruction of the soft-tissue defect. After chemotherapy, a follow-up PET scan showed no signs of re-uptake in any soft tissue or skeletal structures. After 2 years, the patient remains in complete remission.

  12. Studying Genes in Tissue Samples From Younger and Adolescent Patients With Soft Tissue Sarcomas

    ClinicalTrials.gov

    2016-05-13

    Childhood Alveolar Soft-part Sarcoma; Childhood Angiosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Mesenchymoma; Childhood Neurofibrosarcoma; Childhood Synovial Sarcoma; Chordoma; Desmoid Tumor; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Recurrent Childhood Soft Tissue Sarcoma

  13. Epidemiological Evaluation of Head and Neck Sarcomas in Iran (the Study of 105 Cases Over 13 Years)

    PubMed Central

    Alishahi, Batoul; Kargahi, Neda; Homayouni, Solmaz

    2015-01-01

    Background: Head and neck sarcomas are exceedingly rare and they include 4% - 10% of all sarcomas and less than 1% of all neoplasm of head and neck. Objectives: The aim of this study is to evaluate the epidemiological characteristics of head and neck sarcomas of patients in Isfahan, Iran. Patients and Methods: In this retrospective study, from the 16000 patients whose files were evaluated, the total number of 105 head and neck sarcomas were collected. They were evaluated with due attention to age, gender of the patients and the most common location of the lesion. Results: From the total number of 105 (0.6%) patients with sarcomas, 56 were men (53.33%) and 49 women (46.66%). The most common head and neck sarcomas among this population were Osteosarcoma (32 cases, 30.47%), Chondrosarcoma (14 cases, 13.33%), and Ewing sarcoma (11 cases, 10.47%).The most common soft tissue sarcoma was Rabdomiosarcoma. Mandible was the most common location for these lesions. Conclusions: In this study, the hard tissue sarcomas were more prevalent than soft tissue ones. Hence, special attention should be paid to the patients when being diagnosed. PMID:26478791

  14. Principles of treatment for soft tissue sarcoma.

    PubMed

    Dernell, W S; Withrow, S J; Kuntz, C A; Powers, B E

    1998-02-01

    Soft tissue sarcomas (STS) are mesenchymal tumors arising from connective tissue elements and are grouped together based on a common biologic behavior. The most common histologic types include malignant peripheral nerve sheath tumors (schwannoma and neurofibrosarcoma) "hemangiopericytoma," fibrosarcoma, and malignant fibrous histiocytoma. These tumors are relatively slow growing yet locally invasive with a high rate of recurrence following conservative management. Appropriate preoperative planning and aggressive surgical resection often result in long-term remission or cure. Identification and evaluation of resection margins are paramount in appropriate case management. The addition of radiotherapy after surgical resection can aid in remission for incompletely resected masses. Systemic chemotherapy for STS should be considered for high-grade tumors with a moderate metastatic potential. Potential prognostic factors include grade, resection margins, size, location, histologic type, and previous treatment, with grade and margins being the most important. Tumor types classified as STS that differ slightly in their presentation or treatment, including synovial cell sarcoma, rhabdomyosarcoma, liposarcoma, and vaccine-associated STS in cats, are discussed. Soft tissue sarcomas can be a frustrating disease to treat, but adherence to solid surgical oncology principles can greatly increase the odds of good disease control.

  15. The Hippo signal transduction pathway in soft tissue sarcomas.

    PubMed

    Mohamed, Abdalla D; Tremblay, Annie M; Murray, Graeme I; Wackerhage, Henning

    2015-08-01

    Sarcomas are rare cancers (≈1% of all solid tumours) usually of mesenchymal origin. Here, we review evidence implicating the Hippo pathway in soft tissue sarcomas. Several transgenic mouse models of Hippo pathway members (Nf2, Mob1, LATS1 and YAP1 mutants) develop various types of sarcoma. Despite that, Hippo member genes are rarely point mutated in human sarcomas. Instead, WWTR1-CAMTA1 and YAP1-TFE3 fusion genes are found in almost all cases of epithelioid haemangioendothelioma. Also copy number gains of YAP1 and other Hippo members occur at low frequencies but the most likely cause of perturbed Hippo signalling in sarcoma is the cross-talk with commonly mutated cancer genes such as KRAS, PIK3CA, CTNNB1 or FBXW7. Current Hippo pathway-targeting drugs include compounds that target the interaction between YAP and TEAD G protein-coupled receptors (GPCR) and the mevalonate pathway (e.g. statins). Given that many Hippo pathway-modulating drugs are already used in patients, this could lead to early clinical trials testing their efficacy in different types of sarcoma. Crown Copyright © 2015. Published by Elsevier B.V. All rights reserved.

  16. Pharmacokinetics of cephem antibiotics in exudate of pelvic retroperitoneal space after radical hysterectomy and pelvic lymphadenectomy.

    PubMed Central

    Ito, K; Hayasaki, M; Tamaya, T

    1990-01-01

    Many cephalosporin antibiotics have recently been invented and attempts have been made to use them clinically. The choice of which of these drugs should be used has been difficult in gynecology. The efficacies of these drugs depend on their antibacterial spectra, potencies, and concentrations in tissues. This study was designed to investigate the pharmacokinetics of various cephem antibiotics in the exudate of the retroperitoneal space that is formed after radical hysterectomy and pelvic lymphadenectomy. These cephem antibiotics were cefoxitin, cefotiam, cefotetan, cefpiramide, cefminox, cefotaxime, ceftizoxime, cefoperazone, cefmenoxime, cefbuperazone, ceftazidime, cefpimizole, flomoxef, and cefuzonam. The maximum concentrations after administration of a 1-g dose in the exudate of the pelvic retroperitoneal space were 37.9 micrograms/ml with cefminox, 30.3 micrograms/ml with cefpimizole, 21.6 micrograms/ml with flomoxef, 21.5 micrograms/ml with ceftazidime, and 17.6 micrograms/ml with cefbuperazone, which were relatively high. When selecting antibiotics for prophylactic use against infections in the retroperitoneal space after radical hysterectomy and pelvic lymphadenectomy, on the basis of drug transfer, flomoxef, cefminox, cefbuperazone, ceftazidime, and cefpimizole were considered to be the drugs of first choice at a dose of 1 g. PMID:2393276

  17. Primary extraskeletal Ewing's sarcoma/primitive neuroectodermal tumour of breast.

    PubMed

    Ikhwan, S M; Kenneth, V K T; Seoparjoo, A; Zin, A A M

    2013-06-21

    Primary primitive neuroectodermal tumour (PNET) and extraskeletal Ewing's sarcoma belongs to the Ewing's family of tumours. Primary tumours arising from breast are very rare. There are only a few case reports published on primary extraskeletal Ewing's sarcoma and PNET arising from breast. We present an extremely rare case of an inoperable primary Ewing's sarcoma arising from left breast with contralateral breast, lymphatic and lung metastasis.

  18. Primary extra-skeletal Ewing's sarcoma mimicking a disc protrusion.

    PubMed

    Ruelle, A; Boccardo, M

    1987-07-01

    One of the rarest cases of primary epidural neoplasm is a soft tissue sarcoma histologically similar to Ewing's sarcoma of the bone. In the literature only eleven cases of such an extra-skeletal Ewing's sarcoma have been described. The authors report an additional case presenting as a disc protrusion in a young male. The authors include some diagnostic, prognostic and nosologic remarks about this condition.

  19. Granulocytic sarcoma of the ovary in a nonleukemic patient.

    PubMed

    Aguiar, R C; Pozzi, D H; Chamone, D A

    1993-01-01

    We report a case of granulocytic sarcoma of the ovary preceding acute myeloid leukemia by twelve months, with no evidence of any hematological involvement at the time of first diagnosis. The patient was initially treated with surgery and chemotherapy for undifferentiated lymphoma and, although this aggressive protocol resulted in a complete response, granulocytic sarcoma recurred as extramedullary disease, followed by the appearance of acute myeloid leukemia. We discuss the clinical, histopathological and immunohistochemical features of the disease, the differential diagnosis and, in particular, the role of early aggressive treatment on the outcome of the patient.

  20. Extra osseous primary Ewing's sarcoma.

    PubMed

    Ali, Syed Asad; Muhammad, Agha Taj; Soomro, Abdul Ghani; Siddiqui, Akmal Jamal

    2010-01-01

    The case of 20 years old boy with an extra osseous Ewing's sarcoma is described. He was initially diagnosed as a case of infiltrative malignant tumour of left suprarenal gland on the basis of preoperative workup but postoperative biopsy of surgically excised specimen confirmed Extra-osseous Ewing's Sarcoma (EES) suprarenal gland with no evidence of malignancy on skeletal scintiscan, bone marrow aspirate and histopathology Suprarenal location of primary EES is unknown and probably has not been reported in literature. We report a unique case of EES.

  1. Cell Cycle Deregulation in Ewing's Sarcoma Pathogenesis

    PubMed Central

    Kowalewski, Ashley A.; Randall, R. Lor; Lessnick, Stephen L.

    2011-01-01

    Ewing's sarcoma is a highly aggressive pediatric tumor of bone that usually contains the characteristic chromosomal translocation t(11;22)(q24;q12). This translocation encodes the oncogenic fusion protein EWS/FLI, which acts as an aberrant transcription factor to deregulate target genes necessary for oncogenesis. One key feature of oncogenic transformation is dysregulation of cell cycle control. It is therefore likely that EWS/FLI and other cooperating mutations in Ewing's sarcoma modulate the cell cycle to facilitate tumorigenesis. This paper will summarize current published data associated with deregulation of the cell cycle in Ewing's sarcoma and highlight important questions that remain to be answered. PMID:21052502

  2. Extraskeletal Ewing sarcoma of the abdominal wall

    PubMed Central

    Farhat, L. Ben; Ghariani, B.; Rabeh, A.; Dali, N.; Said, W.; Hendaoui, L.

    2008-01-01

    Abstract Ewing sarcoma is most commonly a bone tumour which has usually extended into the soft tissues at the time of diagnosis. Exceptionally, this tumour can have an extraskeletal origin. Clinical or imaging findings are non-specific and diagnosis is based on histology. We report a case of an extraskeletal Ewing sarcoma developed in the soft tissues of the abdominal wall in a 35-year-old woman who presented a painful abdominal wall tumefaction. Ultrasongraphy and computed tomography showed a large, well-defined soft tissue mass developed in the left anterolateral muscle group of the abdominal wall. Surgical biopsy was performed and an extraskeletal Ewing sarcoma was identified histologically. PMID:18818133

  3. Molecular Pathogenesis and Diagnostic, Prognostic and Predictive Molecular Markers in Sarcoma.

    PubMed

    Mariño-Enríquez, Adrián; Bovée, Judith V M G

    2016-09-01

    Sarcomas are infrequent mesenchymal neoplasms characterized by notable morphological and molecular heterogeneity. Molecular studies in sarcoma provide refinements to morphologic classification, and contribute diagnostic information (frequently), prognostic stratification (rarely) and predict therapeutic response (occasionally). Herein, we summarize the major molecular mechanisms underlying sarcoma pathogenesis and present clinically useful diagnostic, prognostic and predictive molecular markers for sarcoma. Five major molecular alterations are discussed, illustrated with representative sarcoma types, including 1. the presence of chimeric transcription factors, in vascular tumors; 2. abnormal kinase signaling, in gastrointestinal stromal tumor; 3. epigenetic deregulation, in chondrosarcoma, chondroblastoma, and other tumors; 4. deregulated cell survival and proliferation, due to focal copy number alterations, in dedifferentiated liposarcoma; 5. extreme genomic instability, in conventional osteosarcoma as a representative example of sarcomas with highly complex karyotype. Copyright © 2016 Elsevier Inc. All rights reserved.

  4. Therapeutic Angiotensin-(1-7) in Treating Patients With Metastatic Sarcoma That Cannot Be Removed By Surgery

    ClinicalTrials.gov

    2018-02-27

    Bone Cancer; Chondrosarcoma; Clear Cell Sarcoma of the Kidney; Metastatic Osteosarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Osteosarcoma; Recurrent Uterine Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma

  5. State of the art in myeloid sarcoma.

    PubMed

    Klco, J M; Welch, J S; Nguyen, T T; Hurley, M Y; Kreisel, F H; Hassan, A; Lind, A C; Frater, J L

    2011-12-01

    Myeloid sarcomas are extramedullary lesions composed of myeloid lineage blasts that typically form tumorous masses and may precede, follow, or occur in the absence of systemic acute myeloid leukemia. They most commonly involve the skin and soft tissues, lymph nodes, and gastrointestinal tract and are particularly challenging to diagnose in patients without an antecedent history of acute myeloid leukemia. We conducted a search of the English language medical literature for recent studies of interest to individuals involved in the diagnosis of myeloid sarcoma. The differential diagnosis includes non-Hodgkin lymphoma, blastic plasmacytoid dendritic cell neoplasm, histiocytic sarcoma, melanoma, carcinoma, and (in children) small round blue cell tumors. The sensitivity and specificity of immunohistochemical markers must be considered when evaluating a suspected case of myeloid sarcoma. A high percentage of tested cases have cytogenetic abnormalities. A minimal panel of immunohistochemical markers should include anti-CD43 or anti-lysozyme as a lack of immunoreactivity for either of these sensitive markers would be inconsistent with a diagnosis of myeloid sarcoma. Use of more specific markers of myeloid disease, such as CD33, myeloperoxidase, CD34 and CD117 is necessary to establish the diagnosis. Other antibodies may be added depending on the differential diagnosis. Identification of acute myeloid leukemia-associated genetic lesions may be helpful in arriving at the correct diagnosis. © 2011 Blackwell Publishing Ltd.

  6. Ewing Sarcoma Treatment (PDQ®)—Health Professional Version

    Cancer.gov

    Ewing sarcoma is derived from a primordial bone marrow–derived mesenchymal stem cell. Get comprehensive information about the presentation, genomics, diagnostic evaluation, prognosis, and treatment of newly diagnosed and recurrent Ewing sarcoma in this summary for clinicians.

  7. Safe and Effective Sarcoma Therapy through Bispecific Targeting of EGFR and uPAR

    PubMed Central

    Borgatti, Antonella; Koopmeiners, Joseph S.; Sarver, Aaron L.; Winter, Amber L.; Stuebner, Kathleen; Todhunter, Deborah; Rizzardi, Anthony E.; Henriksen, Jonathan C.; Schmechel, Stephen; Forster, Colleen L.; Kim, Jong-Hyuk; Froelich, Jerry; Walz, Jillian; Henson, Michael S.; Breen, Matthew; Lindblad-Toh, Kerstin; Oh, Felix; Pilbeam, Kristy; Modiano, Jaime F.; Vallera, Daniel A.

    2017-01-01

    Sarcomas differ from carcinomas in their mesenchymal origin. Therapeutic advancements have come slowly so alternative drugs and models are urgently needed. These studies report a new drug for sarcomas that simultaneously targets both tumor and tumor neovasculature. eBAT is a bispecific angiotoxin consisting of truncated, deimmunized Pseudomonas exotoxin fused to epidermal growth factor (EGF) and the amino terminal fragment (ATF) of urokinase. Here, we study the drug in an in vivo “ontarget” companion dog trial since eBAT effectively kills canine hemangiosarcoma (HSA) and human sarcoma cells in vitro. We reasoned the model has value due to the common occurrence of spontaneous sarcomas in dogs and a limited lifespan allowing for rapid accrual and data collection. Splenectomized dogs with minimal residual disease were given one cycle of eBAT followed by adjuvant doxorubicin in an adaptive dose-finding, phase I–II study of 23 dogs with spontaneous, stage I–II, splenic HSA. eBAT improved 6-month survival from <40% in a comparison population to ~70% in dogs treated at a biologically active dose (50 μg/kg). Six dogs were long-term survivors, living >450 days. eBAT abated expected toxicity associated with EGFR-targeting, a finding supported by mouse studies. Urokinase plasminogen activator receptor (uPAR) and EGFR are targets for human sarcomas, so thorough evaluation is crucial for validation of the dog model. Thus, we validated these markers for human sarcoma targeting in the study of 212 human and 97 canine sarcoma samples. Our results support further translation of eBAT for human patients with sarcomas and perhaps other EGFR-expressing malignancies. PMID:28193671

  8. Observation, Radiation Therapy, Combination Chemotherapy, and/or Surgery in Treating Young Patients With Soft Tissue Sarcoma

    ClinicalTrials.gov

    2017-09-07

    Adult Alveolar Soft-part Sarcoma; Adult Angiosarcoma; Adult Epithelioid Sarcoma; Adult Extraskeletal Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Malignant Hemangiopericytoma; Adult Malignant Mesenchymoma; Adult Neurofibrosarcoma; Adult Synovial Sarcoma; Childhood Alveolar Soft-part Sarcoma; Childhood Angiosarcoma; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Mesenchymoma; Childhood Neurofibrosarcoma; Childhood Synovial Sarcoma; Dermatofibrosarcoma Protuberans; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  9. Mesenchymal Stem Cells and the Origin of Ewing's Sarcoma

    PubMed Central

    Lin, Patrick P.; Wang, Yongxing; Lozano, Guillermina

    2011-01-01

    The origin of Ewing's sarcoma is a subject of much debate. Once thought to be derived from primitive neuroectodermal cells, many now believe it to arise from a mesenchymal stem cell (MSC). Expression of the EWS-FLI1 fusion gene in MSCs changes cell morphology to resemble Ewing's sarcoma and induces expression of neuroectodermal markers. In murine cells, transformation to sarcomas can occur. In knockdown experiments, Ewing's sarcoma cells develop characteristics of MSCs and the ability to differentiate into mesodermal lineages. However, it cannot be concluded that MSCs are the cell of origin. The concept of an MSC still needs to be rigorously defined, and there may be different subpopulations of mesenchymal pluripotential cells. Furthermore, EWS-FLI1 by itself does not transform human cells, and cooperating mutations appear to be necessary. Therefore, while it is possible that Ewing's sarcoma may originate from a primitive mesenchymal cell, the idea needs to be refined further. PMID:20953407

  10. Like or Dislike? Impact of Facebook on Ewing Sarcoma Treatment.

    PubMed

    Ruckenstuhl, Paul; Schippinger, Michael; Liebmann, Paul; Leithner, Andreas; Bernhardt, Gerwin

    2016-08-25

    ' and relatives' results. During the last 6 months most activities in the group concerned sharing destiny and handling the diagnosis. The Facebook group Ewing Sarcoma Awareness has a relevant impact on group members regarding their choice of treatment. Moreover, participants turn toward the group to receive mental and emotional support in everyday life. Statements made within the group are in part questionable from a medical point of view and the impact made by these statements on patients' care requires further evaluation. ©Paul Ruckenstuhl, Michael Schippinger, Paul Liebmann, Andreas Leithner, Gerwin Bernhardt. Originally published in JMIR Cancer (http://cancer.jmir.org), 25.08.2016.

  11. Primary Intimal Sarcoma of Thoracic Aorta Presenting as Hypertensive Crisis.

    PubMed

    Lin, Shu-I; Su, Min-I; Tsai, Cheng-Ting

    2015-11-01

    We report a 45-year-old woman who presented to our facility in a hypertensive crisis. Computed tomography (CT) revealed a thoracic aortic tumor, and tissues obtained via endovascular biopsy revealed undifferentiated sarcoma. A final diagnosis of intimal sarcoma was made by intra-operative pathological examination. Despite undergoing surgical resection followed by adjuvant chemotherapy, the patient died from progressive multiple metastasis and severe sepsis. Although aortic sarcoma is rarely diagnosed, it should be considered a possible etiology of hypertensive crisis. Aortic tumor; Endovascular biopsy; Hypertension crisis; Intimal sarcoma.

  12. [Clinical Evaluation of Diagnostic and Treatment Protocol of Idiopathic Retroperitoneal Fibrosis Incorporating Consideration of Possible IgG4-Related Disease].

    PubMed

    Iyoki, Takaya; Maehana, Takeshi; Tanaka, Toshiaki; Yamamoto, Motohisa; Takahashi, Hiroki; Masumori, Naoya

    2017-11-01

    About half of idiopathic retroperitoneal fibrosis might be classified as a IgG4-related disease, a newly characterized disease that is especially known to be sensitive to steroid therapy. We developed a new protocol for diagnosis and treatment of retroperitoneal fibrosis, which included aggressive diagnosis of IgG4- related disease. We retrospectively reviewed 22 cases with idiopathic retroperitoneal fibrosis that were diagnosed and treated according to our protocol. Of them, 10 patients (45.5%) had no evidence of IgG4- related disease (non-IgG4RD group), whereas 12 patients (54.5%) were diagnosed with IgG4-related disease (IgG4RD group). All patients received steroid therapy, and 13 patients (59.1%) underwent ureteral stenting or received prednisolone (PNS). There was no severe adverse event and planned steroid therapy was completed in all patients. In principle, maintenance steroid therapy was continued after induction therapy in the IgG4RD group, whereas steroid therapy was discontinued in the non-IgG4RD group. Regression of retroperitoneal plaque was achieved in all 22 patients. Four (57.1%) out of 7 patients and 3 (50.0%) out of 6 patients achieved freedom from ureteral stent or PNS in the non-IgG4RD group and IgG4RD group, respectively. All 3 patients with PNS became catheter-free after treatment, whereas only 4 (40.0%) of the 10 patients withureteral stent could become stent-free. Steroid therapy could be discontinued in 7 patients (70.0%) in the non-IgG4RD group. The results of this study suggest that similar efficacy of steroid therapy can be expected in the non-IgG4RD group and IgG4RD group.

  13. Advances in sarcoma genomics and new therapeutic targets

    PubMed Central

    Taylor, Barry S.; Barretina, Jordi; Maki, Robert G.; Antonescu, Cristina R.; Singer, Samuel; Ladanyi, Marc

    2012-01-01

    Preface Increasingly, human mesenchymal malignancies are classified by the abnormalities that drive their pathogenesis. While many of these aberrations are highly prevalent within particular sarcoma subtypes, few are currently targeted therapeutically. Indeed, most subtypes of sarcoma are still treated with traditional therapeutic modalities and in many cases are resistant to adjuvant therapies. In this Review, we discuss the core molecular determinants of sarcomagenesis and emphasize the emerging genomic and functional genetic approaches that, coupled to novel therapeutic strategies, have the potential to transform the care of patients with sarcoma. PMID:21753790

  14. Molecular profiling of sarcomas: new vistas for precision medicine.

    PubMed

    Al-Zaid, Tariq; Wang, Wei-Lien; Somaiah, Neeta; Lazar, Alexander J

    2017-08-01

    Sarcoma is a large and heterogeneous group of malignant mesenchymal neoplasms with significant histological overlap. Accurate diagnosis can be challenging yet important for selecting the appropriate treatment approach and prognosis. The currently torrid pace of new genomic discoveries aids our classification and diagnosis of sarcomas, understanding of pathogenesis, development of new medications, and identification of alterations that predict prognosis and response to therapy. Unfortunately, demonstrating effective targets for precision oncology has been elusive in most sarcoma types. The list of potential targets greatly outnumbers the list of available inhibitors at the present time. This review will discuss the role of molecular profiling in sarcomas in general with emphasis on selected entities with particular clinical relevance.

  15. Kaposi Sarcoma Treatment (PDQ®)—Health Professional Version

    Cancer.gov

    Kaposi sarcoma treatment depends upon the type and can include curettage and desiccation, radiation therapy, surgery, and sometimes chemotherapy. Get detailed information about the diagnosis and treatment of newly diagnosed and recurrent Kaposi sarcoma in this summary for clinicians.

  16. [Kaposi's sarcoma of the penis in a HIV-seronegative patient].

    PubMed

    Touzani, Mohammed Alae; Yddoussalah, Othmane

    2017-01-01

    Sarcomas of the penis account for less than 5% of all tumors of the penis. They are dominated by Kaposi's sarcoma that mainly affects HIV-positive patients. However, recent studies have shown a relationship between Kaposi's sarcoma and HHV-8 infection (Human herpes virus-8), which explains why this sarcoma occurs in non-immunocompromised and HIV-seronegative patients. We here report the case of a 72-year old patient, with no previous medical history, reporting of gradual onset of tumor-like granulation tissue of 3 years duration at the level of the gland, without secondary location. Given the patient's clinical condition, epidermoid carcinoma or sarcomatoid carcinoma of the penis were suspected. Initial biopsy was negative, the second was in favor of Kaposi's sarcoma, confirmed by immunohistochemistry. The patient underwent chemotherapy.

  17. Principles of treatment for vaccine-associated sarcomas.

    PubMed

    Novosad, C Andrew

    2003-05-01

    In the last decade, there has been a great deal of information surrounding the etiology, diagnosis, and treatment of feline vaccine-associated sarcomas. The presence of a mass in areas used for subcutaneous or intramuscular injections should alert the clinician to the possibility of a vaccine-associated sarcoma. Early detection and subsequent treatment is paramount to limit local invasion and distant metastasis. The current data are suggesting that a team approach with multi-modality therapy is the appropriate way to address this disease. In the following article, we will discuss the history/incidence, pathology, diagnosis, and current treatment options, which include a combination of surgery, radiation, and chemotherapy for vaccine-associated sarcomas.

  18. Correlation between DNA ploidy, metaphase high-resolution comparative genomic hybridization results and clinical outcome of synovial sarcoma

    PubMed Central

    2011-01-01

    Background Although synovial sarcoma is the 3rd most commonly occurring mesenchymal tumor in young adults, usually with a highly aggressive clinical course; remarkable differences can be seen regarding the clinical outcome. According to comparative genomic hybridization (CGH) data published in the literature, the simple and complex karyotypes show a correlation between the prognosis and clinical outcome. In addition, the connection between DNA ploidy and clinical course is controversial. The aim of this study was using a fine-tuning interpretation of our DNA ploidy results and to compare these with metaphase high-resolution CGH (HR-CGH) results. Methods DNA ploidy was determined on Feulgen-stained smears in 56 synovial sarcoma cases by image cytometry; follow up was available in 46 cases (average: 78 months). In 9 cases HR-CGH analysis was also available. Results 10 cases were found DNA-aneuploid, 46 were DNA-diploid by image cytometry. With fine-tuning of the diploid cases according to the 5c exceeding events (single cell aneuploidy), 33 cases were so called "simple-diploid" (without 5c exceeding events) and 13 cases were "complex-diploid"; containing 5c exceeding events (any number). Aneuploid tumors contained large numbers of genetic alterations with the sum gain of at least 2 chromosomes (A-, B- or C-group) detected by HR-CGH. In the "simple-diploid" cases no or few genetic alterations could be detected, whereas the "complex-diploid" samples numerous aberrations (equal or more than 3) could be found. Conclusions Our results show a correlation between the DNA-ploidy, a fine-tuned DNA-ploidy and the HR-CGH results. Furthermore, we found significant correlation between the different ploidy groups and the clinical outcome (p < 0.05). PMID:22053830

  19. Granulocytic sarcoma.

    PubMed

    Hutchison, R E; Kurec, A S; Davey, F R

    1990-12-01

    Granulocytic sarcoma is a variant presentation of acute myeloblastic leukemia, occurring in extramedullary locations. It is uncommon, but it may occur at any site and at any age, which necessitates its inclusion in the differential diagnosis of all undifferentiated tumors. Histology, touch-imprint cytology, cytochemistry, immunocytochemistry, electron microscopy, and molecular studies all contribute to the diagnosis.

  20. Variable Expression of PIK3R3 and PTEN in Ewing Sarcoma Impacts Oncogenic Phenotypes

    PubMed Central

    Niemeyer, Brian F.; Parrish, Janet K.; Spoelstra, Nicole S.; Joyal, Teresa; Richer, Jennifer K.; Jedlicka, Paul

    2015-01-01

    Ewing Sarcoma is an aggressive malignancy of bone and soft tissue affecting children and young adults. Ewing Sarcoma is driven by EWS/Ets fusion oncoproteins, which cause widespread alterations in gene expression in the cell. Dysregulation of receptor tyrosine kinase signaling, particularly involving IGF-1R, also plays an important role in Ewing Sarcoma pathogenesis. However, the basis of this dysregulation, including the relative contribution of EWS/Ets-dependent and independent mechanisms, is not well understood. In the present study, we identify variable expression of two modifiers of PI3K signaling activity, PIK3R3 and PTEN, in Ewing Sarcoma, and examine the consequences of this on PI3K pathway regulation and oncogenic phenotypes. Our findings indicate that PIK3R3 plays a growth-promotional role in Ewing Sarcoma, but suggest that this role is not strictly dependent on regulation of PI3K pathway activity. We further show that expression of PTEN, a well-established, potent tumor suppressor, is lost in a subset of Ewing Sarcomas, and that this loss strongly correlates with high baseline PI3K pathway activity in cell lines. In support of functional importance of PTEN loss in Ewing Sarcoma, we show that re-introduction of PTEN into two different PTEN-negative Ewing Sarcoma cell lines results in downregulation of PI3K pathway activity, and sensitization to the IGF-1R small molecule inhibitor OSI-906. Our findings also suggest that PTEN levels may contribute to sensitivity of Ewing Sarcoma cells to the microtubule inhibitor vincristine, a relevant chemotherapeutic agent in this cancer. Our studies thus identify PIK3R3 and PTEN as modifiers of oncogenic phenotypes in Ewing Sarcoma, with potential clinical implications. PMID:25603314

  1. Congenital extraskeletal Ewing's sarcoma of chest wall--a rare case report.

    PubMed

    Atla, Bhagyalakshmi; Prasad, B Satya Vara; Sri, K Satya; Vandana, Geeta

    2011-01-01

    Congenital extraskeletal Ewing's sarcoma or peripheral primitive neuroectodermal tumor is an extremely uncommon and invariably fatal tumor. We report a case of extraskeletal congenital Ewing's sarcoma in a female fetus delivered at 34 weeks of gestation who died immediately after birth. In English literature, majority of cases of Ewing's sarcoma in neonates reported were skeletal. To the best of our knowledge, very few cases of extra-skeletal Ewing's sarcoma in neonates are reported in the literature.

  2. Establishment and characterization of the NCC-SS1-C1 synovial sarcoma cell line.

    PubMed

    Kito, Fusako; Oyama, Rieko; Takai, Yoko; Sakumoto, Marimu; Shiozawa, Kumiko; Qiao, Zhiwei; Uehara, Takenori; Yoshida, Akihiko; Kawai, Akira; Kondo, Tadashi

    2018-04-01

    Synovial sarcoma is an aggressive mesenchymal malignancy characterized by unique gene fusions. Tissue culture cells are essential tools for further understanding tumorigenesis and anti-cancer drug development; however, only a limited number of well-characterized synovial sarcoma cell lines exist. Thus, the objective of this study was to establish a patient-derived synovial sarcoma cell line. We established a synovial sarcoma cell line from tumor tissue isolated from a 72-year-old female patient. Prepared cells were analyzed for the presence of gene fusions by fluorescence in situ hybridization, RT-PCR, and karyotyping. In addition, the resulting cell line was characterized by viability, short tandem repeat, colony and spheroid formation, and invasion analyses. Differences in gene enrichment between the primary tumor and cell line were examined by mass spectrometric protein expression profiling and KEGG pathway analysis. Our analyses revealed that the primary tumor and NCC-SS1-C1 cell line harbored the SS18-SSX1 fusion gene typical of synovial sarcoma and similar proteomics profiles. In vitro analyses also confirmed that the established cell line harbored invasive, colony-forming, and spheroid-forming potentials. Moreover, drug screening with chemotherapeutic agents and tyrosine kinase inhibitors revealed that doxorubicin, a subset of tyrosine kinase inhibitors, and several molecular targeting drugs markedly decreased NCC-SS1-C1 cell viability. Results from the present study support that the NCC-SS1-C1 cell line will be an effective tool for sarcoma research.

  3. Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma

    ClinicalTrials.gov

    2017-11-01

    Adult Alveolar Soft Part Sarcoma; Adult Angiosarcoma; Adult Desmoplastic Small Round Cell Tumor; Adult Epithelioid Hemangioendothelioma; Adult Epithelioid Sarcoma; Adult Extraskeletal Myxoid Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Mesenchymoma; Adult Malignant Peripheral Nerve Sheath Tumor; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Adult Undifferentiated Pleomorphic Sarcoma; Malignant Adult Hemangiopericytoma; Recurrent Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  4. Microarray-based DNA methylation study of Ewing's sarcoma of the bone.

    PubMed

    Park, Hye-Rim; Jung, Woon-Won; Kim, Hyun-Sook; Park, Yong-Koo

    2014-10-01

    Alterations in DNA methylation patterns are a hallmark of malignancy. However, the majority of epigenetic studies of Ewing's sarcoma have focused on the analysis of only a few candidate genes. Comprehensive studies are thus lacking and are required. The aim of the present study was to identify novel methylation markers in Ewing's sarcoma using microarray analysis. The current study reports the microarray-based DNA methylation study of 1,505 CpG sites of 807 cancer-related genes from 69 Ewing's sarcoma samples. The Illumina GoldenGate Methylation Cancer Panel I microarray was used, and with the appropriate controls (n=14), a total of 92 hypermethylated genes were identified in the Ewing's sarcoma samples. The majority of the hypermethylated genes were associated with cell adhesion, cell regulation, development and signal transduction. The overall methylation mean values were compared between patients who survived and those that did not. The overall methylation mean was significantly higher in the patients who did not survive (0.25±0.03) than in those who did (0.22±0.05) (P=0.0322). However, the overall methylation mean was not found to significantly correlate with age, gender or tumor location. GDF10 , OSM , APC and HOXA11 were the most significant differentially-methylated genes, however, their methylation levels were not found to significantly correlate with the survival rate. The DNA methylation profile of Ewing's sarcoma was characterized and 92 genes that were significantly hypermethylated were detected. A trend towards a more aggressive behavior was identified in the methylated group. The results of this study indicated that methylation may be significant in the development of Ewing's sarcoma.

  5. Gemcitabine Hydrochloride, Docetaxel, and Radiation Therapy in Treating Patients With Uterine Sarcoma That Has Been Removed By Surgery

    ClinicalTrials.gov

    2015-01-16

    Stage IA Uterine Sarcoma; Stage IB Uterine Sarcoma; Stage IC Uterine Sarcoma; Stage IIA Uterine Sarcoma; Stage IIB Uterine Sarcoma; Stage IIIA Uterine Sarcoma; Stage IIIB Uterine Sarcoma; Stage IIIC Uterine Sarcoma; Stage IVA Uterine Sarcoma; Stage IVB Uterine Sarcoma; Uterine Corpus Leiomyosarcoma

  6. Differentiating retroperitoneal liposarcoma tumors with optical coherence tomography

    NASA Astrophysics Data System (ADS)

    Lev, Dina; Baranov, Stepan A.; Carbajal, Esteban F.; Young, Eric D.; Pollock, Raphael E.; Larin, Kirill V.

    2011-03-01

    Liposarcoma (LS) is a rare and heterogeneous group of malignant mesenchymal neoplasms exhibiting characteristics of adipocytic differentiation. Currently, radical surgical resection represents the most effective and widely used therapy for patients with abdominal/retroperitoneal LS, but the presence of contiguous essential organs, such as the kidney, pancreas, spleen, adrenal glands, esophagus or colon, as well as often reoccurrence of LS in A/RP calls for the enhancement of surgical techniques to minimize resection and avoid LS reoccurrences. Difficulty in detecting the margins of neoplasms due to their affinity to healthy fat tissue accounts for the high reoccurrence of LS within A/RP. Nowadays, the microscopic detection of margins is possible only by use of biopsy, and the minimization of surgical resection of healthy tissues is challenging. In this presentation we'll demonstrate the initial OCT results for the imaging and distinction of LS and normal human fat tissues and clear detection of tumor boundaries.

  7. Serum vascular endothelial growth factor in dogs with soft tissue sarcomas.

    PubMed

    de Queiroz, G Fernandes; Dagli, M Lúcia Zaidan; Meira, S Aparecida; Matera, J Maria

    2013-09-01

    This work aimed to evaluate serum vascular endothelial growth factor (VEGF) in 25 dogs with soft tissue sarcoma, and in 30 healthy dogs. Blood was collected once time from the control animals and three times, in the same way, from animals with sarcoma. Blood count was performed in the blood collected, and serum VEGF was measured by enzyme-linked immunosorbent assay quantitative method. Serum VEGF in control animals was similar to patients with soft tissue sarcoma. There was a reduction in serum VEGF after the sarcoma resection. There was positive correlation between serum VEGF and neutrophil counts, and negative between VEGF and hemoglobin content in animals with sarcoma. Animals with hemangiopericytoma showed higher serum VEGF levels compared to the patients with malignant peripheral nerve sheath. Circulating blood cells can contribute to elevate VEGF serum concentrations in dogs with soft tissue sarcomas and a possible role of VEGF in the angiogenesis of these tumors. © 2012 John Wiley & Sons Ltd.

  8. Unstable patients with retroperitoneal vascular trauma: an endovascular approach.

    PubMed

    Boufi, Mourad; Bordon, Sébastien; Dona, Bianca; Hartung, Olivier; Sarran, Anthony; Nadeau, Sébastien; Maurin, Charlotte; Alimi, Yves S

    2011-04-01

    In hemodynamically unstable patients, the management of retroperitoneal vascular trauma is both difficult and challenging. Endovascular techniques have become an alternative to surgery in several trauma centers. Between 2004 and 2006, 16 patients (nine men, mean age: 46 years, range: 19-79 years) with retroperitoneal vascular trauma and hemodynamic instability were treated using an endovascular approach. The mean injury severity score was 30.7 ± 13.1. Mean systolic blood pressure and the shock index were 74 mm Hg and 1.9, respectively. Vasopressor drugs were required in 68.7% of cases (n = 11). Injuries were attributable to road traffic accidents (n = 15) and falls (n = 1). The hemorrhage sites included the internal iliac artery or its branches (n = 12) with bilateral injury in one case, renal artery (n = 2), abdominal aorta (n = 1), and lumbar artery (n = 1). In all, 14 coil embolizations and three stent-grafts were implanted. The technical success rate was 75%, as early re-embolization was necessary in one case and three patients died during the perioperative period. Six patients died during the period of hospitalization (37.5%). No surgical conversion or major morbidity was reported. In comparison with particulates, coil ± stent-graft may provide similar efficacy with regard to survival, and thus may be a valuable solution when particulate embolization is not available or feasible. Copyright © 2011 Annals of Vascular Surgery Inc. Published by Elsevier Inc. All rights reserved.

  9. Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma

    ClinicalTrials.gov

    2016-06-09

    Adult Alveolar Soft Part Sarcoma; Adult Angiosarcoma; Adult Desmoplastic Small Round Cell Tumor; Adult Epithelioid Hemangioendothelioma; Adult Epithelioid Sarcoma; Adult Extraskeletal Myxoid Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Mesenchymoma; Adult Malignant Peripheral Nerve Sheath Tumor; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Adult Unclassified Pleomorphic Sarcoma; Chondrosarcoma; Clear Cell Sarcoma of the Kidney; Conjunctival Kaposi Sarcoma; Dermatofibrosarcoma Protuberans; Gastrointestinal Stromal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Osteosarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Adult Unclassified Pleomorphic Sarcoma of Bone; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Kaposi Sarcoma; Recurrent Osteosarcoma; Recurrent Uterine Corpus Sarcoma; Small Intestine Leiomyosarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma; Unclassified Pleomorphic Sarcoma of Bone

  10. Copy Number Alterations and Methylation in Ewing's Sarcoma

    PubMed Central

    Jahromi, Mona S.; Jones, Kevin B.; Schiffman, Joshua D.

    2011-01-01

    Ewing's sarcoma is the second most common bone malignancy affecting children and young adults. The prognosis is especially poor in metastatic or relapsed disease. The cell of origin remains elusive, but the EWS-FLI1 fusion oncoprotein is present in the majority of cases. The understanding of the molecular basis of Ewing's sarcoma continues to progress slowly. EWS-FLI1 affects gene expression, but other factors must also be at work such as mutations, gene copy number alterations, and promoter methylation. This paper explores in depth two molecular aspects of Ewing's sarcoma: copy number alterations (CNAs) and methylation. While CNAs consistently have been reported in Ewing's sarcoma, their clinical significance has been variable, most likely due to small sample size and tumor heterogeneity. Methylation is thought to be important in oncogenesis and balanced karyotype cancers such as Ewing's, yet it has received only minimal attention in prior studies. Future CNA and methylation studies will help to understand the molecular basis of this disease. PMID:21437220

  11. Bone Sarcoma Pathology: Diagnostic Approach for Optimal Therapy.

    PubMed

    Rosenberg, Andrew E

    2017-01-01

    The pathologic interpretation of malignant bone tumors is one of the more challenging areas in surgical pathology. This is based on the reality that primary bone sarcomas are uncommon, demonstrate significant morphologic heterogeneity, and have a broad spectrum of biology. Accordingly, it is difficult for pathologists to acquire the necessary experience to confidently and accurately diagnose bone sarcomas. The task is further complicated by the fact that it requires the integration of clinical and radiologic information into the diagnostic process. Lastly, molecular aberrations in sarcomas are being newly discovered and their identification is often critical to make specific diagnoses. The pathologist's role in guiding optimal treatment in biopsy specimens is to make an accurate diagnosis and provide the grade and molecular aberrations when appropriate. The pathology report of resected tumors must confirm this information and assess the surgical resection margins and the percentage of necrosis if the sarcoma has been treated with neoadjuvant systemic therapy.

  12. Doxorubicin With Upfront Dexrazoxane Plus Olaratumab for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma

    ClinicalTrials.gov

    2018-02-08

    Sarcoma, Soft Tissue; Soft Tissue Sarcoma; Undifferentiated Pleomorphic Sarcoma; Leiomyosarcoma; Liposarcoma; Synovial Sarcoma; Myxofibrosarcoma; Angiosarcoma; Fibrosarcoma; Malignant Peripheral Nerve Sheath Tumor; Epithelioid Sarcoma

  13. NKX2.2 is a useful immunohistochemical marker for Ewing sarcoma.

    PubMed

    Yoshida, Akihiko; Sekine, Shigeki; Tsuta, Koji; Fukayama, Masashi; Furuta, Koh; Tsuda, Hitoshi

    2012-07-01

    Ewing sarcoma is a high-grade round cell sarcoma that affects bones and soft tissues in children and young adults. Its diagnosis can be challenging, and the differential diagnoses include a wide variety of small round cell tumors. CD99 and FLI-1 are the currently accepted immunohistochemical markers for Ewing sarcoma, but their accuracy has been controversial. NKX2.2 is a homeodomain-containing transcription factor that plays a critical role in neuroendocrine/glial differentiation. The NKX2.2 gene was recently identified as a target of EWS-FLI-1, the fusion protein specific to Ewing sarcoma, and was shown to be differentially upregulated in Ewing sarcoma on the basis of array-based gene expression analysis. However, the immunohistochemical diagnostic potential of this marker has not been tested. We immunostained representative sections of 30 genetically confirmed Ewing sarcomas and 130 non-Ewing small round cell tumors by using an antibody to NKX2.2. Nuclear staining in at least 5% of the cells was deemed positive. Twenty-eight (93%) of the 30 Ewing sarcomas were positive for NKX2.2. The staining was diffuse (>50%) in all the positive cases and was moderate or strong in intensity for most cases (25 of 28). NKX2.2 was also positive in 14 non-Ewing tumors, including all the olfactory neuroblastomas and a minor subset of small cell carcinomas, synovial sarcomas, mesenchymal chondrosarcomas, and malignant melanomas. All the other non-Ewing tumors tested were negative for this marker. NKX2.2 is a valuable marker for Ewing sarcoma, with a sensitivity of 93% and a specificity of 89%, and aids in the differential diagnosis of small round cell tumors.

  14. Cutaneous myeloid sarcoma associated with chronic myeloid leukemia*

    PubMed Central

    Vasconcelos, Erica Rodrigues de Araujo; Bauk, Alexander Richard; Rochael, Mayra Carrijo

    2017-01-01

    Myeloid sarcoma is an extramedullary tumor of malignant myeloid cells often associated with acute myeloid leukemia, chronic myeloproliferative disorders and myelodysplastic syndromes. The skin is one of the most commonly affected sites. We report a rare case of cutaneous myeloid sarcoma associated with chronic myeloid leukemia. PMID:29267445

  15. Leukosis/Sarcoma Group

    USDA-ARS?s Scientific Manuscript database

    The leukosis/sarcoma (L/S) group of diseases designates a variety of transmissible benign and malignant neoplasms of chickens caused by members that belong to the family Retroviridae. Because the expansion of the literature on this disease, it is no longer feasible to cite all relevant publications ...

  16. Ewing’s sarcoma precursors are highly enriched in embryonic osteochondrogenic progenitors

    PubMed Central

    Tanaka, Miwa; Yamazaki, Yukari; Kanno, Yohei; Igarashi, Katsuhide; Aisaki, Ken-ichi; Kanno, Jun; Nakamura, Takuro

    2014-01-01

    Ewing’s sarcoma is a highly malignant bone tumor found in children and adolescents, and the origin of this malignancy is not well understood. Here, we introduced a Ewing’s sarcoma–associated genetic fusion of the genes encoding the RNA-binding protein EWS and the transcription factor ETS (EWS-ETS) into a fraction of cells enriched for osteochondrogenic progenitors derived from the embryonic superficial zone (eSZ) of long bones collected from late gestational murine embryos. EWS-ETS fusions efficiently induced Ewing’s sarcoma–like small round cell sarcoma formation by these cells. Analysis of the eSZ revealed a fraction of a precursor cells that express growth/differentiation factor 5 (Gdf5), the transcription factor Erg, and parathyroid hormone-like hormone (Pthlh), and selection of the Pthlh-positive fraction alone further enhanced EWS-ETS–dependent tumor induction. Genes downstream of the EWS-ETS fusion protein were quite transcriptionally active in eSZ cells, especially in regions in which the chromatin structure of the ETS-responsive locus was open. Inhibition of β-catenin, poly (ADP-ribose) polymerase 1 (PARP1), or enhancer of zeste homolog 2 (EZH2) suppressed cell growth in a murine model of Ewing’s sarcoma, suggesting the utility of the current system as a preclinical model. These results indicate that eSZ cells are highly enriched in precursors to Ewing’s sarcoma and provide clues to the histogenesis of Ewing’s sarcoma in bone. PMID:24911143

  17. Cyclin D1 and Ewing's sarcoma/PNET: A microarray analysis.

    PubMed

    Fagone, Paolo; Nicoletti, Ferdinando; Salvatorelli, Lucia; Musumeci, Giuseppe; Magro, Gaetano

    2015-10-01

    Recent immunohistochemical analyses have showed that cyclin D1 is expressed in soft tissue Ewing's sarcoma/peripheral neuroectodermal tumor (PNET) of childhood and adolescents, while it is undetectable in both embryonal and alveolar rhabdomyosarcoma. In the present paper, microarray analysis provided evidence of a significant upregulation of cyclin D1 in Ewing's sarcoma as compared to normal tissues. In addition, we confirmed our previous findings of a significant over-expression of cyclin D1 in Ewing sarcoma as compared to rhabdomyosarcoma. Bioinformatic analysis also allowed to identify some other genes, strongly correlated to cyclin D1, which, although not previously studied in pediatric tumors, could represent novel markers for the diagnosis and prognosis of Ewing's sarcoma/PNET. The data herein provided support not only the use of cyclin D1 as a diagnostic marker of Ewing sarcoma/PNET but also the possibility of using drugs targeting cyclin D1 as potential therapeutic strategies. Copyright © 2015 Elsevier GmbH. All rights reserved.

  18. PI3K inhibition enhances doxorubicin-induced apoptosis in sarcoma cells.

    PubMed

    Marklein, Diana; Graab, Ulrike; Naumann, Ivonne; Yan, Tiandong; Ridzewski, Rosalie; Nitzki, Frauke; Rosenberger, Albert; Dittmann, Kai; Wienands, Jürgen; Wojnowski, Leszek; Fulda, Simone; Hahn, Heidi

    2012-01-01

    We searched for a drug capable of sensitization of sarcoma cells to doxorubicin (DOX). We report that the dual PI3K/mTOR inhibitor PI103 enhances the efficacy of DOX in several sarcoma cell lines and interacts with DOX in the induction of apoptosis. PI103 decreased the expression of MDR1 and MRP1, which resulted in DOX accumulation. However, the enhancement of DOX-induced apoptosis was unrelated to DOX accumulation. Neither did it involve inhibition of mTOR. Instead, the combination treatment of DOX plus PI103 activated Bax, the mitochondrial apoptosis pathway, and caspase 3. Caspase 3 activation was also observed in xenografts of sarcoma cells in nude mice upon combination of DOX with the specific PI3K inhibitor GDC-0941. Although the increase in apoptosis did not further impact on tumor growth when compared to the efficient growth inhibition by GDC-0941 alone, these findings suggest that inhibition of PI3K may improve DOX-induced proapoptotic effects in sarcoma. Taken together with similar recent studies of neuroblastoma- and glioblastoma-derived cells, PI3K inhibition seems to be a more general option to sensitize tumor cells to anthracyclines.

  19. PI3K Inhibition Enhances Doxorubicin-Induced Apoptosis in Sarcoma Cells

    PubMed Central

    Marklein, Diana; Graab, Ulrike; Naumann, Ivonne; Yan, Tiandong; Ridzewski, Rosalie; Nitzki, Frauke; Rosenberger, Albert; Dittmann, Kai; Wienands, Jürgen; Wojnowski, Leszek; Fulda, Simone; Hahn, Heidi

    2012-01-01

    We searched for a drug capable of sensitization of sarcoma cells to doxorubicin (DOX). We report that the dual PI3K/mTOR inhibitor PI103 enhances the efficacy of DOX in several sarcoma cell lines and interacts with DOX in the induction of apoptosis. PI103 decreased the expression of MDR1 and MRP1, which resulted in DOX accumulation. However, the enhancement of DOX-induced apoptosis was unrelated to DOX accumulation. Neither did it involve inhibition of mTOR. Instead, the combination treatment of DOX plus PI103 activated Bax, the mitochondrial apoptosis pathway, and caspase 3. Caspase 3 activation was also observed in xenografts of sarcoma cells in nude mice upon combination of DOX with the specific PI3K inhibitor GDC-0941. Although the increase in apoptosis did not further impact on tumor growth when compared to the efficient growth inhibition by GDC-0941 alone, these findings suggest that inhibition of PI3K may improve DOX-induced proapoptotic effects in sarcoma. Taken together with similar recent studies of neuroblastoma- and glioblastoma-derived cells, PI3K inhibition seems to be a more general option to sensitize tumor cells to anthracyclines. PMID:23300809

  20. RNAi phenotype profiling of kinases identifies potential therapeutic targets in Ewing's sarcoma.

    PubMed

    Arora, Shilpi; Gonzales, Irma M; Hagelstrom, R Tanner; Beaudry, Christian; Choudhary, Ashish; Sima, Chao; Tibes, Raoul; Mousses, Spyro; Azorsa, David O

    2010-08-18

    Ewing's sarcomas are aggressive musculoskeletal tumors occurring most frequently in the long and flat bones as a solitary lesion mostly during the teen-age years of life. With current treatments, significant number of patients relapse and survival is poor for those with metastatic disease. As part of novel target discovery in Ewing's sarcoma, we applied RNAi mediated phenotypic profiling to identify kinase targets involved in growth and survival of Ewing's sarcoma cells. Four Ewing's sarcoma cell lines TC-32, TC-71, SK-ES-1 and RD-ES were tested in high throughput-RNAi screens using a siRNA library targeting 572 kinases. Knockdown of 25 siRNAs reduced the growth of all four Ewing's sarcoma cell lines in replicate screens. Of these, 16 siRNA were specific and reduced proliferation of Ewing's sarcoma cells as compared to normal fibroblasts. Secondary validation and preliminary mechanistic studies highlighted the kinases STK10 and TNK2 as having important roles in growth and survival of Ewing's sarcoma cells. Furthermore, knockdown of STK10 and TNK2 by siRNA showed increased apoptosis. In summary, RNAi-based phenotypic profiling proved to be a powerful gene target discovery strategy, leading to successful identification and validation of STK10 and TNK2 as two novel potential therapeutic targets for Ewing's sarcoma.

  1. [Nonsurgical therapy of abdominal and retroperitoneal abscesses].

    PubMed

    Reuss, J A; Seitz, K

    1987-06-01

    From 1976 to March 1987 intraabdominal or retroperitoneal abscesses were confirmed by ultrasound in 40 patients. 13 patients had surgical drainage with zero-mortality. 3 of 27 patients were treated with antibiotics (only systemically). 24 patients were treated by percutaneous catheter drainage or needle aspiration. Elective cholecystectomy was performed later in 3 patients. Nonsurgical treatment was successful in 22/27 cases. 3 patients died despite percutaneous drainage. Failure of percutaneous drainage required surgical intervention in 3 patients. The considerably poorer primary condition of the patients receiving nonsurgical treatment allows no comparison with the surgical group. Advantages of percutaneous drainage and needle aspiration are a high success rate and low mortality. These techniques can be used even in critically ill persons.

  2. A review of soft-tissue sarcomas: translation of biological advances into treatment measures

    PubMed Central

    Mann, Michael J; Tolani, Bhairavi

    2018-01-01

    Soft-tissue sarcomas are rare malignant tumors arising from connective tissues and have an overall incidence of about five per 100,000 per year. While this diverse family of malignancies comprises over 100 histological subtypes and many molecular aberrations are prevalent within specific sarcomas, very few are therapeutically targeted. Instead of utilizing molecular signatures, first-line sarcoma treatment options are still limited to traditional surgery and chemotherapy, and many of the latter remain largely ineffective and are plagued by disease resistance. Currently, the mechanism of sarcoma oncogenesis remains largely unknown, thus necessitating a better understanding of pathogenesis. Although substantial progress has not occurred with molecularly targeted therapies over the past 30 years, increased knowledge about sarcoma biology could lead to new and more effective treatment strategies to move the field forward. Here, we discuss biological advances in the core molecular determinants in some of the most common soft-tissue sarcomas – liposarcoma, angiosarcoma, leiomyosarcoma, rhabdomyosarcoma, Ewing’s sarcoma, and synovial sarcoma – with an emphasis on emerging genomic and molecular pathway targets and immunotherapeutic treatment strategies to combat this confounding disease. PMID:29785138

  3. Spontaneous Retroperitoneal Hematoma Simulating Ruptured Infrarenal Aortic Aneurysm in a Patient with End-Stage Renal Disease

    PubMed Central

    Li, JYY; Chan, YC; Qing, KX; Cheng, SW

    2014-01-01

    We reported a case of spontaneous retroperitoneal hematoma (SRH) simulating a ruptured infrarenal aortic aneurysm. A 72-year-old man with a history of infrarenal aortic aneurysm and end-stage renal disease on hemodialysis presented with malaise and nonspecific central abdominal pain and left loin discomfort. An emergency computed tomography scan showed a large retroperitoneal hematoma and clinical suspicion of ruptured infrarenal aortic aneurysm. However, the hematoma was discontinuous with the aneurysm sac and raised the clinical suspicion on dual pathology. The SRH was treated conservatively with transfusion of blood products, and the aneurysm was treated with nonemergency endovascular repair electively. This case demonstrates the importance of recognizing different clinical and radiological characteristics and be aware of dual pathology. PMID:28031651

  4. Cabozantinib-s-malate in Treating Patients With Relapsed Osteosarcoma or Ewing Sarcoma

    ClinicalTrials.gov

    2018-05-23

    Metastatic Ewing Sarcoma; Metastatic Osteosarcoma; Recurrent Ewing Sarcoma; Recurrent Osteosarcoma; Stage III Osteosarcoma AJCC v7; Stage IV Osteosarcoma AJCC v7; Stage IVA Osteosarcoma AJCC v7; Stage IVB Osteosarcoma AJCC v7; Unresectable Ewing Sarcoma; Unresectable Osteosarcoma

  5. A Unique Case of Classic Kaposi's sarcoma restricted to the toes.

    PubMed

    Renteria, Anne S; Marshall, Vickie A; Sun, Yanyu; Chockalingam, Porselvi; Cooper, Jay S; Huang, Yiwu; Whitby, Denise

    2013-01-01

    Kaposi's sarcoma associated-herpesvirus causes all forms of Kaposi's sarcoma, and six major subtypes have been described based on the amino acid sequences of the open reading frame K1. A 71-year-old man from China, HIV negative, presented with nodules on the dorsal aspect of his toes. Biopsy confirmed the diagnosis of Kaposi's sarcoma and virology studies of his blood and saliva confirmed the presence of Kaposi's sarcoma associated-herpesvirus infection. Viral genotyping was consistent with subtype C3. Intervention has been deferred as our patient has remained clinically asymptomatic and without evident growth of his lesions over a 2-year follow up. We herein report the first known case of Kaposi's sarcoma restricted to the toes caused by the viral subtype C3 in an HIV-negative patient from Harbin, China.

  6. Primary Fibro Sarcoma of the Heart

    PubMed Central

    Kabashi, Serbeze; Hoxha, Naim; Gashi, Shkelzen; Ahmegjekaj, Ilir; Bejta, Ilir; Sadiku, Muharrem; Ymeri, Halit; Kabashi, Antigona; Bicaj, Xhavit; Mucaj, Sefedin

    2013-01-01

    Primary malignant heart tumors represent rare entities where fibro sarcoma represents about 3% of all. Introducing the patient: A 15 years old patient with cardiac insufficiency (heart failure) symptoms, such as weakness, cyanosis, palpitations and breathing difficulties; enlargement of upper mediastinum and pleural effusion. Through echocardiography a pericardial effusion and intracavitary thrombus in atrium was diagnosed. With computed tomography is diagnosed a tumoral mass in right atrium which is also spread in the right ventricle of the heart. Tumor is completely removed; pat histology result showed primary fibro sarcoma of the heart. At that time no metastasis was found. Conclusion. Primary malignant heart tumors may manifest like cardiac insufficiency or like systemic diseases. Fibrosarcomas are rare and have bad prognosis. On average patients can live around six months after initial symptoms appeared and diagnosis of the tumor was done. In the case of cardiac insufficiency with differential diagnosis we should also think of heart tumors, which could certainly be proved for or eliminated by echocardiography. PMID:24167396

  7. Primary fibro sarcoma of the heart.

    PubMed

    Kabashi, Serbeze; Hoxha, Naim; Gashi, Shkelzen; Ahmegjekaj, Ilir; Bejta, Ilir; Sadiku, Muharrem; Ymeri, Halit; Kabashi, Antigona; Bicaj, Xhavit; Mucaj, Sefedin

    2013-01-01

    Primary malignant heart tumors represent rare entities where fibro sarcoma represents about 3% of all. Introducing the patient: A 15 years old patient with cardiac insufficiency (heart failure) symptoms, such as weakness, cyanosis, palpitations and breathing difficulties; enlargement of upper mediastinum and pleural effusion. Through echocardiography a pericardial effusion and intracavitary thrombus in atrium was diagnosed. With computed tomography is diagnosed a tumoral mass in right atrium which is also spread in the right ventricle of the heart. Tumor is completely removed; pat histology result showed primary fibro sarcoma of the heart. At that time no metastasis was found. Conclusion. Primary malignant heart tumors may manifest like cardiac insufficiency or like systemic diseases. Fibrosarcomas are rare and have bad prognosis. On average patients can live around six months after initial symptoms appeared and diagnosis of the tumor was done. In the case of cardiac insufficiency with differential diagnosis we should also think of heart tumors, which could certainly be proved for or eliminated by echocardiography.

  8. Successfully treated case of epithelioid sarcoma of the vulva.

    PubMed

    Chiyoda, Tatsuyuki; Ishikawa, Mitsuya; Nakamura, Masaru; Ogawa, Mariko; Takamatsu, Kiyoshi

    2011-12-01

    Epithelioid sarcoma of the vulva is an extremely rare and aggressive tumor. This tumor most commonly occurs on the labia majora in women of reproductive age. The molecular pathogenesis remains largely unknown. Only 20 cases of vulvar epithelioid sarcoma have been reported to date and more than half have had poor outcomes. We report a successfully treated case of vulvar epithelioid sarcoma in a 33-year-old woman. We performed a radical vulvectomy with flap reconstruction. Three years after surgery, the patient remains well, showing no evidence of recurrence. Early tissue diagnosis of vulvar epithelioid sarcoma is essential because this tumor can be clinically misdiagnosed as a benign lesion. Gynecologists should be aware of this rare tumor variant and carefully plan the treatment. © 2011 The Authors. Journal of Obstetrics and Gynaecology Research © 2011 Japan Society of Obstetrics and Gynecology.

  9. Epithelioid sarcoma of the spine: case report and literature review.

    PubMed

    Chamadoira, Clara; Pereira, Paulo; Silva, Pedro S; Castro, Ligia; Vaz, Rui

    2014-01-01

    Epithelioid sarcomas are rare mesenchymal neoplasms mainly arising in the limbs of young adults. We report the case of a 24-year-old male presenting low back pain radiating to both lower limbs, constipation and urinary retention. The MRI scan showed an intraspinal lesion extending from L4 to S2. Surgery resulted in gross total removal of the extradural lesion and partial removal of the intradural component. The immunohistological study of the lesion was consistent with an epithelioid sarcoma. The patient was submitted to radiotherapy and chemotherapy, but a local recurrence of the lesion and dissemination along the neuraxis were observed 3 months after surgery. Despite treatment, the patient died 4 months after the surgical procedure due to multiorgan failure. Despite there being isolated reports of epithelioid sarcomas appearing in the spine, this is, to our knowledge, the first case with intradural extension. Copyright © 2013 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.

  10. Genetic susceptibility to bone and soft tissue sarcomas: a field synopsis and meta-analysis

    PubMed Central

    Benna, Clara; Simioni, Andrea; Pasquali, Sandro; De Boni, Davide; Rajendran, Senthilkumar; Spiro, Giovanna; Colombo, Chiara; Virgone, Calogero; DuBois, Steven G.; Gronchi, Alessandro; Rossi, Carlo Riccardo; Mocellin, Simone

    2018-01-01

    Background The genetic architecture of bone and soft tissue sarcomas susceptibility is yet to be elucidated. We aimed to comprehensively collect and meta-analyze the current knowledge on genetic susceptibility in these rare tumors. Methods We conducted a systematic review and meta-analysis of the evidence on the association between DNA variation and risk of developing sarcomas through searching PubMed, The Cochrane Library, Scopus and Web of Science databases. To evaluate result credibility, summary evidence was graded according to the Venice criteria and false positive report probability (FPRP) was calculated to further validate result noteworthiness. Integrative analysis of genetic and eQTL (expression quantitative trait locus) data was coupled with network and pathway analysis to explore the hypothesis that specific cell functions are involved in sarcoma predisposition. Results We retrieved 90 eligible studies comprising 47,796 subjects (cases: 14,358, 30%) and investigating 1,126 polymorphisms involving 320 distinct genes. Meta-analysis identified 55 single nucleotide polymorphisms (SNPs) significantly associated with disease risk with a high (N=9), moderate (N=38) and low (N=8) level of evidence, findings being classified as noteworthy basically only when the level of evidence was high. The estimated joint population attributable risk for three independent SNPs (rs11599754 of ZNF365/EGR2, rs231775 of CTLA4, and rs454006 of PRKCG) was 37.2%. We also identified 53 SNPs significantly associated with sarcoma risk based on single studies. Pathway analysis enabled us to propose that sarcoma predisposition might be linked especially to germline variation of genes whose products are involved in the function of the DNA repair machinery. Conclusions We built the first knowledgebase on the evidence linking DNA variation to sarcomas susceptibility, which can be used to generate mechanistic hypotheses and inform future studies in this field of oncology. PMID:29719630

  11. Spindle cell sarcoma of the vulva with myofibroblastic differentiation.

    PubMed

    Adeleye, Amanda J; Palmeri, Nicholas; Wang, Shih-Hsiu J; Liu-Jarin, Xiaolin; Wright, Jason D

    2015-04-01

    Primary vulvar sarcomas are rare lesions of the lower genital tract. We report the case of a patient with a spindle cell sarcoma of the vulva. A 44-year-old woman presented with a painless vulvar mass. Vulvar biopsy demonstrated a spindle cell sarcoma with myofibroblastic differentiation. Pretreatment evaluation revealed no evidence of metastatic disease, and magnetic resonance imaging found no local masses. The patient underwent right radical vulvectomy with negative margins and tolerated the procedure well. Women undergoing gynecologic care should have routine evaluation of the vulva to detect these rare neoplasms.

  12. Clinical effect of molecular methods in sarcoma diagnosis (GENSARC): a prospective, multicentre, observational study.

    PubMed

    Italiano, Antoine; Di Mauro, Ilaria; Rapp, Jocelyn; Pierron, Gaëlle; Auger, Nathalie; Alberti, Laurent; Chibon, Frédéric; Escande, Fabienne; Voegeli, Anne-Claire; Ghnassia, Jean-Pierre; Keslair, Frédérique; Laé, Marick; Ranchère-Vince, Dominique; Terrier, Philippe; Baffert, Sandrine; Coindre, Jean-Michel; Pedeutour, Florence

    2016-04-01

    Advances in molecular genetics of sarcoma have enabled the identification of type-specific aberrations. We aimed to assess the clinical effect of systematic implementation of molecular assays to improve sarcoma misdiagnosis. In this multicentre, observational study, we recruited patients from 32 centres of the French Sarcoma Group/Reference Network in Pathology of Sarcomas. Eligibility criteria included: biopsy or surgical resection; suspicion of: dermatofibrosarcoma protuberans (cohort 1), dedifferentiated liposarcoma (cohort 2), Ewing's sarcoma family of tumours (cohort 3), synovial sarcoma (cohort 4), alveolar rhabdomyosarcoma (cohort 5), and myxoid or round cell liposarcoma (cohort 6); review by one sarcoma-expert pathologist; availability of frozen material (except for cohort 1 of patients with dermatofibrosarcoma protuberans because anti-CD34 immunohistochemistry is performed on paraffin-embedded tissue); and patient information. For each case, the pathologist made one primary diagnosis followed by up to two differential diagnoses, based on histological characteristics only. Each diagnosis was classified as certain, probable, or possible. For each case to determine the molecular classification, we did fluorescence in-situ hybridisation on paraffin-embedded samples. We also did comparative genomic hybridisation and quantitative PCR (cohort 2) or reverse transcriptase PCR (cohorts 3-6) on frozen and paraffin-embedded samples. We made a final diagnosis based on the molecular results. The clinical effect of diagnosis correction was assessed by a board of experts. Between June 22, 2009, and Oct 30, 2012, 395 patients were enrolled in the study, of which 384 were eligible for inclusion. The diagnosis was eventually modified by molecular genetics for 53 patients: eight (16%) of 50 patients with dermatofibrosarcoma (cohort 1), seven (23%) of 30 patients with dedifferentiated liposarcoma (cohort 2), 13 (12%) of 112 with Ewing's sarcoma family of tumours (cohort 3), 16

  13. Clinical features and outcomes in patients with extraskeletal Ewing sarcoma

    PubMed Central

    Applebaum, Mark A.; Worch, Jennifer; Matthay, Katherine K.; Goldsby, Robert; Neuhaus, John; West, Daniel C.; DuBois, Steven G.

    2010-01-01

    Background Ewing sarcoma can arise in either bone or soft tissue locations. We sought to investigate if patient characteristics, treatment strategies, and outcomes differ between skeletal Ewing sarcoma and extraskeletal Ewing sarcoma (EES). Procedure Patients < 40 years of age with Ewing sarcoma or peripheral primitive neuroectodermal tumor (PNET) reported to the US SEER database from 1973 to 2007 were evaluated based on skeletal (n=1519) vs. extraskeletal (n=683) site of origin. Patient characteristics were compared using Fisher exact tests. Overall survival was estimated by Kaplan-Meier methods and compared using log-rank tests and Cox models. Results Patients with EES had a higher mean age (19.5 vs. 16.3 years; p < 0.001) and were less likely to be male (53.4% vs. 63.3%; p < 0.001) or white (84.8% vs. 92.5%; p < 0.001) compared to patients with skeletal tumors. Extraskeletal tumors were more likely to arise in axial locations (72.9% vs. 54.2%; p = 0.001), though less likely to arise specifically in the pelvis (19.8% vs. 26.6%; p < 0.001). Metastatic status or tumor size did not differ by group. Five-year overall survival was superior for localized EES compared to localized skeletal tumors (69.7% vs. 62.6%; p = 0.02). The hazard ratio for death in patients with localized skeletal tumors compared to localized EES was 2.36 (95% CI 1.61-3.44) beyond 24 months from initial diagnosis. Conclusions Patient characteristics and outcomes differ among patients with EES compared to patients with skeletal Ewing sarcoma. These findings may have important implications for patient care. PMID:21692057

  14. Childhood Soft Tissue Sarcoma Treatment (PDQ®)—Patient Version

    Cancer.gov

    Childhood soft tissue sarcoma treatment options include surgery, radiation therapy, chemotherapy, observation, targeted therapy, immunotherapy and other medications.  Learn more about the diagnosis and treatment of the many types of childhood soft tissue sarcoma in this expert-reviewed summary.

  15. Retroperitoneal margin involvement by adenocarcinoma of the caecum and ascending colon: what does it mean?

    PubMed

    Scott, N; Jamali, A; Verbeke, C; Ambrose, N S; Botterill, I D; Jayne, D G

    2008-03-01

    Circumferential margin involvement (CRM) is a powerful predictor of local recurrence, distant metastasis and patient survival in rectal cancer. In this study, we aimed to determine the frequency of retroperitoneal margin involvement in right colon cancer and describe its relationship to tumour stage and outcome of surgical treatment. Two hundred and twenty-eight consecutive resections for adenocarcinoma of the ascending colon and caecum were identified between 1998 and 2006. Tumour involvement of the posterior retroperitoneal surgical resection margin (RSRM) was recorded and correlated with tumour stage, grade and clinical outcome. RSRM positive patients were compared with CRM positive rectal tumours resected in the same surgical unit. Nineteen of 228 right hemicolectomies (8.4%) showed tumour involvement of the RSRM (defined as < or = 1 mm). Approximately half of the RSRM positive patients underwent palliative resections because of synchronous distant metastases. Out of nine 'potentially curative' resections where the RSRM was involved, five patients subsequently developed metastatic recurrence and two isolated local recurrence. RSRM positivity was associated with advanced tumour stage and more extensive extramural spread than CRM positive rectal cancers. Retroperitoneal surgical resection margin involvement by caecal and ascending colon carcinoma is a marker of advanced tumour stage and associated with a high incidence of synchronous and metachronous distant metastasis. More aggressive surgery to obtain a clear margin or postoperative radiotherapy to the tumour bed is likely to benefit only a minority of patients.

  16. Cixutumumab and Doxorubicin Hydrochloride in Treating Patients With Unresectable, Locally Advanced, or Metastatic Soft Tissue Sarcoma

    ClinicalTrials.gov

    2016-05-16

    Adult Angiosarcoma; Adult Desmoplastic Small Round Cell Tumor; Adult Epithelioid Sarcoma; Adult Extraskeletal Myxoid Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Mesenchymoma; Adult Malignant Peripheral Nerve Sheath Tumor; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Adult Undifferentiated High Grade Pleomorphic Sarcoma of Bone; Childhood Angiosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Mesenchymoma; Childhood Malignant Peripheral Nerve Sheath Tumor; Childhood Pleomorphic Rhabdomyosarcoma; Childhood Rhabdomyosarcoma With Mixed Embryonal and Alveolar Features; Childhood Synovial Sarcoma; Dermatofibrosarcoma Protuberans; Malignant Adult Hemangiopericytoma; Malignant Childhood Hemangiopericytoma; Metastatic Childhood Soft Tissue Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Untreated Childhood Rhabdomyosarcoma

  17. Bioenergetic properties of human sarcoma cells help define sensitivity to metabolic inhibitors

    PubMed Central

    Issaq, Sameer H; Teicher, Beverly A; Monks, Anne

    2014-01-01

    Sarcomas represent a diverse group of malignancies with distinct molecular and pathological features. A better understanding of the alterations associated with specific sarcoma subtypes is critically important to improve sarcoma treatment. Renewed interest in the metabolic properties of cancer cells has led to an exploration of targeting metabolic dependencies as a therapeutic strategy. In this study, we have characterized key bioenergetic properties of human sarcoma cells in order to identify metabolic vulnerabilities between sarcoma subtypes. We have also investigated the effects of compounds that inhibit glycolysis or mitochondrial respiration, either alone or in combination, and examined relationships between bioenergetic parameters and sensitivity to metabolic inhibitors. Using 2-deoxy-D-glucose (2-DG), a competitive inhibitor of glycolysis, oligomycin, an inhibitor of mitochondrial ATP synthase, and metformin, a widely used anti-diabetes drug and inhibitor of complex I of the mitochondrial respiratory chain, we evaluated the effects of metabolic inhibition on sarcoma cell growth and bioenergetic function. Inhibition of glycolysis by 2-DG effectively reduced the viability of alveolar rhabdomyosarcoma cells vs. embryonal rhabdomyosarcoma, osteosarcoma, and normal cells. Interestingly, inhibitors of mitochondrial respiration did not significantly affect viability, but were able to increase sensitivity of sarcomas to inhibition of glycolysis. Additionally, inhibition of glycolysis significantly reduced intracellular ATP levels, and sensitivity to 2-DG-induced growth inhibition was related to respiratory rates and glycolytic dependency. Our findings demonstrate novel relationships between sarcoma bioenergetics and sensitivity to metabolic inhibitors, and suggest that inhibition of metabolic pathways in sarcomas should be further investigated as a potential therapeutic strategy. PMID:24553119

  18. Cytogenetically confirmed primary Ewing's sarcoma of the pancreas.

    PubMed

    Golhar, Ankush; Ray, Samrat; Haugk, Beate; Singhvi, Suresh Kumar

    2017-05-04

    Ewing's sarcoma is a highly aggressive malignant tumour most commonly affecting long bones in children and adolescents. It is part of the Ewing's sarcoma family of tumours (ESFTs) that also include peripheral primitive neuroectodermal tumour and Askin's tumours. ESFTs share common cytogenetic aberrations, antigenic profiles and proto-oncogene expression with an overall similar clinical course. In 99% of ESFTs, genetic translocation with molecular fusion involves the EWSR1 gene on 22q12. Approximately 30% of ESFTs are extraosseous, most commonly occurring in the soft tissues of extremities, pelvis, retroperitoneum and chest wall. Primary presentation in solid organs is very rare but has been described in multiple sites including the pancreas. Accurate diagnosis of a Ewing's sarcoma in a solid organ is critical in facilitating correct treatment. We report the case of a 17-year-old girl with cytogenetically confirmed primary pancreatic Ewing's sarcoma and provide a brief review of the published literature. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  19. Safety, pharmacokinetics, and preliminary activity of the anti-IGF-1R antibody figitumumab (CP-751,871) in patients with sarcoma and Ewing's sarcoma: a phase 1 expansion cohort study.

    PubMed

    Olmos, David; Postel-Vinay, Sophie; Molife, L Rhoda; Okuno, Scott H; Schuetze, Scott M; Paccagnella, M Luisa; Batzel, Gretchen N; Yin, Donghua; Pritchard-Jones, Kathryn; Judson, Ian; Worden, Francis P; Gualberto, Antonio; Scurr, Michelle; de Bono, Johann S; Haluska, Paul

    2010-02-01

    Figitumumab is a fully human IgG2 monoclonal antibody targeting the insulin-like growth-factor-1 receptor (IGF-1R). Preclinical data suggest a dependence on insulin-like growth-factor signalling for sarcoma subtypes, including Ewing's sarcoma, and early reports show antitumour activity of IGF-1R-targeting drugs in these diseases. Between January, 2006, and August, 2008, patients with refractory, advanced sarcomas received figitumumab (20 mg/kg) in two single-stage expansion cohorts within a solid-tumour phase 1 trial. The first cohort (n=15) included patients with multiple sarcoma subtypes, age 18 years or older, and the second cohort (n=14) consisted of patients with refractory Ewing's sarcoma, age 9 years or older. The primary endpoint was to assess the safety and tolerability of figitumumab. Secondary endpoints included pharmacokinetic profiling and preliminary antitumour activity (best response by Response Evaluation Criteria in Solid Tumours [RECIST]) in evaluable patients who received at least one dose of medication. This study is registered with ClinicalTrials.gov, number NCT00474760. 29 patients, 16 of whom had Ewing's sarcoma, were enrolled and received a total of 177 cycles of treatment (median 2, mean 6.1, range 1-24). Grade 3 deep venous thrombosis, grade 3 back pain, and grade 3 vomiting were each noted once in individual patients; one patient had grade 3 increases in aspartate aminotransferase and gammaglutamyltransferase concentrations. This patient also had grade 4 increases in alanine aminotransferase concentrations. The only other grade 4 adverse event was raised concentrations of uric acid, noted in one patient. Pharmacokinetics were comparable between patients with sarcoma and those with other solid tumours. 28 patients were assessed for response; two patients, both with Ewing's sarcoma, had objective responses (one complete response and one partial response) and eight patients had disease stabilisation (six with Ewing's sarcoma, one with

  20. Microarray-based DNA methylation study of Ewing’s sarcoma of the bone

    PubMed Central

    PARK, HYE-RIM; JUNG, WOON-WON; KIM, HYUN-SOOK; PARK, YONG-KOO

    2014-01-01

    Alterations in DNA methylation patterns are a hallmark of malignancy. However, the majority of epigenetic studies of Ewing’s sarcoma have focused on the analysis of only a few candidate genes. Comprehensive studies are thus lacking and are required. The aim of the present study was to identify novel methylation markers in Ewing’s sarcoma using microarray analysis. The current study reports the microarray-based DNA methylation study of 1,505 CpG sites of 807 cancer-related genes from 69 Ewing’s sarcoma samples. The Illumina GoldenGate Methylation Cancer Panel I microarray was used, and with the appropriate controls (n=14), a total of 92 hypermethylated genes were identified in the Ewing’s sarcoma samples. The majority of the hypermethylated genes were associated with cell adhesion, cell regulation, development and signal transduction. The overall methylation mean values were compared between patients who survived and those that did not. The overall methylation mean was significantly higher in the patients who did not survive (0.25±0.03) than in those who did (0.22±0.05) (P=0.0322). However, the overall methylation mean was not found to significantly correlate with age, gender or tumor location. GDF10, OSM, APC and HOXA11 were the most significant differentially-methylated genes, however, their methylation levels were not found to significantly correlate with the survival rate. The DNA methylation profile of Ewing’s sarcoma was characterized and 92 genes that were significantly hypermethylated were detected. A trend towards a more aggressive behavior was identified in the methylated group. The results of this study indicated that methylation may be significant in the development of Ewing’s sarcoma. PMID:25202378

  1. Nivolumab With or Without Ipilimumab in Treating Patients With Metastatic Sarcoma That Cannot Be Removed by Surgery

    ClinicalTrials.gov

    2018-06-20

    Dedifferentiated Liposarcoma; Gastrointestinal Stromal Tumor; Metastatic Liposarcoma; Metastatic Undifferentiated Pleomorphic Sarcoma; Pleomorphic Liposarcoma; Stage III Bone Sarcoma AJCC v7; Stage III Soft Tissue Sarcoma AJCC v7; Stage IV Bone Sarcoma AJCC v7; Stage IV Soft Tissue Sarcoma AJCC v7; Stage IVA Bone Sarcoma AJCC v7; Stage IVB Bone Sarcoma AJCC v7; Unresectable Liposarcoma

  2. Prognostic significance of serum lactate dehydrogenase levels in Ewing's sarcoma: A meta-analysis.

    PubMed

    Li, Suoyuan; Yang, Qing; Wang, Hongsheng; Wang, Zhuoying; Zuo, Dongqing; Cai, Zhengdong; Hua, Yingqi

    2016-12-01

    A number of studies have investigated the role of serum lactate dehydrogenase (LDH) levels in patients with Ewing's sarcoma, although these have yielded inconsistent and inconclusive results. Therefore, the present study aimed to systematically review the published studies and conduct a meta-analysis to assess its prognostic value more precisely. Cohort studies assessing the prognostic role of LDH levels in patients with Ewing's sarcoma were included. A pooled hazard ratio (HR) with 95% confidence intervals (CIs) of overall survival (OS) or 5-year disease-free survival (DFS) was used to assess the prognostic role of the levels of serum LDH. Nine studies published between 1980 and 2014, with a total of 1,412 patients with Ewing's sarcoma, were included. Six studies, with a total of 644 patients, used OS as the primary endpoint and four studies, with 795 patients, used 5-year DFS. Overall, the pooled HR evaluating high LDH levels was 2.90 (95% CI: 2.09-4.04) for OS and 2.40 (95% CI: 1.93-2.98) for 5-year DFS. This meta-analysis demonstrates that high levels of serum LDH are associated with lower OS and 5-year DFS rates in patients with Ewing's sarcoma. Therefore, serum LDH levels are an effective biomarker of Ewing's sarcoma prognosis.

  3. Functional adrenal cortex preservation: A good reason for posterior retroperitoneal endoscopic approach.

    PubMed

    Vidal, Óscar; Delgado-Oliver, Eduardo; Díaz Del Gobbo, Rafael; Hanzu, Felicia; Squarcia, Mattia; Martínez, Daniel; Fuster, David; Fondevila, Constantino

    2018-05-24

    Cortical-sparing adrenalectomy is a suitable treatment for hereditary and sporadic bilateral pheochromocytoma, in cases of low risk of malignancy, to reduce the possibility of adrenal insufficiency assuming the chance of local recurrence. The aim of the study is to analyze the functional results of partial adrenalectomy by retroperitoneal endoscopic approach in single-adrenal patients or patients requiring bilateral adrenalectomy. Prospective study between January 2015 and February 2016 including pheochromocytoma patients diagnosed with low risk of malignant mutations. All patients agreed to be included in the study. Experienced endocrine surgeons who have been trained in minimally invasive endocrine surgery performed the procedure using the same surgical technique. Demographic variables and clinical characteristics were collected, subsequently carrying out the descriptive analysis of the data. A total of 6 patients were registered, four associated with MEN type 2 syndrome and two in the context of VHL syndrome. Retroperitoneoscopic resection was performed without laparoscopic or open conversion and no postoperative complications; the average hospital stay was 2.5 days. Preservation of the functional cortex without corticosteroids was achieved in 5 (83%) of out 6 cases with a follow-up of 26.2 ± 6 months. Today, these 5 patients have a preserved adrenal function without hormone replacement. Cortical-sparing adrenalectomy by the retroperitoneal endoscopic approach, in expert hands, is safe and feasible for the treatment of hereditary and sporadic pheochromocytoma in a context of low malignancy, making it possible to avoid the need for corticoid replacement in most cases. Copyright © 2018 AEC. Publicado por Elsevier España, S.L.U. All rights reserved.

  4. [Extraoseus Ewings Sarcoma, Primary Affection of Uterine Cervix--Case Report].

    PubMed

    Bílek, O; Holánek, M; Zvaríková, M; Fabian, P; Robešová, B; Procházková, M; Adámková Krákorová, D

    2015-01-01

    Ewing's sarcoma is usually diagnosed in adolescents and young adults, peak of incidence is around 15 years of age. Primary localization is mostly in the skeleton of long bones and chest wall. Primary extraosseous involvement rarely occurs, incidence increases with age. We present a case report of a 57-year-old patient with locally advanced tumors of the cervix, clinical stage IIB. Due to histological and molecular genetic examination revealing EWS -ERG fusion gene, Ewing's sarcoma was diagnosed. CT revealed pathological pelvic lymphadenopathy and multiple pulmonary bilateral methastases, scintigraphy did not prove any affection of skeleton. The patient underwent a two-stage intensive chemotherapy regimens VIDE (vincristine, ifosfamide, doxorubicin, etoposide) and VAI (vincristine, actinomycin D, ifosfamide). During the second phase, concomitant radiotherapy of pelvis was aplied. According to PET/CT, complete remission was achieved. Whole-lung irradiation was applied in consolidation of the result. Primary Ewing's sarcoma of the cervix is an extremely rare disease. To our knowledge, only 12 cases was presented until this time. The average age at time of dia-gnosis was 35 years. Unlike the previous reports, we initially diagnosed distant metastases. The treatment was led according to the protocol Ewing 2008 designed for primary skeletal Ewing's sarcoma. Currently, 18 months after the therapy, the patient is without signs of disease. However, long-term follow-up is necessary.

  5. Imaging Primary Mouse Sarcomas After Radiation Therapy Using Cathepsin-Activatable Fluorescent Imaging Agents

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Cuneo, Kyle C.; Mito, Jeffrey K.; Javid, Melodi P.

    2013-05-01

    Purpose: Cathepsin-activated fluorescent probes can detect tumors in mice and in canine patients. We previously showed that these probes can detect microscopic residual sarcoma in the tumor bed of mice during gross total resection. Many patients with soft tissue sarcoma (STS) and other tumors undergo radiation therapy (RT) before surgery. This study assesses the effect of RT on the ability of cathepsin-activated probes to differentiate between normal and cancerous tissue. Methods and Materials: A genetically engineered mouse model of STS was used to generate primary hind limb sarcomas that were treated with hypofractionated RT. Mice were injected intravenously with cathepsin-activatedmore » fluorescent probes, and various tissues, including the tumor, were imaged using a hand-held imaging device. Resected tumor and normal muscle samples were harvested to assess cathepsin expression by Western blot. Uptake of activated probe was analyzed by flow cytometry and confocal microscopy. Parallel in vitro studies using mouse sarcoma cells were performed. Results: RT of primary STS in mice and mouse sarcoma cell lines caused no change in probe activation or cathepsin protease expression. Increasing radiation dose resulted in an upward trend in probe activation. Flow cytometry and immunofluorescence showed that a substantial proportion of probe-labeled cells were CD11b-positive tumor-associated immune cells. Conclusions: In this primary murine model of STS, RT did not affect the ability of cathepsin-activated probes to differentiate between tumor and normal muscle. Cathepsin-activated probes labeled tumor cells and tumor-associated macrophages. Our results suggest that it would be feasible to include patients who have received preoperative RT in clinical studies evaluating cathepsin-activated imaging probes.« less

  6. DNA methylation heterogeneity defines a disease spectrum in Ewing sarcoma

    PubMed Central

    Sheffield, Nathan C; Pierron, Gaelle; Klughammer, Johanna; Datlinger, Paul; Schönegger, Andreas; Schuster, Michael; Hadler, Johanna; Surdez, Didier; Guillemot, Delphine; Lapouble, Eve; Freneaux, Paul; Champigneulle, Jacqueline; Bouvier, Raymonde; Walder, Diana; Ambros, Ingeborg M; Hutter, Caroline; Sorz, Eva; Amaral, Ana T; de Álava, Enrique; Schallmoser, Katharina; Strunk, Dirk; Rinner, Beate; Liegl-Atzwanger, Bernadette; Huppertz, Berthold; Leithner, Andreas; de Pinieux, Gonzague; Terrier, Philippe; Laurence, Valérie; Michon, Jean; Ladenstein, Ruth; Holter, Wolfgang; Windhager, Reinhard; Dirksen, Uta; Ambros, Peter F; Delattre, Olivier; Kovar, Heinrich; Bock, Christoph; Tomazou, Eleni M

    2018-01-01

    Developmental tumors in children and young adults carry few genetic alterations, yet they have diverse clinical presentation. Focusing on Ewing sarcoma, we sought to establish the prevalence and characteristics of epigenetic heterogeneity in genetically homogeneous cancers. We performed genome-scale DNA methylation sequencing for a large cohort of Ewing sarcoma tumors and analyzed epigenetic heterogeneity on three levels: between cancers, between tumors, and within tumors. We observed consistent DNA hypomethylation at enhancers regulated by the disease-defining EWS-FLI1 fusion protein, thus establishing epigenomic enhancer reprogramming as a ubiquitous and characteristic feature of Ewing sarcoma. DNA methylation differences between tumors identified a continuous disease spectrum underlying Ewing sarcoma, which reflected the strength of an EWS-FLI1 regulatory signature and a continuum between mesenchymal and stem cell signatures. There was substantial epigenetic heterogeneity within tumors, particularly in patients with metastatic disease. In summary, our study provides a comprehensive assessment of epigenetic heterogeneity in Ewing sarcoma and thereby highlights the importance of considering nongenetic aspects of tumor heterogeneity in the context of cancer biology and personalized medicine. PMID:28134926

  7. Generation and comparison of CRISPR-Cas9 and Cre-mediated genetically engineered mouse models of sarcoma

    PubMed Central

    Huang, Jianguo; Chen, Mark; Whitley, Melodi Javid; Kuo, Hsuan-Cheng; Xu, Eric S.; Walens, Andrea; Mowery, Yvonne M.; Van Mater, David; Eward, William C.; Cardona, Diana M.; Luo, Lixia; Ma, Yan; Lopez, Omar M.; Nelson, Christopher E.; Robinson-Hamm, Jacqueline N.; Reddy, Anupama; Dave, Sandeep S.; Gersbach, Charles A.; Dodd, Rebecca D.; Kirsch, David G.

    2017-01-01

    Genetically engineered mouse models that employ site-specific recombinase technology are important tools for cancer research but can be costly and time-consuming. The CRISPR-Cas9 system has been adapted to generate autochthonous tumours in mice, but how these tumours compare to tumours generated by conventional recombinase technology remains to be fully explored. Here we use CRISPR-Cas9 to generate multiple subtypes of primary sarcomas efficiently in wild type and genetically engineered mice. These data demonstrate that CRISPR-Cas9 can be used to generate multiple subtypes of soft tissue sarcomas in mice. Primary sarcomas generated with CRISPR-Cas9 and Cre recombinase technology had similar histology, growth kinetics, copy number variation and mutational load as assessed by whole exome sequencing. These results show that sarcomas generated with CRISPR-Cas9 technology are similar to sarcomas generated with conventional modelling techniques and suggest that CRISPR-Cas9 can be used to more rapidly generate genotypically and phenotypically similar cancers. PMID:28691711

  8. Histone deacetylase inhibitor (HDACI) PCI-24781 enhances chemotherapy induced apoptosis in multidrug resistant sarcoma cell lines

    PubMed Central

    Yang, Cao; Choy, Edwin; Hornicek, Francis J.; Wood, Kirkham B; Schwab, Joseph H; Liu, Xianzhe; Mankin, Henry; Duan, Zhenfeng

    2013-01-01

    The anti-tumor activity of histone deacetylase inhibitors (HDACI) on multi-drug resistant sarcoma cell lines has never been previously described. Four multidrug resistant sarcoma cell lines treated with HDACI PCI-24781 resulted in dose-dependent accumulation of acetylated histones, p21 and PARP cleavage products. Growth of these cell lines was inhibited by PCI-24781 at IC50 of 0.43 to 2.7. When we looked for synergy of PCI-24781 with chemotherapeutic agents, we found that PCI-24781 reverses drug resistance in all four multidrug resistant sarcoma cell lines and synergizes with chemotherapeutic agents to enhance caspase-3/7 activity. Expression of RAD51 (a marker for DNA double-strand break repair) was inhibited and the expression of GADD45α (a marker for growth arrest and DNA-damage) was induced by PCI-24781 in multidrug resistant sarcoma cell lines. In conclusion, HDACI PCI-24781 synergizes with chemotherapeutic drugs to induce apoptosis and reverses drug resistance in multidrug resistant sarcoma cell lines. PMID:21508354

  9. Eotaxin/CCL11 in idiopathic retroperitoneal fibrosis.

    PubMed

    Mangieri, Domenica; Corradi, Domenico; Martorana, Davide; Malerba, Giovanni; Palmisano, Alessandra; Libri, Irene; Bartoli, Veronica; Carnevali, Maria L; Goldoni, Matteo; Govoni, Paolo; Alinovi, Rossella; Buzio, Carlo; Vaglio, Augusto

    2012-10-01

    Idiopathic retroperitoneal fibrosis (IRF) is a rare fibro-inflammatory disorder characterized by a periaortic tissue which often encases the ureters causing acute renal failure. IRF histology shows fibrosis and a chronic inflammatory infiltrate with frequent tissue eosinophilia. We assessed a panel of molecules promoting eosinophilia and fibrosis in IRF patients and performed an immunogenetic study. Serum levels of eotaxin/CCL11, regulated and normal T-cell expressed and secreted (RANTES), granulocyte colony-stimulating factor (G-CSF), interleukin (IL)-5, platelet-derived growth factor (PDGF) and fibroblast growth factor (FGF) were measured using a multiplex assay in 24 newly diagnosed, untreated IRF patients and 14 healthy controls. Retroperitoneal biopsies (available in 8/24 patients) were histologically evaluated to assess eosinophil infiltration, whereas mast cells (MCs) were identified by immunohistochemical analysis for human tryptase. Immunohistochemistry for eotaxin/CCL11 and its receptor CCR3 was also performed. Six single nucleotide polymorphisms (SNPs) within the CCL11 gene (rs6505403, rs1860184, rs4795896, rs17735961, rs16969415 and rs17809012) were investigated in 142 IRF patients and 214 healthy controls. Serum levels of eotaxin/CCL11 were higher in IRF patients than in controls (P = 0.009). Eotaxin/CCL11 drives tissue infiltration of eosinophils and MCs, which can promote fibrosis. Eosinophilic infiltration was prominent (>5 cells/hpf) in five (62.5%) cases, and abundant tryptase-positive MCs were found in all cases; notably, MCs were in a degranulating state. Immunohistochemistry showed that CCL11 was highly produced by infiltrating mononuclear cells and that its receptor CCR3 was expressed by infiltrating eosinophils, MCs, lymphocytes and fibroblasts. None of the tested CCL11 SNPs showed disease association, but the TTCCAT haplotype was significantly associated with IRF (P = 0.0005). These findings suggest that the eotaxin/CCL11-CCR3 axis is active

  10. Non-metastatic Ewing's sarcoma family of tumors of bone in adolescents and adults: prognostic factors and clinical outcome-single institution results.

    PubMed

    Oksüz, Didem Colpan; Tural, Deniz; Dincbas, Fazilet Öner; Dervisoglu, Sergülen; Turna, Hande; Hiz, Murat; Kantarci, Fatih; Ceylaner, Beyhan; Koca, Sedat; Mandel, Nil Molinas

    2014-01-01

    There is limited data regarding outcomes of Ewing's sarcoma family of tumors in adolescents and adults compared with the same tumors in childhood. The aim of the study was to analyze prognostic factors and treatment results in a cohort of adolescents and adults with non-metastatic skeletal Ewing's sarcoma family of tumors. From 1992-2008, 90 adolescents and adults with Ewing's sarcoma family of tumors of the bone were referred to our institution. Sixty-five (72%) non-metastatic patients with analyzable data and treated in our institution were retrospectively evaluated. All patients were treated with alternated chemotherapy regimens administered every 3 weeks. The local treatment modality was selected according to tumor and patient characteristics. The median age was 21 years (range, 13-50). Most patients (74%) were >17 years of age. Forty-six percent of the tumors were located in the extremities. Local therapy was surgery in 45 patients and radiotherapy alone in 19 patients. Twenty-one patients received preoperative and 13 patients postoperative radiotherapy. Median follow-up was 43 months (range, 7-167). The 5-year event-free and overall survival rates for all patients were 44% and 49%, respectively. On univariate survival analysis, event-free and overall survival were worse for patients >17 years of age, tumor size >8 cm in diameter, an axial location, positive surgical margins, and poor histopathological response (<90% necrosis). Age, tumor site and tumor size on event-free and overall survival remained significant on multivariate analysis. We identified age, tumor size, and tumor site as independent prognostic factors, in accord with the Western literature. These patients require novel treatment modalities.

  11. Somatic mutations in histiocytic sarcoma identified by next generation sequencing.

    PubMed

    Liu, Qingqing; Tomaszewicz, Keith; Hutchinson, Lloyd; Hornick, Jason L; Woda, Bruce; Yu, Hongbo

    2016-08-01

    Histiocytic sarcoma is a rare malignant neoplasm of presumed hematopoietic origin showing morphologic and immunophenotypic evidence of histiocytic differentiation. Somatic mutation importance in the pathogenesis or disease progression of histiocytic sarcoma was largely unknown. To identify somatic mutations in histiocytic sarcoma, we studied 5 histiocytic sarcomas [3 female and 2 male patients; mean age 54.8 (20-72), anatomic sites include lymph node, uterus, and pleura] and matched normal tissues from each patient as germ line controls. Somatic mutations in 50 "Hotspot" oncogenes and tumor suppressor genes were examined using next generation sequencing. Three (out of five) histiocytic sarcoma cases carried somatic mutations in BRAF. Among them, G464V [variant frequency (VF) of 43.6 %] and G466R (VF of 29.6 %) located at the P loop potentially interfere with the hydrophobic interaction between P and activating loops and ultimately activation of BRAF. Also detected was BRAF somatic mutation N581S (VF of 7.4 %), which was located at the catalytic loop of BRAF kinase domain: its role in modifying kinase activity was unclear. A similar mutational analysis was also performed on nine acute monocytic/monoblastic leukemia cases, which did not identify any BRAF somatic mutations. Our study detected several BRAF mutations in histiocytic sarcomas, which may be important in understanding the tumorigenesis of this rare neoplasm and providing mechanisms for potential therapeutical opportunities.

  12. Primary Intimal Sarcoma of Thoracic Aorta Presenting as Hypertensive Crisis

    PubMed Central

    Lin, Shu-I; Su, Min-I; Tsai, Cheng-Ting

    2015-01-01

    We report a 45-year-old woman who presented to our facility in a hypertensive crisis. Computed tomography (CT) revealed a thoracic aortic tumor, and tissues obtained via endovascular biopsy revealed undifferentiated sarcoma. A final diagnosis of intimal sarcoma was made by intra-operative pathological examination. Despite undergoing surgical resection followed by adjuvant chemotherapy, the patient died from progressive multiple metastasis and severe sepsis. Although aortic sarcoma is rarely diagnosed, it should be considered a possible etiology of hypertensive crisis. PMID:27122923

  13. Effectiveness of Vascular Markers (Immunohistochemical Stains) in Soft Tissue Sarcomas.

    PubMed

    Naeem, Namra; Mushtaq, Sajid; Akhter, Noreen; Hussain, Mudassar; Hassan, Usman

    2018-05-01

    To ascertain the effectiveness of IHC markers of vascular origin like CD31, CD34, FLI1 and ERG in vascular soft tissue sarcomas including angiosarcomas, Kaposi sarcomas, epithelioid hemangioendothelioma and a non-vascular soft tissue sarcoma (Epithelioid sarcoma). Descriptive study. Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, from 2011 to 2017. Diagnosed cases of angiosarcomas (n=48), epithelioid hemangioendothelioma (n=9), Kaposi sarcoma (n=9) and epithelioid sarcoma (n=20) were selected. Immunohistochemical staining as performed on formalin fixed paraffin embedded sections. The sections were stained for the following markers: CD34 (VENTANA clone Q Bend 10), CD31 (Leica clone 1 A 10), FLI1 (CELL MARQUE clone MRQ-1) and ERG (CELL MARQUE clone EP111). A complete panel of CD34, CD31 and ERG was applied on 8/48 cases of angiosarcomas with triple positivity in 6 cases. Eight cases showed positivity for only CD31 and ERG and 2 cases showed positivity for only ERG. A complete panel of CD34, CD31 and ERG was applied on 3/9 cases of epithelioid hemangioendothelioma with positivity for all markers in 2 cases. Combined positivity for ERG and CD34 was seen in 2 cases and on 4 cases only CD31 immunohistochemical was solely applied with 100% positivity. FLI1 was not applied on any case. Among 9 cases of Kaposi sarcoma, ERG, CD34 and CD31 in combination were applied on only 1 case with triple positivity. Remaining cases show positivity for either CD34, CD31 or FLI1. Majority of cases of epithelioid sarcomas were diagnosed on the basis of cytokeratin and CD34 positivity with loss of INI1. The other vascular markers showed negativity in all cases. Among these four markers, ERG immunohistochemical stain is highly effective for endothelial differentiation due to its specific nuclear staining pattern in normal blood vessel endothelial cells (internal control) as well as neoplastic cells of vascular tumors and lack of background staining.

  14. Reduction of MDSCs with all-trans retinoic acid improves CAR therapy efficacy for sarcomas

    PubMed Central

    Long, Adrienne H.; Highfill, Steven L.; Cui, Yongzhi; Smith, Jillian P.; Walker, Alec J.; Ramakrishna, Sneha; El-Etriby, Rana; Galli, Susana; Tsokos, Maria G.; Orentas, Rimas J.; Mackall, Crystal L.

    2016-01-01

    Genetically engineered T cells expressing CD19-specific chimeric antigen receptors (CARs) have shown impressive activity against B cell malignancies, and preliminary results suggest that T cells expressing a first generation disialoganglioside (GD2)-specific CAR can also provide clinical benefit in patients with neuroblastoma. We sought to assess the potential of GD2-CAR therapies to treat pediatric sarcomas. We observed that 18/18 (100%) of osteosarcomas, 2/15 (13%) of rhabdomyosarcomas, and 7/35 (20%) of Ewing sarcomas expressed GD2. T cells engineered to express a third generation GD2-CAR incorporating the 14g2a-scFv with the CD28, OX40, and CD3ζ signaling domains (14g2a.CD28.OX40.ζ) mediated efficient and comparable lysis of both GD2+ sarcoma and neuroblastoma cell lines in vitro. However in xenograft models, GD2-CAR T cells had no antitumor effect against GD2+ sarcoma, despite effectively controlling GD2+ neuroblastoma. We observed that pediatric sarcoma xenografts, but not neuroblastoma xenografts, induced large populations of monocytic and granulocytic murine myeloid-derived suppressor cells (MDSCs) that inhibited human CAR T-cell responses in vitro. Treatment of sarcoma-bearing mice with all-trans retinoic acid (ATRA) largely eradicated monocytic MDSCs and diminished the suppressive capacity of granulocytic MDSCs. Combined therapy using GD2-CAR T cells plus ATRA significantly improved antitumor efficacy against sarcoma xenografts. We conclude that retinoids provide a clinically accessible class of agents capable of diminishing the suppressive effects of MDSCs, and that co-administration of retinoids may enhance the efficacy of CAR therapies targeting solid tumors. PMID:27549124

  15. Stages of Uterine Sarcoma

    MedlinePlus

    ... stage of the cancer being treated. External and internal radiation therapy are used to treat uterine sarcoma, and may also be used as palliative therapy to relieve symptoms and improve quality of life . Chemotherapy Chemotherapy is a cancer treatment ...

  16. Long-term risk of sarcoma following radiation treatment for breast cancer

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Taghian, A.; de Vathaire, F.; Terrier, P.

    1991-07-01

    Between 1954 and 1983, 7620 patients were treated for breast carcinoma at Institut Gustave Roussy (France). Of these patients, 6919 were followed for at least 1 year. Out of these, 11 presented with sarcomas thought to be induced by irradiation, 2 of which were Steward-Treves Syndrome, and 9 of which were sarcomas within the irradiated fields. All histological slides were reviewed and a comparison with those of breast cancer was done. The sites of these sarcomas were: parietal wall, 1 case; second costal cartilage, 1 case; infraclavicular region, 1 case; supraclavicular region, 2 cases; internal third of the clavicle, 2more » cases; axillary region 2 cases; and the internal side of the upper arm (Stewart-Treves syndrome), 2 cases. The median age of these 11 patients at the diagnosis of sarcomas was 65.8 (49-83). The mean latent period was 9.5 years (4-24). Three patients underwent radical mastectomy and nine modified radical mastectomy. Only one patient received chemotherapy. The radiation doses received at the site of the sarcoma were 45 Gy/18 fr. for 10 cases and 90-100 Gy for 1 case (due to overlapping between two fields). The histology was as follows: malignant fibrous histiocytoma, 5 cases; fibrosarcoma, 3 cases; lymphangiosarcoma, 2 cases; and osteochondrosarcoma, 1 case. The median survival following diagnosis of sarcoma was 2.4 years (4 months-9 years). Two patients are still alive: one with recurrence of her breast cancer, the other in complete remission, with 7 and 3 years follow-up, respectively. All other patients died from their sarcomas. The cumulative incidence of sarcoma following irradiation of breast cancer was 0.2% (0.09-0.47) at 10 years. The standardized incidence ratio (SIR) of sarcoma (observed n{number sign} of cases (Obs)/expected n{number sign} of cases (Exp) computed from the Danish Cancer Registry for the same period) was 1.81 (CI 0.91-3.23).« less

  17. Sarcomas in north west England: I. Histopathological peer review.

    PubMed

    Harris, M; Hartley, A L; Blair, V; Birch, J M; Banerjee, S S; Freemont, A J; McClure, J; McWilliam, L J

    1991-08-01

    A total of 468 cases of bone, soft tissue and visceral sarcomas (and certain other tumours) diagnosed during the years 1982-84 in North West England were entered in a study of histopathological peer review, incidence and survival. This paper describes the effects of peer review. Material was reviewed by a panel of five pathologists for 413 of the 450 cases originally registered as sarcomas with the Regional Cancer Registry. The diagnosis of sarcomas was confirmed in 76% cases and and there was agreement on sub-type for 53% cases. Measures of agreement were lowest for the two sub-types most commonly diagnosed i.e. malignant fibrous histiocytoma and leiomyosarcoma. Degree of agreement between individual pathologists and final panel diagnosis was also very variable but never less than 65%. It is concluded that second opinion is essential in cases of presumed sarcomas for studies of incidence and aetiology and to ensure that appropriate treatment is selected.

  18. Ziv-aflibercept in Treating Patients With Locally Advanced, Unresectable, or Metastatic Gynecologic Soft Tissue Sarcoma

    ClinicalTrials.gov

    2015-12-03

    Fallopian Tube Cancer; Female Reproductive Cancer; Ovarian Carcinosarcoma; Ovarian Sarcoma; Recurrent Ovarian Epithelial Cancer; Recurrent Uterine Sarcoma; Stage III Ovarian Epithelial Cancer; Stage III Uterine Sarcoma; Stage IV Ovarian Epithelial Cancer; Stage IV Uterine Sarcoma; Uterine Carcinosarcoma; Uterine Leiomyosarcoma

  19. Collecting and Storing Tissue, Blood, and Bone Marrow Samples From Patients With Rhabdomyosarcoma or Other Soft Tissue Sarcoma

    ClinicalTrials.gov

    2017-12-11

    Adult Rhabdomyosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Chordoma; Desmoid Tumor; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Previously Untreated Childhood Rhabdomyosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  20. Strengthening health data on a rare and heterogeneous disease: sarcoma incidence and histological subtypes in Germany.

    PubMed

    Ressing, Meike; Wardelmann, Eva; Hohenberger, Peter; Jakob, Jens; Kasper, Bernd; Emrich, Katharina; Eberle, Andrea; Blettner, Maria; Zeissig, Sylke Ruth

    2018-02-12

    The population-based incidence of sarcoma and its histological subtypes in Germany is unknown. Up-to-date information on a disease with an incidence comparable to other cancer entities is of high public health relevance. The aim of this study was to determine this incidence and to detect significant changes in incidence trends using data from German epidemiological cancer registries. Pooled data from the German Centre for Cancer Registry Data with a primary diagnosis occurring in 2013 were used. To date, this is the latest data on cancer incidence available for Germany. All German cancer registries with sufficient completeness were included (10 out of 11), covering a population of 70.0 million people, representing 87% of the German population. All malignant sarcomas according to the RARECARE Project and the WHO classification 2002 were considered for analysis and, above all, gastrointestinal stromal tumours (GIST) of uncertain behaviour. Sensitivity analysis was performed excluding certain histologies. The analysis included 3404 cases in men and 3442 cases in women diagnosed in 2013. The age adjusted sarcoma incidence (European standard) was 7.4 (men) and 6.6 (women) per 100,000 inhabitants. About 70% of sarcomas were soft tissue sarcomas, about 22% GIST, and about 9% bone sarcomas. The most common histological subtypes besides GIST were fibrosarcomas (14%) and liposarcomas (12%) in men and complex mixed and stromal neoplasms (22%), non-uterine leiomysarcomas (10%) and fibrosarcomas (9%) in women. Considering the trend for the years of diagnosis 2004 to 2013, there was a significant increase in incidence for GIST while the incidence of soft tissue sarcomas (only men) as well as of bone sarcoma stayed constant over time. As to soft tissue sarcoma in women, the incidence stayed constant up to the year 2009 and significantly decreased afterwards. This study is the first detailed analysis of a German-wide population-based sarcoma incidence showing results comparable to

  1. Primary Synovial Sarcoma of External Auditory Canal: A Case Report

    PubMed Central

    Jayakumar, Krishnannair l L

    2017-01-01

    Synovial sarcoma is a rare malignant tumor of mesenchymal origin. Primary synovial sarcoma of the ear is extremely rare and to date only two cases have been published in English medical literature. Though the tumor is reported to have an aggressive nature, early diagnosis and treatment may improve the outcome. Here, we report a rare case of synovial sarcoma of the external auditory canal in an 18-year-old male who was managed by chemotherapy and referred for palliation due to tumor progression. PMID:28948118

  2. Primary Synovial Sarcoma of External Auditory Canal: A Case Report.

    PubMed

    Devi, Aarani; Jayakumar, Krishnannair L L

    2017-07-20

    Synovial sarcoma is a rare malignant tumor of mesenchymal origin. Primary synovial sarcoma of the ear is extremely rare and to date only two cases have been published in English medical literature. Though the tumor is reported to have an aggressive nature, early diagnosis and treatment may improve the outcome. Here, we report a rare case of synovial sarcoma of the external auditory canal in an 18-year-old male who was managed by chemotherapy and referred for palliation due to tumor progression.

  3. Sarcoma of the thyroid region mimicking Riedel's thyroiditis

    PubMed Central

    Torres-Montaner, A; Beltran, M; d Romero; Oliva, H

    2001-01-01

    Because sarcomas of the anterior lower neck region occur so infrequently, they are not usually considered in the differential diagnosis of Riedel's thyroiditis. Riedel's thyroiditis itself may be confused on clinical grounds alone with malignant neoplasms because of its invasive features. Sarcomatoid carcinoma is the main entity to be discarded in this regard. This is accomplished through histological examination by the finding of carcinomatous areas and/or reactivity with epithelial markers. These features also set apart sarcomatoid carcinoma from true sarcomas. This report concerns a patient with a sarcoma of the anterior lower neck region which was initially suspected to be Riedel's thyroiditis or sarcomatoid carcinoma on clinical and radiological grounds. A peroperative biopsy was interpreted by two independent pathologists as consistent with Riedel's thyroiditis. The subsequent clinical course and postmortem examination demonstrated a high grade sarcoma with metastasis to both lungs and the pleura, and invasion of adjacent neck structures. Nevertheless, some areas of the postmortem material showed a microscopic pattern similar to mediastinal fibrosis, raising the possibility of the malignant transformation of a fibrosclerotic lesion. Key Words: Riedel's thyroiditis • sarcomatoid carcinoma • fibrous histiocytoma • differential diagnosis PMID:11429435

  4. Head and Neck Sarcomas: The UCLA Experience

    PubMed Central

    Tajudeen, Bobby A.; Fuller, Jennifer; Lai, Chi; Grogan, Tristan; Elashoff, David; Abemayor, Elliot; St. John, Maie

    2014-01-01

    Purpose To profile the clinical presentation, subtype distribution, and treatment results of sarcomas of the head and neck at a single tertiary academic center over an 11-year period. Materials and Methods A retrospective review was performed by examining the records and reviewing the pathology of 186 patients with head and neck sarcomas treated at UCLA Medical Center from 2000 to 2011. Results The mean age of the study population was 49 +/− 22 years. 58% of the patients were male and 42% were female. Median duration of follow-up for the entire group was 18.5 months. The most common presenting symptom was a mass lesion in 59.9% of patients. The nasal cavity/sinus was the most common presenting site seen in 22% of patients. Solitary fibrous tumor/hemangiopericytoma was the most common subtype. 15% of patients had evidence of prior radiation exposure. 26.3% of tumors were greater than 5cm and 35.5% were high-grade. Margins were positive in 31.2% of patients. Lymph node metastasis was rare at 6.5%. Perineural invasion was identified in 6.5%. Among all subtypes, 5-yr recurrence-free survival and overall survival were 50% and 49%, respectively. Multivariate analysis demonstrated that grade and margin status were predictors of recurrence-free survival while grade and age affected overall survival. Conclusions Head and neck sarcomas are a rare entity frequently presenting as a mass lesion. In our series, lesions tended to be high-grade with a significant portion of surgical specimens having positive margins. Grade and margin status were the most important predictors of survival. PMID:24721744

  5. Cellular immunotherapy for soft tissue sarcomas

    PubMed Central

    Finkelstein, Steven Eric; Fishman, Mayer; Conley, Anthony P.; Gabrilovich, Dmitry; Antonia, Scott; Chiappori, Alberto

    2015-01-01

    SUMMARY Soft tissue sarcomas are rare neoplasms, with approximately 9,000 new cases in the United States every year. Unfortunately, there is little progress in the treatment of metastatic soft tissue sarcomas in the past two decades beyond the standard approaches of surgery, chemotherapy, and radiation. Immunotherapy is a modality complementary to conventional therapy,. It is appealing because functional anti-tumor activity could affect both local-regional and systemic disease and act over a prolonged period of time. In this report, we review immunotherapeutic investigative strategies being developed, including several tumor vaccine, antigen vaccine, and dendritic cell vaccine strategies. PMID:22401634

  6. Malignant inguinal monophasic synovial sarcoma: report of a case and review of the literature.

    PubMed

    Xu, Ji; Wang, Jia; Cui, Long; Wu, Xiangru

    2010-11-21

    A synovial sarcoma (SS) is an aggressive soft tissue tumor that classically occurs in the extremities near, but rarely within large joints, in young adults. Variable symptoms and clinical manifestations may be encountered and a definite diagnosis should depend on pathological results. This poses certain difficulties in arriving at a prompt diagnosis and appropriate treatment. We report the case of a 68-year-old woman patient who presented an inguinal mass with swelling and pain in the right lower limb. She underwent surgery, and later received systematic intravenous chemotherapy. The pathological studies, especially the specific chromosomal translocation of a t(X;18) (p11.2;q11.2), confirmed the diagnosis as a synovial sarcoma. To the best of our knowledge, this is the first report of a monophasic synovial sarcoma in the inguinal region. Besides making the readership aware of the rarity of location and age of this present case, this report distinctly highlights the great value of a molecular analysis of an SYT associated genetic alteration in the diagnosis of synovial sarcoma occurring at rare sites especially when immunochemical results are equivocal.

  7. Genetic susceptibility to bone and soft tissue sarcomas: a field synopsis and meta-analysis.

    PubMed

    Benna, Clara; Simioni, Andrea; Pasquali, Sandro; De Boni, Davide; Rajendran, Senthilkumar; Spiro, Giovanna; Colombo, Chiara; Virgone, Calogero; DuBois, Steven G; Gronchi, Alessandro; Rossi, Carlo Riccardo; Mocellin, Simone

    2018-04-06

    The genetic architecture of bone and soft tissue sarcomas susceptibility is yet to be elucidated. We aimed to comprehensively collect and meta-analyze the current knowledge on genetic susceptibility in these rare tumors. We conducted a systematic review and meta-analysis of the evidence on the association between DNA variation and risk of developing sarcomas through searching PubMed, The Cochrane Library, Scopus and Web of Science databases. To evaluate result credibility, summary evidence was graded according to the Venice criteria and false positive report probability (FPRP) was calculated to further validate result noteworthiness. Integrative analysis of genetic and eQTL (expression quantitative trait locus) data was coupled with network and pathway analysis to explore the hypothesis that specific cell functions are involved in sarcoma predisposition. We retrieved 90 eligible studies comprising 47,796 subjects (cases: 14,358, 30%) and investigating 1,126 polymorphisms involving 320 distinct genes. Meta-analysis identified 55 single nucleotide polymorphisms (SNPs) significantly associated with disease risk with a high (N=9), moderate (N=38) and low (N=8) level of evidence, findings being classified as noteworthy basically only when the level of evidence was high. The estimated joint population attributable risk for three independent SNPs (rs11599754 of ZNF365/EGR2 , rs231775 of CTLA4 , and rs454006 of PRKCG ) was 37.2%. We also identified 53 SNPs significantly associated with sarcoma risk based on single studies.Pathway analysis enabled us to propose that sarcoma predisposition might be linked especially to germline variation of genes whose products are involved in the function of the DNA repair machinery. We built the first knowledgebase on the evidence linking DNA variation to sarcomas susceptibility, which can be used to generate mechanistic hypotheses and inform future studies in this field of oncology.

  8. Vaccine-associated sarcomas in cats: a unique cancer model.

    PubMed

    McNiel, E A

    2001-01-01

    Epidemiologic evidence supports a relationship between vaccination of cats for rabies and feline leukemia virus with the development of soft tissue sarcomas at the site of administration. These tumors are locally invasive and histologically aggressive. As with high-grade soft tissue sarcoma in humans, combination treatment with radiation therapy and surgery provides for optimum tumor control. Feline vaccine-associated sarcoma has become a difficult issue for the veterinary profession for legal, ethical, and clinical reasons. Although most research efforts have focused on therapeutic intervention, this tumor has great potential to provide an informative model for carcinogenesis and genetic susceptibility applicable to cancer in all species, including humans.

  9. Endometrial stromal sarcoma diagnosed after uterine morcellation in laparoscopic supracervical hysterectomy.

    PubMed

    Della Badia, Carl; Karini, Homa

    2010-01-01

    Endometrial stromal sarcoma is a rare uterine cancer with no reliable method for preoperative diagnosis. A 30-year-old parous woman underwent laparoscopic supracervical hysterectomy because of a leiomyoma. The uterus was removed from the abdominal cavity with an electric morcellator with a spinning blade. The pathology report revealed low-grade endometrial stromal sarcoma. Two months after the initial surgery, a second laparoscopic procedure was performed. The final pathology report confirmed low-grade endometrial stromal sarcoma involving the ovary, fallopian tube, and ovarian artery. It was concluded that morcellation of leiomyomas at laparoscopic supracervical hysterectomy may potentially increase metastasis if the tumor is a sarcoma. Copyright © 2010 AAGL. Published by Elsevier Inc. All rights reserved.

  10. Synovial sarcoma of the jaw in a dog.

    PubMed

    Griffith, J W; Frey, R A; Sharkey, F E

    1987-05-01

    A case of synovial sarcoma of the jaw with pulmonary metastasis is described in a dog. It appears to be a rare or underdiagnosed neoplasm in animals and not previously reported in the jaw. Its diagnostic microscopic features are the biphasic cellular pattern and cleft formations. It may otherwise resemble haemangiopericytoma, malignant fibrous histiocytoma, reticulum cell sarcoma, fibrosarcoma, or giant-cell tumour of soft tissue.

  11. Transperitoneal approach versus retroperitoneal approach: a meta-analysis of laparoscopic partial nephrectomy for renal cell carcinoma.

    PubMed

    Ren, Tong; Liu, Yan; Zhao, Xiaowen; Ni, Shaobin; Zhang, Cheng; Guo, Changgang; Ren, Minghua

    2014-01-01

    To compare the efficiency and safety of the transperitoneal approaches with retroperitoneal approaches in laparoscopic partial nephrectomy for renal cell carcinoma and provide evidence-based medicine support for clinical treatment. A systematic computer search of PUBMED, EMBASE, and the Cochrane Library was executed to identify retrospective observational and prospective randomized controlled trials studies that compared the outcomes of the two approaches in laparoscopic partial nephrectomy. Two reviewers independently screened, extracted, and evaluated the included studies and executed statistical analysis by using software STATA 12.0. Outcomes of interest included perioperative and postoperative variables, surgical complications and oncological variables. There were 8 studies assessed transperitoneal laparoscopic partial nephrectomy (TLPN) versus retroperitoneal laparoscopic partial nephrectomy (RLPN) were included. RLPN had a shorter operating time (SMD = 1.001,95%confidence interval[CI] 0.609-1.393,P<0.001), a lower estimated blood loss (SMD = 0.403,95%CI 0.015-0.791,P = 0.042) and a shorter length of hospital stay (WMD = 0.936 DAYS,95%CI 0.609-1.263,P<0.001) than TLPN. There were no significant differences between the transperitoneal and retroperitoneal approaches in other outcomes of interest. This meta-analysis indicates that, in appropriately selected patients, especially patients with intraperitoneal procedures history or posteriorly located renal tumors, the RLPN can shorten the operation time, reduce the estimated blood loss and shorten the length of hospital stay. RLPN may be equally safe and be faster compared with the TLPN.

  12. Undifferentiated granulocytic sarcoma: a case with epidural onset preceding acute promyelocytic leukemia.

    PubMed

    Tosi, A; De Paoli, A; Fava, S; Luoni, M; Sironi, M; Tocci, A; Assi, A; Cassi, E

    1995-01-01

    This study reports a case of granulocytic sarcoma that developed in the epidural zone 25 days before clinical evidence of an acute promyelocytic leukemia. The case presented the diagnostic difficulties that are common to all aleukemic granulocytic sarcomas. Moreover, it highlights the very rare association between granulocytic sarcoma and acute promyelocytic leukemia, which is far from being explained.

  13. Patients with Advanced, Rare Sarcoma Respond to Cediranib | Center for Cancer Research

    Cancer.gov

    Alveolar soft part sarcomas (ASPS) are highly vascular tumors that usually affect adolescents and young adults. Comprising less than one percent of soft tissue sarcomas, ASPS can be cured with surgery. However, its tendency to metastasize and its lack of response to standard soft tissue sarcoma chemotherapy regimens makes ASPS a particularly lethal cancer with a five-year

  14. [Primary breast synovial sarcoma].

    PubMed

    Alfaro-Cervelló, Clara; Burgués, Octavio

    Primary synovial sarcoma of the breast is very rare. We report a case of a 33-year-old woman, who had previously undergone a radical mastectomy, having been diagnosed with fusocellular breast carcinoma. Histopathology revealed a hypercellular lesion formed by spindle cells with storiform and herringbone patterns. Immunohistochemistry showed strong expression of vimentin and CD99, and focal bcl2, EMA, CK AE1-AE3, actin and desmin, with negativity for S100, CD34, CK7, CK14, CK19, hormone receptors, caldesmon and myosin. Molecular biology revealed the expression of the fusion product of the SS18 and SSX genes, indicative of the translocation t(X;18)(p11.2;q11.2), which confirmed the diagnosis of synovial sarcoma. Copyright © 2017 Sociedad Española de Anatomía Patológica. Publicado por Elsevier España, S.L.U. All rights reserved.

  15. Paediatric and adult soft tissue sarcomas with NTRK1 gene fusions: a subset of spindle cell sarcomas unified by a prominent myopericytic/haemangiopericytic pattern.

    PubMed

    Haller, Florian; Knopf, Jasmin; Ackermann, Anne; Bieg, Matthias; Kleinheinz, Kortine; Schlesner, Matthias; Moskalev, Evgeny A; Will, Rainer; Satir, Ali Abdel; Abdelmagid, Ibtihalat E; Giedl, Johannes; Carbon, Roman; Rompel, Oliver; Hartmann, Arndt; Wiemann, Stefan; Metzler, Markus; Agaimy, Abbas

    2016-04-01

    Neoplasms with a myopericytomatous pattern represent a morphological spectrum of lesions encompassing myopericytoma of the skin and soft tissue, angioleiomyoma, myofibromatosis/infantile haemangiopericytoma and putative neoplasms reported as malignant myopericytoma. Lack of reproducible phenotypic and genetic features of malignant myopericytic neoplasms have prevented the establishment of myopericytic sarcoma as an acceptable diagnostic category. Following detection of a LMNA-NTRK1 gene fusion in an index case of paediatric haemangiopericytoma-like sarcoma by combined whole-genome and RNA sequencing, we identified three additional sarcomas harbouring NTRK1 gene fusions, termed 'spindle cell sarcoma, NOS with myo/haemangiopericytic growth pattern'. The patients were two children aged 11 months and 2 years and two adults aged 51 and 80 years. While the tumours of the adults were strikingly myopericytoma-like, but with clear-cut atypical features, the paediatric cases were more akin to infantile myofibromatosis/haemangiopericytoma. All cases contained numerous thick-walled dysplastic-like vessels with segmental or diffuse nodular myxohyaline myo-intimal proliferations of smooth muscle actin-positive cells, occasionally associated with thrombosis. Immunohistochemistry showed variable expression of smooth muscle actin and CD34, but other mesenchymal markers, including STAT6, were negative. This study showed a novel variant of myo/haemangiopericytic sarcoma with recurrent NTRK1 gene fusions. Given the recent introduction of a novel therapeutic approach targeting NTRK fusion-positive neoplasms, recognition of this rare but likely under-reported sarcoma variant is strongly encouraged. Copyright © 2016 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

  16. CT-guided percutaneous ethanol injection with disposable curved needle for treatment of malignant liver neoplasms and their metastases in retroperitoneal lymph nodes

    PubMed Central

    Zuo, Chang-Jing; Wang, Pei-Jun; Shao, Cheng-Wei; Wang, Min-Jie; Tian, Jian-Ming; Xiao, Yi; Ren, Fang-Yuan; Hao, Xi-Yan; Yuan, Min

    2004-01-01

    AIM: To explore the feasibility of computed tomography (CT)-guided percutaneous ethanol injection (PEI) using a disposable curved needle for treatment of malignant liver neoplasms and their metastases in retroperitoneal lymph nodes. METHODS: CT-guided PEI was conducted using a disposable curved needle in 26 malignant liver tumors smaller than 5 cm in diameter and 5 lymph node metastases of liver cancer in the retroperitoneal space. The disposable curved needle was composed of a straight trocar (21G) and stylet, a disposable curved tip (25 G) and a fine stylet. For the tumors found in deep sites and difficult to reach, or for hepatic masses inaccessible to the injection using a straight needle because of portal vein and bile ducts, the straight trocar was used at first to reach the side of the tumor. Then, the disposable curved needle was used via the trocar. When the needle reached the tumor center, appropriate amount of ethanol was injected. For relatively large malignant liver tumors, multi-point injection was carried out for a better distribution of the ethanol injected throughout the masses. The curved needle was also used for treatment of the metastasis in retroperitoneal lymph nodes blocked by blood vessels and inaccessible by the straight needle. RESULTS: All of the 26 liver tumors received 2 or more times of successful PEI, through which ethanol was distributed throughout the whole tumor mass. Effect of the treatment was monitored by contrast-enhanced multi-phase CT and magnetic resonance imaging (MRI) examinations three months later. Of the 18 lesions whose diameters were smaller than 3 cm, the necrotic change across the whole mass and that in most areas were observed in 15 and 3 tumors, respectively. Among the 8 tumors sizing up to 5 cm, 5 were completely necrotic and 3 largely necrotic. Levels of tumor seromarkers were significantly reduced in some of the cases. In 5 patients with metastases of liver cancer in retroperitoneal lymph nodes who received 1

  17. Overcoming cetuximab resistance in Ewing's sarcoma by inhibiting lactate dehydrogenase-A.

    PubMed

    Fu, Jiaxin; Jiang, Han; Wu, Chenxuan; Jiang, Yi; Xiao, Lianping; Tian, Yonggang

    2016-07-01

    Ewing's sarcoma, the second most common type of malignant bone tumor, generally occurs in children and young adults. The current treatment of Ewing's sarcoma comprises systemic anti‑cancer chemotherapy with complete surgical resection. However, the majority of patients with Ewing's sarcoma develop resistance to chemotherapy. The present study revealed an oncogenic role of lactate dehydrogenase‑A (LDHA) in the resistance of Ewing's sarcoma to cetuximab. LDHA was shown to be upregulated at the protein and mRNA level in cetuximab‑resistant Ewing's sarcoma tissues and a cell line. In addition, a link between LDHA‑induced glycolysis and cetuximab resistance in Ewing's sarcoma cells was revealed. Of note, inhibition of LDHA by either small interfering RNA or LDHA inhibitor oxamate significantly re‑sensitized cetuximab‑resistant cells to cetuximab. Combined treatment with LDHA inhibitor and cetuximab synergistically reduced the viability of cetuximab-resistant cells through the suppression of LDHA. The present study revealed a novel mechanism of cetuximab resistance from the perspective of cancer‑cell metabolism and provided a sensitization approach, which may aid in the development of anti-chemoresistance strategies for the treatment of cetuximab-resistant Ewing's sarcoma.

  18. Preclinical Evaluation of Vemurafenib as Therapy for BRAFV600E Mutated Sarcomas.

    PubMed

    Gouravan, Sarina; Meza-Zepeda, Leonardo A; Myklebost, Ola; Stratford, Eva W; Munthe, Else

    2018-03-23

    The BRAF V600E mutation, which in melanoma is targetable with vemurafenib, is also found in sarcomas and we here evaluate the therapeutic potential in sarcoma cell lines. Four sarcoma cell lines harboring the BRAFV600E mutation, representing liposarcomas (SA-4 and SW872), Ewing sarcoma (A673) and atypical synovial sarcoma (SW982), were treated with vemurafenib and the effects on cell growth, apoptosis, cell cycle progression and cell signaling were determined. Vemurafenib induced a strong cytostatic effect in SA-4 cells, mainly due to cell cycle arrest, whereas only moderate levels of apoptosis were observed. However, a high dose was required compared to BRAF V600E mutated melanoma cells, and removal of vemurafenib demonstrated that the continuous presence of drug was required for sustained growth inhibition. A limited growth inhibition was observed in the other three cell lines. Protein analyses demonstrated reduced phosphorylation of ERK during treatment with vemurafenib in all the four sarcoma cell lines confirming that the MAPK pathway is active in these cell lines, and that the pathway can be inhibited by vemurafenib, but also that these cells can proliferate despite this. These findings indicate that vemurafenib alone would not be an efficient therapy against BRAF V600E mutated sarcomas. However, further investigations of combination with other drugs are warranted.

  19. Sarcoma spreads primarily through the vascular system: are there biomarkers associated with vascular spread?

    PubMed

    Pennacchioli, Elisabetta; Tosti, Giulio; Barberis, Massimo; De Pas, Tommaso M; Verrecchia, Francesco; Menicanti, Claudia; Testori, Alessandro; Mazzarol, Giovanni

    2012-10-01

    Sarcomas are a heterogeneous group of tumors with specific molecular characteristics and currently classified on the basis of their tissue of origin and histologic appearance. Except for epithelioid sarcoma, clear cell sarcoma, angiosarcoma and rhabdomyosarcoma, which may spread to regional lymph nodes, the other histotypes spread via the vascular system to the lungs most of the time. A variety of molecular approaches, including gene expression profiling, have identified candidate biomarkers and generated insights into sarcoma biology. The comprehension of the pathogenesis of this malignancy according to the mesenchymal stem cell hypothesis parallels the description of several molecular pathways deregulated in sarcoma. Individuation of vascular spread biomarkers is actually focused on the study of factors involved both in hemostasis and angiogenesis. Interestingly the microenvironment of sarcomas showed the very same mesenchymal origin of the surrounding stromal cells. The presence of circulating tumor cells and miRNAs in blood samples of sarcoma patients represents the possibility not only to better stratify patients group according to the prognosis but also to tailor new individualized therapy. So, it could be predicted that some genes expressed in a specific sarcoma might have prognostic significance or therapeutic targeting potential and molecular targets can be identified in the tumor or in the tumor microenvironment. Therefore the initial evaluation of a sarcoma patient should include in-depth genetic evaluation including karyotyping and c-DNA/protein expression profiling. The chemokine signaling demonstrated to be deeply implicated in sarcoma development as well as to have a significant role in development of metastatic disease, especially in directing tumor cells towards the preferential sites of metastases in sarcoma, lung and bone. It is unsolved if the blood stream is a more favorable environment compared to lymphatic or if lymph nodes are more efficient

  20. Subungual Ewing sarcoma/PNET tumor family of the great toe: a case report

    PubMed Central

    Binesh, Fariba; Sobhanardekani, Mohammad; Zare, Saeedeh; Behniafard, Nasim

    2016-01-01

    Ewing’s sarcoma is seen mainly in patients less than 18. This aggressive tumor generally affects the axial skeleton and only rarely involves the acral regions. Ewing’s sarcoma in the foot is inordinately scarce. Clinical features are uncertain and can imitate other common diseases. This paper presents a case of 62-year-old malewith complaints of pain and swelling of the subungual area of his right great toe. The lesion was excised, and histopathological diagnosis of Ewing’s sarcoma was made. Histopathological examination, supported by immunochemical methods, remains the mainstay of diagnosis. Surgical ablation along with chemotherapy is the therapy of choice. To our knowledge, this is the first report of Ewing’s sarcoma involving the nail bed of the great toe without bone erosion. The key messages of this case report is “Subungual Ewing sarcoma is a rare case, and Ewing’s sarcoma must be kept in mind for acral lesions, especially in the adult population.” PMID:27279998

  1. Retroperitoneal composite pheochromocytoma-ganglioneuroma : a case report and review of literature

    PubMed Central

    2013-01-01

    Abstract Composite pheochromocytoma/paraganglioma is a rare tumor with elements of pheochromocytoma/paraganglioma and neurogenic tumor. Most were located in the adrenal glands, and extra-adrenal composite pheochromocytoma is extremely rare. Only 4 cases in the retroperitoneum have been described in the online database PUBMED. Here, we report a case of retroperitoneal extra-adrenal composite pheochromocytoma and review the related literature. Virtual slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1700539911908679 PMID:23587063

  2. Biphasic synovial sarcoma in the cervical spine: Case report.

    PubMed

    Foreman, Stephen M; Stahl, Michael J

    2011-05-23

    Synovial sarcoma is a rare malignant neoplasm of soft tissue that typically arising near large joints of the upper and lower extremities in young adult males. Only 3% of these neoplasms have been found to arise in the head and neck region. To our knowledge, there are limited reports in the literature of this neoplasm in the cervical spine.A case of biphasic synovial sarcoma of the cervical spine is reviewed. A 29 year-old male presented with pain on the left side of the cervical spine. Physical examination revealed a global loss of cervical motion and large, palpable mass in the left paravertebral area. The long-delayed Magnetic Resonance (MR) scan revealed a soft tissue mass measuring 8.3 centimeters (cm) × 5.7 cm that was surgically removed. A malignant biphasic synovial sarcoma was diagnosed on pathologic examination.The clinical and imaging findings of an atypically located synovial sarcoma are reviewed. This case report emphasizes the consequences of a limited differential diagnosis, prolonged treatment and the failure to perform timely diagnostic imaging in the presence of a paraspinal mass.

  3. Multidetector Computed Tomography Features in Differentiating Exophytic Renal Angiomyolipoma from Retroperitoneal Liposarcoma

    PubMed Central

    Wang, Qiushi; Juan, Yu-Hsiang; Li, Yong; Xie, Jia-Jun; Liu, Hui; Huang, Hongfei; Liu, Zaiyi; Zheng, Junhui; Saboo, Ujwala S.; Saboo, Sachin S.; Liang, Changhong

    2015-01-01

    Abstract This study aims to evaluate the multidetector computed tomography (CT) imaging features in differentiating exophytic renal angiomyolipoma (AML) from retroperitoneal liposarcoma. We retrospectively enrolled 42 patients with confirmed exophytic renal AML (31 patients) or retroperitoneal liposarcoma (11 patients) during 8 years period to assess: renal parenchymal defect at site of tumor contact, supply from branches of renal artery, tumoral vessel extending through the renal parenchyma, dilated intratumoral vessels, hemorrhage, non–fat-containing intratumoral nodules with postcontrast enhancement, calcification, renal sinus enlargement, anterior displacement of kidneys, and other associated AML. Renal parenchymal defect, renal arterial blood supply, tumoral vessel through the renal parenchyma, dilated intratumoral vessels, intratumoral/perirenal hemorrhage, renal sinus enlargement, and associated AML were seen only or mainly in exophytic renal AML (all P value < 0.05); however, non–fat-attenuating enhancing intratumoral nodules, intratumoral calcification, and anterior displacement of the kidney were more common in liposarcoma (all P value < 0.05). AMLs reveal renal parenchymal defect at the site of tumor contact, supply from renal artery, tumoral vessel extending through the renal parenchyma, dilated intratumoral vessels, intratumoral and/or perirenal hemorrhage, renal sinus enlargement, and associated AML. Non–fat-attenuating enhancing intratumoral nodules, intratumoral calcifications, and anterior displacement of kidney were more commonly seen in liposarcoma. PMID:26376398

  4. Talimogene Laherparepvec and Radiation Therapy in Treating Patients With Newly Diagnosed Soft Tissue Sarcoma That Can Be Removed by Surgery

    ClinicalTrials.gov

    2018-05-23

    FNCLCC Sarcoma Grade 2; FNCLCC Sarcoma Grade 3; Leiomyosarcoma; Liposarcoma; Stage I Soft Tissue Sarcoma AJCC v7; Stage IA Soft Tissue Sarcoma AJCC v7; Stage IB Soft Tissue Sarcoma AJCC v7; Stage II Soft Tissue Sarcoma AJCC v7; Stage IIA Soft Tissue Sarcoma AJCC v7; Stage IIB Soft Tissue Sarcoma AJCC v7; Undifferentiated Pleomorphic Sarcoma

  5. Primary pericranial Ewing's sarcoma on the temporal bone: A case report.

    PubMed

    Kawano, Hiroto; Nitta, Naoki; Ishida, Mitsuaki; Fukami, Tadateru; Nozaki, Kazuhiko

    2016-01-01

    Primary Ewing's sarcoma originating in the pericranium is an extremely rare disease entity. A 9-year-old female patient was admitted to our department due to a left temporal subcutaneous mass. The mass was localized under the left temporal muscle and attached to the surface of the temporal bone. Head computed tomography revealed a mass with bony spicule formation on the temporal bone, however, it did not show bone destruction or intracranial invasion. F-18 fluorodeoxyglucose positron emission tomography showed no lesions other than the mass on the temporal bone. Magnetic resonance imaging showed that the mass was located between the temporal bone and the pericranium. The mass was completely resected with the underlying temporal bone and the overlying deep layer of temporal muscle, and was diagnosed as primary Ewing's sarcoma. Because the tumor was located in the subpericranium, we created a new classification, "pericranial Ewing's sarcoma," and diagnosed the present tumor as pericranial Ewing's sarcoma. We herein present an extremely rare case of primary pericranial Ewing's sarcoma that developed on the temporal bone.

  6. Pre- and postoperative radiotherapy for extremity soft tissue sarcoma: Evaluation of inter-observer target volume contouring variability among French sarcoma group radiation oncologists.

    PubMed

    Sargos, P; Charleux, T; Haas, R L; Michot, A; Llacer, C; Moureau-Zabotto, L; Vogin, G; Le Péchoux, C; Verry, C; Ducassou, A; Delannes, M; Mervoyer, A; Wiazzane, N; Thariat, J; Sunyach, M P; Benchalal, M; Laredo, J D; Kind, M; Gillon, P; Kantor, G

    2018-04-01

    The purpose of this study was to evaluate, during a national workshop, the inter-observer variability in target volume delineation for primary extremity soft tissue sarcoma radiation therapy. Six expert sarcoma radiation oncologists (members of French Sarcoma Group) received two extremity soft tissue sarcoma radiation therapy cases 1: one preoperative and one postoperative. They were distributed with instructions for contouring gross tumour volume or reconstructed gross tumour volume, clinical target volume and to propose a planning target volume. The preoperative radiation therapy case was a patient with a grade 1 extraskeletal myxoid chondrosarcoma of the thigh. The postoperative case was a patient with a grade 3 pleomorphic undifferentiated sarcoma of the thigh. Contour agreement analysis was performed using kappa statistics. For the preoperative case, contouring agreement regarding GTV, gross tumour volume GTV, clinical target volume and planning target volume were substantial (kappa between 0.68 and 0.77). In the postoperative case, the agreement was only fair for reconstructed gross tumour volume (kappa: 0.38) but moderate for clinical target volume and planning target volume (kappa: 0.42). During the workshop discussion, consensus was reached on most of the contour divergences especially clinical target volume longitudinal extension. The determination of a limited cutaneous cover was also discussed. Accurate delineation of target volume appears to be a crucial element to ensure multicenter clinical trial quality assessment, reproducibility and homogeneity in delivering RT. radiation therapy RT. Quality assessment process should be proposed in this setting. We have shown in our study that preoperative radiation therapy of extremity soft tissue sarcoma has less inter-observer contouring variability. Copyright © 2018 Société française de radiothérapie oncologique (SFRO). Published by Elsevier SAS. All rights reserved.

  7. Interactomic approach for evaluating nucleophosmin-binding proteins as biomarkers for Ewing's sarcoma.

    PubMed

    Haga, Ayako; Ogawara, Yoko; Kubota, Daisuke; Kitabayashi, Issay; Murakami, Yasufumi; Kondo, Tadashi

    2013-06-01

    Nucleophosmin (NPM) is a novel prognostic biomarker for Ewing's sarcoma. To evaluate the prognostic utility of NPM, we conducted an interactomic approach to characterize the NPM protein complex in Ewing's sarcoma cells. A gene suppression assay revealed that NPM promoted cell proliferation and the invasive properties of Ewing's sarcoma cells. FLAG-tag-based affinity purification coupled with liquid chromatography-tandem mass spectrometry identified 106 proteins in the NPM protein complex. The functional classification suggested that the NPM complex participates in critical biological events, including ribosome biogenesis, regulation of transcription and translation, and protein folding, that are mediated by these proteins. In addition to JAK1, a candidate prognostic biomarker for Ewing's sarcoma, the NPM complex, includes 11 proteins known as prognostic biomarkers for other malignancies. Meta-analysis of gene expression profiles of 32 patients with Ewing's sarcoma revealed that 6 of 106 were significantly and independently associated with survival period. These observations suggest a functional role as well as prognostic value of these NPM complex proteins in Ewing's sarcoma. Further, our study suggests the potential applications of interactomics in conjunction with meta-analysis for biomarker discovery. © 2013 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  8. AIDS associated Kaposi's sarcoma.

    PubMed

    Thomas, Sumi; Sindhu, C B; Sreekumar, S; Sasidharan, P K

    2011-06-01

    Epidemic Kaposi's sarcoma (KS) is the most common malignant neoplasm occuring in HIV patients. Although the prevalence of HIV infection is high in Asian countries, KS is rarely reported. We report a case of fatal KS involving the skin and mucous membranes in a homosexual male.

  9. Myositis Ossificans Mimicking Sarcoma, the Importance of Diagnostic Imaging – Case Report

    PubMed Central

    Łuczyńska, Elżbieta; Kasperkiewicz, Hanna; Domalik, Agnieszka; Cwierz, Anna; Bobek-Billewicz, Barbara

    2014-01-01

    Summary Background Myositis ossificans is localized inflammatory process affecting skeletal muscles. Very rarely it can affect one of the neck muscles and present as a neck tumor, it can be misdiagnosed as the clinical, radiological and histological examinations can mimic a sarcoma. Case Report We report a 29 year old female patient with neck tumor suspected to be a sarcoma who underwent full diagnostics imaging and open bipsy with histopatological examination, afterwards surgical excision was performed. Conclusions The aim of this study was to present the differential diagnosis based on diagnostics imaging between MO and malignant tumors, such as parosteal sarcoma, synovial sarcoma and malignant fibrous histiocytoma. PMID:25077008

  10. The Scandinavian Sarcoma Group Central Register: 6,000 patients after 25 years of monitoring of referral and treatment of extremity and trunk wall soft-tissue sarcoma.

    PubMed

    Trovik, Clement; Bauer, Henrik C F; Styring, Emelie; Sundby Hall, Kirsten; Vult Von Steyern, Fredrik; Eriksson, Sigvard; Johansson, Ingela; Sampo, Mika; Laitinen, Minna; Kalén, Anders; Jónsson, Halldór; Jebsen, Nina; Eriksson, Mikael; Tukiainen, Erkki; Wall, Najme; Zaikova, Olga; Sigurðsson, Helgi; Lehtinen, Tuula; Bjerkehagen, Bodil; Skorpil, Mikael; Egil Eide, Geir; Johansson, Elisabeth; Alvegard, Thor A

    2017-06-01

    Purpose - We wanted to examine the potential of the Scandinavian Sarcoma Group (SSG) Central Register, and evaluate referral and treatment practice for soft-tissue sarcomas in the extremities and trunk wall (STS) in the Nordic countries. Background - Based on incidence rates from the literature, 8,150 (7,000-9,300) cases of STS of the extremity and trunk wall should have been diagnosed in Norway, Finland, Iceland, and Sweden from 1987 through 2011. The SSG Register has 6,027 cases registered from this period, with 5,837 having complete registration of key variables. 10 centers have been reporting to the Register. The 5 centers that consistently report treat approximately 90% of the cases in their respective regions. The remaining centers have reported all the patients who were treated during certain time periods, but not for the entire 25-year period. Results - 59% of patients were referred to a sarcoma center untouched, i.e. before any attempt at open biopsy. There was an improvement from 52% during the first 5 years to 70% during the last 5 years. 50% had wide or better margins at surgery. Wide margins are now achieved less often than 20 years ago, in parallel with an increase in the use of radiotherapy. For the centers that consistently report, 97% of surviving patients are followed for more than 4 years. Metastasis-free survival (MFS) increased from 67% to 73% during the 25-year period. Interpretation - The Register is considered to be representative of extremity and trunk wall sarcoma disease in the population of Scandinavia, treated at the reporting centers. There were no clinically significant differences in treatment results at these centers.

  11. The Scandinavian Sarcoma Group Central Register: 6,000 patients after 25 years of monitoring of referral and treatment of extremity and trunk wall soft-tissue sarcoma

    PubMed Central

    Trovik, Clement; Bauer, Henrik C F; Styring, Emelie; Sundby Hall, Kirsten; Vult Von Steyern, Fredrik; Eriksson, Sigvard; Johansson, Ingela; Sampo, Mika; Laitinen, Minna; Kalén, Anders; Jónsson, Halldór; Jebsen, Nina; Eriksson, Mikael; Tukiainen, Erkki; Wall, Najme; Zaikova, Olga; Sigurðsson, Helgi; Lehtinen, Tuula; Bjerkehagen, Bodil; Skorpil, Mikael; Egil Eide, Geir; Johansson, Elisabeth; Alvegard, Thor A

    2017-01-01

    Purpose We wanted to examine the potential of the Scandinavian Sarcoma Group (SSG) Central Register, and evaluate referral and treatment practice for soft-tissue sarcomas in the extremities and trunk wall (STS) in the Nordic countries. Background Based on incidence rates from the literature, 8,150 (7,000–9,300) cases of STS of the extremity and trunk wall should have been diagnosed in Norway, Finland, Iceland, and Sweden from 1987 through 2011. The SSG Register has 6,027 cases registered from this period, with 5,837 having complete registration of key variables. 10 centers have been reporting to the Register. The 5 centers that consistently report treat approximately 90% of the cases in their respective regions. The remaining centers have reported all the patients who were treated during certain time periods, but not for the entire 25-year period. Results 59% of patients were referred to a sarcoma center untouched, i.e. before any attempt at open biopsy. There was an improvement from 52% during the first 5 years to 70% during the last 5 years. 50% had wide or better margins at surgery. Wide margins are now achieved less often than 20 years ago, in parallel with an increase in the use of radiotherapy. For the centers that consistently report, 97% of surviving patients are followed for more than 4 years. Metastasis-free survival (MFS) increased from 67% to 73% during the 25-year period. Interpretation The Register is considered to be representative of extremity and trunk wall sarcoma disease in the population of Scandinavia, treated at the reporting centers. There were no clinically significant differences in treatment results at these centers. PMID:28266233

  12. Epidemiology and survivorship of soft tissue sarcomas in adults: a national cancer database reporta

    PubMed Central

    Corey, Robert M; Swett, Katrina; Ward, William G

    2014-01-01

    The National Cancer Data Base (NCDB) of the American College of Surgeons gather demographic and survival data on ∼70% of cancers in the USA. We wanted to investigate the demographic and survivorship data on this potentially more representative cohort of patients with soft tissue sarcomas. We selected 34 of the most commonly encountered soft tissue sarcomas reported to the NCDB, provided that each entity contained a minimum of 50 cases. This report summarizes the demographic and survivorship data on 63,714 patients with these 34 histologically distinct soft tissue sarcomas reported to the NCDB from 1998 to 2010. The overall survivorships of these sarcomas were near the lower limits of many prior reports due to the all-inclusive, minimally biased inclusion criteria. The overall best prognosis was Dermatofibrosarcoma NOS (not otherwise specified). (5-year survivorship 92%). The worst prognosis was Dedifferentiated Chondrosarcoma (5-year survivorship 19%). New observations included Biphasic Synovial Sarcoma demonstrating a better 5-year survivorship (65%) compared to spindle-cell synovial sarcoma (56%, P < 0.031) and Synovial Sarcoma, NOS (52%, P < 0.001). The demographic and 2- and 5-year survivorship data for all 34 soft tissue sarcomas are presented herein. This extent of demographic and survival data in soft tissue sarcomas is unprecedented. Because of the large number of cases and the inclusive nature of the NCDB, without restriction to certain stages, categories, or treatments, it is less subject to selection bias. Therefore, these data are thought to be more reflective of the true overall prognosis given the current management of sarcoma across the NCDB contributing sites. PMID:25044961

  13. Drugs Approved for Kaposi Sarcoma

    Cancer.gov

    This page lists cancer drugs approved by the Food and Drug Administration (FDA) for Kaposi sarcoma. The list includes generic names and brand names. The drug names link to NCI's Cancer Drug Information summaries.

  14. Assessing the reading level of online sarcoma patient education materials.

    PubMed

    Patel, Shaan S; Sheppard, Evan D; Siegel, Herrick J; Ponce, Brent A

    2015-01-01

    Cancer patients rely on patient education materials (PEMs) to gather information regarding their disease. Patients who are better informed about their illness have better health outcomes. The National Institutes of Health (NIH) recommends that PEMs be written at a sixth- to seventh-grade reading level. The purpose of this study was to evaluate the readability of online PEMs of bone and soft-tissue sarcomas and related conditions. We identified relevant online PEMs from the following websites: American Academy of Orthopaedic Surgeons, academic training centers, sarcoma specialists, Google search hits, Bonetumor.org, Sarcoma Alliance, Sarcoma Foundation of America, and Medscape. We used 10 different readability instruments to evaluate the reading level of each website's PEMs. In assessing 72 websites and 774 articles, we found that none of the websites had a mean readability score at or below 7 (seventh grade). Collectively, all websites had a mean readability score of 11.4, and the range of scores was grade level 8.9 to 15.5. None of the PEMs in this study of bone and soft-tissue sarcomas and related conditions met the NIH recommendation for PEM reading levels. Concerted efforts to improve the reading level of orthopedic oncologic PEMs are necessary.

  15. Ewing's Sarcoma as a Second Malignancy in Long-Term Survivors of Childhood Hematologic Malignancies.

    PubMed

    Wolpert, Fabian; Grotzer, Michael A; Niggli, Felix; Zimmermann, Dieter; Rushing, Elisabeth; Bode-Lesniewska, Beata

    2016-01-01

    Modern multimodal treatment has significantly increased survival for patients affected by hematologic malignancies, especially in childhood. Following remission, however, the risk of developing a further malignancy is an important issue. The long-term estimated risk of developing a sarcoma as a secondary malignancy is increased severalfold in comparison to the general population. Ewing's sarcoma family encompasses a group of highly aggressive, undifferentiated, intra- and extraosseous, mesenchymal tumors, caused by several types of translocations usually involving the EWSR1 gene. Translocation associated sarcomas, such as Ewing sarcoma, are only rarely encountered as therapy associated secondary tumors. We describe the clinical course and management of three patients from a single institution with Ewing's sarcoma that followed successfully treated lymphoblastic T-cell leukemia or non-Hodgkin lymphoma. The literature on secondary Ewing's sarcoma is summarized and possible pathogenic mechanisms are critically discussed.

  16. Ribociclib and Doxorubicin in Treating Patients With Metastatic or Advanced Soft Tissue Sarcomas That Cannot Be Removed by Surgery

    ClinicalTrials.gov

    2018-05-09

    Metastatic Angiosarcoma; Metastatic Epithelioid Sarcoma; Metastatic Fibrosarcoma; Metastatic Leiomyosarcoma; Metastatic Liposarcoma; Metastatic Malignant Peripheral Nerve Sheath Tumor; Metastatic Synovial Sarcoma; Metastatic Undifferentiated Pleomorphic Sarcoma; Myxofibrosarcoma; Pleomorphic Rhabdomyosarcoma; Stage III Soft Tissue Sarcoma; Stage IV Soft Tissue Sarcoma; Undifferentiated (Embryonal) Sarcoma

  17. Poppers, Kaposi's sarcoma, and HIV infection: empirical example of a strong confounding effect?

    PubMed

    Morabia, A

    1995-01-01

    Are there empirical examples of strong confounding effects? Textbooks usually show examples of weak confounding or use hypothetical examples of strong confounding to illustrate the paradoxical consequences of not separating out the effect of the studied exposure from that of second factor acting as a confounder. HIV infection is a candidate strong confounder of the spuriously high association reported between consumption of poppers, a sexual stimulant, and risk of Kaposi's sarcoma in the early phase of the AIDS epidemic. To examine this hypothesis, assumptions must be made on the prevalence of HIV infection among cases of Kaposi's sarcoma and on the prevalence of heavy popper consumption according to HIV infection in cases and controls. Results show that HIV infection may have confounded the poppers-Kaposi's sarcoma association. However, it cannot be ruled out that HIV did not qualify as a confounder because it was either an intermediate variable or an effect modifier of the association between popper inhalation and Kaposi's sarcoma. This example provides a basis to discuss the mechanism by which confounding occurs as well as the practical importance of confounding in epidemiologic research.

  18. Diagnostic value of MRI signs in differentiating Ewing sarcoma from osteomyelitis.

    PubMed

    Kasalak, Ömer; Overbosch, Jelle; Adams, Hugo Ja; Dammann, Amelie; Dierckx, Rudi Ajo; Jutte, Paul C; Kwee, Thomas C

    2018-01-01

    Background The value of magnetic resonance imaging (MRI) signs in differentiating Ewing sarcoma from osteomyelitis has not be thoroughly investigated. Purpose To investigate the value of various MRI signs in differentiating Ewing sarcoma from osteomyelitis. Material and Methods Forty-one patients who underwent MRI because of a bone lesion of unknown nature with a differential diagnosis that included both Ewing sarcoma and osteomyelitis were included. Two observers assessed several MRI signs, including the transition zone of the bone lesion, the presence of a soft-tissue mass, intramedullary and extramedullary fat globules, and the penumbra sign. Results Diagnostic accuracies for discriminating Ewing sarcoma from osteomyelitis were 82.4% and 79.4% for the presence of a soft-tissue mass, and 64.7% and 58.8% for a sharp transition zone of the bone lesion, for readers 1 and 2 respectively. Inter-observer agreement with regard to the presence of a soft-tissue mass and the transition zone of the bone lesion were moderate (κ = 0.470) and fair (κ = 0.307), respectively. Areas under the receiver operating characteristic curve of the diameter of the soft-tissue mass (if present) were 0.829 and 0.833, for readers 1 and 2 respectively. Mean inter-observer difference in soft-tissue mass diameter measurement ± limits of agreement was 35.0 ± 75.0 mm. Diagnostic accuracies of all other MRI signs were all < 50%. Conclusion Presence and size of a soft-tissue mass, and sharpness of the transition zone, are useful MRI signs to differentiate Ewing sarcoma from osteomyelitis, but inter-observer agreement is relatively low. Other MRI signs are of no value in this setting.

  19. [Multivisceral organ procurement for transplantation derived mobilization maneouvres: very helpful auxiliary techniques in the excision of large retroperitoneal masses].

    PubMed

    González, Javier; Shirodkar, S P; Ciancio, G

    2011-04-01

    The excision of large retroperitoneal masses poses a challenge for every surgeon. Sometimes the urologist must face situations that do not fit to any conventional approach or technique previously described. Obtaining adequate exposure for safe and oncologically correct management of these masses is based, on many cases, in the mobilization of anatomical adjacent structures to generate a sufficient field in abdominal areas of difficult access. Complex visceral mobilization maneuvers derived from multivisceral transplantation organ procurement surgery provides ancillary techniques that used properly facilitate their successful resolution. The main purpose of this paper is the description of these surgical maneuvers essential to increase both exposure and vascular control in addressing the ever-dreaded high-volume retroperitoneal masses.

  20. The importance of being dead: cell death mechanisms assessment in anti-sarcoma therapy.

    PubMed

    Rello-Varona, Santiago; Herrero-Martín, David; Lagares-Tena, Laura; López-Alemany, Roser; Mulet-Margalef, Núria; Huertas-Martínez, Juan; Garcia-Monclús, Silvia; García Del Muro, Xavier; Muñoz-Pinedo, Cristina; Tirado, Oscar Martínez

    2015-01-01

    Cell death can occur through different mechanisms, defined by their nature and physiological implications. Correct assessment of cell death is crucial for cancer therapy success. Sarcomas are a large and diverse group of neoplasias from mesenchymal origin. Among cell death types, apoptosis is by far the most studied in sarcomas. Albeit very promising in other fields, regulated necrosis and other cell death circumstances (as so-called "autophagic cell death" or "mitotic catastrophe") have not been yet properly addressed in sarcomas. Cell death is usually quantified in sarcomas by unspecific assays and in most cases the precise sequence of events remains poorly characterized. In this review, our main objective is to put into context the most recent sarcoma cell death findings in the more general landscape of different cell death modalities.

  1. Dedifferentiated liposarcoma of retroperitoneum: spectrum of imaging findings in 15 patients.

    PubMed

    Hong, Sun Hwa; Kim, Kyeong Ah; Woo, Ok Hee; Park, Cheol Min; Kim, Chul Hwan; Kim, Myeong-Jin; Chung, Jae-Joon; Han, Joon Koo; Rha, Sung Eun

    2010-01-01

    Dedifferentiated liposarcoma is a high-grade nonlipogenic sarcoma that arises in the background of a preexisting well-differentiated liposarcoma. The purpose of this work is to evaluate the spectrum of radiologic appearance of retroperitoneal dedifferentiated liposarcoma with clinicopathologic features. Radiologic images and clinical histories of 15 patients with histologically verified retroperitoneal dedifferentiated liposarcoma were retrospectively studied. Patients included 11 men and 4 women, with mean age of 55.8 years (range, 36-74 years), and they underwent computed tomography (CT) (n=15) and magnetic resonance imaging (MRI) (n=5). The mean size of the tumor was 22.6 cm with a range of 9.5-38 cm. Both CT and MRI showed well-circumscribed, large round, or lobulated retroperitoneal mass. The appearance of the tumor was classified as follows: Category I, nonfatty component within predominant fatty mass (n=5); Category II, focal fatty component within large nonfatty mass (n=6); Category III, well-defined fatty mass and well-defined nonfatty mass (n=1); Category IV, two masses with predominantly nonfatty component (n=3). Retroperitoneal dedifferentiated liposarcomas present various spectra of imaging findings. While Categories I and III may suggest dedifferentiated liposarcoma, Categories II and IV may appear as other types of liposarcoma or other malignant retroperitoneal tumor.

  2. Childhood Soft Tissue Sarcoma Treatment (PDQ®)—Health Professional Version

    Cancer.gov

    Pediatric soft tissue sarcomas are a heterogenous group of malignant tumors that originate from primitive mesenchymal tissue and account for 7% of all childhood tumors. Get detailed information about clinical presentation, diagnosis, prognosis, and treatment of newly diagnosed and recurrent soft tissue sarcoma in this summary for clinicians.

  3. PML expression in soft tissue sarcoma: prognostic and predictive value in alkylating agents/antracycline-based first line therapy.

    PubMed

    Vincenzi, Bruno; Santini, Daniele; Schiavon, Gaia; Frezza, Anna Maria; Silletta, Marianna; Crucitti, Pierfilippo; Casali, Paolo; Dei Tos, Angelo P; Rossi, Sabrina; Rizzo, Sergio; Badalamenti, Giuseppe; Tomasino, Rosa Maria; Russo, Antonio; Butrynski, James E; Tonini, Giuseppe

    2012-04-01

    Soft tissue sarcomas are aggressive tumors representing <1% of all adult neoplasms. Aim of our study was to evaluate promyelocytic leukemia gene expression value as prognostic factor and as a factor predicting response to alkylating agents/antracycline-based first line therapy. One hundred eleven patients affected by locally advanced and metastatic soft tissue sarcoma were selected. PML expression was evaluated by immunohistochemical analysis in pathological samples and in the corresponding normal tissue from each case. PML immunohistochemical results were correlated with prognosis and with radiological response to alkylating agents/antracycline-based first line therapy. PML expression was significantly reduced in synovial sarcomas (P < 0.0001), in myofibroblastic sarcomas (P < 0.0001), angiosarcomas (P < 0.0001), in leiomyosarcomas (P = 0.003), in mixoid liposarcomas (P < 0.0001), and in dedifferentiated liposarcomas (P < 0.0001). No significant difference was found for pleomorphic sarcoma [31.8 (95% CI: 16.7-41.0); P = 0.21]. and pleomorphic liposarcomas (P = 0.51). Loss of PML expression was found to be statistically correlated with TTP (P < 0.0001), median duration of response (P = 0.007), and OS (P = 0.02). No correlation was observed between PML expression and treatment efficacy. PML IHC expression is down-regulated in synovial sarcomas, myofibroblastic sarcomas, angiosarcomas, liposarcoma, and leiomyosarcomas and its expression correlated with prognosis. Copyright © 2011 Wiley Periodicals, Inc.

  4. Prospective, randomized comparison of transperitoneal versus retroperitoneal laparoscopic adrenalectomy.

    PubMed

    Rubinstein, Mauricio; Gill, Inderbir S; Aron, Monish; Kilciler, Mete; Meraney, Anoop M; Finelli, Antonio; Moinzadeh, Ali; Ukimura, Osamu; Desai, Mihir M; Kaouk, Jihad; Bravo, Emmanuel

    2005-08-01

    We report a prospective, randomized comparison of transperitoneal laparoscopic adrenalectomy (TLA) vs retroperitoneal laparoscopic adrenalectomy (RLA) for adrenal lesions with long-term followup. Between December 1997 and November 1999, 57 consecutive eligible patients with surgical adrenal disease were prospectively randomized to undergo TLA (25) or RLA (32). Study exclusion criteria were patient age greater than 80 years, body mass index greater than 40, bilateral adrenalectomy and significant prior abdominal surgery in the quadrant of interest. Mean followup was 5.96 years in the 2 groups. The groups were matched in regard to patient age (p = 0.84), body mass index (p = 0.43), American Society of Anesthesiologists class (p = 0.81) and laterality (p = 0.12). Median adrenal mass size was 2.7 cm (range 1 to 9) in the TLA group and 2.6 cm (range 0.5 to 6) in the RLA group (p = 0.83). TLA was comparable to RLA in terms of operative time (130 vs 126.5 minutes, p = 0.64), estimated blood loss (p = 0.92), specimen weight (p = 0.81), analgesic requirements (p = 0.25), hospital stay (p = 0.56) and the complication rate (p = 0.58). One case per group was electively converted to open surgery. Pathology data on the intact extracted specimens were similar between the groups. Averaged convalescence was 4.7 weeks in the TLA group and 2.3 weeks in the RLA group (p = 0.02). During a mean followup of 6 years 2 patients in the TLA group had a late complication (port site hernia). Mortality occurred in 5 patients, including 1 with TLA and 4 with RLA, during the 6-year followup. For most benign adrenal lesions requiring surgery laparoscopic adrenalectomy can be performed safely and effectively by the transperitoneal or the retroperitoneal approach.

  5. EWS-FLI1 increases transcription to cause R-loops and block BRCA1 repair in Ewing sarcoma.

    PubMed

    Gorthi, Aparna; Romero, July Carolina; Loranc, Eva; Cao, Lin; Lawrence, Liesl A; Goodale, Elicia; Iniguez, Amanda Balboni; Bernard, Xavier; Masamsetti, V Pragathi; Roston, Sydney; Lawlor, Elizabeth R; Toretsky, Jeffrey A; Stegmaier, Kimberly; Lessnick, Stephen L; Chen, Yidong; Bishop, Alexander J R

    2018-03-15

    Ewing sarcoma is an aggressive paediatric cancer of the bone and soft tissue. It results from a chromosomal translocation, predominantly t(11;22)(q24:q12), that fuses the N-terminal transactivation domain of the constitutively expressed EWSR1 protein with the C-terminal DNA binding domain of the rarely expressed FLI1 protein. Ewing sarcoma is highly sensitive to genotoxic agents such as etoposide, but the underlying molecular basis of this sensitivity is unclear. Here we show that Ewing sarcoma cells display alterations in regulation of damage-induced transcription, accumulation of R-loops and increased replication stress. In addition, homologous recombination is impaired in Ewing sarcoma owing to an enriched interaction between BRCA1 and the elongating transcription machinery. Finally, we uncover a role for EWSR1 in the transcriptional response to damage, suppressing R-loops and promoting homologous recombination. Our findings improve the current understanding of EWSR1 function, elucidate the mechanistic basis of the sensitivity of Ewing sarcoma to chemotherapy (including PARP1 inhibitors) and highlight a class of BRCA-deficient-like tumours.

  6. Ewing sarcoma of the rib with normal blood flow and blood pool imagings on a 3-phase bone scan.

    PubMed

    Alfeeli, Mahmoud A; Naddaf, Sleiman Y; Syed, Ghulam M S

    2005-09-01

    Ewing sarcoma is the second most common pediatric malignant bone tumor. It usually presents as a hot spot on a 3-phase bone scan as a result of increased vascularity of the tumor and new bone formation. However, aggressive Ewing sarcoma can also appear as a cold lesion. We present the features of a Ewing sarcoma of the rib on a 3-phase bone scan in a child who was being investigated for rib fracture after trauma.

  7. Gene Expression Profiling of Histiocytic Sarcomas in a Canine Model: The Predisposed Flatcoated Retriever Dog

    PubMed Central

    Boerkamp, Kim M.; van Wolferen, Monique E.; Groot Koerkamp, Marian J. A.; van Leenen, Dik; Grinwis, Guy C. M.; Penning, Louis C.; Wiemer, Erik A. C.; Rutteman, Gerard R.

    2013-01-01

    Background The determination of altered expression of genes in specific tumor types and their effect upon cellular processes may create insight in tumorigenesis and help to design better treatments. The Flatcoated retriever is a dog breed with an exceptionally high incidence of histiocytic sarcomas. The breed develops two distinct entities of histiocytic neoplasia, a soft tissue form and a visceral form. Gene expression studies of these tumors have value for comparable human diseases such as histiocytic/dendritic cell sarcoma for which knowledge is difficult to accrue due to their rare occurrence. In addition, such studies may help in the search for genetic aberrations underlying the genetic predisposition in this dog breed. Methods Microarray analysis and pathway analyses were performed on fresh-frozen tissues obtained from Flatcoated retrievers with localized, soft tissue histiocytic sarcomas (STHS) and disseminated, visceral histiocytic sarcomas (VHS) and on normal canine spleens from various breeds. Expression differences of nine genes were validated with quantitative real-time PCR (qPCR) analyses. Results QPCR analyses identified the significantly altered expression of nine genes; PPBP, SpiC, VCAM1, ENPEP, ITGAD (down-regulated), and GTSF1, Col3a1, CD90 and LUM (up-regulated) in the comparison of both the soft tissue and the visceral form with healthy spleen. DAVID pathway analyses revealed 24 pathways that were significantly involved in the development of HS in general, most of which were involved in the DNA repair and replication process. Conclusions This study identified altered expression of nine genes not yet implicated in histiocytic sarcoma manifestations in the dog nor in comparable human histiocytic/dendritic sarcomas. Exploration of the downside effect of canine inbreeding strategies for the study of similar sarcomas in humans might also lead to the identification of genes related to these rare malignancies in the human. PMID:23936488

  8. The First European Interdisciplinary Ewing Sarcoma Research Summit

    PubMed Central

    Kovar, Heinrich; Alonso, Javier; Aman, Pierre; Aryee, Dave N. T.; Ban, Jozef; Burchill, Sue A.; Burdach, Stefan; De Alava, Enrique; Delattre, Olivier; Dirksen, Uta; Fourtouna, Argyro; Fulda, Simone; Helman, Lee J.; Herrero-Martin, David; Hogendoorn, Pancras C. W.; Kontny, Udo; Lawlor, Elizabeth R.; Lessnick, Stephen L.; Llombart-Bosch, Antonio; Metzler, Markus; Moriggl, Richard; Niedan, Stephan; Potratz, Jenny; Redini, Françoise; Richter, Günther H. S.; Riedmann, Lucia T.; Rossig, Claudia; Schäfer, Beat W.; Schwentner, Raphaela; Scotlandi, Katia; Sorensen, Poul H.; Staege, Martin S.; Tirode, Franck; Toretsky, Jeffrey; Ventura, Selena; Eggert, Angelika; Ladenstein, Ruth

    2012-01-01

    The European Network for Cancer Research in Children and Adolescents (ENCCA) provides an interaction platform for stakeholders in research and care of children with cancer. Among ENCCA objectives is the establishment of biology-based prioritization mechanisms for the selection of innovative targets, drugs, and prognostic markers for validation in clinical trials. Specifically for sarcomas, there is a burning need for novel treatment options, since current chemotherapeutic treatment protocols have met their limits. This is most obvious for metastatic Ewing sarcoma (ES), where long term survival rates are still below 20%. Despite significant progress in our understanding of ES biology, clinical translation of promising laboratory results has not yet taken place due to fragmentation of research and lack of an institutionalized discussion forum. To fill this gap, ENCCA assembled 30 European expert scientists and five North American opinion leaders in December 2011 to exchange thoughts and discuss the state of the art in ES research and latest results from the bench, and to propose biological studies and novel promising therapeutics for the upcoming European EWING2008 and EWING2012 clinical trials. PMID:22662320

  9. The first European interdisciplinary ewing sarcoma research summit.

    PubMed

    Kovar, Heinrich; Alonso, Javier; Aman, Pierre; Aryee, Dave N T; Ban, Jozef; Burchill, Sue A; Burdach, Stefan; De Alava, Enrique; Delattre, Olivier; Dirksen, Uta; Fourtouna, Argyro; Fulda, Simone; Helman, Lee J; Herrero-Martin, David; Hogendoorn, Pancras C W; Kontny, Udo; Lawlor, Elizabeth R; Lessnick, Stephen L; Llombart-Bosch, Antonio; Metzler, Markus; Moriggl, Richard; Niedan, Stephan; Potratz, Jenny; Redini, Françoise; Richter, Günther H S; Riedmann, Lucia T; Rossig, Claudia; Schäfer, Beat W; Schwentner, Raphaela; Scotlandi, Katia; Sorensen, Poul H; Staege, Martin S; Tirode, Franck; Toretsky, Jeffrey; Ventura, Selena; Eggert, Angelika; Ladenstein, Ruth

    2012-01-01

    The European Network for Cancer Research in Children and Adolescents (ENCCA) provides an interaction platform for stakeholders in research and care of children with cancer. Among ENCCA objectives is the establishment of biology-based prioritization mechanisms for the selection of innovative targets, drugs, and prognostic markers for validation in clinical trials. Specifically for sarcomas, there is a burning need for novel treatment options, since current chemotherapeutic treatment protocols have met their limits. This is most obvious for metastatic Ewing sarcoma (ES), where long term survival rates are still below 20%. Despite significant progress in our understanding of ES biology, clinical translation of promising laboratory results has not yet taken place due to fragmentation of research and lack of an institutionalized discussion forum. To fill this gap, ENCCA assembled 30 European expert scientists and five North American opinion leaders in December 2011 to exchange thoughts and discuss the state of the art in ES research and latest results from the bench, and to propose biological studies and novel promising therapeutics for the upcoming European EWING2008 and EWING2012 clinical trials.

  10. Gynecological sarcomas: what's new in 2018, a brief review of published literature.

    PubMed

    Gantzer, Justine; Ray-Coquard, Isabelle

    2018-05-26

    In this article, we focus on recent published data (2017) on the management of gynecologic sarcomas. The most significant data published in 2017 develop definition of a new molecular subtype of high grade endometrial stromal sarcoma (ESS) using molecular technics added to histological analysis. The identification of a new translocation on presumed uterine leiomyosarcoma (LMS) points to refinement of nosological classification, with fragmentation of even rare tumors into distinct molecular entities: gynecologic sarcomas are now distinguished into distinct entities from a heterogeneous group of tumors. Other articles have discussed the real incidence of unsuspected sarcomas after fibroid mini-invasive surgery and evaluate the risk of relapse and dissemination after morcellation. Among several criteria, preoperative imagery could become a useful tool. For systemic treatment, no clinical trials changing practices were published, only one positive nonrandomized phase II with carboplatin and pegylated liposomal doxorubicin (PLD) in the treatment of uterine sarcomas after the conventional first line, especially in LMSs and ESSs. Many articles were published on this confidential domain in oncology demonstrating interests on rare sarcomas. All specialties were represented in the literature, even though we are still waiting for urgent improvements in early diagnosis and therapeutic strategies to transform the poor prognostic of these tumors.

  11. Cabozantinib-S-Malate in Treating Younger Patients With Recurrent, Refractory, or Newly Diagnosed Sarcomas, Wilms Tumor, or Other Rare Tumors

    ClinicalTrials.gov

    2018-06-25

    Adrenal Cortex Carcinoma; Adult Alveolar Soft Part Sarcoma; Adult Clear Cell Sarcoma of Soft Parts; Adult Hepatocellular Carcinoma; Adult Rhabdomyosarcoma; Adult Soft Tissue Sarcoma; Childhood Alveolar Soft Part Sarcoma; Childhood Central Nervous System Neoplasm; Childhood Clear Cell Sarcoma of Soft Parts; Childhood Hepatocellular Carcinoma; Childhood Rhabdomyosarcoma; Childhood Soft Tissue Sarcoma; Childhood Solid Neoplasm; Ewing Sarcoma; Hepatoblastoma; Hepatocellular Carcinoma; Recurrent Adrenal Cortex Carcinoma; Recurrent Adult Hepatocellular Carcinoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Alveolar Soft Part Sarcoma; Recurrent Childhood Central Nervous System Neoplasm; Recurrent Childhood Hepatocellular Carcinoma; Recurrent Childhood Soft Tissue Sarcoma; Recurrent Ewing Sarcoma; Recurrent Hepatoblastoma; Recurrent Malignant Solid Neoplasm; Recurrent Osteosarcoma; Recurrent Renal Cell Carcinoma; Recurrent Rhabdomyosarcoma; Refractory Osteosarcoma; Renal Cell Carcinoma; Thyroid Gland Medullary Carcinoma; Wilms Tumor

  12. Geographic and birth cohort associations of Kaposi's sarcoma among homosexual men in Canada.

    PubMed

    Schechter, M T; Marion, S A; Elmslie, K D; Ricketts, M N; Nault, P; Archibald, C P

    1991-09-01

    The authors conducted an analysis of all 677 cases of Kaposi's sarcoma among the 3,047 cases of acquired immunodeficiency syndrome diagnosed in homosexual/bisexual men in Canada between 1980 and 1989. The proportion with Kaposi's sarcoma declined from 32.2% during 1980-1985 to 15.0% in 1989. The proportion with Kaposi's sarcoma was significantly higher in primary epidemic centers (Vancouver, Toronto, and Montreal) and in men in the 1945-1954 birth cohort independent of year of diagnosis. These data are consistent with an environmental cofactor for Kaposi's sarcoma which is likely to be a sexually transmitted agent.

  13. Conservative Treatment of Ewing's Sarcoma of the Uterus in Young Women.

    PubMed

    Loverro, Giuseppe; Resta, Leonardo; Di Naro, Edoardo; Caringella, Anna Maria; Mastrolia, Salvatore Andrea; Vicino, Mario; Tartagni, Massimo; Schonauer, Luca Maria

    2015-01-01

    Ewing sarcoma-primitive neuroectodermal tumors (ES/PNETs) constitute a family of neoplasms characterized by a continuum of neuroectodermal differentiations. ES/PNET of the uterus is rare. There are 48 cases of ES/PNET of the uterus published in the literature as far as we know. We describe a case of Ewing sarcoma of the uterus occurring in a 17-year-old woman presenting with a two-month history of pelvic pain. After surgical excision and microscopic, immunohistochemical, and electron microscopy examination, the diagnosis of Ewing sarcoma of the uterus was suggested. This report will discuss the diagnosis and surgical and clinical management of Ewing uterine sarcoma in young women, according to the available literature. In spite of the rarity of ES/PNETs, they should be taken into account in the differential diagnosis of uterine neoplasms in young women.

  14. Risk Factors Associated With Secondary Sarcomas in Childhood Cancer Survivors: A Report From the Childhood Cancer Survivor Study

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Henderson, Tara O., E-mail: thenderson@peds.bsd.uchicago.edu; Rajaraman, Preetha; Stovall, Marilyn

    Purpose: Childhood cancer survivors have an increased risk of secondary sarcomas. To better identify those at risk, the relationship between therapeutic dose of chemotherapy and radiation and secondary sarcoma should be quantified. Methods and Materials: We conducted a nested case-control study of secondary sarcomas (105 cases, 422 matched controls) in a cohort of 14,372 childhood cancer survivors. Radiation dose at the second malignant neoplasm (SMN) site and use of chemotherapy were estimated from detailed review of medical records. Odds ratios (ORs) and 95% confidence intervals were estimated by conditional logistic regression. Excess odds ratio (EOR) was modeled as a functionmore » of radiation dose, chemotherapy, and host factors. Results: Sarcomas occurred a median of 11.8 years (range, 5.3-31.3 years) from original diagnosis. Any exposure to radiation was associated with increased risk of secondary sarcoma (OR = 4.1, 95% CI = 1.8-9.5). A dose-response relation was observed, with elevated risks at doses between 10 and 29.9 Gy (OR = 15.6, 95% CI = 4.5-53.9), 30-49.9 Gy (OR = 16.0, 95% CI 3.8-67.8) and >50 Gy (OR = 114.1, 95% CI 13.5-964.8). Anthracycline exposure was associated with sarcoma risk (OR = 3.5, 95% CI = 1.6-7.7) adjusting for radiation dose, other chemotherapy, and primary cancer. Adjusting for treatment, survivors with a first diagnosis of Hodgkin lymphoma (OR = 10.7, 95% CI = 3.1-37.4) or primary sarcoma (OR = 8.4, 95% CI = 3.2-22.3) were more likely to develop a sarcoma. Conclusions: Of the risk factors evaluated, radiation exposure was the most important for secondary sarcoma development in childhood cancer survivors; anthracycline chemotherapy exposure was also associated with increased risk.« less

  15. Patients with Advanced, Rare Sarcoma Respond to Cediranib | Center for Cancer Research

    Cancer.gov

    Alveolar soft part sarcomas (ASPS) are highly vascular tumors that usually affect adolescents and young adults. Comprising less than one percent of soft tissue sarcomas, ASPS can be cured with surgery. However, its tendency to metastasize and its lack of response to standard soft tissue sarcoma chemotherapy regimens makes ASPS a particularly lethal cancer with a five-year survival rate of 20 percent in patients with metastatic disease who are not candidates for surgery.

  16. The progression of CD56+ myeloid sarcoma: A case report and literature review

    PubMed Central

    WANG, XIN; LI, WEN-SHENG; ZHENG, YAN; YING, ZHAO-XIA; WANG, YONG-XIAN; WANG, YING-MEI; ZHENG, JUN-FENG; XIAO, SHENG-XIANG

    2016-01-01

    The current study presents a case of cluster of differentiation (CD)56+ myeloid sarcoma in a patient that initially presented with skin lesions, and provides evidence for the clinical and differential diagnosis of myeloid sarcoma. The patient of the present case report was a 65-year-old man who was admitted to hospital with a six-month history of bilateral purple-red papules and nodules, which were present on the upper limbs of the patient and had spread over his whole body one month prior to admission to the hospital. Pathological examination demonstrated a diffuse infusion of primitive round cells at the papillary dermis and subcutaneous tissues. The infiltrated cells were 40–60 µm in diameter and morphologically identical. Immunohistochemical examination revealed that the cells expressed myeloperoxidase, CD56, CD43 and T-cell intracytoplasmic antigen. In addition, several cells expressed CD34, and 90% of the cells expressed Ki67. While the majority of cells in myeloid sarcoma do not express CD56, the present case was a myeloid sarcoma that expressed CD56, which is extremely rare. The sarcoma in the present patient progressed rapidly, and the patient died eight months following the onset of disease. Clinicians should be aware of CD56+ myeloid sarcoma, which is easily misdiagnosed and inappropriately treated. Consequently, myeloid sarcoma may have a high malignancy and poor outcome for patients. PMID:27123069

  17. Treatment Option Overview (Uterine Sarcoma)

    MedlinePlus

    ... stage of the cancer being treated. External and internal radiation therapy are used to treat uterine sarcoma, and may also be used as palliative therapy to relieve symptoms and improve quality of life . Chemotherapy Chemotherapy is a cancer treatment ...

  18. Diagnostic Accuracy of Selective 3-T MR Neurography-guided Retroperitoneal Genitofemoral Nerve Blocks for the Diagnosis of Genitofemoral Neuralgia.

    PubMed

    Fritz, Jan; Dellon, A Lee; Williams, Eric H; Rosson, Gedge D; Belzberg, Allan J; Eckhauser, Frederick E

    2017-10-01

    Purpose To determine if 3-T magnetic resonance (MR) neurography-guided retroperitoneal genitofemoral nerve (GFN) blocks are safe and effective for the diagnosis of genitofemoral neuralgia. Materials and Methods Following institutional review board approval and informed consent, 26 subjects (16 men, 10 women; mean age, 42 years [range, 24-78 years]; mean body mass index, 28 kg/m 2 [range, 20-35 kg/m 2 ]) with intractable groin pain were included. By using a 3-T MR imaging system, intermediate-weighted turbo spin-echo pulse sequences, and MR-conditional needles, diagnostic MR neurography-guided GFN blocks were performed in the retroperitoneum. Outcome variables included technical success, procedure time, complications, and rates of positive and negative GFN blocks in association with therapeutic outcomes. For the assessment of a learning curve, Mann-Whitney test was used. P values ≤ .05 were considered to indicate a statistically significant difference. Results In 26 subjects, 30 retroperitoneal GFN blocks were performed. Twelve (40%) were performed with an anterior needle path, 12 (40%) with a lateral needle path, and six (20%) with a posterior needle path. GFN blocks were technically successful in 24 of 26 (92%) subjects, achieving appropriate scrotal anesthesia. No complications occurred. The time required for a GFN block was 40 minutes (range, 18-67 minutes). The rate of a successful GFN intervention after a positive GFN block was 88% (14 of 16). The rate of a successful intervention of an alternative target after a negative GFN block was 71% (five of seven). Conclusion Selective retroperitoneally directed MR neurography-guided GFN blocks are safe and effective with high technical success and positive effect on surgical decision making in patients with presumed genitofemoral neuralgia. © RSNA, 2017 Online supplemental material is available for this article.

  19. Retroperitoneal laparoendoscopic single-site ureterolithotomy and ureteroureterostomy for retrocaval ureter with ureteral calculus: first case report.

    PubMed

    Chen, Zhi; Chen, Xiang; Luo, Yan-Cheng; He, Yao; Li, Nan-Nan; Xie, Chao-Qun; Lai, Chen; Fang, Xiao-Long

    2012-12-01

    A 53-year-old man presented with a 6-month history of intermittent right flank pain. Radiological imaging confirmed the diagnosis of retrocaval ureter (RCU) and ureteral calculus. Retroperitoneal laparoendoscopic single-site surgery (LESS) ureterolithotomy and ureteroureterostomy was successfully performed. The operative time was 185 min and the blood loss was approximately 20 ml. The patient's postoperative course was uneventful. Postoperative analgesia was not needed. The patient was discharged on the third postoperative day. The drain and double-J stent were respectively removed at 1 and 8 weeks postoperatively. At the 3-month follow-up, nuclear scan showed no evidence of obstruction of the right kidney and the patient also remained symptom free. It may be concluded that retroperitoneal LESS repair for RCU is a feasible and safe procedure, which can be considered as a option for the management of RCU even if it is complicated by the presence of a ureteral calculus.

  20. Therapeutic Trial for Patients With Ewing Sarcoma Family of Tumor and Desmoplastic Small Round Cell Tumors

    ClinicalTrials.gov

    2017-09-18

    Desmoplastic Small Round Cell Tumor; Ewing Sarcoma of Bone or Soft Tissue; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  1. [Ewing sarcoma located in the mandible: A case report].

    PubMed

    Hernandez, M; Droz, D; Mansuy, L; Simon, E; Chastagner, P

    2015-06-01

    Ewing sarcoma is the second most common primary malignant bone cancer in children and adolescents. Clinical presentation is usually dominated by local pain and a palpable mass. These symptoms justify imaging investigations: the first one, when an osseous lesion is suspected, is usually a conventional radiograph in two planes. Ewing sarcoma appears as a poorly defined osteolytic lesion that may frequently be associated with cortical erosion or laminar periosteal response ("onion skin"). However, this aspect is not pathognomonic and the definitive diagnosis is made by biopsy. Absence of pain or an unusual localization can lead to misdiagnosis. We report the case of a 7-year-old boy with Ewing sarcoma located in the mandible with a clinical picture including progressive mandibular swelling but no pain. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  2. Epidemiological Evaluation of Head and Neck Sarcomas in Iran (the Study of 105 Cases Over 13 Years).

    PubMed

    Alishahi, Batoul; Kargahi, Neda; Homayouni, Solmaz

    2015-08-01

    Head and neck sarcomas are exceedingly rare and they include 4% - 10% of all sarcomas and less than 1% of all neoplasm of head and neck. The aim of this study is to evaluate the epidemiological characteristics of head and neck sarcomas of patients in Isfahan, Iran. In this retrospective study, from the 16000 patients whose files were evaluated, the total number of 105 head and neck sarcomas were collected. They were evaluated with due attention to age, gender of the patients and the most common location of the lesion. From the total number of 105 (0.6%) patients with sarcomas, 56 were men (53.33%) and 49 women (46.66%). The most common head and neck sarcomas among this population were Osteosarcoma (32 cases, 30.47%), Chondrosarcoma (14 cases, 13.33%), and Ewing sarcoma (11 cases, 10.47%).The most common soft tissue sarcoma was Rabdomiosarcoma. Mandible was the most common location for these lesions. In this study, the hard tissue sarcomas were more prevalent than soft tissue ones. Hence, special attention should be paid to the patients when being diagnosed.

  3. Primary Pulmonary Ewing's Sarcoma: Rare Cause of Superior Vena Cava Syndrome in Children.

    PubMed

    Mehra, Shibani; Atwal, Swapndeep Singh; Garga, Umesh Chandra

    2014-08-01

    Ewing's sarcoma is a common malignant bone tumour presenting in children and young adults. Rarely extra- skeletal soft tissues and visceral organs can also be the site of origin of Ewing's sarcoma. Primary pulmonary Ewing's sarcoma is an extremely rare malignancy which occurs in the paediatric population. We report an unusual case of primary pulmonary Ewing's sarcoma in a nine year old girl who presented with features of superior vena cava syndrome in the emergency department. The diagnosis was confirmed pathologically both by light microscopy and immunohistochemistry. The patient was put on chemotherapy and surgery was planned but the patient expired within three days of starting chemotherapy.

  4. Percutaneous Hepatic Perfusion with Melphalan for Unresectable Metastatic Melanoma or Sarcoma to the Liver: A Single Institution Experience

    PubMed Central

    Forster, Meghan R.; Rashid, Omar M.; Perez, Matthew; Choi, Junsung; Chaudhry, Tariq; Zager, Jonathan S.

    2015-01-01

    Background Patients with unresectable melanoma or sarcoma hepatic metastasis have a poor prognosis with few therapeutic options. Percutaneous hepatic perfusion (PHP), isolating and perfusing the liver with chemotherapy, provides a promising minimally invasive management option. We reviewed our institutional experience with PHP. Methods We retrospectively reviewed patients with unresectable melanoma or sarcoma hepatic metastasis treated with PHP from 2008 to 2013 and evaluated therapeutic response, morbidity, hepatic progression free survival (hPFS), and overall survival (OS). Results Ten patients were treated with 27 PHPs (median 3). Diagnoses were ocular melanoma (n=5), cutaneous melanoma (n=3), unknown primary melanoma (n=1), and sarcoma (n=1). Median hPFS was 240 days, 9 of 10 patients (90%) demonstrated stable disease or partial response to treatment. At a median follow up of 11.5 months, 4 of 10 (40%) remain alive. There were no perioperative mortalities. Myelosuppresion was the most common morbidity, managed on an outpatient basis with growth factors. The median hospital stay was 3 days. Conclusions Patients with metastatic melanoma and sarcoma to the liver have limited treatment options. Our experience with PHP demonstrates promising results with minimal morbidity and should be considered (pending FDA approval) as a management option for unresectable melanoma or sarcoma hepatic metastasis. PMID:24249545

  5. EWS/ATF1 expression induces sarcomas from neural crest–derived cells in mice

    PubMed Central

    Yamada, Kazunari; Ohno, Takatoshi; Aoki, Hitomi; Semi, Katsunori; Watanabe, Akira; Moritake, Hiroshi; Shiozawa, Shunichi; Kunisada, Takahiro; Kobayashi, Yukiko; Toguchida, Junya; Shimizu, Katsuji; Hara, Akira; Yamada, Yasuhiro

    2013-01-01

    Clear cell sarcoma (CCS) is an aggressive soft tissue malignant tumor characterized by a unique t(12;22) translocation that leads to the expression of a chimeric EWS/ATF1 fusion gene. However, little is known about the mechanisms underlying the involvement of EWS/ATF1 in CCS development. In addition, the cellular origins of CCS have not been determined. Here, we generated EWS/ATF1-inducible mice and examined the effects of EWS/ATF1 expression in adult somatic cells. We found that forced expression of EWS/ATF1 resulted in the development of EWS/ATF1-dependent sarcomas in mice. The histology of EWS/ATF1-induced sarcomas resembled that of CCS, and EWS/ATF1-induced tumor cells expressed CCS markers, including S100, SOX10, and MITF. Lineage-tracing experiments indicated that neural crest–derived cells were subject to EWS/ATF1-driven transformation. EWS/ATF1 directly induced Fos in an ERK-independent manner. Treatment of human and EWS/ATF1-induced CCS tumor cells with FOS-targeted siRNA attenuated proliferation. These findings demonstrated that FOS mediates the growth of EWS/ATF1-associated sarcomas and suggest that FOS is a potential therapeutic target in human CCS. PMID:23281395

  6. Aortic intimal sarcoma masquerading as bilateral renal artery stenosis.

    PubMed

    Sethi, Supreet; Pothineni, Naga Krishna; Syal, Gaurav; Ali, Syed Mujtaba; Krause, Michelle W

    2013-01-01

    Aortic intimal sarcoma is a rare tumor with poor prognosis. The most common manifestations are thromboembolic phenomena and vascular obstruction. We present a case of aortic intimal sarcoma causing bilateral renal artery stenosis which manifested as resistant hypertension and acute kidney inury. Multiple attempts to stent the renal arteries were unsuccessful. Eventually the patient developed acute limb ischemia and oliguric kidney failure as complications of the primary tumor.

  7. Monogenic and polygenic determinants of sarcoma risk: an international genetic study.

    PubMed

    Ballinger, Mandy L; Goode, David L; Ray-Coquard, Isabelle; James, Paul A; Mitchell, Gillian; Niedermayr, Eveline; Puri, Ajay; Schiffman, Joshua D; Dite, Gillian S; Cipponi, Arcadi; Maki, Robert G; Brohl, Andrew S; Myklebost, Ola; Stratford, Eva W; Lorenz, Susanne; Ahn, Sung-Min; Ahn, Jin-Hee; Kim, Jeong Eun; Shanley, Sue; Beshay, Victoria; Randall, Robert Lor; Judson, Ian; Seddon, Beatrice; Campbell, Ian G; Young, Mary-Anne; Sarin, Rajiv; Blay, Jean-Yves; O'Donoghue, Seán I; Thomas, David M

    2016-09-01

    Sarcomas are rare, phenotypically heterogeneous cancers that disproportionately affect the young. Outside rare syndromes, the nature, extent, and clinical significance of their genetic origins are not known. We aimed to investigate the genetic basis for bone and soft-tissue sarcoma seen in routine clinical practice. In this genetic study, we included 1162 patients with sarcoma from four cohorts (the International Sarcoma Kindred Study [ISKS], 966 probands; Project GENESIS, 48 probands; Asan Bio-Resource Center, 138 probands; and kConFab, ten probands), who were older than 15 years at the time of consent and had a histologically confirmed diagnosis of sarcoma, recruited from specialist sarcoma clinics without regard to family history. Detailed clinical, pathological, and pedigree information was collected, and cancer diagnoses in probands and relatives were independently verified. Targeted exon sequencing using blood (n=1114) or saliva (n=48) samples was done on 72 genes (selected due to associations with increased cancer risk) and rare variants were stratified into classes approximating the International Agency for Research on Cancer (IARC) clinical classification for genetic variation. We did a case-control rare variant burden analysis using 6545 Caucasian controls included from three cohorts (ISKS, 235 controls; LifePool, 2010 controls; and National Heart, Lung, and Blood Institute Exome Sequencing Project [ESP], 4300 controls). The median age at cancer diagnosis in 1162 sarcoma probands was 46 years (IQR 29-58), 170 (15%) of 1162 probands had multiple primary cancers, and 155 (17%) of 911 families with informative pedigrees fitted recognisable cancer syndromes. Using a case-control rare variant burden analysis, 638 (55%) of 1162 sarcoma probands bore an excess of pathogenic germline variants (combined odds ratio [OR] 1·43, 95% CI 1·24-1·64, p<0·0001), with 227 known or expected pathogenic variants occurring in 217 individuals. All classes of pathogenic

  8. Nature and distribution of feline sarcoma virus nucleotide sequences.

    PubMed Central

    Frankel, A E; Gilbert, J H; Porzig, K J; Scolnick, E M; Aaronson, S A

    1979-01-01

    The genomes of three independent isolates of feline sarcoma virus (FeSV) were compared by molecular hybridization techniques. Using complementary DNAs prepared from two strains, SM- and ST-FeSV, common complementary DNA'S were selected by sequential hybridization to FeSV and feline leukemia virus RNAs. These DNAs were shown to be highly related among the three independent sarcoma virus isolates. FeSV-specific complementary DNAs were prepared by selection for hybridization by the homologous FeSV RNA and against hybridization by fline leukemia virus RNA. Sarcoma virus-specific sequences of SM-FeSV were shown to differ from those of either ST- or GA-FeSV strains, whereas ST-FeSV-specific DNA shared extensive sequence homology with GA-FeSV. By molecular hybridization, each set of FeSV-specific sequences was demonstrated to be present in normal cat cellular DNA in approximately one copy per haploid genome and was conserved throughout Felidae. In contrast, FeSV-common sequences were present in multiple DNA copies and were found only in Mediterranean cats. The present results are consistent with the concept that each FeSV strain has arisen by a mechanism involving recombination between feline leukemia virus and cat cellular DNA sequences, the latter represented within the cat genome in a manner analogous to that of a cellular gene. PMID:225544

  9. Ewing's sarcoma: analysis of single nucleotide polymorphism in the EWS gene.

    PubMed

    Silva, Deborah S B S; Sawitzki, Fernanda R; De Toni, Elisa C; Graebin, Pietra; Picanco, Juliane B; Abujamra, Ana Lucia; de Farias, Caroline B; Roesler, Rafael; Brunetto, Algemir L; Alho, Clarice S

    2012-11-10

    We aimed to investigate single nucleotide polymorphisms (SNPs) in the EWS gene breaking region in order to analyze Ewing's sarcoma susceptibility. The SNPs were investigated in a healthy subject population and in Ewing's sarcoma patients from Southern Brazil. Genotyping was performed by TaqMan® assay for allelic discrimination using Real-Time PCR. The analysis of incidence of SNPs or different SNP-arrangements revealed a higher presence of homozygote TT-rs4820804 in Ewing's sarcoma patients (p=0.02; Chi Square Test). About 300 bp from the rs4820804 SNP lies a palindromic hexamer (5'-GCTAGC-3') and three nucleotides (GTC), which were previously identified to be in close vicinity of the breakpoint junction in both EWS and FLI1 genes. This DNA segment surrounding the rs4820804 SNP is likely to indicate a breakpoint region. If the T-rs4820804 allele predisposes a DNA fragment to breakage, homozygotes (TT-rs4820804) would have double the chance of having a chromosome break, increasing the chances for a translocation to occur. In conclusion, the TT-rs4820804 EWS genotype can be associated with Ewing's sarcoma and the SNP rs4820804 can be a candidate marker to understand Ewing's sarcoma susceptibility. Copyright © 2012 Elsevier B.V. All rights reserved.

  10. EWING'S SARCOMA: EPIDEMIOLOGY AND PROGNOSIS FOR PATIENTS TREATED AT THE PEDIATRIC ONCOLOGY INSTITUTE, IOP-GRAACC-UNIFESP.

    PubMed

    Bellan, Davi Gabriel; Filho, Reynaldo Jesus-Garcia; Garcia, Jairo Greco; de Toledo Petrilli, Marcelo; Maia Viola, Dan Carai; Schoedl, Murillo Ferri; Petrilli, Antonio Sérgio

    2012-01-01

    To outline the epidemiological profile and prognosis for Ewing's sarcoma in the Brazilian population. The medical records of 64 patients with intraosseous Ewing's sarcoma who were treated at the Pediatric Oncology Institute, IOP-GRAACC-Unifesp, between 1995 and 2010, were retrospectively evaluated. The statistical analysis on the data obtained did not correlate factors such as sex, trauma, pathological fracture and time taken for case diagnosis with the treatment outcome. Factors such as initial metastasis, lung metastasis, tumor site, age, recurrence and type of surgery showed results corroborating what has been established in the literature. The prognosis in cases of Ewing's sarcoma was mainly influenced by the presence of metastases at the time of diagnosis.

  11. Durable clinical activity of single-agent bevacizumab in a nonagenarian patient with metastatic alveolar soft part sarcoma.

    PubMed

    Mir, Olivier; Boudou-Rouquette, Pascaline; Larousserie, Frédérique; Blanchet, Benoit; Babinet, Antoine; Anract, Philippe; Goldwasser, François

    2012-08-01

    Alveolar soft part sarcoma is a rare malignancy usually considered resistant to conventional chemotherapy, but recent data suggest that the multikinase inhibitors sunitinib and cediranib could be active in this setting. A 90-year-old lady with alveolar soft part sarcoma of the leg and lung metastases was started on sunitinib 37.5 mg daily. The treatment was poorly tolerated with grade 3 hypertension and grade 3 thrombocytopenia, which persisted after dose reduction to 25 mg daily. The patient was subsequently started on bevacizumab 10 mg/kg every 2 weeks, resulting in a marked improvement in pain and a partial response on lung metastases for 16 months and ongoing. Agents targeting the vascular endothelial growth factor-signalling pathway seem to exert clinically relevant and prolonged activity against alveolar soft part sarcoma and deserve further evaluation in the treatment of this rare soft tissue sarcoma.

  12. Risk factor analysis for massive lymphatic ascites after laparoscopic retroperitonal lymphadenectomy in gynecologic cancers and treatment using intranodal lymphangiography with glue embolization

    PubMed Central

    2016-01-01

    Objective To evaluate risk factors for massive lymphatic ascites after laparoscopic retroperitoneal lymphadenectomy in gynecologic cancer and the feasibility of treatments using intranodal lymphangiography (INLAG) with glue embolization. Methods A retrospective analysis of 234 patients with gynecologic cancer who received laparoscopic retroperitonal lymphadenectomy between April 2006 and November 2015 was done. In June 2014, INLAG with glue embolization was initiated to manage massive lymphatic ascites. All possible clinicopathologic factors related to massive lymphatic ascites were determined in the pre-INLAG group (n=163). Clinical courses between pre-INLAG group and post-INLAG group (n=71) were compared. Results In the pre-INLAG group (n=163), four patients (2.5%) developed massive lymphatic ascites postoperatively. Postoperative lymphatic ascites was associated with liver cirrhosis (three cirrhotic patients, p<0.001). In the post-INLAG group, one patient with massive lymphatic ascites had a congestive heart failure and first received INLAG with glue embolization. She had pelvic drain removed within 7 days after INLAG. The mean duration of pelvic drain and hospital stay decreased after the introduction of INLAG (13.2 days vs. 10.9 days, p=0.001; 15.2 days vs. 12.6 days, p=0.001). There was no evidence of recurrence after this procedure. Conclusion Underlying medical conditions related to the reduced effective circulating volume, such as liver cirrhosis and heart failure, may be associated with massive lymphatic ascites after retroperitoneal lymphadenectomy. INLAG with glue embolization can be an alternative treatment options to treat leaking lymphatic channels in patients with massive lymphatic leakage. PMID:27171674

  13. Evaluation of Cytarabine Against Ewing Sarcoma Xenografts by the Pediatric Preclinical Testing Program

    PubMed Central

    Houghton, Peter J.; Morton, Christopher L.; Kang, Min; Reynolds, C. Patrick; Billups, Catherine A.; Favours, Edward; Payne-Turner, Debbie; Tucker, Chandra; Smith, Malcolm A.

    2015-01-01

    Treatment with the nucleoside analog cytarabine has been shown to mimic changes in gene expression associated with down-regulation of the EWS-FLI1 oncogene in Ewing sarcoma cell lines, selectively inhibit their growth in vitro, and cause tumor regression in athymic nude mice. For this report cytarabine was studied in vitro against a panel of 23 pediatric cancer cell lines and in vivo against 6 Ewing sarcoma xenografts. Acute lymphoblastic leukemia cell lines were the most sensitive to cytarabine in vitro (median IC50 9 nM), while Ewing sarcoma cell lines showed intermediate sensitivity (median IC50 232 nM). Cytarabine at a dose of 150 mg/kg administered daily 5× failed to significantly inhibit growth of five xenograft models, but reduced growth rate of the A673 xenograft by 50%. Cytarabine shows no differential in vitro activity against Ewing sarcoma cell lines and is ineffective in vivo against Ewing sarcoma xenografts at the dose and schedule studied. PMID:20979180

  14. Differences in Activities of Daily Living Performance Between Long-Term Pediatric Sarcoma Survivors and a Matched Comparison Group on Standardized Testing

    PubMed Central

    Parks, Rebecca; Rasch, Elizabeth K.; Mansky, Patrick J.; Oakley, Frances

    2009-01-01

    BACKGROUND: In a cross-sectional study examining late effects of pediatric sarcoma therapy, long-term survivors were evaluated on their activities of daily living (ADL) performance. PROCEDURE: Thirty-two persons with Ewing sarcoma family of tumors, rhabdomyosarcoma, and non-rhabdomysarcoma-soft tissue sarcoma enrolled an average of 17 years after treatment. Participants were evaluated using the Assessment of Motor and Process Skills (AMPS) [1], a standardized observational evaluation of ADL task performance. Means and 95% confidence intervals for ADL motor and ADL process ability measures were calculated for four groups: 1) sarcoma survivors, 2) “well” adults matched for age and gender, 3) “well” adults matched for gender that were 10 years older; and 4) “well” adults matched for gender that were 20 years older. RESULTS: ADL motor ability was significantly lower for sarcoma survivors than for the age and gender matched comparison group (p<0.05). There was no significant difference between ADL motor ability of sarcoma survivors and the comparison group 10 years older, but sarcoma survivors had significantly better ADL motor ability (p<0.05) than the oldest comparison group (20 years older). Sarcoma survivors had significantly worse ADL process ability than the age matched group (p<0.05). There was no difference in ADL process ability between the sarcoma survivors and comparison groups that were 10 and 20 years older. CONCLUSIONS: This first report of a clinical evaluation of ADL limitation in pediatric sarcoma survivors treated with intensive multimodal cancer therapy suggests that influences on performance of daily life activities are more common than previously reported. PMID:19533662

  15. Cutaneous myeloid sarcoma of the penile foreskin.

    PubMed

    Afrose, Ruquiya; Nebhnani, Deepa; Wadhwa, Neelam

    2015-01-01

    Myeloid sarcoma, considered to herald the onset of a blast crisis in the setting of chronic myeloproliferative neoplasm/dysplasia, typically presents during the course of the disorder. Cutaneous involvement is uncommon and lesions on genital skin are seldom seen. We present a case of a well-differentiated myeloid sarcoma in the penile foreskin in an apparently healthy 29-year-old male presenting with phimosis. The unusual composition of the inflammatory cell infiltrate, and characteristic sparing of dermal blood vessels, nerves and smooth muscle fibres led to the correct diagnosis. Absence of commonly observed changes in the circumcision skin like those of balanitis xerotica was also helpful. Detailed hematological work up revealed a previously undiagnosed chronic myeloid leukemia in chronic phase. The patient also had simultaneous priapism, another rare presentation of chronic myeloid leukemia. One year hence, the patient is in hematological remission with no evidence of extramedullary disease. Although priapism has been described as a rare presenting symptom in chronic myeloid leukemia, the present case is unique as this is the first time a cutaneous myeloid sarcoma has been documented in the penile foreskin.

  16. [Association of Kaposi sarcoma--multiple myeloma. A new case].

    PubMed

    Cohen, J D; Thomas, E; Garnier, N; Hellier, I; Durand, L; Guilhou, J J; Baldet, P; Blotman, F

    2000-11-01

    Kaposi's disease is an angiogenic multifocal cancer process that has several forms, namely Mediterranean, African, HIV-associated, and secondary to a preexisting immunodepressive state (hematological disorder, corticosteroid therapy, immunodepressive treatment). Whatever its form, Kaposi's sarcoma is probably associated with a chronic viral human herpes type 8 infection (HHV8). This virus has been implicated in the pathogenesis of multiple myeloma (17 cases recorded to date). In the present study, a further case of Kaposi's sarcoma associated with multiple myeloma has been reported. However, Epstein-Barr virus, cytomegalovirus, hepatitis B and C, HIV and HHV8 serologies were negative. Radiotherapy on the lower limbs was initiated. It is concluded that HHV8 does not appear to play a pathogenic role in cases of multiple myeloma, given the rarity of the association between Kaposi's sarcoma/multiple myeloma/HHV8.

  17. Synovial sarcoma of the temporomandibular joint and infratemporal fossa.

    PubMed

    Nomura, Fuminori; Kishimoto, Seiji

    2014-12-01

    Synovial sarcoma in the head and neck region is rare, and is difficult to resect with adequate safety margins because of its anatomical complexity. We herein report our experiences with synovial sarcoma in this region, and review the literature regarding the management of such cases. We retrospectively examined four cases of synovial sarcoma arising from the temporomandibular joint (TMJ) area and infratemporal fossa. Only one patient remains alive without disease, while the other three patients have died. The local control of these tumors has improved because of the progress in the surgical operation methods, while it is expected that there is still a high rate of deaths due to distant metastasis increase. The development of strong chemotherapy is needed for the use after the initial treatment and surgery. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  18. [Clinical analysis of 48 cases sarcoma in nasal cavity and sinuses].

    PubMed

    Yang, Chengzhang; Zhang, Dan

    2004-10-01

    To enhance the level of diagnose and treatment of sarcoma in nasal cavity and sinuses by studying the clinical features, diagnosis and treatment of 48 cases sarcoma in nasal cavity and sinuses. Forty-eight cases sarcoma in nasal cavity and sinuses treated from 1995 to 2000 were analyzed retrospectively. Fibrosarcoma in 4 cases, malignant fibrous histiocytoma in 3 cases, liposarcoma in 4 cases, leiomyosarcoma in 5 cases, rhabdomyosarcoma in 5 cases, osteosarcoma in 2 cases, chondrosarcoma in 3 cases, malignant melanoma in 4 cases, non-Hogkin's in 4 cases, extramedullary plasmacytoma in 1 case, Schwannoma in 5 cases, esthesioneuroblastoma in 5 cases, angiosarcoma in 3 cases. Following-up after synthetic treatment, the 1, 3, 5 year survival rates were 62.5%, 46.7%, 35.7% respectively. Sarcoma in nasal cavity and sinuses is not easy to diagnose definitely, immunohistochemistry is helpful for pathological diagnose. It's easy to recurrent and metastasis and it's beneficial to improve prognosis with synthetic treatment.

  19. Biomarkers of Osteosarcoma, Chondrosarcoma, and Ewing Sarcoma.

    PubMed

    Evola, Francesco R; Costarella, Luciano; Pavone, Vito; Caff, Giuseppe; Cannavò, Luca; Sessa, Andrea; Avondo, Sergio; Sessa, Giuseppe

    2017-01-01

    Osteosarcoma is the most frequent malignant bone neoplasm, followed by chondrosarcoma and Ewing sarcoma. The diagnosis of bone neoplasms is generally made through histological evaluation of a biopsy. Clinical and radiological features are also important in aiding diagnosis and to complete the staging of bone cancer. In addition to these, there are several non-specific serological or specific molecular markers for bone neoplasms. In bone tumors, molecular markers increase the accuracy of the diagnosis and assist in subtyping bone tumors. Here, we review these markers and discuss their role in the diagnosis and prognosis of the three most frequent malignant bone neoplasms, namely osteosarcoma, chondrosarcoma, and Ewing sarcoma.

  20. [DESCRIPTION OF A RETROPERITONEAL ACCESS ROUTE TO THE VESSELS OF THE SPLEEN FOR SPLENORENAL ARTERIAL AND VENOUS ANASTOMOSIS].

    PubMed

    Gil-Vernet Vila, José María

    2014-01-01

    To perform arterial or venous spleno-renal anastomoses, surgeons have so far systematically used the transperitoneal way whic is burdened by a high mortality an morbility percentage. On the basis of anatomo-surgical considerations, a retroperitoneal approach has been found reaching the hilus of the spleen via the lumbar region; the first arterial spleno-renal anastomosis by this way was performed in 1972 and the first venous spleno-renal anastomosis due to portal hipertension also by this way was performed in 1974, the alter proving to be the least aggresive by avoiding damaging the páncreas, the most surgical and direct for reaching the splenic vessels thereby enabling a better exposure and an easier performing of the anastomoses. By being retroperitoneal, the loss or infección of the ascitic liquid in the cirrhotic patient is prevented.

  1. Reconstruction after musculoskeletal sarcomas: how to avoid a surgical trap.

    PubMed

    Zoccali, Giovanni; Zoccali, Carmine; Costantini, Maurizio; Buccheri, Ernesto M; Biagini, Roberto; DE Vita, Roy

    2017-04-01

    Musculoskeletal sarcomas comprise 1% of all malignancies in adults. Unfortunately, sometimes they are addressed in non-appropriate way requiring a more invasive procedure to achieve radical surgery at a later date. Due to incomplete predictability of their extension, scheduled reconstruction cannot be performed at times, forcing plans to change or clogging up immediate reconstruction. In this paper, the authors provide an insight in the treatment of musculoskeletal sarcomas, particularly focusing on the preoperative planning of reconstructive strategies, which is crucial in order to prevent unpleasant surprises during reconstruction. Fifty-six consecutive patients requiring reconstructive procedures following the extirpation of tumors were recruited. All data collected during the diagnostic phase were analyzed collectively during a multi-disciplinary meeting where the surgical procedure was planned. A score system was created and results were then classified into "excellent", "good", "sufficient" and "poor". After a minimum follow up of 12 months, we recorded the following results: excellent in 10 patients (17.9%), good in 28 patients (50%), sufficient in 12 cases (21.4%) and poor in 6 cases (10.7%). The improvement of treatment and the long-lasting survival in musculoskeletal sarcoma have shifted the goal of therapeutic protocol to obtaining radical tumor removal and maximum functional restoration. When facing unpredictable extension of the resections, reconstruction may be a challenging or even impossible task to fulfil. Only meticulous preoperative planning can prevent surgeons from falling into all sorts of surgical traps following wide resections.

  2. Unusual Presentation of a Primary Ewing's Sarcoma of the Spine with Paraplegia: A Case Report.

    PubMed

    Kannan, Karthik Kailash; Sundarapandian, Rajkumar Jayachandran; Surulivel, Vignesh Jayabalan

    2015-03-01

    Ewing's sarcoma is a primary malignancy of the bone affecting individuals in the second decade of life. Primary sarcomas of the spine are rare and the occurrence of Primary Ewing's sarcoma in the spine is very rare. Ewing's sarcoma occurring in the spine is divided into two types, Ewing's sarcoma of sacral spine which are very aggressive with poor prognosis and Ewing's sarcoma of the non sacral spine which is an extremely rare occurrence. Patient may present with neurological deficit when the tumour extends into the spinal canal causing spinal cord compression. Magnetic resonance imaging (MRI) is very sensitive in diagnosing the tumour and defining the extent of the tumour. Here we report an 18-year-old boy who presented with back pain and complete paraplegia of two months duration. The MRI gave a differential diagnosis of infective pathology due to the fluid collection in the paraspinal region, followed by primary malignancy as the second diagnosis. Patient underwent posterior spinal decompression and stabilization, and intaoperatively there was significant collection of pus whose culture showed no growth. The histopathology and immunohistochemistry studies confirmed the diagnosis of Ewing's sarcoma and patient was started on combination chemotherapy and radiotherapy.

  3. T1-201 chloride scintigraphy for bone tumors and soft part sarcomas

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Terui, S.; Oyamada, H.; Nishikawa, K.

    1984-01-01

    The author investigated T1-201 chloride as a tumor scanning agent of both tumors and soft part sarcomas. Six bone tumors (2 with Ewing sarcoma, 3 with osteosarcoma and 1 with giant cell tumor) and 3 soft part sarcoma (1 with liposarcoma and 2 with malignant fibrous histiocytoma (MFH)) were examined. All but one MFH were untreated primary cases. The diagnosis was determined from biopsy specimen. One patient with Ewing sarcoma had bone metastases. All cases were subsequently received chemotherpeutic agents. Surgery or local irradiation were also used in treatment. T1-201 scintigraphy were performed with intravenous administration of 2 mCi ofmore » T1-201 chloride before initiation of therapy. In addition, follow-up examinations were done in 4 patients (2 with Ewing sarcoma and 2 with osteosarcoma) to study the effect of chemotherapy on T1-201 uptake by the tumor. Tc-99m bone scans were available for comparison in 6 tumor. Ga-67 citrate scans were also examined for the 3 soft part sarcomas. The untreated tumors even in the metastatic lesions of Ewing sarcoma were distinctly visualized with T1-201 in all cases. The distribution of T1-201 in the tumors was sometimes different from that of Tc-99m and similar to that of Ga-67. Of 3 out of the 4 follow-up patients, the post-therapy scan showed reduction in T1-201 uptake more markedly than Tc-99m uptake during effective chemotherapy. The other one patient had not responded to the treatment so that the scan showed no changes in T1-201 uptake. These findings indicate that the tumor imaging with T1-201 is useful in the diagnosis of these malignant tumors and may be of value in assessing the response of bone tumors to chemotherapy.« less

  4. [Lymph drainage therapy in secondary lymphedema caused by Kaposi sarcoma].

    PubMed

    Einfeldt, H

    1989-07-01

    For reasons not yet known HIV infected patients in the final state of their aids disease often tend to develop Kaposi's sarcoma. These tumours result in secondary lymphatic edemas which are found on both sides of the sarcoma. They reach up to the regional lymphatic nodes blocked by the tumour cells. Depending on the state of the edema a lymphaticdrainage treatment is indicated palliatively; the patients can thus be relieved. A fundamental deterioration of prognosis is not to be expected, as all patients are anyway in the final stage of this not yet curable disease. Differing from treatment of other lymphatic edema, it is of special importance to the therapist - apart from the particular and difficult psychic burden - to pay attention to infection protection by using gloves for each single treatment.

  5. Immune reconstitution inflammatory syndrome associated with Kaposi sarcoma: higher incidence and mortality in Africa than in the UK.

    PubMed

    Letang, Emilio; Lewis, James J; Bower, Mark; Mosam, Anisa; Borok, Margareth; Campbell, Thomas B; Naniche, Denise; Newsom-Davis, Tom; Shaik, Fahmida; Fiorillo, Suzanne; Miro, Jose M; Schellenberg, David; Easterbrook, Philippa J

    2013-06-19

    To assess the incidence, predictors, and outcomes of Kaposi sarcoma-associated paradoxical immune reconstitution inflammatory syndrome (KS-IRIS) in antiretroviral therapy (ART)-naive HIV-infected patients with Kaposi sarcoma initiating ART in both well resourced and limited-resourced settings. Pooled analysis of three prospective cohorts of ART-naive HIV-infected patients with Kaposi sarcoma from sub-Saharan Africa (SSA) and one from the UK. KS-IRIS case definition was standardized across sites. Cox regression and Kaplan-Meier survival analysis were used to identify the incidence and predictors of KS-IRIS and Kaposi sarcoma-associated mortality. Fifty-eight of 417 (13.9%) eligible individuals experienced KS-IRIS with an incidence 2.5 times higher in the African vs. European cohorts (P=0.001). ART alone as initial Kaposi sarcoma treatment (hazard ratio 2.97, 95% confidence interval (CI) 1.02-8.69); T1 Kaposi sarcoma stage (hazard ratio 2.96, 95% CI 1.26-6.94); and plasma HIV-1 RNA more than 5 log₁₀ copies/ml (hazard ratio 2.14, 95% CI 1.25-3.67) independently predicted KS-IRIS at baseline. Detectable plasma Kaposi sarcoma-associated herpes virus (KSHV) DNA additionally predicted KS-IRIS among the 259 patients with KSHV DNA assessed (hazard ratio 2.98, 95% CI 1.23-7.19). Nineteen KS-IRIS patients died, all in SSA. Kaposi sarcoma mortality was 3.3-fold higher in Africa, and was predicted by KS-IRIS (hazard ratio 19.24, CI 7.62-48.58), lack of chemotherapy (hazard ratio 2.35, 95% CI 1.09-5.05), pre-ART CD4 cell count less than 200 cells/μl (hazard ratio 2.04, 95% CI 0.99-4.2), and detectable baseline KSHV DNA (hazard ratio 2.12, 95% CI 0.94-4.77). KS-IRIS incidence and mortality are higher in SSA than in the UK. This is largely explained by the more advanced Kaposi sarcoma disease and lower chemotherapy availability. KS-IRIS is a major contributor to Kaposi sarcoma-associated mortality in Africa. Our results support the need to increase awareness on KS

  6. Histological variants of cutaneous Kaposi sarcoma

    PubMed Central

    Grayson, Wayne; Pantanowitz, Liron

    2008-01-01

    This review provides a comprehensive overview of the broad clinicopathologic spectrum of cutaneous Kaposi sarcoma (KS) lesions. Variants discussed include: usual KS lesions associated with disease progression (i.e. patch, plaque and nodular stage); morphologic subtypes alluded to in the older literature such as anaplastic and telangiectatic KS, as well as several lymphedematous variants; and numerous recently described variants including hyperkeratotic, keloidal, micronodular, pyogenic granuloma-like, ecchymotic, and intravascular KS. Involuting lesions as a result of treatment related regression are also presented. PMID:18655700

  7. Hypoxia and hypoglycaemia in Ewing's sarcoma and osteosarcoma: regulation and phenotypic effects of Hypoxia-Inducible Factor.

    PubMed

    Knowles, Helen J; Schaefer, Karl-Ludwig; Dirksen, Uta; Athanasou, Nicholas A

    2010-07-16

    Hypoxia regulates gene expression via the transcription factor HIF (Hypoxia-Inducible Factor). Little is known regarding HIF expression and function in primary bone sarcomas. We describe HIF expression and phenotypic effects of hypoxia, hypoglycaemia and HIF in Ewing's sarcoma and osteosarcoma. HIF-1alpha and HIF-2alpha immunohistochemistry was performed on a Ewing's tumour tissue array. Ewing's sarcoma and osteosarcoma cell lines were assessed for HIF pathway induction by Western blot, luciferase assay and ELISA. Effects of hypoxia, hypoglycaemia and isoform-specific HIF siRNA were assessed on proliferation, apoptosis and migration. 17/56 Ewing's tumours were HIF-1alpha-positive, 15 HIF-2alpha-positive and 10 positive for HIF-1alpha and HIF-2alpha. Expression of HIF-1alpha and cleaved caspase 3 localised to necrotic areas. Hypoxia induced HIF-1alpha and HIF-2alpha in Ewing's and osteosarcoma cell lines while hypoglycaemia specifically induced HIF-2alpha in Ewing's. Downstream transcription was HIF-1alpha-dependent in Ewing's sarcoma, but regulated by both isoforms in osteosarcoma. In both cell types hypoglycaemia reduced cellular proliferation by >or= 45%, hypoxia increased apoptosis and HIF siRNA modulated hypoxic proliferation and migration. Co-localisation of HIF-1alpha and necrosis in Ewing's sarcoma suggests a role for hypoxia and/or hypoglycaemia in in vivo induction of HIF. In vitro data implicates hypoxia as the primary HIF stimulus in both Ewing's and osteosarcoma, driving effects on proliferation and apoptosis. These results provide a foundation from which to advance understanding of HIF function in the pathobiology of primary bone sarcomas.

  8. The molecular biology of soft-tissue sarcomas and current trends in therapy.

    PubMed

    Quesada, Jorge; Amato, Robert

    2012-01-01

    Basic research in sarcoma models has been fundamental in the discovery of scientific milestones leading to a better understanding of the molecular biology of cancer. Yet, clinical research in sarcoma has lagged behind other cancers because of the multiple clinical and pathological entities that characterize sarcomas and their rarity. Sarcomas encompass a very heterogeneous group of tumors with diverse pathological and clinical overlapping characteristics. Molecular testing has been fundamental in the identification and better definition of more specific entities among this vast array of malignancies. A group of sarcomas are distinguished by specific molecular aberrations such as somatic mutations, intergene deletions, gene amplifications, reciprocal translocations, and complex karyotypes. These and other discoveries have led to a better understanding of the growth signals and the molecular pathways involved in the development of these tumors. These findings are leading to treatment strategies currently under intense investigation. Disruption of the growth signals is being targeted with antagonistic antibodies, tyrosine kinase inhibitors, and inhibitors of several downstream molecules in diverse molecular pathways. Preliminary clinical trials, supported by solid basic research and strong preclinical evidence, promises a new era in the clinical management of these broad spectrum of malignant tumors.

  9. Histopathologic and Radiologic Assessment of Chemotherapeutic Response in Ewing's Sarcoma: A Review.

    PubMed

    García-Castellano, José M; Atallah Yordi, Nagib; Reyes, Carolina; Healey, John H

    2012-01-01

    Ewing's sarcoma is a highly malignant tumor that metastasizes rapidly and is thus associated with a low survival rate. The intensification of chemotherapy has been shown to improve the overall survival of patients with Ewing's sarcoma. However, intensified chemotherapy can lead to increased toxicity or even the development of secondary malignancies. The stratification of patients with Ewing's sarcoma into "good" and "poor" responders may help guide the administration of progressively more intensified chemotherapy. Thus, an accurate assessment of the chemotherapeutic response, as well as the extent of chemotherapy-induced tumor necrosis, is critical for avoiding potential treatment-related complications in these patients. This paper reviews the methods currently used to evaluate chemotherapeutic response in Ewing's sarcoma, focusing specifically on histopathologic and imaging analyses, and discusses novel therapies and imaging methods that may help improve the overall survival of these patients.

  10. Isolated Limb Perfusion With Melphalan in Treating Patients With Stage IIIB-IV Melanoma or Sarcoma

    ClinicalTrials.gov

    2015-07-22

    Basal Cell Carcinoma of the Skin; Eccrine Carcinoma of the Skin; Recurrent Adult Soft Tissue Sarcoma; Recurrent Melanoma; Recurrent Skin Cancer; Squamous Cell Carcinoma of the Skin; Stage III Adult Soft Tissue Sarcoma; Stage IIIB Melanoma; Stage IIIC Melanoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Melanoma

  11. Lymphatic endothelial cell line (CH3) from a recurrent retroperitoneal lymphangioma.

    PubMed

    Way, D; Hendrix, M; Witte, M; Witte, C; Nagle, R; Davis, J

    1987-09-01

    An endothelial cell line derived from a massive recurrent chyle-containing retroperitoneal lymphangioma was isolated in monolayer culture. Scanning and transmission electron microscopy and immunohistochemistry confirmed a close resemblance to blood vascular endothelium with typical cobblestone morphology, positive immunofluorescence staining for endothelial marker Factor VIII-associated antigen and fibronectin, and prominent Weibel-Palade bodies. The endothelial cells also exhibited other ultrastructural features characteristic of lymphatic endothelium, including sparse microvillous surface projections, overlapping intercellular junctions, and abundant intermediate filaments. This endothelial cell line represents a new source of proliferating lymphatic endothelium for future study, including structural and functional comparison to blood vascular endothelium.

  12. The retrospective analysis of patients with uterine sarcomas: A single-center experience.

    PubMed

    Terek, Mustafa Cosan; Akman, Levent; Hursitoglu, Behiye Seda; Sanli, Ulus Ali; Ozsaran, Zeynep; Tekindal, Mustafa Agah; Dikmen, Yilmaz; Zekioglu, Osman; Ozsaran, Ahmet Aydin

    2016-01-01

    Uterine sarcomas are rare, malignant, gynecological tumors and show diverse histopathological features. Therefore, there is no consensus on risk factors for poor outcome and optimal treatment. The aim of this retrospective analysis is to report the clinical outcome of patients with uterine sarcoma treated at a single center. The data was obtained regarding the patient's demographic characteristics, pathological results, treatments given, survival, and complications of all uterine sarcoma patients treated in a single center between the years 2000 and 2012. The 80.month overall survival. (OS) was determined with respect to prognostic factors including age, stage of disease, histopathological type, and adjuvant treatment. A total of 57 case records are retrieved for this retrospective analysis. The mean age of the patients is 62.5 ± 11.2 years. International Federation of Gynecology and Obstetrics (FIGO) stage distribution is stage I: 29; stage II: 13; stage III: 9; stage IV: 6. Fifty-seven patients underwent surgery, 33 received postoperative radiotherapy (PORT), and 32 received chemotherapy. Median follow-up period was 25 months (range 2-85 months). The 80-month OS for the entire group of patients was 36.7%. The significant prognostic factors for survival are age under 50 years, stage of disease, and adjuvant chemotherapy. Although limited by small sample size and retrospective nature, age under 50 years, stage of disease, and adjuvant chemotherapy are significant prognostic factors for survival for uterine sarcomas.

  13. Primary cardiac sarcoma complicated with cerebral infarction and brain metastasis: A case report and literature review.

    PubMed

    Sun, Yun-Peng; Wang, Xuan; Gao, Yong-Sheng; Zhao, Song; Bai, Yang

    2017-12-12

    In large autopsy series, the estimated frequency of primary tumors of the heart ranges from 0.0017% to 0.33%. Approximately 25% of primary cardiac tumors are malignant, and nearly 20% of these are sarcomas. To date, a completely feasible surgical resection remains the major treatment measure of cardiac sarcoma, especially for recurrent focal cardiac sarcoma and the recurrence of a restrictive metastasis. Although characteristically medical treatments are recommended, there is no consistent opinion for adjuvant radiotherapy and chemotherapy following an operation. Since these tumors usually undergo extensive spread by the time that the diagnosis is established, the prognosis of cardiac sarcoma remains poor. In this report, we described a case who underwent initial cardiac tumor resection, and was confirmed to be a pleomorphic undifferentiated sarcoma based on pathological findings. However, the patient complicated with cerebral infarction and subsequent brain metastasis sarcoma after the initial surgery, which was confirmed by brain tissue pathology. During the course of therapy, the patient underwent three surgical operations and refused to accept any chemotherapy and radiotherapy intervention. To the best of our knowledge, this is the first case report describing a primary cardiac sarcoma complicated with cerebral infarction and brain metastasis. The management of primary cardiac sarcoma is also discussed.

  14. Validation of the Complexity INdex in SARComas prognostic signature on formalin-fixed, paraffin-embedded, soft tissue sarcomas.

    PubMed

    Le Guellec, S; Lesluyes, T; Sarot, E; Valle, C; Filleron, T; Rochaix, P; Valentin, T; Pérot, G; Coindre, J-M; Chibon, F

    2018-05-31

    Prediction of metastatic outcome in sarcomas is challenging for clinical management since they are aggressive and carry a high metastatic risk. A 67-gene expression signature, the Complexity INdex in SARComas (CINSARC), has been identified as a better prognostic factor than the reference pathological grade. Since it cannot be applied easily in standard laboratory practice, we assessed its prognostic value using nanoString on formalin-fixed, paraffin-embedded (FFPE) blocks to evaluate its potential in clinical routine practice and guided therapeutic management. A code set consisting of 67 probes derived from the 67 genes of the CINSARC signature was built and named NanoCind®. To compare the performance of RNA-seq and nanoString (NanoCind®), we used expressions of various sarcomas (n=124, frozen samples) using both techniques and compared predictive values based on CINSARC risk groups and clinical annotations. We also used nanoString on FFPE blocks (n=67) and matching frozen and FFPE samples (n=45) to compare their level of agreement. Metastasis-free survival and agreement values in classification groups were evaluated. CINSARC strongly predicted metastatic outcome using nanoString on frozen samples (HR = 2.9, 95% CI 1.23-6.82) with similar risk-group classifications (86%). While more than 50% of FFPE blocks were not analyzable by RNA-seq owing to poor RNA quality, all samples were analyzable with nanoString. When similar (risk-group) classifications were measured with frozen tumors (RNA-seq) compared to FFPE blocks (84% agreement), the CINSARC signature was still a predictive factor of metastatic outcome with nanoString on FFPE samples (HR = 4.43, 95% CI 1.25-15.72). CINSARC is a material-independent prognostic signature for metastatic outcome in sarcomas and outperforms histological grade. Unlike RNA-seq, nanoString is not influenced by the poor quality of RNA extracted from FFPE blocks. The CINSARC signature can potentially be used in combination with

  15. Drugs Approved for Soft Tissue Sarcoma

    Cancer.gov

    This page lists cancer drugs approved by the Food and Drug Administration (FDA) for soft tissue sarcoma. The list includes generic names and brand names. The drug names link to NCI's Cancer Drug Information summaries.

  16. Pre-clinical and clinical significance of heparanase in Ewing’s sarcoma

    PubMed Central

    Shafat, Itay; Ben-Arush, Myriam Weyl; Issakov, Josephine; Meller, Isaac; Naroditsky, Inna; Tortoreto, Monica; Cassinelli, Giuliana; Lanzi, Cinzia; Pisano, Claudio; Ilan, Neta; Vlodavsky, Israel; Zunino, Franco

    2011-01-01

    Abstract Heparanase is an endoglycosidase that specifically cleaves heparan sulphate side chains of heparan sulphate proteoglycans, activity that is strongly implicated in cell migration and invasion associated with tumour metastasis, angiogenesis and inflammation. Heparanase up-regulation was documented in an increasing number of human carcinomas, correlating with reduced post-operative survival rate and enhanced tumour angiogenesis. Expression and significance of heparanase in human sarcomas has not been so far reported. Here, we applied the Ewing’s sarcoma cell line TC71 and demonstrated a potent inhibition of cell invasion in vitro and tumour xenograft growth in vivo upon treatment with a specific inhibitor of heparanase enzymatic activity (compound SST0001, non-anticoagulant N-acetylated, glycol split heparin). Next, we examined heparanase expression and cellular localization by immunostaining of a cohort of 69 patients diagnosed with Ewing’s sarcoma. Heparanase staining was noted in all patients. Notably, heparanase staining intensity correlated with increased tumour size (P = 0.04) and with patients’ age (P = 0.03), two prognostic factors associated with a worse outcome. Our study indicates that heparanase expression is induced in Ewing’s sarcoma and associates with poor prognosis. Moreover, it encourages the inclusion of heparanase inhibitors (i.e. SST0001) in newly developed therapeutic modalities directed against Ewing’s sarcoma and likely other malignancies. PMID:21029368

  17. Retroperitoneal haematoma causing gastric outflow obstruction following endovascular repair of a ruptured abdominal aortic aneurysm

    PubMed Central

    Hunter, Benjamin; Tod, Laura; Ghosh, Jonathan

    2012-01-01

    A 74-year-old man presented with back pain and collapse. A ruptured infrarenal abdominal aortic aneurysm was successfully managed by endovascular aneurysm repair. Postoperatively, he developed gastric outlet obstruction owing to duodenal compression from the unevacuated retroperitoneal haematoma. In the absence of abdominal compartment syndrome, conservative management with gastric decompression and parenteral nutrition led to a full recovery. PMID:23162028

  18. FOXM1 in sarcoma: role in cell cycle, pluripotency genes and stem cell pathways.

    PubMed

    Kelleher, Fergal C; O'Sullivan, Hazel

    2016-07-05

    FOXM1 is a pro-proliferative transcription factor that promotes cell cycle progression at the G1-S, and G2-M transitions. It is activated by phosphorylation usually mediated by successive cyclin - cyclin dependent kinase complexes, and is highly expressed in sarcoma. p53 down regulates FOXM1 and FOXM1 inhibition is also partly dependent on Rb and p21. Abnormalities of p53 or Rb are frequent in sporadic sarcomas with bone or soft tissue sarcoma, accounting for 36% of index cancers in the high penetrance TP53 germline disorder, Li-Fraumeni syndrome.FOXM1 stimulates transcription of pluripotency related genes including SOX2, KLF4, OCT4, and NANOG many of which are important in sarcoma, a disorder of mesenchymal stem cell/ partially committed progenitor cells. In a selected specific, SOX2 is uniformly expressed in synovial sarcoma. Embryonic pathways preferentially used in stem cell such as Hippo, Hedgehog, and Wnt dominate in FOXM1 stoichiometry to alter rates of FOXM1 production or degradation. In undifferentiated pleomorphic sarcoma, liposarcoma, and fibrosarcoma, dysregulation of the Hippo pathway increases expression of the effector co-transcriptional activator Yes-Associated Protein (YAP). A complex involving YAP and the transcription factor TEAD elevates FOXM1 in these sarcoma subtypes. In another scenario 80% of desmoid tumors have nuclear localization of β-catenin, the Wnt pathway effector molecule. Thiazole antibiotics inhibit FOXM1 and because they have an auto-regulator loop FOXM1 expression is also inhibited. Current systemic treatment of sarcoma is of limited efficacy and inhibiting FOXM1 represents a potential new strategy.

  19. FOXM1 in sarcoma: role in cell cycle, pluripotency genes and stem cell pathways

    PubMed Central

    Kelleher, Fergal C.; O'sullivan, Hazel

    2016-01-01

    FOXM1 is a pro-proliferative transcription factor that promotes cell cycle progression at the G1-S, and G2-M transitions. It is activated by phosphorylation usually mediated by successive cyclin – cyclin dependent kinase complexes, and is highly expressed in sarcoma. p53 down regulates FOXM1 and FOXM1 inhibition is also partly dependent on Rb and p21. Abnormalities of p53 or Rb are frequent in sporadic sarcomas with bone or soft tissue sarcoma, accounting for 36% of index cancers in the high penetrance TP53 germline disorder, Li-Fraumeni syndrome. FOXM1 stimulates transcription of pluripotency related genes including SOX2, KLF4, OCT4, and NANOG many of which are important in sarcoma, a disorder of mesenchymal stem cell/ partially committed progenitor cells. In a selected specific, SOX2 is uniformly expressed in synovial sarcoma. Embryonic pathways preferentially used in stem cell such as Hippo, Hedgehog, and Wnt dominate in FOXM1 stoichiometry to alter rates of FOXM1 production or degradation. In undifferentiated pleomorphic sarcoma, liposarcoma, and fibrosarcoma, dysregulation of the Hippo pathway increases expression of the effector co-transcriptional activator Yes-Associated Protein (YAP). A complex involving YAP and the transcription factor TEAD elevates FOXM1 in these sarcoma subtypes. In another scenario 80% of desmoid tumors have nuclear localization of β-catenin, the Wnt pathway effector molecule. Thiazole antibiotics inhibit FOXM1 and because they have an auto-regulator loop FOXM1 expression is also inhibited. Current systemic treatment of sarcoma is of limited efficacy and inhibiting FOXM1 represents a potential new strategy. PMID:27074562

  20. The Neurological Compromised Spine Due to Ewing Sarcoma. What First: Surgery or Chemotherapy? Therapy, Survival, and Neurological Outcome of 15 Cases With Primary Ewing Sarcoma of the Vertebral Column.

    PubMed

    Mirzaei, Lida; Kaal, Suzanne E J; Schreuder, Hendrik W B; Bartels, Ronald H M A

    2015-11-01

    The vertebral column is an infrequent site of primary involvement in Ewing sarcoma. Yet when Ewing sarcoma is found in the spine, the urge for decompression is high because of the often symptomatic compression of neural structures. It is unclear in alleviating a neurological deficit whether chemotherapy is preferred over decompressive laminectomy. To underline, in this case series, the efficiency of initial chemotherapy before upfront surgery in the setting of high-grade spinal cord or cauda equina compression of primary Ewing sarcoma. Fifteen patients with Ewing sarcoma primarily located in the spine were treated at our institution between 1983 and 2015. Localization, neurological deficit expressed as Frankel grade, and outcome expressed as Rankin scale before and after initial chemotherapy, the recurrence rate, and overall survival were evaluated. The multidisciplinary approach of 1 case will be discussed in detail. Nine patients (60%) were female. The age at presentation was 15.0 ± 5.5 years (range: 0.9-22.8 years). Ten patients (67%) were initially treated with chemotherapy, and 1 patient (7%) was treated primarily with radiotherapy followed by chemotherapy. The remaining 4 patients (27%) were initially treated with decompressive surgery. All patients treated primarily nonsurgically improved neurologically at follow-up, showing the importance of chemotherapy as an effective initial treatment option. Adequate and quick decompression of neural structures with similar results can be achieved by chemotherapy and radiotherapy, avoiding the local spill of malignant cells.

  1. IGF-1R/MDM2 relationship confers enhanced sensitivity to RITA in Ewing sarcoma cells.

    PubMed

    Di Conza, Giusy; Buttarelli, Marianna; Monti, Olimpia; Pellegrino, Marsha; Mancini, Francesca; Pontecorvi, Alfredo; Scotlandi, Katia; Moretti, Fabiola

    2012-06-01

    Ewing sarcoma is one of the most frequent bone cancers in adolescence. Although multidisciplinary therapy has improved the survival rate for localized tumors, a critical step is the development of new drugs to improve the long-term outcome of recurrent and metastatic disease and to reduce side effects of conventional therapy. Here, we show that the small molecule reactivation of p53 and induction of tumor cell apoptosis (RITA, NSC652287) is highly effective in reducing growth and tumorigenic potential of Ewing sarcoma cell lines. These effects occur both in the presence of wt-p53 as well as of mutant or truncated forms of p53, or in its absence, suggesting the presence of additional targets in this tumor histotype. Further experiments provided evidence that RITA modulates an important oncogenic mark of these cell lines, insulin-like growth factor receptor 1 (IGF-1R). Particularly, RITA causes downregulation of IGF-1R protein levels. MDM2 degradative activity is involved in this phenomenon. Indeed, inhibition of MDM2 function by genetic or pharmacologic approaches reduces RITA sensitivity of Ewing sarcoma cell lines. Overall, these data suggest that in the cell context of Ewing sarcoma, RITA may adopt additional mechanism of action besides targeting p53, expanding its field of application. Noteworthy, these results envisage the promising utilization of RITA or its derivative as a potential treatment for Ewing sarcomas. ©2012 AACR

  2. Targeting the Insulin-Like Growth Factor 1 Receptor in Ewing's Sarcoma: Reality and Expectations

    PubMed Central

    Olmos, David; Martins, Ana Sofia; Jones, Robin L.; Alam, Salma; Scurr, Michelle; Judson, Ian R.

    2011-01-01

    Ewing's sarcoma family of tumours comprises a group of very aggressive diseases that are potentially curable with multimodality treatment. Despite the undoubted success of current treatment, approximately 30% of patients will relapse and ultimately die of disease. The insulin-like growth factor 1 receptor (IGF-1R) has been implicated in the genesis, growth, proliferation, and the development of metastatic disease in Ewing's sarcoma. In addition, IGF1-R has been validated, both in vitro and in vivo, as a potential therapeutic target in Ewing's sarcoma. Phase I studies of IGF-1R monoclonal antibodies reported several radiological and clinical responses in Ewing's sarcoma patients, and initial reports of several Phase II studies suggest that about a fourth of the patients would benefit from IGF-1R monoclonal antibodies as single therapy, with approximately 10% of patients achieving objective responses. Furthermore, these therapies are well tolerated, and thus far severe toxicity has been rare. Other studies assessing IGF-1R monoclonal antibodies in combination with traditional cytotoxics or other targeted therapies are expected. Despite, the initial promising results, not all patients benefit from IGF-1R inhibition, and consequently, there is an urgent need for the identification of predictive markers of response. PMID:21647361

  3. Histopathologic and Radiologic Assessment of Chemotherapeutic Response in Ewing's Sarcoma: A Review

    PubMed Central

    García-Castellano, José M.; Atallah Yordi, Nagib; Reyes, Carolina; Healey, John H.

    2012-01-01

    Ewing's sarcoma is a highly malignant tumor that metastasizes rapidly and is thus associated with a low survival rate. The intensification of chemotherapy has been shown to improve the overall survival of patients with Ewing's sarcoma. However, intensified chemotherapy can lead to increased toxicity or even the development of secondary malignancies. The stratification of patients with Ewing's sarcoma into “good” and “poor” responders may help guide the administration of progressively more intensified chemotherapy. Thus, an accurate assessment of the chemotherapeutic response, as well as the extent of chemotherapy-induced tumor necrosis, is critical for avoiding potential treatment-related complications in these patients. This paper reviews the methods currently used to evaluate chemotherapeutic response in Ewing's sarcoma, focusing specifically on histopathologic and imaging analyses, and discusses novel therapies and imaging methods that may help improve the overall survival of these patients. PMID:22550418

  4. An aza-macrocycle containing maltolic side-arms (maltonis) as potential drug against human pediatric sarcomas

    PubMed Central

    2014-01-01

    Background Identification of new drugs against paediatric sarcomas represents an urgent clinical need that mainly relies on public investments due to the rarity of these diseases. In this paper we evaluated the in vitro and in vivo efficacy of a new maltol derived molecule (maltonis), belonging to the family of molecules named hydroxypyrones. Methods Maltonis was screened for its ability to induce structural alteration of DNA molecules in comparison to another maltolic molecule (malten). In vitro antitumour efficacy was tested using a panel of sarcoma cell lines, representative of Ewing sarcoma, osteosarcoma and rhabdomyosarcoma, the three most common paediatric sarcomas, and in normal human mesenchymal primary cell cultures. In vivo efficacy was tested against TC-71 Ewing sarcoma xenografts. Results Maltonis, a soluble maltol-derived synthetic molecule, was able to alter the DNA structure, inhibit proliferation and induce apoptotic cell death in paediatric sarcoma cells, either sensitive or resistant to some conventional chemotherapeutic drugs, such as doxorubicin and cisplatin. In addition, maltonis was able to induce: i) p21, p15 and Gadd45a mRNA upregulation; ii) Bcl-2, survivin, CDK6 and CDK8 down-regulation; iii) formation of γ-H2AX nuclear foci; iv) cleavage of PARP and Caspase 3. Two independent in vivo experiments demonstrated the tolerability and efficacy of maltonis in the inhibition of tumour growth. Finally maltonis was not extruded by ABCB1, one of the major determinants of chemotherapy failure, nor appeared to be a substrate of the glutathione-related detoxification system. Conclusions Considering that treatment of poorly responsive patients still suffers for the paucity of agents able to revert chemoresistance, maltonis may be considered for the future development of new therapeutic approaches for refractory metastatic patients. PMID:24575739

  5. Diagnostic Yield of Computed Tomography-Guided Coaxial Core Biopsy of Undetermined Masses in the Free Retroperitoneal Space: Single-Center Experience

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Stattaus, Joerg, E-mail: joerg.stattaus@uni-due.de; Kalkmann, Janine, E-mail: janine.kalkmann@uk-essen.de; Kuehl, Hilmar, E-mail: hilmar.kuehl@uni-due.d

    2008-09-15

    The purpose of this study was to evaluate the diagnostic yield of core biopsy in coaxial technique under guidance of computed tomography (CT) for retroperitoneal masses. We performed a retrospective analysis of CT-guided coaxial core biopsies of undetermined masses in the non-organ-bound retroperitoneal space in 49 patients. In 37 cases a 15-G guidance needle with a 16-G semiautomated core biopsy system, and in 12 cases a 16-G guidance needle with an 18-G biopsy system, was used. All biopsies were technically successful. A small hematoma was seen in one case, but no relevant complication occurred. With the coaxial technique, up tomore » 4 specimens were obtained from each lesion (mean, 2.8). Diagnostic accuracy in differentiation between malignant and benign diseases was 95.9%. A specific histological diagnosis could be established in 39 of 42 malignant lesions (92.9%). Correct subtyping of malignant lymphoma according to the WHO classification was possible in 87.0%. Benign lesions were correctly identified in seven cases, although a specific diagnosis could only be made in conjunction with clinical and radiological information. In conclusion, CT-guided coaxial core biopsy provides safe and accurate diagnosis of retroperitoneal masses. A specific histological diagnosis, which is essential for choosing the appropriate therapy, could be established in most cases of malignancy.« less

  6. [Anorectal manifestations of sexually transmissible diseases. Kaposi's sarcoma].

    PubMed

    Libeskind, M; Malbran, J; Agard, D; Pannetier, C; Lecouillard, C; Ivanovic, A

    1984-01-01

    The proctologist is above all concerned with the known recrudescence of venereal diseases. Examples reviewed are diseases of bacterial origin (syphilis, gonorrhea, soft chancre, donovanosis and chlamydiosis), appropriate antibiotic therapy and diseases of viral origin (herpes, condyloma acuminatum). Also noted are other bacterial, viral and parasitic diseases and, indeed, cancers of which Kaposi's sarcoma is the example, even though these are not manifested anorectally. New data on Kaposi's sarcoma, its' relationships with venereal disease and AIDS are presented. With these complex problems, the central role of male homosexuality and lowered cellular immunity widens considerably the professional scope of the proctologist.

  7. Ewing's sarcoma of the cervix, a diagnostic dilemma: a case report and review of the literature.

    PubMed

    Mashriqi, Nazia; Gujjarlapudi, Jaya Kranthi; Sidhu, Jagmohan; Zur, Michael; Yalamanchili, Madhuri

    2015-11-09

    Ewing's sarcoma belongs to a spectrum of neoplastic diseases known as Ewing's family of tumors. This family of tumors is usually seen in osseous sites. Ewing's sarcoma of the cervix is extremely rare, with only 18 cases reported in the English literature. The immunohistochemical profile of Ewing's sarcoma overlaps with other malignancies like small cell carcinoma. The rarity and complex pathologic picture of Ewing's sarcoma of the cervix creates the potential for misdiagnosis. Hence, we believe this case needs to be reported to add to the available literature. A 49-year-old white Caucasian woman presented with vaginal bleeding. A pelvic examination revealed a cystic lesion arising from her cervix. Examination of a biopsy specimen revealed a poorly differentiated neoplasm, with sheets of small hyperchromatic cells, staining weakly for neuroendocrine markers. She was diagnosed with small cell carcinoma and started on concurrent chemotherapy and radiation. However, additional positive immunostaining for CD99 was strongly suggestive of Ewing's sarcoma. Fluorescence in situ hybridization revealed ESWR1 gene rearrangement, confirming Ewing's sarcoma. Our patient underwent surgery, which confirmed stage IIB Ewing's sarcoma. She received adjuvant chemotherapy but died from progressive metastatic disease after four cycles. With early diagnosis and appropriate treatment, Ewing's sarcoma of the cervix can be a potentially curable disease. However, owing to overlapping clinical and histopathological features, the diagnosis poses a challenge to oncologists and pathologists. This article guides pathologists to consider Ewing's sarcoma in the differential diagnosis of small cell carcinoma with weak staining for neuroendocrine markers. This literature review will benefit oncologists encountering this rare entity.

  8. Robust diagnosis of Ewing sarcoma by immunohistochemical detection of super-enhancer-driven EWSR1-ETS targets

    PubMed Central

    Marchetto, Aruna; Gerke, Julia S.; Rubio, Rebeca Alba; Kiran, Merve M.; Musa, Julian; Knott, Maximilian M. L.; Ohmura, Shunya; Li, Jing; Akpolat, Nusret; Akatli, Ayse N.; Özen, Özlem; Dirksen, Uta; Hartmann, Wolfgang; de Alava, Enrique; Baumhoer, Daniel; Sannino, Giuseppina; Kirchner, Thomas; Grünewald, Thomas G. P.

    2018-01-01

    Ewing sarcoma is an undifferentiated small-round-cell sarcoma. Although molecular detection of pathognomonic EWSR1-ETS fusions such as EWSR1-FLI1 enables definitive diagnosis, substantial confusion can arise if molecular diagnostics are unavailable. Diagnosis based on the conventional immunohistochemical marker CD99 is unreliable due to its abundant expression in morphological mimics. To identify novel diagnostic immunohistochemical markers for Ewing sarcoma, we performed comparative expression analyses in 768 tumors representing 21 entities including Ewing-like sarcomas, which confirmed that CIC-DUX4-, BCOR-CCNB3-, EWSR1-NFATc2-, and EWSR1-ETS-translocated sarcomas are distinct entities, and revealed that ATP1A1, BCL11B, and GLG1 constitute specific markers for Ewing sarcoma. Their high expression was validated by immunohistochemistry and proved to depend on EWSR1-FLI1-binding to highly active proximal super-enhancers. Automated cut-off-finding and combination-testing in a tissue-microarray comprising 174 samples demonstrated that detection of high BCL11B and/or GLG1 expression is sufficient to reach 96% specificity for Ewing sarcoma. While 88% of tested Ewing-like sarcomas displayed strong CD99-immunoreactivity, none displayed combined strong BCL11B- and GLG1-immunoreactivity. Collectively, we show that ATP1A1, BCL11B, and GLG1 are EWSR1-FLI1 targets, of which BCL11B and GLG1 offer a fast, simple, and cost-efficient way to diagnose Ewing sarcoma by immunohistochemistry. These markers may significantly reduce the number of misdiagnosed patients, and thus improve patient care. PMID:29416716

  9. The cost-saving effect of centralized histological reviews with soft tissue and visceral sarcomas, GIST, and desmoid tumors: The experiences of the pathologists of the French Sarcoma Group

    PubMed Central

    Rascle, Pauline; Morelle, Magali; Toulmonde, Maud; Ranchere Vince, Dominique; Le Cesne, Axel; Terrier, Philippe; Neuville, Agnès; Meeus, Pierre; Farsi, Fadila; Ducimetière, Françoise; Blay, Jean-Yves; Ray Coquard, Isabelle; Coindre, Jean-Michel

    2018-01-01

    Objective This study examined the types of discordance occurring in the diagnosis of soft tissue and visceral sarcomas, gastrointestinal stromal tumors (GIST), and desmoid tumors, as well as the economic impact of diagnostic discrepancies. Methods We carried out a retrospective, multicenter analysis using prospectively implemented databases performed on a cohort of patients within the French RRePS network in 2010. Diagnoses were deemed to be discordant based on the 2013 World Health Organization (WHO) classification. Predictive factors of discordant diagnoses were explored. A decision tree was used to assess the expected costs of two strategies of disease management: one based on revised diagnoses after centralized histological review (option 1), the other on diagnoses without centralized review (option 2). Both were defined based on the patient and the disease characteristics, according to national or international guidelines. The time horizon was 12 months and the perspective of the French National Health Insurance (NHI) was retained. Costs were expressed in Euros for 2013. Sensitivity analyses were performed using low and high scenarios that included ± 20% estimates for cost. Results A total of 2,425 patients were included. Three hundred forty-one patients (14%) had received discordant diagnoses. These discordances were determined to mainly be benign tumors diagnosed as sarcomas (n = 124), or non-sarcoma malignant tumors diagnosed as sarcomas (n = 77). The probability of discordance was higher for a final diagnosis of desmoid tumors when compared to liposarcomas (odds ratio = 5.1; 95%CI [2.6–10.4]). The expected costs per patient for the base-case analysis (low- and high-case scenarios) amounted to €8,791 (€7,033 and €10,549, respectively) for option 1 and €8,904 (€7,057 and €10,750, respectively) for option 2. Conclusions Our findings highlight misdiagnoses of sarcomas, which were found to most often be confused with benign tumors. Centralized

  10. Massive retroperitoneal haemorrhage after extracorporeal shock wave lithotripsy (ESWL).

    PubMed

    Inoue, Hiromasa; Kamphausen, Thomas; Bajanowski, Thomas; Trübner, Kurt

    2011-01-01

    A 76-year-old male suffering from nephrolithiasis developed a shock syndrome 5 days after extracorporal shock wave lithotripsy (ESWL). CT scan of the abdomen showed massive haemorrhage around the right kidney. Although nephrectomy was performed immediately, the haemorrhage could not be controlled. Numerous units of erythrocytes were transfused, but the patient died. The autopsy revealed massive retroperitoneal haemorrhage around the right kidney. The kidney showed a subcapsular haematoma and a rupture of the capsule. The right renal artery was dissected. The inferior vena cava was lacerated. Accordingly, a hemorrhagic shock as the cause of death was determined, which might mainly have resulted from the laceration of the inferior vena cava due to ESWL. ESWL seems to be a relatively non-invasive modality, but one of its severe complications is perirenal hematoma. The injuries of the blood vessels might have been caused by excessive shock waves. Subsequently, anticoagulation therapy had been resumed 3 days after EWSL, which might have triggered the haemorrhage. Physicians should note that a haemorrhage after an ESWL can occur and they should pay attention to the postoperative management in aged individuals especially when they are under anticoagulation therapy.

  11. Anaplastic sarcoma of the kidney.

    PubMed

    Labanaris, Apostolos; Zugor, Vahudin; Smiszek, Robert; Nützel, Reinhold; Kühn, Reinhard

    2009-02-15

    Wilms tumor can appear with a wide spectrum of morphologic features and can sometimes cover or delay the recognition of other clinicopathologic entities of the kidney. We present a case of a new tumor entity of the kidney, namely the anaplastic sarcoma of the kidney, a tumor of high malignancy.

  12. Next-Generation Sequence Analysis of the Genome of RFHVMn, the Macaque Homolog of Kaposi's Sarcoma (KS)-Associated Herpesvirus, from a KS-Like Tumor of a Pig-Tailed Macaque

    PubMed Central

    Bruce, A. Gregory; Ryan, Jonathan T.; Thomas, Mathew J.; Peng, Xinxia; Grundhoff, Adam; Tsai, Che-Chung

    2013-01-01

    The complete sequence of retroperitoneal fibromatosis-associated herpesvirus Macaca nemestrina (RFHVMn), the pig-tailed macaque homolog of Kaposi's sarcoma-associated herpesvirus (KSHV), was determined by next-generation sequence analysis of a Kaposi's sarcoma (KS)-like macaque tumor. Colinearity of genes was observed with the KSHV genome, and the core herpesvirus genes had strong sequence homology to the corresponding KSHV genes. RFHVMn lacked homologs of open reading frame 11 (ORF11) and KSHV ORFs K5 and K6, which appear to have been generated by duplication of ORFs K3 and K4 after the divergence of KSHV and RFHV. RFHVMn contained positional homologs of all other unique KSHV genes, although some showed limited sequence similarity. RFHVMn contained a number of candidate microRNA genes. Although there was little sequence similarity with KSHV microRNAs, one candidate contained the same seed sequence as the positional homolog, kshv-miR-K12-10a, suggesting functional overlap. RNA transcript splicing was highly conserved between RFHVMn and KSHV, and strong sequence conservation was noted in specific promoters and putative origins of replication, predicting important functional similarities. Sequence comparisons indicated that RFHVMn and KSHV developed in long-term synchrony with the evolution of their hosts, and both viruses phylogenetically group within the RV1 lineage of Old World primate rhadinoviruses. RFHVMn is the closest homolog of KSHV to be completely sequenced and the first sequenced RV1 rhadinovirus homolog of KSHV from a nonhuman Old World primate. The strong genetic and sequence similarity between RFHVMn and KSHV, coupled with similarities in biology and pathology, demonstrate that RFHVMn infection in macaques offers an important and relevant model for the study of KSHV in humans. PMID:24109218

  13. Giant kidney worm (Dioctophyma renale) infection mimicking retroperitoneal neoplasm.

    PubMed

    Sun, T; Turnbull, A; Lieberman, P H; Sternberg, S S

    1986-07-01

    A 50-year-old Chinese man was found by ultrasound and computed tomography to have a retroperitoneal mass in the right upper quadrant of the abdomen. At operation, a hemorrhagic cyst was detected at the upper pole of the right kidney adjacent to the adrenal gland. Microscopic examination revealed that the cyst wall was composed of granulomatous tissue loaded with eggs and cross-sections of parasites, identified as Dioctophyma renale. The eggs were characterized by a birefringent striated double wall. The presence of cross sections of adult worms of D. renale in human tissue has not been previously described. Another unique feature of this case was that the right kidney was intact, as examined grossly at laparotomy and by intravenous pyelography. Eggs were not detected in the urine.

  14. Pitfalls in soft tissue sarcoma imaging: chronic expanding hematomas.

    PubMed

    Jahed, Kiarash; Khazai, Behnaz; Umpierrez, Monica; Subhawong, Ty K; Singer, Adam D

    2018-01-01

    Solid or nodular enhancement is typical of soft tissue sarcomas although high grade soft tissue sarcomas and those with internal hemorrhage often appear heterogeneous with areas of nonenhancement and solid or nodular enhancement. These MRI findings often prompt an orthopedic oncology referral, a biopsy or surgery. However, not all masses with these imaging findings are malignant. We report the multimodality imaging findings of two surgically proven chronic expanding hematomas (CEH) with imaging features that mimicked sarcomas. A third case of nonenhancing CEH of the lower extremity is also presented as a comparison. It is important that in the correct clinical scenario with typical imaging findings, the differential diagnosis of a chronic expanding hematoma be included in the workup of these patients. An image-guided biopsy of nodular tissue within such masses that proves to be negative for malignancy should not necessarily be considered discordant. A correct diagnosis may prevent a morbid unnecessary surgery and may indicate the need for a conservative noninvasive follow-up with imaging.

  15. Fine-needle aspiration cytology of postirradiation sarcomas, including angiosarcoma, with immunocytochemical confirmation

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Silverman, J.F.; Lannin, D.L.; Larkin, E.W.

    1989-01-01

    Postirradiation sarcomas are an unusual but well-recognized late effect of cancer therapy. In this article, a fine-needle aspiration (FNA) series of four cases is presented. There were three female patients and one male patient, with an age range of 28-55 yr (mean, 41). Two of the patients were irradiated for uterine cervical carcinoma while the other two received irradiation for malignant lymphoma. The time interval to the development of the postirradiation sarcoma ranged from 10 to greater than 20 yr. There were a postirradiation synovial sarcoma of the buttock region, malignant fibrous histiocytoma of the bone (femur), and rhabdomyosarcoma andmore » angiosarcoma of the retroperitoneum. A spectrum of cytologic findings was encountered, reflecting the specific types of sarcomas. Immunocytochemical studies performed on the aspirated material from the angiosarcoma demonstrated the utility of immunoperoxidase stains for ULEX europaeus agglutinin-1 (UEA-1) and, to a lesser degree, factor VIII-related antigen antibody, confirming the vascular nature of this malignancy. The FNA findings from all four cases demonstrated cytologic features that allowed recognition of this unusual complication of irradiation treatment. This article confirms the utility of FNA cytology in following patients with previous malignancies and differentiating a postirradiation sarcoma from recurrent carcinoma.« less

  16. Kaposi's sarcoma: an opportunistic infection by human herpesvirus-8 in ulcerative colitis.

    PubMed

    Rodríguez-Peláez, María; Fernández-García, María Soledad; Gutiérrez-Corral, Natalia; de Francisco, Ruth; Riestra, Sabino; García-Pravia, Carmen; Rodríguez, José Ignacio; Rodrigo, Luis

    2010-11-01

    Kaposi's sarcoma is a vascular tumor caused by human herpesvirus-8 infection. Iatrogenic Kaposi's sarcoma often occurs in patients receiving immunosuppressive therapy. To date, a few cases of colonic Kaposi's sarcoma have been reported in ulcerative colitis patients treated with immunomodulators. We describe a 65-year-old male diagnosed with left-sided ulcerative colitis who was treated with methotrexate and low-dose steroids for greater than 6 years. He presented with several papular, violet lesions on both legs. Colonoscopy revealed the presence of multiple reddish, elevated lesions in the sigmoid colon and rectum. Histological evaluation of skin and colonic biopsies showed findings suggestive of Kaposi's sarcoma; immunohistochemistry for human herpesvirus-8 was positive in the colonic lesions. To avoid the need for further immunosuppressive treatment, the patient underwent a colectomy. Following immunomodulator discontinuation, the patient experienced spontaneous regression of his skin lesions. With the present case, we wish to stress the important interaction of immunosuppressive therapy (mainly corticosteroids) used in ulcerative colitis patients in relation to the development of colonic Kaposi's sarcoma. Human herpesvirus-8 infection should be recognized as a possible opportunistic infection in patients with inflammatory bowel disease. Copyright © 2010 European Crohn's and Colitis Organisation. Published by Elsevier B.V. All rights reserved.

  17. A Case of Myxoid Liposarcoma of the Retroperitoneum: A Challenging Tumour for Diagnosis and Treatment

    PubMed Central

    Marino, Fabio; Simone, Michele

    2014-01-01

    Retroperitoneal sarcomas are rare neoplasms that account for only 1%-2% of all solid tumors and liposarcomas represent the most frequent histological type. We describe the case of a 44-year-old female with a retroperitoneal myxoid liposarcoma of 22 × 19 × 8 cm in size. The only symptoms were persistent pain and progressive tenderness of the abdomen lasting for two months. The mass was radically excised during laparotomy. CT and MRI were useful to clarify the site of origin of the tumor, relationships with other organs, and planning surgery but final diagnosis was based on histological findings. Here we review the literature about the challenging diagnosis, treatment, and prognostic factors of this disease. PMID:25024863

  18. Recursive partitioning analysis (RPA) classification predicts survival in patients with brain metastases from sarcoma.

    PubMed

    Grossman, Rachel; Ram, Zvi

    2014-12-01

    Sarcoma rarely metastasizes to the brain, and there are no specific treatment guidelines for these tumors. The recursive partitioning analysis (RPA) classification is a well-established prognostic scale used in many malignancies. In this study we assessed the clinical characteristics of metastatic sarcoma to the brain and the validity of the RPA classification system in a subset of 21 patients who underwent surgical resection of metastatic sarcoma to the brain We retrospectively analyzed the medical, radiological, surgical, pathological, and follow-up clinical records of 21 patients who were operated for metastatic sarcoma to the brain between 1996 and 2012. Gliosarcomas, sarcomas of the head and neck with local extension into the brain, and metastatic sarcomas to the spine were excluded from this reported series. The patients' mean age was 49.6 ± 14.2 years (range, 25-75 years) at the time of diagnosis. Sixteen patients had a known history of systemic sarcoma, mostly in the extremities, and had previously received systemic chemotherapy and radiation therapy for their primary tumor. The mean maximal tumor diameter in the brain was 4.9 ± 1.7 cm (range 1.7-7.2 cm). The group's median preoperative Karnofsky Performance Scale was 80, with 14 patients presenting with Karnofsky Performance Scale of 70 or greater. The median overall survival was 7 months (range 0.2-204 months). The median survival time stratified by the Radiation Therapy Oncology Group RPA classes were 31, 7, and 2 months for RPA class I, II, and III, respectively (P = 0.0001). This analysis is the first to support the prognostic utility of the Radiation Therapy Oncology Group RPA classification for sarcoma brain metastases and may be used as a treatment guideline tool in this rare disease. Copyright © 2014 Elsevier Inc. All rights reserved.

  19. Solitary Bone Plasmacytoma Progressing into Retroperitoneal Plasma Cell Myeloma with No Related End Organ or Tissue Impairment: A Case Report and Review of the Literature

    PubMed Central

    Tikku, Gargi; Jain, Monica; Mridha, Asit; Grover, Rajesh

    2014-01-01

    Solitary bone plasmacytomas and plasma cell myeloma are clonal proliferations of plasma cells. Many patients with solitary bone plasmacytomas develop plasma cell myeloma on follow-up. We present a case of a 70-year-old man who presented with fracture and a lytic lesion in the subtrochanteric region of the left femur and was assigned a diagnosis of solitary bone plasmacytoma. He received local curative radiotherapy. However, 4 months later his serum M protein and β2-microglobulin levels increased to 2.31 g/dL and 5.965 mg/L, respectively. He complained of abdominal fullness and constipation. Ultrasound and non-contrast CT imaging revealed multiple retroperitoneal masses. Colonoscopic examination was normal. Biopsy of the a retroperitoneal mass confirmed it to be a plasmacytoma. Repeat hemogram, blood urea, serum creatinine, skeletal survey, and bone marrow examination revealed no abnormalities. This is an unusual presentation of plasma cell myeloma, which manifested as multiple huge extramedullary retroperitoneal masses and arose from a solitary bone plasmacytoma, without related end organ or tissue impairment and bone marrow plasmacytosis. The patient succumbed to his disease 8 months after the appearance of the retroperitoneal masses. This case highlights the importance of close monitoring of patients diagnosed with solitary bone plasmacytoma with increased serum M protein and serum β2-microglobulin levels, so that early therapy can be instituted to prevent conversion to plasma cell myeloma. PMID:25330522

  20. Treatment Options by Stage (Uterine Sarcoma)

    MedlinePlus

    ... stage of the cancer being treated. External and internal radiation therapy are used to treat uterine sarcoma, and may also be used as palliative therapy to relieve symptoms and improve quality of life . Chemotherapy Chemotherapy is a cancer treatment ...

  1. Isolated Limb Perfusion of Melphalan With or Without Tumor Necrosis Factor in Treating Patients With Soft Tissue Sarcoma of the Arm or Leg

    ClinicalTrials.gov

    2012-03-14

    Stage IVB Adult Soft Tissue Sarcoma; Stage IIB Adult Soft Tissue Sarcoma; Stage IIC Adult Soft Tissue Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Stage IVA Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma

  2. [Inhibitory effect of taspine on mouse S180 sarcoma and its mechanism].

    PubMed

    Zhang, Yan-Min; He, Lang-Chong; Wang, Hong-Ying

    2007-05-01

    To study the inhibition effect of taspine on mouse S180 sarcoma and its mechanism. The mouse S180 sarcoma model was established and used to observe the antitumor activity of taspine. The microvessel density and protein expressing of the VEGF, bFGF, Bcl-2 and Bax in the tumor were measured by immunohistochemistry. Taspine showed antitumor activity on the mouse S180 sarcoma in a good dose-dependent manner. The inhibition rates on tumor of taspine at low, middle and high concentrations were 39.08% , 43.99% and 48.60%, respectively. The microvessel density and protein expressing of the VEGF, bFGF, Bcl-2 and Bax in the tumor were decreased compared with the negative control. The ratio of Bax to Bcl-2 was increased. Taspine has antitumor effect on the S180 sarcoma, and the mechanism may be through the way of decreasing the expressing of the VEGF, bFGF, Bcl-2 and Bax and inducing the vascular endothelial cell apoptosis.

  3. Characterization of a distinct subgroup of high-risk persons with Kaposi's sarcoma and good prognosis who present with normal T4 cell number and T4:T8 ratio and negative HTLV-III/LAV serologic test results.

    PubMed

    Afrasiabi, R; Mitsuyasu, R T; Nishanian, P; Schwartz, K; Fahey, J L

    1986-12-01

    Three homosexual male patients with biopsy-proved Kaposi's sarcoma were classified as having the acquired immune deficiency syndrome (AIDS) by Centers for Disease Control criteria when first seen in 1983 and 1984. These patients, however, differed from most patients with AIDS and Kaposi's sarcoma in having normal CD4 cell numbers and normal CD4:CD8 ratio. Furthermore, these immunologic parameters remained normal for eight to 24 months of follow-up, and the disease did not progress. Results of recent testing of serum from these patients were negative for HTLV-III/LAV antibodies. The Kaposi's sarcoma was limited to skin (stage I tumors) and the patients did not have persistent lymphadenopathy, fever, night sweats, or weight loss. In contrast to AIDS, the serum immunoglobulin levels (IgG, IgA, IgM) and number of B cells that were spontaneously forming immunoglobulin were within normal range with no evidence of polyclonal activation. The lymphocyte proliferative responses to phytohemagglutinin and Candida were reduced in two of the three patients, and skin test anergy was observed in the two patients tested. These findings are not frequently encountered in other healthy, homosexually active men or in classic Kaposi's sarcoma. They may be indicative of functional T cell changes (without numerical changes) induced by factors other than HTLV-III/LAV virus, which made these homosexually active men susceptible to development of low-grade Kaposi's sarcoma lesions.

  4. Application of modified R.E.N.A.L. nephrometry score system in evaluating the retroperitoneal partial nephrectomy for T1 renal cell carcinoma.

    PubMed

    Wang, Qinzhang; Qian, Biao; Li, Qiang; Ni, Zhao; Li, Yinglong; Wang, Xinmin

    2015-01-01

    This study aims to investigate the application of the modified R.E.N.A.L. nephrometry score system in evaluating the operation difficulty of retroperitoneal partial nephrectomy in T1 renal cell carcinoma patients. A total of 52 patients with T1 renal cell carcinoma were enrolled. They all had retroperitoneal partial nephrectomy. Their clinical data was retrospectively analyzed. R.E.N.A.L. nephrometry score system was modified based on the features of retroperitoneal partial nephrectomy. The specificity, sensitivity and Youden index were compared between R.E.N.A.L. nephrometry score system and the modified R.E.N.A.L. nephrometry score system. The effect of the modified R.E.N.A.L. nephrometry score system on perioperative outcomes was analyzed. Three degrees of operation difficulty were defined by the modified R.E.N.A.L. nephrometry score system, which included the low, medium and high degree of operation difficulty. The specificity, sensitivity and Youden index of the modified R.E.N.A.L. nephrometry score system were better than those of the original R.E.N.A.L. nephrometry score system. Compared with low degree of operation difficulty, patients with medium and high degree of operation difficulty had significantly higher levels of operative time, warm ischemia time, and intraoperative blood loss (P < 0.05). And, the levels of operative time, warm ischemia time, and intraoperative blood loss in patients with high degree were significantly higher than those in patients with medium degree (P < 0.05). The modified R.E.N.A.L. nephrometry score system has a good effect in evaluating the operation difficulty of retroperitoneal partial nephrectomy.

  5. Profiling of potential driver mutations in sarcomas by targeted next generation sequencing.

    PubMed

    Andersson, Carola; Fagman, Henrik; Hansson, Magnus; Enlund, Fredrik

    2016-04-01

    Comprehensive genetic profiling by massively parallel sequencing, commonly known as next generation sequencing (NGS), is becoming the foundation of personalized oncology. For sarcomas very few targeted treatments are currently in routine use. In clinical practice the preoperative diagnostic workup of soft tissue tumours largely relies on core needle biopsies. Although mostly sufficient for histopathological diagnosis, only very limited amounts of formalin fixated paraffin embedded tissue are often available for predictive mutation analysis. Targeted NGS may thus open up new possibilities for comprehensive characterization of scarce biopsies. We therefore set out to search for driver mutations by NGS in a cohort of 55 clinically and morphologically well characterized sarcomas using low input of DNA from formalin fixated paraffin embedded tissues. The aim was to investigate if there are any recurrent or targetable aberrations in cancer driver genes in addition to known chromosome translocations in different types of sarcomas. We employed a panel covering 207 mutation hotspots in 50 cancer-associated genes to analyse DNA from nine gastrointestinal stromal tumours, 14 synovial sarcomas, seven myxoid liposarcomas, 22 Ewing sarcomas and three Ewing-like small round cell tumours at a large sequencing depth to detect also mutations that are subclonal or occur at low allele frequencies. We found nine mutations in eight different potential driver genes, some of which are potentially actionable by currently existing targeted therapies. Even though no recurrent mutations in driver genes were found in the different sarcoma groups, we show that targeted NGS-based sequencing is clearly feasible in a diagnostic setting with very limited amounts of paraffin embedded tissue and may provide novel insights into mesenchymal cell signalling and potentially druggable targets. Interestingly, we also identify five non-synonymous sequence variants in 4 established cancer driver genes in DNA

  6. Long noncoding RNA EWSAT1-mediated gene repression facilitates Ewing sarcoma oncogenesis

    PubMed Central

    Marques Howarth, Michelle; Simpson, David; Ngok, Siu P.; Nieves, Bethsaida; Chen, Ron; Siprashvili, Zurab; Vaka, Dedeepya; Breese, Marcus R.; Crompton, Brian D.; Alexe, Gabriela; Hawkins, Doug S.; Jacobson, Damon; Brunner, Alayne L.; West, Robert; Mora, Jaume; Stegmaier, Kimberly; Khavari, Paul; Sweet-Cordero, E. Alejandro

    2014-01-01

    Chromosomal translocation that results in fusion of the genes encoding RNA-binding protein EWS and transcription factor FLI1 (EWS-FLI1) is pathognomonic for Ewing sarcoma. EWS-FLI1 alters gene expression through mechanisms that are not completely understood. We performed RNA sequencing (RNAseq) analysis on primary pediatric human mesenchymal progenitor cells (pMPCs) expressing EWS-FLI1 in order to identify gene targets of this oncoprotein. We determined that long noncoding RNA-277 (Ewing sarcoma–associated transcript 1 [EWSAT1]) is upregulated by EWS-FLI1 in pMPCs. Inhibition of EWSAT1 expression diminished the ability of Ewing sarcoma cell lines to proliferate and form colonies in soft agar, whereas EWSAT1 inhibition had no effect on other cell types tested. Expression of EWS-FLI1 and EWSAT1 repressed gene expression, and a substantial fraction of targets that were repressed by EWS-FLI1 were also repressed by EWSAT1. Analysis of RNAseq data from primary human Ewing sarcoma further supported a role for EWSAT1 in mediating gene repression. We identified heterogeneous nuclear ribonucleoprotein (HNRNPK) as an RNA-binding protein that interacts with EWSAT1 and found a marked overlap in HNRNPK-repressed genes and those repressed by EWS-FLI1 and EWSAT1, suggesting that HNRNPK participates in EWSAT1-mediated gene repression. Together, our data reveal that EWSAT1 is a downstream target of EWS-FLI1 that facilitates the development of Ewing sarcoma via the repression of target genes. PMID:25401475

  7. Risk of second bone sarcoma following childhood cancer: role of radiation therapy treatment.

    PubMed

    Schwartz, Boris; Benadjaoud, Mohamed Amine; Cléro, Enora; Haddy, Nadia; El-Fayech, Chiraz; Guibout, Catherine; Teinturier, Cécile; Oberlin, Odile; Veres, Cristina; Pacquement, Hélène; Munzer, Martine; N'guyen, Tan Dat; Bondiau, Pierre-Yves; Berchery, Delphine; Laprie, Anne; Hawkins, Mike; Winter, David; Lefkopoulos, Dimitri; Chavaudra, Jean; Rubino, Carole; Diallo, Ibrahima; Bénichou, Jacques; de Vathaire, Florent

    2014-05-01

    Bone sarcoma as a second malignancy is rare but highly fatal. The present knowledge about radiation-absorbed organ dose-response is insufficient to predict the risks induced by radiation therapy techniques. The objective of the present study was to assess the treatment-induced risk for bone sarcoma following a childhood cancer and particularly the related risk of radiotherapy. Therefore, a retrospective cohort of 4,171 survivors of a solid childhood cancer treated between 1942 and 1986 in France and Britain has been followed prospectively. We collected detailed information on treatments received during childhood cancer. Additionally, an innovative methodology has been developed to evaluate the dose-response relationship between bone sarcoma and radiation dose throughout this cohort. The median follow-up was 26 years, and 39 patients had developed bone sarcoma. It was found that the overall incidence was 45-fold higher [standardized incidence ratio 44.8, 95 % confidence interval (CI) 31.0-59.8] than expected from the general population, and the absolute excess risk was 35.1 per 100,000 person-years (95 % CI 24.0-47.1). The risk of bone sarcoma increased slowly up to a cumulative radiation organ absorbed dose of 15 Gy [hazard ratio (HR) = 8.2, 95 % CI 1.6-42.9] and then strongly increased for higher radiation doses (HR for 30 Gy or more 117.9, 95 % CI 36.5-380.6), compared with patients not treated with radiotherapy. A linear model with an excess relative risk per Gy of 1.77 (95 % CI 0.6213-5.935) provided a close fit to the data. These findings have important therapeutic implications: Lowering the radiation dose to the bones should reduce the incidence of secondary bone sarcomas. Other therapeutic solutions should be preferred to radiotherapy in bone sarcoma-sensitive areas.

  8. Hysteroscopic diagnosis of uterine sarcomas at the Department of Gynaecology, Sant Joan de Déu University Hospital.

    PubMed

    Gonzalez-Bosquet, E; Suñol, M; Torralba, A; Lozada, C; Miñano, J; Lailla, J M

    2011-01-01

    Uterine sarcomas are rare and the clinical diagnosis of sarcoma is difficult. Diagnostic and surgical hysteroscopy is a minimally invasive outpatient procedure that makes an accurate diagnosis of malignant intrauterine pathology and could play a role in the diagnosis of the uterine sarcomas. Uterine sarcomas diagnosed at the Department of Gynecology of Sant Joan University Hospital by hysteroscopy between January 2004 and August 2010 are described. In this period 2,441 hysteroscopies were performed; a total of 67 adenocarcinomas of the endometrium and five sarcomas were diagnosed by hysteroscopy. The data are presented with a review of the literature, focusing on the diagnostic value of hysteroscopy in these tumors.

  9. Survival of patients with Ewing's sarcoma in Yazd-Iran.

    PubMed

    Akhavan, Ali; Binesh, Fariba; Shamshiri, Hadi; Ghanadi, Fazllolah

    2014-01-01

    The Ewing's sarcoma family is a group of small round cell tumors which accounts for 10-15% of all primary bone neoplasms. The aim of this study was to evaluate the survival of Ewing's sarcoma patients in our province and to determine of influencing factors. All patients with documented Ewing's sarcoma/ primitive neuroectodermal tumor(PNET) family pathology were enrolled in this study during a period of eight years. For all of them local and systemic therapy were carried out. Overall and event free survival and prognostic factors were evaluated. Thirty two patients were enrolled in the study. The median age was 17.5 years. Twenty (65.2%) were male and 9 (28.1%) were aged 14 years or less. Mean disease free survival was 26.8 (95%CI; 13.8-39.9) months and five year disease free survival was 26%. Mean overall survival was 38.7 months (95%CI; 25.9-50.6) and median overall survival was 24 months. Five year overall survival was 25%. From the variables evaluated , only presence of metastatic disease at presentation (p value=0. 028) and complete response (p value =0. 006) had significant relations to overall survival. Survival of Ewing's sarcoma in our province is disappointing. It seems to be mostly due to less effective treatment. Administration of adequate chemotherapy dosage, resection of tumor with negative margins and precise assessment of irradiation volume may prove helpful.

  10. Blocking autophagy enhances the apoptotic effect of 18β-glycyrrhetinic acid on human sarcoma cells via endoplasmic reticulum stress and JNK activation.

    PubMed

    Shen, Shuying; Zhou, Menglu; Huang, Kangmao; Wu, Yizheng; Ma, Yan; Wang, Jiying; Ma, Jianjun; Fan, Shunwu

    2017-09-21

    Sarcoma, a rare form of cancer, is unlike the much more common carcinomas as it occurs in a distinct type of tissue. The potent antitumor effects of 18β-glycyrrhetinic acid (GA), a novel naturally derived agent, have been demonstrated in various cancers. However, the effect of GA on human sarcoma, and the underlying mechanisms, remain to be elucidated. In the current study, we show that GA inhibits sarcoma cell proliferation by inducing G0/G1-phase arrest. Exposure to GA resulted in the activation of caspase-3, -8, and -9, indicating that GA induced apoptosis through both extrinsic and intrinsic pathways. In addition, the autophagy pathway, characterized by the conversion of LC3-I to LC3- II, was activated, resulting in increased Beclin-1 protein levels, decreased p62 expression, and stimulation of autophagic flux. The present findings showed that GA stimulated autophagy by inducing endoplasmic reticulum (ER) stress via the IRE1-JNK pathway. Our data supported the prosurvival role of GA-induced autophagy when the autophagy pathway was blocked with specific chemical inhibitors. Finally, GA markedly reduced sarcoma growth, with little organ-related toxicity, in vivo. The present results suggest that the combination of GA with a specific autophagy inhibitor represents a promising therapeutic approach for the treatment of sarcoma.

  11. Ewing's Sarcoma: Development of RNA Interference-Based Therapy for Advanced Disease

    PubMed Central

    Simmons, Olivia; Maples, Phillip B.; Senzer, Neil; Nemunaitis, John

    2012-01-01

    Ewing's sarcoma tumors are associated with chromosomal translocation between the EWS gene and the ETS transcription factor gene. These unique target sequences provide opportunity for RNA interference(i)-based therapy. A summary of RNAi mechanism and therapeutically designed products including siRNA, shRNA and bi-shRNA are described. Comparison is made between each of these approaches. Systemic RNAi-based therapy, however, requires protected delivery to the Ewing's sarcoma tumor site for activity. Delivery systems which have been most effective in preclinical and clinical testing are reviewed, followed by preclinical assessment of various silencing strategies with demonstration of effectiveness to EWS/FLI-1 target sequences. It is concluded that RNAi-based therapeutics may have testable and achievable activity in management of Ewing's sarcoma. PMID:22523703

  12. Reverse transcriptase polymerase chain reaction on fine needle aspirates for rapid detection of translocations in synovial sarcoma.

    PubMed

    Nilsson, G; Wang, M; Wejde, J; Kanter, L; Karlén, J; Tani, E; Kreicbergs, A; Larsson, O

    1998-01-01

    To evaluate the utilization of fine needle aspiration (FNA) biopsy to obtain material for reverse-transcriptase polymerase chain reaction (RT-PCR) in the detection of the t(X;18)(p11.2;q11.2) translocation in synovial sarcomas. We applied RT-PCR to detection of synovial sarcoma fusion gene transcripts on fine needle aspirates. Five clinical samples were first analyzed: one was a tumor previously diagnosed as malignant hemangiopericytoma, one was a poorly defined tumor, and three were suspected synovial sarcomas. FNA material was transferred directly to the RT-PCR reaction tube without RNA extraction. The t(X;18) translocation could be detected on the limited amount of material that FNA provides. In each of the cases studied the representivity of the tumor samples was confirmed microscopically. Our protocol permits analysis directly on representative samples without extraction of RNA. The results imply that RT-PCR offers reliable detection of sarcoma fusion gene transcripts on fine needle aspirates. The procedure, apart from being applicable to outpatients, is rapid and sensitive.

  13. Preclinical Justification of pbi-shRNA EWS/FLI1 Lipoplex (LPX) Treatment for Ewing's Sarcoma.

    PubMed

    Rao, Donald D; Jay, Christopher; Wang, Zhaohui; Luo, Xiuquan; Kumar, Padmasini; Eysenbach, Hilary; Ghisoli, Maurizio; Senzer, Neil; Nemunaitis, John

    2016-08-01

    The EWS/FLI1 fusion gene is well characterized as a driver of Ewing's sarcoma. Bi-shRNA EWS/FLI1 is a functional plasmid DNA construct that transcribes both siRNA and miRNA-like effectors each of which targets the identical type 1 translocation junction region of the EWS/FLI1 transcribed mRNA sequence. Previous preclinical and clinical studies confirm the safety of this RNA interference platform technology and consistently demonstrate designated mRNA and protein target knockdown at greater than 90% efficiency. We initiated development of pbi-shRNA EWS/FLI1 lipoplex (LPX) for the treatment of type 1 Ewing's sarcoma. Clinical-grade plasmid was manufactured and both sequence and activity verified. Target protein and RNA knockdown of 85-92% was demonstrated in vitro in type 1 human Ewing's sarcoma tumor cell lines with the optimal bi-shRNA EWS/FLI1 plasmid. This functional plasmid was placed in a clinically tested, liposomal (LP) delivery vehicle followed by in vivo verification of activity. Type 1 Ewing's sarcoma xenograft modeling confirmed dose related safety and tumor response to pbi-shRNA EWS/FLI1 LPX. Toxicology studies in mini-pigs with doses comparable to the demonstrated in vivo efficacy dose resulted in transient fever, occasional limited hypertension at low- and high-dose assessment and transient liver enzyme elevation at high dose. These results provide the justification to initiate clinical testing.

  14. Preclinical Justification of pbi-shRNA EWS/FLI1 Lipoplex (LPX) Treatment for Ewing's Sarcoma

    PubMed Central

    Rao, Donald D.; Jay, Christopher; Wang, Zhaohui; Luo, Xiuquan; Kumar, Padmasini; Eysenbach, Hilary; Ghisoli, Maurizio; Senzer, Neil; Nemunaitis, John

    2016-01-01

    The EWS/FLI1 fusion gene is well characterized as a driver of Ewing's sarcoma. Bi-shRNA EWS/FLI1 is a functional plasmid DNA construct that transcribes both siRNA and miRNA-like effectors each of which targets the identical type 1 translocation junction region of the EWS/FLI1 transcribed mRNA sequence. Previous preclinical and clinical studies confirm the safety of this RNA interference platform technology and consistently demonstrate designated mRNA and protein target knockdown at greater than 90% efficiency. We initiated development of pbi-shRNA EWS/FLI1 lipoplex (LPX) for the treatment of type 1 Ewing's sarcoma. Clinical-grade plasmid was manufactured and both sequence and activity verified. Target protein and RNA knockdown of 85–92% was demonstrated in vitro in type 1 human Ewing's sarcoma tumor cell lines with the optimal bi-shRNA EWS/FLI1 plasmid. This functional plasmid was placed in a clinically tested, liposomal (LP) delivery vehicle followed by in vivo verification of activity. Type 1 Ewing's sarcoma xenograft modeling confirmed dose related safety and tumor response to pbi-shRNA EWS/FLI1 LPX. Toxicology studies in mini-pigs with doses comparable to the demonstrated in vivo efficacy dose resulted in transient fever, occasional limited hypertension at low- and high-dose assessment and transient liver enzyme elevation at high dose. These results provide the justification to initiate clinical testing. PMID:27166877

  15. An integrated chemical biology approach identifies specific vulnerability of Ewing's sarcoma to combined inhibition of Aurora kinases A and B.

    PubMed

    Winter, Georg E; Rix, Uwe; Lissat, Andrej; Stukalov, Alexey; Müllner, Markus K; Bennett, Keiryn L; Colinge, Jacques; Nijman, Sebastian M; Kubicek, Stefan; Kovar, Heinrich; Kontny, Udo; Superti-Furga, Giulio

    2011-10-01

    Ewing's sarcoma is a pediatric cancer of the bone that is characterized by the expression of the chimeric transcription factor EWS-FLI1 that confers a highly malignant phenotype and results from the chromosomal translocation t(11;22)(q24;q12). Poor overall survival and pronounced long-term side effects associated with traditional chemotherapy necessitate the development of novel, targeted, therapeutic strategies. We therefore conducted a focused viability screen with 200 small molecule kinase inhibitors in 2 different Ewing's sarcoma cell lines. This resulted in the identification of several potential molecular intervention points. Most notably, tozasertib (VX-680, MK-0457) displayed unique nanomolar efficacy, which extended to other cell lines, but was specific for Ewing's sarcoma. Furthermore, tozasertib showed strong synergies with the chemotherapeutic drugs etoposide and doxorubicin, the current standard agents for Ewing's sarcoma. To identify the relevant targets underlying the specific vulnerability toward tozasertib, we determined its cellular target profile by chemical proteomics. We identified 20 known and unknown serine/threonine and tyrosine protein kinase targets. Additional target deconvolution and functional validation by RNAi showed simultaneous inhibition of Aurora kinases A and B to be responsible for the observed tozasertib sensitivity, thereby revealing a new mechanism for targeting Ewing's sarcoma. We further corroborated our cellular observations with xenograft mouse models. In summary, the multilayered chemical biology approach presented here identified a specific vulnerability of Ewing's sarcoma to concomitant inhibition of Aurora kinases A and B by tozasertib and danusertib, which has the potential to become a new therapeutic option.

  16. Kaposi's sarcoma with visceral involvement after intraarticular and epidural injections of corticosteroids.

    PubMed

    Trattner, A; Hodak, E; David, M; Neeman, A; Sandbank, M

    1993-11-01

    Kaposi's sarcoma has been reported in patients receiving immunosuppressive therapy, most of whom are organ transplant recipients. The development of Kaposi's sarcoma after treatment with corticosteroids has been reported in only 38 patients who have not had acquired immunodeficiency syndrome or undergone organ transplantation. Cutaneous Kaposi's sarcoma developed 2 months after intraarticular steroid injections in a man with ulnar nerve entrapment. The lesions regressed spontaneously after 3 months but reappeared with visceral involvement 18 months later, shortly after initiation of a course of epidural steroid injections for treatment of low back pain. The cutaneous lesions and some visceral lesions rapidly regressed after cessation of treatment.

  17. Combination Therapy for Advanced Kaposi Sarcoma

    Cancer.gov

    In this clinical trial, adult patients with any form of advanced Kaposi sarcoma will be treated with liposomal doxorubicin and bevacizumab every 3 weeks for a maximum of six treatments.  Patients who respond to this therapy or have stable disease will rec

  18. BCOR-CCNB3-positive soft tissue sarcoma with round-cell and spindle-cell histology: a series of four cases highlighting the pitfall of mimicking poorly differentiated synovial sarcoma.

    PubMed

    Li, Wan-Shan; Liao, I-Chuang; Wen, Mei-Chin; Lan, Howard Haw-Chang; Yu, Shih-Chen; Huang, Hsuan-Ying

    2016-11-01

    BCOR-CCNB3 sarcoma is a genetically defined undifferentiated malignancy with Ewing sarcoma (ES)-like round cells, and preferentially affects the bones of male adolescents. Sarcomas harbouring BCOR-CCNB3 rarely arise from soft tissues; therefore, we aimed to report four cases to expand the clinicopathological spectrum. By reverse transcription polymerase chain reaction and confirmatory sequencing, we detected a BCOR-CCNB3 transcript in primary undifferentiated sarcomas of the deep musculature of four male patients, comprising two teenagers (aged 14 and 17 years) and two adults (aged 34 and 44 years). The tumours originated in the back (n = 2), pelvis (n = 1), and thigh (n = 1), and were 70-140 mm in size (mean, 107 mm). All tumours showed sheets of primitive round or ovoid cells with vesicular nuclei, active mitosis (28-41/10 high-power fields), variably prominent nucleoli, and geographical necrosis. This major component transformed into fascicles of elongated spindle cells with staghorn vessels and a myxoid reticular stroma, accounting for 10-50% of areas. All cases were positive for CD99, three were positive for TLE1, and one was positive for EMA, indicating poorly differentiated synovial sarcomas (PDSSs). Nuclear cyclin B3 reactivity was present in all cases, but not in molecularly confirmed atypical ESs and PDSSs. At the last follow-up (median, 13.5 months), one patient had died of lung metastasis, two were alive with tumours, and one was tumour-free. BCOR-CCNB3-positive sarcomas may primarily occur in soft tissues of adults and show PDSS-mimicking round-cell and spindle-cell histology with aggressive behaviour. Cyclin B3 is useful for selecting candidates for BCOR-CCNB3 molecular testing. © 2016 John Wiley & Sons Ltd.

  19. Survey among German gynecologists on the clinical management of patients with sarcomas of the uterus.

    PubMed

    Chen, Frank Chih-Kang; David, Matthias; Richter, Rolf; Muallem, Mustafa Zelal; Chekerov, Radoslav; Sehouli, Jalid

    2013-08-01

    To gain more information about the knowledge of the clinical management of uterine sarcoma. This survey was performed among members of the North-Eastern German Society of Gynecological Oncology (NOGGO) and the German Society of Psychosomatic Medicine in Gynecology and Obstetrics (DGPFG) on the treatment of uterine sarcomas. Altogether, 374 gynecologists took part. When asked about the surgical therapy of leiomyosarcoma, 64% indicated hysterectomy with bilateral adenectomy and lymph node dissection. Answers on the extent of lymphadenectomy in leiomyosarcoma differed widely. When asked about the preferred chemotherapy regimen for metastatic uterine sarcoma, more than 60% of all gynecologists would not apply any chemotherapy. Almost 40% recommended any kind of radiotherapy in this situation. There is a great uncertainty about the standard treatment of uterine sarcoma, even among specialists of gynecological oncology. It is time for organized efforts to improve the treatment of uterine sarcoma.

  20. Case report 673: Telangiectic osteogenic sarcoma.

    PubMed

    Liu, S K; Thacher, C

    1991-01-01

    A case is presented of a telangiectatic osteogenic sarcoma involving the left third metatarsal of a 4-year-old male Great Dane dog. Radiographs revealed a diaphyseal, expanding, lytic lesion with minimal intralesional sclerosis and a sclerotic rim in the proximal portion. The lesion contained a large amount of blood. The biopsy specimens consisted of spaces which were outlined by fibrous osteoid or granulation tissue. There were islands of multinuclear giant cells and/or fibrous osteoid tissue. A diagnosis of aneurysmal bone cyst was made. The lesion was treated by curettage and insertion of cancellous bone graft but was progressive 10 weeks after treatment. The lesion was further curetted, and these biopsy specimens consisted of aneurysmally dilated spaces and areas of anaplastic sarcomatous cells with mitotic figures and osteoid production, characteristic of telangiectatic osteogenic sarcoma. The dog was euthanized as the owner requested; an autopsy was not performed.