Science.gov

Sample records for retroperitoneal sarcoma results

  1. Giant retroperitoneal sarcomas.

    PubMed

    Ianoşi, G; Neagoe, Daniela; Buteică, Elena; Ianoşi, Simona; Drighiciu, Corina; Stănoiu, B; Burada, F; Mercuţ, D

    2007-01-01

    Retroperitoneal sarcomas are rare malignant tumors, which are developing from mesenchymal stem cells residing in muscle, fat, and connective tissues. Underlying the rarity of this kind of tumors in general population, the aim of this paper is to present three cases of retroperitoneal sarcomas operated in Surgical Department of Military Hospital of Craiova (a retroperitoneal liposarcoma, a dedifferentiated liposarcoma and a malignant fibrous histiocytoma). From clinical point of view, we note the poverty of symptoms and non-specificity of these and a great tolerability of retroperitoneal space that offers the possibility for a great development of the tumor. CT-scan and MRI are the best investigations for diagnosis but surgical exploration is the best way for a good evaluation of these tumors. From histological point of view, we try to present new features about these kinds of tumors in order to classify them. Results of surgery correlated with complementary therapies were good without per-operative mortality or postoperative morbidity but we noted a recidive of one tumor (with different histological pattern) 21 months after the surgical intervention. The rarity of retroperitoneal sarcomas, combined with the vast array of histologic subtypes, has complicated our understanding of these tumors and impeded the development of effective therapies.

  2. Practical Issues for Retroperitoneal Sarcoma.

    PubMed

    Pham, Vicky; Henderson-Jackson, Evita; Doepker, Matthew P; Caracciolo, Jamie T; Gonzalez, Ricardo J; Druta, Mihaela; Ding, Yi; Bui, Marilyn M

    2016-07-01

    Retroperitoneal sarcoma is rare. Using initial specimens on biopsy, a definitive diagnosis of histological subtypes is ideal but not always achievable. A retrospective institutional review was performed for all cases of adult retroperitoneal sarcoma from 1996 to 2015. A review of the literature was also performed related to the distribution of retroperitoneal sarcoma subtypes. A meta-analysis was performed. Liposarcoma is the most common subtype (45%), followed by leiomyosarcoma (21%), not otherwise specified (8%), and undifferentiated pleomorphic sarcoma (6%) by literature review. Data from Moffitt Cancer Center demonstrate the same general distribution for subtypes of retroperitoneal sarcoma. A pathology-based algorithm for the diagnosis of retroperitoneal sarcoma is illustrated, and common pitfalls in the pathology of retroperitoneal sarcoma are discussed. An informative diagnosis of retroperitoneal sarcoma via specimens on biopsy is achievable and meaningful to guide effective therapy. A practical and multidisciplinary algorithm focused on the histopathology is helpful for the management of retroperitoneal sarcoma.

  3. [Radiotherapy for retroperitoneal sarcomas].

    PubMed

    Sargos, P; Stoeckle, E; Henriques de Figueiredo, B; Antoine, M; Delannes, M; Mervoyer, A; Kantor, G

    2016-10-01

    The management of retroperitoneal sarcoma can be very challenging, and the quality of initial treatment strategy appears to be a crucial prognostic factor. En bloc surgery is currently the standard of care for these rare tumours and perioperative treatments such as chemotherapy or radiotherapy have not been validated yet. However, local-regional relapse constitutes the most common disease course. While adjuvant radiotherapy is less and less common due to gastrointestinal toxicities, preoperative radiation therapy offers numerous advantages and is being evaluated as part of a national multicentre phase II study (TOMOREP trial) and is the subject of a European randomized phase III study (STRASS trial). The objective of this article is to present data on preoperative irradiation in terms of dose, volumes and optimal radiotherapy techniques for the treatment of this rare disease.

  4. Radiation therapy for retroperitoneal sarcoma.

    PubMed

    Tuan, Jeffrey; Vitolo, Viviana; Vischioni, Barbara; Iannalfi, Alberto; Fiore, Maria Rosaria; Fossati, Piero; Orecchia, Roberto

    2014-10-01

    Retroperitoneal sarcomas (RPS) are rare tumours with an annual reported incidence of 2.7 per million persons. In spite of improvements in both diagnostic imaging and therapeutic strategies, patients afflicted by RPS still have poor prognoses. There are currently many different therapeutic strategies for these rare tumours and combining several different multi-modality strategies have not proved to have superior long-term clinical results. This review analyses the available published data and discusses multi-modality management of this rare entity. In particular, the role of radiation therapy, treatment-related side effects and the use of modern radiation treatment techniques will be discussed. A comprehensive literature search was conducted using PubMed in January 2011. Relevant international articles published from January 1980 to January 2011 were assessed. The keywords for search purposes were: retroperitoneum, sarcoma, radiotherapy, and radiation therapy. The search was limited to articles published in English. All articles were read in full by the authors and selected for inclusion based on relevance to this article. The addition of radiation therapy (RT) to wide surgical excision for RPS has improved local control rates when compared with surgery alone. Preoperative RT is preferred over postoperative RT. New types and delivery techniques in radiation therapy could further improve patient outcomes. Emerging therapies that employ charged particles (such as protons and carbon ions) are expected to be superior in sparing of normal tissues and efficacy over conventional photon therapy radiation, due to their physical and radiobiological properties.

  5. Carbon Ion Radiotherapy for Unresectable Retroperitoneal Sarcomas

    SciTech Connect

    Serizawa, Itsuko; Kagei, Kenji; Kamada, Tadashi; Imai, Reiko; Sugahara, Shinji; Okada, Tohru; Tsuji, Hiroshi; Ito, Hisao; Tsujii, Hirohiko

    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 histologic 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.

  6. Multimodality Local Therapy for Retroperitoneal Sarcoma

    SciTech Connect

    Paryani, Nitesh N.; Zlotecki, Robert A.; Swanson, Erika L.; Morris, Christopher G.; Grobmyer, Stephen R.; Hochwald, Steven N.; Marcus, Robert B.; Indelicato, Daniel J.

    2012-03-01

    Purpose: Soft-tissue sarcomas of the retroperitoneum are rare tumors comprising less than 1% of all malignancies. Although surgery continues as the mainstay of treatment, the large size of these tumors coupled with their proximity to critical structures make resection with wide margins difficult to achieve. The role and timing of radiotherapy are controversial. This study updates our institutional experience using multimodality local therapy for resectable retroperitoneal sarcoma and identifies prognostic factors impacting disease control and survival. Methods and Materials: Between 1974 and 2007, 58 patients with nonmetastatic retroperitoneal sarcoma were treated with surgery and radiation at University of Florida. The median age at radiotherapy was 57 years old (range, 18-80 years). Forty-two patients received preoperative radiotherapy and 16 received postoperative radiotherapy. Nineteen patients received 1.8 Gy once daily and 39 patients received 1.2 Gy twice daily. Variables analyzed for prognostic value included age, grade, kidney involvement, histology, de novo versus recurrent presentation, tumor diameter, margin status, radiotherapy sequencing (preoperative vs. postoperative), total radiation dose, fractionation scheme, and treatment era. Results: The 5-year overall survival, cause-specific survival, and local control rates were 49%, 58%, and 62%, respectively. Nearly two-thirds of disease failures involved a component of local progression. On multivariate analysis, only margin status was significantly associated with improved 5-year local control (85%, negative margins; 63%, microscopic positive margins; 0%, gross positive margins; p < 0.0001) and 5-year overall survival (64%, negative margins; 56%, microscopic positive margins; 13%, gross positive margins; p = 0.0012). Thirty-one Grade 3 or greater toxicities were observed in 22 patients, including two treatment-related deaths (3%). Conclusion: For retroperitoneal sarcoma, local control remains a

  7. Tumor Lysis Syndrome in a Retroperitoneal Sarcoma.

    PubMed

    Zakharia, Yousef; Mansour, Joshua; Vasireddi, Srinivasa; Zakharia, Kais; Fatakhov, Eduard; Koch, Christopher; Hrinczenko, Borys

    2014-01-01

    In the present case, a 49-year-old white female presented to the clinic with a 2-month history of nausea, vomiting, and right upper quadrant pain. On examination a 3-cm mass on the right anterior scalene muscle was noted. A computed tomography scan was performed revealing a 8.7 × 7.7 × 6.1 cm retroperitoneal mass with possible invasion of the inferior vena cava and right renal and left common iliac veins. An excisional biopsy was performed with pathology compatible with spindle cell sarcoma. The patient was then sent for follow-up at the sarcoma clinic as an outpatient. However, before chemotherapy was to be started the patient would be admitted to the hospital with progressively worse nausea and vomiting. At that time the patient's lab work showed lactic acidosis, acute renal failure, hyperuricemia, hyperphosphatemia, and hypocalcemia, which met the Cairo-Bishop criteria for tumor lysis syndrome (TLS). The patient was admitted to the intensive care unit and kidney dialysis initiated. The patient would become progressively obtunded at which time the family opted for hospice care. The patient eventually succumbed peacefully 3 days after her last admission. In this case report, we briefly review the literature on TLS in solid tumors, and we present a rare case of spontaneous TLS in a retroperitoneal sarcoma.

  8. [Retroperitoneal sarcoma: report of 6 cases].

    PubMed

    Joual, A; Faik, H; Rabii, R; Hafiani, M; Bennani, S; el Mrini, M; Benjelloun, S

    2000-06-01

    Retroperitoneal soft tissue sarcomas (RPS) are uncommon tumors. The diagnosis is frequently made later in the evolution of the disease due to the absence of specific symptomatology. Surgery with total resection of the tumor is the treatment of choice, but is only possible in 38 to 75% of cases. Six cases of RPS have been retrospectively reviewed; the mean time to diagnosis was 5 months; diagnosis was established via CT scan, which determined the retroperitoneal tumor location and its relationship to the neighboring organs. The most common symptoms were the presence of an abdominal mass and accompanying abdominal pain; signs of urinary and vascular compression were not found until later. Surgery with total resection was performed in 4 cases, and with partial resection in 2 cases. The histological findings were as follows: 3 liposarcomas, 2 rhabdomyosarcomas, and 1 fibrosarcoma. Tumor recurrence developed in 2 cases (liposarcomas), necessitating further surgery and complete resection. Two patients were lost to follow-up. RPS are characterized by locoregional relapse and metastases. Disease outcome depends on the histological type, tumor grade, and on the possibility of carrying out complete resection.

  9. Primary retroperitoneal mucinous cystadenoma with sarcoma-like mural nodule

    PubMed Central

    Bakker, R. F. R.; Stoot, J. H. M. B.; Blok, P.

    2007-01-01

    Primary retroperitoneal cystadenomas are extremely rare. This is the first report in literature to describe a primary retroperitoneal cystadenoma with a sarcoma-like mural nodule. A 45-year-old woman complained of a left-sided abdominal mass. A computed tomography scan revealed a cystic mass with a mural nodule, which seemed to originate from the tail of the pancreas. At laparotomy the cyst was not adhered to the pancreas but localized retroperitoneally. Histologic examination showed a mucinous cystadenoma with only foci of borderline malignancy with a mural “sarcoma-like” nodule. In view of the surgical and histopathological findings, the mucinous cystadenoma was regarded as primary retroperitoneal. This case demonstrates that in the era of radiological preoperative refinement, pathological diagnosis remains of utmost importance, especially for rare cases. PMID:17690906

  10. Prognostic factors predictive of survival for truncal and retroperitoneal soft-tissue sarcoma.

    PubMed Central

    Singer, S; Corson, J M; Demetri, G D; Healey, E A; Marcus, K; Eberlein, T J

    1995-01-01

    OBJECTIVE: The authors identified prognostic factors relevant to clinical outcomes (especially survival) in truncal and retroperitoneal soft-tissue sarcoma. SUMMARY BACKGROUND DATA: These results can be used to optimize surgical management and select patients most likely to benefit from novel therapeutic strategies in future trials. METHODS: A retrospective analysis was performed of a prospectively compiled database of 183 consecutive patients with truncal and retroperitoneal sarcomas seen at the Brigham and Women's Hospital and the Dana Farber Cancer Institute between 1970 to 1994. RESULTS: For truncal sarcoma, multivariate analysis showed that high-grade histology was associated with an eightfold increased risk of death compared with low-grade histology (p = 0.001). In addition to grade, gross positive margin of resection (p = 0.001), microscopic positive margin (p = 0.023), and tumors greater than 5 cm in size (p = 0.018) were important independent prognostic factors for survival. In this series, postoperative radiation therapy for truncal sarcoma was associated with a 2.4-fold decreased risk of death compared with truncal sarcoma patients receiving no adjuvant radiation therapy, having adjusted for the other prognostic factors (p = 0.030). In contrast, for retroperitoneal sarcoma, multivariate analysis showed that high-grade and intermediate-grade histology were associated with a five- to sixfold increased risk of death compared with low-grade histology (p = 0.009). In addition to grade, gross positive margin of resection (p = 0.001) and microscopic positive margin (p = 0.004) were important independent prognostic factors for survival in retroperitoneal sarcoma. Patients who received either preoperative or postoperative chemotherapy for retroperitoneal sarcoma had a 4.6-fold (p = 0.002) and 3-fold (p = 0.010) increased risk of death, respectively, compared with patients receiving no adjuvant chemotherapy, having adjusted for the other prognostic factors

  11. Needle tract seeding following core biopsies in retroperitoneal sarcoma.

    PubMed

    Van Houdt, W J; Schrijver, A M; Cohen-Hallaleh, R B; Memos, N; Fotiadis, N; Smith, M J; Hayes, A J; Van Coevorden, F; Strauss, D C

    2017-09-01

    Retroperitoneal tumours often require a preoperative core needle biopsy to establish a histological diagnosis. Literature is scarce regarding the risk of biopsies in retroperitoneal sarcomas, so the aim of this study is to identify the potential risks of core needle biopsies causing needle tract recurrences or local recurrences. Patients who underwent resection of a primary retroperitoneal sarcoma between 1990 and 2014 were identified from a prospectively maintained database from two tertiary referral centres. Patient demographics, tumour characteristics and biopsy techniques were examined. The primary endpoint was needle tract recurrence and local intra-abdominal recurrence. 498 patients were included in the analysis. The most common histological subtypes were liposarcoma (66%) and leiomyosarcoma (18%). Of the 498 patients that underwent resection, 255 patients were diagnosed with a preoperative biopsy. Five patients (2%) developed a biopsy site recurrence: 3 patients with leiomyosarcomas and 2 patients with dedifferentiated liposarcomas. All biopsy site recurrences occurred after trans-abdominal biopsies and were not performed with a co-axial technique. There was no significant difference in local recurrence rate between the patients with or without a biopsy (=0.30) or for the biopsy route (trans-abdominal or trans-retroperitoneal (p = 0.72)). The risk of a needle tract metastasis after core needle biopsy for retroperitoneal sarcoma is very low but not zero. The safest method seems a trans-retroperitoneal approach with a co-axial technique. Local recurrence rate is not altered after doing a core needle biopsy. Copyright © 2017 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.

  12. Primary retroperitoneal soft tissue sarcoma: Imaging appearances, pitfalls and diagnostic algorithm.

    PubMed

    Messiou, C; Moskovic, E; Vanel, D; Morosi, C; Benchimol, R; Strauss, D; Miah, A; Douis, H; van Houdt, W; Bonvalot, S

    2017-07-01

    Although retroperitoneal sarcomas are rare tumours, they can be encountered by a wide variety of clinicians as they can be incidental findings on imaging or present with non specific symptoms and signs. Surgical resection can offer hope of cure and patient outcomes are improved when patients are managed in high-volume specialist centers. Failure to recognize retroperitoneal sarcomas on imaging can lead to inappropriate management in inexperienced centers. Therefore it is critical that a diagnosis of retroperitoneal sarcoma should be considered in the differential diagnosis of a retroperitoneal mass with prompt referral to a soft tissue sarcoma unit. In particular, the most common retroperitoneal sarcoma subtypes, liposarcoma and leiomyosarcoma, have characteristic imaging appearances which are discussed. This review therefore aims to set the context and guide clinicians through a diagnostic pathway for retroperitoneal masses in adults which arise extrinsic to the solid abdominal viscera. Copyright © 2016 The Authors. Published by Elsevier Ltd.. All rights reserved.

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

    SciTech Connect

    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

    2015-08-01

    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.

  14. Treatment of retroperitoneal sarcoma: current standards and new developments.

    PubMed

    van Houdt, Winan J; Zaidi, Shane; Messiou, Christina; Thway, Khin; Strauss, Dirk C; Jones, Robin L

    2017-07-01

    Retroperitoneal sarcomas are rare tumors and with complex treatment. In this manuscript we give an overview of current standards in treatment of this disease and discuss new developments. Surgery with complete resection of the primary tumor is still the only curative modality. The role of preoperative radiotherapy is not clear and is currently being investigated in a clinical trial. Neo-adjuvant chemotherapy is not the standard of care but can be considered occasionally when complete resection is uncertain. Local and distant recurrent disease carries a dismal prognosis, although long-term survival can be achieved. Liposarcomas tend to recur locally, whereas distant recurrences are more often seen in leiomyosarcoma and other subtypes. Outcome improves when patients are treated in high volume sarcoma centers. In the metastatic setting, newer systemic agents have recently been approved. Treatment of retroperitoneal sarcomas is complex and all patients should be treated in multidisciplinary sarcoma centers. Increasing international collaboration of expert centers in sharing expertise and performing clinical trials might lead to better treatment and improved survival.

  15. 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.

  16. Histology-based classification predicts pattern of recurrence and improves risk stratification in primary retroperitoneal sarcoma

    PubMed Central

    Tan, Marcus C.B.; Brennan, Murray F.; Kuk, Deborah; Agaram, Narasimhan P.; Antonescu, Cristina; Qin, Li-Xuan; Moraco, Nicole; Crago, Aimee M.; Singer, Samuel

    2015-01-01

    Objective To determine the prognostic significance of histologic type/subtype in a large series of patients with primary resected retroperitoneal sarcoma. Summary Background Data The histologic diversity and rarity of retroperitoneal sarcoma has hampered the ability to predict patient outcome. Methods From a single-institution, prospective database, 675 patients treated surgically for primary, non-metastatic retroperitoneal sarcoma during 1982–2010 were identified and histologic type/subtype was reviewed. Clinicopathologic variables were analyzed for association with disease-specific death (DSD), local recurrence (LR), and distant recurrence (DR). Results Median follow-up for survivors was 7.5 years. The predominant histologies were well-differentiated liposarcoma, dedifferentiated liposarcoma, and leiomyosarcoma. Five-year cumulative incidence of DSD was 31%, and factors independently associated with DSD were R2 resection, resection of ≥3 contiguous organs, and histologic type. Five-year cumulative incidence for LR was 39% and for DR was 24%. R1 resection, age, tumor size, and histologic type were independently associated with LR; size, resection of ≥3 organs, and histologic type were independently associated with DR. Liposarcoma and leiomyosarcoma were associated with late recurrence and DSD (as long as 15 years from diagnosis). For solitary fibrous tumor, local recurrence was uncommon (<10%), but early distant recurrence was common (36% at 5 years). Nomograms were developed to predict DSD, LR, and DR. Conclusions Histologic type/subtype is the most important independent predictor of DSD, LR, and DR in primary retroperitoneal sarcoma. Histology predicts the pattern and incidence of LR and DR and will aid in more accurate patient counseling and selection of patients for adjuvant therapy trials. PMID:25915910

  17. Surgical outcome and patterns of recurrence for retroperitoneal sarcoma at a single centre

    PubMed Central

    Hogg, HDJ; Manas, DM; Lee, D; Dildey, P; Scott, J; Lunec, J; French, JJ

    2016-01-01

    Introduction Retroperitoneal sarcoma is a surgically managed condition that can recur locally following macroscopically complete resection. Owing to the low incidence of the condition, advances in treatment are reported infrequently but complete compartmental resection and adjuvant or neoadjuvant radiotherapy are areas under investigation. Given the practical difficulty of randomised trials, observational data can highlight advantages from progressive treatment approaches. Methods A retrospective database of consecutive retroperitoneal sarcoma resections performed at a single referral centre between March 1997 and March 2013 was interrogated. Histological, radiological and clinical data were collected. Univariate and multivariate analyses for disease free and overall survival were performed to establish independent predictors of disease recurrence and patient survival. Results A total of 79 patients underwent 90 resections (63 primary). The mean five-year overall and disease free survival rates were 55.3% and 24.8% respectively. Higher patient age, high tumour grade, presence of extraretroperitoneal disease and invasive tumour phenotype were found to significantly predict survival following multivariate analysis. Half (50%) of the tumours displayed invasive behaviour on histopathology and 42% of locoregional recurrence was intraperitoneal. Conclusions Retroperitoneal sarcoma is commonly an infiltrative tumour and often recurs outside of the retroperitoneum. These features limit the therapeutic impact of interventions that focus on gaining local control such as complete compartmental resection and radiotherapy. It seems likely that future advances in the management of this cancer will involve new systemic agents to treat this frequently systemic disease. PMID:26876538

  18. Intraabdominal and retroperitoneal soft-tissue sarcomas - outcome of surgical treatment in primary and recurrent tumors

    PubMed Central

    2010-01-01

    Background Surgery is the only curative treatment for intraabdominal and retroperitoneal sarcoma (IaRS). Little is known about how to treat patients with recurrence. We here report the outcome in primary and recurrent sarcoma treated at the Sarcoma Center in Aarhus, Denmark. Methods All patients evaluated for IaRS from June 1998 to May 2008 were enrolled and data on symptoms, signs, means of diagnosis, extent of surgery, perioperative complications, mortality and long time survival were registered. Primary and first-recurrence sarcomas were analyzed separately. Results Sixty-five of 73 primary and 22 of 28 first-recurrence IaRS had surgery. Fifty-three (82%) and 11 (50%) patients achieved radical R0 resection. Age and radicality of surgery were independent predictors of death, while recurrence of sarcoma was not. Perioperative mortality was 2.3%. 5-year survival was 70.2% for primary and 51.8% for first-recurrent sarcomas. However, patients with radical surgery had 5-year survival of over 70% in both the primary and recurrent group. Conclusions The radicality of surgery is the most important prognostic factor. Patients with recurrence have an equally good prognosis as those with primary sarcoma if radicality is achieved and such surgery should not be considered only as a palliative effort. PMID:20831829

  19. External validation of a multi-institutional retroperitoneal sarcoma nomogram.

    PubMed

    Raut, Chandrajit P; Miceli, Rosalba; Strauss, Dirk C; Swallow, Carol J; Hohenberger, Peter; van Coevorden, Frits; Rutkowski, Piotr; Fiore, Marco; Callegaro, Dario; Casali, Paolo G; Haas, Rick L; Hayes, Andrew J; Honore, Charles; Cannell, Amanda J; Jakob, Jens; Szacht, Milena; Fairweather, Mark; Pollock, Raphael E; Bonvalot, Sylvie; Gronchi, Alessandro

    2016-05-01

    A multi-institutional nomogram for predicting disease-free survival (DFS) and overall survival (OS) in patients with primary retroperitoneal sarcoma (RPS) incorporating relevant prognostic factors not included in the American Joint Committee on Cancer staging system for soft tissue sarcoma has been reported. The authors validated this nomogram with an independent, transatlantic cohort. Data from patients with RPS who were undergoing definitive resection at 1 of 6 sarcoma centers in Europe and North America ("validation set") were used to validate a RPS nomogram developed from 3 other centers ("development set"). The nomogram incorporated 6 variables: age, tumor size, grade, histologic subtype, multifocality, and quality of surgery. Nomogram-predicted probabilities were stratified into 6 subgroups and compared with observed outcomes. Discriminative ability was quantified by Harrell C statistics. The validation and development sets included 631 and 523 patients, respectively, all of whom underwent surgical resection at the institutions represented. The 7-year DFS and OS rates for the validation set were 38% (95% confidence interval, 34%-43%) and 58% (95% confidence interval, 53%-63%), respectively. All 6 nomogram variables were found to be independently prognostic. The corrected Harrell C statistics concordance index values for the validation set were 0.69 for DFS and 0.73 for OS, which were similar to those for the development set, suggesting good calibration of the nomogram in the validation cohort. The RPS nomogram was externally validated using a larger, independent cohort. The nomogram can be generalized to patients undergoing surgery for RPS by specialized sarcoma surgeons at sarcoma centers. The nomogram provides a more individualized and disease-relevant estimation of OS compared with the American Joint Committee on Cancer classification. Cancer 2016;122:1417-1424. © 2016 American Cancer Society. © 2015 American Cancer Society.

  20. Vascular reconstruction after retroperitoneal and lower extremity sarcoma resection.

    PubMed

    Wortmann, M; Alldinger, I; Böckler, D; Ulrich, A; Hyhlik-Dürr, A

    2017-02-01

    Soft tissue sarcomas (STS) of the retroperitoneum and the lower limb with invasion of major blood vessels are very rare malignancies. This study analyses the outcome of patients with vascular replacement during resection of STS of the retroperitoneum and the lower extremity with either arterial or concomitant arterial and venous infiltration. Patients with vascular replacement during resection of sarcoma of the retroperitoneum and the lower extremity between 1990 and 2014 were included in this retrospective single center study. Patients with a sole infiltration of a major vein were excluded. The follow up was obtained from medical records, the general practitioner and a clinical examination whenever possible. The main endpoints were survival, graft patency and the rate of major amputations. Fourty seven patients were included in this study. Twenty patients have received an operation for a retroperitoneal STS, twenty seven for a STS of the lower extremity. The median follow-up was 24.5 months. The median survival was 113 months with a median tumor-free survival of 25 months. The two-year patency for arterial bypasses in the retroperitoneum and the lower extremity was 88% and 66%, respectively. Limb salvage rate was 89%. Invasion of major blood vessels is no contraindication for a resection of a STS in the retroperitoneum and the lower extremity, but it is accompanied by a high postoperative morbidity. Since surgical resection is the only curative therapy in these patients, it should also be offered to patients with infiltration of major blood vessels. Copyright © 2016 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.

  1. [Clinicopathological Study and Treatment Outcome of Retroperitoneal Soft Tissue Sarcoma].

    PubMed

    Kurosawa, Kazuhiro; Urakami, Shinji; Ikeda, Masaomi; Tabata, Mariko; Murata, Hirokatsu; Okaneya, Toshikazu

    2015-11-01

    We analyzed the pathological findings, surgical margin, the first site of local or distant recurrence, treatment for postoperative recurrence and the characteristics of 25 patients who were surgically treated for retroperitoneal sarcomas between December 2000 and January 2014. The median tumor diameter was 11 cm. The pathological diagnosis was liposarcoma (n = 14), leiomyosarcoma (n = 7) and others (n = 4). Tumors were resected en-bloc with adjacent organs in 17 of the patients. In median follow up period of 39 months, 11 of the 14 patients with liposarcoma experienced local tumor recurrence and distant metastasis did not precede local recurrence in any of these patients. Leiomyosarcoma recurred in all patients and distant metastases appeared before local recurrence in four of them. The five-year recurrence-free and over all survival rates were 28 and 58%, respectively. The recurrence-free and overall survival rates significantly differed between well-differentiated and other subtypes of liposarcoma (both p < 0.05). The overall survival was significantly better for patients with a tumor diameter of < 11 cm than for those with ≥ 11 cm (p 0.05). Furthermore, overall survival was significantly better for patients who were able to undergo re-operation at the time of recurrence than for those who could not (p < 0.005). Although we resected adjacent organs when the margin was insufficient, the rate of local recurrence was high in liposarcoma. On the other hand, the rate of distant metastasis was high in leiomyosarcoma.

  2. Singapore Cancer Network (SCAN) Guidelines for the Initial Evaluation, Diagnosis and Management of Retroperitoneal Soft Tissue Sarcoma.

    PubMed

    2015-10-01

    The SCAN sarcoma workgroup aimed to develop Singapore Cancer Network (SCAN) clinical practice guidelines for the initial evaluation, diagnosis and management of patients with retroperitoneal soft tissue sarcoma. The workgroup utilised a consensus approach to create high quality evidence-based clinical practice guidelines suited for our local setting. Various international guidelines from the fields of radiology, pathology, surgical, medical and radiation oncology were reviewed. Recommendations on the role of radiological imaging, pathology, surgery, radiotherapy and systemic therapy in the management of retroperitoneal soft tissue sarcoma were developed. These guidelines form the SCAN Guidelines 2015 for the diagnosis, staging and optimal management of patients with retroperitoneal soft tissue sarcoma.

  3. Surgical Resection of Retroperitoneal Sarcomas: Analysis of Factors Determining Outcome.

    PubMed

    Amersi, Farin; Forscher, Charles; Silberman, Allan W

    2016-01-01

    Retroperitoneal sarcomas (RS) are rare malignant tumors characterized by high local recurrence rates and poor survival, Aggressive surgical resection may improve local recurrence rates and disease-specific survival (DSS), The aim of our study was to determine predictors of survival and local recurrence in primary RS. We performed a retrospective analysis and identified 68 patients who underwent surgical resection of a primary RS between 1985 and 2010, Clinical and pathologic variables were used to create univariate and multivariate models for both survival and recurrence. 68 patients (37% male) with mean age 59 (range 25-84) underwent surgical resection for RS. Median tumor size was 12.0 cm (range 7.0-18.0 cm). 75% of tumors were intermediate/high grade, Incontinuity organ resection was performed in 29 (43%) patients. Seven patients (10%) underwent vascular resection with graft placement. Overall survival at 5, 10 and 15 years was 55%, 42%, and 33%, respectively. Grade (p<0.007), tumor size (p=0.048) and margin status (p<0.05) were found to significantly affect local recurrence. In a multivariate analysis, recurrent disease (p<0.001), age (p<0.003) and high/intermediate grade (p<0.001) significantly affected DSS. Incontinuity organ resection did not significantly affect recurrence (HR = 1.1, CI 0.63 - 1.85) or survival (HR = 1.4, CI 0.8 - 2.9). Surgical resection of RS affords the best chance of survival. Incontinuity organ resection did not affect outcome on multivariate analysis; however, margin status did significantly affect recurrence and could not be achieved without aggressive resection of incontinuity organs.

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

    SciTech Connect

    Baldini, Elizabeth H.; Wang, Dian; Haas, Rick L.M.; Catton, Charles N.; Indelicato, Daniel J.; Kirsch, David G.; Roberge, David; Salerno, Kilian; Deville, Curtiland; Guadagnolo, B. Ashleigh; O'Sullivan, Brian; Petersen, Ivy A.; Le Pechoux, Cecile; Abrams, Ross A.; DeLaney, Thomas F.

    2015-07-01

    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 systematic 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 efficacy

  5. Percutaneous core needle biopsy in retroperitoneal sarcomas does not influence local recurrence or overall survival.

    PubMed

    Wilkinson, M J; Martin, J L; Khan, A A; Hayes, A J; Thomas, J M; Strauss, D C

    2015-03-01

    Tumours within the retroperitoneum can cause a diagnostic dilemma. A preoperative core needle biopsy often is required to establish a histological diagnosis. Preoperative core needle biopsy for extremity soft-tissue sarcoma is oncologically safe and biopsy site recurrence is extremely rare, attributed to placing the biopsy site within the planned resection field. This study investigates whether preoperative core needle biopsy affects oncological outcomes in retroperitoneal sarcomas. Patients undergoing resection of intermediate- and high-grade primary retroperitoneal sarcoma from 1990 until 2011 were included. Primary endpoints were immediate biopsy-related complications, local recurrence, and overall survival. A total of 150 patients were included in the analysis. Of these, 90 patients underwent resection after a preoperative biopsy. Median follow-up was 48 months. One patient required hospital admission postbiopsy for an abdominal wall rectus sheath haematoma. No patient developed intra-abdominal complications that required hospitalisation or early operation related to core needle biopsy. No patient developed a biopsy site recurrence. There was no significant increase in either local recurrence (p = 0.101) or overall survival (p = 0.191) after core needle biopsy. Preoperative core needle biopsy for retroperitoneal sarcomas is safe and does not affect oncological outcome.

  6. Surgical outcome and patterns of recurrence for retroperitoneal sarcoma at a single centre.

    PubMed

    Hogg, H D J; Manas, D M; Lee, D; Dildey, P; Scott, J; Lunec, J; French, J J

    2016-03-01

    Retroperitoneal sarcoma is a surgically managed condition that can recur locally following macroscopically complete resection. Owing to the low incidence of the condition, advances in treatment are reported infrequently but complete compartmental resection and adjuvant or neoadjuvant radiotherapy are areas under investigation. Given the practical difficulty of randomised trials, observational data can highlight advantages from progressive treatment approaches. A retrospective database of consecutive retroperitoneal sarcoma resections performed at a single referral centre between March 1997 and March 2013 was interrogated. Histological, radiological and clinical data were collected. Univariate and multivariate analyses for disease free and overall survival were performed to establish independent predictors of disease recurrence and patient survival. A total of 79 patients underwent 90 resections (63 primary). The mean five-year overall and disease free survival rates were 55.3% and 24.8% respectively. Higher patient age, high tumour grade, presence of extraretroperitoneal disease and invasive tumour phenotype were found to significantly predict survival following multivariate analysis. Half (50%) of the tumours displayed invasive behaviour on histopathology and 42% of locoregional recurrence was intraperitoneal. Retroperitoneal sarcoma is commonly an infiltrative tumour and often recurs outside of the retroperitoneum. These features limit the therapeutic impact of interventions that focus on gaining local control such as complete compartmental resection and radiotherapy. It seems likely that future advances in the management of this cancer will involve new systemic agents to treat this frequently systemic disease.

  7. Preoperative or postoperative radiotherapy versus surgery alone for retroperitoneal sarcoma: a case-control, propensity score-matched analysis of a nationwide clinical oncology database.

    PubMed

    Nussbaum, Daniel P; Rushing, Christel N; Lane, Whitney O; Cardona, Diana M; Kirsch, David G; Peterson, Bercedis L; Blazer, Dan G

    2016-07-01

    Recruitment into clinical trials for retroperitoneal sarcoma has been challenging, resulting in termination of the only randomised multicentre trial in the USA investigating perioperative radiotherapy. Nonetheless, use of radiotherapy for retroperitoneal sarcoma has increased over the past decade, substantiated primarily by its established role in extremity sarcoma. In this study, we used a nationwide clinical oncology database to separately compare overall survival for patients with retroperitoneal sarcoma who had surgery and preoperative radiotherapy or surgery and postoperative radiotherapy versus surgery alone. We did two case-control, propensity score-matched analyses of the National Cancer Data Base, which included adult patients with retroperitoneal sarcoma who were diagnosed from 2003 to 2011. Patients were included if they had localised, primary retroperitoneal sarcoma. Patients were classified into three groups based on use of radiotherapy: preoperative radiotherapy, postoperative radiotherapy, and no radiotherapy (surgery alone). Patients were excluded if they received both preoperative radiotherapy and postoperative radiotherapy, or if they received intraoperative radiotherapy. Parallel propensity score-matched datasets were created for patients who received preoperative radiotherapy versus those who received no radiotherapy and for patients who received postoperative therapy versus those who received no radiotherapy. Propensity scores were calculated with logistic regression, with multiple imputation and backwards elimination, with a significance level to stay of 0·05. Matching was done with a nearest-neighbour algorithm and matched 1:2 for the preoperative radiotherapy dataset and 1:1 for the postoperative radiotherapy dataset. The primary objective of interest was overall survival for patients who received preoperative radiotherapy or postoperative radiotherapy compared with those who received no radiotherapy within the propensity score

  8. Luteinizing hormone, sex steroids and extracorporeal circulation - a promising link to treat retroperitoneal sarcomas. A reconsideration of cancer treatment.

    PubMed

    Lazar, Angela Madalina

    2012-10-01

    Retroperitoneal sarcomas are rare and aggressive tumors with a negative prognosis as there is currently no satisfactory treatment for them. The only proven factor that can significantly increase the otherwise poor survival of sarcoma patients is the radically of resection. However, the completeness of resection is hindered by the hypervascularized nature of sarcomas and the frequent involvement of major blood vessels. In this context, we propose to operate on retroperitoneal sarcomas only with the use of extracorporeal circulation, applying vascular clamps above and below the tumor, even with short periods of hypothermic circulatory arrest in complex cases. This technique would allow the surgeon to achieve complete tumor resections, approach large blood vessels easier and perform sofisticated vascular reconstructions with no fear of hemorrhage which is fundamental to achieve a bloodless surgical field. Also, we speculate on the etiology of retroperitoneal sarcomas that appear mostly during the period of menopause/andropause. Although both estrogens and androgens have been incriminated in inducing various cancer types, including sarcomas, an endogenous estradiol cathabolyte has been shown to have anti-tumor effects. Considering that during menopause/andropause sex steroid levels actually decrease, our second working hypothesis is that the increasing follicle-stimulating hormone (FSH) and especially luteinizing hormone (LH) levels, together with the relative estrogen/androgen imbalance, may be the triggering cause. Also, a certain level of estrogens (Methoxyestradiol) may be essential in limiting tumor development and dedifferentiation. Given that extragonadal sarcomas appear to behave as endocrine tumors, a targeted hormonal therapy, together with controlled radical resections in complex cases of tumor vascular involvement, would certainly provide a strong link to both prevention and treatment of retroperitoneal sarcomas and even of cancer in general.

  9. Retroperitoneal Sarcoma (RPS) High Risk Gross Tumor Volume Boost (HR GTV Boost) Contour Delineation Agreement Among NRG Sarcoma Radiation and Surgical Oncologists

    PubMed Central

    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

    2015-01-01

    Purpose 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. Methods 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. Results 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. Conclusions 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. PMID:26018727

  10. Retroperitoneal soft tissue sarcoma: An analysis of radiation and surgical treatment

    SciTech Connect

    Ballo, Matthew T. . E-mail: mballo@mdanderson.org; Zagars, Gunar K.; Pollock, Raphael E.; Benjamin, Robert S.; Feig, Barry W.; Cormier, Janice N.; Hunt, Kelly K.; Patel, Shreyaskumar R.; Trent, Jonathan C.; Beddar, Sam; Pisters, Peter W.T.

    2007-01-01

    Purpose: To evaluate the clinical outcomes of patients with localized retroperitoneal soft tissue sarcoma (STS) treated with complete surgical resection and radiation. Methods and Materials: The medical records of 83 patients were reviewed retrospectively. Sixty patients presented with primary disease and the remaining 23 had recurrence after previous surgical resection. Results: With a median follow-up of 47 months, the actuarial overall disease-specific survival (DSS), distant metastasis-free survival, and local control (LC) rates were 44%, 67%, and 40%, respectively. Of the 38 patients dying of disease, local disease progression was the sole site of recurrence for 16 patients and was a component of progression for another 11 patients. Multivariate analysis indicated that histologic grade was associated with the 5-year rates of DSS (low-grade, 92%; intermediate-grade, 51%; and high-grade, 41%, p = 0.006). Multivariate analysis also indicated an inferior 5-year LC rate for patients presenting with recurrent disease, positive or uncertain resection margins, and age greater than 65 years. The data did not suggest an improved local control with higher doses of external-beam radiation (EBRT) or with the specific use of intraoperative radiotherapy (IORT). Radiation-related complications (10% at 5 years) developed in 5 patients; all had received their EBRT postoperatively. Conclusions: Although preoperative radiation therapy and aggressive surgical resection is well tolerated in patients, local disease progression continues to be a significant component of disease death. In this small cohort of patients, the use of higher doses of EBRT or IORT did not result in clinically apparent improvements in outcomes.

  11. Retroperitoneal sarcoma: a population-based analysis of epidemiology, surgery, and radiotherapy.

    PubMed

    Porter, Geoffrey A; Baxter, Nancy N; Pisters, Peter W T

    2006-04-01

    No population-based studies of retroperitoneal sarcoma (RPS) have been conducted, and the use and timing of adjuvant radiotherapy for RPS is controversial. The objective of this study was to examine the incidence and treatment of RPS, specifically regarding the use of adjuvant radiotherapy. The Surveillance, Epidemiology, and End Results (SEER) database was used to evaluate the incidence of RPS over a 29-year period (1973-2001). The rate of surgery, the rate and timing of adjuvant radiotherapy, and the influence of demographic factors on treatment were evaluated. A total of 2348 cases of RPS were identified. The mean annual incidence of RPS was 2.7 cases per 10(6) persons and did not change significantly over time (2.6 in 1973 vs. 2.8 in 2001; P = .92). Most patients (1654; 70.4%) underwent surgical resection. Radiotherapy was used in 428 patients (25.9%) who underwent surgery; radiation was given postoperatively in 366 (85.5%), preoperatively in 20 (4.7%), and intraoperatively or unknown in 42 (9.8%). Patients who received any adjuvant radiotherapy were on average 5 years younger than those who underwent surgery alone (P < .0001). Radiotherapy was more commonly used among whites than African Americans (25.8% vs. 16.7%; P = .02) and there was significant variation in the use of adjuvant radiotherapy by geographic location (P = .003). On multivariate analysis, race (P = 0.004), age (P < .0001), and geographic location (P = .006) were independently associated with the use of adjuvant radiotherapy. The incidence of RPS, a rare disease, appears stable. Most patients who undergo surgery do not receive any adjuvant radiotherapy, and very few receive preoperative radiotherapy. Differences in adjuvant radiotherapy use related to demographic and geographic factors suggest that at least some treatment variations reflect differences in individual and institutional practice patterns. Copyright 2006 American Cancer Society.

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

    SciTech Connect

    Bishop, Andrew J.; Zagars, Gunar K.; Torres, Keila E.; Hunt, Kelly K.; Cormier, Janice N.; Feig, Barry W.; Guadagnolo, B. Ashleigh

    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) 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.

  13. Clinical outcomes of tissue expanders on adjuvant radiotherapy of resected retroperitoneal sarcoma

    PubMed Central

    Yu, Jeong Il; Lim, Do Hoon; Park, Hee Chul; Nam, Heerim; Kim, Bo Kyoung; Kim, Sung-Joo; Park, Jae Berm

    2016-01-01

    Abstract We investigated the efficacy and safety of a tissue expander (TE) for adjuvant radiotherapy (RT) of resected retroperitoneal sarcoma (RPS). This study was conducted with 37 patients with RPS who received resection with or without TE insertion followed by RT from August 2006 to June 2012 at Samsung Medical Center. Among the 37 patients, TE was inserted in 19. The quality of TE insertion was evaluated according to the correlation of clinical target volume and retroperitoneal surface volume covered by TE and was defined as follows: excellent, ≥85%; good, 70% to 85%; fair, 50% to 70%; and poor, <50%. The median follow-up period after surgery was 47.9 months (range, 5.5–85.5 months). The quality of TE insertion was excellent in 7 (36.8%), good in 5 (26.3%), fair in 4 (21.0%), and poor in 3 (16.7%) patients. A significantly higher biologically equivalent dose (BED, α/β = 10) was used in patients who had TE insertion (median, 64.8 vs. 60.0 Gy, P = 0.01). Local control was 39.7%, and overall survival was 76.4% at 5 years. Local control was significantly higher in patients who received ≥65 Gy of BED, 100.0% in contrast to 22.8% (P = 0.01). One patient with a history of multiple tumor resections showed abdominal infection with duodenal perforation of uncertain cause but had the potential of being related to TE and/or RT. Otherwise there were no ≥grade III acute or late toxicities. TE for adjuvant RT in RPS is feasible for delivering a higher RT dose with acceptable toxicity. PMID:27428199

  14. Clinical outcomes of tissue expanders on adjuvant radiotherapy of resected retroperitoneal sarcoma.

    PubMed

    Yu, Jeong Il; Lim, Do Hoon; Park, Hee Chul; Nam, Heerim; Kim, Bo Kyoung; Kim, Sung-Joo; Park, Jae Berm

    2016-07-01

    We investigated the efficacy and safety of a tissue expander (TE) for adjuvant radiotherapy (RT) of resected retroperitoneal sarcoma (RPS).This study was conducted with 37 patients with RPS who received resection with or without TE insertion followed by RT from August 2006 to June 2012 at Samsung Medical Center. Among the 37 patients, TE was inserted in 19. The quality of TE insertion was evaluated according to the correlation of clinical target volume and retroperitoneal surface volume covered by TE and was defined as follows: excellent, ≥85%; good, 70% to 85%; fair, 50% to 70%; and poor, <50%. The median follow-up period after surgery was 47.9 months (range, 5.5-85.5 months).The quality of TE insertion was excellent in 7 (36.8%), good in 5 (26.3%), fair in 4 (21.0%), and poor in 3 (16.7%) patients. A significantly higher biologically equivalent dose (BED, α/β = 10) was used in patients who had TE insertion (median, 64.8 vs. 60.0 Gy, P = 0.01). Local control was 39.7%, and overall survival was 76.4% at 5 years. Local control was significantly higher in patients who received ≥65 Gy of BED, 100.0% in contrast to 22.8% (P = 0.01). One patient with a history of multiple tumor resections showed abdominal infection with duodenal perforation of uncertain cause but had the potential of being related to TE and/or RT. Otherwise there were no ≥grade III acute or late toxicities.TE for adjuvant RT in RPS is feasible for delivering a higher RT dose with acceptable toxicity.

  15. CT-guided 125I seed implantation for inoperable retroperitoneal sarcoma: A technique for delivery of local tumor brachytherapy

    PubMed Central

    Yang, Biao; Guo, Wen-Hao; Lan, Ting; Yuan, Fang; Liu, Guan-Jian; Zan, Rui-Yu; You, Xin; Tan, Qiao-Yue; Liao, Zheng-Yin

    2016-01-01

    Radical surgery is currently the first treatment of choice for retroperitoneal soft tissue sarcoma (RSTS). However, the prognosis of RSTS remains poor due to ineffective local control and a high incidence of metastasis after surgical resection. Brachytherapy has been shown to safely provide local radiotherapy for numerous types of cancer when used alone or in combination with surgical resection, but has not been well characterized in the management of RSTS. The aim of this study was to evaluate CT-guided 125I seed implantation for local control and pain relief in the treatment of inoperable RSTS. A total of 23 patients with RSTS were treated with 125I implantation. Pain was assessed using a visual analog scale. Other endpoints were evaluated via computed tomography scan or phone call/e-mail records. The occurrence of complications was assessed preoperatively (baseline) and during postoperatively follow-up or until patient succumbed. All patients were successfully treated with 125I implantation. A mean number of 70.87 radioactive seeds were applied in each patient. During the follow-up, two patients were unaccounted for, local recurrence occurred in three patients, five succumbed and complications were observed in sixteen. The patient's VAS score changed from 7.4 preoperatively to 7.6, 2.3, 2.0, 1.2, 1.5, 1.4 and 2.5 at 24 h, 1, 3, 6, 12, 24 and 36 months after the procedure, respectively. Good local control and significant pain relief after 125I seed implantation was observed in patients with inoperable RSTS. Thus, the present results suggest that this method could be an effective treatment option for patients with inoperable RSTS. PMID:28101168

  16. Significant benefits in survival by the use of surgery combined with radiotherapy for retroperitoneal soft tissue sarcoma.

    PubMed

    Hager, Sven; Makowiec, Frank; Henne, Karl; Hopt, Ulrich T; Wittel, Uwe A

    2017-01-26

    To report the effect of intraoperative electron beam radiotherapy (IOERT) and external beam radiotherapy (EBRT) in addition to surgery as well as to evaluate the role of resectable local recurrence for long-term prognosis. In 53 patients who underwent surgery for retroperitoneal soft tissue sarcoma (RSTS) from 2001 to 2014 prognostic and epidemiologic factors were reviewed retrospectively to analyze their impact on survival and recurrence. Twenty three patients (50%) had surgery plus radiotherapy, 23 (50%) had surgery only. Histology showed 73.9% liposarcoma, 15.2% leiomyosarcoma and 6.5% pleomorphic undifferentiated sarcoma respectively. Low grade sarcoma were observed in 52.2%, high grade sarcoma in 47.8%. The latter showed a trend towards a decreased 5-year survival rate (p = 0.125). Margin status was: R0: 60.9%, R1: 23.9%, R2: 15.2%; leading to significant changes in 5-year survival rate (R0: 77.6%; R1: 70.0%; R2: 42.9%; p = 0.03). Age younger than 55 years significantly improved 5-year survival rate (p = 0.039). Patients receiving resection of multiple sarcoma recurrence showed an almost identical improved 5-year survival rate compared to patients without recurrence (no recurrence: 100.0%; single recurrence: 35.0%; multiple recurrence: 91.7%; p = 0.001). Surgery plus radiotherapy led to significantly improved survival (p = 0.04). There is a significant benefit in terms of 5-year survival after surgery plus some form of radiotherapy and a good prognosis for patients when the recurrence from RSTS was resected. Age older than 55 years and incomplete resection lowered 5-year survival rate significantly.

  17. 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.

  18. Proton-Beam, Intensity-Modulated, and/or Intraoperative Electron Radiation Therapy Combined with Aggressive Anterior Surgical Resection for Retroperitoneal Sarcomas

    PubMed Central

    Yoon, Sam S.; Chen, Yen-Lin; Kirsch, David G.; Maduekwe, Ugwuji N.; Rosenberg, Andrew E.; Nielsen, G. Petur; Sahani, Dushyant V.; Choy, Edwin; Harmon, David C.; DeLaney, Thomas F.

    2010-01-01

    Background We sought to reduce local recurrence for retroperitoneal sarcomas by using a coordinated strategy of advanced radiation techniques and aggressive en-bloc surgical resection. Methods Proton-beam radiation therapy (PBRT) and/or intensity-modulated radiation therapy (IMRT) were delivered to improve tumor target coverage and spare selected adjacent organs. Surgical resection of tumor and adjacent organs was performed to obtain a disease-free anterior margin. Intraoperative electron radiation therapy (IOERT) was delivered to any close posterior margin. Results Twenty patients had primary tumors and eight had recurrent tumors. Tumors were large (median size 9.75 cm), primarily liposarcomas and leiomyosarcomas (71%), and were mostly of intermediate or high grade (81%). PBRT and/or IMRT were delivered to all patients, preferably preoperatively (75%), to a median dose of 50 Gy. Surgical resection included up to five adjacent organs, most commonly the colon (n = 7) and kidney (n = 7). Margins were positive for disease, usually posteriorly, in 15 patients (54%). IOERT was delivered to the posterior margin in 12 patients (43%) to a median dose of 11 Gy. Surgical complications occurred in eight patients (28.6%), and radiation-related complications occurred in four patients (14%). After a median follow-up of 33 months, only two patients (10%) with primary disease experienced local recurrence, while three patients (37.5%) with recurrent disease experienced local recurrence. Conclusions Aggressive resection of retroperitoneal sarcomas can achieve a disease-negative anterior margin. PBRT and/or IMRT with IOERT may possibly deliver sufficient radiation dose to the posterior margin to control microscopic residual disease. This strategy may minimize radiation-related morbidity and reduce local recurrence, especially in patients with primary disease. PMID:20151216

  19. Comparison of Three-Dimensional (3D) Conformal Proton Radiotherapy (RT), 3D Conformal Photon RT, and Intensity-Modulated RT for Retroperitoneal and Intra-Abdominal Sarcomas

    SciTech Connect

    Swanson, Erika L.; Indelicato, Daniel J.; Louis, Debbie; Flampouri, Stella; Li, Zuofeng; Morris, Christopher G.; Paryani, Nitesh; Slopsema, Roelf

    2012-08-01

    Purpose: To compare three-dimensional conformal proton radiotherapy (3DCPT), intensity-modulated photon radiotherapy (IMRT), and 3D conformal photon radiotherapy (3DCRT) to predict the optimal RT technique for retroperitoneal sarcomas. Methods and Materials: 3DCRT, IMRT, and 3DCPT plans were created for treating eight patients with retroperitoneal or intra-abdominal sarcomas. The clinical target volume (CTV) included the gross tumor plus a 2-cm margin, limited by bone and intact fascial planes. For photon plans, the planning target volume (PTV) included a uniform expansion of 5 mm. For the proton plans, the PTV was nonuniform and beam-specific. The prescription dose was 50.4 Gy/Cobalt gray equivalent CGE. Plans were normalized so that >95% of the CTV received 100% of the dose. Results: The CTV was covered adequately by all techniques. The median conformity index was 0.69 for 3DCPT, 0.75 for IMRT, and 0.51 for 3DCRT. The median inhomogeneity coefficient was 0.062 for 3DCPT, 0.066 for IMRT, and 0.073 for 3DCRT. The bowel median volume receiving 15 Gy (V15) was 16.4% for 3DCPT, 52.2% for IMRT, and 66.1% for 3DCRT. The bowel median V45 was 6.3% for 3DCPT, 4.7% for IMRT, and 15.6% for 3DCRT. The median ipsilateral mean kidney dose was 22.5 CGE for 3DCPT, 34.1 Gy for IMRT, and 37.8 Gy for 3DCRT. The median contralateral mean kidney dose was 0 CGE for 3DCPT, 6.4 Gy for IMRT, and 11 Gy for 3DCRT. The median contralateral kidney V5 was 0% for 3DCPT, 49.9% for IMRT, and 99.7% for 3DCRT. Regardless of technique, the median mean liver dose was <30 Gy, and the median cord V50 was 0%. The median integral dose was 126 J for 3DCPT, 400 J for IMRT, and 432 J for 3DCRT. Conclusions: IMRT and 3DCPT result in plans that are more conformal and homogenous than 3DCRT. Based on Quantitative Analysis of Normal Tissue Effects in Clinic benchmarks, the dosimetric advantage of proton therapy may be less gastrointestinal and genitourinary toxicity.

  20. Retroperitoneal Sarcoma Involving Unilateral Double Ureter: Management, Treatment and Psychological Implications

    PubMed Central

    Leanza, Vito; Garraffo, Claudia; Leanza, Gianluca; Leanza, Antonio

    2014-01-01

    The case of a 45-year-old woman who was admitted to our university hospital for polymenorrhea, weight gain and pain in the left iliac region is reported. An abdominal ultrasound revealed a 9.5 × 5.2-cm, hypoechoic and inhomogeneous mass located on the left side of the pelvis and behind the ovary. The patient underwent surgery. The pelvic mass was firmly anchored to the small intestine, colon, sigma and uterine fundus. After removing the adhesions, double ureters, which had been incorporated in the mass, were observed on the left side. Resection of the unilateral double ureters was necessary in order to remove the entire mass, and thereafter, a left salpingoophorectomy was performed. A histological examination showed a malignant retroperitoneal mass. Termino-terminal ureteral anastomosis with two double-J stents was carried out. Total hysterectomy with preservation of the right adenexum and regional lymphadenectomy was performed. The purpose of this case report is to discuss the physical and psychological implications related to the combination of two rare entities: leiomyosarcoma and a double ureter located within the mass. A literature review on the clinical management and psychological aspects from a female cancer patient's perspective undergoing surgery with the aforementioned disorders will be discussed. PMID:24932171

  1. [Sonographical diagnosis of pneumoretroperitoneum as a result of retroperitoneal perforation].

    PubMed

    Nürnberg, D; Mauch, M; Spengler, J; Holle, A; Pannwitz, H; Seitz, K

    2007-12-01

    A retroperitoneal perforation is a rare incident. It can occur as a complication of ERCP with papillotomy (0.2-0.5%). Leakage of contrast agent during endoscopy raises the suspicion that this complication has occurred but doesn't always give sufficient information about the leakage extent. In the case of extreme gas emission, a plain abdominal X-ray shows classic pneumoretroperitoneum. The abdominal CT scan can display small amounts of free air which is why it is used for diagnosis in such cases. Ultrasonography also provides a reliable diagnosis and is a good method for monitoring the progression of the condition. Alternative causes of pneumoretroperitoneum can be: trauma, inflammation, infection, tumor as well as ERCP and other interventional procedures, especially endoscopies. Presacral retroperitoneal pneumoradiography was used for the diagnosis of retroperitoneal tumors in the 70 s but is no longer used today. Perforations into the retroperitoneal space come from several locations in the gastrointestinal tract. In the different types of lesions the gas can penetrate the compartments and reach as far as the mediastinum, the intraabdominal cavity, subcutaneum (cervical) or the scrotal compartment (compartment shift). Based on 11 cases (7 perforations during ERCP, 2 perforation during colonoscopy, 2 cases with damage of the distal esophagus), we show the most extensive presentation of the sonographical picture of pneumoretroperitoneum. Typical signs on abdominal ultrasound are an increased echogenicity around the right kidney ("overcasted" or "covered" kidney), air dorsal to the gallbladder, around the duodenum and the head of the pancreas and especially ventral to the great abdominal vessel which can lead to the picture of "vanishing" vessels. The extent of free air is easy to assess. Even very small amounts are detectable ventral to the right kidney. In most cases, a conservative approach with no oral intake, antibiotic coverage, and analgesia in close

  2. Retroperitoneal liposarcomas: a representative literature review occasioned by a rare case of laterelapse abdominal liposarcoma.

    PubMed

    Pisani, Michela; Al-Buheissi, Salah; Whittlestone, Tim

    2016-05-24

    Soft tissue sarcomas (STS) are rare and heterogeneous tumours representing approximately 0.7%-1% of all adult tumours. In the adults and among the retroperitoneal sarcomas (RPS), Liposarcoma (LS) is the most common variant accounting for 12% -20% of all sarcomas and up to 45% of sarcomas at retroperitoneal localization. A rare case of LS relapsed after 15 years is giving the occasion to review the published literature and emphasise the followings concepts: 1) Despite extensive surgery remains the mainstay of treatment for localized STS at present, anatomical complexity and occult localization result in local recurrence in the majority of patients; 2) The role of imaging and tumour markers is still limited; 3) Indefinite prolonged surveillance is a key point of treatment; 4) Referral to tertiary centres with dedicated Retroperitonal Surgeons and Oncology expertise is mandatory.

  3. Combination of a fillet flap, free tissue transfer, and autologous tissue grafts in pelvic reconstruction following retroperitoneal sarcoma: a case report.

    PubMed

    Saksena, Anshuman; Aho, Johnathon; Winocour, Sebastian; Maricevich, Renata; Senchenkov, Alex; Rose, Peter; Leibovich, Bradley; Zietlow, Scott; Saint-Cyr, Michel

    2015-05-01

    The resection of large pelvic tumors is challenging due to their infiltrative nature into multiple structures and organ systems. In this report, we describe the use of multiple vascularized and nonvascularized spare parts to reconstruct a pelvic defect in a patient with a uniquely large pelvic sarcoma invading the spinal canal. A 39-year-old Caucasian female who presented with a large retroperitoneal sarcoma where the tumor encased the left ureter, kidney, colon, and external iliac vessels and invaded the L3-S1 vertebral bodies. An extensive hemipelvectomy and reconstruction was performed over two days. A free thigh and leg fillet flap together with ipsilateral fibula flap, based on the superficial femoral artery and venae comitantes, was used for spinal reinforcement as well as abdominal and pelvic wall reconstruction. The postoperative course was uneventful without complications, no flap compromise or wound healing problems. After a follow-up period of 4 months, the patient had no complications and returned to activities of daily living with mild limitations. The success of this flap procedure shows the practicality and usefulness of using the full spectrum of tissue transfer for the purposes of a large pelvic reconstruction.

  4. Retroperitoneal inflammation

    MedlinePlus

    ... page: //medlineplus.gov/ency/article/001255.htm Retroperitoneal inflammation To use the sharing features on this page, please enable JavaScript. Retroperitoneal inflammation is swelling that occurs in the retroperitoneal space. ...

  5. Gallium-67 imaging in retroperitoneal fibrosis: Significance of a negative result

    SciTech Connect

    Jacobson, A.F. )

    1991-03-01

    A patient with retroperitoneal fibrosis and right peritracheal and hilar lymphadenopathy was studied using gallium-67-citrate. No abnormal uptake was seen in the regions of retroperitoneal fibrosis, while there was avid uptake in chest lesions later shown to represent small cell lung carcinoma. Retroperitoneal fibrosis which does not show gallium uptake is most likely mature, with few inflammatory elements. In patients with multiple retroperitoneal and/or mediastinal masses, gallium imaging may be useful in identifying the most active sites of disease for possible biopsy and for subsequent monitoring of response to therapy.

  6. Post-relapse outcomes after primary extended resection of retroperitoneal sarcoma: A report from the Trans-Atlantic RPS Working Group.

    PubMed

    MacNeill, Andrea J; Miceli, Rosalba; Strauss, Dirk C; Bonvalot, Sylvie; Hohenberger, Peter; Van Coevorden, Frits; Rutkowski, Piotr; Callegaro, Dario; Hayes, Andrew J; Honoré, Charles; Fairweather, Mark; Cannell, Amanda; Jakob, Jens; Haas, Rick L; Szacht, Milena; Fiore, Marco; Casali, Paolo G; Pollock, Raphael E; Raut, Chandrajit P; Gronchi, Alessandro; Swallow, Carol J

    2017-06-01

    Despite a radical surgical approach to primary retroperitoneal sarcoma (RPS), many patients experience locoregional and/or distant recurrence. The objective of this study was to analyze post-relapse outcomes for patients with RPS who had initially undergone surgical resection of their primary tumor at a specialist center. All consecutive patients who underwent macroscopically complete resection for primary RPS at 8 high volume centers from January 2002 to December 2011 were identified, and those who developed local recurrence (LR) only, distant metastasis (DM) only, or synchronous local recurrence and distant metastasis (LR+DM) during the follow-up period were included. Overall survival (OS) was calculated for all groups, as was the crude cumulative incidence of a second recurrence after the first LR. Multivariate analyses for OS were performed. In an initial series of 1007 patients with primary RPS, 408 patients developed recurrent disease during the follow-up period. The median follow-up from the time of recurrence was 41 months. The median OS was 33 months after LR (n = 219), 25 months after DM (n = 146), and 12 months after LR+DM (n = 43), and the 5-year OS rates were 29%, 20%, and 14%, respectively. Predictors of OS after LR were the time interval to LR and resection of LR, while histologic grade approached significance. For DM, significant predictors of OS were the time interval to DM and histologic subtype. The subgroup of patients who underwent resection of recurrent disease had a longer median OS than patients who did not undergo resection. Relapse of RPS portends high disease-specific mortality. Patients with locally recurrent or metastatic disease should be considered for resection. Cancer 2017;123:1971-1978. © 2017 American Cancer Society. © 2017 American Cancer Society.

  7. Analysis of results of soft tissue sarcoma margins revision surgery.

    PubMed

    García-Jiménez, A; Trullols-Tarragó, L; Peiró-Ibáñez, A; Gracia-Alegría, I

    Patients from other centres where they have had an unplanned surgical resection of a soft tissue sarcoma are often referred to hospitals specialised in sarcomas. A study was conducted on 35 patients who required this type of surgery were referred to our center between November 2001 and July 2013. Surgery had been performed on 29% of the patients without any complementary tests being done. In 75% of cases, the sarcoma diagnosis was discovered in the post-surgical histological study. Synovial sarcoma was the most common, affecting 38% of the patients. A surgical revision of the margins was performed on all of them, and adjuvant treatment was performed on 86% of them. The histopathology study found that 69% of the patients had residual disease. At the end of follow-up, 12% had a local recurrence, another 12% distant metastases, and 3% had died. Given the results, it is concluded that any tumour of the soft tissues in which malignancy is suspected has to be resected in a reference centre. If an unplanned esection was performed in another centre, it should be referred immediately in order to perform an imaging study, revision surgery, and if required, adjuvant treatment. Copyright © 2016 SECOT. Publicado por Elsevier España, S.L.U. All rights reserved.

  8. Indian data on bone and soft tissue sarcomas: A summary of published study results

    PubMed Central

    Ramaswamy, Anant; Rekhi, Bharat; Bakhshi, Sameer; Hingmire, Sachin; Agarwal, Manish

    2016-01-01

    Bone sarcomas are rare tumors, approximating 0.2% of all cancers, with osteosarcoma (OGS), chondrosarcoma, and Ewing sarcoma being the most common cancers in this subset. The formation of disease management groups/clinics focused on sarcomas has resulted in better understanding and management of these uncommon tumors. Multiple large-scale retrospective data from Tata Memorial Hospital (TMH) and All India Institute of Medical Sciences have reported outcomes comparable to Western data in the field of OGS and Ewing sarcoma, with interesting prognostic factors identified for further evaluation. Soft tissue sarcomas are a rare heterogeneous group of tumors, more than 50 different tumor entities. The common subtypes identified in India include Ewing sarcoma and synovial sarcoma. Valuable work regarding brachytherapy has been done by radiation oncologists from the TMH, especially in pediatric patients. PMID:27606300

  9. [Retroperitoneal tumors: a case of liposarcoma].

    PubMed

    Cassinelli, G; Arena, E; Bronzino, P; Cuneo, A E; Partipilo, F; Rusca, I; Rassu, P C; Casaccia, M

    2002-01-01

    The Authors report a case of retroperitoneal sarcoma, in the histological subtype "liposarcoma", occasionally diagnosed in a 57 year old woman, who presented aspecific symptoms, characterized by dry cough, vomiting and chest pain. Chest TC showed a neoplasm of the retroperitoneum, close to liver, right colon, duodenum and right kidney. The arteriography of the right renal artery showed a neovascolarization inside the mass. A surgical resection was performed. The histological examination confirmed the diagnosis of liposarcoma. Liposarcomas account for 30% of the retroperitoneal soft tissue sarcomas; symptoms and diagnosis are late and the surgical resection of primary neoplasm, metastases and local recurrences representing the only therapeutic choice.

  10. Heart failure resulting from giant left atrial synovial sarcoma metastasis.

    PubMed

    Winkler, B; Grapow, M; Seeberger, M; Matt, P; Aulitzky, W; Eckstein, F

    2012-02-01

    Synovial sarcoma metastasis affecting the heart and infiltrating the mitral valve is a very rare pathology. We report the case of a 44-year-old male treated with chemotherapy for atypical synovial sarcoma of the oral mucosa who presented to our clinic after cardiac decompensation with a presumptive diagnosis of myxoma of the left atrium. A large necrotic tumour positive for CK 22, EMA, CD 99 and BCL-2 but negative for translocation in COBRA-FISH analysis by break-apart probe could be excised and revealed a very rare subtype of synovial sarcoma metastasis arising from the endocard of the left atrium. The tumour was resected and the mitral valve reconstructed through ring annuloplasty. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  11. [Giant retroperitoneal liposarcoma--case report].

    PubMed

    Bánky, Balázs; Bányász, Zsolt; Mayer, Arpád; Almási, Kálmán; Szucs, Iván

    2005-06-01

    Retroperitoneal liposarcomas are the most frequent soft tissue sarcomas and the second most frequent retroperitoneal tumours. They represent less then 0.1% of all human malignancies. Hereby we describe the treatment of our patient where of a 15-kg giant retroperitoneal liposarcoma was successfully removed and a local recurrence two years later was operated on as well. This was the 3rd largest retroperitoneal malignant tumor that was successfully removed according to the available literature of the last five decades. These typically symptom-free tumors usually grow extreme size before diagnosed. The "gold-standard" of treatment remains surgical total excision, but the high local recurrence rate (50-60%) hopefully can be reduced by adjuvant radio- and chemotherapy. Multidisciplinary treatment and long-time follow-up can provide as high as 40% 5-year survival rate. We summarize the recent clinical, diagnostic and therapeutic methods of this rare condition.

  12. [Retroperitoneal fibrosis].

    PubMed

    Babski, Paweł; Wojtuń, Stanisław; Gil, Jerzy

    2007-05-01

    Retroperitoneal fibrosis is a rare clinical entity characterised by the presence of patologic collagen tissue in a retroperitoneal space. The fibrous mass covers abdominal organs causing their disfunctions. RPF was described at the begining of XX century but its etiology is not clear yet. Usually it causes an ureter obstuction and hydronephrosis, that is why most commonly is diagnosed by urologists and nephrologists. However, retroperitoneal fibrosis can be multifacial disease. In some patients localisation of fibrosis is atypical and manifestationns can be varied. Gastrological symptoms like jaundice, bowel obstuction, ascites can occure. Besides, some early signs of RPF are nonspecific and can imitate alarming symptoms of neoplasma, e.g.: weight loss, anemia, malaise, anorexia, fever. This force us to initiate gastrological investigation. The awareness of this disease is important. The early diagnosis and treatment improves prognosis and alows to avoid heavy complications. In typical cases radiology is often enough for diagnosis. However, histological examination is needed in many cases, especialy when patological mass is located atypical. A treatment is made up of farmacology and surgery. The first one is based on steroids, immunossuppressant and tamoxifen. Surgery is needed to eliminate organs obstruction.

  13. CT characteristics of primary retroperitoneal neoplasms in children.

    PubMed

    Xu, Yufeng; Wang, Jichen; Peng, Yun; Zeng, Jinjin

    2010-09-01

    Primary retroperitoneal neoplasms are uncommon in children. Retroperitoneal neoplasms are either mesodermal, neurogenic, germ cell ectodermal or lymphatic in origin. In general, primary retroperitoneal neoplasms in children have different spectrum and prevalence compared to those in adults. Neuroblastoma, rhabdomyosarcoma, benign teratoma and lymphoma are the common retroperitoneal neoplasms. In this review, the clinical and CT futures of common retroperitoneal neoplasms in children are described. Coarse, amorphous, and mottled calcification are very common in neuroblastoma. Paraganglioma tends to show marked and early enhancement and may present with clinical symptoms associated with the excess catecholamine. Sarcomas are often very large and have heterogeneous appearance. Imaging cannot be reliably used to identify the type of retroperitoneal sarcomas due to overlapped radiographic features. In children, lipoblastoma is the most common lipomatous tumor in the retroperitoneum. The percentage of visible fat in tumor varies depending on the cellular composition of the lesion. The CT characteristics of teratoma are quite variable, which may be cystic, solid, on a combination of both. Typically teratoma appears as a large complex mass containing fluid, fat, fat-fluid level, and calcifications. Lymphoma is often homogeneous on both enhanced and unenhanced CT scans. Necrosis and calcification are rare on CT. In conclusion, making a final histological diagnosis of retroperitoneal tumor base on CT features is not often possible; however, CT can help to develop a differential diagnosis and determine the size and extent of the retroperitoneal neoplasms.

  14. [Sarcoma developed in irradiated area: Preliminary results of the SARI trial].

    PubMed

    Maingon, P; Mirjolet, C; Diallo, I; Veres, C; Collin, F; Italiano, A; Chibon, F; Merlin, J L; Coindre, J M

    2016-10-01

    Radiotherapy and surgery are the two main pillars of the locoregional treatment of cancer. The risk of second malignancy is better evaluated and constitutes a major issue regarding radioprotection of the patients. Among malignant disease observed in the surviving irradiated patients, the occurrence of sarcoma is a rare event but associated with a poor outcome since the 5 year overall survival is estimated at 10 to 35 %. The SARI protocol, written in 2011, included 120 patients and 240 controlled patients, irradiated in the same conditions but without sarcoma observed during the follow up. The main objective was to identify the clinical and biological factors associated with the occurrence of such a complication. The secondary objective was to identify the dosimetric characteristics of the treatment of the primary. Preliminary results will be presented during the 2016 meeting of the French radiation oncology society.

  15. [Giant retroperitoneal liposarcoma].

    PubMed

    Mezzour, Mohamed Hicham; El Messaoudi, Yasser Arafat; Fekak, Hamid; Rabii, Redouane; Marnissi, Farida; Karkouri, Mehdi; Salam, Siham; Iraki, Moulay Ahmed; Joual, Abdenbi; Meziane, Fathi

    2006-02-01

    The authors report a case of giant retroperitoneal liposarcoma. The diagnosis was suspected after scanography and magnetic resonance imaging and confirmed by the histological analysis of the extracted piece after surgical treatment. Postoperative evolution was favourable after one year without recurrence or distant metastasis. The authors discuss the pathologic and therapeutic aspects and the prognosis of retroperitoneal liposarcoma.

  16. Combination of Trabectedin and Gemcitabine for Advanced Soft Tissue Sarcomas: Results of a Phase I Dose Escalating Trial of the German Interdisciplinary Sarcoma Group (GISG)

    PubMed Central

    Kasper, Bernd; Reichardt, Peter; Pink, Daniel; Sommer, Michaela; Mathew, Monika; Rauch, Geraldine; Hohenberger, Peter

    2015-01-01

    Background: Evaluation of the potential efficacy and safety of combination therapies for advanced soft tissue sarcomas (STS) has increased substantially after approval of trabectedin and pazopanib. Trabectedin’s introduction in Europe in 2007 depended mainly on its activity in so-called L-sarcomas (liposarcoma and leiomyosarcoma); combination of trabectedin with other chemotherapies used in STS seems of particular interest. Methods: We initiated within the German Interdisciplinary Sarcoma Group (GISG) a phase I dose escalating trial evaluating the combination of trabectedin and gemcitabine in patients with advanced and/or metastatic L-sarcomas (GISG-02; ClinicalTrials.gov NCT01426633). Patients were treated with increasing doses of trabectedin and gemcitabine. The primary endpoint was to determine the maximum tolerated dose. Results: Five patients were included in the study. Two patients were treated on dose level 1 comprising trabectedin 0.9 mg/m2 on day 1 and gemcitabine 700 mg/m2 on days 1 + 8, every 3 weeks. Due to dose-limiting toxicity (DLT) in both patients (elevated transaminases and thrombocytopenia), an additional three patients were treated on dose level −1 with trabectedin 0.7 mg/m2 plus gemcitabine 700 mg/m2. Of these three patients, two demonstrated another DLT; therefore, the trial was stopped and none of the dose levels could be recommended for phase II testing. Conclusion: The GISG-02 phase I study was stopped with the conclusion that the combination of gemcitabine and trabectedin is generally not recommended for the treatment of patients with advanced and/or metastatic leiomyosarcoma or liposarcoma. Also, this phase I study strongly supports the necessity for careful evaluation of combination therapies. PMID:25591040

  17. [Surgery of retroperitoneal pheochromocytoma].

    PubMed

    Duff, C; van Segesser, L; Schmid, E R; Ziegler, W; Turina, M

    1989-06-01

    Retroperitoneal pheochromocytomas are very uncommon tumors. During the last 10 years only 3 cases have been operated on in our clinic. Two of them were primary retroperitoneal paragangliomas. These two patients are now well and without recurrence 1 and 10 years respectively after operation. The third patient had retroperitoneal metastases of an adrenal pheochromocytoma. Now, after 1 year he has disseminated metastases without response to therapy with (131I)metaiodobenzylguanidine (MIBG). The surgical procedure includes often extended resection and replacement of retroperitoneal blood vessels and requires therefore an experienced surgical team and optimal anesthetic management (Swan-Ganz-katheter). In two cases the aorta was resected and replaced with a Dacron-graft. Reimplantation of both renal arteries into the graft was necessary in one case. Further interventions were: nephrectomy (2x), resection and ligation of the inferior vena cava (1x), resection and replacement of the left renal vein (1x).

  18. An unusual sarcomatous retroperitoneal metastasis. A rare case report with a brief literature review.

    PubMed

    Lazar, A M; Straja, N D; Brătucu, E

    2012-01-01

    Retroperitoneal sarcomas are rare tumors associated with a high rate of recurrence and very bad prognosis. Their only efficient treatment is a negative- margin surgical resection that is extremely difficult to achieve. Retroperitoneal metastases from extremity sarcomas are considered unusual. In literature, such a metastatic pattern is described extremely rarely. In this paper we report a case of a very aggressive extremity chondrosarcoma, associated with local recurrence, multiple distant metastases, that finally led to a retroperitoneal metastasis. The recurrence and progression of the sarcoma in this localization were impressive, with a fast overcome of therapeutic options. Chemo- and radiotherapy have not proved to be efficacious in this context and they could have had a role in the deterioration of patient state of health. New tumor markers for the detection and follow-up of these tumors should be considered.

  19. [Retroperitoneal hematoma in pelvic fractures].

    PubMed

    Purghel, F; Jemna, C; Ciuvică, R

    2011-01-01

    Retroperitoneal trauma implies a wide variety of organs in multiple systems (digestive, urinary, circulatory, musculoskeletal); although their common result is the retroperitoneal hematoma, their management is completely different, an intervention indicated for a particular lesion being able to completely decompensate other lesions in case of insufficient diagnostic. The present material highlights the recent diagnostic and therapeutic particularities in retroperitoneal hematoma from pelvic fractures. We noted a recent trend in diminishing the role of the fracture pattern on standard pelvis X-ray in assessing the risk of hemodinamic instability, new markers being indicated as more predictive. CT scan with contrast substance, when applies, remains the gold standard in identifying the source of the vascular bleeding and in guiding the subsequent therapeutic maneuvers. The angiographic embolisation in arterial lesions remains the main therapeutic procedure in hemodinamical unstable patients, with the possibility of repeating it when needed; the C-clamp external fixator application is associated. The pre-peritoneal packing constantly gains support as an emergency hemostasis maneuver. The treatment should be adapted in each case, the hemodinamic instability being the trigger in initiation and repetition of the emergency therapeutic interventions mentioned above.

  20. [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.

  1. Long-term results in 144 localized Ewing's sarcoma patients treated with combined therapy

    SciTech Connect

    Bacci, G.; Toni, A.; Avella, M.; Manfrini, M.; Sudanese, A.; Ciaroni, D.; Boriani, S.; Emiliani, E.; Campanacci, M.

    1989-04-15

    The results of 144 previously untreated cases of primary Ewing's sarcoma of bone are reported with a minimum follow-up of 5 years. This series was treated between 1972 and 1982 at Istituto Ortopedico Rizzoli with a combined therapy. The local control of the disease consisted of amputation (ten cases), resection followed by radiation therapy (35-45 Gy) (48 cases) and radiation therapy alone (40-60 Gy) (86 cases). Adjuvant chemotherapy, rigorously standardized, was performed according two different protocols: the first (85 cases treated in the period 1972-1978) consisted of vincristine (VCR) Adriamycin (doxorubicin) (ADM), and cyclophosphamide (EDX); the second (59 cases treated in the period 1979-1982) of VCR, ADM, EDX and dactinomycin (DACT). At a follow-up of 5 to 16 years (median, 9), 59 patients (41%) are continuously disease-free (CDF), 81 (56%) developed metastatic disease and/or local recurrence, and four (3%) had a second malignancy. Three factors seem to be correlated to prognosis: the site of the initial lesion (only 23% of the pelvic lesions are represented in the CDF group versus 46% of the other locations); the chemotherapy protocol (32% of the cases in the first protocol are CDF versus 54% in the second); the type of local treatment (60% of the patients treated with amputation or resection plus radiotherapy versus 28% of those treated with radiation therapy alone are CDF). A local recurrence was observed in 24% of the patients (8% in the group locally treated with surgery or surgery plus radiation therapy versus 36% in the group treated with radiation therapy alone). These data suggest that even though adjuvant chemotherapy can improve the long-term results in localized Ewing's sarcoma patients, this disease still represents, in a high percentage of cases, a lethal process whose final prognosis widely depends on the local control of the lesion.

  2. Results of the Scandinavian Sarcoma Group XIV protocol for classical osteosarcoma

    PubMed Central

    2011-01-01

    Background and purpose The Scandinavian Sarcoma Group (SSG) XIV protocol is based on experience from previous SSG trials and other osteosarcoma intergroup trials, and has been considered the best standard of care for patients with extremity localized, non-metastatic osteosarcoma. We analyzed the outcome in 63 consecutive patients. Patients and methods From 2001 through 2005, 63 patients recruited from centers in Sweden, Norway, and Finland were included. They received preoperative chemotherapy consisting of 2 cycles of paired methotrexate (12 g/m2), cisplatin (90 mg/m2), and doxorubicin (75 mg/m2). 3 cycles were administered postoperatively, and poor histological responders were given 3 additional cycles of ifosfamide (10–12 g/m2) as a salvage strategy. Results With a median follow-up of 77 months for survivors, the estimated metastasis-free and sarcoma-related survival at 5 years was 70% and 76%, respectively. 53 patients were treated with limb salvage surgery or rotationplasty and 2 patients experienced a local recurrence. 3 toxic deaths were recorded, all related to acute toxicity from chemotherapy. The 5-year metastasis-free survival of poor histological responders receiving add-on treatment with ifosfamide was 47%, as compared to 89% for good histological responders. Interpretation Outcome from the SSG XIV protocol compares favorably with the results of previous SSG trials and other published osteosarcoma trials. However, salvage therapy given to poor responders did not improve outcome to a similar degree as for good responders. In a multi-institutional setting, more than four-fifths of the patients were operated with limb salvage surgery or rotationplasty, with few local recurrences. PMID:21434784

  3. Ewing sarcoma

    MedlinePlus

    Bone cancer - Ewing sarcoma; Ewing family of tumors; Primitive neuroectodermal tumors (PNET); Bone neoplasm - Ewing sarcoma ... Ewing sarcoma can occur anytime during childhood and young adulthood. But it usually develops during puberty, when bones ...

  4. [Retroperitoneal germ cell tumor].

    PubMed

    Borrell Palanca, A; García Garzón, J; Villamón Fort, R; Domenech Pérez, C; Martínez Lorente, A; Gunthner, S; García Sisamón, F

    1999-03-01

    We report a case of retroperitoneal extragonadal germ-cell tumor in an 17 years old patient who presented with aedema and pain in left inferior extremity asociated with hemopthysis caused by pulmonar metastasis, who was treated with chemotherapy and resection of residual mass and pulmonary nodes. Dyagnosis was stableshed by fine neadle aspiration biopsy of the wass. We comment on the difficult of stableshing differential dyagnosis between retroperitoneal extragonadal germ-cell tumor and metastasis of a testicular tumor. Dyagnosis is stableshed by the finding of a histologically malignant germ-cell tumor with normal testis. We considered physical examination and ecographyc exploration enough for a correct dyagnosis.

  5. Concealed primary aortic sarcoma induced hypertensive encephalopathy resulting from a thoracic aortic occlusion: a case report.

    PubMed

    Choi, Hyunmin; Yoon, Hee-Jeoung; Jang, Woo-Ik; Kim, Chang-Young; Doh, Joon-Hyung

    2013-04-18

    Primary aortic sarcoma is a rare condition that is frequently associated with distal embolization. In addition, growth characteristics of primary aortic sarcoma lead to the narrowing of the involved aortic lumen. A 72-year-old Korean male with primary aortic sarcoma showed progressive unexplained blood pressure elevation that didn't improve with additional antihypertensive drug therapy. Because follow-up measures were not taken, the patient ultimately developed hypertensive encephalopathy with concurrent embolic dissemination. Although we successfully performed open transcatheter embolectomy in both legs, the patient died because of multiple organ failure 3 days after surgery. Given the ominous prognosis for this condition, this case report highlights the fact that the value of early detection and prompt evaluation of altered vital signs should not be overemphasized. We describe a rare case of primary aortic sarcoma that showed hypertensive encephalopathy caused by thoracic aortic occlusion and also had embolic metastases to the lower extremities.

  6. [A CASE OF GRANULOCYTIC SARCOMA IN THE BILATERAL URETER].

    PubMed

    Miyagawa, Tomoaki; Tanaka, Ken; Ikeda, Atsushi; Komine, Manabu; Tsutsumi, Masakazu; Shinagawa, Atsushi

    2015-07-01

    A 61-year-old female presented with the complaints of fever and left back pain. She had previously undergone bone marrow transplantation for acute myeloid leukemia and achieved remission. Abdominal computed tomography (CT) revealed left hydronephrosis and suspected tumor lesions in the right upper ureter and left lower ureter. Ureteroscopy was performed for a diagnosis. A yellowish tumor was detected in the left lower ureter, and tissue biopsy was performed. Two weeks after the examination, abdominal CT revealed ascites and retroperitoneal dissemination. The results of the ascites cytology showed infiltration by leukemia cells, and the patient was diagnosed as having recurrent leukemia. A week later, she died. The ureteral tumor was diagnosed as a granulocytic sarcoma. Granulocytic sarcoma, a condition characterized by mass formation outside the bone marrow by granulocytic cells, is classified as a myeloid sarcoma, occurring in an estimated 2-8% of patients with myeloid leukemia. The possibility of granulocytic sarcoma should be considered when treating patients with a history of leukemia.

  7. The radiation response of sarcomas by histologic subtypes: a review with special emphasis given to results achieved with razoxane.

    PubMed

    Rhomberg, Walter

    2006-01-01

    Purpose. Relatively few results are available in the literature about the radiation response of unresectable sarcomas in relation to their histology. Therefore, an attempt was made to summarize the present situation. Materials and methods. This report is based on a review of the literature and the author's own experience. Adult-type soft tissue sarcomas, chondrosarcomas, and chordomas were analyzed. Radioresponse was mainly associated with the degree of tumor shrinkage, that is, objective responses. Histopathologic responses, that is, the degree of necrosis, are only discussed in relation to radiation treatment reports of soft tissue sarcomas as a group. Results. Radiation therapy alone leads to major responses in about 50% of lipo-, fibro-, leiomyo-, or chondrosarcomas. The response rate is less than 50% in malignant fibrous histiocytomas, synovial, neurogenic, and other rare soft tissue sarcomas. The response rates may increase up to 75% through the addition of radiosensitizers such as halogenated pyrimidines or razoxane, or by the use of high-LET irradiation. Angiosarcomas become clearly more responsive if biologicals, angiomodulating, and/or tubulin affinic substances are given together with radiation therapy. Razoxane is able to increase the duration and quality of responses even in difficult-to-treat tumors like chondrosarcomas or chordomas. Conclusions. The available data demonstrate that the radioresponsiveness of sarcomas is very variable and dependent on histology, kind of radiation, and various concomitantly given drugs. The rate of complete sustained remissions by radiation therapy alone or in combination with drugs is still far from satisfactory although progress has been made through the use of sensitizing agents.

  8. The Radiation Response of Sarcomas by Histologic Subtypes: A Review With Special Emphasis Given to Results Achieved With Razoxane

    PubMed Central

    Rhomberg, Walter

    2006-01-01

    Purpose. Relatively few results are available in the literature about the radiation response of unresectable sarcomas in relation to their histology. Therefore, an attempt was made to summarize the present situation. Materials and methods. This report is based on a review of the literature and the author's own experience. Adult-type soft tissue sarcomas, chondrosarcomas, and chordomas were analyzed. Radioresponse was mainly associated with the degree of tumor shrinkage, that is, objective responses. Histopathologic responses, that is, the degree of necrosis, are only discussed in relation to radiation treatment reports of soft tissue sarcomas as a group. Results. Radiation therapy alone leads to major responses in about 50% of lipo-, fibro-, leiomyo-, or chondrosarcomas. The response rate is less than 50% in malignant fibrous histiocytomas, synovial, neurogenic, and other rare soft tissue sarcomas. The response rates may increase up to 75% through the addition of radiosensitizers such as halogenated pyrimidines or razoxane, or by the use of high-LET irradiation. Angiosarcomas become clearly more responsive if biologicals, angiomodulating, and/or tubulin affinic substances are given together with radiation therapy. Razoxane is able to increase the duration and quality of responses even in difficult-to-treat tumors like chondrosarcomas or chordomas. Conclusions. The available data demonstrate that the radioresponsiveness of sarcomas is very variable and dependent on histology, kind of radiation, and various concomitantly given drugs. The rate of complete sustained remissions by radiation therapy alone or in combination with drugs is still far from satisfactory although progress has been made through the use of sensitizing agents. PMID:17040092

  9. Pregnancy and genital sarcoma: a systematic review of the literature.

    PubMed

    Matsuo, Koji; Eno, Michele L; Im, Dwight D; Rosenshein, Neil B

    2009-08-01

    We conducted a literature review to determine the clinical characteristics of genital sarcoma during pregnancy. The systematic literature search was conducted using the search engines PubMed and MEDLINE with keywords "sarcoma" and "pregnancy" and was limited to female genital organs such as ovary, uterus, cervix, vagina, vulva, and retroperitoneal sarcoma. Kaposi's sarcoma, metastatic sarcoma, history of sarcoma, bone sarcoma located in pelvis, and fetal sarcoma were excluded in this study. There were 40 cases of genital sarcoma during pregnancy between 1955 and 2007. The majority of the cases were uterine sarcoma (37.5%), followed by retroperitoneal sarcoma (27.5%), vulvar sarcoma (22.5%), and vaginal sarcoma (12.5%). Mean age of the patient was 27.8 +/- 7.0. The distribution in the onset of symptoms had two peaks: first trimester (27.5%) and third trimester (50.0%). Growing mass (42.5%), abdominal pain (30.0%), and vaginal bleeding (22.5%) were the three most common symptoms. Incidental diagnosis was made in 22.5% and included during cesarean section (12.5%) and routine pelvic exam (7.5%). The cases initially not suspicious for malignancy were 42.5%. Thirty-three (82.5%) cases had live-born infants with term delivery in 55.2%. Mean birth weight was 2843 +/- 791 g, and male infants were more common (66.7%). Intrauterine growth retardation was seen in 12.5% of cases. Preterm labor was a common complication. Median survival period was 2.5 years (95% confidence, 1.9 to 3.1). The 2-, 3-, and 5-year cumulative survival rates were 60%, 38%, and 17%, respectively. Genital sarcomas in pregnancy are rare. There is a delay in diagnosis due to low index of suspicion. A majority had live births, and the 5-year survival is similar to that of advanced-stage sarcoma in nonpregnant women.

  10. Surgical ‘damage control’ treatment of a large retroperitoneal liposarcoma encasing a horseshoe kidney

    PubMed Central

    Andreoni, B; Chiappa, A; Pace, U; Bertani, E; Verweij, F; Orsi, F; Petralia, G; Tullii, M; Venturino, M; Pelosi, G

    2008-01-01

    Damage control is a surgical strategy for severely compromised trauma patients based on speed control of life-threatening injuries that aims to rapidly resuscitate patients in an intensive care unit (ICU). We report on the use of such therapeutic strategy in a patient affected by a retroperitoneal sarcoma concomitant to a horseshoe kidney, a relatively rare anatomical malformation. PMID:22275967

  11. Laser treatment of retroperitoneal lymphangiomas in children

    NASA Astrophysics Data System (ADS)

    Waldschmidt, U.; Waldschmidt, Juergen; Cholewa, D.; Stroedter, L.

    2000-06-01

    Retroperitoneal Lymphangiomas are rare tumor-like lesions, predominantly found in the new-born period and pediatric age. They are always benign. About 1 percent of Lymphangiomas arise in the retroperitoneal space. Until now surgical excision was the only consistently effective method. Compete excision of the tumor is in more than a half of the cases not possible, and the rate of occurrences is about 50 percent. Because of this background new therapeutic methods were sought. In 1986 we have introduced the interstitial laser therapy in the treatment of the Lymphangiomas and treated more than 200 cases, 10 of them were located in the retroperitoneum. Technique and results are discussed.

  12. Organized hematoma mimicking retroperitoneal cystic tumors

    PubMed Central

    Ivankiv, Taras; Ogurtsov, Oleksii; Pokhylevych, Galina

    2015-01-01

    Introduction Isolated retroperitoneal cysts are uncommon with an estimated incidence of 1/5750–1/250,000. In women they occur about 1.5–2 times more often than in men. The largest numbers of patients are young or middle aged (20–50 years). Lack of knowledge about the causes of these rare entities and asymptomatic clinical picture often leads to diagnostic and tactical mistakes. Methods The medical history of 54-year old male patient B., who has been hospitalized at Surgical Department №1 of Danylo Halytsky Lviv National Medical University (Surgical Department of Lviv Regional Clinical Hospital), was processed retrospectively. Result Diagnosing of retroperitoneal organized hematoma in the early stages is not always possible, because exploration of retroperitoneal space can be difficult. General tests and tumor markers are usually normal range and not prognostically informative in this case. Decisively important were imaging diagnostic methods—USG and CT. As clinical cases of organized hematoma are quite rare, finding out retroperitoneal formation with irregular contours and infiltrative component indicates for retroperitoneal tumors. Thus, this formation accumulated contrast that says for increased vascularization. Intraoperative: formation with thick walls and heterogeneous structure. Histological diagnosis: hematoma in a phase of deep organization. On our opinion, taking into account location and structure of tumors, laparoscopic intervention was not appropriate, open surgery was reasonable approach. Preoperative biopsy has a crucial role to set preliminary diagnosis. Conclusion Despite the fact that organized retroperitoneal hematomas are quite rare, their diagnosis requires detailed examination and histological verification. PMID:26764890

  13. Combined therapy of localized Ewing's sarcoma of bone: Analysis of results in 100 patients

    SciTech Connect

    Barbieri, E.; Emiliani, E.; Zini, G.; Mancini, A.; Toni, A.; Frezza, G.; Neri, S.; Putti, C.; Babini, L. )

    1990-11-01

    From 1979 to 1986, 182 patients with biopsy proven diagnosis of Ewing's sarcoma of bone were observed. One hundred of the 182 patients (72 males, 28 females, median age 15.8 years) with localized disease and no previous treatment were treated with chemotherapy (VCR, ADM, CTX, D-ACT) for 15-18 months. Local treatment was radiotherapy (42 patients), surgery (31 patients), or a combination of both (27 pts). Radiation doses ranged from 45 to 64 Gy given with conventional fractionation. Median follow-up was 51.2 months (24-106). Overall and disease-free survival were, respectively, 58.7 and 42.6%. Resected patients tended to have a better local control (Surgery 93.6%, Surgery + Radiation therapy 92.6%, Radiation therapy 69.1%). Disease-free survival was significantly related to the volume of the primary tumor (bulky: 33.2%, not-bulky: 57.7%), to site (extremities 54.6%, central sites 16.6%, other sites 40.9%), and to local treatment (Radiation therapy 30.3%, Surgery + Radiation therapy 47.9%, Surgery 59.1%). These results are, however, biased because resected patients tended to have smaller tumors in favorable sites.

  14. Neuropraxia following resection of a retroperitoneal liposarcoma.

    PubMed

    Tsiao, Stevenson; Aydin, Nail; Misra, Subhasis

    2017-01-01

    This is a unique case of neuropraxia of femoral nerve seen after resection of retroperitoneal liposarcoma which has not been reported before in the literature. Neuropraxia, a transient paralysis due to blockage of nerve conduction, commonly associated with athletes and orthopedic procedures, has not been previously reported as a complication following resection of retroperitoneal sarcoma. This is an 81-year-old female who, on CT for evaluation of her atherosclerosis, was found to have an incidental right-sided retroperitoneal mass extending from the right renal capsule inferiorly through the inguinal canal. At this point, the patient reported mild right sided abdominal pain and right lower back pain, but reported no neuromotor deficits of the right lower extremity. Given the symptoms of the patient as well as the size, location and the density of the lesion, surgical intervention was pursued. On exploration, the lipomatous lesion, suggestive of liposarcoma, was invading the right genitofemoral nerve and ilioinguinal nerve which were sacrificed to ensure a complete oncologic resection. Following complete removal of the mass, she developed right side femoral nerve neuropraxia, suffering complete loss of motor function in the femoral distribution. Pathology revealed the mass to be a low grade liposarcoma. The patient required only physical therapy and oral prednisone following surgery for treatment of the neuropraxia. She responded well and has regained significant neuromotor function of the affected limb. Cases presenting with post-resection neurological sequelae without any known intraoperative nerve injury may respond very well to conservative treatment. Hence, it is very important to collaborate with Neurology and Physical Therapy to achieve best possible outcome. Copyright © 2017 The Author(s). Published by Elsevier Ltd.. All rights reserved.

  15. Intensity Modulated Radiation Therapy for Primary Soft Tissue Sarcoma of the Extremity: Preliminary Results

    SciTech Connect

    Alektiar, Kaled M. . E-mail: alektiak@mskcc.org; Hong, Linda; Brennan, Murray F.; Della-Biancia, Cesar; Singer, Samuel

    2007-06-01

    Purpose: To report preliminary results on using intensity modulated radiation therapy (IMRT) as an adjuvant treatment in primary soft tissue sarcoma (STS) of the extremity. Methods and Materials: Between February 2002 and March 2005, 31 adult patients with primary STS of the extremity were treated with surgery and adjuvant IMRT. Tumor size was >10 cm in 74% of patients and grade was high in 77%. Preoperative IMRT was given to 7 patients (50 Gy) and postoperative IMRT (median dose, 63 Gy) was given to 24 patients. Complete gross resection including periosteal stripping or bone resection was required in 10, and neurolysis or nerve resection in 20. The margins were positive or within 1 mm in 17. Complications from surgery and radiation therapy (RT) were assessed using the Common Terminology Criteria for Adverse Events grading system. Results: Median follow-up time was 23 months. Grade 1 RT dermatitis developed in 71% of patients, Grade 2 in 16%, and Grade 3 in 10%. Infectious wound complications developed in 13% and noninfectious complications in 10%. Two patients (6.4%) developed fractures. Grade 1 neuropathy developed in 28% of patients and Grade 2 in 5%. The rates of Grade 1 and 2 joint stiffness were each 19%. Grade 1 edema was observed in 19% of patients and Grade 2 in 13%. The 2-year local control, distant control, and overall survival were 95%, 65%, and 81%, respectively. Conclusion: Intensity modulated RT appears to provide excellent local control in a difficult group of high-risk patients. The morbidity profile is also favorable, but longer follow-up is needed to confirm the results from this study.

  16. Pediatric Sarcomas.

    PubMed

    Williams, Regan F; Fernandez-Pineda, Israel; Gosain, Ankush

    2016-10-01

    Pediatric sarcomas are a heterogeneous group of tumors accounting for approximately 10% of childhood solid tumors. Treatment is focused on multimodality therapy, which has improved the prognosis over the past two decades. Current regimens focus on decreasing treatment for low-risk patients to decrease the long-term side effects while maximizing therapy for patients with metastatic disease to improve survival. Pediatric sarcomas can be divided into soft tissue sarcomas and osseous tumors. Soft tissue sarcomas are further delineated into rhabdomyosarcomas, which affect young children and nonrhabdomyosarcomas, which are most common in adolescents. The most common bone sarcomas are osteosarcomas and Ewing's sarcoma.

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

    PubMed Central

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

    2016-01-01

    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. PMID:27746879

  18. Treatment for soft tissue sarcoma in childhood and adolescence. Austrian results within the CWS 96 study.

    PubMed

    Modritz, Ditha; Ladenstein, Ruth; Pötschger, Ulrike; Amman, Gabriele; Dieckmann, Karin; Horcher, Ernst; Urban, Christian; Meister, Bernhard; Schmitt, Klaus; Jones, Regina; Kaulfersch, Wilhelm; Haas, Hans; Moser, Reinhard; Stöllinger, Olaf; Peham, Martina; Gadner, Helmut; Koscielniak, Ewa; Treuner, Jörn

    2005-03-01

    The aim of the CWS 96 Study was to achieve an optimal treatment in children and adolescents with soft tissue sarcoma (STS) implementing a further refinement of risk-adapted allocation to chemotherapy, surgery and radiotherapy. Treatment stratification was based on tumour histology, TNM status, postsurgical stage, localisation and age. Local tumour control was ensured by surgery and risk-adapted radiotherapy. From 1995 to 2002, 89 patients were registered in Austria. The 3-year event-free survival (EFS) and overall survival rates (OS) were 63% +/- 6% and 71% +/- 6%, respectively. 59/89 patients had localised RMS-like (rhabdomayosarcoma) STS (EFS 73% +/- 7%), 14 had localised NON-RMS STS (EFS 54% +/- 16%) and 15 patients had metastatic disease at diagnosis (EFS 33% +/- 12%), 1 patient had fibromatosis. The EFS rates at 3 years in patients with localised RMS-like tumours according to risk group were 92% +/- 8% for low and standard risk (12 patients) and 67% +/- 8% for high risk (47 patients). Favourable primary tumour sites of nonmetastatic RMS-like STS i.e. orbit, head/neck nonparameningeal or genitourinary non-bladder/prostate were diagnosed in 15 patients (1/15 patients died). In 44 patients with unfavourable localisation such as parameningeal, genitourinary bladder/prostate, extremity and others, 7 deceased. The 3 year EFS according to histology in patients with RMS-like STS was 61% +/- 11% for RME (embryonal RMS ) (28 patients) and 71% +/- 15% for RMA (alveolar RMS) (10 patients). The most common treatment failure was local relapse occurring in 21% of patients in the high-risk group. Risk-adapted individualisation of treatment led to a reduction of chemotherapy in the low and standard risk group without compromising survival. The outcome of RME and RMA was similar in this cohort of patients. These preliminary results after a median observation time of 2.5 years confirm the CWS 96 strategy.

  19. Radiation therapy for Ewing's sarcoma: Results from Memorial Sloan-Kettering in the modern era

    SciTech Connect

    La, Trang H.; Meyers, Paul A.; Wexler, Leonard H.; Alektiar, Kaled M.; Healey, John H.; Laquaglia, Michael P.; Boland, Patrick J.; Wolden, Suzanne L. . E-mail: woldens@mskcc.org

    2006-02-01

    Purpose: To evaluate the outcomes of patients with Ewing's sarcoma family of tumors (ESFT) treated with modern radiotherapy techniques with MRI along with optimal chemotherapy. Methods and Materials: The records of all 60 patients with ESFT who received radiation to the primary site between 1990 and 2004 were reviewed. All patients received chemotherapy, including vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide. Radiation was used as the sole modality for local control in 31 patients and was given either before (n = 3) or after surgical resection (n = 26) in the remainder. All patients had MRI and CT scan-based treatment planning, and 43% received intensity-modulated radiation therapy. Radiation doses ranged from 30 Gy to 60 Gy (median, 51 Gy), and 35% received hyperfractionated radiotherapy. Results: Median age was 16 years (range, 2-40 years). Because of selection bias for radiotherapy, the majority of primary tumors were centrally located (72%): spine (n = 18), pelvis (n = 15), extremities (n 12), chest wall (n = 5), head and neck (n = 5), and other (n = 5). Thirty-eight percent of patients presented with metastatic disease, and 52% of primary tumors were {>=}8 cm. Actuarial 3-year local control was 77%. The presence of metastases at diagnosis was an adverse prognostic factor for local control (84% vs. 61%, p = 0.036). No other predictive factors for local failure were identified. In patients without metastatic disease, 3-year disease-free and overall survival rates were 70% and 86%, respectively, whereas in patients with metastases they were both 21%. Follow-up of surviving patients was 6-178 months (median, 41 months). Conclusion: In this unfavorable cohort of ESFT patients, radiation therapy was an effective modality for local control, especially for patients without metastases. The presence of metastases at diagnosis is a predictive factor not only for death but also for local failure.

  20. Long-Term Results Following Postoperative Radiotherapy for Soft Tissue Sarcomas of the Extremity

    SciTech Connect

    McGee, Lisa; Indelicato, Daniel J.; Dagan, Roi; Morris, Christopher G.; Knapik, Jacquelyn A.; Reith, John D.; Scarborough, Mark T.; Gibbs, C. Parker; Marcus, Robert B.; Zlotecki, Robert A.

    2012-11-15

    Purpose: To review long-term outcomes following postoperative radiotherapy (RT) for extremity soft tissue sarcoma (STS) and identify variables affecting the therapeutic ratio. Methods and Materials: Between 1970 and 2008, 173 patients with localized extremity STS were treated with postoperative radiation. No patients received prior irradiation. Sixteen percent of tumors had recurred after initial surgery alone; 89% of tumors were high grade. The median patient age was 57 years (range, 18-86 years). Sixty-one percent underwent >1 surgery before RT in an attempt to achieve wide negative margins. Final margin status was negative in 70% and marginal or microscopically positive in 30%. The median time between final surgery and start of RT was 40 days. The median RT dose was 65 Gy (range, 49-74 Gy). The median follow-up for all patients was 10.4 years and 13.2 years among survivors. Results: At 10 years, local control (LC), cause-specific survival (CSS), and overall survival (OS) rates were 87%, 80%, and 70%, respectively, with 89% of local failures occurring within 3 years after treatment. On multivariate analysis, age >55 years (82% vs 93%, P<.05) and recurrent presentation (67% vs 91%, P<.05) were associated with inferior 10-year LC. The LC according to final margin status was 90% for wide negative margins vs 79% for marginal/microscopically positive margins (P=.08). Age >55 years and local recurrence were associated with inferior CSS and OS on multivariate analysis. Twelve percent of patients experienced grade 3+ toxicity; 12 of these occurred in patients with tumors of the proximal lower extremity, with the most common toxicity of pathologic fracture occurring in 6.3%. Conclusions: This large single-institution series confirms that postoperative RT for STS of the extremities provides good long-term disease control with acceptable toxicity. Our experience supports recurrent presentation and older age as adverse prognostic factors for LC.

  1. Soft Tissue Sarcoma Across the Age Spectrum: A Population-Based Study from the Surveillance Epidemiology and End Results Database

    PubMed Central

    Ferrari, Andrea; Sultan, Iyad; Huang, Tseng Tien; -Galindo, Carlos Rodriguez; Shehadeh, Ahmad; Meazza, Cristina; Ness, Kirsten K.; Casanova, Michela; Spunt, Sheri L.

    2014-01-01

    Background Soft tissue sarcomas (STS) are a heterogeneous group of mesenchymal malignancies that occur throughout the lifespan. The impact of age on disease features and outcome is unclear. Methods We analyzed the clinical features and outcome of all STS cases registered between 1973 and 2006 in the SEER database. Results There were 48,012 cases that met the selection criteria. Individuals less than 20 years of age represented 5.6%, with rhabdomyosarcoma being the most common subtype. In adults, the most common types were Kaposi sarcoma, fibrohistiocytic tumors, and leiomyosarcoma. Rhabdomyosarcoma was the only entity with a median age < 20 years. Male predominance (male:female of 1.5:1) was noticed for almost all types of STS, except for alveolar soft part sarcoma and leiomyosarcoma. Tumor stage was similar across different age groups. Younger patients (<50 years) had significantly better survival than older patients (88.8%±0.2% vs. 40%±0.3%, P<0.001), but for most histologies the survival decline with advancing age was gradual and did not occur abruptly at the onset of adulthood. The decline in survival with advancing age was particularly significant for rhabdomyosarcoma. Conclusion With few exceptions, the clinical features of STS are similar in children and adults. However, individuals over 50 years of age have an inferior survival. PMID:21793180

  2. [A rare case of nerve-sheath sarcoma with rhabdomyoblastic differentiation (malignant triton tumor)].

    PubMed

    Malerba, M; Garofalo, A

    2003-01-01

    Malignant peripheral nerve sheath tumors (MPNST) are spindle-cell sarcomas that appear in a setting of neurofibroma or schwannoma or are associated with peripheral nerves or demonstrate nerve sheath differentiation. Malignant triton tumor (MTT) is a subtype of MPNST that also contain tissue with skeletal muscle differentiation (embryonal, plemorphic and botryoid rhabdomyosarcoma). The estimated incidence of MPNSTs in patients with NF1 is 2-5% compared with 0.0001% in the general population and approximately 69% of the reported cases of MTT are associated with von Recklinghausen disease. In July 2002 a 37-year old man was readmitted to the Department of Oncologic Surgery of the S. Camillo-Forlanini Hospital in Rome for both a right-sided retroperitoneal paravertebral not palpable mass, incidentally detected at a follow-up MRI, and a left-sided popliteal mass, discovered at clinical evaluation. Seventeen months before, when the patient underwent surgery at the same Department for both a left-sided paravertebral inferior mediastinal neurofibroma and a right-sided axillary neurofibroma, diagnosis of von Recklinghausen disease (NF1) was made, according to the criteria established by the NIH Consensus Development. Conference on Neurofibromatosis of 1987. A xifopubic laparotomy was performed: the tumor appeared to be localized, well-capsulated and strictly associated to the lumbar and sacral nervous radicles (L4, L5, S1) without evidence of invasion. The tumor was completely resected with sparing of the psoas muscle and the lumbar plexus through a subperineural dissection technique. No intra-operative pathologic examination was performed. Postoperative pathologic findings showed evidence for a trition tumor. The popliteal mass was resected too and resulted to be a neurofibroma just like the tumors resected 17 months before when diagnosis of von Recklinghausen disease was made. The patient was disease free 6 months after initial surgery. Sarcoma arising in anatomic site

  3. Kaposi sarcoma

    MedlinePlus

    Kaposi's sarcoma; HIV - Kaposi; AIDS - Kaposi ... Before the HIV/AIDS epidemic, Kaposi sarcoma was seen mainly in older Italian and Jewish men, and rarely, in older women. Among this group, the tumors developed slowly. In ...

  4. Laparoscopic renal surgery after spontaneous retroperitoneal hemorrhage.

    PubMed

    Hernandez, Fernando; Ong, Albert M; Rha, Koon H; Pinto, Peter A; Kavoussi, Louis R

    2003-09-01

    We assessed the role of laparoscopic management in patients following spontaneous retroperitoneal hemorrhage from a renal tumor. A retrospective chart review revealed 4 patients with spontaneous retroperitoneal hemorrhage treated at our institution in the last 2 years. After conservative management elsewhere patients were referred for definitive therapy. Patient characteristics and tumor size were examined and correlated with ease of surgical dissection and surgical outcome. No patient had any history of trauma. Computerized tomography was used to identify the initial extent of hemorrhage in all patients. All patients underwent successful laparoscopic exploration without the need for open conversion. Three patients underwent radical nephrectomy and 1 underwent laparoscopic partial nephrectomy. Renal hemorrhage extending outside of the renal capsule was associated with significantly more adhesions than renal hemorrhage confined to the renal capsule. Mean patient age was 56 years (range 36 to 70). Mean retroperitoneal tumor size was 5.3 cm (range 2.5 to 10). Three renal hematomas were extracapsular and 1 was subcapsular. Mean operative time was 182.3 minutes (range 59 to 235). Average estimated blood loss was 800 cc (range 150 to 2,100). Nontraumatic retroperitoneal hemorrhage of renal origin may be managed using traditional laparoscopic techniques with results similar to those achieved with open renal exploration. These cases may prove technically challenging due to fibrosis and associated tissue plane loss.

  5. Laparoscopic resection of retroperitoneal benign neurilemmoma

    PubMed Central

    Park, Joon Seong; Kang, Chang Moo; Yoon, Dong Sup; Lee, Woo Jung

    2017-01-01

    Purpose The aim of this study was to verify that laparoscopic resection for treating retroperitoneal benign neurilemmoma (NL) is expected to be favorable for complete resection of tumor with technical feasibility and safety. Methods We retrospectively analyzed 47 operations for retroperitoneal neurogenic tumor at Yonsei University College of Medicine, Severance Hospital and Gangnam Severance Hospital between January 2005 and September 2015. After excluding 21 patients, the remaining 26 were divided into 2 groups: those who underwent open surgery (OS) and those who underwent laparoscopic surgery (LS). We compared clinicopathological features between the 2 groups. Results There was no significant difference in operation time, estimated blood loss, transfusion, complication, recurrence, or follow-up period between 2 groups. Postoperative hospital stay was significantly shorter in the LS group versus the OS group (OS vs. LS, 7.00 ± 3.43 days vs. 4.50 ± 2.16 days; P = 0.031). Conclusion We suggest that laparoscopic resection of retroperitoneal benign NL is feasible and safe by obtaining complete resection of the tumor. LS for treating retroperitoneal benign NL could be useful with appropriate laparoscopic technique and proper patient selection. PMID:28289669

  6. Symptoms at diagnosis as independent prognostic factors in retroperitoneal liposarcoma.

    PubMed

    Taguchi, Satoru; Kume, Haruki; Fukuhara, Hiroshi; Morikawa, Teppei; Kakutani, Shigenori; Takeshima, Yuta; Miyazaki, Hideyo; Suzuki, Motofumi; Fujimura, Tetsuya; Nakagawa, Tohru; Ishikawa, Akira; Igawa, Yasuhiko; Homma, Yukio

    2016-02-01

    The prognostic factors of retroperitoneal liposarcoma have yet to be clearly determined due to its rarity, whereas the prognostic value of symptoms at diagnosis has never been evaluated to date. In this context, we reviewed 24 consecutive patients with primary retroperitoneal liposarcoma who underwent surgical resection with curative intent at our institution. The Kaplan-Meier analysis and the log-rank test were used to estimate progression-free survival (PFS; primary endpoint) and sarcoma-specific survival (SSS; secondary endpoint). The effect of various clinicopathological factors, including symptoms at diagnosis, on these two endpoints was assessed with a Cox proportional hazards model. During the study period, 11 patients (45.8%) developed recurrence after the initial surgery and 8 (33.3%) succumbed to retroperitoneal liposarcoma, with a median follow-up of 64 months. A total of 16 patients (66.7%) had symptoms at diagnosis, while the remaining 8 (33.3%) were diagnosed incidentally. The symptoms were palpability of the tumor (n=8); abdominal pain/fullness (n=3); flank pain/fullness (n=2); lower extremity pain (n=1); testicular pain due to varicocele (n=1); and discomfort on urination (n=1). Patients with symptoms at diagnosis were significantly more likely to develop recurrence (log-rank test, P=0.0196) and were also more likely to succumb to sarcoma (P=0.0778) compared with asymptomatic patients. On the multivariate analysis, symptoms at diagnosis and dedifferentiated components were independent predictors of poor PFS, while positive surgical margins were predictors of poor SSS. Given that symptoms at diagnosis are easily accessible for physicians, they may prove to be useful additional prognostic factors for primary retroperitoneal liposarcoma.

  7. Comparison between preoperative biopsy and post-excision histology results in sarcoma: experience at Chris Hani Baragwanath Academic Hospital, Johannesburg, South Africa.

    PubMed

    Panda, Kitela Ghislain; Hale, Martin J; Kruger, Deirdre; Luvhengo, Thifhelimbilu Emmanuel

    2014-06-06

    Tumour size, grade and subtype are the main prognostic factors in adult patients presenting with soft-tissue sarcoma. Planning for appropriate management, including the need for additional staging investigations and neoadjuvant therapy, is dependent on reliable preoperative histopathological results. To determine whether there is agreement between preoperative and post-excision histological findings in patients presenting with soft-tissue sarcoma, and whether the agreement is influenced by the subtypes of sarcomas. Records of adult patients who had soft-tissue sarcomas excised were reviewed. Kaposi's sarcoma and gastrointestinal stromal tumours were excluded. Data were retrieved from the Department of Anatomical Pathology of the National Health Laboratory Service and theatre records at Chris Hani Baragwanath Academic Hospital, and included patient demography, tumour sites and size, HIV status, biopsy types and post-excision histological findings. Records of 153 patients were found (median age 44 years). The majority of the sarcomas were >5 cm in diameter, deep seated and localised in extremities. The commonest subtype, irrespective of HIV status, was dermatofibrosarcoma protuberans. Fine-needle aspiration biopsy (FNAB) results were inaccurate in determining the malignant nature, grade and subtype of sarcoma. Rates of accurate tumour subtype classification following core needle and incision biopsies when compared with post-excision histological findings were 73.1% and 78.3%, respectively. FNAB should not be used in the primary evaluation of soft-tissue tumours. A report of spindle cells on the FNAB smear should be followed by core needle or incision biopsy. Incision biopsy is superior to core needle biopsy in the classification of sarcomas by subtype.

  8. Transacatheter arterial embolization for malignant osseous and soft tissue sarcomas. II. Clinical results

    SciTech Connect

    Nagata, Yasushi; Mitsumori, Michihide; Okajima, Kaoru; Mizowaki, Takashi; Fujiwara, Kazuhisa; Sasai, Keisuke; Nishimura, Yasumasa; Hiraoka, Masahiro; Abe, Mitsuyuki; Shimizu, Katsuji; Kotoura, Yoshihiko

    1998-05-15

    Purpose: To evaluate the clinical effects of transcatheter arterial embolization (TAE) on malignant bone and soft tissue tumors.Methods: TAE was performed in 10 patients with primary bone and soft tissue sarcomas and in 31 patients with metastatic bone tumors. The embolized arteries were the internal iliac artery in 30 cases, the intercostal artery in six cases, the lumbar artery in five cases, the suprascapular artery in three cases, and the iliolumbar artery, the internal pudendal artery, and the lateral sacral artery in one case each. The embolized material was gelatin sponge particles. The chemotherapeutic drugs were usually 20-40 mg of doxorubicin for primary and metastatic tumors and 50-100 mg of cisplatin only for primary tumors. In addition, 50-60 Gy of 10-MV radiotherapy with or without radiofrequency (RF)-capacitive hyperthermia in four sessions was administered before TAE for primary tumors only.Results: Even though the pain score increased immediately after TAE, 30 of 38 (79%) patients with pain (8 of 9 with primary tumors, and 22 of 29 with metastases) achieved pain control after TAE. A necrotic low-density area shown by computed tomography (CT) after TAE was found in 31 of 41 (76%) tumors [8 of 10 (80%) with primary tumors, and 23 of 31 (74%) with metastatic tumors]. The tumor size decreased in 14 of 25 (56%) primary and metastatic tumors after 3 months. Osteosclerotic changes appeared in two cases of metastatic tumors after 6 months. In five tumors resected after TAE, large areas of necrosis within the tumor were confirmed histologically. Transient local pain and numbness appeared after TAE, but were relieved by drug treatment within 1 week. No severe complications except a case of gluteal muscle necrosis were encountered after TAE. The 1-year survival rate of the patients with primary tumors was 38.1%, and the median survival was 18 months. The longest survival was 84 months. The 1-year survival rate of the patients with metastatic bone tumors was

  9. Transcatheter Arterial Embolization for Malignant Osseous and Soft Tissue Sarcomas. II. Clinical Results

    SciTech Connect

    Nagata, Yasushi; Mitsumori, Michihide; Okajima, Kaoru; Mizowaki, Takashi; Fujiwara, Kazuhisa; Sasai, Keisuke; Nishimura, Yasumasa; Hiraoka, Masahiro; Abe, Mitsuyuki; Shimizu, Katsuji; Kotoura, Yoshihiko

    1998-05-15

    Purpose: To evaluate the clinical effects of transcatheter arterial embolization (TAE) on malignant bone and soft tissue tumors. Methods: TAE was performed in 10 patients with primary bone and soft tissue sarcomas and in 31 patients with metastatic bone tumors. The embolized arteries were the internal iliac artery in 30 cases, the intercostal artery in six cases, the lumbar artery in five cases, the suprascapular artery in three cases, and the iliolumbar artery, the internal pudendal artery, and the lateral sacral artery in one case each. The embolized material was gelatin sponge particles. The chemotherapeutic drugs were usually 20-40 mg of doxorubicin for primary and metastatic tumors and 50-100 mg of cisplatin only for primary tumors. In addition, 50-60 Gy of 10-MV radiotherapy with or without radiofrequency (RF)-capacitive hyperthermia in four sessions was administered before TAE for primary tumors only. Results: Even though the pain score increased immediately after TAE, 30 of 38 (79%) patients with pain (8 of 9 with primary tumors, and 22 of 29 with metastases) achieved pain control after TAE. A necrotic low-density area shown by computed tomography (CT) after TAE was found in 31 of 41 (76%) tumors [8 of 10 (80%) with primary tumors, and 23 of 31 (74%) with metastatic tumors]. The tumor size decreased in 14 of 25 (56%) primary and metastatic tumors after 3 months. Osteosclerotic changes appeared in two cases of metastatic tumors after 6 months. In five tumors resected after TAE, large areas of necrosis within the tumor were confirmed histologically. Transient local pain and numbness appeared after TAE, but were relieved by drug treatment within 1 week. No severe complications except a case of gluteal muscle necrosis were encountered after TAE. The 1-year survival rate of the patients with primary tumors was 38.1%, and the median survival was 18 months. The longest survival was 84 months. The 1-year survival rate of the patients with metastatic bone tumors was

  10. Positive psoas sign in presentation of retroperitoneal malignant triton tumour

    PubMed Central

    Winterbottom, Christopher Toby

    2010-01-01

    This article describes a case of a rare malignant neoplasm presenting to the emergency department with common symptomatology and its subsequent identification using a simple physical examination technique. Discussion includes a description of this rare soft tissue sarcoma and a consideration of the value of the psoas sign as a part of the routine abdominal exam to detect intra-abdominal and retroperitoneal pathology. In conclusion, this article acts as a reminder to all clinicians that uncommon and significant pathology may present to the emergency department masquerading as a common, seemingly benign, complaint, but can be clinically identified using simple techniques available to all and rapidly investigated using appropriate special investigations. PMID:22479296

  11. The Epidemiology of Sarcoma

    PubMed Central

    2012-01-01

    Sarcomas account for over 20% of all pediatric solid malignant cancers and less than 1% of all adult solid malignant cancers. The vast majority of diagnosed sarcomas will be soft tissue sarcomas, while malignant bone tumors make up just over 10% of sarcomas. The risks for sarcoma are not well-understood. We evaluated the existing literature on the epidemiology and etiology of sarcoma. Risks for sarcoma development can be divided into environmental exposures, genetic susceptibility, and an interaction between the two. HIV-positive individuals are at an increased risk for Kaposi’s sarcoma, even though HHV8 is the causative virus. Radiation exposure from radiotherapy has been strongly associated with secondary sarcoma development in certain cancer patients. In fact, the risk of malignant bone tumors increases as the cumulative dose of radiation to the bone increases (p for trend <0.001). A recent meta-analysis reported that children with a history of hernias have a greater risk of developing Ewing’s sarcoma (adjusted OR 3.2, 95% CI 1.9, 5.7). Bone development during pubertal growth spurts has been associated with osteosarcoma development. Occupational factors such as job type, industry, and exposures to chemicals such as herbicides and chlorophenols have been suggested as risk factors for sarcomas. A case-control study found a significant increase in soft tissue sarcoma risk among gardeners (adjusted OR 4.1, 95% CI 1.00, 14.00), but not among those strictly involved in farming. A European-based study reported an increased risk in bone tumors among blacksmiths, toolmakers, or machine-tool operators (adjusted OR 2.14, 95% CI 1.08, 4.26). Maternal and paternal characteristics such as occupation, age, smoking status, and health conditions experienced during pregnancy also have been suggested as sarcoma risk factors and would be important to assess in future studies. The limited studies we identified demonstrate significant relationships with sarcoma risk, but many of

  12. Adult soft tissue sarcoma

    MedlinePlus

    STS; Leiomyosarcoma; Hemangiosarcoma; Kaposi's sarcoma; Lymphangiosarcoma; Synovial sarcoma; Neurofibrosarcoma; Liposarcoma; Fibrosarcoma; Malignant fibrous histiocytoma; Dermatofibrosarcoma; Angiosarcoma

  13. Successful enucleation of retroperitoneal cyst.

    PubMed

    Singal, Rikki; Gupta, Samita; Singh, Bir

    2012-01-01

    Retroperitoneal mesenteric cyst is a rare entity among the other mesenteric cysts and intra-abdominal tumours. A 42-year-old woman reported with pain abdomen off and on since one month. There were no other complaints. On ultrasonography a mesenteric cyst was diagnosed. Surgery was planned which revealed a retroperitoneal mesenteric cyst. Enucleation of the cyst was done. In follow-up of 6 months patient is asymptomatic. We are reporting a rarely reported retroperitoneal mesenteric cyst in the mesentery of the descending colon or sigmoid.

  14. Treatment of AIDS-associated Kaposi's sarcoma in Zimbabwe: results of a randomized quality of life focused clinical trial.

    PubMed

    Olweny, Charles L M; Borok, Margaret; Gudza, Ivy; Clinch, Jennifer; Cheang, Mary; Kiire, Clem F; Levy, Lorraine; Otim-Oyet, David; Nyamasve, Joseph; Schipper, Harvey

    2005-02-10

    Kaposi's sarcoma is currently the most common tumor in Zimbabwe. The purpose of our study is to compare the effectiveness of supportive care vs. 3 intervention approaches, namely oral Etoposide, a 3-drug combination, and radiotherapy using quality of life (QOL) as the primary measure of success. In addition, our study was to determine whether a disease-specific module has greater sensitivity to group differences than a generic QOL questionnaire and to determine the most pragmatic approach to treating epidemic Kaposi's sarcoma (EKS) in Zimbabwe. Histologically confirmed HIV-positive patients with Kaposi's sarcoma were randomized to receive supportive care only or supportive care plus either radiotherapy, oral Etoposide or a 3-drug combination consisting of actinomycin-D, vincristine and bleomycin. No patient received antiretroviral therapy. The primary outcome was QOL measured by the functional living index-cancer (FLI-C) and supplemented by the Kaposi's sarcoma module (KSM). From 1994-1999, 495 EKS patients were accrued, and 470 were evaluable. Of these, 433 are known to be dead, 26 are lost to follow-up and 11 are still alive. The group treated with oral Etoposide had a significantly better QOL than the radiotherapy group for the total FLI-C score (adjusted mean plus standard error at 3-months 89 +/- 3 vs. 76 +/- 3; p = 0.004) and for the hardship (11 +/- 0.4 vs. 9 +/- 0.4; p = 0.001); social (10 +/- 0.4 vs. 8 +/- 0.4; p = 0.001) and nausea (9 +/- 0.4 vs. 8 +/- 0.4; p = 0.002) subscales. In addition, on the physical and psychological subscales, the Etoposide group had a significantly better QOL than the other 3 treatment groups (p < 0.04). The 3-drug combination, supportive care and radiotherapy groups did not differ significantly from each other with respect to the total FLI-C score or its subscales. There were no group differences with respect to survival. Oral Etoposide therapy resulted in better total FLI-C QOL score than radiotherapy. As well, Etoposide

  15. Can Kaposi Sarcoma Be Prevented?

    MedlinePlus

    ... Kaposi Sarcoma Causes, Risk Factors, and Prevention Can Kaposi Sarcoma Be Prevented? Kaposi sarcoma (KS) is caused ... Sarcoma? Can Kaposi Sarcoma Be Prevented? More In Kaposi Sarcoma About Kaposi Sarcoma Causes, Risk Factors, and ...

  16. Extremity preservation by combined modality therapy in sarcomas of the hand and foot: an analysis of local control, disease free survival and functional result

    SciTech Connect

    Kinsella, T.J.; Loeffler, J.S.; Fraass, B.A.; Tepper, J.

    1983-08-01

    A primary tumor arising in the hand or foot represents an uncommon presentation for patients with Ewing's sarcoma (ES) or soft tissue sarcoma (STS). While there exists considerable literature on the treatment of extremity sarcomas, very little deals specifically with lesions of the hand or foot. It remains controversial whether these lesions can be successfully treated with combined modality therapy which preserves the extremity and maintains function. From 1972 to 1979, 10 patients with sarcomas arising in the hand or foot were treated with combined modality therapy at the National Cancer Institute. Seven patients with ES of bone received local irradiation to 5000 rad and combination chemotherapy following an incisional biopsy. Three patients with STS received a gross tumor excision and local irradiation to 6000 rad. Local control was achieved in nine patients (90%) with a follow-up of 30 to 119 months (median 56 months). These patients have complete or almost complete function of the treated extremity. Nine patients are alive with five patients remaining disease-free following the initial combined modality treatment. We conclude that for selected patients with sarcomas arising in the hand or foot, combined modality therapy which leaves the extremity intact results in excellent local tumor control and preserves function. Careful treatment planning is an essential aspect of successful radiation therapy of a hand or foot primary. Our treatment recommendations are outlined. This approach is a viable alternative to amputation in these patients.

  17. [Indications and results of preventive endoprosthezing of the abdominal wall during operations on organs of the abdominal cavity and retroperitoneal space].

    PubMed

    Sukovatykh, B S; Valuĭskaia, N M; Netiaga, A A; Zhukovskiĭ, V A; Pravednikova, N V; Kas'ianova, M A

    2011-01-01

    Complex examination and treatment of 120 patients with anatomo-functional insufficiency of the abdominal wall was made after operations on organs of the abdominal cavity and retroperitoneal space. In the 1st group (60 patients) the abdominal wall was sutured by traditional methods. In the 2nd group (60 patients) laparotomy was followed by implantation of polypropylene endoprosthesis by over-aponeurotic method, and suturing of the lateral wall--by sub-aponeurotic method. At the long-term postoperative period the postoperative ventral hernias were formed in 21.6% of patients of the 1st group, in the 2nd group of patients hernias were not detected. Physical component of quality of life of the 2nd group was 1.5 times, and mental component 1.7 times higher than in the 1st group.

  18. Vorinostat in refractory soft tissue sarcomas - Results of a multi-centre phase II trial of the German Soft Tissue Sarcoma and Bone Tumour Working Group (AIO).

    PubMed

    Schmitt, Thomas; Mayer-Steinacker, Regine; Mayer, Frank; Grünwald, Viktor; Schütte, Jochen; Hartmann, Jörg T; Kasper, Bernd; Hüsing, Johannes; Hajda, Jacek; Ottawa, Gregor; Mechtersheimer, Gunhild; Mikus, Gerd; Burhenne, Jürgen; Lehmann, Lorenz; Heilig, Christoph E; Ho, Anthony D; Egerer, Gerlinde

    2016-09-01

    New treatment options for patients with metastatic Soft Tissue Sarcoma are urgently needed. Preclinical studies suggested activity of vorinostat, a histone deacetylase inhibitor. A multi-centre, open-label, non-randomised phase II trial to investigate the efficacy and safety of vorinostat in patients with locally advanced or metastatic Soft Tissue Sarcoma failing 1st-line anthracycline-based chemotherapy was initiated. Patients were treated with vorinostat 400 mg po qd for 28 d followed by a treatment-free period of 7 d, representing a treatment cycle of 5 weeks. Restaging was performed every three cycles or at clinical progression. Between 06/10 and 09/13, 40 Soft Tissue Sarcoma patients were treated with vorinostat at seven participating centres. Patients had received 1 (n=8, 20%), 2 (n=10, 25%) or ≥3 (n=22, 55%) previous lines of chemotherapy. Best response after three cycles of treatment was stable disease (n=9, 23%). Median progression-free survival and overall survival were 3.2 and 12.3 months, respectively. Six patients showed long-lasting disease stabilisation for up to ten cycles. Statistical analyses failed to identify baseline predictive markers in this subgroup. Major toxicities (grade ≥III) included haematological toxicity (n=6, 15%) gastrointestinal disorders (n=5, 13%), fatigue (n=4, 10%), musculoskeletal pain (n=4, 10%), and pneumonia (n=2, 5%). In a heavily pre-treated patient population, objective response to vorinostat was low. However, a small subgroup of patients had long-lasting disease stabilisation. Further studies aiming to identify predictive markers for treatment response as well as exploration of combination regimens are warranted. NCT00918489 (ClinicalTrials.gov) EudraCT-number: 2008-008513-19. Copyright © 2016 Elsevier Ltd. All rights reserved.

  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. Angiography of nonneoplastic retroperitoneal masses

    SciTech Connect

    Lois, J.F.; Levin, D.C.; Hooshmand, I.

    1982-01-01

    Although noninvasive imaging modalities can be used to initially detect retroperitoneal masses, angiography is still desirable for several reasons in those cases in which surgery is contemplated. The latter can forewarn the surgeon as to possible hemorrhagic complications in highly vascular lesions. In some cases it can predict the malignant potential of the lesion. Finally, since these masses may derive blood supply from multiple sources, a vascular ''road map'' is provided to the surgeon. The angiographic findings of malignant retroperitoneal tumors are well known, but very little has been published dealing with benign nonneoplastic retroperitoneal masses. We have performed angiography in 11 surgically proven nonneoplastic retroperitoneal masses (9 inflammatory lesions, 2 hematomas). Major arterial or renal displacement occurred in 10. The 2 hematomas and 3 inflammatory lesions were totally avascular; 6 of the 9 inflammatory lesions revealed fine neovascularity and 5 of these 6 had an associated capillary blush. Sources of vascular supply included the lumbar, renal capsular, superior mesenteric, and gastroduodenal arteries. Although angiography is helpful in preoperative evaluation of retroperitoneal masses for the aforementioned reasons, it may be difficult or impossible to differentiate benign from malignant lesions based on the angiographic findings alone.

  1. Primary metastatic Ewing's family tumors: results of the Italian Sarcoma Group and Scandinavian Sarcoma Group ISG/SSG IV Study including myeloablative chemotherapy and total-lung irradiation.

    PubMed

    Luksch, R; Tienghi, A; Hall, K Sundby; Fagioli, F; Picci, P; Barbieri, E; Gandola, L; Eriksson, M; Ruggieri, P; Daolio, P; Lindholm, P; Prete, A; Bisogno, G; Tamburini, A; Grignani, G; Abate, M E; Podda, M; Smeland, S; Ferrari, S

    2012-11-01

    The Italian Sarcoma Group and the Scandinavian Sarcoma Group designed a joint study to improve the prognosis for patients with Ewing's family tumors and synchronous metastatic disease limited to the lungs, or the pleura, or a single bone. The study was opened in 1999 and closed to the enrollment in 2008. The program consisted of intensive five-drug combination chemotherapy, surgery and/or radiotherapy as local treatment, and consolidation treatment with high-dose busulfan/melphalan plus autologous stem cell rescue and total-lung irradiation. During the study period, 102 consecutive patients were enrolled. The median follow-up was 62 months (range 24-124). The 5-year event-free survival probability was 0.43 [standard deviation (SD) = 0.05] and the 5-year overall survival probability was 0.52 (SD = 0.052). Unfavorable prognostic factors emerging on multivariate analysis were a poor histological/radiological response at the site of the primary tumor [relative risk (RR) = 3.4], and incomplete radiological remission of lung metastases after primary chemotherapy (RR = 2.6). One toxic death and one secondary leukemia were recorded. This intensive approach is feasible and long-term survival is achievable in ∼50% of patients. New treatment approaches are warranted for patients responding poorly to primary chemotherapy.

  2. Results of treatment for soft tissue sarcoma in childhood and adolescence: a final report of the German Cooperative Soft Tissue Sarcoma Study CWS-86.

    PubMed

    Koscielniak, E; Harms, D; Henze, G; Jürgens, H; Gadner, H; Herbst, M; Klingebiel, T; Schmidt, B F; Morgan, M; Knietig, R; Treuner, J

    1999-12-01

    The goal of the second German Soft Tissue Sarcoma Study CWS-86 (1985 to 1990) was to improve the prognosis in children and adolescents with soft tissue sarcoma by means of a clinical trial comprising intensive chemotherapy and risk-adapted local therapy. There were 372 eligible patients. A staging system based on the postsurgical extent of disease was used. Chemotherapy consisted of vincristine, dactinomycin, doxorubicin, and ifosfamide. Radiotherapy was administered early at 10 to 13 weeks simultaneously with the second chemotherapy cycle (32 Gy or 54. 4 Gy). The single dose was reduced to 1.6 Gy and given twice daily (accelerated hyperfractionation). The event-free survival (EFS) and overall survival rates at 5 years were 59% +/- 3% and 69% +/- 3%, respectively. The 5-year EFS rate according to stage was as follows: stage I, 83% +/- 5%; stage II, 69% +/- 6%; stage III, 57% +/- 4%; and stage IV, 19% +/- 6%. The outcome for patients with stage III disease who required radiotherapy was much better in the CWS-86 study compared with the CWS-81 study (5-year EFS, 60% +/- 5% v 44% +/- 6%; P =.053). The most common treatment failure was isolated local relapse, with 14% of patients relapsing at the primary tumor site. The improved design of the study incorporating risk-adapted radiotherapy allowed treatment to be reduced for selected groups of patients without compromising survival.

  3. Ewing sarcoma of the rib: results of an intergroup study with analysis of outcome by timing of resection.

    PubMed

    Shamberger, R C; Laquaglia, M P; Krailo, M D; Miser, J S; Pritchard, D J; Gebhardt, M C; Healey, J H; Tarbell, N J; Fryer, C J; Meyers, P A; Grier, H E

    2000-06-01

    We sought to establish the outcome and optimal therapeutic sequence for patients with nonmetastatic Ewing sarcoma/primitive neuroectodermal tumor of the chest wall. Patients 30 years of age or younger with nonmetastatic Ewing sarcoma/primitive neuroectodermal tumor of the bone were randomly assigned to receive vincristine, doxorubicin, cyclophosphamide, and dactinomycin or those drugs alternating with ifosfamide and etoposide. Local control was obtained with an operation, radiotherapy, or both. Fifty-three (13.4%) of 393 patients had primary tumors of the chest wall (all rib). Event-free survival at 5 years was 57% for the chest wall compared with 61% for other sites (P >.2). Ifosfamide and etoposide improved outcome in the overall group (5-year event-free survival, 68% vs 54%; P =.002), and a similar trend occurred in chest wall lesions (5-year event-free survival, 64% vs 51%). Patients with chest wall lesions had more attempts at initial surgical resection (30%) than those with other primary tumor sites (8%, P <.01). The attempt at initial resection for chest wall lesions did not correlate with size. Initial resections at other sites were restricted to smaller tumors. Initial resection resulted in negative pathologic margins in 6 of 16 patients, whereas the delayed resection resulted in negative margins in 17 of 24 patients (P =.05). Although there was no difference in survival by timing of the operation in rib lesions, a higher percentage of patients with initial surgical resection received radiation than those with resection after initial chemotherapy (P =. 13). Although rib primary tumors are significantly larger than tumors found in other sites, their outcome is similar. We favor delayed resection whenever possible to minimize the number of patients requiring radiation therapy.

  4. [Laparoscopic treatment of retroperitoneal fibrosis].

    PubMed

    Joual, Abdenbi; Rabii, Redouane; El Mejjad, Amine; Fekak, Hamid; Debbagh, Adil; El Mrini, Mohamed

    2004-04-01

    The authors report a case of idiopathic retroperitoneal fibrosis (RPF) in a 38-year-old man presenting with obstructive acute renal failure. The initial management consisted of urinary diversion by bilateral double-J ureteric stenting. After restoration of normal renal function, CT urography demonstrated retroperitoneal fibrosis surrounding the two ureters. Surgical treatment was performed by laparoscopy using four trocars. The operation consisted of detachment of the ascending and descending colon followed by release of the ureters from the lumbar segment to the pelvic segment and finally intraperitonealization of the ureters. The operating time was six hours, the postoperative course was uneventful and the double-J stents were removed at the third week. Laparoscopic treatment of RPF is a treatment option providing all of the benefits of minimally invasive surgery. In the light of this case and a review of the literature, the authors describe the laparoscopic treatment of idiopathic retroperitoneal fibrosis.

  5. 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.

  6. Displaced plaque in retroperitoneal adenopathy.

    PubMed

    Al-Okaili, Riyadh N; Schable, Stephen I; Marlow, Troy J

    2002-08-01

    This study was designed to determine when to consider incidental retroperitoneal masses on the basis of a displaced calcified atheromatous abdominal aorta on lateral radiographs. We did a retrospective review of 143 normal abdominal helical computed tomography scans of individuals aged 50 years and older to measure the distance between the posterior aortic wall and anterior cortex of vertebral bodies from T12 through L3. The normal abdominal aorta maintains a close relationship to the vertebral column. The distance should not be more than 10 mm in men and 7.3 mm in women. Displacement of aortic calcified atheroma greater than these distances should prompt a search for a retroperitoneal mass.

  7. Epidemic Kaposi Sarcoma

    MedlinePlus

    ... Kaposi sarcoma is found in patients who have acquired immunodeficiency syndrome (AIDS). Epidemic Kaposi sarcoma occurs in patients who have ... combines treatment for Kaposi sarcoma with treatment for AIDS. For the treatment of epidemic Kaposi sarcoma, combined ...

  8. What Is Kaposi Sarcoma?

    MedlinePlus

    ... Treatment? Kaposi Sarcoma About Kaposi Sarcoma What Is Kaposi Sarcoma? Cancer starts when cells in the body ... the lungs may cause trouble breathing. Types of Kaposi sarcoma The different types of KS are defined ...

  9. 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 ...

  10. Combined mediastinal and retroperitoneal fibrosis

    PubMed Central

    Salmon, H. W.

    1968-01-01

    A case of combined idiopathic mediastinal fibrosis and retroperitoneal fibrosis is described. It is possibly the twelfth case to be reported during life. A review of the literature reveals the `ubiquity' of localized collagenosis and the trend of opinion as regards aetiology and treatment. Images PMID:5654073

  11. Light ion irradiation for unfavorable soft tissue sarcoma

    SciTech Connect

    Linstadt, D.; Castro, J.R.; Phillips, T.L.; Petti, P.L.; Collier, J.M.; Daftari, I.; Schoethaler, R.; Rayner, A.

    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 macroscopic 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.

  12. Retroperitoneal fibrosis and obstructive uropathy due to actinomycosis: case report of a treatment approach.

    PubMed

    Yagmurdur, Mahmut Can; Akbulut, Sami; Colak, Aysel; Aygun, Cem; Haberal, Mehmet

    2009-01-01

    An actinomycotic retroperitoneal infection usually occurs in the presence of an intrauterine device (IUD). It can result in pelvic inflammatory disease and diffuse retroperitoneal fibrosis. A 39-year-old patient was admitted to the emergency unit with left flank pain. A computed tomography scan of the abdomen showed bilateral hydroureteronephrosis and a retroperitoneal malignant mass. Other tumors were excluded with a colonoscopy and an upper gastrointestinal endoscopy. Results of a fine needle aspiration biopsy showed fibrosis compatible with retroperitoneal mesenteritis. Double-J stents were placed in both ureters, and immunosuppressive therapy was started. The patient had clinical and radiologic responses to the therapy. A bilateral ureterolysis and sigmoid colon resection were done. The pathology report showed fibrosis and Actinomyces israelii infection. Parenteral and oral penicillins were administered. The probability of an Actinomyces infection in patients with retroperitoneal fibrosis should be kept in mind, especially in cases in which the patient has an intrauterine device.

  13. Surgery for Soft Tissue Sarcomas

    MedlinePlus

    ... Tissue Sarcomas Chemotherapy for Soft Tissue Sarcomas Targeted Therapy for Soft Tissue Sarcoma Treatment of Soft Tissue Sarcomas, by Stage ... Cancer Information Cancer Prevention & Detection Cancer Basics ...

  14. Non-Rhabdomyosarcoma Soft Tissue Sarcomas in Children: A Surveillance, Epidemiology, and End Results Analysis Validating COG Risk Stratifications

    SciTech Connect

    Waxweiler, Timothy V.; Rusthoven, Chad G.; Proper, Michelle S.; Cost, Carrye R.; Cost, Nicholas G.; Donaldson, Nathan; Garrington, Timothy; Greffe, Brian S.; Heare, Travis; Macy, Margaret E.; Liu, Arthur K.

    2015-06-01

    Purpose: Non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) are a heterogeneous group of sarcomas that encompass over 35 histologies. With an incidence of ∼500 cases per year in the United States in those <20 years of age, NRSTS are rare and therefore difficult to study in pediatric populations. We used the large Surveillance, Epidemiology, and End Results (SEER) database to validate the prognostic ability of the Children's Oncology Group (COG) risk classification system and to define patient, tumor, and treatment characteristics. Methods and Materials: From SEER data from 1988 to 2007, we identified patients ≤18 years of age with NRSTS. Data for age, sex, year of diagnosis, race, registry, histology, grade, primary size, primary site, stage, radiation therapy, and survival outcomes were analyzed. Patients with nonmetastatic grossly resected low-grade tumors of any size or high-grade tumors ≤5 cm were considered low risk. Cases of nonmetastatic tumors that were high grade, >5 cm, or unresectable were considered intermediate risk. Patients with nodal or distant metastases were considered high risk. Results: A total of 941 patients met the review criteria. On univariate analysis, black race, malignant peripheral nerve sheath (MPNST) histology, tumors >5 cm, nonextremity primary, lymph node involvement, radiation therapy, and higher risk group were associated with significantly worse overall survival (OS) and cancer-specific survival (CSS). On multivariate analysis, MPNST histology, chemotherapy-resistant histology, and higher risk group were significantly poor prognostic factors for OS and CSS. Compared to low-risk patients, intermediate patients showed poorer OS (hazard ratio [HR]: 6.08, 95% confidence interval [CI]: 3.53-10.47, P<.001) and CSS (HR: 6.27; 95% CI: 3.44-11.43, P<.001), and high-risk patients had the worst OS (HR: 13.35, 95% CI: 8.18-21.76, P<.001) and CSS (HR: 14.65, 95% CI: 8.49-25.28, P<.001). Conclusions: The current COG risk group

  15. Five-Year Results From a Scandinavian Sarcoma Group Study (SSG XIII) of Adjuvant Chemotherapy Combined With Accelerated Radiotherapy in High-Risk Soft Tissue Sarcoma of Extremities and Trunk Wall

    SciTech Connect

    Jebsen, Nina L.; Bruland, Oyvind S.; Eriksson, Mikael; Engellau, Jacob; Turesson, Ingela; Folin, Annika; Trovik, Clement S.; Hall, Kirsten Sundby

    2011-12-01

    Purpose: To evaluate adjuvant chemotherapy and interpolated accelerated radiotherapy (RT) for adult patients with high-risk soft tissue sarcoma in the extremities or trunk wall. Methods and Materials: High-risk soft tissue sarcoma was defined as high-grade malignancy and at least two of the following criteria: size {>=}8 cm, vascular invasion, or necrosis. Six cycles of doxorubicin and ifosfamide were prescribed for all patients. RT to a total dose of 36 Gy (1.8 Gy twice daily) was inserted between two chemotherapy cycles after marginal margin resection regardless of tumor depth or after wide-margin resection for deep-seated tumors. RT was boosted to 45 Gy in a split-course design in the case of intralesional margin resection. Results: A total of 119 patients were eligible, with a median follow-up of 5 years. The 5-year estimate of the local recurrence, metastasis-free survival, and overall survival rate was 12%, 59%, and 68%, respectively. The group receiving RT to 36 Gy had a local recurrence rate of 10%. In contrast, the local recurrence rate was 29% in the group treated with RT to 45 Gy. The presence of vascular invasion and low chemotherapy dose intensity had a negative effect on metastasis-free and overall survival. Toxicity was moderate after both the chemotherapy and the RT. Conclusions: Accelerated RT interposed between chemotherapy cycles in a selected population of patients with high-risk soft tissue sarcoma resulted in good local and distant disease control, with acceptable treatment-related morbidity. The greater radiation dose administered after intralesional surgery was not sufficient to compensate for the poorer surgical margin. Vascular invasion was the most important prognostic factor for metastasis-free and overall survival.

  16. Retroperitoneal approach for recurrent benign multicystic peritoneal mesothelioma.

    PubMed

    Bakhshi, Girish D; Wankhede, Kishor R; Tayade, Mukund B; Bhandarwar, Ajay H; Gore, Sandeep T; Choure, Dayanand D

    2013-01-25

    Benign multicystic peritoneal mesothelioma (BMPM) is an uncommon lesion. It presents as a lump in abdomen or a finding seen on imaging modalities. Surgery is the primary modality of treatment. However, it has a high recurrence rate; this results in adhesions and subsequent surgeries difficult. We present a case of recurrent BMPM in a female operated twice earlier in a rural centre. Imaging modalities showed majority of the lesion in paracolic and retroperitoneal region. Hence, retroperitoneal approach for surgery was taken. This avoided previous surgical adhesions. A brief case report on this novel approach and review of literature is presented.

  17. Retroperitoneal Approach for Recurrent Benign Multicystic Peritoneal Mesothelioma

    PubMed Central

    Bakhshi, Girish D.; Wankhede, Kishor R; Tayade, Mukund B.; Bhandarwar, Ajay H.; Gore, Sandeep T.; Choure, Dayanand D.

    2013-01-01

    Benign multicystic peritoneal mesothelioma (BMPM) is an uncommon lesion. It presents as a lump in abdomen or a finding seen on imaging modalities. Surgery is the primary modality of treatment. However, it has a high recurrence rate; this results in adhesions and subsequent surgeries difficult. We present a case of recurrent BMPM in a female operated twice earlier in a rural centre. Imaging modalities showed majority of the lesion in paracolic and retroperitoneal region. Hence, retroperitoneal approach for surgery was taken. This avoided previous surgical adhesions. A brief case report on this novel approach and review of literature is presented. PMID:24765496

  18. TRALI Syndrome Complicated by Retroperitoneal Bleeding.

    PubMed

    Singh, Vijay A; Zeltsman, David

    2011-09-01

    Transfusion-related acute lung injury (TRALI) is an underdiagnosed and underreported syndrome which by itself is the third leading cause of transfusion-related mortality. The incidence of TRALI is reported to be 1 in 2000 to 5000 transfusions. When combined with uncontrollable bleeding, survival is unachievable. We report the case of a 25-year-old man, who underwent open heart surgery as an infant to correct his congenital heart disease in association with right pulmonary artery atresia. He presented with hemoptysis secondary to aspergilloma and required a pneumonectomy of the nonfunctional right lung. During pneumolysis, significant bleeding occurred from the superior vena cava. The patient required a blood transfusion and was placed on cardiopulmonary bypass to control the bleeding. Simultaneous occurrence of severe pulmonary edema and retroperitoneal bleeding were noted. Approximately 8 L of frothy edema fluid were drained from the only functional left lung starting ~15 minutes after the transfusion and lasting for several hours until the end of the case. It most likely represented TRALI syndrome. Increasing abdominal girth and poor volume return to the pump were consistent with and pathognomonic for retroperitoneal bleeding. Though primary surgical bleeding in the chest was controlled successfully and a pneumonectomy performed without further difficulty, we were unable to separate the patient from cardiopulmonary bypass due to the inability to oxygenate. As a result, we could not reverse the anti-coagulation which potentially exacerbated the retroperitoneal bleeding. After multiple unsuccessful attempts the patient succumbed. This ill-fated case demonstrates the quandary of obtaining vascular access for emergency cardiopulmonary bypass while in the right thoracotomy position. It may be beneficial to have both the femoral artery and vein cannulated before positioning a patient in a lateral decubitus position. In addition, early direct access to the right atrium

  19. False-Negative Results of Endoscopic Biopsy in the Diagnosis of Gastrointestinal Kaposi's Sarcoma in HIV-Infected Patients.

    PubMed

    Nagata, Naoyoshi; Sekine, Katsunori; Igari, Toru; Hamada, Yohei; Yazaki, Hirohisa; Ohmagari, Norio; Akiyama, Junichi; Shimbo, Takuro; Teruya, Katsuji; Oka, Shinichi; Uemura, Naomi

    2012-01-01

    Kaposi's sarcoma (KS) is a rare endothelial neoplasm mainly involving the skin, but it is often associated with AIDS. Diagnosis of gastrointestinal (GI) tract KS, a common site of visceral involvement in AIDS, is important, but endoscopic biopsy carries a risk of false-negative results (FNRs) due to its submucosal appearance. This study sought to determine the rate and causes of FNR for endoscopic biopsy of GI-KS lesions. Endoscopic biopsy samples of 116 GI-KS lesions were reviewed retrospectively. All GI-KS lesions were confirmed to be resolved following KS therapy. FNRs were yielded for 41 of the lesions (35.3%). Among upper and lower GI sites, the esophagus was the only site significantly associated with FNRs (P < 0.01). Small size (<10 mm) and patches found on endoscopy were significantly associated with FNRs (P < 0.05). Findings of submucosal tumor (SMT) with ulceration were significantly associated with true-positive results (P < 0.05). In conclusion, FNRs were found in 35.3% of GI-KS lesions and were especially related to the site of the esophagus and endoscopic early stage (small size or patch appearance). An SMT with ulceration may be relatively easy to diagnose on endoscopic biopsy. Caution should be exercised when performing endoscopic biopsy of these lesions in AIDS patients and evaluating the histological features.

  20. Long-term recurrence of soft tissue sarcomas: prognostic factors and implications for prolonged follow-up.

    PubMed

    Toulmonde, Maud; Le Cesne, Axel; Mendiboure, Jean; Blay, Jean-Yves; Piperno-Neumann, Sophie; Chevreau, Christine; Delcambre, Corinne; Penel, Nicolas; Terrier, Philippe; Ranchère-Vince, Dominique; Lae, Marick; Le Guellec, Sophie; Michels, Jean-Jacques; Robin, Yves-Marie; Bellera, Carine; Italiano, Antoine

    2014-10-01

    To the authors' knowledge, the incidence of late recurrence (> 5 years after initial management) is unknown and no prognostic factors for late events have been characterized in patients with soft tissue sarcomas. Follow-up data from patients with localized soft tissue sarcoma who were included in the French Sarcoma Group database from January 1990 to June 2005 were reviewed. The outcomes of interest were the cumulative probabilities of late (> 5 years) local and metastatic disease recurrence with death as a competing event. Estimations and 95% confidence intervals (95% CIs) were computed with the cumulative incidence function. A total of 719 patients who were alive and event free > 5 years after their initial diagnosis were included in the current study. Sixty-seven patients (9.3%) developed a late local recurrence and 42 patients (5.8%) developed a late metastatic recurrence, respectively. On multivariate analysis, internal trunk location (hazard ratio [HR], 3.9; 95% CI, 2.2-6.7 [P < .001]) and tumor size > 100 mm (HR, 2.1; 95% CI, 1.1-4 [P = .035]) were the 2 factors found to be independently associated with an increased risk of late local recurrence. Grade > 1 (graded according to the French Federation of Cancer Centers Sarcoma Group) (HR, 4.7; 95% CI 1.1-21 [P = .04]) was the sole factor found to be independently associated with an increased risk of late metastatic recurrence. Late recurrence of soft tissue sarcoma is relatively uncommon. However, the results of the current study emphasize the critical role of long-term follow-up to detect late local disease recurrence in patients with retroperitoneal or very large soft tissue sarcomas, and late metastatic recurrence in patients with high-grade disease. Conversely, the prolonged follow-up of patients with grade 1 disease is not needed. © 2014 American Cancer Society.

  1. Successful management of unsuspected retroperitoneal paraganglioma via the use of combined epidural and general anesthesia: a case report

    PubMed Central

    2013-01-01

    Introduction Similar to pheochromocytomas, paragangliomas can secrete catecholamines, although they are usually non-functional and clinical presentation is non-specific. We present a case of accidental, intra-operatively diagnosed neuroendocrine-active sympathetic paraganglioma, which was suspected and confirmed during elective retroperitoneal tumor removal. Case presentation A 25-year-old Caucasian Croatian man, American Society of Anesthesiologists status 1, underwent elective surgery for retroperitoneal tumor removal. The tumor had been discovered by chance during a routine examination and was suspected to be a sarcoma. Our patient had no history of previous medical conditions nor did he have symptoms characteristic of a neuroendocrine secreting tumor. The results of ultrasound and magnetic resonance imaging studies showed a large, well demarcated retroperitoneal tumor mass in his upper abdomen localized between the aorta and vena cava, measuring approximately 9×6×4.5cm. In the operating room an epidural catheter was inserted at the T7 to T8 level prior to induction of general anesthesia. Epidural analgesia was maintained by an infusion pump with local anesthetic and opiate mixture. During the surgical excision of the tumor, hemodynamic changes occurred, with hypertension (205/110mmHg) and tachycardia (up to 120 beats/minute). In spite of the fact that the surgical field of work did not include adrenal glands whose direct manipulation could explain this occurrence, there was a high degree of suspicion for the presence of a neurosecreting tumor. His clinical symptoms were relieved after administration of urapidil, esmolol and magnesium sulfate. After tumor excision, our patient developed severe hypotension. Hemodynamic stability was reinstated with aggressive volume replacement, with crystalloids and colloids, vasopressors and hydrocortisone. His post-operative course was unremarkable and on the eighth post-operative day our patient was discharged from hospital

  2. Follicular dendritic cell sarcoma of the abdomen: the imaging findings.

    PubMed

    Kang, Tae Wook; Lee, Soon Jin; Song, Hye Jong

    2010-01-01

    Follicular dendritic cell sarcoma is a rare neoplasm that originates from follicular dendritic cells in lymphoid follicles. This disease usually involves the lymph nodes, and especially the head and neck area. Rarely, extranodal sites may be affected, including tonsil, the oral cavity, liver, spleen and the gastrointestinal tract. We report here on the imaging findings of follicular dendritic cell sarcoma of the abdomen that involved the retroperitoneal lymph nodes and colon. It shows as a well-defined, enhancing homogenous mass with internal necrosis and regional lymphadenopathy.

  3. Neoadjuvant and adjuvant chemotherapy combined with anatomical resection of feline injection-site sarcoma: results in 21 cats.

    PubMed

    Bray, J; Polton, G

    2016-06-01

    This study assesses the outcome of two combined treatment strategies for the treatment of feline injection-site sarcoma (FISS). Twenty-one cats with primary or recurrent FISS received 3 cycles of neoadjuvant chemotherapy with epirubicin (25 mg m(-2) ), then an anatomical resection of the entire muscle compartment containing the tumour was performed based on the findings of co-axial imaging. Cats then received a further 3 cycles of adjuvant chemotherapy. Follow-up was performed by telephone contact with a median follow-up time of 1072 days. Three cats (14%) developed local tumour recurrence at days 264, 664 and 1573 after surgery. A median survival time could not be calculated as over 80% of the study population remained alive or were censored due to death from other causes. When compared to historical controls, the results of this study demonstrate superior rates of tumour-free survival and disease-free interval.

  4. Retroperitoneal Angiomyolipoma: A Case Report and Review of the Literature

    PubMed Central

    King, Jonathan; McClintock, Scott

    2013-01-01

    Retroperitoneal angiomyolipoma is a rare tumour that is difficult to diagnose preoperatively. We present a case of retroperitoneal angiomyolipoma that highlights its diagnostic dilemma. We also performed a literature review and present a review of retroperitoneal angiomyolipoma. PMID:24303222

  5. Myeloid sarcoma derived from the gastrointestinal tract: A case report and review of the literature

    PubMed Central

    YU, TENG; XU, GENBO; XU, XIAOHUA; YANG, JING; DING, LUYIN

    2016-01-01

    Myeloid sarcoma is a type of malignant neoplasm composed of myeloblasts that locates extramedullary. The present study reports the case of a 31-year-old female who presented with upper abdominal pain, melena, vomiting and jaundice. The abdominal computed tomography revealed a mass in gastric antrum area and possible infiltration of the duodenum, gallbladder and head of the pancreas, with possible retroperitoneal lymph node metastasis. The tumor grew quickly and led to serious obstructive jaundice. New masses developed in the bilateral orbits and left breast within 2 months of admission. The pathological results of the gastroscopic biopsy and the fine-needle biopsy of the breast revealed myeloid sarcoma. Transhepatic cholangial drainage, radiotherapy and chemotherapy were administered, but the disease reoccurred and became resistant to chemotherapy, so salvage allogenetic peripheral blood stem cell transplantation was performed. The disease relapsed at 5 months post-transplantation, and chemotherapy and donor lymphocytes transfusions were then administered. The patient declined further treatment and succumbed to disease on May 19, 2015. The present study could improve the understanding of myeloid sarcoma and provide a reference for standardized and individualized treatments for this disease. PMID:27313759

  6. Primary retroperitoneal acinar cell cystadenoma.

    PubMed

    Pesci, Anna; Castelli, Paola; Facci, Enrico; Romano, Luigi; Zamboni, Giuseppe

    2012-03-01

    In this report, we describe a case of hitherto unreported primary retroperitoneal acinar cell cystadenoma that morphologically and immunophenotypically resembled pancreatic acinar cell cystadenoma. Pancreatic acinar cell cystadenoma is a very uncommon benign lesion characterized by acinar cell differentiation, the evidence of pancreatic exocrine enzyme production, and the absence of cellular atypia. Our case occurred in a 55-year-old woman presenting a 10-cm multilocular cystic lesion in the retroperitoneum thought to be a mucinous cystic neoplasm. At laparotomy, the cystic mass, which showed no connection with any organ, was completely resected with a clinical diagnosis of cystic lymphangioma. The diagnosis of retroperitoneal acinar cell cystadenoma was based on the recognition of morphological acinar differentiation, the immunohistochemical demonstration of the acinar marker trypsin, and the absence of cellular atypia. These peculiar features can be used in the differential diagnosis with all the other cystic lesions of the retroperitoneum.

  7. CT-guided fine-needle aspiration of abdominal and retroperitoneal small lesions with the coaxial technique using MPR images.

    PubMed

    De Filippo, Massimo; Saba, Luca; Azzali, Emanuele; Milanese, Gianluca; Mostardi, Maurizio; Borgia, Daniele; Capasso, Raffaella; Nizzoli, Rita

    2016-07-28

    To demonstrate the advantages of CT-guided fine-needle aspiration (FNA) of abdominal and retroperitoneal small lesions with the coaxial technique using MPR images. The study included retrospectively 50 patients who underwent CT-guided FNA of abdominal and/or retroperitoneal small lesion (<30 mm). Patients with suspected lymphomas or sarcomas were excluded. Cytology reports were the reference standard. The cytology was diagnostic in 48/50 biopsies (96%): out of 41 neoplastic lesions (85%), 37 were malignant (90.2%) and 4 were benign (9.8%); 7 out of 48 were non-neoplastic (14.6%). No procedural complications were observed (0%). By using MPR images there is an effective improvement in coaxial CT-guided FNA of abdominal and retroperitoneal small lesions.

  8. Kaposi sarcoma.

    PubMed

    Radu, Oana; Pantanowitz, Liron

    2013-02-01

    Kaposi sarcoma (KS) is a low-grade vascular tumor associated with Kaposi sarcoma herpesvirus/human herpesvirus 8 (KSHV/HHV8) infection. Kaposi sarcoma lesions predominantly present at mucocutaneous sites, but may involve all organs and anatomic locations. Recognized epidemiologic-clinical forms of KS include classic, African (endemic), AIDS-associated (epidemic), and iatrogenic KS. New clinical manifestations have been described, such as antiretroviral therapy-related KS regression or flares. Kaposi sarcoma lesions evolve from early (patch stage) macules into plaques (plaque stage) that grow into larger nodules (tumor stage). Newer histologic variants include anaplastic, hyperkeratotic, lymphangioma-like, bullous, telangiectatic, ecchymotic, keloidal, pyogenic granuloma-like, micronodular, intravascular, glomeruloid and pigmented KS, as well as KS with sarcoidlike granulomas and KS with myoid nodules. Latency-associated nuclear antigen (HHV8) is the most specific immunohistochemical marker available to help distinguish KS from its mimics. Since KS remains one of the most common AIDS-defining malignancies, it is important that pathologists be able to recognize KS and its contemporary manifestations.

  9. Retroperitoneal Cystic Lymphangioma: A Diagnostic and Surgical Challenge

    PubMed Central

    Gümüştaş, Oguzhan Güven; Sanal, Murat; Güner, Osman; Tümay, Volkan

    2013-01-01

    A lymphangioma is a benign proliferation of lymph vessels, producing fluid-filled cysts that result from a blockage of the lymphatic system. The incidence of abdominal lymphangiomas is unknown; however they account for from 3% to 9.2% of all pediatric lymphangiomas, with retroperitoneal lymphangioma representing less than 1% of abdominal lymphangiomas. Due to rarity, preoperative diagnosis is often difficult. PMID:23533897

  10. Femoral nerve dysfunction after retroperitoneal hemorrhage: pathophysiology revealed by computed tomography.

    PubMed

    Reinstein, L; Alevizatos, A C; Twardzik, F G; DeMarco, S J

    1984-01-01

    In three patients receiving anticoagulation therapy who developed retroperitoneal hemorrhage computed tomography (CT) clearly localized the resulting hematoma in each case. Three distinct syndromes are described. A hemorrhage within the iliacus muscle resulted in femoral nerve dysfunction. A large hemorrhage within the iliacus muscle which extended into the psoas muscle produced both femoral and obturator nerve dysfunction. A retroperitoneal hemorrhage extrinsic to both the iliacus and psoas muscles did not produce peripheral nerve dysfunction. The pathophysiology of peripheral nerve dysfunction in retroperitoneal hemorrhage is reviewed in detail.

  11. Retroperitoneal fibromatosis and acquired immunodeficiency syndrome in macaques. Pathologic observations and transmission studies.

    PubMed Central

    Giddens, W. E.; Tsai, C. C.; Morton, W. R.; Ochs, H. D.; Knitter, G. H.; Blakley, G. A.

    1985-01-01

    A peculiar fibroproliferative syndrome called retroperitoneal fibromatosis (RF) has been observed in Macaca nemestrina, Macaca mulatta, Macaca fascicularis, and Macaca fuscata at the Washington Regional Primate Research Center. RF is characterized by the aggressive proliferation of highly vascular fibrous tissue subjacent to the peritoneum covering the ileocecal junction and associated mesenteric lymph nodes. In the early, proliferative phase of the disease, most of the fibroblastlike cells contain Factor VIII-related antigen. Two syndromes have been recognized: localized, in which fibroproliferative lesions occur only in solitary nodules; and progressive, in which fibromatosis occurs throughout the abdominal cavity. RF-affected monkeys often develop a simian acquired immunodeficiency syndrome (SAIDS) with severe thymic and lymphoid atrophy, chronic enterocolitis, and wasting. Experimental intraperitoneal inoculation with suspensions of RF tissue in two separate experiments resulted in the development of SAIDS in 5 of 16 and RF-SAIDS in 3 of 16 macaques. RF associated with SAIDS appears to be an excellent model for the Kaposi's sarcoma associated with AIDS. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Figure 7 Figure 8 Figure 9 Figure 10 Figure 11 Figure 12 Figure 13 PMID:3993741

  12. Fetal Cyst Reveling Retroperitoneal Enteric Duplication

    PubMed Central

    Ayadi, Imene Dahmane; Bezzine, Ahlem; Hamida, Emira Ben; Marrakchi, Zahra

    2017-01-01

    Retroperitoneum is a very uncommon site of enteric duplication (ED). We report a new case of retroperitoneal ED cyst suspected in utero. Prenatal ultrasound showed an abdominal cystic mass. Noncommunicating retroperitoneal ED cyst measuring 70 mm × 30 mm was resected. Histopathologic examination confirmed the diagnosis. PMID:28082784

  13. 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.

  14. Retroperitoneal fibrosis associated with Riedel's struma

    PubMed Central

    Rao, Chandar R.; Ferguson, George C.; Kyle, Victor N.

    1973-01-01

    A case of retroperitoneal fibrosis with bilateral ureteral obstruction in association with Riedel's struma of the thyroid is reported. There has been a definite increase in incidence of retroperitoneal fibrosis, but with prompt recognition and adequate treatment the mortality rate has been decreased from the original 14%. The association of Riedel's struma with retroperitoneal fibrosis has been noted in the past and its association with sclerosing cholangitis has also been mentioned. It is not known whether Riedel's thyroiditis originates in the thyroid gland and spreads or whether true thyroiditis is part of a generalized process. The temporal relationship of thyroiditis and retroperitoneal fibrosis suggests an extension of fibrosis from the thyroid, but one cannot be sure which condition occurred first. Whatever the cause, the treatment remains the same as for retroperitoneal fibrosis from other causes. ImagesFIG. 1FIG. 2 PMID:4699273

  15. Retroperitoneal Laparoscopy in Dogs: Access Technique, Working Space, and Surgical Anatomy

    PubMed Central

    Jeong, Junemoe; Ko, Jonghyeok; Lim, Hyunjoo; Kweon, Oh‐Kyeong

    2016-01-01

    Objective To develop and describe a laparoscopic retroperitoneal access technique, investigate working space establishment, and describe the surgical anatomy in the retroperitoneal space as an initial step for clinical application of retroperitoneal laparoscopy in dogs. Study Design Cadaveric and experimental study. Animals Cadaveric (n=8) and healthy (n=6) adult dogs. Methods The retroperitoneal access technique was developed in 3 cadavers based on the human technique and transperitoneal observation. Its application and working space establishment with carbon dioxide (CO2) insufflation alone was evaluated in 5 cadavers by observing with a transperitoneal telescope and in 6 live dogs by repeated computed tomography (CT) scans at pressure of 0, 5, 10, and 15 mmHg. Recordings of retroperitoneoscopy as well as working space volume and linear dimensions measured on CT images were analyzed. Results Retroperitoneal access and working space establishment with CO2 insufflation alone were successfully performed in all 6 live dogs. The only complication observed was in 1 dog that developed subclinical pneumomediastinum. As pressure increased, working space was established from the ipsilateral to the contralateral side, and peritoneal tearing eventually developed. Working space volume increased significantly from 5 mmHg and linear dimensions increased significantly from 0 to 10 mmHg. With pneumo‐retroperitoneum above 5 mmHg, retroperitoneal organs, including kidneys and adrenal glands, were easily visualized. Conclusion The retroperitoneal access technique and working space establishment with CO2 insufflation starting with 5 mmHg and increasing to 10 mmHg provided adequate working space and visualization of retroperitoneal organs, which may allow direct access for retroperitoneal laparoscopy in dogs. PMID:27731512

  16. The role of radiotherapy in the management of localized soft tissue sarcomas

    PubMed Central

    Tiong, Siaw Sze; Dickie, Colleen; Haas, Rick L.; O’Sullivan, Brian

    2016-01-01

    The combination of radiotherapy (RT) and function-preserving surgery is the most usual contemporary approach in the management of soft tissue sarcomas (STS). Pre- and postoperative RT result in similar local control rates, as shown by a landmark trial in extremity STS. In this review, the role of RT in the management of extremity STS will be discussed, but STS in other sites, including retroperitoneal STS, will also be addressed. The focus will consider various aspects of RT including strategies to reduce the volume of tissue being irradiated, dose, scheduling, and the possible of omission of RT in selected cases. Finally, technology advances through the use of intensity-modulated radiotherapy (IMRT), image-guided IMRT, intraoperative radiotherapy (IORT) and particle therapy will also be discussed. PMID:27807504

  17. Intra articular synovial sarcoma.

    PubMed

    Sistla, Radha; Tameem, Afroz; Vidyasagar, J V S

    2010-01-01

    Synovial sarcoma is a soft tissue neoplasm with a characteristic biphasic pattern. Incidence in soft tissues is 5-10%. Intra articularly synovial sarcoma is extremely rare. Fewer than 5% of all synovial sarcomas arise within the joint space. We report a case of intra articular synovial sarcoma in a young male who presented as internal derangement of the knee.

  18. [Renal angiomyolipoma complicated by retroperitoneal hematoma].

    PubMed

    Rabii, R; Fekak, H; Moufid, K; Joual, A; Benjelloun, S; Khaleq, K; Idali, B; Harti, A; Barrou, L

    2002-07-01

    Renal angiomyolipoma (AML) is a benign tumor, they are generally asymptomatic or can manifested by abdominal pain, palpable mass or hematuria. We report an uncommoun case of 65 years old women who consulted for retroperitoneal hemorrhage by spontaneous rupture of renal AML with palpable mass. The ultrasound and CT abdominopelvic scan were performed in the preoperative diagnosis and showed a typical right renal AML with retroperitoneal hematoma. The right nephrectomy by transperitoneal approach was performed with a good follow-up. The histological examination confirmed the diagnosis for renal AML. About this case, the authors discuses the diagnosis and the management for AML with retroperitoneal hemorrhage.

  19. Sarcomas related to the heart and vasculature.

    PubMed

    Raaf, H N; Raaf, J H

    1994-01-01

    Soft tissue sarcoma is the most common malignant neoplasm of the heart, pericardium, and great vessels. Its presentation is infrequent, nonspecific, and subtle. For example, emboli from these tumors to the lungs or peripheral arteries may mimic thrombotic embolic disease. New noninvasive techniques such as echocardiography and magnetic resonance imaging (MRI) aid in diagnosis and preoperative assessment. Angiosarcoma, the most common cardiac sarcoma, is aggressive and usually arises in the right atrium. Kaposi's sarcoma of the heart has been found in patients with AIDS and in immunosuppressed organ transplant recipients. Most primary sarcomas of the aorta and pulmonary artery (the elastic arteries) show minimal differentiation and are classified as "intimal, sarcomas," whereas leiomyosarcomas predominate in the muscular arteries and great veins. Surgical resection of any sarcoma of the vasculature, when feasible, is technically challenging but may result in cure or palliation. Adjuvant chemotherapy and radiation therapy can also relieve symptoms and prolong survival.

  20. General Information about Kaposi Sarcoma

    MedlinePlus

    ... Kaposi sarcoma is found in patients who have acquired immunodeficiency syndrome (AIDS). Epidemic Kaposi sarcoma occurs in patients who have ... combines treatment for Kaposi sarcoma with treatment for AIDS. For the treatment of epidemic Kaposi sarcoma, combined ...

  1. Organized hematoma mimicking retroperitoneal cystic tumors.

    PubMed

    Ivankiv, Taras; Ogurtsov, Oleksii; Pokhylevych, Galina

    2016-01-01

    Isolated retroperitoneal cysts are uncommon with an estimated incidence of 1/5750-1/250,000. In women they occur about 1.5-2 times more often than in men. The largest numbers of patients are young or middle aged (20-50 years). Lack of knowledge about the causes of these rare entities and asymptomatic clinical picture often leads to diagnostic and tactical mistakes. The medical history of 54-year old male patient B., who has been hospitalized at Surgical Department №1 of Danylo Halytsky Lviv National Medical University (Surgical Department of Lviv Regional Clinical Hospital), was processed retrospectively. Diagnosing of retroperitoneal organized hematoma in the early stages is not always possible, because exploration of retroperitoneal space can be difficult. General tests and tumor markers are usually normal range and not prognostically informative in this case. Decisively important were imaging diagnostic methods-USG and CT. As clinical cases of organized hematoma are quite rare, finding out retroperitoneal formation with irregular contours and infiltrative component indicates for retroperitoneal tumors. Thus, this formation accumulated contrast that says for increased vascularization. Intraoperative: formation with thick walls and heterogeneous structure. Histological diagnosis: hematoma in a phase of deep organization. On our opinion, taking into account location and structure of tumors, laparoscopic intervention was not appropriate, open surgery was reasonable approach. Preoperative biopsy has a crucial role to set preliminary diagnosis. Despite the fact that organized retroperitoneal hematomas are quite rare, their diagnosis requires detailed examination and histological verification. Copyright © 2016. Published by Elsevier Ltd.

  2. Giant primary retroperitoneal seminoma: A case report.

    PubMed

    Jovanović, Milan; Janjusević, Natasa; Mirković, Darko; Vulović, Maja; Milev, Bosko; Mitrović, Miroslav; Trifunović, Bratislav

    2016-02-01

    Primary extragonadal seminomas are rare tumors. There have been only a few cases of the primary retroperitoneal seminomas reported in the literature up to date. We reported a 56-year-old man with giant primary retroperitoneal seminoma presented with the enlargement of the left side of the abdomen and deep venous thrombosis of the left leg. Computed tomography of the abdomen showed a large tumor occupying the left part of the retroperitoneal space with 23 x 13 cm in diameter. Firm tumor mass having 25 x 15 cm in diameter was surgically removed from the left retroperitoneum. The tumor adhered the tunica adventitia of the aorta and it was carefully resected from the aortic wall. The diagnosis of seminoma was made during histopathological examination. The patient underwent chemotherapy. Two years after finished chemotherapy the patient accepted left orchiectomy with the aim of eliminating the possibility of the occult malignancy of the testicle. Histopathological analysis of the testicular tissue was normal and the diagnosis of primary retroperitoneal seminoma was confirmed. CONCLUSION. Despite its small incidence in general population, the diagnosis of retroperitoneal seminoma should be considered in male patients with nonspecific symptoms and with retroperitoneal tumor mass.

  3. Robotic retroperitoneal surgery: a contemporary review.

    PubMed

    Patel, Mayank; Porter, James

    2013-01-01

    Robotic-assisted renal surgery is being increasingly utilized for various kidney diseases; however, the majority of these are performed via a transperitoneal approach. Retroperitoneal robotic surgery is a relatively new technique, which allows direct access to the posterolateral surface of the kidney, as well as posterior hilar structures. In this review, we summarize the most recent publications and review our experience of retroperitoneal robotic surgery. Retroperitoneal robotic surgery has been successfully applied to radical nephrectomy, partial nephrectomy and pyeloplasty. The current series, although few, find this approach ideal for posterior and lateral renal masses, and technically feasible with the advances in robotic technology. The retroperitoneal approach has been shown to decrease operative times, narcotic need and permit quicker return of bowel function. Furthermore, there does not appear to be any increase in perioperative complications using this approach. The limited data using this technique offer an encouraging outlook on robotic retroperitoneal surgery. The retroperitoneal approach permits direct access to the renal hilum, no need for bowel mobilization and excellent visualization for posteriorly located renal disease.

  4. [Brachytherapy for sarcomas].

    PubMed

    Ducassou, A; Haie-Méder, C; Delannes, M

    2016-10-01

    The standard of care for local treatment for extremities soft tissue sarcomas relies on conservative surgery combined with external beam radiotherapy. Brachytherapy can be realized instead of external beam radiotherapy in selected cases, or more often used as a boost dose on a limited volume on the area at major risk of relapse, especially if a microscopic positive resection is expected. Close interaction and communication between radiation oncologists and surgeons are mandatory at the time of implantation to limit the risk of side effects. Long-term results are available for low-dose rate brachytherapy. Nowadays, pulsed dose rate or high-dose-rate brachytherapy are more often used. Brachytherapy for paediatric sarcomas is rare, and has to be managed in reference centres.

  5. Utility of PET/CT to Evaluate Retroperitoneal Lymph Node Metastasis in High-Risk Endometrial Cancer: Results of ACRIN 6671/GOG 0233 Trial.

    PubMed

    Atri, Mostafa; Zhang, Zheng; Dehdashti, Farrokh; Lee, Susanna I; Marques, Helga; Ali, Shamshad; Koh, Wui-Jin; Mannel, Robert S; DiSilvestro, Paul; King, Stephanie A; Pearl, Michael; Zhou, XunClare; Plante, Marie; Moxley, Katherine M; Gold, Michael

    2017-05-01

    Purpose To assess the diagnostic accuracy of fluorine 18 fluorodeoxyglucose (FDG) positron emission tomography (PET) combined with diagnostic contrast material-enhanced computed tomography (CT) in detecting lymph node (LN) metastasis in high-risk endometrial cancer. Materials and Methods This prospective multicenter HIPAA-compliant study had institutional review board approval, and all participants gave written informed consent. Data were accrued between January 2010 and June 2013. Patients underwent PET/CT and pelvic and abdominal lymphadenectomy. Two hundred seven of 215 enrolled patients had PET/CT and pathologic examination results for the abdomen and pelvis. Mean patient age was 62.7 years ± 9.6 (standard deviation). Data in all 23 patients with a positive abdominal examination and in 26 randomly selected patients with a negative abdominal examination were used for this central reader study. Seven independent blinded readers reviewed diagnostic CT and PET/CT results in different sessions 1 month apart. Accuracy was calculated at the participant level, correlating abdominal (right and left para-aortic and common iliac) and pelvic (right and left external iliac and obturator) LN regions with pathologic results, respecting laterality. Reader-average sensitivities, specificities, and areas under the receiver operating characteristic curve (AUCs) of PET/CT and diagnostic CT were compared. Power calculation was for sensitivity and specificity in the abdomen. Results Sensitivities of PET/CT versus diagnostic CT for the detection of LN metastasis were 0.65 (95% confidence interval [CI]: 0.57, 0.72) versus 0.50 (95% CI: 0.43, 0.58) (P = .01) in the abdomen and 0.65 (95% CI: 0.57, 0.72) versus 0.48 (95% CI: 0.41, 0.56) (P = .004) in the pelvis. Corresponding specificities were 0.88 (95% CI: 0.83, 0.92) versus 0.93 (95% CI: 0.89, 0.96) (P = .11) and 0.93 (95% CI: 0.86, 0.96) versus 0.89 (95% CI: 0.82, 0.94) (P = .27), and AUCs were 0.78 (95% CI: 0.66, 0.89) versus 0

  6. Stages of Adult Soft Tissue Sarcoma

    MedlinePlus

    ... soft tissue sarcomas: Childhood Soft Tissue Sarcoma Treatment Ewing Sarcoma Family of Tumors Treatment Gastrointestinal Stromal Tumors Treatment ... Sarcoma Home Page Childhood Soft Tissue Sarcoma Treatment Ewing Sarcoma Family of Tumors Treatment Gastrointestinal Stromal Tumors Treatment ...

  7. Treatment Options for Adult Soft Tissue Sarcoma

    MedlinePlus

    ... soft tissue sarcomas: Childhood Soft Tissue Sarcoma Treatment Ewing Sarcoma Family of Tumors Treatment Gastrointestinal Stromal Tumors Treatment ... Sarcoma Home Page Childhood Soft Tissue Sarcoma Treatment Ewing Sarcoma Family of Tumors Treatment Gastrointestinal Stromal Tumors Treatment ...

  8. Treatment Option Overview (Adult Soft Tissue Sarcoma)

    MedlinePlus

    ... soft tissue sarcomas: Childhood Soft Tissue Sarcoma Treatment Ewing Sarcoma Family of Tumors Treatment Gastrointestinal Stromal Tumors Treatment ... Sarcoma Home Page Childhood Soft Tissue Sarcoma Treatment Ewing Sarcoma Family of Tumors Treatment Gastrointestinal Stromal Tumors Treatment ...

  9. Do We Know What Causes Kaposi Sarcoma?

    MedlinePlus

    ... Factors, and Prevention Do We Know What Causes Kaposi Sarcoma? Kaposi sarcoma (KS) is caused by infection ... Sarcoma? Can Kaposi Sarcoma Be Prevented? More In Kaposi Sarcoma About Kaposi Sarcoma Causes, Risk Factors, and ...

  10. Retroperitoneal fibrosis in three siblings with the sickle cell trait

    PubMed Central

    Phills, James A.; Geggie, Peter; Hidvegi, Robert I.; Oliva, Luis A.

    1973-01-01

    Three West-Indian black siblings with the sickle cell trait developed retroperitoneal fibrosis, a previously unreported association. Other well known renal manifestations associated with the sickle cell trait were also present in some of these cases and included renal medullary necrosis and spontaneous hematuria. It is postulated that the sickling of the erythrocytes in the periureteral vessels resulted in thrombosis, ischemia, reactive scarring and progressive fibrosis indistinguishable from the known histological picture of retroperitoneal fibrosis. The finding of fibrin thrombi in the small veins of the fibrotic tissue of one of these patients would support this explanation. ImagesFIG. 1AFIG. 1BFIG. 2FIG. 3AFIG. 3BFIG. 4AFIG. 4BFIG. 4C PMID:4699274

  11. Surgical anatomy of the retroperitoneal spaces, Part IV: retroperitoneal nerves.

    PubMed

    Mirilas, Petros; Skandalakis, John E

    2010-03-01

    We present surgicoanatomical topographic relations of nerves and plexuses in the retroperitoneal space: 1) six named parietal nerves, branches of the lumbar plexus: iliohypogastric, ilioinguinal, genitofemoral, lateral femoral cutaneous, obturator, femoral. 2) The sacral plexus is formed by the lumbosacral trunk, ventral rami of S1-S3, and part of S4; the remainder of S4 joining the coccygeal plexus. From this plexus originate the superior gluteal nerve, which passes backward through the greater sciatic foramen above the piriformis muscle; the inferior gluteal nerve also courses through the greater sciatic foramen, but below the piriformis; 3) sympathetic trunks: right and left lumbar sympathetic trunks, which comprise four interconnected ganglia, and the pelvic chains; 4) greater, lesser, and least thoracic splanchnic nerves (sympathetic), which pass the diaphragm and join celiac ganglia; 5) four lumbar splanchnic nerves (sympathetic), which arise from lumbar sympathetic ganglia; 6) pelvic splanchnic nerves (nervi erigentes), providing parasympathetic innervation to the descending colon and pelvic splanchna; and 7) autonomic (prevertebral) plexuses, formed by the vagus nerves, splanchnic nerves, and ganglia (celiac, superior mesenteric, aorticorenal). They include sympathetic, parasympathetic, and sensory (mainly pain) fibers. The autonomic plexuses comprise named parts: aortic, superior mesenteric, inferior mesenteric, superior hypogastric, and inferior hypogastric (hypogastric nerves).

  12. Sarcomas other than Kaposi sarcoma occurring in immunodeficiency: interpretations from a systematic literature review.

    PubMed

    Bhatia, Kishor; Shiels, Meredith S; Berg, Alexandra; Engels, Eric A

    2012-09-01

    In immunodeficiency, an increased sarcoma risk is confirmed for Kaposi's sarcoma. Whether rates of other sarcoma subtypes are elevated in the setting of immunodeficiency is not known. We therefore reviewed published case reports on HIV and AIDS patients and organ transplant recipients with sarcomas. For comparison, we assessed sarcomas in the U.S. general population using Surveillance Epidemiology End Results (SEER) data. A total of 176 non-Kaposi sarcoma were identified, 75 in people with HIV and AIDS and 101 in transplant recipients. Leiomyosarcomas (n = 101) were the most frequently reported sarcomas, followed by angiosarcomas (n = 23) and fibrohistiocytic tumors (n = 17). Leiomyosarcomas were reported with two age peaks, in children and young adults. Epstein-Barr virus (EBV) was detected in the tumor cells in 85 and 88% of leiomyosarcomas in HIV-infected people and transplant recipients, respectively. Angiosarcomas and fibrohistiocytic tumors were most frequently reported in men. Among kidney transplant recipients, 20% of sarcomas arose at the site of an arteriovenous fistula. In comparison, leiomyoscarcomas, angiosarcomas, and fibrohistiocytic tumors comprised 16.9, 3.8, and 18.7% of sarcomas in the U.S. general population. Leiomyosarcoma and angiosarcoma may occur disproportionately in immunodeficiency. Leiomyosarcomas appear causatively linked to EBV, whereas angiosarcomas might be correlated with an arteriovenous fistula. Additional studies are necessary to understand the contribution of immunodeficiency to the cause of these sarcomas.

  13. A Review of the Literature on Extrarenal Retroperitoneal Angiomyolipoma

    PubMed Central

    Venyo, Anthony Kodzo-Grey

    2016-01-01

    Background. Extrarenal retroperitoneal angiomyolipomas are rare. Aim. To review the literature. Results. Angiomyolipomas, previously classified as hamartomas, are now classified as benign tumours. Thirty cases of primary retroperitoneal angiomyolipomas have been reported. Diagnosis of the disease upon is based radiological and pathological findings of triphasic features of (a) fat and (b) blood vessels and myoid tissue. Immunohistochemistry tends to be positive for HMB45, MART1, HHF35, calponin, NKI-C3, and CD117. The lesion is common in women. Treatment options have included the following: (a) radical surgical excision of the lesion with renal sparing surgery or radical nephrectomy in cases where malignant tumours could not be excluded and (b) selective embolization of the lesion alone or prior to surgical excision. One case of retroperitoneal angiomyolipoma was reported in a patient 15 years after undergoing radical nephrectomy for angiomyolipoma of kidney and two cases of distant metastases of angiomyolipoma have been reported following radical resection of the tumour. Conclusions. With the report of two cases of metastases ensuing surgical resection of the primary lesions there is need for academic pathologists to debate and review angiomyolipomas to decide whether to reclassify angiomyolipomas as slow-growing malignant tumours or whether the reported cases of metastases were de novo tumours or metastatic lesions. PMID:26989509

  14. [A case of tuberculous pyothorax with retroperitoneal gravitation abscess].

    PubMed

    Inada, T; Ide, H; Shiomi, K; Tsumura, M; Nakamura, Y; Kageyama, H

    1994-07-01

    A 65-year-old woman being treated under the diagnosis of pneumonia was subjected to further evaluation for right back tenderness. Chest radiography revealed a granular, net-like shadow in the right lower lung field, and right pleural thickening with pleural fluid retention. Ultrasonography demonstrated a cystic mass of uniformly low echo density, corresponding to the site of tenderness. This lesion was located on the right renal dorsal, right iliopsoas muscle ventral side. Based on CT findings of internal water density and marginal enhancement, the lesion was diagnosed as an abscess. With the suspicion of right tuberculous pyothorax with retroperitoneal gravitation abscess, puncture was attempted. The diagnosis was confirmed by the presence of Mycobacterium tuberculosis in the specimen. Antituberculosis chemotherapy was initiated, with drainage for symptom relief and lesion range definition. The abscess cavity communicated with the right pleural cavity via s stalk. The stalk path was confirmed by CT, and surgical curettage was performed. Tuberculous pyothorax with retroperitoneal gravitation abscess is rare and is not discussed in standard textbooks. In large series of cases it is not mentioned. In this patient we assumed that retroperitoneal gravitation abscess occurred as a result of the advanced state of tuberculous pyothorax.

  15. Soft Tissue Sarcoma

    MedlinePlus

    ... muscles, tendons, fat, and blood vessels. Soft tissue sarcoma is a cancer of these soft tissues. There ... have certain genetic diseases. Doctors diagnose soft tissue sarcomas with a biopsy. Treatments include surgery to remove ...

  16. Sarcoma Foundation of America

    MedlinePlus

    ... Make a Donation Matching Gifts Sarcoma Dedication Page Stocks and Securities Workplace Giving FAQ's Dedications Join Donate ... Make a Donation Matching Gifts Sarcoma Dedication Page Stocks and Securities Workplace Giving

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

    SciTech Connect

    Yoon, Sam S.; Duda, Dan G.; Karl, Daniel L.; Kim, Tae-Min; Kambadakone, Avinash R.; Chen, Yen-Lin; Rothrock, Courtney; Rosenberg, Andrew E.; Nielsen, G. Petur; Kirsch, David G.; Choy, Edwin; Harmon, David C.; Hornicek, Francis J.; Dreyfuss, Jonathan; Ancukiewicz, Marek; and others

    2011-11-15

    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 size 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 warrants

  18. Gallium-67 imaging and computed tomography in early retroperitoneal fibrosis

    SciTech Connect

    Leibowich, S.; Tumeh, S.S.

    1988-11-01

    Retroperitoneal fibrosis is characterized by symptoms associated with gradual compression of retroperitoneal structures. This case report demonstrates the use of Ga-67 imaging and CT in the early diagnostic work-up of this disorder.

  19. Retroperitoneal hematoma following radical orchiectomy: Two cases

    PubMed Central

    Glicksman, Rachel; Hamilton, Robert J.; Chung, Peter

    2017-01-01

    Treatment of testicular cancer is dependent on the stage of disease at presentation. Stage 1 testicular cancer is treated with radical orchiectomy, followed by active surveillance, radiotherapy, or chemotherapy. Occasionally, unusual and unexpected postoperative changes can be seen on computed tomography (CT), and may raise concern for metastatic disease. Here, we present two cases of testicular cancer patients who developed retroperitoneal hematomas post-radical orchiectomy, one as a classical clinical presentation, and the other as an atypical radiological entity only. The first is a case of a 38-year-old male with a non-seminoma testicular cancer, who developed severe flank pain, hemodynamic instability, and progressive anemia from a retroperitoneal hematoma in the immediate (<24 hours) postoperative period, requiring urgent surgical evacuation. The second is a case of a 33-year-old male with a testicular seminoma who had a large, suspicious retroperitoneal mass on a staging CT scan concerning for metastatic disease, which was later diagnosed as a retroperitoneal hematoma. These cases reveal the clinical variability with which a retroperitoneal hematoma post-radical orchiectomy may present. In addition, the second case demonstrates the importance of recognizing radiological postoperative changes and ensuring that these findings are not mistaken for and treated as metastatic disease. PMID:28163811

  20. [Retroperitoneal perforations of the colon. Apropos of 2 cases].

    PubMed

    Jurczak, F; Likholatnikov, D; Courant, O; Hamy, A; Visset, J; Paineau, J

    1994-01-01

    The retroperitoneal perforation of the colon is rare and our observations illustrate its two modes of revelation: a retroperitoneal suppuration; it must be traited quickly in order to decrease the mortality. Note that the abscess of the thigh is exceptional. Retroperitoneal perforations during colonoscopy whose treatment (initially medical) become surgical if there is no clinical improvement.

  1. [Low grade fibromixoid sarcoma: a purpose of 3 cases and review of the bibliography].

    PubMed

    Citores-Pascual, Miguel Angel; Tinoco-Carrasco, Claudia; Arenal-Vera, Juan José; Benito-Fernández, César; Torres-Nieto, María de Los Ángeles; Zamora-Martínez, Tomás

    2013-01-01

    Low grade fibromyxoid sarcoma or tumor Evans is a variety of soft tissue sarcoma that represents 1% of all malignancies. More common in limbs and trunk (50%), we present 3 new cases of retroperitoneal reviewing their characteristics and performing a literature review. In the retroperitoneal location highlights the poor specificity of clinical symptoms, demonstrating, according to their growth and size, as an abdominal tumor, usually painless, or by compression and/or invasion of nearby structures. In cases that present the most important finding was the presence of a palpable abdominal tumor without other symptoms despite remarkable infiltration of other organs that should be included in surgical resection. We emphasize the difficulty of correct diagnosis preoperatively because preoperative studies are inconclusive and only the histological and immuno-histo-typing chemistry allow precise identification.

  2. Robot-assisted laparoscopic retroperitoneal lymph node dissection for stage IIIb mixed germ cell testicular cancer after chemotherapy.

    PubMed

    Lee, Sang Hyub; Kim, Dong Soo; Chang, Sung-Goo; Jeon, Seung Hyun

    2015-07-01

    Laparoscopic retroperitoneal lymph node dissection, especially when performed with the da Vinci Surgical System (Intuitive Surgical), has shown excellent cosmetic results with similar oncologic outcomes to those of open surgery. In this study, we present a case of robot-assisted retroperitoneal lymph node dissection performed in an 18-year-old man who was diagnosed with a stage IIIb mixed germ cell tumor and who was initially treated with radical orchiectomy, followed by chemotherapy. This case shows that robot-assisted retroperitoneal lymph node dissection is technically feasible, safe, and cosmetically favorable, even when performed on patients with high-stage disease or after chemotherapy.

  3. Laparoscopic Resection of Retroperitoneal Neural Tumors

    PubMed Central

    Nozaki, Tetsuo; Kato, Tomonori; Morii, Akihiro; Fuse, Hideki

    2013-01-01

    Purpose Retroperitoneal neural tumor (RNT) is rarely excised laparoscopically, and the laparoscopic management of RNT remains controversial. We herein report 4 cases of laparoscopic excision of RNT that resulted in diverse clinical outcomes. Patients and Methods Between August 2005 and January 2011, we performed laparoscopic excision of RNT in 4 patients. The mean tumor size was 4.5 cm. The mean operative time was 297 minutes and the mean amount of blood loss was 55 ml. The surgeries were uneventful, with no operative complications or evidence of intra-abdominal bleeding. However, 2 patients required reoperation for delayed hemorrhage and urinoma formation, respectively. Results The postoperative pathological diagnoses were schwannoma in 3 patients and ganglioneuroblastoma in 1 patient. All patients were well with no signs of peripheral neuropathy or radiculopathy, and CT and/or 18F-FDG PET/CT performed during follow-up indicated no evidence of disease. Conclusions Obtaining extensive preoperative knowledge of the source neural and vascular anatomy of the tumor is important for the surgical planning of laparoscopic resection of RNT. When a great deal of care is taken to divide the tumor and the source nerves and vital vessels, safe execution of RNT can be achieved for minimal postoperative mortality and morbidity. PMID:24917756

  4. The recurrent primary retroperitoneal liposarcoma.

    PubMed

    Nagy, V; Bober, J; Zavacky, P; Brandebur, O; Svajdler, M

    2013-01-01

    Describe a patient with multiple recurrences of the primary recurrent liposarcoma. A 60-years-old man complained of weight loss (BMI 18.4) with a palpable huge retroperitoneal tumour, which displaced left kidney, and was confirmed on USG and CT. Laboratory examination showed anaemia and pathological blood tests. Chest X-ray initially showed a negative finding. A complete transperitonealy surgical extirpation of the tumour with left side nephrectomy was performed on June 28, 2007. The tumour mass weight was 1900 g. It was lying on the posterior face of the kidney in diameters 170x120x120 mm, completely capsulated by thin grey-pink capsula with peripheral fat tissue on the section grey-pink, lobulary shaped, in ¾ parts with central necrotic changes. Histopathologically was confirmed the primary dedifferentiated (non-lipogenous) liposarcoma low grade of malignancy. Nephrectomy specimen was confirmed as age related finding. There was no evidence of positives surgical margins. Despite oncological and surgical treatment, followed repeated recurrence with eight transperitoneal surgeries in the retroperitoneum and abdomen with extirpation of the metastases, left side hemicolectomy, splenectomy and repeated extirpation tumour metastases from abdomen and radix mesenterii. Last tumour weighed 2900 grams. Patient died on January 9, 2011, after the eight surgeries on multiorgans failure due to hemorrhagic shock and persistent atrial fibrilaton by cardiopulmonary insufficiency. As a speciality, he was treated without transfusion because as Jehovah´s witness he refused blood derivates. Despite complex surgical and oncological treatment, the prognosis in patient with recurrent liposarcoma was fatal (Tab. 1, Fig. 5, Ref. 50).

  5. Efficacy and safety of gemcitabine plus docetaxel in Japanese patients with unresectable or recurrent bone and soft tissue sarcoma: Results from a single-institutional analysis.

    PubMed

    Takahashi, Masanobu; Komine, Keigo; Imai, Hiroo; Okada, Yoshinari; Saijo, Ken; Takahashi, Masahiro; Shirota, Hidekazu; Ohori, Hisatsugu; Takahashi, Shin; Chiba, Natsuko; Mori, Takahiro; Shimodaira, Hideki; Ishioka, Chikashi

    2017-01-01

    Combination therapy with gemcitabine and docetaxel has been reported to be a good therapeutic strategy for patients with soft tissue sarcoma. The aim of the present study was to analyze the efficacy and toxicity of gemcitabine with docetaxel in Japanese patients with advanced bone and soft tissue sarcoma. We retrospectively analyzed the effect of gemcitabine and docetaxel therapy on overall response, progression-free survival, overall survival, and toxicity in 42 patients with bone or soft tissue sarcoma who had received the therapy between October 2006 and September 2015, at Tohoku University Hospital. The median age was 55 years; 23 patients were men, and 19 were women. Eight had bone sarcoma and 34 had soft tissue sarcoma. Forty patients (95%) had previously been treated with one or more chemotherapeutic regimens. The overall response rate was 6.9% and the disease control rate was 55%. The median progression-free survival was 2.3 months and the median overall survival was 14.3 months. Grade 3 or more neutropenia and febrile neutropenia were observed in 74% and 4.8% of all patients, respectively. The response rate was lower and myelosuppression was more frequently observed than in other previous reports. On the other hand, most of toxicities were enough manageable. In addition, some patients had long survival with a good response. Our study supports the notion that gemcitabine and docetaxel therapy is a good therapeutic option for treating patients with advanced soft tissue sarcoma as well as bone sarcoma, also in Asian populations.

  6. Efficacy of trabectedin in advanced soft tissue sarcoma: beyond lipo- and leiomyosarcoma

    PubMed Central

    De Sanctis, Rita; Marrari, Andrea; Marchetti, Silvia; Mussi, Chiara; Balzarini, Luca; Lutman, Fabio Romano; Daolio, Primo; Bastoni, Stefano; Bertuzzi, Alexia Francesca; Quagliuolo, Vittorio; Santoro, Armando

    2015-01-01

    Objective Trabectedin is effective in leiomyosarcoma and liposarcoma, especially the myxoid variant, related to the presence of the FUS-CHOP transcript. We evaluated the efficacy of trabectedin in specific subgroups of patients with soft tissue sarcomas (STS). Methods Seventy-two patients with advanced anthracycline-pretreated STS, who received trabectedin at a dose of 1.5 mg/m2 every 3 weeks by continuous 24-hour infusion, were retrospectively analyzed. Best response rate according to Response Evaluation Criteria In Solid Tumors (RECIST) criteria and severe adverse events (AEs) according to National Cancer Institute Common Terminology Criteria for Adverse Events (NCI-CTCAE v4.02) were evaluated. Secondary endpoints included progression-free survival and overall survival (OS). Results Median age was 48 (range, 20–75) years, with a median Eastern Cooperative Oncology Group performance status of 0. The median number of previous chemotherapy regimens was 1 (range, 0–5). Median number of trabectedin cycles was 3 (range, 1–17). About 69/72 patients (95.8%) were evaluable for response: 9 patients (13%) achieved partial response and 26 (37.7%) stable disease. According to histotype, clinical benefit (partial response + stable disease) was reported in synovial sarcoma (n=5), retroperitoneal liposarcoma (n=10), myxoid liposarcoma (n=5), leiomyosarcoma (n=8), high-grade undifferentiated pleomorphic sarcoma (n=5), Ewing/peripheral primitive neuroectodermal tumor (n=1), and malignant peripheral nerve sheath tumor (n=1). Any grade AEs were noncumulative, reversible, and manageable. G3/G4 AEs included anemia (n=1, 1.4%), neutropenia (n=7, 9.6%), liver toxicity (n=6, 8.3%), and fatigue (n=2, 2.8%). With a median follow-up time of 11 (range, 2–23) months, median progression-free survival and OS of the entire cohort were 2.97 months and 16.5 months, respectively. Conclusion Our experience confirms trabectedin as an effective therapeutic option for metastatic lipo- and

  7. Targeted therapy for sarcomas

    PubMed Central

    Forscher, Charles; Mita, Monica; Figlin, Robert

    2014-01-01

    Sarcomas are tumors of mesenchymal origin that make up approximately 1% of human cancers. They may arise as primary tumors in either bone or soft tissue, with approximately 11,280 soft tissue tumors and 2,650 bone tumors diagnosed each year in the United States. There are at least 50 different subtypes of soft tissue sarcoma, with new ones described with ever-increasing frequency. One way to look at sarcomas is to divide them into categories on the basis of their genetic make-up. One group of sarcomas has an identifiable, relatively simple genetic signature, such as the X:18 translocation seen in synovial sarcoma or the 11:22 translocation seen in Ewing’s sarcoma. These specific abnormalities often lead to the presence of fusion proteins, such as EWS-FLI1 in Ewing’s sarcoma, which are helpful as diagnostic tools and may become therapeutic targets in the future. Another group of sarcomas is characterized by complex genetic abnormalities as seen in leiomyosarcoma, osteosarcoma, and undifferentiated sarcoma. It is important to keep these distinctions in mind when contemplating the development of targeted agents for sarcomas. Different abnormalities in sarcoma could be divided by tumor subtype or by the molecular or pathway abnormality. However, some existing drugs or drugs in development may interfere with or alter more than one of the presented pathways. PMID:24669185

  8. A prospective, randomized study of adjuvant parenteral nutrition in the treatment of sarcomas: results of metabolic and survival studies.

    PubMed

    Shamberger, R C; Brennan, M F; Goodgame, J T; Lowry, S F; Maher, M M; Wesley, R A; Pizzo, P A

    1984-07-01

    A prospective, randomized study was performed to evaluate the use of total parenteral nutrition (TPN) in a group of young patients receiving aggressive chemotherapy for metastatic or locally recurrent sarcomas. Fourteen patients were randomly selected to receive TPN and 18 to receive conventional oral nutritional support (CN). During the study period (from first dose of chemotherapy to recovery from myelosuppression), the TPN patients received between 1020 and 2100 calories/m2/day (median 1650) and between 5.3 and 12.4 gmN/m2/day (median 8.9), while the CN patients received between 380 and 880 calories/m2/day (median 685) and between 0.0 and 3.7 gmN/m2/day (median 1.5). The mean daily nitrogen balance during the study period for the TPN group (-3.0 to + 1.3 gmN/m2/day, median -0.7) was significantly higher (p = 0.005) than that of the CN group (-6.2 to -0.7 gmN/m2/day, median -2.6). Serum protein levels (albumin, total protein, and transferrin) did not differ between the two treatment groups. The proportion of patients responding to therapy and the long-term survival rates were similar in the treatment groups. Thus despite established improvement in nitrogen balance, no survival or therapeutic advantage was demonstrated for the adjuvant parenteral nutrition group. Further studies of the role of parenteral nutrition as an adjuvant to cancer chemotherapy are needed to determine which populations of patients will benefit from its use.

  9. The clinical application of plasma Kaposi sarcoma herpesvirus viral load as a tumour biomarker: results from 704 patients.

    PubMed

    Haq, I-U; Dalla Pria, A; Papanastasopoulos, P; Stegmann, K; Bradshaw, D; Nelson, M; Bower, M

    2016-01-01

    The aim of the study was to evaluate the role of plasma Kaposi sarcoma herpesvirus (KSHV) as a diagnostic and prognostic biomarker in people living with HIV (PLWH) and diagnosed with KSHV-associated diseases. Using quantitative nested polymerase chain reaction (PCR) targeting the open reading frame-26 gene of KSHV, plasma levels of KSHV were measured in consecutive PLWH with KSHV-associated diseases or as part of the investigation of lymphadenopathy. Plasma KSHV assays were performed on samples from 684 PLWH and 20 HIV-seronegative people with KSHV-associated malignancies. In PLWH, plasma KSHV was detected in 39% of those with KS, 99% of those with multicentric Castleman disease (MCD), 9% of those with non-Hodgkin lymphoma (NHL), 2% of those with non-AIDS-defining malignancies and 0% of those with nonmalignant lymphadenopathy. There was no significant difference in plasma KSHV viral load among those with KS, MCD and KSHV-associated NHL. The 5-year overall survival rate from KS diagnosis of 335 PLWH was 95.2% (95% confidence interval 92.6-97.8%). Plasma KSHV viraemia did not predict overall survival in those with KS (P = 0.73), nor when those with T0 stage KS (P = 0.52) or T1 stage KS (P = 0.62) were analysed separately. Measuring the plasma levels of KSHV as a biomarker in KSHV-associated disease has a very limited value in either diagnosis or prognostication. The only potential role of clinical value is the suggestion that an undetectable plasma KSHV excludes a diagnosis of MCD in PLWH. © 2015 British HIV Association.

  10. Results of the Scandinavian Sarcoma Group XIV protocol for classical osteosarcoma: 63 patients with a minimum follow-up of 4 years.

    PubMed

    Smeland, Sigbjørn; Bruland, Oyvind S; Hjorth, Lars; Brosjö, Otte; Bjerkehagen, Bodil; Osterlundh, Gustaf; Jakobson, Ake; Hall, Kirsten Sundby; Monge, Odd R; Björk, Olle; Alvegaard, Thor A

    2011-04-01

    The Scandinavian Sarcoma Group (SSG) XIV protocol is based on experience from previous SSG trials and other osteosarcoma intergroup trials, and has been considered the best standard of care for patients with extremity localized, non-metastatic osteosarcoma. We analyzed the outcome in 63 consecutive patients. Patients and methods From 2001 through 2005, 63 patients recruited from centers in Sweden, Norway, and Finland were included. They received preoperative chemotherapy consisting of 2 cycles of paired methotrexate (12 g/m²), cisplatin (90 mg/m²), and doxorubicin (75 mg/m²). 3 cycles were administered postoperatively, and poor histological responders were given 3 additional cycles of ifosfamide (10-12 g/m²) as a salvage strategy. With a median follow-up of 77 months for survivors, the estimated metastasis-free and sarcoma-related survival at 5 years was 70% and 76%, respectively. 53 patients were treated with limb salvage surgery or rotationplasty and 2 patients experienced a local recurrence. 3 toxic deaths were recorded, all related to acute toxicity from chemotherapy. The 5-year metastasis-free survival of poor histological responders receiving add-on treatment with ifosfamide was 47%, as compared to 89% for good histological responders. Outcome from the SSG XIV protocol compares favorably with the results of previous SSG trials and other published osteosarcoma trials. However, salvage therapy given to poor responders did not improve outcome to a similar degree as for good responders. In a multi-institutional setting, more than four-fifths of the patients were operated with limb salvage surgery or rotationplasty, with few local recurrences.

  11. [Comments on retroperitoneal lymphadenectomy - laparoscopic versus robotic].

    PubMed

    Heidenreich, A

    2012-05-01

    Retroperitoneal lymph node dissection (RPLND) in high risk clinical stage I nonseminomatous germ cell tumors (NSGCT) plays a limited role in modern uro-oncology due to the superior therapeutic efficacy of even one cycle of PEB (cysplatin, etoposide, bleomycin) chemotherapy. There might be an indication for the rare case of pure mature teratoma with unfavorable prognostic risk factors. If RPLND is performed for clinical stage I NSGCT it always has to be performed in a nerve-sparing technique and within the well-defined boundaries of an anatomically adequate template in order to avoid unnecessary adjuvant systemic chemotherapy. In this aspect, laparoscopic RPLND is inferior to open RPLND as basically all patients with lymph node positive disease receive adjuvant chemotherapy. The evidence for robotic-assisted RPLND is too weak to draw any clinically useful conclusions. Currently, it is an experimental procedure.Postchemotherapy RPLND (PC-RPLND) remains a surgery for tertiary referral centres due to the complexity of the surgical intervention and the high probability of adjunctive visceral and/or vascular surgery. In accordance with international guidelines it remains a domain for an open surgical approach. Laparoscopic PC-RPLND is reserved for small residual masses with the option of a unilateral modified template resection in very experienced laparoscopic centres. With regard to robotic-assisted PC-RPLND there is no evidence in the literature with regard to morbidity and complications, short-term and long-term oncological results being in favor of this experimental approach.

  12. Mast cell sarcoma: clinical management.

    PubMed

    Weiler, Catherine R; Butterfield, Joseph

    2014-05-01

    Mast cell sarcoma is a disorder that results in abnormal mast cells as identified by morphology, special stains, and in some publications, c-kit mutation analysis. It affects animal species such as canines more commonly than humans. In humans it is a very rare condition, with variable clinical presentation. There is no standard therapy for the disorder. It can affect any age group. It is occasionally associated with systemic mastocytosis and/or urticaria pigmentosa. The prognosis of mast cell sarcoma in published literature is very poor in humans.

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

    PubMed

    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.

  14. Can Soft Tissue Sarcomas Be Found Early?

    MedlinePlus

    ... Tissue Sarcoma Early Detection, Diagnosis, and Staging Can Soft Tissue Sarcomas Be Found Early? People who have ... Your Doctor About Soft Tissue Sarcomas? More In Soft Tissue Sarcoma About Soft Tissue Sarcoma Causes, Risk ...

  15. Spontaneous Retroperitoneal Hemorrhage from Adrenal Artery Aneurysm

    SciTech Connect

    Gonzalez Valverde, F.M. Balsalobre, M.; Torregrosa, N.; Molto, M.; Gomez Ramos, M.J.; Vazquez Rojas, J.L.

    2007-04-15

    Spontaneous adrenal hemorrhage is a very rare but serious disorder of the adrenal gland that can require emergent treatment. We report on a 42-year-old man who underwent selective angiography for diagnosis and treatment of retroperitoneal hemorrhage from small adrenal artery aneurysm. This case gives further details about the value of transluminal artery embolization in the management of visceral aneurysm rupture.

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

    SciTech Connect

    Lee, V.W.; Rosen, M.P.; Baum, A.; Cohen, S.E.; Cooley, T.P.; Liebman, H.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 Kaposi 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.

  17. Targeting protein kinases to reverse multidrug resistance in sarcoma.

    PubMed

    Chen, Hua; Shen, Jacson; Choy, Edwin; Hornicek, Francis J; Duan, Zhenfeng

    2016-02-01

    Sarcomas are a group of cancers that arise from transformed cells of mesenchymal origin. They can be classified into over 50 subtypes, accounting for approximately 1% of adult and 15% of pediatric cancers. Wide surgical resection, radiotherapy, and chemotherapy are the most common treatments for the majority of sarcomas. Among these therapies, chemotherapy can palliate symptoms and prolong life for some sarcoma patients. However, sarcoma cells can have intrinsic or acquired resistance after treatment with chemotherapeutics drugs, leading to the development of multidrug resistance (MDR). MDR attenuates the efficacy of anticancer drugs and results in treatment failure for sarcomas. Therefore, overcoming MDR is an unmet need for sarcoma therapy. Certain protein kinases demonstrate aberrant expression and/or activity in sarcoma cells, which have been found to be involved in the regulation of sarcoma cell progression, such as cell cycle, apoptosis, and survival. Inhibiting these protein kinases may not only decrease the proliferation and growth of sarcoma cells, but also reverse their resistance to chemotherapeutic drugs to subsequently reduce the doses of anticancer drugs and decrease drug side-effects. The discovery of novel strategies targeting protein kinases opens a door to a new area of sarcoma research and provides insight into the mechanisms of MDR in chemotherapy. This review will focus on the recent studies in targeting protein kinase to reverse chemotherapeutic drug resistance in sarcoma. Copyright © 2015 Elsevier Ltd. All rights reserved.

  18. Targeting Protein Kinases to Reverse Multidrug Resistance in Sarcoma

    PubMed Central

    Chen, Hua; Shen, Jacson; Choy, Edwin; Hornicek, Francis J.; Duan, Zhenfeng

    2015-01-01

    Sarcomas are a group of cancers that arise from transformed cells of mesenchymal origin. They can be classified into over 50 subtypes, accounting for approximately 1% of adult and 15% of pediatric cancers. Wide surgical resection, radiotherapy, and chemotherapy are the most common treatments for the majority of sarcomas. Among these therapies, chemotherapy can palliate symptoms and prolong life for some sarcoma patients. However, sarcoma cells can have intrinsic or acquired resistance after treatment with chemotherapeutics drugs, leading to the development of multidrug resistance (MDR). MDR attenuates the efficacy of anticancer drugs and results in treatment failure for sarcomas. Therefore, overcoming MDR is an unmet need for sarcoma therapy. Certain protein kinases demonstrate aberrant expression and/or activity in sarcoma cells, which have been found to be involved in the regulation of sarcoma cell progression, such as cell cycle, apoptosis, and survival. Inhibiting these protein kinases may not only decrease the proliferation and growth of sarcoma cells, but also reverse their resistance to chemotherapeutic drugs to subsequently reduce the doses of anticancer drugs and decrease drug side-effects. The discovery of novel strategies targeting protein kinases opens a door to a new area of sarcoma research and provides insight into the mechanisms of MDR in chemotherapy. This review will focus on the recent studies in targeting protein kinase to reverse chemotherapeutic drug resistance in sarcoma. PMID:26827688

  19. [Retroperitoneal approach in the treatment of supra- and infrarenal aortic aneurysms].

    PubMed

    Mandolfino, Tommaso; Canciglia, Aldo; Russo, Salvatore; Carmignani, Amedeo; Mastrojeni, Claudio; Spinelli, Francesco

    2004-01-01

    The retroperitoneal approach for the treatment of thoracoabdominal type IV and infrarenal aortic aneurysms is an accepted alternative to thoraco-phrenolaparotomy. The purpose of this retrospective study was to report our experience and results in terms of respiratory and renal complications. From January 1997 to December 2003, 48 patients (36 with thoracoabdominal type IV and 12 with infrarenal aortic aneurysms) were treated by a retroperitoneal extrapleural approach in intercostal space X or XI. We performed 40 aorto-aortic and 8 aorto-basilar reconstructions. The perioperative mortality was 2%. Postoperative respiratory insufficiency was 8%, and postoperative renal insufficiency 12%. Permanent dialysis was necessary in 4% of cases. The survival rates were 98%, 89.4% and 58.7 at 1, 5 and 7 years, respectively. Retroperitoneal extrapleural access with a partial phrenotomy results in a significantly reduced incidence of postoperative respiratory complications.

  20. Survival and Margin Status in Head and Neck Radiation-Induced Sarcomas and De Novo Sarcomas.

    PubMed

    Rosko, Andrew J; Birkeland, Andrew C; Chinn, Steven B; Shuman, Andrew G; Prince, Mark E; Patel, Rajiv M; McHugh, Jonathan B; Spector, Matthew E

    2017-04-01

    Objective To describe histologic subtypes and oncologic outcomes among patients with radiation-induced and de novo sarcomas of the head and neck. Study Design Retrospective case series with chart review. Setting Tertiary academic center. Subject and Methods In total, 166 adult patients with sarcoma of the head and neck treated from January 1, 1985, to January 1, 2010, were included. Tumors were characterized as radiation induced (15.1%) vs de novo sarcomas (84.9%). Clinical and tumor characteristics were compared. The primary outcomes were overall survival (OS) and disease-specific survival (DSS). Results Radiation-induced sarcomas were more likely to be high grade ( P = .006) and advanced stage ( P = .03). Chondrosarcoma was more common in de novo tumors ( P = .02) while leiomyosarcoma ( P = .01), sarcoma not otherwise specified ( P = .02), and undifferentiated pleomorphic sarcoma ( P < .001) were more common in radiation-induced sarcomas. Radiation-induced sarcomas were associated with statistically significantly worse DSS ( P = .019) and OS ( P = .005) compared with de novo sarcomas, but when only high-grade soft tissue sarcomas were analyzed, neither DSS ( P = .48) nor OS ( P = .29) differed. Margin status was a significant predictor of survival as both R0 and R1 resections correlated with statistically better DSS and OS compared with R2 ( P < .001) resections and patients treated with radiation therapy/chemoradiation therapy alone ( P = .005). Conclusion Radiation-induced sarcomas of the head and neck correlate with worse survival compared with de novo tumors; however, when controlling for tumor grade and resection status, there is no statistically significant difference in observed outcomes.

  1. Keratin subsets in spindle cell sarcomas. Keratins are widespread but synovial sarcoma contains a distinctive keratin polypeptide pattern and desmoplakins.

    PubMed Central

    Miettinen, M.

    1991-01-01

    The presence of individual keratin polypeptides and desmoplakins was immunohistochemically studied in 25 spindle cell sarcomas of different types using acetone-fixed frozen sections. Results revealed that keratins 8 and 18 were present in a high number of tumors: 9 of 9 synovial sarcomas, 5 of 7 leiomyosarcomas, 5 of 5 malignant schwannomas, and 1 of 4 undifferentiated spindle cell sarcomas. In addition to keratins 8 and 18, the glandular component of synovial sarcoma showed prominent reactivity with antibodies to keratins 7 and 19. Also the glandular epithelial cells in synovial sarcoma showed desmoplakin immunoreactivity preferentially in a luminal distribution, but desmoplakin was absent in other spindle cell sarcomas. Furthermore keratin 13 was seen focally in 4 of 9 synovial sarcomas. In contrast, keratins 7, 13, and 19 were practically absent in leiomyosarcomas, malignant schwannomas, and undifferentiated spindle cell sarcomas. The widespread presence of keratins 8 and 18 in various spindle cell sarcomas may reflect aberrant keratin expression in mesenchymal cells, previously described in cultured transformed fibroblasts. The presence of keratins 7 and 19 and desmoplakin is highly associated with morphologically observable epithelial differentiation restricted to synovial sarcoma among spindle cell sarcomas. Images Figure 1 Figure 2 Figure 3 PMID:1704194

  2. The Biology of Ewing Sarcoma

    PubMed Central

    Ross, Keir A.; Smyth, Niall A.; Murawski, Christopher D.; Kennedy, John G.

    2013-01-01

    Objective. The goal of this study was to review the current literature on the biology of Ewing's sarcoma, including current treatments and the means by which an understanding of biological mechanisms could impact future treatments. Methods. A search of PubMed and The Cochrane Collaboration was performed. Both preclinical and clinical evidence was considered, but specific case reports were not. Primary research articles and reviews were analyzed with an emphasis on recent publications. Results. Ewing sarcoma is associated with specific chromosomal translocations and the resulting transcripts/proteins. Knowledge of the biology of Ewing sarcoma has been growing but has yet to significantly impact or produce new treatments. Localized cases have seen improvements in survival rates, but the same cannot be said of metastatic and recurrent cases. Standard surgical, radiation, and chemotherapy treatments are reaching their efficacy limits. Conclusion. Improving prognosis likely lies in advancing biomarkers and early diagnosis, determining a cell(s) of origin, and developing effective molecular therapeutics and antiangiogenic agents. Preclinical evidence suggests the utility of molecular therapies for Ewing sarcoma. Early clinical results also reveal potential for novel treatments but require further development and evaluation before widespread use can be advocated. PMID:23346417

  3. Retroperitoneal and intrahepatic metastasis from primary clear cell carcinoma of the liver

    PubMed Central

    Xiong, Junjie; He, Du; Hu, Weiming; Liu, Xubao

    2017-01-01

    Abstract Background: Hepatocellular carcinoma (HCC) is a major cause of cancer-related mortality worldwide and the incidence is increasing as a result of growing hepatitis B and C virus infections. Primary clear cell carcinoma of the liver (PCCCL) is a rare subgroup of primary HCC, which has low metastatic potential and infrequently reported in literature. Retroperitoneal and intrahepatic metastasis of PCCCL has not been reported previously. Case Summary: Here, we present a 55-year-old male with retroperitoneal and intrahepatic metastasis of PCCCL who is managed with surgical method and transcatheter arterial chemoembolization (TACE) at our institution. When the patient is followed up in 16 months after surgery and TACE, he is alive without any extrahepatic metastasis and abnomal liver function. Conclusion: We concluded that surgical resection of retroperitoneal metastasis and TACE of the intrahepatic tumors provided an appropriate strategy for the patient with unresectable PCCCL accompanied with extra-hepatic metastasis. PMID:28328858

  4. 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

  5. The soft tissue sarcomas

    SciTech Connect

    Eilber, F.R.; Morton, D.L.; Sondak, V.K.; Economou, J.S.

    1987-01-01

    New advances in multimodality therapy of sarcomas in all anatomic sites are thoroughly described. Multimodality therapy with limb-salvage surgery for extremity tumors, sarcomas of the head and neck, trunk, intraabdominal, visceral, and genitourinary tract and cardiopulmonary system are presented. Separate sections are devoted to the management of pediatric sarcomas, pulmonary metastasis and to the pathology and radiobiology, chemotherapy, and immunotherapy of sarcomas. The text also stresses the philosophy of achieving adequate local control without radical amputation by combined surgery and chemo/radiotherapy.

  6. MYCOBACTERIUM INTRACELLULARE INFECTION CAUSING A RETROPERITONEAL MASS IN A BINTURONG (ARCTICTIS BINTURONG).

    PubMed

    Adamovicz, Laura; Kennedy-Stoskopf, Suzanne; Talley, Ashley; Cullen, John M; Cohen, Eli B; Bizikova, Petra; Grunkemeyer, Vanessa

    2017-06-01

    A 19-yr-old castrated male binturong ( Arctictis binturong ) with a history of recurrent pyogranulomatous panniculitis, lymphangitis, and dermatitis was presented for evaluation of hyporexia and tenesmus. A large caudal abdominal mass was palpated on physical examination. On ultrasound, the mass encircled and obstructed the left ureter, resulting in hydroureter and hydronephrosis. The animal was euthanized, and necropsy revealed a large retroperitoneal pyogranuloma with acid-fast organisms identified in both the mass and the perineal skin. The acid-fast organisms within the retroperitoneal mass were identified as Mycobacterium intracellulare by PCR. This case represents an unusual presentation of M. intracellulare in a novel species.

  7. Retroperitoneal fibrosis: retrospective descriptive study on clinical features and management

    PubMed Central

    Laroche, Ann-Sophie; Bell, Robert Z; Bezzaoucha, Sarah; Földes, Eva; Lamarche, Caroline; Vallée, Michel

    2016-01-01

    Introduction Retroperitoneal fibrosis (RPF) is a rare condition characterized by the presence of inflammatory and fibrous retroperitoneal tissue that often encases the ureters or abdominal organs. This study describes the clinical characteristics, diagnostic methods, and treatments and their effects on renal function. Methods We conducted a retrospective analysis of patients diagnosed with RPF at Maisonneuve-Rosemont Hospital. Results We identified 17 patients with RPF between 1998 and 2013. Eight patients were females (47%), and the mean age was 62±18 years. Eleven patients were idiopathic. Back pain was the most common symptom. All diagnoses were made based on the finding of a retroperitoneal mass on the computed tomography scan. Three patients had histological diagnosis of RPF and seven patients had unspecific changes on their biopsy. Twelve patients needed double-J stents, three patients had a temporary percutaneous nephrostomy, two patients had to have a nephrectomy for refractory ureteral obstruction, and one patient required hemodialysis. Ten patients with idiopathic RPF received medical treatment. In the treated group, only two patients had complete remission of the disease and five patients had improvement of their lesions. There were no deteriorations and only one relapse. Seven patients did not receive any treatment; two of them achieved complete remission, one of them deteriorated, and two of them had no changes. Conclusion Most of our cases of RPF were idiopathic. Almost all treated patients received prednisone and seemed to respond, at least partially. There was a lot of heterogeneity in patient management, which makes it difficult to compare treatment effects. However, treated patients seemed to have more favorable outcomes than those who were not. PMID:27822461

  8. 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

  9. Postradiation sarcoma involving the spine

    SciTech Connect

    Sundaresan, N.; Huvos, A.G.; Krol, G.; Hughes, J.E.; Cahan, W.G.

    1986-06-01

    Postradiation sarcomas arising many years after treatment of cancer are long term sequelae of therapy. We describe the clinical features, radiographic findings, and results of treatment in 13 patients with such sarcomas encountered over a 6-year period. Of these patients, 9 had bone sarcomas and the remaining 4 had paraspinal tumors arising from adjacent soft tissue and nerve. The primary cancer for which radiation was given included Hodgkin's disease (4 patients), breast cancer (2 patients), cervix cancer (2 patients), and a variety of others (5 patients). The latent interval to the occurrence of the second neoplasm varied from 6 to 30 years (median, 10 years) after treatment of the original tumor. Despite aggressive treatment, the overall prognosis was poor. The median survival was 8 months, with only 3 surviving more than 2 years. Although rare, postradiation sarcoma should be considered in the differential diagnosis of patients presenting with late onset of spinal pain or neurological symptoms after clinical remission of an original cancer.

  10. Retroperitoneal abscess: an extra-abdominal manifestation

    PubMed Central

    Mallia, Alvin James; Ashwood, Neil; Arealis, George; Galanopoulos, Ilias

    2015-01-01

    Retroperitoneal abscesses are unusual occurrences with occult and insidious presentations. There is often a lack of abdominal signs, leading to delays in drainage and high mortality rates. We report a case of thigh emphysema in an 88-year-old patient with diabetes. Prior to admission the patient reported a vague 4-week history of left thigh pain and an inability to fully weight bear. She presented to our emergency department with sepsis and acute kidney impairment. An X-ray of her left femur revealed widespread gas between muscular planes. A retroperitoneal abscess involving the left renal fossa, psoas, iliacus and upper thigh muscles was revealed on an urgent CT scan. The patient was transferred to intensive care unit (ICU) and underwent an emergency drainage. Despite ICU the patient died 2 days after admission. PMID:25576509

  11. Retroperitoneal abscess: an extra-abdominal manifestation.

    PubMed

    Mallia, Alvin James; Ashwood, Neil; Arealis, George; Galanopoulos, Ilias

    2015-01-09

    Retroperitoneal abscesses are unusual occurrences with occult and insidious presentations. There is often a lack of abdominal signs, leading to delays in drainage and high mortality rates. We report a case of thigh emphysema in an 88-year-old patient with diabetes. Prior to admission the patient reported a vague 4-week history of left thigh pain and an inability to fully weight bear. She presented to our emergency department with sepsis and acute kidney impairment. An X-ray of her left femur revealed widespread gas between muscular planes. A retroperitoneal abscess involving the left renal fossa, psoas, iliacus and upper thigh muscles was revealed on an urgent CT scan. The patient was transferred to intensive care unit (ICU) and underwent an emergency drainage. Despite ICU the patient died 2 days after admission.

  12. Large benign retroperitoneal tumour in pregnancy.

    PubMed

    Berczi, Csaba; Osvath, Peter; Flasko, Tibor

    2015-01-01

    A 31-year-old female was in the 13th week of pregnancy when an abdominal ultrasound examination revealed a large retroperitoneal tumour. Magnetic resonance imaging was carried out and the imaging described a 10-cm mass in diameter extending from the right kidney. Given that the patient was in her first trimester and that there was a suspicion of malignancy, further surgical exploration of the tumour was warranted. During the operation, the tumour was removed, but nephrectomy was not necessary. Histologic analysis of the resected tumour showed a mucinous cystic adenoma, and no signs of malignancy were present. Following the surgery, the pregnancy was otherwise uneventful and further complications did not occur. This case illustrates that surgery is recommended in patients with a retroperitoneal tumour early during a pregnancy, when a malignancy cannot be excluded.

  13. Large benign retroperitoneal tumour in pregnancy

    PubMed Central

    Berczi, Csaba; Osvath, Peter; Flasko, Tibor

    2015-01-01

    A 31-year-old female was in the 13th week of pregnancy when an abdominal ultrasound examination revealed a large retroperitoneal tumour. Magnetic resonance imaging was carried out and the imaging described a 10-cm mass in diameter extending from the right kidney. Given that the patient was in her first trimester and that there was a suspicion of malignancy, further surgical exploration of the tumour was warranted. During the operation, the tumour was removed, but nephrectomy was not necessary. Histologic analysis of the resected tumour showed a mucinous cystic adenoma, and no signs of malignancy were present. Following the surgery, the pregnancy was otherwise uneventful and further complications did not occur. This case illustrates that surgery is recommended in patients with a retroperitoneal tumour early during a pregnancy, when a malignancy cannot be excluded. PMID:26609332

  14. Expression of the EWS/FLI-1 oncogene in murine primary bone-derived cells Results in EWS/FLI-1-dependent, ewing sarcoma-like tumors.

    PubMed

    Castillero-Trejo, Yeny; Eliazer, Susan; Xiang, Lilin; Richardson, James A; Ilaria, Robert L

    2005-10-01

    Ewing sarcoma is the second most common malignant pediatric bone tumor. Over 80% of Ewing sarcoma contain the oncogene EWS/FLI-1, which encodes the EWS/FLI-1 oncoprotein, a hybrid transcription factor comprised of NH2-terminal sequences from the RNA-binding protein EWS and the DNA-binding and COOH-terminal regions of the Ets transcription factor FLI-1. Although numerous genes are dysregulated by EWS/FLI-1, advances in Ewing sarcoma cancer biology have been hindered by the lack of an animal model because of EWS/FLI-1-mediated cytotoxicity. In this study, we have developed conditions for the isolation and propagation of murine primary bone-derived cells (mPBDC) that stably express EWS/FLI-1. Early-passage EWS/FLI-1 mPBDCs were immortalized in culture but inefficient at tumor induction, whereas later-passage cells formed sarcomatous tumors in immunocompetent syngeneic mice. Murine EWS/FLI-1 tumors contained morphologically primitive cells that lacked definitive lineage markers. Molecular characterization of murine EWS/FLI-1 tumors revealed that some but not all had acquired a novel, clonal in-frame p53 mutation associated with a constitutive loss of p21 expression. Despite indications that secondary events facilitated EWS/FLI-1 mPBDC tumorigenesis, cells remained highly dependent on EWS/FLI-1 for efficient transformation in clonogenic assays. This Ewing sarcoma animal model will be a useful tool for dissecting the molecular pathogenesis of Ewing sarcoma and provides rationale for the broader use of organ-specific progenitor cell populations for the study of human sarcoma.

  15. Experience with cardiopulmonary bypass and deep hypothermic circulatory arrest in the management of retroperitoneal tumors with large vena caval thrombi.

    PubMed Central

    Novick, A C; Kaye, M C; Cosgrove, D M; Angermeier, K; Pontes, J E; Montie, J E; Streem, S B; Klein, E; Stewart, R; Goormastic, M

    1990-01-01

    From June 1984 to September 1989, 43 patients with large vena caval tumor thrombi from retroperitoneal malignancies underwent surgical treatment with cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA). The primary malignancies were renal cell carcinoma (RCC) (n = 39), renal pelvic transitional cell carcinoma (n = 1), adrenal pheochromocytoma (n = 1), and renal (n = 1) or retroperitoneal (n = 1) sarcoma. The level of the caval thrombus was either suprahepatic (n = 27), intrahepatic (n = 14), or subhepatic (n = 2). In all cases the primary tumor and caval thrombus were completely removed. Concomitant procedures included coronary artery bypass grafting (n = 5), pulmonary resection (n = 2), and hepatic lobectomy (n = 1). The time of circulatory arrest ranged from 10 to 44 minutes (mean, 23.5 minutes). There were two operative deaths (4.7%), neither of them due to to the use of DHCA. Major postoperative complications occurred in 13 patients (30.2%). There were no ischemic or neurologic complications and no cases of perioperative tumor embolization. The median postoperative hospital stay was 9 days. Twenty-two patients (51%) are alive and enjoying a good quality of life. The 3-year patient survival rates in patients with localized (n = 24) versus metastatic (n = 15) RCC are 63.9% and 10.9%, respectively (p = 0.02). We conclude that CPB with DHCA facilities excision of retroperitoneal malignancies with large caval thrombi and provides the potential for cure with low morbidity and mortality rates. PMID:2222013

  16. [Pulmonary artery intimal sarcoma].

    PubMed

    Bourry, N; Chabrot, P; Jeannin, G; Filaire, M; Charpy, C; Bay, J O; Kemeny, J L; Caillaud, D; Escande, G; Boyer, L

    2008-02-01

    Pulmonary artery sarcoma is a rare tumor. We present a case of intimal sarcoma arising from right pulmonary artery and left lower pulmonary vein observed in a 44-year-old man with a non-productive cough. Computed tomographic scans and magnetic resonance imaging showing filling defect enhancement contributed early, suggesting the diagnosis of primary vascular tumor, hypothesis confirmed by pathologist findings.

  17. Pulmonary artery sarcoma mimicking a pulmonary embolism.

    PubMed

    Sandhu, A; Yates, T J; Kuriakose, P

    2008-01-01

    Sarcomas involving the lung are a rare occurrence, often a result of metastatic disease from primary malignancies involving the skin, liver, breast or heart. Primary pulmonary artery sarcomas are rarer still, with limited cases reported world-wide and consequently data regarding treatment modalities are sparse and largely experimental. These tumors are often mistaken for a pulmonary embolism and seemingly supported by radiological findings. Patients will often present without symptom resolution despite therapeutic anticoagulation. The following case illustrates how a soft tissue sarcoma of the pulmonary artery can mimic a pulmonary embolism, thus, resulting in both a diagnostic and therapeutic dilemma. A positron emission tomography scan was an invaluable tool in this case, showing increased radiotracer uptake and placing neoplasm at the top of the differential diagnosis. This ultimately led to a biopsy that was vimentin positive, cytokeratin negative and CD117 negative, thus consistent with soft tissue sarcoma.

  18. Hemorrhagic, calcified, and ossified benign retroperitoneal schwannoma

    PubMed Central

    Xu, Shao-Yan; Sun, Ke; Xie, Hai-Yang; Zhou, Lin; Zheng, Shu-Sen; Wang, Wei-Lin

    2016-01-01

    Abstract Background: Schwannomas are mesenchymal tumors arising from the neural sheaths of peripheral nerves. They can almost develop in any part of the body, while head, neck and extremities are the most common sites. Occurrence in the retroperitoneum is rare. Schwannomas can show secondary degenerative changes including cyst formation, hyalinization, hemorrhage, and calcification, whereas the ossified retroperitoneal schwannoma was only reported in a malignant one. Case summary: We first present a benign ossified retroperitoneal schwannoma in a 61-year-old female. The mass was found by a routine health examination. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a well-defined mass in the area among duodenum, right liver, and kidney. Definitive preoperative diagnosis of the mass was difficult. By laparotomy, the mass was found in the retroperitoneum. We completely removed the tumor and gross specimen showed a mass with a capsule and 6 × 6 × 4.8 cm in size. Microscopic examination showed the tumor is composed of spindle-shaped cells with degenerative changes of hemorrhage, calcification, and ossification. Immunohistochemically, S-100 protein was strongly positive. Finally, the mass was diagnosed as a hemorrhagic, calcified, and ossified benign schwannoma in the retroperitoneum. The patient was followed up for a period of 21 months, during which she was well with no evidence of recurrence. Conclusion: We report the first case of a benign retroperitoneal schwannoma with secondary degenerative changes including hemorrhage, calcification, and ossification. Precise preoperative diagnosis of the tumor is challenging even with multiple preoperative imaging modalities. After complete resection, patients with benign retroperitoneal schwanommas generally have good prognosis. PMID:27472709

  19. [Diagnostic-therapeutic approach for retroperitoneal tumors].

    PubMed

    Cariati, A

    1993-12-01

    After a careful review of the Literature, diagnostic and therapeutic strategies for Primary Retroperitoneal Tumours (PRT) are reported. The Author analyzes the experience of the Institute of Clinica Chirurgica "R" (Chief: Prof. E. Tosatti) as well as that of Anatomia Chirurgica (Chief: Prof. E. Cariati),--University of Genoa--in the management of PRT, stressing the importance of preoperative staging for a correct surgical approach.

  20. Retroperitoneal fibromatosis presenting as a presacral mass

    PubMed Central

    Choi, Seok Jin; Jeon, Ung Bae; Choo, Kie Seok

    2014-01-01

    Aggressive fibromatosis arising from the retroperitoneum is extremely rare. It may occur in association with previous trauma, abdominal surgery, drugs, Gardner’s syndrome, or familial adenomatous polyposis. We report a case of retroperitoneal fibromatosis presenting as a presacral mass with an infiltrating nature, relatively intense enhancement on enhanced computed tomography scanning, and low-signal intensity on T2-weighted magnetic resonance imaging in a patient with no significant medical or surgical history. PMID:24778806

  1. Haemoperitoneurn Secondary to Rupture of Retroperitoneal Variceal

    PubMed Central

    Molina-Perez, M.; Rodriguez-Moreno, F.; Perez-Palma, J.; Santolaria-Fernandez, F.; Essardas-Daryanani, H.; Martinez-Riera, A.

    1997-01-01

    A 45-year-old alcoholic male patient presented with hypovolemic shock and intense anemia (Hemoglobin 04.7 g/dl), and was operated on. A bleeding retroperitoneal varix located near the right colon was responsible for the clinical picture and was sutured. After operation the patient developed haemodynamic instability and pneumonia a situation which was reverted with intensive medical therapy. The patient is now doing well. PMID:9298389

  2. Spontaneous retroperitoneal hemorrhage caused by segmental arterial mediolysis.

    PubMed

    Phillips, Courtney K; Lepor, Herbert

    2006-01-01

    Spontaneous retroperitoneal hemorrhage is a rare clinical entity; signs and symptoms include pain, hematuria, and shock. Spontaneous retroperitoneal hemorrhage can be caused by tumors, such as renal cell carcinoma and angiomyolipoma; polyarteritis nodosa; and nephritis. The least common cause is segmental arterial mediolysis. Although computed tomography is used for the diagnosis of spontaneous retroperitoneal hemorrhage, it can miss segmental arterial mediolysis as the cause of the hemorrhage. The diagnosis of segmental arterial mediolysis as a cause of spontaneous retroperitoneal hemorrhage requires angiography, with pathologic confirmation for a definitive diagnosis.

  3. Spontaneous Retroperitoneal Hemorrhage Caused by Segmental Arterial Mediolysis

    PubMed Central

    Phillips, Courtney K; Lepor, Herbert

    2006-01-01

    Spontaneous retroperitoneal hemorrhage is a rare clinical entity; signs and symptoms include pain, hematuria, and shock. Spontaneous retroperitoneal hemorrhage can be caused by tumors, such as renal cell carcinoma and angiomyolipoma; polyarteritis nodosa; and nephritis. The least common cause is segmental arterial mediolysis. Although computed tomography is used for the diagnosis of spontaneous retroperitoneal hemorrhage, it can miss segmental arterial mediolysis as the cause of the hemorrhage. The diagnosis of segmental arterial mediolysis as a cause of spontaneous retroperitoneal hemorrhage requires angiography, with pathologic confirmation for a definitive diagnosis. PMID:16985559

  4. Pulmonary artery sarcoma.

    PubMed

    Blackmon, Shanda H; Reardon, Michael J

    2010-01-01

    Primary heart tumors are rare, and malignant primary heart tumors are only a small subset of these. Most primary malignant tumors are sarcomas arising from the cells of the structural elements of the heart such as blood vessels, muscle, connective tissue, fat and even bone. Unlike most malignancies, where cell type often dictates treatment choices and prognosis and is used for classification, the histology in primary cardiac sarcoma plays little role in determining therapeutic options or prognosis. We have found that anatomic location within the heart is the major determining factor in clinical presentation, treatment options and prognosis in cardiac sarcoma. Therefore, we accordingly classify primary cardiac sarcomas into right heart sarcomas, left heart sarcomas and pulmonary artery (PA) sarcomas. Since the first autopsy report of a primary PA sarcoma in 1923, there have been fewer than 250 cases reported in the English literature. Most of these reports have been single autopsy or case reports, and patient prognosis has generally been dismal. Since few institutions and even fewer individual physicians acquire much exposure to this disease, the diagnostic and treatment approaches have remained unresolved. Our cardiac sarcoma group working at the Methodist DeBakey Heart & Vascular Center and the MD Anderson Cancer Center has undertaken a systematic study of this disease, and operated on 9 patients using a radical resection with curative intent and multimodality approach. Based on this work, we have suggested a diagnostic strategy, treatment approach and staging system for primary PA sarcoma. A substantial improvement in patient survival over historical controls has also been demonstrated and will be discussed in this review.

  5. Experience with laparoscopy-assisted retroperitoneal pyeloplasty in children.

    PubMed

    Abraham, Mohan K; Nasir, Abdul Rasheed A; Bindu, S; Ramakrishnan, P; Kedari, Prashant M; Unnithan, Gopidas R; Damisetti, Kalyan Ravi Prasad

    2009-07-01

    To describe a laparoscopy-assisted retroperitoneal pyeloplasty (LARP) and results of initial experience. Port placement used by Farhat in retroperitoneal-assisted laparoscopic pyeloplasty was modified for better cosmetic results. Surgery was done using 2-cm incision for 5-mm camera port and two 3-mm working ports. Dissection was done anterior to the kidney. The ureteropelvic junction was brought out through the 2-cm trocar site and the pyeloplasty was performed extracorporeally. Between January 2004 and February 2008, a total of 39 kidneys in 38 children with mean age of 4.1 months underwent LARP. The operative time, hospital stay, functional outcome and follow-up renogram studies were reviewed. The mean operative time was 147 min. 2-cm incision was extended in one patient with malrotated kidney. There was improvement in function in 37 (95%) with no failure. The mean split renal function, preoperative and at follow-up were 35.7 and 44.2%, respectively (P = 0.000). The mean glomerular filtration rate (ml/min), preoperative and at follow-up were 27.4 and 39.1%, respectively (P = 0.000). Mean follow-up period was 24 months. LARP is safe in treating UPJ obstruction in infants. It is recommended especially in small babies where laparoscopic pyeloplasty is difficult.

  6. 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)

  7. Neurogenic sarcomas of the neck in neurofibromatosis.

    PubMed

    Martin, G; Kleinsasser, O

    1981-01-01

    Based on two observations and a review of the literature, the pathological and clinical findings in sarcomas of the neck in patients with neurofibromatosis are described. Histologically these neurogenic tumours show a manifold picture; in addition to spindle-cell sarcomas pleomorphic structures are to be found, which can be similar to rhabdomyo-, lipo-, chondro-, angio-, or osteogenic sarcomas so that a histological diagnosis of a neurogenic sarcoma cannot always be made without clinical details. Up to the present surgical treatment is preferred; the value of cytostatic therapy and irradiation is controversial. The results of treating these tumours are unsatisfactory. Of 29 cases reported in the literature, only two could be found in which the patient survived without a recurrence for more than five years.

  8. [Results of the randomised phase III study of the German Testicular Cancer Study Group. Retroperitoneal lymphadenectomy versus one cycle BEP as adjuvant therapy for non-seminomatous testicular tumours in clinical stage I].

    PubMed

    Hartmann, M; Siener, R; Krege, S; Schmelz, H; Dieckmann, K-P; Heidenreich, A; Kwasny, P; Pechoel, M; Lehmann, J; Kliesch, S; Köhrmann, K-U; Fimmers, R; Weissbach, L; Loy, V; Wittekind, C; Albers, P

    2009-05-01

    As 30% of non-seminomas in clinical stage I will progress during active surveillance, alternative adjuvant strategies of 2 cycles of bleomycin, etoposid, cisplatin (BEP) or nerve sparing retroperitoneal lymphadenectomy (RPLND) can be offered. The risk of relapse is reduced to 2% and 10%, respectively. Without prognostic markers and with lowered toxicity it is postulated that only one cycle of BEP could significantly reduce the recurrence rate in comparison to RPLND. Between 1996 and 2005, 382 patients were randomly assigned to receive either RPLND (n=191) or 1 cycle of BEP (n=191). In accordance with the protocol, 174 patients were treated with 1 cycle of BEP and 173 underwent RPLND. The primary study end-point was a reduction of recurrence from 10% after RPLND to a maximum of 3% after 1 cycle of BEP. After a mean follow-up of 4.7 years, there were 2 and 13 recurrences in the according-to-protocol population with chemotherapy and surgery, respectively. The difference between chemotherapy (1.15%) and surgery (7.5%) was statistically significant (p=0.0033). The tumor-specific survival was 100%. This largest randomized trial investigating treatment strategies in clinical stage I non-seminomas (AUO AH 01/94) showed the superiority of one cycle BEP over RPLND. The data obtained represent the basis for a reduced chemotherapy.

  9. The development of a retroperitoneal dissection model.

    PubMed

    Yousuf, Aisha A; Frecker, Helena; Satkunaratnam, Abheha; Shore, Eliane M

    2017-10-01

    Knowledge of ureteric anatomy is essential for ureteric injury prevention in laparoscopic gynecologic surgery. Rates of injury increase with limited surgical experience and reduced surgical volume. Currently, there are no low-fidelity or high-fidelity simulation models for teaching and practicing ureteric dissection. Our goal was to design a laparoscopic simulation model for retroperitoneal anatomy with high face validity that is low-cost and easily reproducible. A low-fidelity 3-dimensional simulation model was developed that represents key anatomic structures encountered during retroperitoneal dissection and ureteric identification. Materials, construction steps, and costs were determined. The models were trialed by expert laparoscopic surgeons. Demographic information that included age, gender, surgical experience, and complex laparoscopic case volumes was collected. Face validity was assessed with a 5-item Likert-scale. The total cost of 1 model ranged from $65 to $75. The majority of the materials that were used were reusable, except for 2 components that cost <$1 per use. Seven expert surgeons participated in the study, all of whom were fellowship-trained minimally invasive gynecologic surgeons or currently enrolled in this type of fellowship program. Participants agreed or strongly agreed that the model resembled the texture of the ureter, vessels, and peritoneal layer (n=6; 86%), approximated the correct anatomic course of the ureter (n=7; 100%), and closely approximated live surgery (n=5; 71%). They also agreed or strongly agreed that the model would be useful for teaching laparoscopic retroperitoneal dissection (n=7; 100%), for assessing a learner's ability before performing in the operating room (n=6; 86%), was low-cost (n=7; 100%), and was easily reproducible (n=6; 86%). This unique model fills a gap in laparoscopic simulation training. No other low- or high-fidelity models for laparoscopic retroperitoneal ureteric dissection have been identified in the

  10. Clinical outcomes of uterine sarcomas: results from 14 years worth of experience in the Kinki district in Japan (1990-2003).

    PubMed

    Kokawa, K; Nishiyama, K; Ikeuchi, M; Ihara, Y; Akamatsu, N; Enomoto, T; Ishiko, O; Motoyama, S; Fujii, S; Umesaki, N

    2006-01-01

    To review clinical outcomes and therapeutic varieties, we were invited to submit data from the patients who were treated for uterine sarcomas in Japan from 1990 to 2003. Uterine sarcomas were defined as leiomyosarcoma (LMS), endometrial stromal sarcoma (ESS), and carcinosarcoma (CS). Of a total of 97 patients, 36 (37.1%) were diagnosed with LMS of the uterine corpus, 15 (15.5%) with ESS, 46 (47.4%) with CS. Median age at diagnosis was 59 (21-85) years. Clinical stages based on FIGO were 41 (42.3%) with stage I disease, 6 (6.2%) with staged II, 34 (35.1%) with stage III, and 16 (16.5%) with stage IV. The median follow-up period for all patients was 13 (1-108) months and median disease-free period was 9 (0-96) months. The 1-year survival rate and disease-free survival (DFS) rate were calculated in patients with all sarcomas (overall survival [OAS], 61.3%; DFS, 46.6%). Statistical analysis showed that younger age (less than 50 years), early stage (stages I and II), and surgical procedure (extended hysterectomy [EH] and radical hysterectomy [RH]) were associated with significantly better OAS. Histologic types did not affect the survival period. In conclusion, aggressive surgery including EH or RH at the time of initial operation offers the possibility of prolonged survival.

  11. 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.

  12. Pulmonary Artery Sarcoma

    PubMed Central

    Shomaf, Maha; Obeidat, Nathir; Najjar, Saleh

    2014-01-01

    Pulmonary artery sarcomas (PAS) are extremely rare sarcomas of uncertain histogenesis that often mimic pulmonary thromboemboli. This is a report of a 60-year-old female patient who presented with recurrent chest pain and cough. The patient was first diagnosed with pulmonary embolism but she did not improve on anticoagulant therapy. Follow-up imaging studies revealed a mass in the left hilar region extending into the pulmonary trunk and branches of the left pulmonary artery. The tru-cut biopsy revealed an undifferentiated sarcoma. The patient died 10 months after her initial presentation. PMID:26425600

  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. Canine intrathoracic sarcoma with ultrastructural characteristics of human synovial sarcoma - case report.

    PubMed

    Lovell, Ser; Burchell, R K; Roady, P J; Fredrickson, R L; Gal, A

    2017-08-16

    Canine joint sarcomas, designated synovial sarcomas, are uncommon malignant mesenchymal neoplasms that occur in the large joints of the extremities of middle-aged, large-breed dogs. We report the diagnosis of an intrathoracic sarcoma with ultrastructural characteristics reminiscent of human synovial sarcoma in a dog. A 7-year-old female spayed Tibetan terrier crossbred dog was presented for acute severe labored breathing and diagnosed with an intrathoracic neoplastic mass. The neoplasm resulted in the accumulation of substantial amounts of viscous pleural fluid that led to dyspnea. The neoplastic mass consisted of interweaving bundles of large pleomorphic mesenchymal cells, supported by an alcian blue positive myxomatous matrix. The neoplastic cells were immunohistochemically negative for cytokeratin and CD18. Transmission electron microscopy indicated that the neoplastic cells had desmosome junctions, short microvilli-like structures and ample amounts of rough endoplasmic reticulum resembling type B-like synoviocytes and synovial sarcoma as reported in people. Despite complete surgical excision of the neoplastic mass, clinical signs recurred after a month and led to the euthanasia of the dog. Currently, there are no immunohistochemical markers specific for synovial sarcoma. Canine neoplasms with transmission electron microscopy characteristics resembling type B-like synoviocytes should be considered similar to the human sarcomas that carry the specific translocations between chromosomes X and 18.

  15. Current Immunotherapies for Sarcoma: Clinical Trials and Rationale

    PubMed Central

    Mitsis, Demytra; Francescutti, Valerie

    2016-01-01

    Sarcoma tumors are rare and heterogeneous, yet they possess many characteristics that may facilitate immunotherapeutic responses. Both active strategies including vaccines and passive strategies involving cellular adoptive immunotherapy have been applied clinically. Results of these clinical trials indicate a distinct benefit for select patients. The recent breakthrough of immunologic checkpoint inhibition is being rapidly introduced to a variety of tumor types including sarcoma. It is anticipated that these emerging immunotherapies will exhibit clinical efficacy for a variety of sarcomas. The increasing ability to tailor immunologic therapies to sarcoma patients will undoubtedly generate further enthusiasm and clinical research for this treatment modality. PMID:27703409

  16. Survival by Stage of Soft Tissue Sarcoma

    MedlinePlus

    ... Detection, Diagnosis, and Staging Survival by Stage of Soft Tissue Sarcoma Survival rates are often used by ... Your Doctor About Soft Tissue Sarcomas? More In Soft Tissue Sarcoma About Soft Tissue Sarcoma Causes, Risk ...

  17. Retroperitoneal Tumors in the Pelvis: A Diagnostic Challenge in Gynecology

    PubMed Central

    Wee-Stekly, Wei-Wei; Mueller, Michael David

    2014-01-01

    Retroperitoneal tumors can pose a diagnostic and therapeutic challenge to gynecologists because of their rarity, late presentation, and complex anatomical location in the retroperitoneum. This article reviews the diagnosis and management of retroperitoneal tumors in the pelvis, and highlights the potential pitfalls that may be faced by gynecologists. PMID:25593973

  18. 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.

  19. Pancreatic liposarcoma: case report with review of retroperitoneal liposarcomas.

    PubMed

    Elliott, T E; Albertazzi, V J; Danto, L A

    1980-04-01

    A case report of a large pancreatic liposarcoma is presented showing a five-year survival with aggressive surgical excision as the only treatment. The medical literature of the subject is reviewed. This is the only reported case of retroperitoneal liposarcoma which is limited to the pancreas. A discussion of the current types of treatment for retroperitoneal liposarcomas is included.

  20. Cluster analysis of immunohistochemical profiles in synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma.

    PubMed

    Olsen, Stephen H; Thomas, Dafydd G; Lucas, David R

    2006-05-01

    As a result of overlapping morphologic and immunohistochemical features, it can be difficult to distinguish synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma/primitive neuroectodermal tumor in core biopsies. To analyze and compare immunohistochemical profiles, we stained tissue microarrays of 23 synovial sarcomas, 23 malignant peripheral nerve sheath tumors, and 27 Ewing sarcomas with 22 antibodies potentially useful in the differential diagnosis, and analyzed the data with cluster analysis. Stain intensity was scored as none, weak, or strong. For CD99, tumors with membranous accentuation were independently categorized. Cluster analysis sorted five groups, with like tumors clustering together. Synovial sarcoma clustered into two groups: one cytokeratin and EMA positive (n = 11), the other mostly cytokeratin negative, EMA positive, bcl-2 positive and mostly CD56 positive (n = 9). Malignant peripheral nerve sheath tumor clustered into two groups: one S100 positive, with nestin and NGFR positivity in most (n = 10), the other mostly S100 negative, and variably but mostly weakly positive for nestin and NGFR (n = 11). Ewing sarcomas clustered into a single group driven by membranous CD99 staining. Thirteen cases failed to cluster (outliers), while three Ewing sarcomas clustered into groups of other tumor types. Paired antibodies for each tumor type determined by visual assessment of cluster analysis data and statistical calculations of specificity, sensitivity, and predictive values showed that EMA/CK7 for synovial sarcoma, nestin/S100 for malignant peripheral nerve sheath tumor, and membranous CD99/Fli-1 for Ewing sarcoma yielded high specificity and positive predictive values. Cluster analysis also highlighted aberrant staining reactions and diagnostic pitfalls in these tumors. Hierarchical cluster analysis is an effective method for analyzing high-volume immunohistochemical data.

  1. Evaluation of monophosphoryl lipid A as an immune adjuvant for photodynamic therapy in a rat sarcoma model: preliminary results

    NASA Astrophysics Data System (ADS)

    Lucroy, Michael D.; Edwards, Benjamin F.; Griffey, Stephen M.; Madewell, Bruce R.

    1999-06-01

    Photodynamic therapy (PDT) is a treatment option for several forms of human cancer, and like traditional chemotherapy and ionizing radiation therapy, PDT alone is not curative for some cases. Recent efforts have aimed at developing strategies for adjuvant therapy for PDT. Given the nature of PDT-mediated cell damage, immunotherapy is a promising adjuvant for long-term control of solid tumors. A candidate immune stimulant for use with PDT is monophosphoryl lipid A (MLA), a non-toxic fraction of the endotoxin molecule. The hypothesis is that adjuvant MLA immunotherapy with PDT will improve local tumor control and prevent growth of subsequently implanted tumor cells when compared to PDT alone. To date, no significant differences in circulating leukocyte populations or tumor infiltrating lymphocyte populations have been identified in 9L tumor-bearing F344 rats after systemic administrations of MLA. Likewise, no significant difference has been identified in local tumor control following PDT of 9L tumors with or without adjuvant MLA. Further results are pending.

  2. Retroperitoneal unicentric Castleman's disease: A case report.

    PubMed

    Abdessayed, Nihed; Bdioui, Ahlem; Ammar, Houssem; Gupta, Rahul; Mhamdi, Nozha; Guerfela, Marwa; Mokni, Moncef

    2017-01-01

    Castleman's disease (CD) is an angio-follicular lymph node hyperplasia presenting as a localized or a systemic disease masquerading malignancy. The most common sites of CD are mediastinum, neck, axilla and pelvis. Unicentric CD in the peripancreatic region is very rare. We report a case of the 34-year-old lady presenting with epigastric pain for 3 months. Abdominal imaging revealed a retroperitoneal mass arising from the pancreas suspected to be neuroendocrine tumor. Tumor markers were not elevated. Complete surgical excision was performed and patient had uneventful recovery. Pathologic findings demonstrated localized hyaline-vascular type of Castleman's disease. CD is a very rare cause for development of retroperitoneal mass. It is more frequent in young adults without predilection of sex. It can occur anywhere along the lymphoid chain. Abdominal and retroperitoneal locations usually present with symptoms due to the mass effect on adjacent organs. CD appears as a homogeneously hypoechoic mass on ultrasound and non-specific enhancing homogeneous mass with micro calcifications on computed tomography. Histologically, the hyaline vascular type demonstrates a follicular and inter-follicular capillary proliferation with peri-vascular hyalinization, with expansion of the mantle zones by a mixed inflammatory infiltrate of numerous small lymphocytes and plasma cells. The standard therapy of localized form is en bloc surgical excision as performed in our case. Unicentric CD in the peripancreatic region is difficult to differentiate from pancreatic neoplasm preoperatively. However, preoperative biopsy in cases of high clinical suspicion can help in avoiding extensive surgery for this benign disease. Copyright © 2017 The Authors. Published by Elsevier Ltd.. All rights reserved.

  3. Retroperitoneal teratoma in a skunk (Mephitis mephitis).

    PubMed

    Munday, John S; Fairchild, Steve E; Brown, Cathy A

    2004-09-01

    A 4-yr-old striped skunk (Mephitis mephitis) developed an 11- x 7- x 7-cm retroperitoneal mass caudal to the left kidney. The mass was surgically excised and, on the basis of histology and immunohistochemistry, diagnosed as a benign teratoma. Six months later, a second histologically similar neoplasm was excised from the same location and was considered to represent local tumor recurrence. The skunk died 16 mo later without recurrence. Complete excision of the tumor appeared curative in this case and supported the benign diagnosis. An extragonadal teratoma should be considered as a differential diagnosis for an abdominal mass in a skunk.

  4. Immune Therapy for Sarcomas.

    PubMed

    Anderson, Peter M

    2017-01-01

    Absolute lymphocyte count (ALC) recovery rapidly occurring at 14 days after start of chemotherapy for osteosarcoma and Ewing sarcoma is a good prognostic factor. Conversely, lymphopenia is associated with significantly decreased sarcoma survival. Clearly, the immune system can contribute towards better survival from sarcoma. This chapter will describe treatment and host factors that influence immune function and how effective local control and systemic interventions of sarcoma therapy can cause inflammation and/or immune suppression but are currently the standard of care. Preclinical and clinical efforts to enhance immune function against sarcoma will be reviewed. Interventions to enhance immune function against sarcoma have included regional therapy (surgery, cryoablation, radiofrequency ablation, electroporation, and radiotherapy), cytokines, macrophage activators (mifamurtide), vaccines, natural killer (NK) cells, T cell receptor (TCR) and chimeric antigen receptor (CAR) T cells, and efforts to decrease inflammation. The latter is particularly important because of new knowledge about factors influencing expression of checkpoint inhibitory molecules, PD1 and CTLA-4, in the tumor microenvironment. Since these molecules can now be blocked using anti-PD1 and anti-CTLA-4 antibodies, how to translate this knowledge into more effective immune therapies in the future as well as how to augment effectiveness of current interventions (e.g., radiotherapy) is a challenge. Barriers to implementing this knowledge include cost of agents that release immune checkpoint blockade and coordination of cost-effective outpatient sarcoma treatment. Information on how to research clinical trial eligibility criteria and how to access current immune therapy trials against sarcoma are shared, too.

  5. Bronchopulmonary Kaposi's sarcoma.

    PubMed

    Bashar, Nada; Innes, Nicholas; Orrell, Julian

    2015-01-01

    Kaposi's sarcoma (KS) is a highly vascular tumour, which was first described by the Hungarian dermatologist Moritz Kaposi Kohn before the discovery of the human immunodeficiency virus (HIV). Historically, KS has been linked to immunosuppression or to elderly male patients, especially in relation to diffuse cutaneous KS. We describe a case of Bronchopulmonary Kaposi's sarcoma in a patient with AIDS who was successfully treated with HAART and Liposomal Doxorubicin chemotherapy.

  6. Bronchopulmonary Kaposi's sarcoma

    PubMed Central

    Bashar, Nada; Innes, Nicholas; Orrell, Julian

    2015-01-01

    Kaposi's sarcoma (KS) is a highly vascular tumour, which was first described by the Hungarian dermatologist Moritz Kaposi Kohn before the discovery of the human immunodeficiency virus (HIV). Historically, KS has been linked to immunosuppression or to elderly male patients, especially in relation to diffuse cutaneous KS. We describe a case of Bronchopulmonary Kaposi's sarcoma in a patient with AIDS who was successfully treated with HAART and Liposomal Doxorubicin chemotherapy. PMID:26236600

  7. Retroperitoneal Laparoendoscopic Single-Site Ureterolithotomy: A Comparison with Conventional Laparoscopic Surgery

    PubMed Central

    Liu, Xiaopeng; Huang, Huaiqiu; Wu, Jieying; Huang, Wentao; Cai, Songwang; Li, Xiaojuan; Ye, Chunwei; Zhu, Baoyi; Cai, Yi; Gao, Xin

    2012-01-01

    Abstract Background and Purpose Laparoendoscopic single-site (LESS) surgery through the retroperitoneal approach has been seldom reported. We aimed to compare the feasibility and outcomes of LESS and conventional laparoscopic surgery via the retroperitoneal approach in the management of large, impacted ureteral stones. Patients and Methods From June 2010 to May 2011, LESS ureterolithotomy through the retroperitoneal approach was performed in 10 patients (the LESS group). Another 15 patients who underwent conventional retroperitoneal laparoscopic ureterolithotomy (the conventional laparoscopic group) by the same surgeon were involved and compared. The operative time, complications, and surgical outcomes were evaluated. Results All the operations were completed successfully, without conversion to conventional laparoscopic or open surgeries. The operative time of the LESS group and of the conventional laparoscopic group were 132.7±16.3 and 128.1±20.1 minutes, respectively (P=0.782). The estimated blood loss were 30.7±5.9 vs 28.0±4.5 mL (P=0.620). Duration of analgesia postoperatively was 2.0±0.8 vs 3.5±0.5 days (P=0.005). All targeted stones were successfully extracted without major complications. Postoperative urine leakage was noted in one patient in each group. Cosmetic results were superior in the LESS group according to both the study nurse's and the patients' assessments (8.5 vs 5.3; P=0.012, and 8.3 vs 5.6; P=0.025, respectively). All patients showed no obstructions or stricture formations on postoperative follow-up. Conclusions In experienced hands, LESS for ureterolithotomy through the retroperitoneal approach is feasible and can acquire outcomes equal to those of conventional multiport laparoscopic surgery. Prospective long-term follow-up studies with a larger number of patients are needed to further evaluate its benefits. PMID:22103789

  8. Primary CIC-DUX4 round cell sarcoma of the kidney: A treatment-refractory tumor with poor outcome.

    PubMed

    Bergerat, Sébastien; Barthelemy, Philippe; Mouracade, Pascal; Lang, Hervé; Saussine, Christian; Lindner, Véronique; Jacqmin, Didier

    2017-02-01

    The CIC-DUX4 sarcoma is a subset of the undifferentiated small round cell sarcoma family, presently recognized as a new clinicopathological entity. It is a rare and highly aggressive tumor usually arising in the soft parts of the limbs and the trunk. Only a very few cases of primitive visceral CIC-DUX4 have been hitherto described. We report the case of a 29 year-old male patient with a primary CIC-DUX4 sarcoma of the kidney with lung metastasis. The outcome of the disease was rapidly unfavorable. Despite radical nephrectomy, the patient experienced an early local retroperitoneal recurrence associated with lung and liver metastases. The tumor did not respond to four successive lines of chemotherapy nor to palliative radiotherapy. Due to partial morphologic and immunohistochemical overlap with Ewing sarcoma, CIC-DUX4 positive tumors have generally been considered as Ewing-like sarcomas and managed similarly. However, this tumor shows a high propensity to metastasize and is much less sensitive to chemotherapy than Ewing sarcomas. The management of this type of very aggressive sarcoma needs to be defined by comprehensive biological and clinical studies. Copyright © 2016 Elsevier GmbH. All rights reserved.

  9. Complete response of endemic Kaposi sarcoma lesions with high-dose-rate brachytherapy: treatment method, results, and toxicity using skin surface applicators.

    PubMed

    Kasper, Michael E; Richter, Sam; Warren, Nicholas; Benda, Rashmi; Shang, Charles; Ouhib, Zoubir

    2013-01-01

    To analyze the clinical outcome of Kaposi sarcoma skin lesions treated with high-dose-rate (HDR) brachytherapy in patients with a minimum of 2 years of followup. Between February 2006 and July 2008, all patients with Kaposi sarcoma who received (192)Ir HDR brachytherapy using a skin surface applicator were evaluated for clinical response. Responses to treatment and toxicity were scored using standard criteria. Sixteen cases were collected. Treatment was delivered in four to six fractions, over a period of approximately 12 days. The specified dose ranged from 24 to 35Gy. Median followup the lesion was 41.4 months. No lesion was greater than 2cm. All patients had a complete response to treatment, with no evidence of local recurrence or tumor progression. Thirteen lesions developed Grade 1 and two lesions had Grade 2 acute skin reactions. One patient developed late skin changes with telangiectasias and hypopigmentation. HDR brachytherapy treatment seems to be an effective noninvasive option for patients with small cutaneous Kaposi sarcoma lesions, delivering excellent cosmesis and local control in our small series. Fewer fractions over a shorter period used in our group offer patients more convenience compared with other common regimens. Although HDR is being used more frequently for many surface applications, additional clinical studies with larger numbers of patients and longer followup are needed to confirm the general impression that it is an excellent option for many patients. Copyright © 2013 American Brachytherapy Society. Published by Elsevier Inc. All rights reserved.

  10. Postchemotherapy Retroperitoneal Lymph Node Dissection in Patients With Nonseminomatous Testicular Cancer: A Single Center Experiences

    PubMed Central

    Nowroozi, Mohamadreza; Ayati, Mohsen; Arbab, Amir; Jamshidian, Hassan; Ghorbani, Hamidreza; Niroomand, Hassan; Taheri Mahmoodi, Mohsen; Amini, Erfan; Salehi, Sohrab; Hakima, Hamid; Fazeli, Farid; Haghdani, Saeid; Ghadian, Alireza

    2015-01-01

    Background: Testicular cancer accounts for about 1 - 1.5% of all malignancies in men. Radical orchiectomy is curative in 75% of patients with stage I disease, but advance stage with retroperitoneal lymph node involvement needs chemotherapy. All patients who have residual masses ≥ 1 cm after chemotherapy should undergo postchemotherapy retroperitoneal lymph node dissection (PC-RPLND). Objectives: Treatment of advanced nonseminomatous testicular cancer is usually a combination of chemotherapy and surgery. We described our experience about postchemotherapy retroperitoneal lymph node dissection (PC-RPLND) in our center. Patients and Methods: In a retrospective cross-sectional study between 2006 and 2011, patients with a history of postchemotherapy retroperitoneal lymph node dissection (PC-RPLND) in Imam Khomeini hospital were evaluated. All patients had normal postchemotherapy serum tumor markers and primary nonseminomatous cancer. We reviewed retrospectively clinical, pathological, and surgical parameters associated with PC-RPLND in our center. Results: Twenty-one patients underwent bilateral PC-RPLND. Mean age was 26.3 years (ranged 16 - 47). Mean size of retroperitoneal mass after chemotherapy was 7.6 cm. Mean operative time was 198 minutes (120 - 246 minutes). Mean follow-up time was 38.6 months. Pathologic review showed presence of fibrosis/necrosis, viable germ cell tumor and teratoma in 8 (38.1%), 10 (47.6%) and 3 (14.28%) patients, respectively. One patient in postoperative period of surgery and three patients in two first years after surgery were expired. Of 17 alive patients, only two (11.8%) had not retrograde ejaculation. Conclusions: PC-RPLND is one the major operations in the field of urology, which is associated with significant adjunctive surgeries. In appropriate cases, PC-RPLND was associated with good cancer specific survival in tertiary oncology center. PMID:26539420

  11. Olaratumab for soft tissue sarcoma.

    PubMed

    Teyssonneau, Diego; Italiano, Antoine

    2017-08-01

    Soft tissue sarcomas (STS) are rare malignant tumors. Unfortunately, the first-line doxorubicin-based treatment has not been improved since the 1970s. Platelet-derived growth factor (PDGF) receptor alpha (PDGFR-α) and its ligands are co-expressed in many types of cancer, including sarcomas. They are involved in stimulating growth and regulating stromal-derived fibroblasts and angiogenesis. PDGFR-α and its ligand may play an important role in tumorigenesis and be a potential target in the treatment of sarcomas. Olaratumab is a fully human IgG1-type anti-PDGFR-α monoclonal antibody with a high affinity and a low 50% inhibitory concentration (IC50). Areas covered: The authors review the role of olaratumab in the treatment of STS by focusing on the recent, randomized Phase II JDGD trial that challenged patients with unresectable or metastatic STS with doxorubicin in the presence or absence of olaratumab. This trial showed a great improvement in overall survival (OS), with an increase in survival from 14.7 months to 26.5 months for patients in the experimental arm and showed acceptable toxicity. Expert opinion: Results seem promising. However, it must be qualified, as the study includes several uncertainties. These uncertainties should be addressed by the ongoing Phase 3 JGDJ confirmatory trial, for which the final efficacy analysis is expected by 2019.

  12. Retroperitoneal liposarcoma associated with small plaque parapsoriasis

    PubMed Central

    Tartaglia, Francesco; Blasi, Sara; Sgueglia, Monica; Polichetti, Paolo; Tromba, Luciana; Berni, Alberto

    2007-01-01

    Background Extremely rare cases of paraneoplastic syndromes or ectopic production of proteins associated with liposarcoma are reported in literature. Production of Granulocyte-Colony Stimulating Factor, alpha-fetoprotein, paraneoplastic pemphigus and leucocytosis, Acrokeratosis paraneoplastica (Bazex's syndrome) are reported. The present report describes a case of retroperitoneal liposarcoma associated with small plaque parapsoriasis. Our search in the English literature of such a kind of association did not reveal any case reported. Case presentation A 74 year male patient was admitted to our hospital because of the presence of an abdominal mass in right iliac fossa. He also complained of a two-year history of psoriasiform eruptions. The CT scan showed a retroperitoneal pelvic mass. Therefore surgical resection of the tumor was performed. After surgery, the skin eruptions disappeared completely in seven days and so a diagnosis of parapsoriasis syndrome was done. Conclusion Parallel disappearing of skin eruptions after surgery, typical clinical picture and not specific histology of the cutaneous lesions suggest the diagnosis of small plaque parapsoriasis. Therefore we propose to add Small Plaque Parapsoriasis to the list of paraneoplastic syndromes associated to liposarcoma. PMID:17620118

  13. Histiocytic Sarcoma in a Kidney Transplant Patient: A Case Report and Review of the Literature

    PubMed Central

    Pollen, Maressa; El Jamal, Siraj; Lewin, Jack

    2016-01-01

    Objective. Histiocytic sarcoma (HS) is an aggressive neoplasm with only limited number of reported series of cases and rare case reports of occurrence as a posttransplant neoplastic disorder. The etiology and pathogenesis of the disease is unknown and the optimal treatment is still under investigation. We describe an unusual case of HS in a patient with a remote history of kidney transplant. Method and Results. A 54-year-old male with a remote history of renal transplantation under maintenance immunosuppression presented with features of sepsis. CT abdomen revealed multiple heterogeneous masses in bilateral native kidneys and liver and enlarged abdominal and retroperitoneal lymph nodes. Viral serology work-up was negative. Needle core biopsy revealed a highly undifferentiated neoplasm comprised of highly atypical large cells with eosinophilic to vacuolated cytoplasm and hemophagocytosis. Extended panel of immunohistochemistry proved histiocytic lineage for the tumor cells. The patient expired 2 weeks following the diagnosis. Conclusion. Our case along with three previously published case reports raised the possibility of HS as a treatment-related neoplasm or a posttransplantation neoplastic disorder in solid organ transplant recipients. PMID:27795864

  14. HUMAN SARCOMAS IN CULTURE

    PubMed Central

    Giraldo, Gaetano; Beth, Elke; Hirshaut, Yashar; Aoki, Tadao; Old, Lloyd J.; Boyse, Edward A.; Chopra, Harish C.

    1971-01-01

    In a study of human sarcomas maintained in culture for periods up to two years, the following observations were made. The most prominent cell type in serially cultured osteosarcomas was fibroblastic in appearance. After 16–20 wk in culture some lines spontaneously developed foci of altered cells resembling the foci produced in monolayer cultures by oncogenic viruses. The presence of these foci in the sarcoma cultures was transient, and usually they did not reappear; but in one instance they recurred with a characteristic periodicity of several weeks. From one of the sarcoma lines, in which foci appeared after 5 months in culture, two subcultures were established from stored frozen cells and these both exhibited foci after approximately the same lapse of time. The same phenomenon has been seen with another line, suggesting that the time of appearance of foci is characteristic for particular sarcomas. Foci of similar type could sometimes be induced in monolayer cultures of human fibroblasts by filtered medium from cultured sarcomas; this bore no relation to the presence or absence of foci in the sarcoma cultures at the time the filtrate was prepared. Electron microscopy of the spontaneous and induced foci, and of the sarcoma cultures, revealed no demonstrable virus. 12 out of 15 sarcoma cultures contained an antigen (S) demonstrable by indirect immunofluorescence with human sera. It was not present in any of the original sarcoma specimens, nor in any culture lines other than sarcomas. At least 3–4 wk in culture appear to be required for its demonstration. The antigen was cytoplasmic, occurred in only a small proportion of the cells, and was unpredictably variable in its expression, even in the same culture line. It could be induced in monolayer cultures of human fibroblasts by filtrates of medium from sarcoma cultures. As with the foci, the induction of S antigen in indicator cultures was not dependent upon the expression of antigen in the sarcoma line from which

  15. Primary retroperitoneal myxoid/round cell liposarcoma is a nonexisting disease: an immunohistochemical and molecular biological analysis.

    PubMed

    de Vreeze, Ronald S A; de Jong, Daphne; Tielen, Ivon H G; Ruijter, Henrique J; Nederlof, Petra M; Haas, Rick L; van Coevorden, Frits

    2009-02-01

    Almost all primary retroperitoneal liposarcomas can be classified as well-/dedifferentiated liposarcoma. Rarely, however, primary retroperitoneal liposarcoma is classified as myxoid/round cell liposarcoma, based on the presence of myxoid areas and vascular crow's feet pattern, which has resulted in a debate on the classification of liposarcoma in the retroperitoneum. Genetically, myxoid/round cell liposarcoma and well-/dedifferentiated liposarcoma are different diseases. Myxoid/round cell liposarcoma is characterized by a translocation causing FUS-CHOP or EWSR1-CHOP fusion, whereas well-/dedifferentiated liposarcoma is characterized by an amplification of the 12q13-15 region, including MDM2 and CDK4 genes. As myxoid/round cell liposarcoma is highly radio- and chemosensitive, differentiation between subtypes is important to optimize treatment. We studied whether primary retroperitoneal liposarcomas diagnosed as myxoid/round cell liposarcoma represent molecularly true myxoid/round cell liposarcoma or are histopathological mimics and represent well-/dedifferentiated liposarcoma. Primary retroperitoneal myxoid/round cell liposarcoma (n=16) were compared to primary extremity myxoid/round cell liposarcoma (n=20). Histopathological and immunohistochemical features were studied. Amplification status of the 12q13-15 region was studied using a multiplex ligation-dependent probe amplification analysis, and FUS-CHOP or EWS-CHOP translocations were studied using RT-PCR. In primary retroperitoneal myxoid/round cell liposarcoma, MDM2 and CDK4 staining was both positive in 12 of 15 cases. In primary extremity myxoid/round cell liposarcoma, MDM2 was negative in 18/20 and CDK4 was negative in all cases. Multiplex ligation-dependent probe amplification showed the amplification of 12q13-15 region in 16/16 primary retroperitoneal myxoid/round cell liposarcomas and in 1/20 primary extremity myxoid/round cell liposarcomas. Translocation was present in all (18/18) primary extremity myxoid

  16. Explant culture of sarcoma patients' tissue.

    PubMed

    Muff, Roman; Botter, Sander M; Husmann, Knut; Tchinda, Joelle; Selvam, Philomina; Seeli-Maduz, Franziska; Fuchs, Bruno

    2016-07-01

    Human sarcomas comprise a heterogeneous group of rare tumors that affect soft tissues and bone. Due to the scarcity and heterogeneity of these diseases, patient-derived cells that can be used for preclinical research are limited. In this study, we investigated whether the tissue explant technique can be used to obtain sarcoma cell lines from fresh as well as viable frozen tissue obtained from 8 out of 12 soft tissue and 9 out of 13 bone tumor entities as defined by the World Health Organization. The success rate, defined as the percent of samples that yielded sufficient numbers of outgrowing cells to be frozen, and the time to freeze were determined for a total of 734 sarcoma tissue specimens. In 552 cases (75%) enough cells were obtained to be frozen at early passage. Success rates were higher in bone tumors (82%) compared with soft tissue tumors (68%), and the mean time to freezing was lower in bone tumors (65 days) compared with soft tissue tumors (84 days). Overall, from 40% of the tissues cells could be frozen at early passage within <2 month after tissue removal. Comparable results as with fresh tissue were obtained after explant of viable frozen patient-derived material. In a selected number of bone and soft tissue sarcoma entities, conventional karyotyping and/or FISH (fluorescence in situ hybridization) analysis revealed a high amount (>60%) of abnormal cells in 41% of analyzed samples, especially in bone sarcomas (osteosarcoma and Ewing sarcoma). In conclusion, the explant technique is well suited to establish patient-derived cell lines for a large majority of bone and soft tissue sarcoma entities with adequate speed. This procedure thus opens the possibility for molecular analysis and drug testing for therapeutic decision making even during patient treatment.

  17. How Is Uterine Sarcoma Staged?

    MedlinePlus

    ... The American Cancer Society medical and editorial content team Our team is made up of doctors and master’s-prepared ... Sarcoma About Uterine Sarcoma Causes, Risk Factors, and Prevention Early Detection, Diagnosis, and Staging Treatment After Treatment ...

  18. Orbital granulocytic sarcoma

    PubMed Central

    Stockl, F.; Dolmetsch, A.; Saornil, M; Font, R.; Burnier, M.

    1997-01-01

    AIM—Orbital granulocytic sarcoma is a localised tumour composed of cells of myeloid origin. Histological diagnosis can be difficult in patients with poorly differentiated orbital tumours and no evidence of systemic leukaemia. The naphthol AS-D chloracetate esterase (Leder stain) and immunohistochemical stains for lysozyme and MAC387 were used to determine the staining characteristics of these tumours. A case series of seven patients with orbital granulocytic sarcoma is presented.
METHODS—Seven patients with orbital granulocytic sarcoma were studied. Haematoxylin and eosin, Leder, and lysozyme stained sections were available in seven cases. Unstained formalin fixed paraffin embedded sections of seven cases were available for immunohistochemical evaluation using the avidin-biotin-complex technique for MAC387.
RESULTS—The mean age of presentation of the orbital tumour was 8.8 years. Four patients presented with an orbital tumour before any systemic manifestations of leukaemia. In two cases the diagnosis of the orbital tumour and systemic leukaemia was made simultaneously. There was one case of established systemic myeloid leukaemia in remission with the subsequent development of orbital granulocytic sarcoma. Six of seven cases (86%) were positive for the Leder stain. Five of seven cases (71%) showed positive immunoreactivity with lysozyme. The immunohistochemical stain for MAC387 was positive in all seven cases (100%) including one case that was negative for both lysozyme and Leder stains.
CONCLUSIONS—Orbital granulocytic sarcoma is a tumour that affects children and can present with rapidly progressive proptosis. This tumour may develop before, during, or after the occurrence of systemic leukaemia. The combination of Leder and lysozyme stains is useful in the diagnosis of orbital granulocytic sarcoma. MAC387 may be a more reliable marker for orbital granulocytic sarcoma.

 PMID:9497470

  19. Pancreatic Pseudocyst with Splenic Artery Erosion, Retroperitoneal and Splenic Hematoma

    PubMed Central

    Botianu, Petre V. H.; Dobre, Adrian S.; Botianu, Ana-Maria V.; Onisor, Danusia

    2015-01-01

    The erosion of the peripancreatic vascular structures is a rare but life-endangering complication of pancreatic diseases. We report a female patient with a multicompartmentalized pancreatic pseudocyst that eroded the splenic artery resulting in a retroperitoneal and splenic hematoma with hemodynamic instability which required emergency laparotomy with splenectomy, partial cystectomy, ligation of the splenic artery at the level of the vascular erosion, cholecystectomy (lithiasis), and multiple drainage. The postoperative course was difficult (elevated level of platelets, pancreatic fistula) but eventually favourable, with no abdominal complaints and no recurrence at 2-year follow-up. The case shows that the pancreatic pseudocysts may present with acute hemorrhagic complications with life-endangering potential and significant postoperative morbidity. PMID:26783490

  20. 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.

  1. Idiopathic retroperitoneal fibrosis causing unilateral ureteral and sigmoid colon obstruction

    PubMed Central

    Yan, Ting; Wang, Yujuan; Liu, Zhijun; Zhang, Xiaolei; Wu, Qian; Xi, Mingrong

    2017-01-01

    Abstract Objective: The present report aimed to present a unique case of idiopathic retroperitoneal fibrosis (RPF) presenting features of unilateral ureteral and sigmoid colon obstruction. RPF is a rare disorder with unclear etiology. Case report: A 43-year-old female had a 10-day history of lower right abdominal and lumbar pain. Gynecological examination, ultrasound, and computed tomography (CT) were all suggestive of right ovarian tumor. An enhanced CT showed right-sided hydronephrosis. The patient was diagnosed as having ovarian cancer. Ten days after hospitalization, a right intraureteral stent with a double-J catheter was inserted. Upon exploring the abdomen, unyielding RPF was encountered. A partial sigmoidectomy and colostomy were performed. Postoperative pathological results suggested idiopathic RPF. She received steroid treatments. Conclusion: RPF is a rare disease that can be misdiagnosed. Our understanding about its presentation has to be improved and it should be considered as a differential diagnosis for patients presenting with abdominal diseases. PMID:28207528

  2. Differentiation of Lymphoma Presenting as Retroperitoneal Mass and Retroperitoneal Fibrosis: Evaluation with Multidetector-row Computed Tomography

    PubMed Central

    Zhang, Shuai; Chen, Min; Li, Chun-Mei; Song, Guo-Dong; Liu, Ying

    2017-01-01

    Background: Retroperitoneal fibrosis (RPF) and lymphoma presenting as retroperitoneal mass may closely resemble each other and misdiagnosis may occur. This study investigated the differential imaging features of RPF and lymphoma which presented as a retroperitoneal soft tissue using multidetector-row computed tomography (MDCT). Methods: The 42 consecutive patients were included in this retrospective review, including 19 RPF patients (45.2%; including 13 males and 6 females; mean age: 56.7 ± 6.2 years) and 23 patients with lymphoma (54.8%; including 14 males and 9 females; mean age: 57.4 ± 12.3 years). An array of qualitative computed tomography (CT) features of lesions in 42 consecutive patients with newly diagnosed untreated RPF and lymphoma were retrospectively analyzed. The quantitative size of the lesion at the para-aortic region and attenuation in the precontrast, arterial, and portal phases were calculated in regions of interest and compared between the patients with newly diagnosed untreated RPF and with lymphoma. Receiver operating characteristic curve analysis was used to assess the potential diagnostic value of each quantitative parameter. Inter-reader concordance was also calculated. Results: Mean ages between patients with RPF and lymphoma were not significantly different (56.7 ± 6.2 years vs. 57.4 ± 12.3 years P = 0.595). Compared to those in patients with lymphoma, homogeneous enhancement (65.2% vs. 94.7%, P = 0.027) and pelvic extension (52.2% vs. 89.5%, P = 0.017) were significantly more common while the involvement of additional nodes (78.3% vs. 5.3%, P < 0.001), suprarenal extension (60.9% vs. 15.8%, P = 0.004), and aortic displacement (43.5% vs. 5.3%, P = 0.006) were significantly less common in patients with RPF. Lesion size at the para-aorta was significantly greater in patients with lymphoma, compared with RPF patients (3.9 ± 1.2 cm vs. 1.8 ± 0.6 cm; P < 0.001). The attenuation values in three phases were not significantly different

  3. Expression of PSMA in tumor neovasculature of high grade sarcomas including synovial sarcoma, rhabdomyosarcoma, undifferentiated sarcoma and MPNST

    PubMed Central

    Heitkötter, Birthe; Trautmann, Marcel; Grünewald, Inga; Bögemann, Martin; Rahbar, Kambiz; Gevensleben, Heidrun; Wardelmann, Eva; Hartmann, Wolfgang; Steinestel, Konrad; Huss, Sebastian

    2017-01-01

    Aims PSMA (prostate specific membrane antigen) is physiologically expressed in normal prostate tissue. It is overexpressed in prostate cancer cells and has been suggested as a target for antibody-based radioligand therapy. As PSMA expression so far has not been systematically analyzed in soft tissue tumors, the current study aims at investigating a large cohort of different subtypes. Methods and Results Immunohistochemistry was used to detect PSMA expression in 779 samples of soft tissue tumors and Ewing sarcoma as a primary bone malignancy. CD34 coexpression was employed to study PSMA expression in the neovasculature. PSMA expression was found in the tumor-associated neovasculature of 151/779 soft tissue/bone tumors (19.38%) and was more frequent in malignant tumors compared to tumors with intermediate or benign biological potential (p=0.078). Strong neovascular PSMA expression was predominantly observed in subsets of different sarcomas including 3/20 rhabdomyosarcomas (15%), 4/21 malignant peripheral nerve sheath tumors (19.05%), 6/16 synovial sarcomas (35.29%) and 6/33 undifferentiated pleomorphic sarcomas (18.18%). Conclusion We conclude that PSMA is expressed in the neovasculature of a subset of soft tissue tumors to a variable extent. Our observation of strong PSMA expression predominantly occurring in sarcomas might provide a rationale to evaluate PSMA-targeted radioligand therapy in these entities. PMID:28002805

  4. Schwannoma Localized Retroperitoneally in a 14-Year-Old Boy

    PubMed Central

    Cayirli, Hasan; Ozguven, Ali Aykan; Gunsar, Cuneyt; Ersoy, Betul; Kandiloglu, Ali Riza

    2016-01-01

    Schwannomas usually occur in adults being between the second and fifth decades, and such neoplasms are extremely rare in a pediatric population. In addition, they are not normally found in the retroperitoneal region. Here, we present a pediatric case of a retroperitoneal schwannoma in an adrenal location where the tumor was not able to be preoperatively differentiated from other benign or malign adrenal gland tumors. In our opinion, this tumor can be included in the differential diagnosis of a nonfunctioning retroperitoneal adrenal mass in children. PMID:28078159

  5. BCOR-CCNB3 (Ewing-like) sarcoma: a clinicopathologic analysis of 10 cases, in comparison with conventional Ewing sarcoma.

    PubMed

    Puls, Florian; Niblett, Angela; Marland, Gillian; Gaston, Czar Louie L; Douis, Hassan; Mangham, D Chas; Sumathi, Vaiyapuri P; Kindblom, Lars-Gunnar

    2014-10-01

    BCOR-CCNB3 fusion transcripts resulting from an X-chromosomal paracentric inversion were recently identified in a series of unclassifiable soft tissue and bone sarcomas with Ewing sarcoma-like morphology. The morphologic and clinical features of these sarcomas are, as yet, not well characterized. Here we describe the clinicopathologic features of 10 cases of BCOR-CCNB3 sarcoma and compare their clinical course with typical Ewing sarcoma. Nine of 10 patients were male, and all were 11 to 18 years of age. Seven tumors were located in the bone and 3 in the deep soft tissues. The histomorphologic spectrum was quite wide, with 7 tumors predominately showing small primitive cell morphology with angulated nuclei simulating so-called atypical Ewing sarcoma and 3 predominately showing spindle cell morphology. Recurrent and metastatic lesions showed increased cellularity and marked pleomorphism. Immunohistochemistry showed expression of CCNB3 (100%), bcl2 (90%), CD99 (60%), and CD117 (60%). Reverse transcription polymerase chain reaction for BCOR-CCNB3 fusion transcripts was positive in all 9 cases, which yielded sufficient extracted RNA. Five- and 10-year survival rates were 75% and 56%, respectively. BCOR-CCNB3 sarcomas located in axial skeleton and soft tissues showed a significantly shorter survival. The Ewing sarcoma overall survival was not statistically different, although there was a trend for longer survival of patients with BCOR-CCNB3 sarcomas in the extremities. In conclusion, this study provides a detailed description of the histologic spectrum, immunohistochemical features, and clinical characteristic of BCOR-CCNB3 sarcoma justifying distinction from Ewing sarcoma with its typical EWS/FUS-ETS translocations. Ideally immunohistochemistry is used in combination with reverse transcription polymerase chain reaction for definitive diagnosis.

  6. Long-term results of a multicenter SAKK trial on high-dose ifosfamide and doxorubicin in advanced or metastatic gynecologic sarcomas.

    PubMed

    Leyvraz, S; Zweifel, M; Jundt, G; Lissoni, A; Cerny, T; Sessa, C; Fey, M; Dietrich, D; Honegger, H P

    2006-04-01

    Dose intensive chemotherapy has not been tested prospectively for the treatment of gynecologic sarcomas. We investigated the antitumor activity and toxicity of high-dose ifosfamide and doxorubicin, in the context of a multidisciplinary strategy for the treatment of advanced and metastatic, not pretreated, gynecologic sarcomas. Thirty-nine patients were enrolled onto a phase I-II multicenter trial of ifosfamide, 10 g/m2 as a continuous infusion over 5 days, plus doxorubicin intravenously, 25 mg/m2/day for 3 days with Mesna and granulocyte-colony-stimulating factor every 21 days. Salvage therapy was allowed after chemotherapy. Among the 37 evaluable patients, the tumor was locally advanced (n = 11), with concomitant distant metastases (n = 5) or with distant metastases only (n = 21). After a median of three (range 1-7) chemotherapy cycles, six patients experienced a complete response and 12 a partial response for an overall response rate of 49% (95% CI 32% to 66%). The response rate was higher in poorly differentiated tumors (62%) compared with moderately well differentiated ones (18%), but was not different according to histology subtypes. Eleven patients had salvage therapy, either immediately following chemotherapy (n = 7) or at time of progression (n = 4). With a median follow-up time of 5 years, the median overall survival was 30.5 months. Hematological toxicity was as expected neutropenia, thrombopenia and anemia > or = grade 3 at 50%, 34% and 33% of cycles respectively. No toxic death occurred. High-dose ifosfamide plus doxorubicin is an active regimen for all subtypes of gynecological sarcomas. Its toxicity was manageable in a multicentric setting. The prolonged survival might be due to the multidisciplinary strategy that was possible in one-third of the patients.

  7. Retroperitoneal Approach in Single-Port Laparoscopic Hysterectomy

    PubMed Central

    Kim, Tae-Hyun; Kim, Chul Jung; Lee, Yoo-Young; Choi, Chel Hun; Lee, Jeong-Won; Bae, Duk-Soo; Kim, Byoung-Gie

    2016-01-01

    Background and Objectives: In single-port laparoscopic hysterectomy(SP-LH), ligation of the uterine artery is a fundamental step. We analyzed the effectiveness and safety of 2 different surgical approaches to ligate the uterine artery in SP-LH for women with uterine myomas or adenomyosis. Methods: A single surgeon (TJ Kim) performed 36 retroperitoneal single-port laparoscopic hysterectomies (SP-rH) from September 1st 2012 to April 30th 2013. We compared these cases with 36 cases of conventional single-port laparoscopic abdominal hysterectomy (SP-aH) performed by the same surgeon from November 1st 2011 to July 31th 2012 (historic control). In the SP-rH cases, the retroperitoneal space was developed to identify the uterine artery; then, it was ligated where it originates from the internal iliac artery. Results: Estimated blood loss (EBL) was decreased in the SP-rH group compared with the SP-aH group (100 mL vs 200 mL; P = .023). The median total operative time was shorter in the SP-rH group (75 minutes vs 93 minutes; P < .05). The operative time of the Scope I phase, including ligation of the utero-ovarian (or infundibulopelvic) ligament, round ligament, uterine artery, and detachment of the bladder, was longer in the SP-rH group compared with that in the SP-aH group (26.0 minutes vs 24 minutes; P = .043). However, the operative time of the Scope II phase, including detachment of the uterosacral-cardinal ligament, vaginal cutting, and uterus removal, was shorter in the SP-rH group (19.5 minutes vs 30 minutes; P < .05). Operative complications were not significantly different between the groups (P = .374). Conclusion: Although SP-rH may be considered technically difficult, it can be performed safely and efficiently with surgical outcomes comparable to those of SP-aH. PMID:27186067

  8. Surgical anatomy of the retroperitoneal spaces, Part III: Retroperitoneal blood vessels and lymphatics.

    PubMed

    Mirilas, Petros; Skandalakis, John E

    2010-02-01

    In this article, we discuss the surgical anatomy of the blood vessels and the lymphatic vessels and lymph nodes found in the retroperitoneum. Retroperitoneal blood vessels include the aorta and all its branches--parietal and visceral--from the diaphragm to the pelvis, and the inferior vena cava and its tributaries. The retroperitoneal lymphatics form a very rich and extensive chain. As a general rule, lymphatics follow the arteries and named lymph nodes are found at the root of the arteries. Retroperitoneal nodes of the abdomen comprise the inferior diaphragmatic nodes and the lumbar nodes. The latter are classified as left lumbar (aortic), intermediate (interaorticovenous), and right lumbar (caval). These nodes surround the aorta and the inferior vena cava. Around the aorta lie the paraortic nodes, preaortic nodes (include celiac, superior mesenteric, inferior mesenteric nodes collecting lymph from the splanchna supplied by the homonymous arteries), and retroaortic nodes. Similarly, around the vena cava lie the paracaval, precaval, and retrocaval nodes. Pelvic nodes include the common iliac, external and internal iliac, obturator, and sacral nodes.

  9. Dasatinib inhibits migration and invasion in diverse human sarcoma cell lines and induces apoptosis in bone sarcoma cells dependent on SRC kinase for survival.

    PubMed

    Shor, Audrey C; Keschman, Elizabeth A; Lee, Francis Y; Muro-Cacho, Carlos; Letson, G Douglas; Trent, Jonathan C; Pledger, W Jack; Jove, Richard

    2007-03-15

    Sarcomas are rare malignant mesenchymal tumors for which there are limited treatment options. One potential molecular target for sarcoma treatment is the Src tyrosine kinase. Dasatinib (BMS-354825), a small-molecule inhibitor of Src kinase activity, is a promising cancer therapeutic agent with p.o. bioavailability. Dasatinib exhibits antitumor effects in cultured human cell lines derived from epithelial tumors, including prostate and lung carcinomas. However, the action of dasatinib in mesenchymally derived tumors has yet to be shown. Based on our previous findings of Src activation in human sarcomas, we evaluated the effects of dasatinib in 12 cultured human sarcoma cell lines derived from bone and soft tissue sarcomas. Dasatinib inhibited Src kinase activity at nanomolar concentrations in these sarcoma cell lines. Downstream components of Src signaling, including focal adhesion kinase and Crk-associated substrate (p130(CAS)), were also inhibited at similar concentrations. This inhibition of Src signaling was accompanied by blockade of cell migration and invasion. Moreover, apoptosis was induced in the osteosarcoma and Ewing's subset of bone sarcomas at nanomolar concentrations of dasatinib. Inhibition of Src protein expression by small interfering RNA also induced apoptosis, indicating that these bone sarcoma cell lines are dependent on Src activity for survival. These results show that dasatinib inhibits migration and invasion of diverse sarcoma cell types and selectively blocks the survival of bone sarcoma cells. Therefore, dasatinib may provide therapeutic benefit by preventing the growth and metastasis of sarcomas in patients.

  10. A case report of pancreatic metastasis from synovial sarcoma successfully treated by metastasectomy with adjuvant chemotherapy

    PubMed Central

    Makino, Yuki; Shigekawa, Minoru; Kegasawa, Tadashi; Suda, Takahiro; Yoshioka, Teppei; Iwahashi, Kiyoshi; Ikezawa, Kenji; Sakamori, Ryotaro; Yakushijin, Takayuki; Kajihara, Jun; Tomimaru, Yoshito; Eguchi, Hidetoshi; Imura, Yoshinori; Outani, Hidetatsu; Naka, Norifumi; Honma, Keiichiro; Morii, Eiichi; Tatsumi, Tomohide; Hiramatsu, Naoki; Takehara, Tetsuo

    2016-01-01

    Abstract Introduction: Synovial sarcoma is a malignant soft tissue sarcoma which arises near joints. The most frequent metastasis sites of synovial sarcoma are the lungs, lymph nodes, and bone. Pancreatic metastasis is quite rare; only 3 cases have been reported worldwide to date. We herein present the 4th case of pancreatic metastasis from synovial sarcoma. Methods and Results: A 32-year-old man underwent extended excision of synovial sarcoma in the left pelvis and femur in 2009. In 2013, follow-up contrast-enhanced computed tomography revealed a 35-mm heterogeneously enhanced mass in the pancreas body. Endoscopic ultrasound-guided fine needle aspiration of the mass revealed a diffuse proliferation of atypical spindle cells in a fascicular arrangement. Because the histology was quite similar to the resected specimen of synovial sarcoma in 2009, the mass was suspected to be a metastasis from synovial sarcoma. Laparoscopic distal pancreatectomy with adjuvant adriamycin/ifosfamide chemotherapy was subsequently performed. Synovial sarcoma-specific SS18-SSX1 (synovial sarcoma translocation, chromosome 18-synovial sarcoma X1) or SS18-SSX2 chimera mRNA was detected in the resected specimen, confirming the diagnosis of metastasis from synovial sarcoma. The patient did well for 30 months without recurrence. Conclusion: This case suggests that pancreatic metastasis from synovial sarcoma can be successfully treated by metastasectomy with adjuvant chemotherapy. PMID:27684804

  11. Microsatellite instability in sarcoma: fact or fiction?

    PubMed

    Monument, Michael J; Lessnick, Stephen L; Schiffman, Joshua D; Randall, Rl Tx

    2012-01-01

    Microsatellite instability (MSI) is a unique molecular abnormality, indicative of a deficient DNA mismatch repair (MMR) system. Described and characterized in the colorectal cancer literature, the MSI-positive phenotype is predictive of disease susceptibility, pathogenesis, and prognosis. The clinical relevance of MSI in colorectal cancer has inspired similar inquisition within the sarcoma literature, although unfortunately, with very heterogeneous results. Evolving detection techniques, ill-defined sarcoma-specific microsatellite loci and small study numbers have hampered succinct conclusions. The literature does suggest that MSI in sarcoma is observed at a frequency similar to that of sporadic colorectal cancers, although there is little evidence to suggest that MSI-positive tumors share distinct biological attributes. Emerging evidence in Ewing sarcoma has demonstrated an intriguing mechanistic role of microsatellite DNA in the activation of key EWS/FLI-target genes. These findings provide an alternative perspective to the biological implications of microsatellite instability in sarcoma and warrant further investigation using sophisticated detection techniques, sensitive microsatellite loci, and appropriately powered study designs.

  12. INTRAOCULAR SYNOVIAL SARCOMA.

    PubMed

    Richards, Nikisha Q; Kofler, Julia K; Chu, Charleen T; Stefko, S Tonya

    2017-01-01

    To describe the first reported case of intraocular synovial sarcoma. A 29-year-old man was enucleated for a blind, painful eye. Pathologic examination revealed an unexpected intraocular spindle cell tumor. Immunohistochemical characterization revealed diffuse reactivity of the tumor cells for vimentin and focal positivity for epithelial markers pankeratin and epithelial membrane antigen. Melanoma markers were negative. Fluorescent in situ hybridization studies identified a t(X;18) (p11.2;q11.2) translocation, establishing a final diagnosis of synovial sarcoma. There was no evidence of extraocular extension as the resected margin of the optic nerve was free of tumor. Further imaging studies revealed no extraocular primary site or metastasis. The incidental discovery of an intraocular malignancy in this case underscores the importance of routine histopathologic analysis of all enucleated globes. To the authors' knowledge, this is the first reported case of an intraocular synovial sarcoma, either as metastasis or as primary site.

  13. [Sarcoma of the breast].

    PubMed

    Haberthür, F; Almendral, A C; Feichter, G; Torhorst, J K

    1992-05-01

    Sarcoma of the breast represents only 0.2-1% of all mammary malignancies. This study reports 5 such cases, including 2 osteosarcomas, 1 fibro-, 1 lipo-, and 1 malignant fibrous sarcoma. The treatment used was mastectomy in 3 cases with excision of axillary lymph nodes. The remaining 2 patients were treated by simple mastectomy whereby 1 of these received a immediate reconstruction with a prosthesis. 1 patient demonstrated local recurrence and died. The remaining 4 patients did not develop neither metastases nor local recurrence and are still alive after an observing period between 12 months up to 17 years. Today, first-line treatment is wide local excision or simple mastectomy. Excision of the axillary lymphatics, adjuvant radiotherapy, and chemotherapy have been disappointing in the treatment of breast sarcoma.

  14. Large retroperitoneal low-grade extraskeletal osteosarcoma

    PubMed Central

    Jaipuria, Jiten; Kumar, Ashok; Rao, Adla Satyanarayan; Kataria, Satya Pal

    2014-01-01

    Low-grade extraskeletal osteosarcoma is an extremely rare neoplasm with only nine cases reported in the literature, in which only one case involved the retroperitoneum. Tendency to dedifferentiate as well as recur with high-grade variant is known and management principles are not well defined, necessitating a continuous review of literature. We report management of the largest such retroperitoneal tumour (18.0 cm× 18.3 cm×17.8 cm) in a 38-year-old man, which was treated with surgery followed by six cycles of cisplatin (90 mg/m2 days 1 and 2) and doxorubicin (75 mg/m2 day 3), cycled every 3 weeks (with granulocyte colony stimulating factor support as necessary). The patient is disease free after 3 years of follow-up. PMID:24658529

  15. Retroperitoneal hematoma following trauma: its clinical importance.

    PubMed

    Grieco, J G; Perry, J F

    1980-09-01

    Records of 100 consecutive patients treated in 1973 through 1977 with post-traumatic retroperitoneal hematomas (RH) were studied. Eighty RH followed blunt injury and 20 were due to penetrating trauma. Overall mortality was 26%. The worst prognosis was associated with RH from automobile accidents and pedestrian injuries. Pelvic RH were almost uniformly associated with pelvic fracture and were the primary cause of 39% of deaths. Blunt perinephric RH required renal exploration in 47% of patients. Blunt RH in other locations were associated with major visceral or vascular injury in half the patients and were the cause of death in five. Sixty-five per cent of RH due to penetrating trauma had visceral or vascular injury requiring operative correction. Contained rupture of descending choracic aorta presented as retrogastric RH in two patients. RH from penetrating trauma should be explored routinely, since 65% are associated with visceral or vascular injury.

  16. Retroperitoneal fibrosis - a report of five cases.

    PubMed

    Runowska, Marta; Majewski, Dominik; Puszczewicz, Mariusz

    2017-01-01

    Retroperitoneal fibrosis (RPF) is a rare disease, characterized by inflammation and deposition of fibrotic tissue in the vicinity of the abdominal aorta and iliac arteries. We present a report of five patients admitted to our department between January 2014 and February 2017, diagnosed with RPF. Abdominal pain was the most common presenting symptom; however, in one patient, RPF was identified accidentally in routinely performed ultrasonography. In 4 cases, corticosteroids (CS) in combination with azathioprine were applied as first-line therapy, whereas one patient was treated with intravenous methylprednisolone pulses followed by oral CS. In this paper, clinical features as well as laboratory and radiographic findings together with management and treatment outcomes in patients with RPF are discussed. Given the rarity of the condition, it seems important to report every single case of RPF to help establish its management algorithm.

  17. Gasless laparoscopic removal of retroperitoneal leiomyosarcoma.

    PubMed

    Viani, M P; Poggi, R V; Pinto, A; Maruotti, R A

    1995-02-01

    Leiomyosarcoma is a rare malignant tumor originating from the smooth muscular tissue in any part of the organism. The only therapy is its complete removal. We describe herein the operative treatment of a retroperitoneal leiomyosarcoma with gasless laparoscopic complete removal. The procedure was successfully performed in a consenting woman with an abdominal mass. Gasless laparoscopic removal was performed with a mechanical retractor (Laparolift, Origin Medsystem Inc.), obviating the creation of the pneumoperitoneum and of the sealed environment. The technique is a simple, safe, and effective surgical method. Gasless technique guarantees a clear vision, makes possible continuous suction of smoke and fluids, and allows the use of conventional instruments and easy management of suturing. The present case has proved to be another abdominal procedure that can be carried out with all the advantages of gasless miniinvasive surgery.

  18. [Moritz Kaposi and his sarcoma].

    PubMed

    van Kessel, Anne; Quint, Koen D

    2011-01-01

    Nowadays, Kaposi sarcoma is a multidisciplinary condition, not only observed by dermatologists. Since the HIV epidemic in the 80s and 90s of the last century, more insight into the aetiology of Kaposi sarcoma has been acquired. However, this sarcoma had already been described in 1872 by a Hungarian dermatologist named Moritz Kaposi (1832-1902). Kaposi described the entity as 'idiopathic multiple pigmented sarcoma of the skin'. This entity was an extraordinary diagnosis at that time, mostly observed in Jewish or Mediterranean men. In 1912, 10 years after the death of Moritz Kaposi, the entity name was changed to Kaposi sarcoma.

  19. Systemic therapy for cardiac sarcomas.

    PubMed

    Ravi, Vinod; Benjamin, Robert S

    2010-01-01

    Cardiac sarcomas create 2 risks: local problems and metastatic disease. Most frequently, the histologies are angiosarcoma and high-grade pleomorphic unclassified sarcoma (formerly called MFH or malignant fibrous histiocytoma). There is also a clinical-pathological entity without distinctive histological features of tumors that originate in the pulmonary artery and are referred to as pulmonary artery sarcomas or intimal sarcomas of the pulmonary artery. Conventional wisdom indicates that soft-tissue sarcomas are poorly responsive to chemotherapy. Luckily, that is not the case. Attempts to concentrate on the local problem only with therapies up to and including cardiac transplantation have been unsuccessful due to the high rate of fatal metastatic disease.

  20. [Sarcomas, example of a pathologist network organization].

    PubMed

    Neuville, Agnes; Coindre, Jean-Michel

    2013-12-01

    Sarcomas are rare and heterogeneous with many subtypes explaining the high level of diagnostic difficulty with frequent important therapeutic consequences. In 2009, a national network of pathologists has been set up with the main objective to perform a systematic histological review of every new sarcoma, gastro-intestinal stromal tumor (GIST) and desmoid tumor. We describe the network organization and report the results of the first two years of activity. These results clearly show the interest of this organization for the patients as well as for all pathologists. Moreover, data and material collect allows a better knowledge of these tumors and an improvement of the rules for their diagnostic management.

  1. Significant Reduction of Late Toxicities in Patients With Extremity Sarcoma Treated With Image-Guided Radiation Therapy to a Reduced Target Volume: Results of Radiation Therapy Oncology Group RTOG-0630 Trial

    PubMed Central

    Wang, Dian; Zhang, Qiang; Eisenberg, Burton L.; Kane, John M.; Li, X. Allen; Lucas, David; Petersen, Ivy A.; DeLaney, Thomas F.; Freeman, Carolyn R.; Finkelstein, Steven E.; Hitchcock, Ying J.; Bedi, Manpreet; Singh, Anurag K.; Dundas, George; Kirsch, David G.

    2015-01-01

    Purpose We performed a multi-institutional prospective phase II trial to assess late toxicities in patients with extremity soft tissue sarcoma (STS) treated with preoperative image-guided radiation therapy (IGRT) to a reduced target volume. Patients and Methods Patients with extremity STS received IGRT with (cohort A) or without (cohort B) chemotherapy followed by limb-sparing resection. Daily pretreatment images were coregistered with digitally reconstructed radiographs so that the patient position could be adjusted before each treatment. All patients received IGRT to reduced tumor volumes according to strict protocol guidelines. Late toxicities were assessed at 2 years. Results In all, 98 patients were accrued (cohort A, 12; cohort B, 86). Cohort A was closed prematurely because of poor accrual and is not reported. Seventy-nine eligible patients from cohort B form the basis of this report. At a median follow-up of 3.6 years, five patients did not have surgery because of disease progression. There were five local treatment failures, all of which were in field. Of the 57 patients assessed for late toxicities at 2 years, 10.5% experienced at least one grade ≥ 2 toxicity as compared with 37% of patients in the National Cancer Institute of Canada SR2 (CAN-NCIC-SR2: Phase III Randomized Study of Pre- vs Postoperative Radiotherapy in Curable Extremity Soft Tissue Sarcoma) trial receiving preoperative radiation therapy without IGRT (P < .001). Conclusion The significant reduction of late toxicities in patients with extremity STS who were treated with preoperative IGRT and absence of marginal-field recurrences suggest that the target volumes used in the Radiation Therapy Oncology Group RTOG-0630 (A Phase II Trial of Image-Guided Preoperative Radiotherapy for Primary Soft Tissue Sarcomas of the Extremity) study are appropriate for preoperative IGRT for extremity STS. PMID:25667281

  2. Vascular stenosis with retroperitoneal rhabdomyosarcoma in a child: Case report

    SciTech Connect

    Bakody, P.J.; Stanley, P.

    1983-08-01

    Discovery on angiography of arterial stenosis in an 11-month-old girl with a retroperitoneal mass led to a preoperative diagnosis of neuroblastoma. Surgery revealed a rhabdomyosarcoma. The arteriographic appearance in both tumors may be identical.

  3. Transgenic Expression of Walleye Dermal Sarcoma Virus rv-cyclin (orfA) in Zebrafish does not Result in Tissue Proliferation

    PubMed Central

    Paul, Thomas A.; Rovnak, Joel; Quackenbush, Sandra L.; Whitlock, Kathleen; Zhan, Huiqing; Gong, Zhiyuan; Spitsbergen, Jan; Bowser, Paul R.

    2012-01-01

    Walleye dermal sarcoma (WDS) is a benign tumor of walleye fish that develops and completely regresses seasonally. The retrovirus associated with this disease, walleye dermal sarcoma virus, encodes three accessory genes, two of which, rv-cyclin (orfA) and orfb, are thought to play a role in tumor development. In this study, we attempted to recapitulate WDS development by expressing rv-cyclin in chimeric and stable transgenic zebrafish. Six stable transgenic lines expressing rv-cyclin from the constitutive CMVtk promoter were generated. Immunohistochemistry and quantitative reverse transcriptase polymerase chain reaction demonstrate that rv-cyclin is widely expressed in different tissues in these fish. These lines were viable and histologically normal for up to 2 years. No increase in tumors or tissue proliferation was observed following N-ethyl N-nitrosourea exposure or following tail wounding and subsequent tissue regeneration compared to controls. These data indicate that rvcyclin is not independently sufficient for tumor induction in zebrafish. PMID:20349325

  4. 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.

  5. Kaposi sarcoma in unusual locations

    PubMed Central

    Pantanowitz, Liron; Dezube, Bruce J

    2008-01-01

    Kaposi sarcoma (KS) is a multifocal, vascular lesion of low-grade malignant potential that presents most frequently in mucocutaneous sites. KS also commonly involves lymph nodes and visceral organs. This article deals with the manifestation of KS in unusual anatomic regions. Unusual locations of KS involvement include the musculoskeletal system, central and peripheral nervous system, larynx, eye, major salivary glands, endocrine organs, heart, thoracic duct, urinary system and breast. The development of KS within wounds and blood clots is also presented. KS in these atypical sites may prove difficult to diagnose, resulting in patient mismanagement. Theories to explain the rarity and development of KS in these unusual sites are discussed. PMID:18605999

  6. Microenvironmental Targets in Sarcoma

    PubMed Central

    Ehnman, Monika; Larsson, Olle

    2015-01-01

    Sarcomas are rare malignant tumors affecting all age groups. They are typically classified according to their resemblance to corresponding normal tissue. Their heterogeneous features, for example, in terms of disease-driving genetic aberrations and body location, complicate both disease classification and development of novel treatment regimens. Many years of failure of improved patient outcome in clinical trials has led to the conclusion that novel targeted therapies are likely needed in combination with current multimodality regimens. Sarcomas have not, in contrast to the common carcinomas, been the subject of larger systematic studies on how tumor behavior relates to characteristics of the tumor microenvironment. There is consequently an urgent need for identifying suitable molecular targets, not only in tumor cells but also in the tumor microenvironment. This review discusses preclinical and clinical data about potential molecular targets in sarcomas. Studies on targeted therapies involving the tumor microenvironment are prioritized. A greater understanding of the biological context is expected to facilitate more successful design of future clinical trials in sarcoma. PMID:26583076

  7. 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 ...

  8. Management of Bone Sarcoma.

    PubMed

    Gutowski, Christina J; Basu-Mallick, Atrayee; Abraham, John A

    2016-10-01

    Treatment of bone sarcoma requires careful planning and involvement of an experienced multidisciplinary team. Significant advancements in systemic therapy, radiation, and surgery in recent years have contributed to improved functional and survival outcomes for patients with these difficult tumors, and emerging technologies hold promise for further advancement. Copyright © 2016 Elsevier Inc. All rights reserved.

  9. Sarcoma derived from cultured mesenchymal stem cells.

    PubMed

    Tolar, Jakub; Nauta, Alma J; Osborn, Mark J; Panoskaltsis Mortari, Angela; McElmurry, Ron T; Bell, Scott; Xia, Lily; Zhou, Ning; Riddle, Megan; Schroeder, Tania M; Westendorf, Jennifer J; McIvor, R Scott; Hogendoorn, Pancras C W; Szuhai, Karoly; Oseth, Leann; Hirsch, Betsy; Yant, Stephen R; Kay, Mark A; Peister, Alexandra; Prockop, Darwin J; Fibbe, Willem E; Blazar, Bruce R

    2007-02-01

    To study the biodistribution of MSCs, we labeled adult murine C57BL/6 MSCs with firefly luciferase and DsRed2 fluorescent protein using nonviral Sleeping Beauty transposons and coinfused labeled MSCs with bone marrow into irradiated allogeneic recipients. Using in vivo whole-body imaging, luciferase signals were shown to be increased between weeks 3 and 12. Unexpectedly, some mice with the highest luciferase signals died and all surviving mice developed foci of sarcoma in their lungs. Two mice also developed sarcomas in their extremities. Common cytogenetic abnormalities were identified in tumor cells isolated from different animals. Original MSC cultures not labeled with transposons, as well as independently isolated cultured MSCs, were found to be cytogenetically abnormal. Moreover, primary MSCs derived from the bone marrow of both BALB/c and C57BL/6 mice showed cytogenetic aberrations after several passages in vitro, showing that transformation was not a strain-specific nor rare event. Clonal evolution was observed in vivo, suggesting that the critical transformation event(s) occurred before infusion. Mapping of the transposition insertion sites did not identify an obvious transposon-related genetic abnormality, and p53 was not overexpressed. Infusion of MSC-derived sarcoma cells resulted in malignant lesions in secondary recipients. This new sarcoma cell line, S1, is unique in having a cytogenetic profile similar to human sarcoma and contains bioluminescent and fluorescent genes, making it useful for investigations of cellular biodistribution and tumor response to therapy in vivo. More importantly, our study indicates that sarcoma can evolve from MSC cultures.

  10. Immunotherapy of Childhood Sarcomas

    PubMed Central

    Roberts, Stephen S.; Chou, Alexander J.; Cheung, Nai-Kong V.

    2015-01-01

    Pediatric sarcomas are a heterogeneous group of malignant tumors of bone and soft tissue origin. Although more than 100 different histologic subtypes have been described, the majority of pediatric cases belong to the Ewing’s family of tumors, rhabdomyosarcoma and osteosarcoma. Most patients that present with localized stage are curable with surgery and/or chemotherapy; however, those with metastatic disease at diagnosis or those who experience a relapse continue to have a very poor prognosis. New therapies for these patients are urgently needed. Immunotherapy is an established treatment modality for both liquid and solid tumors, and in pediatrics, most notably for neuroblastoma and osteosarcoma. In the past, immunomodulatory agents such as interferon, interleukin-2, and liposomal-muramyl tripeptide phosphatidyl-ethanolamine have been tried, with some activity seen in subsets of patients; additionally, various cancer vaccines have been studied with possible benefit. Monoclonal antibody therapies against tumor antigens such as disialoganglioside GD2 or immune checkpoint targets such as CTLA-4 and PD-1 are being actively explored in pediatric sarcomas. Building on the success of adoptive T cell therapy for EBV-related lymphoma, strategies to redirect T cells using chimeric antigen receptors and bispecific antibodies are rapidly evolving with potential for the treatment of sarcomas. This review will focus on recent preclinical and clinical developments in targeted agents for pediatric sarcomas with emphasis on the immunobiology of immune checkpoints, immunoediting, tumor microenvironment, antibody engineering, cell engineering, and tumor vaccines. The future integration of antibody-based and cell-based therapies into an overall treatment strategy of sarcoma will be discussed. PMID:26301204

  11. What Happens After Treatment for Soft Tissue Sarcomas?

    MedlinePlus

    ... Sarcoma After Treatment What Happens After Treatment for Soft Tissue Sarcomas? For some people with soft tissue ... for Soft Tissue Sarcoma Stops Working More In Soft Tissue Sarcoma About Soft Tissue Sarcoma Causes, Risk ...

  12. Klippel-Trenaunay syndrome presenting with acanthocytosis and splenic and retroperitoneal lymphangioma: a case report.

    PubMed

    Withana, Milinda; Rodrigo, Chaturaka; Shivanthan, Mitrakrishnan Chrishan; Warnakulasooriya, Sachini; Wimalachandra, Manu; Gooneratne, Lallindra; Rajapakse, Senaka

    2014-11-27

    Klippel-Trenaunay syndrome is a rare congenital mesodermal abnormality characterized by bone and soft tissue hypertrophy, extensive hemangioma and venous abnormalities. We report the case of a patient with two additional rare clinical manifestations in the background of Klippel-Trenaunay syndrome, namely, acanthocytosis and splenic and retroperitoneal lymphangioma. A 24-year-old Sri Lankan man from North Central Province in Sri Lanka presented to our general medical unit with symptomatic anaemia. He had been diagnosed with Klippel-Trenaunay syndrome at the age of six years, with hemihypertrophy of his right lower limb and strawberry naevi over both lower limbs. His blood film results were positive for acanthocytes, which accounted for more than 20% of the red blood cell population. He was also found to have extensive splenic lymphangiomas and a large retroperitoneal lymphangioma encasing the mesentric vessels in the right para-aortic region. An extensive battery of tests to identify a secondary cause for the acanthocytosis failed to show any positive results. Retroperitoneal lymphangioma has been reported in association with Klippel-Trenaunay syndrome once before, but an association with acanthocytosis has never been reported. Given the rarity of all three conditions this is not surprising. The cause of acanthocytosis in this setting is currently unresolved. It is plausible that this may be a primary association with Klippel-Trenaunay syndrome, as an alternative aetiology was not found.

  13. Development and evaluation of a retroperitoneal dialysis porcine model.

    PubMed

    Okhunov, Zhamshid; Yoon, Renai; Perez Lanzac, Alberto; Sgroi, Michael; Lau, Wei Ling; Del Junco, Michael; Ordon, Michael; Drysch, Austin; Hwang, Christina; Vernez, Simone L; Fujitani, Roy; Kabutey, Nii-Kabu; Kalantar-Zadeh, Kamyar; Landman, Jaime

    2016-08-01

    We attempted to create a surgical model to evaluate the retroperitoneal space for the ability to transfer solutes through the retroperitoneal membrane. Our dual objectives were to develop a technique to assess the feasibility of retroperitoneal dialysis (RPD) in a porcine model. We incorporated two 35-kg Yorkshire pigs for this pilot study. In the first animal, we clamped renal vessels laparoscopically. In the second animal, we embolized renal arteries. In both animals, we dilated the retroperitoneal space bilaterally and deployed dialysis catheters. We measured serum creatinine (Cr), urea, and electrolytes at baseline 6 hours before the dialysis and every 4 hours after. We successfully created retroperitoneal spaces bilaterally and deployed dialysis catheters in both animals. In the first animal, dialysate and plasma Cr ratio (D/P) on the left and right side were 0.43 and 0.3, respectively. Cr clearance by 40 minutes of dialysis treatment was 6.3 mL/min. The ratio of dialysate glucose at 4 hours dwell time to dialysate glucose at 0 dwell time (D/D0) for left/rights sides were 0.02 and 0.02, respectively. kt/Vurea was 0.43. In the second animal, D/P Cr for left/right sides were 0.34 and 0.33, respectively. kt/Vurea was 0.17. We euthanized the pigs due to fluid collection in the peritoneal space and rapid increase of serum Cr, urea, and electrolytes. We demonstrated the feasibility of creation of a functionally anephric porcine model with successful development of retroperitoneal spaces using balloon inflation. Notwithstanding minimal clearance and limited diffusion capacity in this experiment, additional studies are needed to examine potential use of retroperitoneal space for peritoneal dialysis.

  14. Retroperitoneal Fibrosis Due to Opium Abuse: A Case Series and Literature Review

    PubMed Central

    Mohammadzadeh Rezaei, Mohammad Ali; Akhavan Rezayat, Alireza; Ardalan, Shima

    2016-01-01

    Retroperitoneal fibrosis (RPF) is a rare condition with an unclear etiology, presenting with the development of aberrant chronic nonspecific fibroinflammatory tissue in the retroperitoneal space, which can result in entrapment and obstruction of the retroperitoneal structures. RPF is a subtype of chronic periaortitis, and can be divided into two types: primary (or idiopathic) and secondary. RPF is usually idiopathic, but can also be secondary to malignancies, certain drugs, infections, surgery, and trauma. The systemic clinical manifestations are nonspecific and include low-grade fever, fatigue, anorexia, weight loss, and myalgia. We report five patients admitted to our hospital with clinical, laboratory, imaging, and pathologic findings compatible with RPF, and we describe their treatment and follow-up. We were suspicious that the impurities of some types of opium have an important role in the pathogenesis of RPF. Some of our patients used opium again after the follow-up period; however, they used a different type with a different origin, and we were surprised to see that RPF did not form again. PMID:27896238

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

    ClinicalTrials.gov

    2017-08-29

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

  16. Current classification, treatment options, and new perspectives in the management of adipocytic sarcomas

    PubMed Central

    De Vita, Alessandro; Mercatali, Laura; Recine, Federica; Pieri, Federica; Riva, Nada; Bongiovanni, Alberto; Liverani, Chiara; Spadazzi, Chiara; Miserocchi, Giacomo; Amadori, Dino; Ibrahim, Toni

    2016-01-01

    Sarcomas are a heterogeneous group of mesenchymal tumors arising from soft tissue or bone, with an uncertain etiology and difficult classification. Soft tissue sarcomas (STSs) account for around 1% of all adult cancers. Till date, more than 50 histologic subtypes have been identified. Adipocyte sarcoma or liposarcoma (LPS) is one of the most common STS subtypes, accounting for 15% of all sarcomas, with an incidence of 24% of all extremity STSs and 45% of all retroperitoneal STSs. The new World Health Organization classification system has divided LPS into four different subgroups: atypical lipomatous tumor/well-differentiated LPS, dedifferentiated LPS, myxoid LPS, and pleomorphic LPS. These lesions can develop at any location and exhibit different aggressive potentials reflecting their morphologic diversity and clinical behavior. Patients affected by LPS should be managed in specialized multidisciplinary cancer centers. Whereas surgical resection is the mainstay of treatment for localized disease, the benefits of adjuvant and neoadjuvant chemotherapy are still unclear. Systemic treatment, particularly chemotherapy, is still limited in metastatic disease. Despite the efforts toward a better understanding of the biology of LPS, the outcome of advanced and metastatic patients remains poor. The advent of targeted therapies may lead to an improvement of treatment options and clinical outcomes. A larger patient enrollment into translational and clinical studies will help increase the knowledge of the biological behavior of LPSs, test new drugs, and introduce new methodological studies, that is, on treatment response. PMID:27785071

  17. Kaposi sarcoma incidence in Mozambique: national and regional estimates.

    PubMed

    Meireles, Paula; Albuquerque, Gabriela; Vieira, Mariana; Foia, Severiano; Ferro, Josefo; Carrilho, Carla; Lunet, Nuno

    2015-11-01

    Kaposi sarcoma is expressed in four clinical variants, all associated with human herpes virus type 8 infection, namely, classic, endemic, immunosuppression-related and AIDS-related. The latter currently accounts for most of the burden of Kaposi sarcoma in sub-Saharan Africa, reflecting the frequency of HIV infection and its management. We aimed to estimate the incidence of Kaposi sarcoma in Mozambique and in its provinces. We estimated the number of incident cases of Kaposi sarcoma by adding up the expected number of endemic and AIDS-related cases. The former were estimated from the rates observed in Kyandondo, Uganda (1960-1971). The latter were computed from the number of AIDS-related deaths in each region, assuming that the ratio between the AIDS-related Kaposi sarcoma incident cases and the number of AIDS-related deaths observed in the city of Beira applies to all regions. A total of 3862 Kaposi sarcoma cases were estimated to have occurred in Mozambique in 2007, mostly AIDS-related, in the age group 25-49 years, and in provinces from South/Centre. The age-standardized incidence rates were 36.1/100 000 in men and 11.5/100 000 in women, with a more than three-fold variation across provinces. We estimated a high incidence of Kaposi sarcoma in Mozambique, along with large regional differences. These results can be used to improve disease management and to sustain political decisions on health policies.

  18. 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.

  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. Effective transduction of osteogenic sarcoma cells by a baculovirus vector.

    PubMed

    Song, Sun U; Shin, Seok-Hwan; Kim, Soon-Ki; Choi, Gwang-Seong; Kim, Woo-Chul; Lee, Moon-Hee; Kim, Sei-Joong; Kim, In-Ho; Choi, Mi-Sook; Hong, Young-Jin; Lee, Kwan-Hee

    2003-03-01

    Efficient gene delivery of a baculovirus-derived vector (BV-p53-lacZ) to a human osteogenic sarcoma cell line, Saos-2, was serendipitously found while evaluating the vector for gene delivery to human p53-null tumour cells in a previous study. Therefore, we investigated other human, rat and mouse osteogenic sarcoma and other types of tumour cell lines for transduction efficiency via baculovirus vectors containing a lacZ reporter gene under the control of either a cytomegalovirus or Rous sarcoma virus promoter. The expression of beta-galactosidase protein, assessed by X-Gal staining and beta-galactosidase ELISA, demonstrated an extremely high level of transduction efficiency in some osteogenic sarcoma cell lines, such as U-2OS, Saos-2 and Saos-LM2. These human osteogenic sarcoma cell lines showed levels of beta-galactosidase expression 5-40 times greater than HepG2 cells, which were previously thought to be the mammalian cells most susceptible to baculovirus-mediated gene delivery. The level of acetylated histone proteins in these tumour lines did not correlate well with the high level of reporter gene expression. These results strongly suggest that some osteogenic sarcoma cells are highly susceptible to baculovirus-mediated gene delivery and that a baculovirus-derived vector is an efficient gene delivery vehicle into human osteogenic sarcoma cells.

  1. Pericytes in sarcomas of bone.

    PubMed

    Chang, Le; Nguyen, Vi; Nguyen, Alan; Scott, Michelle A; James, Aaron W

    2015-07-01

    Pericytes are mesenchymal cells that closely enwrap small blood vessels, lying in intimate association with the endothelium. Pericytes have recently gained attention as an important mediator of vascular biology and angiogenesis in cancer. Although better studied in carcinoma, pericytes have known interaction with sarcomas of bone, including Ewing's sarcoma, osteosarcoma, and chondrosarcoma. Best studied is Ewing's sarcoma (ES), which displays a prominent perivascular growth pattern. Signaling pathways of known importance in intratumoral pericytes in ES include Notch, PDGF/PDGFR-β, and VEGF signaling. In summary, pericytes serve important functions in the tumor microenvironment. Improved understanding of pericyte biology may hold significant implications for the development of new therapies in sarcoma.

  2. Primary pulmonary artery sarcoma.

    PubMed

    Jin, Tao; Zhang, Chong; Feng, Zhiying; Ni, Yiming

    2008-08-01

    Primary pulmonary artery sarcoma is an uncommon tumor. We report a case of a 73-year-old male patient with a two-week history of palpitations and shortness of breath, aggravated for two days and was believed to be pulmonary hypertension. Emergency heart ultrasound after admission presented a massive pulmonary embolism in the pulmonary artery. The patient's condition was successfully managed with urgent pulmonary artery embolectomy. The patient demonstrated improvement in hemodynamics after the operation. Histologic and immunohistochemical assays were performed and a diagnosis was made as primary pulmonary artery sarcoma arising from the left pulmonary artery. Resection of the tumor is recommended for the treatment of this rare malignant tumor. The corresponding chemotherapy, follow-up and prognosis are described as well in this case report.

  3. Molecular biology of sarcomas.

    PubMed

    Gebhardt, M C

    1996-07-01

    There has been a virtual explosion of information relating to the biology of sarcomas with which we as orthopaedists deal. Much more is yet to be learned. These findings will teach us more about the etiology of these tumors. More important, the findings will alter the way in which these tumors are treated. It is unlikely that we will continue to treat osteosarcoma or Ewing's sarcoma patients with currently available drug regimens and surgery or make treatment decisions based on the histologic classification of tumors we know today. If we are to remain active in the management of these patients we must be aware of the findings as they occur. That will ensure both that we remain the primary caretakers of these patients, and that we will continue to be stimulated intellectually by these intriguing scientific investigations.

  4. Pseudo-Kaposi sarcoma (acroangiodermatitis): occurring after bullous erysipelas.

    PubMed

    Kutlubay, Zekayi; Yardimci, Gürkan; Engin, Burhan; Demirkesen, Cuyan; Aydin, Övgü; Khatib, Rashid; Tuzun, Yalçın

    2015-05-18

    Pseudo-Kaposi sarcoma is a benign reactive vascular proliferative disorder, which can be seen at any age. It occurs when the chronic venous pressure changes result in vascular proliferation in the upper and mid dermis. This disease is divided into two subtypes: the most frequent subtype is the Mali type and seen in early ages. The Mali type is seen in chronic venous insufficiency and in those patients with arteriovenous shunts. The rare subtype is the Stewart-Bluefarb type. This disease must be distinguished from Kaposi sarcoma because of their clinical resemblance. Herein, we present a patient with pseudo-Kaposi sarcoma, which developed after bullous erysipelas.

  5. Sarcoma epidemiology and etiology: potential environmental and genetic factors.

    PubMed

    Lahat, Guy; Lazar, Alexander; Lev, Dina

    2008-06-01

    Sarcomas are a heterogeneous group of tumors that may have many etiologies. The incidence of histologic subtypes differs significantly between children and adults. The increase in incidence may be due to improved registry systems, diagnostic tools, and pathologic definitions. Environmental causes may contribute to increased incidence. Genetic alternations may play a role in sarcoma development. As a result of rapidly evolving genomic and proteomic technologies, increased knowledge of the oncogenic mechanisms underlying sarcomagenesis is being generated. Understanding the mechanisms involved in sarcomagenesis is rudimentary. Insight into the molecular basis of sarcoma inception, proliferation, and dissemination hopefully will lead to more effective therapies.

  6. Robotic retroperitoneal partial nephrectomy: a four-arm approach.

    PubMed

    Feliciano, Joseph; Stifelman, Michael

    2012-01-01

    Robotic partial nephrectomy is an effective alternative to laparoscopic partial nephrectomy. The 3-arm and 4-arm transperitoneal robotic approaches are well described in the literature. However, a retroperitoneal robotic technique has yet to be fully described. We report our technique and initial experience with robotic retroperitoneal partial nephrectomy with a novel 4-arm approach. We reviewed our current experience with the robotic retroperitoneal approach. Descriptive statistics on patient characteristics, operative parameters, and oncologic outcomes are reported. A total of 67 robotic-assisted partial nephrectomies were performed by one surgeon between October 2009 and October 2010. The 4-arm retroperitoneal approach was used in 8 patients (12%) with no complications. Median tumor size was 2cm. All were posterior renal tumors, with 5 located in the upper pole. The median operative time, warm ischemia time, estimated blood loss, and length of stay were 202 minutes, 18 minutes, 100cc, and 2 days, respectively. Pathology indicated renal cell carcinoma (RCC) in 7 patients with negative margins. The 4-arm robotic approach to retroperitoneal partial nephrectomy is safe, reproducible, and easily used. The fourth arm provides optimal traction on target tissues in key maneuvers and may decrease complications and positive margins secondary to impaired exposure.

  7. Retroperitoneal fibrosis: a rare vascular and immune entity disclosed by chronic lombalgia.

    PubMed

    Blanc, Gaelle; Girard, Nicolas; Alexandre, Christian; Vignon, Eric

    2007-10-01

    Retroperitoneal fibrosis is a rare inflammatory and fibrotic process in the retroperitoneal peri-aortic tissues, associated with ureters and other abdominal organs' entrapment. Here we report an original observation of a 55-year-old patient presenting with chronic lombalgia disclosing idiopathic retroperitoneal fibrosis. After one-year follow-up, treatment with corticosteroids led to a complete clinical, biological, and radiological response. Pathogenesis and therapeutic options in idiopathic retroperitoneal fibrosis are discussed.

  8. A Retroperitoneal Isolated Enteric Duplication Cyst Mimicking a Teratoma: A Case Report and Literature Review

    PubMed Central

    Momosaka, Daichi; Ushijima, Yasuhiro; Asayama, Yoshiki; Ishigami, Kousei; Takayama, Yukihisa; Okamoto, Daisuke; Fujita, Nobuhiro; Ikeda, Tetsuo; Uchida, Keiichiro; Sugimoto, Masaaki; Kohashi, Kenichi; Honda, Hiroshi

    2016-01-01

    Enteric duplication cysts lacking anatomic association with the gastrointestinal tract are called isolated enteric duplication cysts (IEDCs). We present an atypical case of a retroperitoneal IEDC with a tortuous tubular complex shape that enfolded the surrounding retroperitoneal fat and mimicked a retroperitoneal teratoma. Multiplanar reconstruction images should be used to evaluate such a lesion correctly. A tortuous tubular complex shape could be a key finding to differentiate from other retroperitoneal cysts. PMID:28083153

  9. 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

  10. Massive retroperitoneal hematoma as a complication of anticoagulation therapy in a patient treated in a pulmonary intensive care unit.

    PubMed

    Stjepanović, Mihailo; Buha, Ivana; Raljević, Snezana; Babić, Uroš; Savić, Milan; Mašković, Jovana; Roksandić, Marina; Marić, Dragana

    2015-06-01

    Retroperitoneal hematoma may occur as a result of trauma, but also from rapture of arterial aneurysms (aortic or iliac), surgical complications, tumors or anticoagulation therapy. We presented a patient on permanent anticoagulation therapy. On the day of admission to our institudon, the patient had the value of his INR 5.57 which required immediate suspension of the therapy. The main symptom in this patient was pain in the right inguinal canal with propagation along the right leg, which was indicated in clinical picture of spontaneous retroperitoneal haematoma. After three days the fall of hemoglobin occurred, so the additonal diagnostics was done. A computed tomography of the abdomen was performed showing well limited, large retroperitoneal hematoma (213 x 79 x 91 mm). Transfusion of concentrated red blood cells was performed twice with satisfactory correction of hemoglobin level, and four units of fresh frozen plasma. The patient was hemodynamically stabilized and discharged after a two-month long intensive care unit treatment, with the advice to use low-molecular weight heparin 2 x 0.4 mg subcutaneusly, due to persistent arrhythmia. In patients on anticoagulation therapy regular monitoring of the anticoagulant status is extremely important, because of the possibility of fatal complications development, such as retroperitoneal hematoma.

  11. Routes to diagnosis for sarcoma - Describing the sarcoma patient journey.

    PubMed

    Gerrand, C; Francis, M; Dennis, N; Charman, J; Lawrence, G; Evans, T; Grimer, R

    2015-10-01

    Understanding the route to diagnosis for patients with cancer is important to improve the diagnostic pathway and therefore outcomes. We aimed to investigate routes to diagnosis for patients with sarcoma in England. National patient level datasets relating to 7716 soft tissue and 1240 bone sarcoma patients diagnosed between 2006 and 2008 were analysed. Routes to diagnosis were defined as: "Two Week Wait Referral", "GP Referral", "Other Outpatient", "Inpatient Elective", "Emergency Presentation", "Death Certificate Only" and "Unknown". Patients with sarcoma are most likely to be diagnosed after "GP Referral" or "Emergency Presentation" and are less likely to be referred under a two week wait compared with other malignancies. Patients with bone sarcoma under 10 or over 80 years of age were more likely to present by emergency routes, as were patients with vertebral column, pelvis or unspecified site tumours and those with Ewing's sarcoma or sarcoma NOS. Patients with soft tissue sarcoma under 19 or over 80 years of age and patients with GI tract tumours were more likely to present by emergency routes. Patients presenting by emergency routes more often had metastases and had lower 1 year survival. Patients from least deprived quintiles more often presented by unknown routes: those from more deprived quintiles more often presented by emergency routes. Routes to diagnosis for sarcoma patients differ from other cancers. Interventions to improve the diagnostic experience should consider the very young and elderly, tumours in abdominal, pelvic or spinal locations and on reducing emergency presentations. Crown Copyright © 2015. Published by Elsevier Ltd. All rights reserved.

  12. Percutaneous Radiofrequency Ablation for Treatment of Recurrent Retroperitoneal Liposarcoma

    SciTech Connect

    Keil, Sebastian Bruners, Philipp; Brehmer, Bernhard; Mahnken, Andreas Horst

    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 performed 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.

  13. Laparotomized Direct Puncture for Embolization of a Retroperitoneal Arteriovenous Fistula

    SciTech Connect

    Inagawa, Shoichi; Unno, Naoki; Yamashita, Shuhei; Tanaka, Hiroki; Sakahara, Harumi

    2010-02-15

    A 28-year-old woman was referred to our institution with hope for another child after having an abortion several months previously to avoid a potential risk of catastrophic hemorrhage from a retroperitoneal arteriovenous fistula (AVF) with enlarged and twisted draining veins in the pelvis. Multiple branches coming from the right lumbar arteries and the right iliac arteries fed fistulae converging on an enlarged venous pouch anterior to the psoas major muscle in the right retroperitoneal space. It seemed impossible to achieve complete occlusion of the lesion in a single session by either transarterial or transvenous approach. A laparotomy and direct puncture of the enlarged draining vein immediately downstream of the venous pouch was performed and embolization was done with n-butyl cyanoacrylate and the aid of coils. Complete occlusion of the retroperitoneal AVF was achieved and confirmed in control angiography 5 months later.

  14. Guidelines for time-to-event end point definitions in sarcomas and gastrointestinal stromal tumors (GIST) trials: results of the DATECAN initiative (Definition for the Assessment of Time-to-event Endpoints in CANcer trials)†.

    PubMed

    Bellera, C A; Penel, N; Ouali, M; Bonvalot, S; Casali, P G; Nielsen, O S; Delannes, M; Litière, S; Bonnetain, F; Dabakuyo, T S; Benjamin, R S; Blay, J-Y; Bui, B N; Collin, F; Delaney, T F; Duffaud, F; Filleron, T; Fiore, M; Gelderblom, H; George, S; Grimer, R; Grosclaude, P; Gronchi, A; Haas, R; Hohenberger, P; Issels, R; Italiano, A; Jooste, V; Krarup-Hansen, A; Le Péchoux, C; Mussi, C; Oberlin, O; Patel, S; Piperno-Neumann, S; Raut, C; Ray-Coquard, I; Rutkowski, P; Schuetze, S; Sleijfer, S; Stoeckle, E; Van Glabbeke, M; Woll, P; Gourgou-Bourgade, S; Mathoulin-Pélissier, S

    2015-05-01

    The use of potential surrogate end points for overall survival, such as disease-free survival (DFS) or time-to-treatment failure (TTF) is increasingly common in randomized controlled trials (RCTs) in cancer. However, the definition of time-to-event (TTE) end points is rarely precise and lacks uniformity across trials. End point definition can impact trial results by affecting estimation of treatment effect and statistical power. The DATECAN initiative (Definition for the Assessment of Time-to-event End points in CANcer trials) aims to provide recommendations for definitions of TTE end points. We report guidelines for RCT in sarcomas and gastrointestinal stromal tumors (GIST). We first carried out a literature review to identify TTE end points (primary or secondary) reported in publications of RCT. An international multidisciplinary panel of experts proposed recommendations for the definitions of these end points. Recommendations were developed through a validated consensus method formalizing the degree of agreement among experts. Recommended guidelines for the definition of TTE end points commonly used in RCT for sarcomas and GIST are provided for adjuvant and metastatic settings, including DFS, TTF, time to progression and others. Use of standardized definitions should facilitate comparison of trials' results, and improve the quality of trial design and reporting. These guidelines could be of particular interest to research scientists involved in the design, conduct, reporting or assessment of RCT such as investigators, statisticians, reviewers, editors or regulatory authorities. © The Author 2014. Published by Oxford University Press on behalf of the European Society for Medical Oncology. All rights reserved. For permissions, please email: journals.permissions@oup.com.

  15. Matrix Metalloproteinase 1: Role in Sarcoma Biology

    PubMed Central

    Jawad, Muhammad Umar; Garamszegi, Nandor; Garamszegi, Susanna P.; Correa-Medina, Mayrin; Diez, Juan A.; Wen, Rong; Scully, Sean P.

    2010-01-01

    In carcinomas stromal cells participate in cancer progression by producing proteases such as MMPs. The expression MMP1 is a prognostic factor in human chondrosarcoma, however the role in tumor progression is unknown. Laser capture microdissection and In Situ hybridization were used to determine cellular origin of MMP1 in human sarcomas. A xenogenic model of tumor progression was then used and mice were divided in two groups: each harboring either the control or a stably MMP1 silenced cell line. Animals were sacrificed; the neovascularization, primary tumor volumes, and metastatic burden were assessed. LCM and RNA-ISH analysis revealed MMP1 expression was predominantly localized to the tumor cells in all samples of sarcoma (p = 0.05). The percentage lung metastatic volume at 5 weeks (p = 0.08) and number of spontaneous deaths secondary to systemic tumor burden were lower in MMP1 silenced cell bearing mice. Interestingly, this group also demonstrated a larger primary tumor size (p<0.04) and increased angiogenesis (p<0.01). These findings were found to be consistent when experiment was repeated using a second independent MMP1 silencing sequence. Prior clinical trials employing MMP1 inhibitors failed because of a poor understanding of the role of MMPs in tumor progression. The current findings indicating tumor cell production of MMP1 by sarcoma cells is novel and highlights the fundamental differences in MMP biology between carcinomas and sarcomas. The results also emphasize the complex roles of MMP in tumor progression of sarcomas. Not only does metastasis seem to be affected by MMP1 silencing, but also local tumor growth and angiogenesis are affected inversely. PMID:21170377

  16. [A case of seronegative spondylarthropathy with iritis and retroperitoneal fibrosis].

    PubMed

    Takahashi, M; Ishikawa, A; Kondo, H

    1999-02-01

    In 1985 a 41-year old male visited a local hospital because of congestion in the bulbar conjunctiva, which was diagnosed as iritis. In August 1990, right coxalgia and arthralgia of metatarsophalangeal joints appeared, with recurrence of iritis. In October, stiffness in the hands and arthralgia of proximal interphalangeal joints also started. In July 1991, the right coxalgia worsened, resulting in walking difficulty. He was admitted to the Kitasato University Hospital. He presented with bilateral iritis, polyarthritis with limited ranges of motion and sacroilitis. The Schober's test was positive at 3 cm. Serological tests for rheumatoid factor and HLA-B 27 were negative. Abdominal computer tomographic scan revealed low density lesion around the aorta. PSL 10 mg was initiated, and iritis and arthritis remitted. Progression of the periaortic lesion was not observed during the subsequent 5 years. In this case, iritis preceded limited ranges of motion in the vertebrae and sacroilitis. From these findings, seronegative spondylarthropathy with peripheral arthritis was diagnosed. The periaortic lesion seen in this case probably corresponds to chronic periaortitis recently reported as a subset of idiopathic retroperitoneal fibrosis. The two lesions observed in the present case may be interpreted as caused by inflammation of the connective tissue initially either at the vertebrae or around the aorta, which had advanced to involve the other lesion.

  17. 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.

  18. Surgical treatment of uterine sarcoma.

    PubMed

    Nam, Joo-Hyun

    2011-12-01

    Uterine sarcomas are rare, heterogeneous malignant tumours of several histologic types originating from mesenchymal tissues of the uterus. The most common histologic types are carcinosarcoma, leiomyosarcoma, and endometrial stromal sarcoma, accounting for 90% of uterine sarcomas. To date, no effective treatment has been found to achieve a high rate of cure or prolong survival. Although complete surgical excision of the tumour is the only curative treatment modality, the rarity of these tumours and their diversity of histologic types have precluded the development of standard surgical strategies. Surgery may also be optimal for recurrent uterine sarcomas, but indications for secondary surgical treatment have not been established. Here, we describe recent changes in, and updates of, the surgical treatment of the three most common types of malignant uterine sarcomas.

  19. Surgery for retroperitoneal relapse in the setting of a prior retroperitoneal lymph node dissection for germ cell tumor

    PubMed Central

    Gotto, Geoffrey T.; Carver, Brett S.; Sogani, Pramod; Sheinfeld, Joel

    2010-01-01

    Recognition of the therapeutic role of retroperitoneal lymph node dissection (RPLND) in the setting of testicular germ cell tumors (GCTs) is of utmost importance. Although the histologic findings of RPLND provide diagnostic and prognostic information, the adequacy of initial RPLND is an independent predictor of clinical outcome. Despite the advent of effective cisplatin-based chemotherapy for testicular GCTs, patients who have undergone suboptimal surgery at the time of initial RPLND are compromised. Despite the initial enthusiasm surrounding anatomic mapping studies, the use of modified RPLND templates has the potential to leave a significant number of patients with unresected retroperitoneal disease. Teratomatous elements are particularly common. Patients with retroperitoneal relapse following initial RPLND should be treated with reoperative RPLND and chemotherapy and can expect long term survival rates nearing 70% when treated in tertiary centers by experienced surgeons. PMID:20535295

  20. 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

  1. Impact of hemiscrotectomy on outcome of patients with embryonal paratesticular rhabdomyosarcoma: results from the Cooperative Soft Tissue Sarcoma Group Studies CWS-86, 91, 96 and 2002P.

    PubMed

    Seitz, Guido; Dantonello, Tobias M; Kosztyla, Daniel; Klingebiel, Thomas; Leuschner, Ivo; Fuchs, Jörg; Koscielniak, Ewa

    2014-09-01

    Children with paratesticular rhabdomyosarcoma have a favorable prognosis. Surgical treatment problems include inadequate primary transscrotal approaches, incomplete tumor resections and the need for secondary hemiscrotectomy. We evaluated the need for hemiscrotectomy regarding local relapse and outcome. A total of 173 patients with a diagnosis of paratesticular rhabdomyosarcoma were enrolled in the Cooperative Soft Tissue Sarcoma Studies between 1986 and 2008. Of the patients 17 were excluded due to an incomplete data set and alveolar histology. Thus, a total of 156 patients with embryonal subtype were analyzed. All patients were treated according to study protocols, which included multiagent chemotherapy, tumor resection and/or radiotherapy. Mean ± SD 5-year overall survival rate was 91.5% ± 2.4% for patients with embryonal rhabdomyosarcoma. A total of 28 patients underwent transscrotal approaches initially. Of these patients 12 were treated with hemiscrotectomy (mean ± SD 5-year event-free survival 91.7% ± 8%) and 16 without hemiscrotectomy (93.8% ± 6.1%). Additionally 13 of 156 patients underwent an inguinal approach with hemiscrotectomy due to suspicious tumor infiltration of the scrotal skin (mean ± SD 5-year event-free survival 84.6% ± 10%). Relapse was observed in 3 of 12 patients after transscrotal approach with hemiscrotectomy (locoregional lymph node in 1 and metastasis in 2). One metastatic relapse was observed in the group undergoing a transscrotal approach without hemiscrotectomy. One of 13 patients treated with an inguinal approach and hemiscrotectomy had locoregional relapse and died of disease. Hemiscrotectomy seems not to be mandatory in patients after transscrotal approaches regarding outcome and local relapse. Nevertheless, hemiscrotectomy probably should be performed if the scrotal skin is infiltrated. Copyright © 2014 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

  2. Immunostaining for SYT protein discriminates synovial sarcoma from other soft tissue tumors: analysis of 146 cases.

    PubMed

    He, Rui; Patel, Rajiv M; Alkan, Serhan; Hammadeh, Rasheed; Weiss, Sharon W; Goldblum, John R; Venkataraman, Girish; Baila, Horea

    2007-05-01

    Synovial sarcoma in its classic biphasic form can be distinguished readily from other soft tissue lesions; however, monophasic and poorly differentiated forms are diagnostically more problematic. For this reason, we assessed the efficacy of immunostaining for SYT and SSX1 proteins, the gene products resulting from unique synovial sarcoma translocation, to distinguish synovial sarcoma from other soft tissue lesions. A total number of 146 cases were analyzed, including 47 synovial sarcoma cases (all of which were verified by FISH to have t(X; 18) translocation and SYT-SSX fusion gene) and 99 soft tissue tumors of various types. A polyclonal IgG antibody against SYT was used to stain formalin-fixed paraffin embedded tissues. Forty-one out of 47 (87%) synovial sarcoma displayed strong positive nuclear staining (ranging from 80 to 90% of the tumor cells) for SYT antibody. Nineteen of 99 (19%) non-synovial sarcoma cases showed variable nuclear and cytoplasmic staining with SYT, which ranged from 20 to 60% of tumor nuclei, and included malignant peripheral nerve sheath tumor (5/25), solitary fibrous tumor (2/14), Ewing sarcoma (2/6), low grade fibromyxoid tumor (2/4), extraskeletal mesenchymal chondrosarcoma (2/6), gastrointestinal tumor (4/17), epithelioid sarcoma (2/2). The remaining non-synovial sarcomas were negative. This is the first study demonstrating SYT protein expression in tissue sections of synovial sarcoma. This method could provide an easy, rapid and widely applicable means of assisting in the diagnosis of synovial sarcoma, particularly when material and/or resources are unavailable for PCR or FISH-based testing. However, as variable weak staining for SYT may be encountered in a small percentage of non-synovial sarcoma sarcomas, a positive interpretation should be made only when the staining is strong, nuclear and present in the majority of cells.

  3. 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

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

    ClinicalTrials.gov

    2016-11-21

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

  5. Usefulness of F-18 FDG PET/CT in a case of Kaposi sarcoma with an unexpected bone lesion.

    PubMed

    Morooka, Miyako; Ito, Kimiteru; Kubota, Kazuo; Yanagisawa, Kunio; Teruya, Katsuji; Hasuo, Kahehiro; Shida, Yoshitaka; Minamimoto, Rhogo; Kikuchi, Yoshimi; Oka, Shinichi

    2011-03-01

    Bone lesions of Kaposi sarcoma are rare. A 56-year-old man who was HIV positive and was diagnosed with Kaposi sarcoma on the basis of the results of a biopsy of skin lesions, underwent F-18 FDG PET/CT scan for detecting Kaposi sarcoma lesions and other AIDS-related diseases. An abnormal uptake was observed in the lumbar spine. MRI showed a diffuse enhanced spine lesion, and Ga-67 and ²⁰¹Tl scanning were negative. As a result, the lesion was considered to be a Kaposi sarcoma, and the shrinkage of the lesion was noted after the therapy for Kaposi sarcoma.

  6. 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.

  7. Hepatocyte Growth Factor-mediated satellite cells niche perturbation promotes development of distinct sarcoma subtypes.

    PubMed

    Morena, Deborah; Maestro, Nicola; Bersani, Francesca; Forni, Paolo Emanuele; Lingua, Marcello Francesco; Foglizzo, Valentina; Šćepanović, Petar; Miretti, Silvia; Morotti, Alessandro; Shern, Jack F; Khan, Javed; Ala, Ugo; Provero, Paolo; Sala, Valentina; Crepaldi, Tiziana; Gasparini, Patrizia; Casanova, Michela; Ferrari, Andrea; Sozzi, Gabriella; Chiarle, Roberto; Ponzetto, Carola; Taulli, Riccardo

    2016-03-17

    Embryonal Rhabdomyosarcoma (ERMS) and Undifferentiated Pleomorphic Sarcoma (UPS) are distinct sarcoma subtypes. Here we investigate the relevance of the satellite cell (SC) niche in sarcoma development by using Hepatocyte Growth Factor (HGF) to perturb the niche microenvironment. In a Pax7 wild type background, HGF stimulation mainly causes ERMS that originate from satellite cells following a process of multistep progression. Conversely, in a Pax7 null genotype ERMS incidence drops, while UPS becomes the most frequent subtype. Murine EfRMS display genetic heterogeneity similar to their human counterpart. Altogether, our data demonstrate that selective perturbation of the SC niche results in distinct sarcoma subtypes in a Pax7 lineage-dependent manner, and define a critical role for the Met axis in sarcoma initiation. Finally, our results provide a rationale for the use of combination therapy, tailored on specific amplifications and activated signaling pathways, to minimize resistance emerging from sarcomas heterogeneity.

  8. Granulocytic sarcoma of pediatric head and neck: an institutional experience.

    PubMed

    Roby, Brianne Barnett; Drehner, Dennis; Sidman, James D

    2013-08-01

    To demonstrate a case series of granulocytic sarcoma of the head and neck found in the pediatric population and review long-term outcomes. A pathology database at a tertiary hospital was searched for patients with biopsy specimens from the head and neck diagnosed as granulocytic sarcoma. There were 6 cases between 1992 and 2004 that met inclusion criteria. Subjects' age ranged from 22 months to 14 years old. In three cases, the patients were diagnosed with acute myeloid leukemia (AML) based on biopsy results; 2 patients were already diagnosed with AML when diagnosed with granulocytic sarcoma, and in 1 case, a relapse of AML was diagnosed. In all cases, patients began induction chemotherapy. Two patients died during induction chemotherapy from infection. The remaining 4 patients underwent bone marrow transplants. One patient had a relapse post-transplant and died. Only one patient was healthy two years post-transplant. The results of this series suggest granulocytic sarcoma must be on the differential when tumors present in the head and neck region in pediatric patients. In our series, 100% of the patients with granulocytic sarcoma had underlying AML. The long-term prognosis of patients with AML who developed granulocytic sarcoma is quite poor. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

  9. Surgical management of abdominal and retroperitoneal Castleman's disease

    PubMed Central

    Bucher, Pascal; Chassot, Gilles; Zufferey, Guillaume; Ris, Frederic; Huber, Olivier; Morel, Philippe

    2005-01-01

    Background Abdominal and retroperitoneal Castleman's disease could present either as a localized disease or as a systemic disease. Castleman's disease is a lymphoid hyperplasia related to human Herpes virus type 8, which could have an aggressive behavior, similar to that of malignant lymphoid neoplasm mainly with the systemic type, or a benign one in its localized form. Methods The authors report two cases of localized Castleman's disease in the retroperitoneal space and review the current and recent progress in the knowledge of this atypical disease. Cases presentation The two patients were young healthy women presenting with a hyper vascular peri-renal mass suggestive of malignant tumor. Both have been resected in-toto. One of them had an extensive resection with nephrectomy, while the second had a kidney preserving surgery. Pathological examination revealed localized Castleman's disease and surgical margins were free of disease. Postoperative course was uneventful, and after more than 5-years of follow-up no recurrences have been observed. Conclusion Localized Castleman's disease should be considered when facing a solid hypervascular abdominal or retroperitoneal mass. A better knowledge of this disorder and its characteristic would help surgeon to avoid unnecessarily extensive resection for this benign disorder when dealing with abdominal or retroperitoneal tumors. Surgical resection is curative for the localized form, when complete, while splenectomy could be indicated for the systemic form. PMID:15941478

  10. Retroperitoneal bronchogenic cyst mimicking hydatid liver: a case report.

    PubMed

    Parray, Fazl Q; Sherwani, Afak Yusuf; Dangroo, Sajad Ahmad; Bisati, Rafia Aziz; Malik, Nighat Shaffi

    2012-01-01

    Bronchogenic cysts frequently occur in the mediastinum. They may be rarely encountered in the abdomen and retroperitoneum. Bronchogenic cysts can in fact mimic hydatid cysts. We report a case of retroperitoneal bronchogenic cyst below the right hemidiaphragm mimicking a hydatid cyst of the liver in a 30-year-old female.

  11. Retroperitoneal Bronchogenic Cyst Mimicking Hydatid Liver: A Case Report

    PubMed Central

    Parray, Fazl Q.; Sherwani, Afak Yusuf; Dangroo, Sajad Ahmad; Bisati, Rafia Aziz; Malik, Nighat Shaffi

    2012-01-01

    Bronchogenic cysts frequently occur in the mediastinum. They may be rarely encountered in the abdomen and retroperitoneum. Bronchogenic cysts can in fact mimic hydatid cysts. We report a case of retroperitoneal bronchogenic cyst below the right hemidiaphragm mimicking a hydatid cyst of the liver in a 30-year-old female. PMID:22606600

  12. Rare Case of Retroperitoneal Lipomatosis in an Indian Woman

    PubMed Central

    Patil, Saurabh S; Shah, Vaibhav R; Choudhary, Vivek A

    2016-01-01

    Retroperitoneal lipomatosis is a rare but distinct clinicopathological entity characterized by non encapsulated lipoma development in the retroperitoneum. Presenting complaints in the early stages are vague, and patients with retroperitoneal lipomatosis are often misdiagnosed because considerable abnormality is not detected by abdomino-pelvic sonography. However, because of the progressive nature of this lesion, most patients eventually present with varying degrees of urinary outflow obstruction and end stage renal disease, or bladder malignancies in few cases. Here we report a case of a 35-year-old Indian woman presenting with complaints of diffuse lumps in the abdomen and constipation. Based on the findings of the imaging [sonography and computed tomography (CT) scan] studies, benign retroperitoneal lipomatosis was preopreratively diagnosed and a confirmatory exploratory laprotomy was performed. Furthermore, we discuss the imaging findings obtained using various radiological modalities such as plain radiographs, intravenous urography, barium enema, sonography, CT and magnetic resonance imaging (MRI). We also discuss the etiopathogenesis, demographics, and various differential diagnoses of retroperitoneal lipomatosis. PMID:27200169

  13. Postcatheterization Retroperitoneal Hematoma Due to Spontaneous Lumbar Arterial Hemorrhage

    SciTech Connect

    Kalinowski, E. Anthony; Trerotola, Scott O.

    1998-07-15

    A patient developed retroperitoneal hemorrhage after cardiac catheterization, initially thought to be a complication of the puncture. Diagnostic evaluation revealed the source to be spontaneous bleeding from a lumber artery, which was successfully embolized. Spontaneous hemorrhage is a recognized complication of anticoagulation therapy and must be considered in the differential diagnosis even in the face of a seemingly obvious source of bleeding.

  14. A retroperitoneal neuroendocrine tumor in ectopic pancreatic tissue.

    PubMed

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

    2013-07-01

    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.

  15. 'Pipe-organ'-like retroperitoneal drainage in severe necrotizing pancreatitis.

    PubMed

    Sakamoto, Yuichiro; Mashiko, Kunihiro; Matsumoto, Hisashi; Hara, Yoshiaki; Kutsukata, Noriyoshi; Yokota, Hiroyuki

    2010-01-01

    Infected pancreatic necrosis is a severe complication of acute pancreatitis; this complication is the major cause of death. We used the 'pipe-organ' -like retroperitoneal drainage, which is usually used in the management of open pelvic fracture, successfully in a patient with severe infected pancreatic necrosis. This procedure can avoid gastrointestinal fistula and local bleeding without necessitating surgery.

  16. Primary retroperitoneal Merkel cell carcinoma: Case report and literature review

    PubMed Central

    Quiroz-Sandoval, Osvaldo A.; Cuellar-Hubbe, Mario; Lino-Silva, Leonardo S.; Salcedo-Hernández, Rosa A.; López-Basave, Horacio N.; Padilla-Rosciano, Alejandro E.; León-Takahashi, Alberto M.; Herrera-Gómez, Ángel

    2015-01-01

    Background Merkel cell carcinoma (MCC) is an aggressive cutaneous neuroendocrine carcinoma that affects elderly patients and typically arises in sun-exposed skin. The disease is very rare and only few cases present with no apparent skin lesion. In the retroperitoneum there are only two cases reported in the literature. Case presentation We report a case of a 54-year-old Mexican male with MCC, which presented as a large retroperitoneal mass. Pathological and immunohistochemical analysis of the transabdominal CT-guided biopsy specimen revealed a MCC. The patient underwent preoperative chemotherapy followed by a laparotomy and the mass was successfully excised. Discussion There are two possible explanations for what occurred in our patient. The most plausible theory is the retroperitoneal mass could be a massively enlarged lymph node where precursor cells became neoplastic. This would be consistent with a presumptive diagnosis of primary nodal disease. Moreover, metastasis to the retroperitoneal lymph nodes has been reported as relatively common when compared to other sites such as liver, bone, brain and skin. The less probable theory is the non-described “regression” phenomena of a cutaneous MCC, but we are not found a primary skin lesion. Conclusion Preoperative chemotherapy and excision of the primary tumor is the surgical treatment of choice for retroperitoneal MCC. We propose that further studies are needed to elucidate the true efficacy of chemotherapy in conventional and unconventional patients with MCC. PMID:26708276

  17. Demonstration of the route of embryo migration in retroperitoneal ectopic pregnancy using contrast-enhanced computed tomography.

    PubMed

    Liang, Changhu; Li, Xueli; Zhao, Bin; Du, Yinglin; Xu, Shifeng

    2014-03-01

    Retroperitoneal abdominal pregnancy is exceptionally rare. An unusual retroperitoneal pregnancy has a high risk of severe bleeding. Abdominal ultrasonography and whole abdominal computerized tomography scanning should be performed for the early diagnosis of ectopic pregnancy. A case of retroperitoneal ectopic pregnancy located very close to large retroperitoneal blood vessels and treated with resection is presented. Many unusual features of retroperitoneal ectopic pregnancy were highlighted as providing further evidence in support of the main proposed embryo migration mechanism via lymphatic vessels.

  18. MicroRNAs as potential target in human bone and soft tissue sarcoma therapeutics

    PubMed Central

    Varshney, Jyotika; Subramanian, Subbaya

    2015-01-01

    Sarcomas are highly aggressive heterogeneous tumors that are mesenchymal in origin. There have been vast advancements on identifying diagnostic markers for sarcomas including chromosomal translocations, but very little progress has been made to identify targeted therapies against them. The tumor heterogeneity, genetic complexity and the lack of drug studies make it challenging to recognize the potential targets and also accounts for the inadequate treatments in sarcomas. In recent years, microRNAs that are a part of small non-coding RNAs have shown promising results as potential diagnostic and prognostic biomarkers in multiple sarcoma types. This review focuses on the current knowledge of the microRNAs that are deregulated in sarcomas, and an insight on the strategies to target these microRNAs that are essential for developing improved therapies for various human sarcomas. PMID:26137468

  19. Kaposi sarcoma herpesvirus pathogenesis

    PubMed Central

    Koch, Sandra; Schulz, Thomas F.

    2017-01-01

    Kaposi sarcoma herpesvirus (KSHV), taxonomical name human gammaherpesvirus 8, is a phylogenetically old human virus that co-evolved with human populations, but is now only common (seroprevalence greater than 10%) in sub-Saharan Africa, around the Mediterranean Sea, parts of South America and in a few ethnic communities. KSHV causes three human malignancies, Kaposi sarcoma, primary effusion lymphoma, and many cases of the plasmablastic form of multicentric Castleman's disease (MCD) as well as occasional cases of plasmablastic lymphoma arising from MCD; it has also been linked to rare cases of bone marrow failure and hepatitis. As it has colonized humans physiologically for many thousand years, cofactors are needed to allow it to unfold its pathogenic potential. In most cases, these include immune defects of genetic, iatrogenic or infectious origin, and inflammation appears to play an important role in disease development. Our much improved understanding of its life cycle and its role in pathogenesis should now allow us to develop new therapeutic strategies directed against key viral proteins or intracellular pathways that are crucial for virus replication or persistence. Likewise, its limited (for a herpesvirus) distribution and transmission should offer an opportunity for the development and use of a vaccine to prevent transmission. This article is part of the themed issue ‘Human oncogenic viruses’. PMID:28893942

  20. RUNX3 facilitates growth of Ewing sarcoma cells.

    PubMed

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

    2014-12-01

    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.

  1. 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

  2. Retroperitoneal fibrosis associated with immunoglobulin g4-related disease in the differential diagnosis in retroperitoneal tumors. Case report.

    PubMed

    Ruiz Mar, Gabriela; Cárdenas Serrano, Óscar E; Roldan García, Jorge; Cañavera-Constantino, A; Menéndez Trejo, Víctor M; Chapa Azuela, Óscar

    2017-07-19

    The patient was a 55-year-old woman with a 4-month history of pain in left flank that irradiated to the lumbar region. Computed tomography revealed a cystic tumor in the retroperitoneal region that compresses the ureter and left renal pelvis in contact with pancreatic body and tail. Surgery was performed and the definitive pathological report diagnosed nodular retroperitoneal fibrosis associated with IgG4; Ki67-positive in germinal centers (5%) and IgG4-positive (40 plasma cells in 3 fields of 40x) by immunohistochemistry. Retroperitoneal fibrosis is a rare disease, that develops gradually and has an excellent response to steroid management. Surgical treatment is reserved for cases that compromise adjacent structures. Thus, identifying it when studying a retroperitoneal tumor leads to better prognosis and survival. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  3. What Should You Ask Your Doctor about Kaposi Sarcoma?

    MedlinePlus

    ... What Should You Ask Your Doctor About Kaposi Sarcoma? Kaposi Sarcoma Early Detection, Diagnosis, and Staging What Should You Ask Your Doctor About Kaposi Sarcoma? As you cope with Kaposi sarcoma (KS) and ...

  4. Alisertib in Treating Patients With Advanced or Metastatic Sarcoma

    ClinicalTrials.gov

    2016-11-01

    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; Stage IV Soft Tissue Sarcoma

  5. [Clinicopathologic analysis of Ewing-like BCOR-CCNB3 undifferentiated sarcoma].

    PubMed

    Wang, X J; Zhao, D H; Wang, Y M; Liu, Y X; Li, X; Li, M Y; Li, W C; Wang, Z

    2017-02-08

    Objective: To investigate the morphologic, immunohistochemical, genetic, clinical features and prognosis of Ewing-like BCOR-CCNB3 gene fusion undifferentiated sarcoma (BCOR-CCNB3 fusion sarcoma). Method: Seventeen Ewing-like sarcoma cases were screened for CCNB3 expression and BCOR-CCNB3 fusion transcripts by immunohistochemistry and RT-PCR among 260 cases of Ewing-like sarcomas collected during Jan, 2006 to Dec, 2015. Three cases of BCOR-CCNB3 fusion sarcoma were found among 17 atypical Ewing sarcomas, and follow-up were conducted. Results: The harboring of BCOR-CCNB3 fusion transcript was confirmed by RT-PCR and directly sequencing results. The three patients aged between 8 and 11 years old. Two of them were male and the other one was female. One patient achieved a complete response after chemotherapy, the other two died without chemotherapy after surgical excision in 12 months. Tumor cells in all 3 cases showed diffuse nuclear CCNB3, TLE1 and cyclin D1 positivity, while CCNB3 (0/12), TLE1 (1/12) and cyclin D1 (4/12) positivity was infrequent in the 12 cases of classical Ewing's sarcoma. The oval or plump spindle tumor cells with fine chromatin arranged in solid pattern, the nucleoli was inconspicuous. The delicate capillary networks were obvious in the tumor. Conclusion: With a detailed description of the histological spectrum, immunohistochemical features and clinical characteristic of BCOR-CCNB3 sarcoma, justify distinction from Ewing sarcoma could be possible.

  6. Improved Prognosis for Patients with Ewing Sarcoma in the Sacrum Compared with the Innominate Bones: The Scandinavian Sarcoma Group Experience.

    PubMed

    Hesla, Asle Charles; Tsagozis, Panagiotis; Jebsen, Nina; Zaikova, Olga; Bauer, Henrik; Brosjö, Otte

    2016-02-03

    Treatment of Ewing sarcoma of the pelvic bones remains one of the most difficult tasks in the treatment of bone sarcomas. Whether surgery or radiation therapy is the best local treatment is still a matter of debate. The aim of the present study was to compare sacral and nonsacral sites with regard to the treatment and outcome of pelvic Ewing sarcomas. Patients with Ewing sarcoma of the osseous pelvis diagnosed between 1986 and 2011 were identified through the Scandinavian Sarcoma Group registry. Data regarding tumor size, local treatment (surgery or radiation therapy), metastatic disease, surgical margins, local recurrence, and overall survival were analyzed. Of the 117 patients examined, eighty-eight had tumors in the innominate bones and twenty-nine, in the sacrum. Radiation therapy was the sole local treatment for 40% of the innominate bone tumors in contrast to 79% of the sacral tumors. The five-year disease-free survival rate in the latter group (66%) was greater than that in the group with tumors in the innominate bones (40%) (p = 0.02 adjusted for size). Disease-free survival among patients with Ewing sarcoma was improved when the tumor was localized in the sacrum compared with the innominate bones, where these tumors are generally larger. Local radiation therapy alone appears to result in good local tumor control and may be the treatment of choice for sacral tumors. Copyright © 2016 by The Journal of Bone and Joint Surgery, Incorporated.

  7. Nitrogenous subcutaneous emphysema caused by spray application of fibrin glue during retroperitoneal laparoscopic surgery.

    PubMed

    Matsuse, Shinji; Maruyama, Atsushi; Hara, Yoshiki

    2011-06-01

    We report a case of a patient treated by retroperitoneoscopic partial nephrectomy who developed nitrogenous subcutaneous emphysema (SCE) as a complication. The use of a nitrogen gas-pressured fibrin tissue adhesive applied as a spray caused excessively increased pressure in the closed retroperitoneal space and resulted in widespread SCE with protracted clinical course. To the best of our knowledge, this is the first report of nitrogenous SCE associated with pneumoperitoneum. The clinical significance of nitrogenous SCE is emphasized, and the risks associated with the use of fibrin glue as a spray during laparoscopic surgery are discussed.

  8. 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

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

    PubMed

    Lewin, Jeremy; 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.

  10. [Primary pharynx synovial sarcoma: a case report].

    PubMed

    Qingying, Cui; Youmei, Zhang; Shuai, Fu; Changbin, Zhang; Ming, Li

    2017-04-01

    A case of primary pharynx synovial sarcoma was reported in this paper. A 15-year-old male patient experienced painless pharyngeal swelling that gradually proliferated for 1 month. Special examination showed an 8 cm × 4 cm × 3 cm tumor located in the left pharynx and the supratonsillar crypt. Imaging tests revealed an irregular mass on the left side of the oropharynx and an unclear boundary. Immunohistochemical examination yielded the following results: epithelial membrane antigen (+), cytokeratin (CK)19 (+), CD7(+), vimentin (+), CK10(-), E-cadherin (+), B-cell lymphoma-2 (-), CD2 (-), CD10 (-), CD138 (+), CD99 (+), leukocyte common antigen (+), and Ki-67 (20%+). This condition was pathologically diagnosed as primary pharynx synovial sarcoma.

  11. 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.

  12. 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.

  13. 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.

  14. Synovial sarcoma in childhood

    SciTech Connect

    Israels, S.J.; Chan, H.S.L.; Daneman, A.; Weitzman, S.S.

    1984-04-01

    The clinical and radiologic findings in seven children with synovial sarcoma are described. The five boys and two girls had a mean age at presentation of 4.4 years. All seven had the lesion situated in an extremity. Plain radiographs in four revealed the presence of a soft-tissue mass with no calcification or bone and joint involvement. In two patients studied with computed tomography (CT), the primary lesions had peripheral irregular areas of enhancement with central areas of poor enhancement, reflecting the necrotic, cystic, and hemorrhagic changes found in the centers of these tumors. Although the exact margins of these lesions were difficult to define accurately even with intravenous contrast enhancement, CT is still recommended as the best imaging method for assessing the local extent of the primary tumor and is a useful tool in the planning of appropriate therapy as well as the gauging of the tumor response to ongoing treatment.

  15. Suprainguinal retroperitoneal approach for the successful surgical treatment of meralgia paresthetica.

    PubMed

    Alberti, Olaf; Wickboldt, Jürgen; Becker, Ralf

    2009-04-01

    Neurosurgical textbooks describe an infrainguinal approach as the standard or preferred option for the surgical treatment of meralgia paresthetica (MP), the most frequent entrapment neuropathy of the lower limb. However, inhomogeneous results led the authors to adopt a suprainguinal, retroperitoneal approach for decompression of the lateral femoral cutaneous nerve. In this paper the authors' aim was to study the outcome of patients harboring MP treated via this different surgical approach. The outcome of 55 consecutive patients who underwent surgery for MP via the suprainguinal retroperitoneal approach during a 15-year period was ascertained through postal questionnaires (in 47 patients) and follow-up visits (in 8 patients). The male to female ratio was 1:0.67, and the mean patient age was 50 +/- 12.9 years. The mean follow-up was 3.2 +/- 3.3 years. Seven of the patients underwent bilateral surgery. Intraoperatively the lateral femoral cutaneous nerve was consistently found in close anatomical relationship to the anterior superior iliac spine, although some variations regarding the diameter, number of branches, and underlying pathological entity were observed. Eighty-seven percent of patients showed improvement (21 patients) or complete remission (27 patients) of painful dysesthesia in the anterolateral thigh, and 13% (7 patients) remained unchanged. In addition 82% had improvement (31 patients) or complete remission (14 patients) of hypesthesia, leaving 18% with unchanged (9 patients) or worsened (1 patient) hypesthesia. In the patient-evaluated group 66% (31 of 47) were completely satisfied with the outcome, 23% (11 of 47) were partially satisfied, and 11% (5 of 47) were not satisfied with the outcome. Two cases each of recurrence, seroma, wound infection, and 1 case of hematoma requiring revision were encountered as complications. The suprainguinal retroperitoneal approach is a viable first-choice option for the surgical relief of MP.

  16. The surgical experience for retroperitoneal, mesenteric and omental cyst in children

    PubMed Central

    Nam, So Hyun; Kim, Dae Yeon; Kim, In Koo

    2012-01-01

    Purpose Intra-abdominal cystic masses originating from the retroperitoneum, mesentery or omentum are very rare and mostly benign tumors, but sometimes present as a complicated cyst encasing the major organs. Methods We analyzed the clinical findings, histologic diagnosis, and surgical outcomes in children who underwent operation for retroperitoneal, omental, and mesenteric cyst from 1998 to 2010, retrospectively. Results Twenty-three patients (male, 12; female, 11) underwent the operation at a median age of 46 months (range, 9 days to 16 years). Among them, 17 cysts presented one or two symptoms such as abdominal mass, abdominal pain or abdominal distension. The median duration of symptoms was 7 days (range, 1 day to 365 days). Five were detected prenatally. Ten cysts were found in retroperitoneum, 8 in the omentum and 5 in the mesentery. The median diameter was 13 cm (range, 3 to 30 cm). Twenty cysts were completely removed. Five mesenteric cysts required bowel resection and anastomosis. Three of retroperitoneal cysts were impossible to complete excise because of location and extensiveness. Pathologically, 20 cysts were lymphangioma and 3 were pseudocyst. The morbidity was one of adhesive ileus and the mortality was one who had extensive retroperitoneal cyst with mesenteric cyst. He died from sepsis. During follow-up period, there was no recurrence. Conclusion Preoperative diagnosis and localization for these cysts are very difficult. Complete excision was possible in almost all cases despite the size, bringing a favorable outcome. The possibility of this disease entity should be considered as the cause of acute abdomen. PMID:22880185

  17. Retroperitoneal and transperitoneal laparoscopic cryotherapy for small renal masses.

    PubMed

    Domínguez, A; Bellido, J A; Muñoz-Rodríguez, J; Abascal-Junquera, J M; Hannaoui, N; Banús, J M

    2015-11-01

    Cryotherapy is a minimally invasive ablative technique that is considered an alternative to conventional surgery for preserving renal function in small renal tumors and in selected cases. We present our results from laparoscopic renal cryotherapy. We retrospectively analyzed 17 renal tumors diagnosed in 16 patients treated with cryotherapy. The patients' mean age was 66 years (43-80). The mean tumor size was 1.8cm (0.7-3.7cm). Cryotherapy with double-freeze cycle was performed laparoscopically in all cases (10 by transperitoneal approach and 7 by retroperitoneal approach). Perioperative biopsies were performed on all patients and were positive for malignancy in 10 cases (59%). The mean stay was 2.8 days. The mean operative time was 162 minutes. Only 1 case reverted to open surgery due to bleeding. One patient required a blood transfusion in the immediate postoperative period. The majority of complications were Clavien-Dindo grades I and II. Some 76.5% of the patients had no complications. After a mean follow-up of 31 months (6-102), 1 patient died from nontumor-related causes, and 12 patients (75%) still show no evidence of local recurrence or progression. One patient had tumor persistence and therefore underwent partial nephrectomy at 6 months. One patient had a metachronous recurrence in the same kidney at 36 months, and another patient had a recurrence at 23 months. Laparoscopic renal cryotherapy is a safe and feasible technique and is a good alternative to surgery for selected renal tumors. Copyright © 2015 AEU. Publicado por Elsevier España, S.L.U. All rights reserved.

  18. Robot - assisted laparoscopic retroperitoneal lymph node dissection in testicular tumor

    PubMed Central

    Torricelli, Fabio C. M.; Jardim, Denis; Guglielmetti, Giuliano B.; Patel, Vipul; Coelho, Rafael F.

    2017-01-01

    ABSTRACT Introduction and objective Retroperitoneal lymph node dissection (RPLND) is indicated for patients with non-seminomatous germ cell tumor (NSGCT) with residual disease after chemotherapy. Although the gold standard approach is still the open surgery, few cases of robot-assisted laparoscopic RPLND have been described. Herein, we aim to present the surgical technique for robot-assisted laparoscopic RPLND. Patient and method A 30 year-old asymptomatic man presented with left testicular swelling for 2 months. Physical examination revealed an enlarged and hard left testis. Alpha-fetoprotein (>1000ng/mL) and beta-HCG (>24.000U/L) were increased. Beta-HCG increased to >112.000U/L in less than one month. The patient underwent a left orchiectomy. Pathological examination showed a mixed NSGCT (50% embryonal carcinoma; 30% teratoma; 10% yolk sac; 10% choriocarcinoma). Computed tomography scan revealed a large tumor mass close to the left renal hilum (10x4x4cm) and others enlarged paracaval and paraortic lymph nodes (T2N3M1S3-stage III). Patient was submitted to 4 cycles of BEP with satisfactory response. Residual mass was suggestive of teratoma. Based on these findings, he was submitted to a robot-assisted RPLND. Results RPLND was uneventfully performed. Operative time was 3.5 hours. Blood loss was minimal, and there were no intra- or postoperative complications. The patient was discharged from hospital in the 1st postoperative day. Pathological examination showed a pure teratoma. After 6 months of follow-up, patient is asymptomatic with an alpha-fetoprotein of 2.9ng/mL and an undetectable beta-HCG. Conclusion Robot-assisted laparoscopic RPLND is a feasible procedure with acceptable morbidity even for post chemotherapy patients when performed by an experienced surgeon.

  19. Myeloid Sarcoma in the Orbit.

    PubMed

    Qian, Xiaoxiao; Gigantelli, James W; Abromowitch, Minnie; Morgan, Linda A; Suh, Donny W

    2016-12-08

    The authors describe a case of myeloid sarcoma of the orbit in a pediatric patient. An 8-month-old male infant presented to the ophthalmology clinic with a left orbital mass, which had been increasing in size over the previous 2 months. The mass was initially diagnosed at another clinic as an infantile hemangioma, and had been treated with a topical formulation of timolol. In the ophthalmology clinic, orbital magnetic resonance imaging showed a solid enhancing mass. A biopsy was performed, and histopathology revealed myeloid sarcoma. The disease responded well to a standard chemotherapy regimen. Myeloid sarcoma is a rare, extra-medullary presentation that can occur as an isolated tumor, concurrently with or at relapse of acute myeloid leukemia. Because few cases of myeloid sarcoma in the orbit have been reported, this case report aids in the management of myeloid sarcoma in pediatric patients. The report describes an 8-month-old male infant, the youngest patient to develop myeloid sarcoma without preexisting acute myeloid leukemia. [J Pediatr Ophthalmol Strabismus. 2016;53:e64-e68.].

  20. 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

  1. 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.

  2. 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

  3. Feed withdrawal abate regimens lipodystrophy and metabolic syndrome symptoms, such as glucose tolerance, are associated with the diameter of retroperitoneal adipocytes in rats.

    PubMed

    He, Mao L; Sharma, Ranjana; Mir, Priya S; Okine, Erasmus; Dodson, Michael V

    2010-02-01

    Adipocyte numbers were increased by feed withdrawal (FW) regimens in cattle; thus, the effect of FW regimens was studied in male Wistar and fa/fa obese rats, as models for humans, in 2 completely randomized design experiments to abate lipodystrophy and progression of metabolic syndrome symptoms. The hypothesis was that application of FW regimens could alter adipose tissue cellularity, adipocyte size, and affect area under the curve (AUC) during glucose tolerance tests. Objectives were to determine associations among retroperitoneal and inguinal adipose tissue adipocyte number, diameter, and AUC, as affected by fortnightly or a single (at age 50 days) 24-hour FW regimen. Adipocyte marker peroxisome proliferator-activated receptor gamma expression was elevated (P = .054) in the retroperitoneal tissue of fa/fa obese rats in the fortnightly FW treatment because of a 13% increase in tissue cell density (cells per gram; P = .13). Average cell diameter in retroperitoneal adipose and AUC were negatively corelated. Regression analyses after including the square of average cell diameter indicated that average retroperitoneal adipocyte diameter (between 65 and 135 mum) and the AUC were related in a quadratic manner (R(2) = 0.14; n = 49; P = .03) for Wistar rats. Cell number of the inguinal and retroperitoneal adipocytes tended to be positively corelated (r = 0.24; P = .09 and r = 0.26; P = .07, n = 49, respectively) to the AUC and are indexes of adiposity. Results suggest that maintenance of retroperitoneal adipocytes at appropriate diameters may control progression of metabolic syndrome symptoms such as glucose tolerance. Crown Copyright 2010. Published by Elsevier Inc. All rights reserved.

  4. Imaging investigations before referral to a sarcoma center delay the final diagnosis of musculoskeletal sarcoma.

    PubMed

    Dyrop, Heidi Buvarp; Vedsted, Peter; Rædkjær, Mathias; Safwat, Akmal; Keller, Johnny

    2017-04-01

    Background and purpose - The use of point-of-care or local investigations before referral to specialist sarcoma centers as part of a fast-track diagnostic pathway varies, and may affect the time to diagnosis. We wanted to investigate differences in time intervals and proportion of malignancy in patients who were referred after initial diagnostic investigations were performed locally and in patients who were referred without these investigations. Patients and methods - We included 545 consecutive patients who were referred to Aarhus Sarcoma Center for suspected musculoskeletal sarcoma. Data on time intervals and investigations performed were collected from questionnaires and patient records. Patients who were referred from outside Aarhus uptake area after initial MRI/CT or histology performed locally were compared with patients who were referred from Aarhus uptake area without these investigations. Results - The median total interval from first symptom to diagnosis was 166 days for outside patients referred with MRI/CT or histology, which was 91 (95% CI: 76-106) days longer than for local patients who were referred without MRI/CT or histology. Comparing the same groups, the median diagnostic interval was 41 (95% CI: 30-51) days longer for outside patients including both primary care and hospital intervals. Both the proportion of malignancies (38% vs. 14%) and the proportion of sarcomas (24% vs. 7%) were higher in the outside group referred with MRI/CT or histology than in the local group without MRI/CT or histology. Interpretation - Pre-referral investigations at a local hospital increased the diagnostic interval by at least 1 month for 50% of the patients, and the proportion of malignancy was more than doubled-to almost 40%. If investigations are to be performed before referral to a sarcoma center, they should be part of the fast-track pathway in order to ensure timely diagnosis.

  5. Imaging investigations before referral to a sarcoma center delay the final diagnosis of musculoskeletal sarcoma

    PubMed Central

    Dyrop, Heidi Buvarp; Vedsted, Peter; Rædkjær, Mathias; Safwat, Akmal; Keller, Johnny

    2017-01-01

    Background and purpose The use of point-of-care or local investigations before referral to specialist sarcoma centers as part of a fast-track diagnostic pathway varies, and may affect the time to diagnosis. We wanted to investigate differences in time intervals and proportion of malignancy in patients who were referred after initial diagnostic investigations were performed locally and in patients who were referred without these investigations. Patients and methods We included 545 consecutive patients who were referred to Aarhus Sarcoma Center for suspected musculoskeletal sarcoma. Data on time intervals and investigations performed were collected from questionnaires and patient records. Patients who were referred from outside Aarhus uptake area after initial MRI/CT or histology performed locally were compared with patients who were referred from Aarhus uptake area without these investigations. Results The median total interval from first symptom to diagnosis was 166 days for outside patients referred with MRI/CT or histology, which was 91 (95% CI: 76–106) days longer than for local patients who were referred without MRI/CT or histology. Comparing the same groups, the median diagnostic interval was 41 (95% CI: 30–51) days longer for outside patients including both primary care and hospital intervals. Both the proportion of malignancies (38% vs. 14%) and the proportion of sarcomas (24% vs. 7%) were higher in the outside group referred with MRI/CT or histology than in the local group without MRI/CT or histology. Interpretation Pre-referral investigations at a local hospital increased the diagnostic interval by at least 1 month for 50% of the patients, and the proportion of malignancy was more than doubled—to almost 40%. If investigations are to be performed before referral to a sarcoma center, they should be part of the fast-track pathway in order to ensure timely diagnosis. PMID:28077058

  6. A rare case of lethal retroperitoneal abscess caused by Citrobacter koseri.

    PubMed

    Cai, Tommaso; Giubilei, Gianluca; Vichi, Francesca; Farina, Umberto; Costanzi, Antonio; Bartoletti, Riccardo

    2007-01-01

    Retroperitoneal abscesses are very uncommon clinical conditions. The characteristically vague symptomatology of retroperitoneal abscess and the inherent difficulty of identifying retroperitoneal disease by physical examination contributed to these dismal therapeutic outcomes. We present an unusual case of lethal retroperitoneal abscess, caused by Citrobacter diversus(koseri), treated with surgical drainage. Citrobacter species have rarely been involved in deep tissue infection and there is no reported case of lethal retroperitoneal abscess caused by C. koseri. This case is the only reported case of C. koseri as the sole pathogen associated with a lethal retroperitoneal abscess in immunocompetent patient. The case is also notable because it confirms the recent bacterial resistance to beta-lactam antibiotics and to other antimicrobial agents, like chloramphenicol or cotrimoxazol.

  7. RFHVMn ORF73 is structurally related to the KSHV ORF73 latency-associated nuclear antigen (LANA) and is expressed in retroperitoneal fibromatosis (RF) tumor cells

    SciTech Connect

    Burnside, Kellie L.; Ryan, Jonathan T.; Bielefeldt-Ohmann, Helle; Gregory Bruce, A.; Thouless, Margaret E.; Tsai, Che-Chung; Rose, Timothy M. . E-mail: trose@u.washington.edu

    2006-10-10

    Retroperitoneal fibromatosis herpesvirus (RFHV), the macaque homolog of the human rhadinovirus, Kaposi's sarcoma-associated herpesvirus (KSHV), was first identified in retroperitoneal fibromatosis (RF) tumor lesions of macaques with simian AIDS. We cloned and sequenced the ORF73 latency-associated nuclear antigen (LANA) of RFHVMn from the pig-tailed macaque. RFHVMn LANA is structurally analogous to KSHV ORF73 LANA and contains an N-terminal serine-proline-rich region, a large internal glutamic acidic-rich repeat region and a conserved C-terminal domain. RFHVMn LANA reacts with monoclonal antibodies specific for a glutamic acid-proline dipeptide motif and a glutamic acid-glutamine-rich motif in the KSHV LANA repeat region. Immunohistochemical and immunofluorescence analysis revealed that RFHVMn LANA is a nuclear antigen which is highly expressed in RF spindloid tumor cells. These data suggest that RFHV LANA is an ortholog of KSHV LANA and will function similarly to maintain viral latency and play a role in tumorigenicity in macaques.

  8. [Aspergillosis located on polycystic kidney treated with retroperitoneal nephrectomy].

    PubMed

    Rabii, R; Hoznek, A; Salomon, L; Bourg, S; Chopin, D K; Abbou, C C

    2001-03-01

    We reported an uncommon case of 40 years old man, cardiac transplant recipient with chronic renal faillure who consulted for infected left polycystic renal. The serum creatinine level was 750 mmol/L, and urine culture isolated a E. Ecol germe. The abdominopelvic computed tomography showed a bilateral large polycystic renal cortex and suspected the infected cyst in lower pole of left kidney. The retroperitoneal laparoscopic nephrectomy was performed confirming a renal invasive aspergillosa. About this case we should have a high index of suspicion for fungal aetiology in kidney infection in transplant patients and the management of non functioning infected polycystic kidney can use laparoscopic retroperitoneal nephrectomy. This approach can offers a minimal morbidity and alternative to open surgery.

  9. Pseudomyxoma Retroperitonei: ARare Cause of Retroperitoneal Cystic Mass.

    PubMed

    Al-Qahtani, Hamad Hadi; Asalamah, Saleh; Akeely, Mohammed; Alshakaki, Salman Mohammad

    2016-06-01

    Pseudomyxoma retroperitonei is a rare condition, characterized by accumulation of mucinous material in the retroperitoneal space, originating predominantly from the appendiceal mucinous neoplasms. A72-year-old male patient presented with a history of progressive right side abdominal pain for 5 months with a palpable abdominal mass. Ultrasound, computerized tomography, and magnetic resonance imaging showed large right abdominal multiloculated cystic lesion with heterogeneous echoic contents. Colonoscopy revealed normal mucosa with extramural pressure on the right colon and cecum. He underwent complete excision of the mass along with right hemicolectomy. The cystic mass was containing gelatinous material. Histopathology revealed low grade mucinous neoplasm. Pseudomyxoma retroperitonei should be considered in the differential diagnosis of patient presenting with progressive right side abdominal pain and retroperitoneal cystic mass.

  10. [Robot-assisted operations for non-organ retroperitoneal tumors].

    PubMed

    Kriger, A G; Berelavichus, S V; Son, A I; Gorin, D S; Ikramov, R Z; Kalinin, D V

    2015-01-01

    Robot-assisted operations for non-organ retroperitoneal tumors were performed in 20 patients. The biggest tumor's diameter was 160 mm, the smallest--32 mm, mean value was 79.1±37.8 mm. According to morphological investigation data lymphangioma was diagnosed in 4 (20%) patients, lipoma--in 3 (15%) cases, peritoneal cyst--in 2 (10%) patients. Solitary lymphatic node, teratoma, leiomyosarcoma, accessory stomach, bronchogenic cyst, adenogenous cancer, ureterocele, megaureter, schwannoma, leiomyoma, malignant paraganglioma were observed in 1 (5%) case respectively. Surgery duration was 138±55.9 min. Blood loss was 69±112.7 ml (range 0-500 ml). Intraoperative complications (bleeding) were diagnosed in 3 (15%) patients. There were no conversions. Robot-assisted variant has been demonstrated in case of non-organ retroperitoneal tumors with diameter not more than 100-120 mm.

  11. Retroperitoneal calcifying fibrous tumor mimicking an adrenal tumor

    PubMed Central

    Prochaska, Erica C.; Sciallis, Andrew P.; Miller, Barbra S.

    2016-01-01

    Establishing the etiology of a retroperitoneal tumor may be difficult due to close proximity of multiple organs. Evaluation of retroperitoneal tumors often leads to surgery, many times to obtain a definitive diagnosis and rule out malignancy. Calcifying fibrous tumors (CFT) are very rare soft tissue tumors occurring most often in young patients. They are most often found arising in the thoracic cavity, mediastinum, abdominal cavity and extremities and usually have a benign clinical course. Macrocscopically, the tumors are well circumscribed and firm with a white-tan appearance. Histologically, CFT comprised a hypocellular proliferation of bland spindle cells, densely hyalinized collagen, chronic lymphoplasmacytic inflammation and dystrophic calcifications. Other considerations in the pathologic differential diagnosis include solitary fibrous tumor and inflammatory myofibroblastic tumor. PMID:27252518

  12. Retroperitoneal calcifying fibrous tumor mimicking an adrenal tumor.

    PubMed

    Prochaska, Erica C; Sciallis, Andrew P; Miller, Barbra S

    2016-06-01

    Establishing the etiology of a retroperitoneal tumor may be difficult due to close proximity of multiple organs. Evaluation of retroperitoneal tumors often leads to surgery, many times to obtain a definitive diagnosis and rule out malignancy. Calcifying fibrous tumors (CFT) are very rare soft tissue tumors occurring most often in young patients. They are most often found arising in the thoracic cavity, mediastinum, abdominal cavity and extremities and usually have a benign clinical course. Macrocscopically, the tumors are well circumscribed and firm with a white-tan appearance. Histologically, CFT comprised a hypocellular proliferation of bland spindle cells, densely hyalinized collagen, chronic lymphoplasmacytic inflammation and dystrophic calcifications. Other considerations in the pathologic differential diagnosis include solitary fibrous tumor and inflammatory myofibroblastic tumor.

  13. Retroperitoneal fibrosis - the state-of-the-art.

    PubMed

    Runowska, Marta; Majewski, Dominik; Puszczewicz, Mariusz

    2016-01-01

    Retroperitoneal fibrosis (RPF) is a rare disease, hallmarked by inflammation and deposition of fibrous tissue around the abdominal aorta. This process may spread contiguously and involve adjacent structures, leading to many complications, among which the most frequent and most severe is ureteral obstruction. The condition usually has idiopathic origin (idiopathic retroperitoneal fibrosis - IRF), but can also develop secondarily to a number of factors. The etiology of the disease remains unclear. Current research suggests that about half of the cases of IRF may be a symptom of a recently discovered, clinically heterogeneous immunoglobulin G4-related disease (IgG4-RD). Corticosteroids are the first-line treatment for IRF, but effective attempts to use immunosuppressants are also made. This paper presents the current state of knowledge on the etiopathogenesis, clinical presentation, diagnosis and therapeutic possibilities in different forms of RPF. Based on the latest research, an analysis of the relationship between IRF and IgG4-RD was performed.

  14. Talimogene Laherparepvec and Radiation Therapy in Treating Patients With Newly Diagnosed Soft Tissue Sarcoma That Can Be Removed by Surgery

    ClinicalTrials.gov

    2017-01-31

    Leiomyosarcoma; Liposarcoma; Sarcoma Differentiation Score 2; Sarcoma Differentiation Score 3; Stage IA Soft Tissue Sarcoma; Stage IB Soft Tissue Sarcoma; Stage IIA Soft Tissue Sarcoma; Stage IIB Soft Tissue Sarcoma; Undifferentiated Pleomorphic Sarcoma

  15. 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

  16. 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

    2017-07-05

    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.

  17. Femoral Nerve Palsy due to Anticoagulant Induced Retroperitoneal Hematoma

    PubMed Central

    Gurbuz, Orcun; Ercan, Abdulkadir; Kumtepe, Gencehan; Karal, İlker Hasan; Velioglu, Yusuf; Ener, Serdar

    2014-01-01

    A forty-one-year-old man who, sought evaluation for a sudden hip flexion contracture and groin pain with a history of mechanical mitral valve replacement, had been misdiagnosed and treated as having lumbar discopathy for two days. This patient finally was diagnosed with compressive femoral neuropathy due to warfarin-induced retroperitoneal hematoma and successfully managed nonoperatively. This case is reported in order to draw attention to this rare presentation. PMID:25386195

  18. A Huge Cystic Retroperitoneal Lymphangioma Presenting with Back Pain

    PubMed Central

    Kubachev, Kubach; Abdullaev, Elbrus; Babyshin, Valentin; Neronov, Dmitriy; Abdullaev, Abakar

    2016-01-01

    Retroperitoneal lymphangioma is a rare location and type of benign abdominal tumors. The clinical presentation of this rare disease is nonspecific, ranging from abdominal distention to sepsis. Here we present a 73-year-old female patient with 3-month history of back pain. USG and CT revealed a huge cystic mass which was surgically excised and appeared to be lymphangioma on histopathology. PMID:27843456

  19. Retroperitoneal Extraskeletal Osteosarcoma: Imaging Findings and Transarterial Chemoembolization

    SciTech Connect

    Zhang Huojun Yang Jijin Lu Jianping; Sheng Jin; Yuan Min; Jiang Xu; Li Yuxiao; Gupta, Sanjay

    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.

  20. 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

  1. Lytic Replication of Kaposi's Sarcoma-Associated Herpesvirus Results in the Formation of Multiple Capsid Species: Isolation and Molecular Characterization of A, B, and C Capsids from a Gammaherpesvirus

    PubMed Central

    Nealon, K.; Newcomb, W. W.; Pray, T. R.; Craik, C. S.; Brown, J. C.; Kedes, D. H.

    2001-01-01

    Despite the discovery of Epstein-Barr virus more than 35 years ago, a thorough understanding of gammaherpesvirus capsid composition and structure has remained elusive. We approached this problem by purifying capsids from Kaposi's sarcoma-associated herpesvirus (KSHV), the only other known human gammaherpesvirus. The results from our biochemical and imaging analyses demonstrate that KSHV capsids possess a typical herpesvirus icosahedral capsid shell composed of four structural proteins. The hexameric and pentameric capsomers are composed of the major capsid protein (MCP) encoded by open reading frame 25. The heterotrimeric complexes, forming the capsid floor between the hexons and pentons, are each composed of one molecule of ORF62 and two molecules of ORF26. Each of these proteins has significant amino acid sequence homology to capsid proteins in alpha- and betaherpesviruses. In contrast, the fourth protein, ORF65, lacks significant sequence homology to its structural counterparts from the other subfamilies. Nevertheless, this small, basic, and highly antigenic protein decorates the surface of the capsids, as does, for example, the even smaller basic capsid protein VP26 of herpes simplex virus type 1. We have also found that, as with the alpha- and betaherpesviruses, lytic replication of KSHV leads to the formation of at least three capsid species, A, B, and C, with masses of approximately 200, 230, and 300 MDa, respectively. A capsids are empty, B capsids contain an inner array of a fifth structural protein, ORF17.5, and C capsids contain the viral genome. PMID:11222712

  2. Childhood Soft Tissue Sarcoma: Treatment Information

    MedlinePlus

    ... Germ Cell Tumors Kidney/Wilms Tumor Liver Cancer Neuroblastoma Osteosarcoma Rhabdomyosarcoma Skin Cancer Soft Tissue Sarcoma Thyroid ... Tumor Liver Cancer Lymphoma (Non-Hodgkin) Lymphoma (Hodgkin) Neuroblastoma Osteosarcoma Retinoblastoma Rhabdomyosarcoma Skin Cancer Soft Tissue Sarcoma ...

  3. What Is a Soft Tissue Sarcoma?

    MedlinePlus

    ... know has just been diagnosed with sarcoma, this short, simple guide can help. Downloadable PDFs Download free PDFs of our soft tissue sarcoma information About and ... Read More Latest Cancer News Read More Stories ...

  4. Drugs Approved for Soft Tissue Sarcoma

    MedlinePlus

    ... Your Treatment Research Drugs Approved for Soft Tissue Sarcoma This page lists cancer drugs approved by the ... not listed here. Drugs Approved for Soft Tissue Sarcoma Cosmegen (Dactinomycin) Dactinomycin Doxorubicin Hydrochloride Eribulin Mesylate Gleevec ( ...

  5. [Radiotherapy of adult soft tissue sarcoma].

    PubMed

    Le Péchoux, C; Moureau-Zabotto, L; Llacer, C; Ducassou, A; Sargos, P; Sunyach, M P; Thariat, J

    2016-09-01

    Incidence of soft tissue sarcoma is low and requires multidisciplinary treatment in specialized centers. The objective of this paper is to report the state of the art regarding indications and treatment techniques of main soft tissue sarcoma localisations.

  6. Retroperitoneal unicentric Castleman's disease (giant lymph node hyperplasia): case report.

    PubMed

    Waisberg, Jaques; Satake, Marie; Yamagushi, Nagamassa; Matos, Leandro Luongo de; Waisberg, Daniel Reis; Artigiani Neto, Ricardo; Franco, Maria Isete Fares

    2007-07-05

    Castleman's disease, or giant lymph node hyperplasia, is a rare disorder of the lymphoid tissue that causes lymph node enlargement. It is considered benign in its localized form, but aggressive in the multicentric type. The definitive diagnosis is based on postoperative pathological findings. The aim here was to describe a case of retroperitoneal unicentric Castleman's disease in the retroperitoneum. A 61-year old white male with weight loss and listlessness presented with moderate arterial hypertension and leukopenia. Abdominal tomography revealed a 5 x 4 x 5 cm oval mass of low attenuation, with inner calcification and intense enhancement on intravenous contrast, located in the retroperitoneal region, between the left kidney and the aorta, at the renal hilus. Exploratory laparotomy revealed a non-pulsatile solid oval mass situated in the retroperitoneum, adjacent to the left renal hilus. The retroperitoneal lesion was removed in its entirety. Examination of frozen samples revealed benign lymph node tissue and histopathological examination of the surgical sample revealed hyaline-vascular giant lymph node hyperplasia (Castleman's disease). The patient was discharged on the 12th day without significant events. Two months after the operation, the patient was readmitted with severe cardiac insufficiency, acute renal failure and bronchopneumonia, which progressed to acute respiratory insufficiency, sepsis and death.

  7. Cross-sectional Imaging Features of Primary Retroperitoneal Tumors and Their Subsequent Treatment

    PubMed Central

    Acar, Turker; Harman, Mustafa; Guneyli, Serkan; Gemici, Kazim; Efe, Duran; Guler, Ibrahim; Yildiz, Melda

    2015-01-01

    Basically malignant tumors in the retroperitoneal region arise from a heterogeneous group of tissues: mesodermal, neurogenic, germ cell, and lymphoid. Although rare, benign tumors and cystic masses can be also encountered in retroperitoneal space. Developments in computed tomography (CT) and magnetic resonance imaging (MRI) have contributed to both diagnosis and staging of the retroperitoneal tumors. High spatial resolution and superiority in calcification make CT indispensable; on the other hand, MRI has a better soft-tissue contrast resolution which is essential for the assessment of vascular invasion and tissue characterization. The aim of this article is to review the CT and MRI features of retroperitoneal tumors and their subsequent management. PMID:25973288

  8. 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

  9. Alarm symptoms of soft-tissue and bone sarcoma in patients referred to a specialist center

    PubMed Central

    Dyrop, Heidi B; Vedsted, Peter; Safwat, Akmal; Maretty-Nielsen, Katja; Hansen, Bjarne H; Jørgensen, Peter H; Baad-Hansen, Thomas; Keller, Johnny

    2014-01-01

    Background and purpose — The Danish Cancer Patient Pathway for sarcoma defines a set of alarm symptoms as criteria for referral to a sarcoma center. This may exclude cancer patients without alarm symptoms, so we investigated the presence of alarm symptoms (defined as being indicative of a sarcoma) in patients who had been referred to the Aarhus Sarcoma Center. Patients and methods — We reviewed the medical records of all 1,126 patients who had been referred, with suspected sarcoma, from other hospitals in the period 2007–2010 for information on symptoms, clinical findings, and diagnosis. Alarm symptoms were analyzed for predictive values in diagnosing sarcoma. Results — 179 (69%) of 258 sarcoma patients were referred with alarm symptoms (soft-tissue tumor > 5 cm or deep-seated, fast-growing soft-tissue tumor, palpable bone tumor, or deep persisting bone pain). The remaining 79 sarcomas were found accidentally. “Size over 5 cm” for soft-tissue tumors, and “deep persisting bone pain” for bone tumors had the highest sensitivity and positive predictive value. Of the 79 sarcoma patients who were referred without alarm symptoms, 7 were found accidentally on imaging, 5 were referred with suspected recurrence of a sarcoma, 64 were referred with a confirmed histological diagnosis, and 3 were referred for other reasons. Interpretation — Defined alarm symptoms are predictive of sarcoma, but one-third of the patients were found accidentally. Further studies on presenting symptoms in primary care are needed to assess the true value of alarm symptoms. PMID:25175662

  10. Retroperitoneal hemangiosarcoma in a common carp Cyprinus carpio: a case report.

    PubMed

    Hyatt, Michael W; Clauss, Tonya M; Dennison, Sophie E; Camus, Alvin C

    2013-12-12

    A 7.5 kg common carp Cyprinus carpio presented with prominent localized swelling in the caudal right coelomic area, identified by ultrasound as a fluid filled mass. Fine needle aspirate (FNA) and culture results suggested a sterile seroma. Centesis removed 290 ml of serosanguinous fluid that returned within days. Recheck ultrasonography revealed a solid component within the cavity. Radiography demonstrated irregular lysis and misalignment of vertebrae adjacent to the mass, most suggestive of bacterial osteitis or neoplasia. Treatment with antibiotics followed for 2 mo but failed to resolve the lesion. Repeated radiography and ultrasonography showed progressive enlargement of the mass, with vertebral lysis and invasion characteristic of neoplasia. Ultrasound-guided FNA of the solid component of the mass was non-diagnostic. Euthanasia was elected due to the poor clinical response and primary differential of neoplasia. Post-mortem MRI and CT confirmed a retroperitoneal soft tissue mass, partially surrounded by a fluid-filled cavity, causing vertebral lysis and infiltration of the spinal canal. Expansion of the mass caused severe muscle loss and an associated elevation in creatine kinase (>120000 U l-1). Necropsy results corroborated the MRI and CT findings, revealing a retroperitoneal, multilobular, red and tan mass causing dorsal displacement of the vertebral column, with vertebral lysis, pathologic fracture and invasion of the spinal canal. Histopathologic examination revealed a locally aggressive neoplasm exhibiting multiple patterns of growth, including endothelial lined vascular channels and solid areas formed by more pleomorphic polygonal and spindle cells, consistent with hemangiosarcoma.

  11. Cardiac surgery in a patient with retroperitoneal fibrosis and heart valvulopathy, both due to pergolide medication for Parkinson's disease.

    PubMed

    Apostolakis, Efstratios E; Baikoussis, Nikolaos G; Tselikos, Dimitrios; Koniari, Ioanna; Prokakis, Christos; Fokaeas, Eleftherios; Karanikolas, Menelaos

    2009-11-13

    Retroperitoneal fibrosis is best described as a chronic inflammatory process which may be idiopathic, but can rarely be brought about by medications, such as pergolide, used for treating Parkinson's disease. Pergolide can produce a fibrotic process in heart valves, resulting in valve insufficiency in up to 25% of cases. Herein we describe the case of a 68-year-old man who received pergolide for 2 years for Parkinson's disease. The patient developed retroperitoneal fibrosis resulting in renal failure from ureteral obstruction necessitating ureteral stenting, as well as significant aortic and mitral valve insufficiency. He successfully underwent surgery for combined aortic valve, mitral valve and ascending aorta replacement because of severe valve insufficiency and dilated (d = 5.8 cm) ascending aorta. Retroperitoneal fibrosis improved with pergolide cessation and corticosteroid treatment. This is the second case reported in the literature, of a patient who had double valve and ascending aorta replacement surgery because he suffered from this rare but serious adverse effect of dopamine agonists used for managing Parkinson's disease.

  12. Trabectedin in soft tissue sarcomas.

    PubMed

    Petek, Bradley J; Loggers, Elizabeth T; Pollack, Seth M; Jones, Robin L

    2015-02-12

    Soft tissue sarcomas are a group of rare tumors derived from mesenchymal tissue, accounting for about 1% of adult cancers. There are over 60 different histological subtypes, each with their own unique biological behavior and response to systemic therapy. The outcome for patients with metastatic soft tissue sarcoma is poor with few available systemic treatment options. For decades, the mainstay of management has consisted of doxorubicin with or without ifosfamide. Trabectedin is a synthetic agent derived from the Caribbean tunicate, Ecteinascidia turbinata. This drug has a number of potential mechanisms of action, including binding the DNA minor groove, interfering with DNA repair pathways and the cell cycle, as well as interacting with transcription factors. Several phase II trials have shown that trabectedin has activity in anthracycline and alkylating agent-resistant soft tissue sarcoma and suggest use in the second- and third-line setting. More recently, trabectedin has shown similar progression-free survival to doxorubicin in the first-line setting and significant activity in liposarcoma and leiomyosarcoma subtypes. Trabectedin has shown a favorable toxicity profile and has been approved in over 70 countries for the treatment of metastatic soft tissue sarcoma. This manuscript will review the development of trabectedin in soft tissue sarcomas.

  13. Synovial Sarcoma With Myoid Differentiation.

    PubMed

    Qassid, Omar; Ali, Ahmed; Thway, Khin

    2016-09-01

    Synovial sarcoma is a malignant mesenchymal tumor with variable epithelial differentiation, which is defined by the presence of a specific t(X;18)(p11.2;q11.2) chromosomal translocation that generates SS18-SSX fusion oncogenes. Synovial sarcoma typically arises within extremity deep soft tissue (particularly around large joints) of young adults, but has been shown to occur at almost any location. When it arises in more unusual sites, such as the abdomen, it can present a significant diagnostic challenge. We describe a case of intraabdominal monophasic synovial sarcoma that immunohistochemically showed strong expression of smooth muscle actin and calponin but only very scanty cytokeratin, and which showed morphologic and immunohistochemical overlap with other spindle cell neoplasms that can arise at this site, such as gastrointestinal stromal tumor and myofibrosarcoma. As correct diagnosis is of clinical and prognostic importance, surgical pathologists should be aware of the potential for synovial sarcoma to occur at a variety of anatomic sites and of its spectrum of immunoreactivity. Synovial sarcoma should be in the differential diagnosis of spindle cell neoplasms with myoid differentiation that do not fall into any definite tumor category, for which there should be a relatively low threshold for performing fluorescence in situ hybridization or reverse transcription-polymerase chain reaction to assess for the specific SS18 gene rearrangement or SS18-SSX fusion transcripts, which remain the diagnostic gold standard. © The Author(s) 2016.

  14. Pulmonary involvement in Kaposi sarcoma: correlation between imaging and pathology.

    PubMed

    Gasparetto, Taisa Davaus; Marchiori, Edson; Lourenço, Sílvia; Zanetti, Gláucia; Vianna, Alberto Domingues; Santos, Alair A S M D; Nobre, Luiz Felipe

    2009-07-14

    Kaposi sarcoma is a low-grade mesenchymal tumor involving blood and lymphatic vessels. There are four variants of this disease, each presenting a different clinical manifestation: classic or sporadic, African or endemic, organ transplant-related or iatrogenic, and AIDS-related or epidemic. Kaposi sarcoma is the most common tumor among patients with HIV infection, occurring predominantly in homosexual or bisexual men. The pulmonary involvement in Kaposi sarcoma occurs commonly in critically immunosupressed patients who commonly have had preceding mucocutaneous or digestive involvement.The etiology of Kaposi sarcoma is not precisely established; genetic, hormonal, and immune factors, as well as infectious agents, have all been implicated. There is evidence from epidemiologic, serologic, and molecular studies that Kaposi sarcoma is associated with human herpes virus type 8 infection. The disease starts as a reactive polyclonal angioproliferative response towards this virus, in which polyclonal cells change to form oligoclonal cell populations that expand and undergo malignant transformation.The diagnosis of pulmonary involvement in Kaposi sarcoma usually can be made by a combination of clinical, radiographic, and laboratory findings, together with the results of bronchoscopy and transbronchial biopsy. Chest high-resolution computed tomography scans commonly reveal peribronchovascular and interlobular septal thickening, bilateral and symmetric ill-defined nodules in a peribronchovascular distribution, fissural nodularity, mediastinal adenopathies, and pleural effusions. Correlation between the high-resolution computed tomography findings and the pathology revealed by histopathological analysis demonstrate that the areas of central peribronchovascular infiltration represent tumor growth involving the bronchovascular bundles, with nodules corresponding to proliferations of neoplastic cells into the pulmonary parenchyma. The interlobular septal thickening may represent

  15. 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

  16. The two-port laparoscopic retroperitoneal approach for minimal access pancreatic necrosectomy

    PubMed Central

    Cresswell, AB; Nageswaran, H; Belgaumkar, A; Kumar, R; Menezes, N; Riga, A; Worthington, TR

    2015-01-01

    Introduction Despite advances in surgery and critical care, severe pancreatitis continues to be associated with a high rate of mortality, which is increased significantly in the presence of infected pancreatic necrosis. Controversy persists around the optimal treatment for such cases, with specialist units variously advocating open necrosectomy, simple percutaneous drainage or one of several minimal access approaches. We describe our technique and outcomes with a two-port laparoscopic retroperitoneal necrosectomy (2P-LRN). Methods Thirteen consecutive patients with proven infected pancreatic necrosis were treated by 2P-LRN over a three-year period in the setting of a specialist hepatopancreatobiliary unit. The median patient age was 46 years (range: 28–87 years) and 10 of the patients were male. Results The median number of procedures required to clear the necrosis was 2 (range: 1–5), with a median time to discharge following the procedure of 44 days (range: 10–135 days). There was no 90-day mortality and the morbidity rate was 38%, consisting of pancreatic fistula (31%) and bleeding (23%). Conclusions Two-port laparoscopic retroperitoneal necrosectomy has been demonstrated to confer similar or better outcomes to other techniques for necrosectomy. It carries the additional advantages of better visualisation, leading to fewer procedures and the opportunity to deploy simple laparoscopic instruments such as diathermy or haemostatic clips. PMID:26264086

  17. Spectrum of retroperitoneal and genitourinary paraganglioma: Experience at a North Indian tertiary care center

    PubMed Central

    Choudhary, Gautam Ram; Singh, Shivanshu; Prasad, Seema; Singh, Shrawan Kumar; Bhansali, Anil; Bhadada, Sanjay; Dutta, Pinaki

    2015-01-01

    Introduction Genitourinary and retroperitoneal paragangliomas are infrequent tumors with bizarre presentation. A high index of suspicion is required to make a diagnosis in young hypertensive individuals. Our aim is to study the varied clinical presentations and management of these paragangliomas. Herein, we share our experience of clinical presentation, diagnosis, and management of these paragangliomas. Material and methods Seventeen consecutive patients who underwent surgery for paraganglioma at our institute from August 2009 to July 2014 were included. Demographic, peri-operative, surgical, and follow up data were collected and analyzed. Results Mean age of presentation was 34.8 years with female predominance. The majority of the tumors were located in the retroperitoneum and urinary bladder. Most of them presented with classical symptoms of catecholamine excess and hypertension. Complete surgical resection could be performed in 13 cases. At a median follow up of two years, cases with R0 resection (no microscopic malignant cells) did not show recurrence. Among patients on chemotherapy, one died, another had partial response, and yet another had progressive disease. Conclusions Genitourinary and retroperitoneal paragangliomas are a disease of a young age group with variable clinical features at presentation. Appropriate pre-operative optimization and complete surgical resection provide the best chance of cure. PMID:26855794

  18. [Retroperitoneal laparoscopic treatment of parapelvic renal cysts: report of 5 cases].

    PubMed

    Rabii, Redouane; Mezzour, Mohamed Hicham; Essaki, Hicham; Aboutaieb, Rachid; El Moussaoui, Ali; Joual, Abdenbi; Meziane, Fathi

    2005-12-01

    Renal cysts of the sinuses or parapelvic cysts constitute a rare form of renal cyst, usually associated with low back pain. Symptomatic forms may be treated by percutaneous aspiration, but the treatment of these cysts has changed considerably over recent years due to the proximity of the renal hilum. Percutaneous treatment is now contraindicated due to its high complication rate, and open surgery is an invasive technique. Laparoscopy appears to be the most appropriate and most effective solution. The authors report five cases of parapelvic renal cysts all presenting with renal colic and diagnosed by ultrasound, and confirmed by CT in three cases. Treatment consisted of retroperitoneal laparoscopy using four trocars allowing cyst resection with no intraoperative complications or conversion. The mean operating time was 55 minutes (range: 40 to 70 minutes). The postoperative course was uneventful and all patients were discharged on postoperative D1 or D2. No recurrence was observed after a mean follow-up of 6 months (range: 3 to 15 months). Retroperitoneal laparoscopic treatment of parapelvic renal cysts is an effective approach, with a low morbidity and less postoperative pain, a short convalescence period and good cosmetic results.

  19. 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.

  20. Necrotizing cellulitis of the abdominal wall, caused by Pediococcus sp., due to rupture of a retroperitoneal stromal cell tumor

    PubMed Central

    Michalopoulos, Nick; Arampatzi, Stergiani; Papavramidis, Theodossis S.; Kotidis, Efstathios; Laskou, Styliani; Papavramidis, Spiros T.

    2013-01-01

    INTRODUCTION Soft tissue necrotizing infections are a significant cause of morbidity and mortality. The aim of this study is to present a patient with necrotizing infection of abdominal wall resulting from the rupture of a retroperitoneal stromal tumor. PRESENTATION OF CASE We present a 60-year-old Caucasian male patient with necrotizing infection of abdominal wall secondary to the rupture of a retroperitoneal stromal tumor. The patient was initially treated with debridement and fasciotomy of the anterior abdominal wall. Laparotomy revealed purulent peritonitis caused by infiltration and rupture of the splenic flexure by the tumor. Despite prompt intervention the patient died 19 days later. The isolated microorganism causing the infection was the rarely identified as cause of infections in humans Pediococcus sp., a gram-positive, catalase-negative coccus. DISCUSSION Necrotizing infections of abdominal wall are usually secondary either to perineal or to intra-abdominal infections. Gastrointestinal stromal cell tumors could be rarely complicated with perforation and abscess formation. In our case, the infiltrated by the extra-gastrointestinal stromal cell tumor ruptured colon was the source of the infection. The pediococci are rarely isolated as the cause of severe septicemia. CONCLUSION Ruptured retroperitoneal stromal cell tumors are extremely rare cause of necrotizing fasciitis, and before this case, Pediococcus sp. has never been isolated as the responsible agent. PMID:23357010

  1. Herpes virus-like sequences are specifically found in Kaposi sarcoma lesions.

    PubMed Central

    O'Neill, E; Henson, T H; Ghorbani, A J; Land, M A; Webber, B L; Garcia, J V

    1996-01-01

    AIM: To detect the prevalence of herpes virus-like DNA sequences in AIDS associated Kaposi sarcoma (KSHV) lesions and normal tissue. METHODS: KSHV detection was performed by polymerase chain reaction (PCR) using four different sets of primers. PCR products were cloned, sequenced, and analysed. RESULTS: All of four biopsies of Kaposi sarcoma lesions and all of three paraffin embedded Kaposi sarcoma tissues were positive for KSHV, while normal tissue from the same patients was negative. Sequence analysis of amplification products revealed polymorphisms that result in amino acid changes of the predicted sequence. CONCLUSIONS: KSHV is prevalent in tissues from Kaposi sarcoma, suggesting a role in the development of the tumour. On this basis, anti-herpes virus agents should be considered to control Kaposi sarcoma. Images PMID:8655706

  2. 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.

  3. Recent advances in the molecular pathogenesis of Ewing's sarcoma.

    PubMed

    Toomey, E C; Schiffman, J D; Lessnick, S L

    2010-08-12

    Tumor development is a complex process resulting from interplay between mutations in oncogenes and tumor suppressors, host susceptibility factors, and cellular context. Great advances have been made by studying rare tumors with unique clinical, genetic, or molecular features. Ewing's sarcoma serves as an excellent paradigm for understanding tumorigenesis because it exhibits some very useful and important characteristics. For example, nearly all cases of Ewing's sarcoma contain the (11;22)(q24;q12) chromosomal translocation that encodes the EWS/FLI oncoprotein. Besides the t(11;22), however, many cases have otherwise simple karyotypes with no other demonstrable abnormalities. Furthermore, it seems that an underlying genetic susceptibility to Ewing's sarcoma, if it exists, must be rare. These two features suggest that EWS/FLI is the primary mutation that drives the development of this tumor. Finally, Ewing's sarcoma is an aggressive tumor that requires aggressive treatment. Thus, improved understanding of the pathogenesis of this tumor will not only be of academic interest, but may also lead to new therapeutic approaches for individuals afflicted with this disease. The purpose of this review is to highlight recent advances in understanding the molecular pathogenesis of Ewing's sarcoma, while considering the questions surrounding this disease that still remain and how this knowledge may be applied to developing new treatments for patients with this highly aggressive disease.

  4. Synovial Sarcoma in Head and Neck: A Case Report

    PubMed Central

    Fonseca, Adriano Santana; Azevedo, Amanda Canário Andrade; Magalhães, Fabíola Moreira; Andrade, Nilvano Alves de

    2013-01-01

    Introduction Synovial sarcoma is a malignant tumor of mesenchymal pluripotent cells. Objectives We present a case of synovial sarcoma in the posterolateral wall of the oropharynx. Resumed report The patient, a 23-year-old woman, was admitted with a history of dysphagia and difficulty in breathing for 8 months, resulting in progressive deterioration and onset of snoring, muffled voice, and local pain. An oropharyngeal tumor in the left posterolateral wall touched the base of the ipsilateral tongue. The patient underwent endoscopic pharyngectomy to remove the lesion. Pathologic examination revealed synovial sarcoma with positive margins, and Mohs technique was proposed for margin control. The margins were disease-free, without the need for total laryngectomy. The pharynx was reconstructed with a microvascular forearm flap. The patient developed postoperative stability. Conclusion Despite its name, synovial sarcoma is rarely sourced directly from synovial membranes. It is most commonly found in the vicinity of large joints. The location at the head and neck, a location poor in synovial tissue, is unusual. Synovial sarcoma in the head and neck has an aggressive nature and poor prognosis. Resection with negative margins remains the foundation of therapy, which is not so easily achieved in the head and neck. It is important for the otorhinolaryngologist and head and neck surgeon to be familiar with this aggressive tumor, which carries high mortality and morbidity. The appropriate diagnosis and treatment can improve prognosis and patient survival. PMID:25992071

  5. Kaposi sarcoma and lymphadenopathy syndrome: limitations of abdominal CT in acquired immunodeficiency syndrome

    SciTech Connect

    Moon, K.L. Jr.; Federle, M.P.; Abrams, D.I.; Volberding, P.; Lewis, B.J.

    1984-02-01

    Abdominal computed tomography (CT) was performed in 31 patients with Kaposi sarcoma (KS) related to acquired immunodeficiency syndrome (AIDS), three patients with classic KS, and 12 patients with the newly described lymphadenopathy syndrome (LNS). The frequency, distribution, and appearance of lymphadenopathy and splenomegaly were similar in the AIDS-related KS and LNS groups. Rectal and perirectal disease was identified in 86% of homosexual men studied; rectal KS could not be distinguished from proctitis on CT criteria alone. No CT abnormalities were seen in patients with classic KS. The CT demonstration of retroperitoneal, mesenteric, or pelvic adenopathy or of rectal or perirectal disease in patients with AIDS-related KS is not necessarily indicative of widespread involvement with the disease.

  6. Comparative Pathobiology of Kaposi Sarcoma-associated Herpesvirus and Related Primate Rhadinoviruses

    PubMed Central

    Westmoreland, Susan V; Mansfield, Keith G

    2008-01-01

    With the emergence of the AIDS epidemic over the last 2 decades and the more recent identification of Kaposi sarcoma-associated herpesvirus (KSHV, Human herpesvirus 8), the genera of rhadinoviruses have gained importance as a family of viruses with oncogenic potential. First recognized in New World primates more than 30 y ago, the rhadinoviruses Saimiriine herpesvirus 2 and Ateline herpesvirus 2 have well-described transforming capabilities. Recently several new species-specific rhadinoviruses of Old World primates have been described, including retroperitoneal fibromatosis herpesvirus and rhesus rhadinovirus (Cercopithecine herpesvirus 17). Molecular analysis of these viruses has elucidated several functionally conserved genes and properties shared with KSHV involved in cellular proliferation, transformation, and immune evasion that facilitate the oncogenic potential of these viruses. This review examines the comparative pathobiology of KSHV, discusses the role of macaque rhadinoviruses as models of human disease, and outlines the derivation of specific pathogen-free animals. PMID:19793454

  7. 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.

  8. Skin Ultrasound in Kaposi Sarcoma.

    PubMed

    Carrascosa, R; Alfageme, F; Roustán, G; Suarez, M D

    2016-05-01

    The use of ultrasound imaging has recently been increasing in numerous dermatologic diseases. This noninvasive technique provides additional details on the structure and vascularization of skin lesions. Kaposi sarcoma is a vascular tumor that typically arises in the skin and mucosas. It can spread to lymph nodes and internal organs. We performed B-mode and color Doppler ultrasound studies in 3 patients with a clinical diagnosis of Kaposi sarcoma confirmed by histological examination. We found differences in the ultrasound pattern between nodular and plaque lesions, in both B-mode and color Doppler. We believe that skin ultrasound imaging could be a useful technique for studying cutaneous Kaposi sarcoma, providing additional information on the structural and vascular characteristics of the lesion.

  9. Testicular myeloid sarcoma: case report

    PubMed Central

    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. PMID:23580888

  10. Epithelioid sarcoma in FNAB smears.

    PubMed

    Pohar-Marinsek, Z; Zidar, A

    1994-12-01

    FNAB smears of eleven epithelioid sarcomas were reviewed and analysed. Ten cases had a very similar cytomorphologic picture composed predominantly of dissociated epithelioid-like cells with eccentrically placed nuclei. These tumors were clearly malignant but difficult to differentiate morphologically from melanoma, epithelioid leiomiosarcoma, and Schwannoma or adenocarcinoma. One case was composed of spindle cells and was reminiscent of a fibrohistiocytic tumor. Immunocytochemical reactions to vimentin and cytokeratin were performed in six cases on the Papanicolaou stained smears. The reactions to both antigens were positive in all six cases. Ultrastructural characteristics of eight of the tumors are also described. It seems that epithelioid sarcoma has a rather distinct cytomorphologic picture. Taking into consideration clinical data and using also immunocytochemistry, a definitive diagnosis of epithelioid sarcoma can probably be given from FNAB smears.

  11. Reticular schwannoma mimicking myxoid sarcoma.

    PubMed

    Chaurasia, Jai Kumar; Afroz, Nishat; Sahoo, Biswajit; Naim, Mohammed

    2014-02-20

    Reticular/microcystic schwannoma is a recently described rare distinctive variant of schwannoma with a predilection for gastrointestinal tract. Its occurrence in soft tissue is extremely rare. We report a case of reticular/microcystic variant of schwannoma in a 55-year-old Indian woman who presented with a painless slow-growing swelling in the right forearm for the past 6 months. MRI findings suggested myxoid sarcoma. However, histological and immunohistochemical findings in the excised specimen were consistent with reticular/microcystic variant of schwannoma. This case report emphasises that the diagnosis of reticular/microcystic schwannoma should always be considered in cases where myxoid sarcomas are suspected as it can mimic malignant myxoid sarcomas, clinically and radiologically, thereby avoiding aggressive intervention and overtreatment.

  12. [Therapeutic options for synovial sarcoma].

    PubMed

    Deme, Dániel; Telekes, András

    2015-05-31

    Synovial sarcomas account for approximately 5 to 10% of soft tissue sarcomas and 0.05 to 0.1% of all malignant neoplasms. They predominantly affect the extremities but can occur in any part of the body. More than 50% of the patients are expected to develop metastatic disease within 3-5 years. In some patients disease recurrence may develop after 20 years. The 5-year overall survival rate is 10% for patients with metastatic disease and 76% for patients with localized one. Age, tumour size, histological subtype, and adjuvant radiotherapy influence prognosis. The role of adjuvant chemotherapy has not been proven yet. There are several ongoing clinical trials to determine the efficacy of active agents used for therapy of locally advanced, relapsed/refractory or metastatic disease. Better understanding of the biological behaviour of synovial sarcomas would provide the future way for the targeted therapy in combination with conventional treatments.

  13. Intra-Articular Synovial Sarcomas: Incidence and Differentiating Features from Localized Pigmented Villonodular Synovitis.

    PubMed

    Nordemar, D; Öberg, J; Brosjö, O; Skorpil, M

    2015-01-01

    Purpose. To determine the incidence of intra-articular synovial sarcomas and investigate if any radiological variables can differentiate them from localized (unifocal) pigmented villonodular synovitis (PVNS) and if multivariate data analysis could be used as a complementary clinical tool. Methods. Magnetic resonance images and radiographs of 7 cases of intra-articular synovial sarcomas and 14 cases of localized PVNS were blindedly reviewed. Variables analyzed were size, extra-articular growth, tumor border, blooming, calcification, contrast media enhancement, effusion, bowl of grapes sign, triple signal intensity sign, synovial low signal intensity, synovitis, age, and gender. Univariate and multivariate data analysis, the method of partial least squares-discriminant analysis (PLS-DA), were used. Register data on all synovial sarcomas were extracted for comparison. Results. The incidence of intra-articular synovial sarcomas was 3%. PLS-DA showed that age, effusion, size, and gender were the most important factors for discrimination between sarcomas and localized PVNS. No sarcomas were misclassified as PVNS with PLS-DA, while some PVNS were misclassified as sarcomas. Conclusions. The most important variables in differentiating intra-articular sarcomas from localized PVNS were age, effusion, size, and gender. Multivariate data analysis can be helpful as additive information to avoid a biopsy, if the tumor is classified as most likely being PVNS.

  14. Intra-Articular Synovial Sarcomas: Incidence and Differentiating Features from Localized Pigmented Villonodular Synovitis

    PubMed Central

    Nordemar, D.; Öberg, J.; Brosjö, O.; Skorpil, M.

    2015-01-01

    Purpose. To determine the incidence of intra-articular synovial sarcomas and investigate if any radiological variables can differentiate them from localized (unifocal) pigmented villonodular synovitis (PVNS) and if multivariate data analysis could be used as a complementary clinical tool. Methods. Magnetic resonance images and radiographs of 7 cases of intra-articular synovial sarcomas and 14 cases of localized PVNS were blindedly reviewed. Variables analyzed were size, extra-articular growth, tumor border, blooming, calcification, contrast media enhancement, effusion, bowl of grapes sign, triple signal intensity sign, synovial low signal intensity, synovitis, age, and gender. Univariate and multivariate data analysis, the method of partial least squares-discriminant analysis (PLS-DA), were used. Register data on all synovial sarcomas were extracted for comparison. Results. The incidence of intra-articular synovial sarcomas was 3%. PLS-DA showed that age, effusion, size, and gender were the most important factors for discrimination between sarcomas and localized PVNS. No sarcomas were misclassified as PVNS with PLS-DA, while some PVNS were misclassified as sarcomas. Conclusions. The most important variables in differentiating intra-articular sarcomas from localized PVNS were age, effusion, size, and gender. Multivariate data analysis can be helpful as additive information to avoid a biopsy, if the tumor is classified as most likely being PVNS. PMID:26819567

  15. [Fast neutrons in the treatment of soft tissue sarcomas].

    PubMed

    Chernichenko, V A; Tolstopiatov, B A; Monich, A Iu; Konovalenko, V F; Galakhin, K A; Palivets, A Iu; Vorona, A M

    1990-01-01

    Results of treatment of 101 cases of soft tissue sarcoma are presented in the paper. Preoperative irradiation technique and radical program of treatment are described. Combined radiation and surgical treatment was given to 45 patients whereas conservative--to 56. Sixty-three cases received adjuvant combination chemotherapy. Response and three-year survival rates were compared to those in control group treated by photons. The results observed in patients of combined and conservative treatment groups who had been irradiated with fast neutrons proved significantly better than in controls. These data suggest vistas in application of fast neutron irradiation for the treatment of soft tissue sarcomas.

  16. The metabolic comparison of HPD in normal brain tissue and s180 sarcoma in brain

    NASA Astrophysics Data System (ADS)

    Wang, Yu; Zhu, Jing; Zhang, Huiguo

    2005-07-01

    Objective: Search The Metabolic Comparision of HPD in Normal Brain Tissue and S180 sarcoma in brain. Methods: Xe laser photoradiation of normal brain tissue and S180 sarcoma in brain to excite fluorescence was performed at different time after the injection of hematoporphyrin derivative (HpD) in normal rats and rats with S180 sarcoma of Brain. We analysed HPD fluorescence lifetime imaging (wavelength: 630 nm and 690 nm) of two rat team with OMA. Result: We concluded the peak intensity of HPD fluorescence fell obviously in normal brain three hours later after injection, while it still retained high HPD concentration in sarcoma of Brain. Conclusion: first-rank time the 4~5 hours after injection of HPD is first-rank time 0f PDT and photosensitive diagnose in sarcoma of Brain of rat

  17. Response of sarcomas of bone and of soft tissue to neutron beam therapy

    SciTech Connect

    Cohen, L.; Hendrickson, F.; Mansell, J.; Kurup, P.D.; Awschalom, M.; Rosenberg, I.; Ten Haken, R.K.

    1984-06-01

    A total of 51 patients were treated at Fermilab for sarcoma of bone (25 patients) and soft tissue (26 patients). Neutrons were delivered in twice weekly fractions over 6-7 weeks to total doses between 18 and 26 Gy. Long-term local control (greater than 2 years) was achieved in 24 patients (47%). Overall local control rates were 44% in the bone sarcomas and 50% in the soft tissue tumors. The overall survival rate was 40% in the entire series. These results are comparable with international experience in neutron therapy of sarcomas of bone and soft tissues. Out of 263 soft tissue sarcomas treated with neutrons only to full dosage throughout the world, 152 (58%) were locally controlled. Similarly out of 74 sarcomas of bone so treated 44 (60%) were controlled. Complications of the treatment are discussed.

  18. Distinct and overlapping sarcoma subtypes initiated from muscle stem and progenitor cells.

    PubMed

    Blum, Jordan M; Añó, Leonor; Li, Zhizhong; Van Mater, David; Bennett, Brian D; Sachdeva, Mohit; Lagutina, Irina; Zhang, Minsi; Mito, Jeffrey K; Dodd, Leslie G; Cardona, Diana M; Dodd, Rebecca D; Williams, Nerissa; Ma, Yan; Lepper, Christoph; Linardic, Corinne M; Mukherjee, Sayan; Grosveld, Gerard C; Fan, Chen-Ming; Kirsch, David G

    2013-11-27

    Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children, whereas undifferentiated pleomorphic sarcoma (UPS) is one of the most common soft tissue sarcomas diagnosed in adults. To investigate the myogenic cell(s) of origin of these sarcomas, we used Pax7-CreER and MyoD-CreER mice to transform Pax7(+) and MyoD(+) myogenic progenitors by expressing oncogenic Kras(G12D) and deleting Trp53 in vivo. Pax7-CreER mice developed RMS and UPS, whereas MyoD-CreER mice developed UPS. Using gene set enrichment analysis, RMS and UPS each clustered specifically within their human counterparts. These results suggest that RMS and UPS have distinct and overlapping cells of origin within the muscle lineage. Taking them together, we have established mouse models of soft tissue sarcoma from muscle stem and progenitor cells.

  19. Cross Species Genomic Analysis Identifies a Mouse Model as Undifferentiated Pleomorphic Sarcoma/Malignant Fibrous Histiocytoma

    PubMed Central

    Mito, Jeffrey K.; Riedel, Richard F.; Dodd, Leslie; Lahat, Guy; Lazar, Alexander J.; Dodd, Rebecca D.; Stangenberg, Lars; Eward, William C.; Hornicek, Francis J.; Yoon, Sam S.; Brigman, Brian E.; Jacks, Tyler; Lev, Dina; Mukherjee, Sayan; Kirsch, David G.

    2009-01-01

    Undifferentiated pleomorphic sarcoma/Malignant Fibrous Histiocytoma (MFH) is one of the most common subtypes of human soft tissue sarcoma. Using cross species genomic analysis, we define a geneset from the LSL-KrasG12D; Trp53Flox/Flox mouse model of soft tissue sarcoma that is highly enriched in human MFH. With this mouse geneset as a filter, we identify expression of the RAS target FOXM1 in human MFH. Expression of Foxm1 is elevated in mouse sarcomas that metastasize to the lung and tissue microarray analysis of human MFH correlates overexpression of FOXM1 with metastasis. These results suggest that genomic alterations present in human MFH are conserved in the LSL-KrasG12D; p53Flox/Flox mouse model of soft tissue sarcoma and demonstrate the utility of this pre-clinical model. PMID:19956606

  20. 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

  1. Olaratumab for the treatment of advanced soft tissue sarcoma.

    PubMed

    Okuno, Scott H; Maran, Avudaiappan; Robinson, Steven I

    2017-10-01

    Olaratumab, a human monoclonal antibody against platelet derived growth factor receptor alpha (PDGFR- α), is the first drug that in combination with doxorubicin for the treatment of patients with advanced/metastatic soft tissue sarcoma (STS) that has showed an improved overall survival compared to doxorubicin alone. These initial results are exciting and have the potential to change the landscape of treatment for patients with STS. Areas covered: This article reviews the development of olaratumab for oncology use by reviewing articles in PubMed for 'platelet derived growth factor' and 'receptor' and 'soft tissue sarcoma'. We provide an overview of the published studies to date for olaratumab and specifically the use in soft tissue sarcoma. Expert commentary: Olaratumab is a well-tolerated drug that, when combined with doxorubicin, has shown an improved overall survival compared to doxorubicin alone and the phase III confirmatory study is eagerly awaited.

  2. Stewart-Treves syndrome following idiopathic leg lymphoedema: remember sarcoma.

    PubMed

    Harrison, W D; Chandrasekar, C R

    2015-06-01

    Patients with skin complaints secondary to oedema are commonly encountered and can be a diagnostic challenge. Here, we present the case of a 78-year-old lady with Stewart-Treves syndrome, a rare cutaneous angiosarcoma. The histology showed angiocutaneous sarcoma with poorly defined margins. The prognosis remains extremely poor. A successful transfemoral amputation reduced the massive tumour burden and cleared a source of sepsis from this fungating tumour. Our aim is to highlight this rare but high-grade sarcoma which results from chronic lymphoedema. The red flag signs of sarcoma are masses greater than 5 cm, tumours which are rapidly growing, deep to fascia or painful, and tumours that recur after previous surgery. Non-healing or progressive ulceration in the background of chronic lymphoedema should raise the possibility of underlying malignancy. We need a low threshold for biopsy and early referral to an appropriate multidisciplinary team for the optimum management of the patient.

  3. Pulmonary artery sarcoma presenting as an isolated lung mass.

    PubMed

    Mori, Shohei; Uehara, Hirofumi; Motoi, Noriko; Okumura, Sakae

    2017-03-01

    We report a very rare case of pulmonary artery sarcoma that presented as an isolated lung mass, which we attempted to resect via lobectomy, although this resulted in incomplete resection due to unnoticed latent proximal presentations. A 54-year-old man complained of a dry cough that had persisted for 2 months. Enhanced chest computed tomography revealed a lobular mass in his left lower lobe. Therefore, left lower lobectomy was performed as a radical surgery, and the tumor was ultimately diagnosed as pulmonary artery sarcoma. However, follow-up computed tomography at 16 months revealed proximal presentations in the pulmonary trunk and right pulmonary artery, which we retrospectively discovered were present before the surgery. This case highlights the importance of evaluating the lumen of the pulmonary artery, to accurately determine the required extent of any radical surgery, even in cases of pulmonary artery sarcoma that presents as an isolated lung mass.

  4. Clinical Outcomes of Intraoperative Radiation Therapy for Extremity Sarcomas

    PubMed Central

    Tran, Quy N. H.; Kim, Anne C.; Gottschalk, Alexander R.; Wara, William M.; Phillips, Theodore L.; O'Donnell, Richard J.; Weinberg, Vivian; Haas-Kogan, Daphne A.

    2006-01-01

    Purpose. Radiation of extremity lesions, a key component of limb-sparing therapy, presents particular challenges, with significant risks of toxicities. We sought to explore the efficacy of intraoperative radiation therapy (IORT) in the treatment of soft tissue sarcomas of the extremities. Patients. Between 1995 and 2001, 17 patients received IORT for soft tissue sarcomas of the extremities. Indications for IORT included recurrent tumors in a previously radiated field or tumors adjacent to critical structures. Results. Gross total resections were achieved in all 17 patients. Two patients experienced locoregional relapses, six patients recurred at metastatic sites, and one patient died without recurrence. Thirty-six month estimates for locoregional control, disease free survival, and overall survival were 86%, 50%, and 78%, respectively. IORT was extremely well tolerated, with no toxicities referable to IORT. Conclusions. For patients with soft tissue sarcomas of the extremities, IORT used as a boost to EBRT provides excellent local control, with limited acute toxicities. PMID:17040093

  5. [Oral lesions in Kaposi sarcoma: clinical and radiotherapeutic considerations].

    PubMed

    Barberis, M; Brenna Betti, N; Lauritano, D; Sangiani, L; Spadari, F; Villa, S

    1996-01-01

    The epidemic form of Kaposi's sarcoma is the most frequent tumor in sieropositive patients. Every part of the body including oral cavity is affected by these lesions. According to modern acknowledgement in treating oropharynge carcinoma, radiotherapy is used for management of oral Kaposi's sarcoma. This paper reports a study of 10 patients suffering from Kaposi's sarcoma correlated to AIDS (EKS) treated with radiotherapy and chemiotherapy, achieving good results, at the Istituto Nazionale per lo Studio e la Cura dei Tumori of Milan (Divisone di Radioterapia C) from 1988 to 1992. Treatment has been performed using linear accelerator (6 Mev) or Co 60 unity in order to reach the deepest layer of mucosa lesions. Radiotherapy schedule consisted of 150-200 cGy daily fractions given 5 times/week (w) for 4-5 w in split-course.

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

    PubMed Central

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

    2016-01-01

    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. PMID:28050423

  7. Hypopituitarism due to lymphocytic hypophysitis in a patient with retroperitoneal fibrosis.

    PubMed Central

    Alvarez, A.; Cordido, F.; Sacristán, F.

    1997-01-01

    We present a 78-year-old woman with clinical acute hypopituitarism in whom pathologic findings showed lymphocytic hypophysitis and retroperitoneal fibrosis. Lymphocytic hypophysitis should be included in the differential diagnosis of hypopituitarism at any age. The association with idiopathic retroperitoneal fibrosis suggest an autoimmune origin for this entity. Images Figure 1 Figure 2 PMID:9519190

  8. 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

  9. Impact of EWS-ETS fusion type on disease progression in Ewing's sarcoma/peripheral primitive neuroectodermal tumor: prospective results from the cooperative Euro-E.W.I.N.G. 99 trial.

    PubMed

    Le Deley, Marie-Cecile; Delattre, Olivier; Schaefer, Karl-Ludwig; Burchill, Sue A; Koehler, Gabriele; Hogendoorn, Pancras C W; Lion, Thomas; Poremba, Christopher; Marandet, Julien; Ballet, Stelly; Pierron, Gaelle; Brownhill, Samantha C; Nesslböck, Michaela; Ranft, Andreas; Dirksen, Uta; Oberlin, Odile; Lewis, Ian J; Craft, Alan W; Jürgens, Heribert; Kovar, Heinrich

    2010-04-20

    PURPOSE EWS-ETS fusion genes are the driving force in Ewing's sarcoma pathogenesis. Because of the variable breakpoint locations in the involved genes, there is heterogeneity in fusion RNA and protein architecture. Since previous retrospective studies suggested prognostic differences among patients expressing different EWS-FLI1 fusion types, the impact of fusion RNA architecture on disease progression and relapse was studied prospectively within the Euro-E.W.I.N.G. 99 clinical trial. PATIENTS AND METHODS Among 1,957 patients who registered before January 1, 2007, 703 primary tumors were accessible for the molecular biology study. Fusion type was assessed by polymerase chain reaction on frozen (n = 578) or paraffin-embedded materials (n = 125). The primary end point was the time to disease progression or relapse. Results After exclusion of noninformative patients, 565 patients were entered into the prognostic factor analysis comparing type 1 (n = 296), type 2 (n = 133), nontype 1/nontype 2 EWS-FLI1 (n = 91) and EWS-ERG fusions (n = 45). Median follow-up time was 4.5 years. The distribution of sex, age, tumor volume, tumor site, disease extension, or histologic response did not differ between the four fusion type groups. We did not observe any significant prognostic value of the fusion type on the risk of progression or relapse. The only slight difference was that the risk of progression or relapse associated with nontype 1/nontype 2 EWS-FLI1 fusions was 1.38 (95% CI, 0.96 to 2.0) times higher than risk associated with other fusion types, but it was not significant (P = .10). CONCLUSION In contrast to retrospective studies, the prospective evaluation did not confirm a prognostic benefit for type 1 EWS-FLI1 fusions.

  10. Radioimmunoimaging of osteogenic sarcoma xenografts in nude mice using monoclonal antibodies to osteogenic sarcoma

    SciTech Connect

    Sakahara, H.; Endo, K.; Nakashima, T.; Koizumi, M.; Ohta, H.; Kunimatsu, M.; Torizuka, K.; Nakamura, T.; Tanaka, H.; Kotoura, Y.

    1985-05-01

    The authors have developed several monoclonal antibodies against human osteogenic sarcoma, one of which; OST7 (IgGl) selectively localized in osteogenic sarcoma xenografts in nude mice. In the present study, F(ab')/sub 2/ fragment was compared with whole IgG and those labeled with In-111 as well as I-131 were used as a radiotracer for the scintigraphic imaging of tumors. IgC and F(ab')/sub 2/ were labeled with I-131 using chloramine-T method and injected into nude mice bearing human osteogenic sarcoma. Scintigrams at day 2 clearly delineated the site of tumors with almost no radioactivity in other organs with F(ab')/sub 2/, which yielded much better images than whole IgG. Tumor-to-blood ratio of 6.09-27.87 was obtained at day 2 using F(ab')/sub 2/, whereas it was 0.76-1.12 at day 2 and 2.05-3.27 at day 7 with IgG. I-131 labeled nonspecific F(ab')/sub 2/ or IgG resulted in no or very low tumor uptake with tumor-to-blood ratio of 0.94-1.18 at day 2 for F(ab')/sub 2/ and 0.67-0.76 at day 7 for IgG, respectively. In-111 labeled F(ab')/sub 2/ fragment of OST7, which was prepared using DTPA as a bifunctional chelate, also showed a high tumor accumulation with tumor-to-blood ratio of 11.67-17.54 at day 2, but higher background activity in the liver and kidney was observed than I-131 labeled one. These results indicate that F(ab')/sub 2/ fragment of OST7 labeled with either I-131 or In-111, has a great potential for the radioimmunoimaging of osteogenic sarcoma.

  11. mTOR inhibitors block Kaposi sarcoma growth by inhibiting essential autocrine growth factors and tumor angiogenesis.

    PubMed

    Roy, Debasmita; Sin, Sang-Hoon; Lucas, Amy; Venkataramanan, Raman; Wang, Ling; Eason, Anthony; Chavakula, Veenadhari; Hilton, Isaac B; Tamburro, Kristen M; Damania, Blossom; Dittmer, Dirk P

    2013-04-01

    Kaposi sarcoma originates from endothelial cells and it is one of the most overt angiogenic tumors. In Sub-Saharan Africa, where HIV and the Kaposi sarcoma-associated herpesvirus (KSHV) are endemic, Kaposi sarcoma is the most common cancer overall, but model systems for disease study are insufficient. Here, we report the development of a novel mouse model of Kaposi sarcoma, where KSHV is retained stably and tumors are elicited rapidly. Tumor growth was sensitive to specific allosteric inhibitors (rapamycin, CCI-779, and RAD001) of the pivotal cell growth regulator mTOR. Inhibition of tumor growth was durable up to 130 days and reversible. mTOR blockade reduced VEGF secretion and formation of tumor vasculature. Together, the results show that mTOR inhibitors exert a direct anti-Kaposi sarcoma effect by inhibiting angiogenesis and paracrine effectors, suggesting their application as a new treatment modality for Kaposi sarcoma and other cancers of endothelial origin.

  12. A survey of the interactome of Kaposi's sarcoma-associated herpesvirus ORF45 revealed its binding to viral ORF33 and cellular USP7, resulting in stabilization of ORF33 that is required for production of progeny viruses.

    PubMed

    Gillen, Joseph; Li, Wenwei; Liang, Qiming; Avey, Denis; Wu, Jianjun; Wu, Fayi; Myoung, JinJong; Zhu, Fanxiu

    2015-05-01

    The ORF45 protein of Kaposi's sarcoma-associated herpesvirus (KSHV) is a gammaherpesvirus-specific immediate-early tegument protein. Our previous studies have revealed its crucial roles in both early and late stages of KSHV infection. In this study, we surveyed the interactome of ORF45 using a panel of monoclonal antibodies. In addition to the previously identified extracellular regulated kinase (ERK) and p90 ribosomal S6 kinase (RSK) proteins, we found several other copurified proteins, including prominent ones of ∼38 kDa and ∼130 kDa. Mass spectrometry revealed that the 38-kDa protein is viral ORF33 and the 130-kDa protein is cellular USP7 (ubiquitin-specific protease 7). We mapped the ORF33-binding domain to the highly conserved carboxyl-terminal 19 amino acids (aa) of ORF45 and the USP7-binding domain to the reported consensus motif in the central region of ORF45. Using immunofluorescence staining, we observed colocalization of ORF45 with ORF33 or USP7 both under transfected conditions and in KSHV-infected cells. Moreover, we noticed ORF45-dependent relocalization of a portion of ORF33/USP7 from the nucleus to the cytoplasm. We found that ORF45 caused an increase in ORF33 protein accumulation that was abolished if either the ORF33- or USP7-binding domain in ORF45 was deleted. Furthermore, deletion of the conserved carboxyl terminus of ORF45 in the KSHV genome drastically reduced the level of ORF33 protein in KSHV-infected cells and abolished production of progeny virions. Collectively, our results not only reveal new components of the ORF45 interactome, but also demonstrate that the interactions among these proteins are crucial for KSHV lytic replication. Kaposi's sarcoma-associated herpesvirus (KSHV) is the causative agent of several human cancers. KSHV ORF45 is a multifunctional protein that is required for KSHV lytic replication, but the exact mechanisms by which ORF45 performs its critical functions are unclear. Our previous studies revealed that all

  13. A Survey of the Interactome of Kaposi's Sarcoma-Associated Herpesvirus ORF45 Revealed Its Binding to Viral ORF33 and Cellular USP7, Resulting in Stabilization of ORF33 That Is Required for Production of Progeny Viruses

    PubMed Central

    Gillen, Joseph; Li, Wenwei; Liang, Qiming; Avey, Denis; Wu, Jianjun; Wu, Fayi; Myoung, JinJong

    2015-01-01

    ABSTRACT The ORF45 protein of Kaposi's sarcoma-associated herpesvirus (KSHV) is a gammaherpesvirus-specific immediate-early tegument protein. Our previous studies have revealed its crucial roles in both early and late stages of KSHV infection. In this study, we surveyed the interactome of ORF45 using a panel of monoclonal antibodies. In addition to the previously identified extracellular regulated kinase (ERK) and p90 ribosomal S6 kinase (RSK) proteins, we found several other copurified proteins, including prominent ones of ∼38 kDa and ∼130 kDa. Mass spectrometry revealed that the 38-kDa protein is viral ORF33 and the 130-kDa protein is cellular USP7 (ubiquitin-specific protease 7). We mapped the ORF33-binding domain to the highly conserved carboxyl-terminal 19 amino acids (aa) of ORF45 and the USP7-binding domain to the reported consensus motif in the central region of ORF45. Using immunofluorescence staining, we observed colocalization of ORF45 with ORF33 or USP7 both under transfected conditions and in KSHV-infected cells. Moreover, we noticed ORF45-dependent relocalization of a portion of ORF33/USP7 from the nucleus to the cytoplasm. We found that ORF45 caused an increase in ORF33 protein accumulation that was abolished if either the ORF33- or USP7-binding domain in ORF45 was deleted. Furthermore, deletion of the conserved carboxyl terminus of ORF45 in the KSHV genome drastically reduced the level of ORF33 protein in KSHV-infected cells and abolished production of progeny virions. Collectively, our results not only reveal new components of the ORF45 interactome, but also demonstrate that the interactions among these proteins are crucial for KSHV lytic replication. IMPORTANCE Kaposi's sarcoma-associated herpesvirus (KSHV) is the causative agent of several human cancers. KSHV ORF45 is a multifunctional protein that is required for KSHV lytic replication, but the exact mechanisms by which ORF45 performs its critical functions are unclear. Our previous studies

  14. Characteristics of abdominal cavity drainage fluid in Chinese patients without postoperative complications after surgery for gastrointestinal or retroperitoneal tumors

    PubMed Central

    Zhou, Jing; Pi, Hongying; Zheng, Yingying

    2015-01-01

    Background Abdominal cavity drainage fluid can be used as an early diagnostic tool of postoperative complications, and observing its characteristics can help us to judge and handle postoperative complications. There is no accurate standard reference range on its characteristics after surgeries for gastrointestinal or retroperitoneal tumors. This research attempted to analyze its characteristics in Chinese patients without postoperative complications after surgery for gastrointestinal or retroperitoneal tumors, and to offer an experimental basis for establishing a reliable standard reference range for abdominal cavity drainage fluid used to detect postoperative complications. Methods This study enrolled 262 Chinese patients without postoperative complications after surgery for gastrointestinal or retroperitoneal tumors. Results All patients had a median age (range) of 55 (19–72) years, and 150 (57.3%) were men. There were 93 (35.5%), 115 (43.9%), and 54 (20.6%) patients who underwent surgery for upper gastrointestinal tumors, lower gastrointestinal tumors, and retroperitoneal tumors, respectively. The total amount, density, and pH of the abdominal cavity drainage fluid were 204 (0–6,195) mL, 1.032 (1.011–1.047) kg/m3, and pH 7.0 (5.0–7.5), respectively. The total numbers of cells and white blood cells were 1.3×1011 (5.5×108–6.2×1012)/L and 3.7×109 (1.0×107–5.0×1011)/L, respectively. The levels of sugar and protein were 3 (0–37) mmol/L and 39 (1–272) g/L. The total amount of abdominal cavity drainage fluid, the total number of cells, the total number of white blood cells, the number of multinucleated cells, the number of monocytes, and the levels of sugar were statistically significantly different between the three groups (P<0.05 for all). Conclusion This study described the characteristics of abdominal cavity drainage fluid in Chinese patients without postoperative complications after surgery for gastrointestinal or retroperitoneal tumors, and

  15. 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.

  16. Gastroesophageal cancer and retroperitoneal fibrosis: Two case reports and review of the literature

    PubMed Central

    Peixoto, Renata D’Alpino; Al-Barrak, Jasem; Lim, Howard; Renouf, Daniel

    2013-01-01

    Retroperitoneal fibrosis secondary to malignant disease is a rare condition associated with a dismal prognosis. We herein present the first ever reported case of retroperitoneal fibrosis related to esophageal adenocarcinoma in a 63-year-old patient who developed bilateral ureteral obstruction due to extensive retroperitoneal fibrosis 18 mo after having completed neoadjuvant chemoradation followed by surgery for a pT3N0 adenocarcinoma of the distal esophagus. We also report the case of a previously healthy woman who presented with bilateral ureteral obstruction and diffuse narrowing of the common biliary duct and was found to have extensive retroperitoneal fibrosis as a consequence of metastatic gastric adenocarcinoma. Both patients had poor performance status and were unsuitable for palliative chemotherapy. This paper shows that urinary and biliary obstructive symptoms might represent retroperitoneal fibrosis as a consequence of gastroesophageal malignancy. PMID:23671733

  17. Gastroesophageal cancer and retroperitoneal fibrosis: Two case reports and review of the literature.

    PubMed

    Peixoto, Renata D'Alpino; Al-Barrak, Jasem; Lim, Howard; Renouf, Daniel

    2013-03-15

    Retroperitoneal fibrosis secondary to malignant disease is a rare condition associated with a dismal prognosis. We herein present the first ever reported case of retroperitoneal fibrosis related to esophageal adenocarcinoma in a 63-year-old patient who developed bilateral ureteral obstruction due to extensive retroperitoneal fibrosis 18 mo after having completed neoadjuvant chemoradation followed by surgery for a pT3N0 adenocarcinoma of the distal esophagus. We also report the case of a previously healthy woman who presented with bilateral ureteral obstruction and diffuse narrowing of the common biliary duct and was found to have extensive retroperitoneal fibrosis as a consequence of metastatic gastric adenocarcinoma. Both patients had poor performance status and were unsuitable for palliative chemotherapy. This paper shows that urinary and biliary obstructive symptoms might represent retroperitoneal fibrosis as a consequence of gastroesophageal malignancy.

  18. A rare retroperitoneal schwannoma in a patient with neurofibromatosis Type 2.

    PubMed

    Patrinou, Alexandra; Malindretos, Pavlos; Koutroubas, Georgios; Anagnostou, Nikolaos; Argiraki, Elefteria; Syrganis, Christos

    2010-06-01

    Neurofibromatosis Type 2 (NF2) is a dominantly inherited tumour-prone disorder, characterized by the development of multiple schwannomas, meningiomas and ependymomas. Its prevalence is around 1:60 000. Vestibular schwannoma (VS) is the hallmark of NF2. Retroperitoneal schwannomas are expected to occur in only 3% of cases. We present the case of a large retroperitoneal schwannoma in a patient with NF2. A well-circumscribed heterogenic mass (9.5 × 4 × 4 cm) behind and under the left kidney and extending into the left retroperitoneal space was revealed during a lumbar and retroperitoneal space magnetic resonance imaging (MRI). Brain, orbits, cervical, thoracic and lumbar MRI revealed bilateral VS, multiple meningiomas as well as multiple schwannomas and ependymomas in the cervical, thoracic and lumbar spine. The retroperitoneal mass represents a schwannoma probably derived from an intercostal nerve. The patient underwent neurosurgical excision of the VS, and 3 months later, the patient's condition remained stable.

  19. Incidence and management of Kaposi sarcoma in renal transplant recipients: the Greek experience.

    PubMed

    Zavos, G; Moris, D; Vernadakis, S; Bokos, J; Lionaki, S; Mamarelis, G; Panagiotellis, K; Zavvos, V; Boletis, I

    2014-11-01

    One of the most common malignancies in kidney transplant recipients is Kaposi sarcoma. The incidence of Kaposi sarcoma, which develops after renal transplantation, is 400-500 times higher than that in the general population. The aims of this study were to review the experience with Kaposi sarcoma in the highest-volume transplantation Unit in Greece and to analyze clinical characteristics and response to treatment, with respect to both the patients' survival and the renal graft function. The records of 2008 renal graft recipients between March 1983 and December 2012 were retrospectively reviewed. Kaposi sarcoma was diagnosed based on clinical, laboratory, radiological, endoscopic, and histopathologic examinations. The disease was staged according to the classification of Al-Khader et al. The prevalence of Kaposi sarcoma was 1.2% in our renal transplant population. Of these, 1006 recipients underwent living-donor renal transplantation, whereas 1002 received their graft from deceased donors. Post-transplantation malignancy developed in 153 patients, among which, Kaposi sarcoma has been found in 24 cases. Of the 24 cases of Kaposi sarcoma, lesions were mainly cutaneous in 14 cases, visceral and cutaneous in 8, and concomitant visceral and lymph node involvement was observed in 2 patients. With regard to the final outcome, 20 patients (83.3%) showed remission of the disease, whereas 4 patients with visceral involvement (16.6%) did not respond to chemotherapy and discontinuation of immunosuppression and died. Moreover, 8 deaths occurred due to apparently unrelated causes. Kaposi sarcoma is an important part (15.7%) of all post-transplantation neoplasias in our series. Furthermore, our findings confirmed the previously described close association between human herpesvirus-8 and post-transplantation Kaposi sarcoma. Reduction of immunosuppression or discontinuation of calcineurin inhibitors results in remission of the disease in most of the cases. Prognosis in patients with

  20. 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

  1. Kaposi sarcoma associated with lipoedema.

    PubMed

    Ekmekci, T R; Ayabakan, O; Sakiz, D; Koslu, A

    2005-05-01

    Lipoedema is a form of lipodistrophy, which consists of abnormal accumulation of fat in subcutaneous tissue of the lower limbs. It does not cause any disease and it has not been reported association with malignity. We describe a 63-year-old woman occurring of Kaposi sarcoma on the lipoedema base.

  2. Pulmonary Artery Sarcoma - Multimodality Imaging.

    PubMed

    Jeong, Nari; Seol, Sang-Hoon; Kim, Il Hwan; Kim, Ji Yeon

    2016-01-01

    Pulmonary artery sarcoma (PAS) is a rare and fatal disease. PAS can often be misdiagnosed as pulmonary thromboembolism. Moreover, the correct diagnosis is frequently delayed due to nonspecific signs and symptoms. The prognosis of patients with PAS is poor. We report a case of a woman with a primary PAS who was initially diagnosed with pulmonary thromboembolism.

  3. Pulmonary Artery Sarcoma - Multimodality Imaging

    PubMed Central

    Jeong, Nari; Seol, Sang-Hoon; Kim, Il Hwan; Kim, Ji Yeon

    2016-01-01

    Pulmonary artery sarcoma (PAS) is a rare and fatal disease. PAS can often be misdiagnosed as pulmonary thromboembolism. Moreover, the correct diagnosis is frequently delayed due to nonspecific signs and symptoms. The prognosis of patients with PAS is poor. We report a case of a woman with a primary PAS who was initially diagnosed with pulmonary thromboembolism. PMID:27833785

  4. Retroperitoneal anatomy during excision of pelvic side wall endometriosis

    PubMed Central

    Gingold, Julian A.; Falcone, Tommaso

    2016-01-01

    Surgical management of endometriosis has been shown to improve dysmenorrhea at all disease stages and is recommended in severe disease for treatment of infertility. Deeply infiltrating endometriosis (DIE) produces thick inflammatory tissue that precludes visualization of anatomical landmarks and distorts normal anatomy. Excision of DIE poses several technical and surgical challenges that mandate a clear understanding of the anatomy of the pelvic sidewall. This review details relevant surgical anatomy and addresses the principles of safe retroperitoneal entry, ureterolysis and excision of endometriotic lesions. Proper use of these techniques should facilitate safe and successful surgery for management of DIE. PMID:27642583

  5. Retroperitoneal schwannoma with features of gastrointestinal schwannoma. A case report.

    PubMed

    Zámecník, M; Koys, F

    2003-01-01

    A case of retroperitoneal schwannoma with histological features of gastrointestinal (GI) schwannoma occurring in a 67-year-old woman is reported. The tumor was composed of spindle cells with focal pseudoatypism and it showed several features typical of gastrointestinal-type schwannoma such as lymphocytic infiltration, peripheral cuff of lymphocytes, lack of Antoni A pattern, and absence of thick walled vessels. Immunohistochemically, the tumor showed diffuse reactivity for S100 protein and glial fibrillary acidic protein. The authors discuss a phenotypical similarity of the lesion with GI schwannoma as well as the possible existence of GI-type schwannoma outside the tubal GI tract.

  6. Survival analysis of pure seminoma at post-chemotherapy retroperitoneal lymph node dissection.

    PubMed

    Rice, Kevin R; Beck, Stephen D W; Bihrle, Richard; Cary, K Clint; Einhorn, Lawrence H; Foster, Richard S

    2014-11-01

    Viable seminoma encountered at post-chemotherapy retroperitoneal lymph node dissection for pure testicular seminoma is rare due to the chemosensitivity of this germ cell tumor. In this study we define the natural history of viable seminoma at post-chemotherapy retroperitoneal lymph node dissection. The Indiana University testis cancer database was queried from 1988 to 2011 to identify all patients with primary testicular or retroperitoneal pure seminoma and who were found to have pure seminoma at post-chemotherapy retroperitoneal lymph node dissection. Clinical characteristics were reviewed and survival analysis was performed. A total of 36 patients met the study inclusion criteria. All patients received standard first line cisplatin based chemotherapy and 17 received salvage chemotherapy. The decision to proceed to retroperitoneal lymph node dissection was based on enlarging retroperitoneal mass and/or positron emission positivity in the majority of cases. Seven patients had undergone previous retroperitoneal lymph node dissection. Additional surgical procedures were required in 19 patients to achieve a complete resection. The 5-year cancer specific survival rate was 54%. However, only 9 of 36 patients remained continuously free of disease and of these patients 4 received adjuvant chemotherapy. Mean time from post-chemotherapy retroperitoneal lymph node dissection to death was 6.9 months. Second line chemotherapy, reoperative retroperitoneal lymph node dissection and earlier era of treatment were associated with poorer cancer specific survival. A total of 36 patients with pure seminoma were found to have viable pure seminoma at post-chemotherapy retroperitoneal lymph node dissection. While 5-year cancer specific survival was 54%, these surgeries are technically demanding and only a minority of patients achieves a durable cure from surgery alone. Copyright © 2014 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights

  7. Targeted mutation of p53 and Rb in mesenchymal cells of the limb bud produces sarcomas in mice.

    PubMed

    Lin, Patrick P; Pandey, Manoj K; Jin, Fenghua; Raymond, A Kevin; Akiyama, Haruhiko; Lozano, Guillermina

    2009-10-01

    Mice bearing germ line mutations of p53 develop sarcomas at a significant rate. Since they are susceptible to a variety of other malignancies, they are not ideally suited to the study of sarcomas. To test the possibility that targeted mutation of tumor suppressor genes in early mesenchymal cells would induce formation of sarcomas, the Prx1-cre transgenic mouse was crossed to mice-bearing floxed alleles of p53 and Rb. Mice with homozygous deletion of p53 (Prx1-cre p53(lox/lox)) developed sarcomas in the extremities at a mean time of 50 weeks. Osteosarcomas (OS) were the most common type of sarcoma (61%) followed by poorly differentiated soft tissue sarcomas (PDSTS) (32%). Homozygous deletion of p53 produced sarcomas significantly more rapidly than heterozygous deletion, which resulted in sarcoma formation after a mean of 96 weeks. Mice with homozygous Rb mutation (Prx1-cre Rb(lox/lox)) developed normally and had no ostensible defects in the limbs. In contrast to p53, targeted deletion of Rb did not produce sarcomas in the limbs. However, simultaneous deletion of Rb and p53 accelerated the time to sarcoma formation, and a greater percentage of PDSTS were found. Deletion of p53 in committed osteoblasts by the Col1a1-cre transgenic mouse bearing an osteoblast-specific enhancer resulted in a high percentage of OS. These findings suggest that deletion of p53 in mesenchymal cells that give rise to osteoblasts is a powerful initiator of OS. Deletion of Rb does not initiate sarcoma formation in mice, but it accelerates formation of both soft tissue sarcomas and OS.

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

    ClinicalTrials.gov

    2017-10-12

    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

  9. CCI-779 in Treating Patients With Soft Tissue Sarcoma or Gastrointestinal Stromal Tumor

    ClinicalTrials.gov

    2013-06-03

    Gastrointestinal Stromal Tumor; Recurrent Adult 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

  10. What Are the Key Statistics about Kaposi Sarcoma?

    MedlinePlus

    ... Sarcoma About Kaposi Sarcoma What Are the Key Statistics About Kaposi Sarcoma? Before the AIDS epidemic, Kaposi ... and children. Visit the American Cancer Society’s Cancer Statistics Center for more key statistics. Written by References ...

  11. What's New in Kaposi Sarcoma Research and Treatment?

    MedlinePlus

    ... and Treatment? Kaposi Sarcoma About Kaposi Sarcoma What’s New in Kaposi Sarcoma Research and Treatment? A great ... once it has developed. Treatment Researchers are studying new and different ways to treat KS. Imiquimod (Aldara) ...

  12. What's New in Uterine Sarcoma Research and Treatment?

    MedlinePlus

    ... and Treatment? Uterine Sarcoma About Uterine Sarcoma What's New in Uterine Sarcoma Research and Treatment? Molecular pathology ... the chromosomes leads to the formation of a new gene, called JAZF1/JJAZ. This gene may help ...

  13. What Are the Key Statistics about Uterine Sarcoma?

    MedlinePlus

    ... Sarcoma About Uterine Sarcoma What Are the Key Statistics About Uterine Sarcoma? The American Cancer Society's estimates ... uterine corpus. Visit the American Cancer Society’s Cancer Statistics Center for more key statistics. Written by References ...

  14. Clitoris metastasis from a retroperitoneal leiomyosarcoma: A case report

    PubMed Central

    Cokmert, Suna; Demir, Lutfiye; Akyol, Murat; Bayoglu, Ibrahim Vedat; Can, Alper; Unek, Ilkay Tugba; Bolat, Filiz Aka

    2014-01-01

    Leiomyosarcoma is a rare form of cancer commonly found in the retroperitoneum, uterus, stomach, small intestine and vascular tissue. Surgery with a wide margin of resection is the most effective treatment. Nevertheless, metastasis is common and generally occurs within the first 3 years. The liver and lungs are the most common sites of metastasis in leiomyosarcoma. Other sites of metastasis include bone, spleen, soft tissues and brain. Metastatic tumours of the clitoris are extremely rare. As cited in the literature, the most common cancers that metastasize to the clitoris are breast, bladder, renal and gastric. Here, we report a case of a clitoral mass in a 64-year-old woman who received an operation for retroperitoneal leiomyosarcoma 4 years prior. Mass resection was performed. The pathological diagnosis was a leiomyosarcoma metastasis. The patient also presented with brain and lung metastases at the time of the clitoral metastasis. This is the first case of clitoral and brain metastases originating from a retroperitoneal leiomyosarcoma. PMID:24527400

  15. Retroperitoneal fibrosis – the state-of-the-art

    PubMed Central

    Majewski, Dominik; Puszczewicz, Mariusz

    2016-01-01

    Retroperitoneal fibrosis (RPF) is a rare disease, hallmarked by inflammation and deposition of fibrous tissue around the abdominal aorta. This process may spread contiguously and involve adjacent structures, leading to many complications, among which the most frequent and most severe is ureteral obstruction. The condition usually has idiopathic origin (idiopathic retroperitoneal fibrosis – IRF), but can also develop secondarily to a number of factors. The etiology of the disease remains unclear. Current research suggests that about half of the cases of IRF may be a symptom of a recently discovered, clinically heterogeneous immunoglobulin G4-related disease (IgG4-RD). Corticosteroids are the first-line treatment for IRF, but effective attempts to use immunosuppressants are also made. This paper presents the current state of knowledge on the etiopathogenesis, clinical presentation, diagnosis and therapeutic possibilities in different forms of RPF. Based on the latest research, an analysis of the relationship between IRF and IgG4-RD was performed. PMID:27994271

  16. Retroperitoneal perforation of the duodenum from biliary stent erosion.

    PubMed

    Miller, George; Yim, Duke; Macari, Michael; Harris, Marsha; Shamamian, Peter

    2005-01-01

    Endoscopically placed biliary stents have supplanted surgical decompression as the preferred treatment option for patients with obstructive jaundice from advanced pancreatic cancer. An unusual complication of indewelling biliary stents is duodenal perforation into the retroperitoneum. We describe the case of a patient with end-stage pancreatic cancer who presented with an acute abdomen from erosion of a previously placed bile duct stent through the wall of the second portion of the duodenum. Although our patient presented with advanced symptoms, clinical presentations can vary from mild abdominal discomfort and general malaise to overt septic shock. Definitive diagnosis is best made with computed tomography (CT) imaging, which can detect traces of retroperitoneal air and fluid. Treatment options vary from nonoperative management with antibiotics, bowel rest, and parenteral alimentation in the most stable patients to definitive surgery with complete diversion of gastric contents and biliary flow from the affected area in patients with clinical symptoms or radiologic evidence suggesting extensive contamination. Complications of management can include duodenal fistulization, residual retroperitoneal or intrabdominal abscess, and ongoing sepsis. This report highlights the salient issues in the presentation, diagnosis, and modern management of patients with this rare complication of indwelling biliary stents.

  17. Kaposi Sarcoma Incidence and Survival Among HIV-Infected Homosexual Men After HIV Seroconversion

    PubMed Central

    Guiguet, Marguerite; Costagliola, Dominique; Fisher, Martin; de Luca, Andrea; Porter, Kholoud

    2010-01-01

    Background Despite the success of combination antiretroviral therapy (cART) in reducing the incidence of Kaposi sarcoma, HIV-infected individuals who have responded to treatment continue to be diagnosed with Kaposi sarcoma. We examine factors associated with the incidence of Kaposi sarcoma among cART-treated HIV-infected homosexual men and changes in their survival after its diagnosis over calendar time. Methods Data were from HIV-infected homosexual men with well-estimated dates of HIV seroconversion (ie, change in status from being HIV negative to having HIV antibodies detected). Incidence of Kaposi sarcoma was calculated. We used Kaplan–Meier methods to determine survival after Kaposi sarcoma diagnosis in three calendar periods: before 1996, 1996–2000, and 2001–2006. Poisson models were used to examine the effect of risk factors such as current and nadir CD4 cell count (ie, the lowest CD4 cell count ever recorded for a person), duration of infection, and age at diagnosis for Kaposi sarcoma incidence in cART-treated men. All statistical tests were two-sided. Results Among the 9473 men, 555 were diagnosed with Kaposi sarcoma in the period 1986–2006, of whom 319 died. The percentage surviving 24 months after Kaposi sarcoma diagnosis rose statistically significantly during the study period from 35% (95% confidence interval [CI] = 29% to 42%) before 1996 to 84% (95% CI = 76% to 90%) in 1996–2000 and to 81% (95% CI = 70% to 88%) in 2001–2006 (P < .001). Seventy men were diagnosed with Kaposi sarcoma after starting cART. Current (ie, within 6 months) CD4 cell count was associated with incidence of Kaposi sarcoma among cART-treated men (rate ratios [RRs] = 18.91, 95% CI = 8.50 to 42.09, for CD4 level category <200 cells per cubic millimeter; RR = 3.55, 95% CI = 1.40 to 9.00, for 200–349 cells per cubic millimeter; and RR = 4.11, 95% CI = 1.74 to 9.70, for 350–499 cells per cubic millimeter; all compared with ≥500 cells per cubic millimeter). After

  18. Acquired myasthenia gravis associated with oral sarcoma in a dog.

    PubMed

    Stepaniuk, Kevin; Legendre, Loïc; Watson, Shelby

    2011-01-01

    Acquired myasthenia gravis is a common neuromuscular disorder resulting from autoantibody directed against the post-synaptic acetylcholine nicotinic receptors in skeletal muscle. Myasthenia gravis has been reported previously as a paraneoplastic syndrome. This case report presents myasthenia gravis secondary to an oral sarcoma in a juvenile Mastiffdog.

  19. EXPERIMENTAL PRODUCTION AND TRANSMISSION OF TAR SARCOMAS IN CHICKENS

    PubMed Central

    Murphy, James B.; Landsteiner, Karl

    1925-01-01

    Two spindle cell sarcomas have developed as the result, of injection of coal tar into induced embryomas in chickens. One of these, transplanted to other chickens for eleven generations, is highly invasive in character, and metastasizes freely. Attempts to transmit this neoplasm by a filtrate or desiccate of the tumor have failed of success. PMID:19869028

  20. Ribociclib and Doxorubicin in Treating Patients With Metastatic or Advanced Soft Tissue Sarcomas That Cannot Be Removed by Surgery

    ClinicalTrials.gov

    2017-04-10

    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

  1. 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.

  2. Noncoding RNA in drug resistant sarcoma

    PubMed Central

    Li, Xiaoyang; Shen, Jacson K.; Hornicek, Francis J.; Xiao, Tao; Duan, Zhenfeng

    2017-01-01

    Sarcomas are a group of malignant tumors that arise from mesenchymal origin. Despite significant development of multidisciplinary treatments for sarcoma, survival rates have reached a plateau. Chemotherapy has been extensively used for sarcoma treatment; however, the development of drug resistance is a major obstacle limiting the success of many anticancer agents. Sarcoma biology has traditionally focused on genomic and epigenomic deregulation of protein-coding genes to identify the therapeutic potential for reversing drug resistance. New and more creative approaches have found the involvement of noncoding RNAs, including microRNAs and long noncoding RNAs in drug resistant sarcoma. In this review, we discuss the current knowledge of noncoding RNAs characteristics and the regulated genes involved in drug resistant sarcoma, and focus on their therapeutic potential in the future.

  3. Soft Tissue Sarcomas of the Kidney

    PubMed Central

    Köhle, Olivia; Abt, Dominik; Rothermundt, Christian; Öhlschlegel, Christian; Brugnolaro, Christiane; Schmid, Hans-Peter

    2015-01-01

    Soft tissue sarcomas are rare mesenchymal tumors. Amongst others, primitive neuroectodermal tumors (PNET) of the kidney and synovial sarcoma of the kidney belong to the group of soft tissue sarcomas. Synovial sarcomas can occur almost anywhere in the body, most frequently, however, in the lower (62%) or upper extremities (21%). Metastases occur in 50-70% of cases, and thus the prognosis is poor. PNETs are rare, highly aggressive neoplastic lesions which mainly occur in the torso or axial skeleton in young adults. The prognosis is poor with a 5-year disease-free survival rate of 45-55%. The primary therapeutic approach is surgical resection. Most randomized studies assessing adjuvant chemotherapy for all types of localized soft tissue sarcomas did not show statistically significantly better overall survival times after chemotherapy, although they did show longer progression-free survival. We report on two cases of primary renal synovial sarcoma and one case of PNET of the kidney. PMID:25918607

  4. TERT promoter hotspot mutations are recurrent in myxoid liposarcomas but rare in other soft tissue sarcoma entities

    PubMed Central

    2014-01-01

    Background Recently, recurrent point mutations in the telomerase reverse transcriptase (TERT) promoter region have been found in many human cancers, leading to a new transcription factor binding site, increased induction of TERT and subsequently to telomere maintenance. We determined the prevalence of TERT promoter mutations in soft tissue sarcomas of 341 patients comprising 16 entities and in 16 sarcoma cell lines covering 7 different soft tissue sarcoma types. Methods The sarcoma tissue samples were collected from the archives of the Institute of Pathology, University of Heidelberg and were composed of 39 myxoid liposarcomas (MLS), 61 dedifferentiated liposarcomas, 15 pleomorphic liposarcomas, 27 leiomyosarcomas, 25 synovial sarcomas (SS), 35 malignant peripheral nerve sheath tumors (MPNST), 40 undifferentiated pleomorphic sarcomas, 17 myxofibrosarcomas, 9 low grade fibromyxoid sarcomas, 10 cases of dermatofibrosarcoma protuberans, 31 solitary fibrous tumors (SFT), 8 extraskeletal myxoid chondrosarcomas, 9 angiosarcomas, 6 alveolar soft part sarcomas, 5 clear cell sarcomas and 4 epithelioid sarcomas. Sarcoma cell lines were obtained from the raising laboratories. A 193 bp fragment of the TERT promoter region covering the hot-spot mutations C228T and C250T was amplified, and direct sequencing of the PCR products was performed. Results TERT promoter mutations were detected in 36/341 sarcomas. They were highly recurrent in MLS (29/39; 74%) and were in the present MLS series not associated with the phenotype (myxoid vs. round cell variant), tumor grade, tumor site and patients’ median age or gender. In the remaining cases, TERT promoter mutations were found only in 7/302 sarcoma samples and confined to SFTs (4/31; 13%), MPNSTs (2/35; 6%), and SSs (1/25; 4%). Within the collection of sarcoma cell lines examined, TERT promoter mutations were detected in two MLS and in one of three MPNST cell lines. Conclusions TERT promoter mutations are frequent in MLSs including

  5. Radiation-induced sarcoma of the thyroid

    SciTech Connect

    Griem, K.L.; Robb, P.K.; Caldarelli, D.D.; Templeton, A.C. )

    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.

  6. Retroperitoneal Approach for Dissection of Inflamed Pelvic Viscera in Acute Pelvic Inflammatory Disease- Case Report

    PubMed Central

    Ganesh, Deepa; Srinivasan, Jayashree; S, Jayakumar; Mathew, Renu

    2014-01-01

    Tubo-ovarian abscesses can rupture spontaneously after a manual examination or an accidental trauma. A critically ill patient with septic peritonitis will only deteriorate if timely surgical removal of pus is not done. The operation of choice is removal of free pus, together with the abscess, the uterus, the tubes and usually, the ovaries. Anatomy is distorted, dependable landmarks are obscured and tissues are thick and oedematous. Loops of densely adhered intestine are difficult to separate. If an intra peritoneal approach is used, it is likely that the fragments of ovary will be left behind. This can subsequently cause signs and symptoms of ovarian remnant syndrome. Injury to the serosa of distended bowel occurs inadvertently, thus increasing the morbidity which results from the procedure. We are hereby presenting a unique case of a ruptured tubo-ovarian abscess where a retroperitoneal approach was used. PMID:24995219

  7. Pneumomediastinum and retroperitoneal air after removal of papillomas with the microdebrider and jet ventilation.

    PubMed Central

    Sims, H. Steven; Lertsburapa, Keith

    2007-01-01

    OBJECTIVE: To discuss the complication of pneumothorax from alveolar rupture after transtracheal high-frequency jet ventilation and to present a case of pneumothorax, pneumomediastinum and pneumoperitoneum after jet ventilation coupled with use of the microdebrider. METHOD: Detailed case report. RESULTS: Unilateral pnuemothorax, subcutaneous emphysema, pneumomediastinum and retroperitoneal air discovered after jet ventilation for removal of airway papillomas resolved with conservative management. DISCUSSION: We discuss the difference between the respective patterns of air seepage in a peripheral alveolar injury versus a probable microperforation in the trachea. We also review the epidemiology of this rare disorder and its incidence in the African-American community. CONCLUSION: The recurrent nature of this disorder mandates multiple surgical procedures. Great care must be taken to eradicate disease and avoid complications. Pneumomediastinum in this setting can be managed conservatively. Images Figure 1 Figure 2 PMID:17913120

  8. Retroperitoneal Fasciitis: Spectrum of CT Findings in the Abdomen and Pelvis.

    PubMed

    Chingkoe, Christina M; Jahed, Ali; Loreto, Michael P; Sarrazin, Josée; McGregor, Caitlin T; Blaichman, Jason I; Glanc, Phyllis

    2015-01-01

    Retroperitoneal fasciitis is a rare but potentially lethal complication of infection. Early diagnosis is crucial and is usually made when there is a high degree of clinical suspicion combined with characteristic imaging findings leading to early surgical intervention. Computed tomography (CT) can play a central role in demonstrating early findings, assessing the extent of disease to help determine the best surgical approach, identifying the primary source of infection, and evaluating the treatment response. The possible presence of retroperitoneal fasciitis should be considered in patients presenting with symptoms of sepsis, including pain that is disproportionate with the clinical abnormality. When retroperitoneal fasciitis is suspected, emergency CT can facilitate early diagnosis and evaluation of the extent of disease. Common findings at CT include fascial thickening and enhancement, muscular edema, fat stranding, fluid collections, and abscess formation. Gas tracking along fascial planes in the retroperitoneum is the hallmark of retroperitoneal fasciitis but is not seen in all cases. Another important clue to the diagnosis is asymmetric involvement of the retroperitoneal fascial planes and deep tissues. Fasciitis in the retroperitoneum may originate from infected retroperitoneal organs or from infection that spreads along indirect and/or direct pathways from a primary source elsewhere in the body. Findings of indirect tracking and transgression of fascial planes may indicate more severe infection associated with the necrotizing form of retroperitoneal fasciitis. Despite aggressive antibiotic treatment, early and repeated surgical débridement may be required to remove nonviable tissue in patients with the necrotizing form of retroperitoneal fasciitis. Awareness of the anatomy of the retroperitoneum, potential routes of spread of infection, and the spectrum of CT findings in retroperitoneal fasciitis is needed to achieve prompt diagnosis and guide treatment.

  9. Retroperitoneal Laparoscopy in Dogs: Access Technique, Working Space, and Surgical Anatomy.

    PubMed

    Jeong, Junemoe; Ko, Jonghyeok; Lim, Hyunjoo; Kweon, Oh-Kyeong; Kim, Wan Hee

    2016-11-01

    To develop and describe a laparoscopic retroperitoneal access technique, investigate working space establishment, and describe the surgical anatomy in the retroperitoneal space as an initial step for clinical application of retroperitoneal laparoscopy in dogs. Cadaveric and experimental study. Cadaveric (n=8) and healthy (n=6) adult dogs. The retroperitoneal access technique was developed in 3 cadavers based on the human technique and transperitoneal observation. Its application and working space establishment with carbon dioxide (CO2 ) insufflation alone was evaluated in 5 cadavers by observing with a transperitoneal telescope and in 6 live dogs by repeated computed tomography (CT) scans at pressure of 0, 5, 10, and 15 mmHg. Recordings of retroperitoneoscopy as well as working space volume and linear dimensions measured on CT images were analyzed. Retroperitoneal access and working space establishment with CO2 insufflation alone were successfully performed in all 6 live dogs. The only complication observed was in 1 dog that developed subclinical pneumomediastinum. As pressure increased, working space was established from the ipsilateral to the contralateral side, and peritoneal tearing eventually developed. Working space volume increased significantly from 5 mmHg and linear dimensions increased significantly from 0 to 10 mmHg. With pneumo-retroperitoneum above 5 mmHg, retroperitoneal organs, including kidneys and adrenal glands, were easily visualized. The retroperitoneal access technique and working space establishment with CO2 insufflation starting with 5 mmHg and increasing to 10 mmHg provided adequate working space and visualization of retroperitoneal organs, which may allow direct access for retroperitoneal laparoscopy in dogs. © 2016 The Authors. Veterinary Surgery published by Wiley Periodicals, Inc., on behalf of The American College of Veterinary Surgeons.

  10. The roles and therapeutic potential of cyclin-dependent kinases (CDKs) in sarcoma.

    PubMed

    Liao, Yunfei; Feng, Yong; Shen, Jacson; Hornicek, Francis J; Duan, Zhenfeng

    2016-06-01

    Uncontrolled proliferation and cell growth is the hallmark of many different malignant diseases, including sarcomas. Cyclin-dependent kinases (CDKs) are members of the serine/threonine protein kinase family and play crucial roles in tumor cell proliferation and growth by controlling cell cycle, transcription, and RNA splicing. In addition, several CDKs influence multiple targets and phosphorylate transcription factors involved in tumorigenesis. There are many examples linking dysregulated activation and expression of CDKs to tumors, and targeting CDKs in tumor cells has become a promising therapeutic strategy. More recently, the Food and Drug Administration (FDA) has approved the CDK4/6 inhibitor palbociclib for treating metastatic breast cancer. In sarcomas, high levels of CDK mRNA and protein expression have been found in most human sarcoma cells and patient tissues. Many studies have demonstrated consistent results in which inhibition of different CDKs decrease sarcoma cell growth and induce apoptosis. Therefore, CDKs comprise an attractive set of targets for novel anti-sarcoma drug development. In this review, we discuss the roles of different members of CDKs in various sarcomas and provide a pre-clinical overview of promising therapeutic potentials of targeting CDKs with a special emphasis on sarcoma.

  11. Uterine sarcoma part III-Targeted therapy: The Taiwan Association of Gynecology (TAG) systematic review.

    PubMed

    Yen, Ming-Shyen; Chen, Jen-Ruei; Wang, Peng-Hui; Wen, Kuo-Chang; Chen, Yi-Jen; Ng, Heung-Tat

    2016-10-01

    Uterine sarcoma is a very aggressive and highly lethal disease. Even after a comprehensive staging surgery or en block cytoreduction surgery followed by multimodality therapy (often chemotherapy and/or radiation therapy), many patients relapse or present with distant metastases, and finally die of diseases. The worst outcome of uterine sarcomas is partly because of their rarity, unknown etiology, and highly divergent genetic aberration. Uterine sarcomas are often classified into four distinct subtypes, including uterine leiomyosarcoma, low-grade uterine endometrial stromal sarcoma, high-grade uterine endometrial stromal sarcoma, and undifferentiated uterine sarcoma. Currently, evidence from tumor biology found that these tumors showed alternation and/or mutation of genomes and the intracellular signal pathway. In addition, some preclinical studies showed promising results for targeting receptor tyrosine kinase signaling, phosphatidylinositol 3-kinase/AKT/mammalian target of rapamycin pathway, various kinds of growth factor pathways, Wnt/beta-catenin signaling pathway, transforming growth factor β/bone morphogenetic protein signal pathway, aurora kinase A, MDM2 proto-oncogene, histone deacetylases, sex hormone receptors, certain types of oncoproteins, and/or loss of tumor suppressor genes. The current review is attempted to summarize the recurrent advance of targeted therapy for uterine sarcomas. Copyright © 2016. Published by Elsevier B.V.

  12. Germline Mutations in Cancer Predisposition Genes are Frequent in Sporadic Sarcomas.

    PubMed

    Chan, Sock Hoai; Lim, Weng Khong; Ishak, Nur Diana Binte; Li, Shao-Tzu; Goh, Wei Lin; Tan, Gek San; Lim, Kiat Hon; Teo, Melissa; Young, Cedric Ng Chuan; Malik, Simeen; Tan, Mann Hong; Teh, Jonathan Yi Hui; Chin, Francis Kuok Choon; Kesavan, Sittampalam; Selvarajan, Sathiyamoorthy; Tan, Patrick; Teh, Bin Tean; Soo, Khee Chee; Farid, Mohamad; Quek, Richard; Ngeow, Joanne

    2017-09-06

    Associations of sarcoma with inherited cancer syndromes implicate genetic predisposition in sarcoma development. However, due to the apparently sporadic nature of sarcomas, little attention has been paid to the role genetic susceptibility in sporadic sarcoma. To address this, we performed targeted-genomic sequencing to investigate the prevalence of germline mutations in known cancer-associated genes within an Asian cohort of sporadic sarcoma patients younger than 50 years old. We observed 13.6% (n = 9) amongst 66 patients harbour at least one predicted pathogenic germline mutation in 10 cancer-associated genes including ATM, BRCA2, ERCC4, FANCC, FANCE, FANCI, MSH6, POLE, SDHA and TP53. The most frequently affected genes are involved in the DNA damage repair pathway, with a germline mutation prevalence of 10.6%. Our findings suggests that genetic predisposition plays a larger role than expected in our Asian cohort of sporadic sarcoma, therefore clinicians should be aware of the possibility that young sarcoma patients may be carriers of inherited mutations in cancer genes and should be considered for genetic testing, regardless of family history. The prevalence of germline mutations in DNA damage repair genes imply that therapeutic strategies exploiting the vulnerabilities resulting from impaired DNA repair may be promising areas for translational research.

  13. Malignant sarcoma of the pelvic bones: treatment outcomes and prognostic factors vary by histopathology.

    PubMed

    Jawad, Muhammad Umar; Haleem, Abdul Ahad; Scully, Sean P

    2011-04-01

    Treatment of malignant sarcomas of the pelvis poses a challenge for local disease control and oncologic outcome. Many reports have described the dismal outcomes. Most studies are retrospective series coming out of single centers, thus biased toward patient selection and are of limited statistical power. The authors used the Surveillance, Epidemiology, and End Results database to analyze 1185 pelvic sarcoma cases from 1987 to 2006. Kaplan-Meier and Cox regression were used to analyze the significance of prognostic factors. The analysis was repeated for different histopathological subtypes to determine specific prognostic factors in each case. Incidence of pelvic sarcoma in 2006 was 89 per 100,000 persons; it has significantly increased since 1973 (P < .05). The overall 5-year survival for all the patients with pelvic sarcoma was 47%, with osteosarcoma having the worst 5-year survival at 19% and patients with chordoma having the best 5-year survival at 60%. Independent prognostic factors included age, stage, grade, size of primary lesion, histopathology, and treatment-related factors. Comparing the patients only with high-grade lesions, patients with Ewing sarcoma have the best prognosis. This is an analysis of patients with pelvic sarcomas derived from a population-based registry. Survival and prognostics vary with histopathological diagnoses. Although surgical resection was associated with superior outcomes for osteosarcoma and chondrosarcoma, there was no significant difference in outcomes of patients with Ewing sarcoma treated with surgery and/or radiotherapy. Copyright © 2010 American Cancer Society.

  14. 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.

  15. Kaposi sarcoma: review and medical management update.

    PubMed

    Fatahzadeh, Mahnaz

    2012-01-01

    Despite recent advances in our understanding of pathogenic mechanisms involved, the true nature of Kaposi sarcoma remains an enigma. Four clinical variants have been described for the disease, differing in natural history, site of predilection, and prognosis. All forms of Kaposi sarcoma may manifest in the oral cavity and Kaposi sarcoma-associated virus appears essential to development of all clinical variants. The spectrum of therapeutic strategies is broad and selection of appropriate intervention mandates a thorough understanding of disease spread and the patient's symptomatology, as well as risks and benefits of therapy. This article provides an overview of epidemiology, subtypes, clinical course, pathogenesis, and management strategies for Kaposi sarcoma.

  16. Intimal sarcoma of the pulmonary valve.

    PubMed

    Scheidl, Stefan; Taghavi, Shahrokh; Reiter, Ursula; Tröster, Natascha; Kovacs, Gabor; Rienmüller, Rainer; Lang, Susanna; Klepetko, Walter; Olschewski, Horst

    2010-04-01

    Pulmonary artery intimal sarcoma is a rare tumor of the cardiovascular system. Intimal sarcoma of the pulmonary valve itself has not been described. Embolization into pulmonary arteries originating from the pulmonary valve intimal sarcoma can mimic chronic thromboembolic pulmonary hypertension and mislead the diagnosis. We present and discuss a patient initially diagnosed as chronic thromboembolic pulmonary hypertension, treated by pulmonary endarterectomy. After 24 months, a tumor of the pulmonary valve was detected by echocardiography. The patient underwent removal and replacement of the pulmonary valve. Histology revealed pulmonary valve intimal sarcoma.

  17. Retroperitoneal fibrosis-clinical presentation and outcome analysis from urological perspective

    PubMed Central

    Kumar, Vikash; Punatar, Chirag B.; Joshi, Vinod S.; Sagade, Sharad N.

    2017-01-01

    Purpose To study clinical presentation, laboratory results, imaging findings and treatment options and outcomes of retroperitoneal fibrosis (RPF). To determine whether it follows the same natural course and response to treatment in the Asian population as in the Western world. Materials and Methods Medical records of patients diagnosed with RPF on imaging and histopathology between February 2010 and April 2016 were reviewed. Results Of the 21 patients analyzed, mean age at presentation was 50.81 years. The male to female ratio was 0.9:1. Pain was most common presenting complaint (95.23% cases), almost 85% cases were idiopathic and rests were postradiation induced. The median creatinine level was 1.8 mg/dL. The mean erythrocyte sedimentation rate (ESR) was 53.2 mm/h. Hydronephrosis was present in all patients and 47.6% had atrophic kidneys. Diffuse retroperitoneal mass was present in 61.1%. Ureterolysis with lateralization, omental wrapping or gonadal pedicle wrap was done in 17 cases. Two patients underwent uretero-ureterostomy. One patient underwent ileal replacement of ureter, and one ileal conduit. Eighteen patients received concurrent medical treatment, 11 were given tamoxifen, 2 steroids (Prednisolone), and five were given both. Of the 20 patients with follow-up, 70% had complete symptomatic relief; ESR improvement was seen in 77.8%. Follow-up ultrasound showed resolved and decreased hydronephrosis in 20% and 55% respectively. One patient had treatment failure and 17.65% had disease recurrence. Conclusions RPF is a rare disease with varied presentation and outcomes. The male to female ratio may be equal in Asians and smoking could be lesser contributing factor. More Asian cohort studies are required to support same. PMID:28868510

  18. 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.

  19. Molecular genetics of sarcomas: applications to diagnoses and therapy.

    PubMed

    Toguchida, Junya; Nakayama, Tomitaka

    2009-09-01

    Sarcomas are mesenchymal cancers consisting of tumors with various clinical and pathological features. Some of them compel affected individuals to lose important musculoskeletal functions, and some of them are highly malignant and life-threatening. A great amount of genetic information for sarcomas has accumulated during the past two decades, contributing diagnoses and treatments. From the standpoint of molecular genetics, sarcomas are classified into two groups: those with defined genetic alterations and those with various genetic alterations. The genetic alterations in the first group include reciprocal translocations resulting in fusion oncoproteins and oncogenic mutations of defined genes such as those of the c-kit gene in gastrointestinal stromal tumors. The function of fusion proteins includes transcription regulator, signal transducer, chromatic remodeling factor, and growth factor, some of which are suitable targets for the molecular therapy. In tumors belonging to the second group, the number of which is far larger than those of the first group, considerable genetic heterogeneity was found even among tumors with same pathological diagnosis. The disruption of the RB and p53 pathways was frequently found, resulting in the dysregulation of cell cycle and the genomic instability. The application of molecular target therapy for tumors in this group requires novel strategies to overcome cross talk between different signal pathways. Recent evidence from in vitro and in vivo experiments has indicated that the cells of origin of sarcomas are tissue stem cells such as mesenchymal stem cells, and the application of stem cell biology holds the promise of novel treatment options.

  20. Myeloid sarcoma causing airway obstruction

    PubMed Central

    Krause, John R.

    2017-01-01

    Myeloid sarcoma is an extramedullary collection of blasts of the myeloid series that partially or totally effaces the architecture of the tissue in which it is found. These tumors have been described in many sites of the body, but the skin, lymph nodes, gastrointestinal tract, bone, soft tissue, and testes are most common. They can arise in a patient following the diagnosis of acute myeloid leukemia, but they may also be precursors of leukemia and should be considered diagnostic for acute myeloid leukemia. The differential diagnosis of this neoplasm includes malignant lymphoma, with which it is often mistaken, leading to diagnostic and therapeutic delays. We present the case of an 84-year-old African American man with a history of renal disease secondary to hypertension and coronary artery disease without any prior history of malignancies who presented with airway obstruction. He was diagnosed with a myeloid sarcoma of the mediastinum compressing his trachea. PMID:28405080

  1. Angiogenesis and Vascular Targeting in Ewing Sarcoma: A Review of Preclinical and Clinical Data

    PubMed Central

    DuBois, Steven G.; Marina, Neyssa; Glade-Bender, Julia

    2009-01-01

    Ewing sarcoma is the second most common type of bone cancer in children and young adults. In recent years, the mechanisms by which these tumors develop and maintain their vascular supply have been elucidated. Additional work has demonstrated that inhibition of angiogenic pathways or disruption of established vasculature can attenuate the growth of Ewing sarcoma mouse xenografts. Early clinical data suggest that these results may also extend to patients with Ewing sarcoma treated with anti-angiogenic or anti-vascular therapies. This review summarizes the available data supporting this approach. PMID:20029966

  2. Kaposi’s Sarcoma of the Rectum in a Homosexual Male with HIV-AIDS

    PubMed Central

    Nautsch, Deborah

    2016-01-01

    Visceral involvement in AIDS-related Kaposi’s sarcoma is common, but it is rarely seen in the absence of cutaneous disease. Most patients with gastrointestinal tract Kaposi’s sarcoma remain asymptomatic and are often diagnosed on endoscopy or autopsy. We report a case of a 24-year-old homosexual man who presented with rectal pain and bleeding and was found to have skin-sparing, disseminated Kaposi’s sarcoma with rectum, liver, lungs, and lymph node involvement. Despite treatment with highly active anti-retroviral therapy, he developed multiorgan failure resulting in death. PMID:28119943

  3. 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.

  4. 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

  5. [Primary sarcoma of the mammary gland].

    PubMed

    Coronel-Brizio, Pedro Guillermo; Quistian Navarrete, Fernando; Guzmán Garcia, Raúl E; Zoloeta Dominguez, Pedro A; Venegas Espinosa, Berenice

    2012-12-01

    Breast cancer occupies the chief place in incidence after the cervix. This gland sarcomas are rare with less than 1% at this location. Its diagnosis is difficult and its evolution is aggressive. Primary breast osteogenic sarcomas are a subset of lower frequency so that their behavior, evolution, prognosis ytratamiento no much experience and often are diagnosed as benign tumors. We report the case of a woman of 59 years which initially arose from a tumor in the right breast was performed with tru-cut biopsy with inconclusive results, using the tumor 7 months after the presence of a multilobulated solid tumor 20 cm, tumor compatible with mammography Phylodes (BIRADS 2). It was decided to perform right total mastectomy and pectoral muscle resection reconstruction with latissimus dorsi more and histochemical review pathology diagnosed with malignant mesenchymal neoplasm, osteosarcoma osteoblast osteoclast type and complemented the treatment with radiotherapy and chemotherapy. The prognosis is poor because the five-year survival is less than 40%. In the fast-growing breast tumors and large mammographic calcifications with signs and antecedentesde irradiation, trauma and Phyllodes tumor must be aware of this disease and that early diagnosis improves survival. In the surgical treatment of axillary dissection is not indicated and adjuvant treatment with radiotherapy and chemotherapy.

  6. [A case of retroperitoneal teratoma in an adult resembling adrenal tumors].

    PubMed

    Megumi, Y; Ohmori, K; Nishimura, K

    1999-01-01

    A retroperitoneal teratoma arising from the vicinity of the upper pole of the right kidney in a 61-year-old woman is described. Computed tomographic (CT) scan showed well marginated fat density mass with calcification. Magnetic resonance imaging (MRI) on T1 weighted image and T2 weighted image showed a high-intensity mass above the upper pole of the right kidney. Needle biopsy under computed tomography was performed, but no specimen was obtained. The preoperative diagnosis was retroperitoneal teratoma or right adrenal myelolipoma. The tumor was resected and the histological diagnosis was benign retroperitoneal cystic teratoma.

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

    PubMed Central

    Minocha, Priyanka; Setia, Ankur

    2016-01-01

    Summary 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. PMID:27904827

  8. Retroperitoneal tumor: giant cavernous hemangioma – case presentation and literature review

    PubMed Central

    Haponiuk, Ireneusz; Jaworski, Radoslaw; Peksa, Rafal; Irga-Jaworska, Ninela; Jaskiewicz, Janusz

    2016-01-01

    Retroperitoneal hemangiomas are very rare. This paper presents the case of a 71-year-old female patient with giant cavernous hemangioma of the retroperitoneum who underwent surgical treatment for abdominal pain and left lower limb edema. Interventional staged treatment with percutaneous transcatheter arterial embolization prior to surgery was considered. Radical resection of the tumor was performed, which caused the symptoms to abate. Additionally a literature review of cases involving cavernous hemangioma in the retroperitoneal space is presented. No description of retroperitoneal cavernous hemangioma originating from the bowel was found in the analyzed reports. PMID:28096841

  9. [Primary sarcomas of the breast].

    PubMed

    Chirife, Ana M; Bello, Lorena; Celeste, Francisco; Giménez, Liliana; Gorostidy, Susana

    2006-01-01

    Primary sarcomas of the breast are extremely rare with less than 1% of all malignant tumours of the breast reported in literature. At our Institution 1315 malignant tumours of the breast were diagnosed between 1999-2004; nine of them corresponded to primary sarcomas: angiosarcoma (3), leiomyosarcoma (1), low-grade fibromyxoid sarcoma (1), dematofibrosarcoma protuberans (1), liposarcoma (1), osteosarcoma (1), malignant peripheral nerve sheath tumour (1). Histopathological specimens stained with routine techniques and immunoperoxidase were reviewed; proliferation index and p53 over-expression were also determined. Patients' clinical reports were also reviewed to determine prognosis (favorable and unfavorable). The incidence observed (0.7%) is similar to those already published by others authors. Proliferation index was correlated with type of evolution, being an unfavourable prognosis factor when it was equal or major to 30%. Most of the tumours (67%) showed p53 (mayor or equal to 20% of nuclear staining) over-expression but this did not show a direct relationship with the evolution of each neoplasm.

  10. Pathology of soft tissue sarcomas.

    PubMed

    Thway, K

    2009-11-01

    Sarcomas are a rare, complex group of childhood and adult neoplasms with differentiation towards mesenchymal tissue, which may arise almost anywhere in the body. Although pathologically diverse, they frequently exhibit similar clinical presentations and radiological features. Correct histopathological diagnosis is therefore crucial, but there is overlap between histological patterns of malignant tumours, between benign and malignant lesions, and with non-mesenchymal tumours. Immunohistochemistry and molecular genetic techniques, the latter to detect tumour-specific alterations, add significantly to histological interpretation, but several groups of tumours still lack reliable immunohistochemical markers or reproducible genetic changes. The classification of sarcomas is incomplete and continues to evolve, and although the biology of many remains relatively poorly understood, our increasing insight into molecular events occurring in these tumours is certain to aid future diagnosis and therapy. This paper aims to give a broad overview of several of the main soft tissue sarcomas from a clinicopathological perspective, discussing laboratory diagnosis and the use and limitations of ancillary investigations, including recent developments in molecular diagnosis.

  11. NF1 deletion generates multiple subtypes of soft-tissue sarcoma that respond to MEK inhibition.

    PubMed

    Dodd, Rebecca D; Mito, Jeffrey K; Eward, William C; Chitalia, Rhea; Sachdeva, Mohit; Ma, Yan; Barretina, Jordi; Dodd, Leslie; Kirsch, David G

    2013-09-01

    Soft-tissue sarcomas are a heterogeneous group of tumors arising from connective tissue. Recently, mutations in the neurofibromin 1 (NF1) tumor suppressor gene were identified in multiple subtypes of human soft-tissue sarcomas. To study the effect of NF1 inactivation in the initiation and progression of distinct sarcoma subtypes, we have developed a novel mouse model of temporally and spatially restricted NF1-deleted sarcoma. To generate primary sarcomas, we inject adenovirus containing Cre recombinase into NF1(flox/flox); Ink4a/Arf(flox/flox) mice at two distinct orthotopic sites: intramuscularly or in the sciatic nerve. The mice develop either high-grade myogenic sarcomas or malignant peripheral nerve sheath tumor (MPNST)-like tumors, respectively. These tumors reflect the histologic properties and spectrum of sarcomas found in patients. To explore the use of this model for preclinical studies, we conducted a study of mitogen-activated protein kinase (MAPK) pathway inhibition with the MEK inhibitor PD325901. Treatment with PD325901 delays tumor growth through decreased cyclin D1 mRNA and cell proliferation. We also examined the effects of MEK inhibition on the native tumor stroma and find that PD325901 decreases VEGFα expression in tumor cells with a corresponding decrease in microvessel density. Taken together, our results use a primary tumor model to show that sarcomas can be generated by loss of NF1 and Ink4a/Arf, and that these tumors are sensitive to MEK inhibition by direct effects on tumor cells and the surrounding microenvironment. These studies suggest that MEK inhibitors should be further explored as potential sarcoma therapies in patients with tumors containing NF1 deletion.

  12. NF1 deletion generates multiple subtypes of soft-tissue sarcoma that respond to MEK inhibition

    PubMed Central

    Dodd, Rebecca D.; Mito, Jeffrey K.; Eward, William C.; Chitalia, Rhea; Sachdeva, Mohit; Ma, Yan; Barretina, Jordi; Dodd, Leslie; Kirsch, David G.

    2013-01-01

    Soft-tissue sarcomas are a heterogeneous group of tumors arising from connective tissue. Recently, mutations in the neurofibromin 1 (NF1) tumor suppressor gene were identified in multiple subtypes of human soft-tissue sarcomas. To study the effect of NF1 inactivation in the initiation and progression of distinct sarcoma subtypes, we have developed a novel mouse model of temporally and spatially restricted NF1-deleted sarcoma. To generate primary sarcomas, we inject adenovirus containing Cre recombinase into NF1flox/flox; Ink4a/Arf flox/flox mice at two distinct orthotopic sites: intramuscularly or in the sciatic nerve. The mice develop either high-grade myogenic sarcomas or MPNST-like tumors, respectively. These tumors reflect the histological properties and spectrum of sarcomas found in patients. To explore the utility of this model for preclinical studies, we performed a study of MAPK pathway inhibition with the MEK inhibitor PD325901. Treatment with PD325901 delays tumor growth through decreased cyclin D1 mRNA and cell proliferation. We also examined the effects of MEK inhibition on the native tumor stroma and find that PD325901 decreases VEGFα expression in tumor cells with a corresponding decrease in microvessel density. Taken together, our results utilize a primary tumor model to demonstrate that sarcomas can be generated by loss of NF1 and Ink4a/Arf, and that these tumors are sensitive to MEK inhibition by direct effects on tumor cells and the surrounding microenvironment. These studies suggest that MEK inhibitors should be further explored as potential sarcoma therapies in patients with tumors containing NF1 deletion. PMID:23858101

  13. Impact of hybrid molecular imaging in retroperitoneal fibrosis: a systematic review.

    PubMed

    Grozdic Milojevic, Isidora T; Milojevic, Bogomir; Sobic-Saranovic, Dragana P; Artiko, Vera M

    2017-08-24

    The aim of this article was to critically assess the usefulness of hybrid molecular imaging (FDG PET/CT and FDG PET/MR) procedures in the evaluation of inflammatory activity in retroperitoneal fibrosis (RPF). A systematic review of the literature was performed using PubMed without timeline restriction and using the following keywords: retroperitoneal fibrosis, disease activity, diagnostic techniques, PET/CT, PET/MR. We evaluated full text articles written in the English language. Case reports, review articles or editorials and articles not in the field of interest of this review were excluded. Nine articles comprising a total of 186 patients met the inclusion criteria and were included and described in this systematic review. The new hybrid molecular imaging methods give promising results in the evaluation of the activity of the disease, quantification and prediction of therapeutic response and in tailoring medical therapy in RPF. FDG PET/CT can be a valuable tool in detecting disease activity, particularly in asymptomatic patients with RPF with acute phase reactant increase. Hybrid imaging can predict therapy response outcome and the best time for stent removal. Although PET/MR has potential advantage in small lesions and has reduced radiation exposure in comparison to PET/CT, PET quantification parameters have potentially higher diagnostic value over MR parameters in the evaluation of RPF. Acute phase reactants alone may not be reliable for the management and follow-up assessment of the disease. Hybrid imaging in RFP could be more comfortable, more accurate, with less radiation burden than different separate imaging studies acquired at different points in time.

  14. Clinical significance of systematic retroperitoneal lymphadenectomy during interval debulking surgery in advanced ovarian cancer patients

    PubMed Central

    Takada, Toshio; Iitsuka, Chiaki; Nomura, Hidetaka; Abe, Akiko; Taniguchi, Tomoko; Takizawa, Ken

    2015-01-01

    Objective To investigate the clinical significance of systematic retroperitoneal lymphadenectomy during interval debulking surgery (IDS) in advanced epithelial ovarian cancer (EOC) patients. Methods We retrospectively reviewed the medical records of 124 advanced EOC patients and analyzed the details of neoadjuvant chemotherapy (NACT), IDS, postoperative treatment, and prognoses. Results Following IDS, 98 patients had no gross residual disease (NGRD), 15 had residual disease sized <1 cm (optimal), and 11 had residual disease sized ≥1 cm (suboptimal). Two-year overall survival (OS) and progression-free survival (PFS) rates were 88.8% and 39.8% in the NGRD group, 40.0% and 13.3% in the optimal group (p<0.001 vs. NGRD for both), and 36.3% and 0% in the suboptimal group, respectively. Five-year OS and 2-year PFS rates were 62% and 56.1% in the lymph node-negative (LN-) group and 26.2% and 24.5% in the lymph node-positive (LN+) group (p=0.0033 and p=0.0024 vs. LN-, respectively). Furthermore, survival in the LN+ group, despite surgical removal of positive nodes, was the same as that in the unknown LN status group, in which lymphadenectomy was not performed (p=0.616 and p=0.895, respectively). Multivariate analysis identified gross residual tumor during IDS (hazard ratio, 3.68; 95% confidence interval, 1.31 to 10.33 vs. NGRD) as the only independent predictor of poor OS. Conclusion NGRD after IDS improved prognosis in advanced EOC patients treated with NACT-IDS. However, while systematic retroperitoneal lymphadenectomy during IDS may predict outcome, it does not confer therapeutic benefits. PMID:26197771

  15. CT-Guided Radioactive {sup 125}I Seed Implantation Therapy of Symptomatic Retroperitoneal Lymph Node Metastases

    SciTech Connect

    Wang, Zhongmin; Lu, Jian; Gong, Ju; Zhang, Liyun; Xu, Yingjia; Song, Shaoli; Chen, Kemin; Liu, Fenju; Gang, Huang

    2013-04-12

    PurposeThis study explored the clinical efficacy of CT-guided radioactive {sup 125}I seed implantation in treating patients with symptomatic retroperitoneal lymph node metastases.MethodsTwenty-five patients with pathologically confirmed malignant tumors received CT-guided radioactive {sup 125}I seed implantation to treat metastatic lymph nodes. The diameter of the metastatic lymph nodes ranged from 1.5 to 4.5 cm. Treatment planning system (TPS) was used to reconstruct the three-dimensional image of the tumor and then calculate the corresponding quantity and distribution of {sup 125}I seeds.ResultsFollow-up period for this group of patients was 2–30 months, and median time was 16 months. Symptoms of refractory pain were significantly resolved postimplantation (P < 0.05), and Karnofsky score rose dramatically (P < 0.05). Most patients reported pain relief 2–5 days after treatment. Follow-up imaging studies were performed 2 months later, which revealed CR in 7 patients, PR in 13 patients, SD in 3 patients, and PD in 2 patients. The overall effective rate (CR + PR) was 80 %. Median survival time was 25.5 months. Seven patients died of recurrent tumor; 16 patients died of multiorgan failure or other metastases. Two patients survived after 30 months follow-up. Two patients reported localized skin erythema 1 week postimplantation, which disappeared after topical treatment.ConclusionsCT-guided radioactive {sup 125}I seed implantation, which showed good palliative pain relief with acceptable short-term effects, has proved in our study to be a new, safe, effective, and relatively uncomplicated treatment option for symptomatic retroperitoneal metastatic lymph nodes.

  16. Neoplastic fever in patients with bone and soft tissue sarcoma

    PubMed Central

    Nakamura, Tomoki; Matsumine, Akihiko; Matsubara, Takao; Asanuma, Kunihiro; Sudo, Akihiro

    2016-01-01

    The development of fever is a common complication in the clinical course of cancer. If all other potential causes of fever are excluded, the possibility of neoplastic fever should be considered. The aim of the present study was to determine the incidence of neoplastic fever in patients with bone and soft tissue sarcomas. Between January 2009 and December 2014, 195 patients with bone and soft tissue sarcoma (111 men and 84 women; mean age, 55 years) were admitted to the Department of Orthopaedic Surgery of Mie University Graduate School of Medicine (Tsu, Japan). Episodes of fever were observed in 58 patients (30%), of whom 11 (5.5%) had neoplastic fever (mean maximum temperature, 38.9°C). The causes of neoplastic fever were as follows: Primary tumor (n=3), local recurrence (n=1), metastasis (n=5), and local recurrence with metastasis (n=2). Of the 11 patients, 9 were treated with naproxen and 8 exhibited a complete response, with their temperature normalizing to <37.3°C within 24 h. The 2 patients who were not treated with naproxen underwent surgical tumor resection, which resulted in prompt and complete lysis of the fever. In conclusion, neoplastic fever occurred in 5.5% of the 195 patients with bone and soft tissue sarcomas investigated herein. Naproxen may be effective for treating neoplastic fever in patients with bone and soft tissue sarcoma; however, radical tumor treatment may have to be considered to achieve permanent lysis of the fever. PMID:27900101

  17. Survival and Recurrence Rate after Treatment for Primary Spinal Sarcomas

    PubMed Central

    Cho, Wonik

    2013-01-01

    Objective We have limited understanding on the presentation and survival of primary spinal sarcomas. The survival, recurrence rate, and related prognostic factors were investigated after treatment for primary sarcomas of the spine. Methods Retrospective analysis of medical records and radiological data was done for 29 patients in whom treatment was performed due to primary sarcoma of the spine from 2000 to 2010. As for treatment method, non-radical operation, radiation therapy, and chemotherapy were simultaneously or sequentially combined. Overall survival (OS), progression free survival (PFS), ambulatory function, and pain status were analyzed. In addition, factors affecting survival and recurrence were analyzed : age (≤42 or ≥43), gender, tumor histologic type, lesion location (mobile spine or rigid spine), weakness at diagnosis, pain at diagnosis, ambulation at diagnosis, initial treatment, radiation therapy, kind of irradiation, surgery, chemotherapy and distant metastasis. Results Median OS was 60 months, the recurrence rate was 79.3% and median PFS was 26 months. Patients with distant metastasis showed significantly shorter survival than those without metastasis. No factors were found to be significant relating to recurrence. Prognostic factor associated with walking ability was the presence of weakness at diagnosis. Conclusion Primary spinal sarcomas are difficult to cure and show high recurrence rate. However, the development of new treatment methods is improving survival. PMID:23826479

  18. [Radiotherapy for sarcoma: hadrontherapy, for whom and what for?].

    PubMed

    Pommier, P; Sunyach, M-P; Hu, Yi; Amsalem, E; Claude, L; Moncort-Boulch, D; Toutenu, P; Balosso, J

    2010-06-01

    The radiobiological properties of the hadrons (neutrons, protons, carbon ions) led to their therapeutic use in sarcomas, as a referent therapy or as an alternative to photon therapy. An extensive review of the literature has been conducted to assess the present indications and the perspectives for hadrontherapy. Compared to photons, neutrons are characterized by a higher biological efficiency that is on particular importance for these tumours usually considered as radio-resistant. Neutrons have been considered as a standard therapy for sarcoma' patients, contra-indicated for surgery or with a definitive R2 resection, but their indications and use have been restricted due to the occurrence of late severe toxicities related to their poor ballistic' properties. Thanks to their physical properties (Bragg Peak), protons are characterized by a higher conformity index compared to photons (and neutrons) with optimal organs at risk preservation that permits a dose escalation. Protontherapy is to date the standard of care for base of skull, spinal and paraspinal sarcomas. Carbon ions combined both advantages from protons and neutrons. Literature data permits to consider this radiation modality as a referent therapy for unresectable sarcomas. The ongoing diffusions of protons and carbon ions radiotherapy facilities will permit to offer these therapies to more patients and to conduct studies that are warranted to determine their indications and their results.

  19. Functional Reconstruction of Sarcoma Defects Utilising Innervated Free Flaps

    PubMed Central

    Grinsell, Damien; Di Bella, Claudia; Choong, Peter F. M.

    2012-01-01

    Soft-tissue reconstruction following preoperative radiotherapy and wide resection of soft tissue sarcoma remains a challenge. Pedicled and free tissue transfers are an essential part of limb sparing surgery. We report 22 cases of sarcoma treated with radiotherapy and wide excision followed by one-stage innervated free or pedicled musculocutaneous flap transfers. The resection involved the upper limb in 3 cases, the lower limb in 17, and the abdominal wall in 2. The flaps used for the reconstruction were mainly latissimus dorsi and gracilis. The range of motion was restored fully in 14 patients. The muscle strength of the compartment reconstructed was of grades 4 and 5 in all patients except one. The overall function was excellent in all the cases with functional scores of 71.2% in the upper limb and 84% in the lower limb. The only 2 major complications were flap necrosis, both revised with another flap, one of which was innervated with restoration of function. Innervated flaps are valuable alternatives for reconstruction after sarcoma resection in the extremity and in the abdominal wall. The excellent functional results are encouraging, and we believe that innervated muscle reconstruction should be encouraged in the treatment of sarcoma after radiotherapy and wide resection. PMID:22969309

  20. Needle tract seeding after percutaneous biopsy of sarcoma: Risk/benefit considerations.

    PubMed

    Berger-Richardson, David; Swallow, Carol J

    2017-02-15

    To evaluate histologic subtype and grade, which in turn guide the decision making for multimodality therapy, the workup of suspected sarcoma requires more material than can be obtained from a fine-needle aspiration. Either open or percutaneous core needle biopsy is indicated before a management decision is made. Percutaneous biopsy of a potentially malignant lesion is controversial, given the perceived potential for tumor seeding along the needle tract. However, the evidence that the latter is a significant risk is weak at best. To the authors' knowledge, among cases of patients with extremity sarcoma who have undergone core needle biopsy, only a few cases of needle tract seeding have been reported to date. Although en bloc excision of the needle tract with the primary tumor is often performed, this practice is not associated with improved oncologic outcomes; the evidence for excision of the needle tract is poor. For patients with gastrointestinal stromal tumors, there is a theoretical risk of peritoneal dissemination after percutaneous biopsy, but to the authors' knowledge this remains unproven. Although endoscopic ultrasound is the preferred route for biopsy among patients with gastrointestinal stromal tumors, percutaneous biopsy is indicated if endoscopic ultrasound is unsuitable or unavailable. In the setting of retroperitoneal sarcoma, a review of pooled data from 4 large tertiary care referral centers demonstrated a risk of needle tract seeding of 0.37%. The authors concluded that the benefits of pretreatment biopsy in patients with mesenchymal tumors outweigh the potential risks of needle tract seeding. Cancer 2017;123:560-567. © 2016 American Cancer Society. © 2016 American Cancer Society.

  1. Efficacy of trabectedin in advanced soft tissue sarcoma: beyond lipo- and leiomyosarcoma.

    PubMed

    De Sanctis, Rita; Marrari, Andrea; Marchetti, Silvia; Mussi, Chiara; Balzarini, Luca; Lutman, Fabio Romano; Daolio, Primo; Bastoni, Stefano; Bertuzzi, Alexia Francesca; Quagliuolo, Vittorio; Santoro, Armando

    2015-01-01

    Trabectedin is effective in leiomyosarcoma and liposarcoma, especially the myxoid variant, related to the presence of the FUS-CHOP transcript. We evaluated the efficacy of trabectedin in specific subgroups of patients with soft tissue sarcomas (STS). Seventy-two patients with advanced anthracycline-pretreated STS, who received trabectedin at a dose of 1.5 mg/m(2) every 3 weeks by continuous 24-hour infusion, were retrospectively analyzed. Best response rate according to Response Evaluation Criteria In Solid Tumors (RECIST) criteria and severe adverse events (AEs) according to National Cancer Institute Common Terminology Criteria for Adverse Events (NCI-CTCAE v4.02) were evaluated. Secondary endpoints included progression-free survival and overall survival (OS). Median age was 48 (range, 20-75) years, with a median Eastern Cooperative Oncology Group performance status of 0. The median number of previous chemotherapy regimens was 1 (range, 0-5). Median number of trabectedin cycles was 3 (range, 1-17). About 69/72 patients (95.8%) were evaluable for response: 9 patients (13%) achieved partial response and 26 (37.7%) stable disease. According to histotype, clinical benefit (partial response + stable disease) was reported in synovial sarcoma (n=5), retroperitoneal liposarcoma (n=10), myxoid liposarcoma (n=5), leiomyosarcoma (n=8), high-grade undifferentiated pleomorphic sarcoma (n=5), Ewing/peripheral primitive neuroectodermal tumor (n=1), and malignant peripheral nerve sheath tumor (n=1). Any grade AEs were noncumulative, reversible, and manageable. G3/G4 AEs included anemia (n=1, 1.4%), neutropenia (n=7, 9.6%), liver toxicity (n=6, 8.3%), and fatigue (n=2, 2.8%). With a median follow-up time of 11 (range, 2-23) months, median progression-free survival and OS of the entire cohort were 2.97 months and 16.5 months, respectively. Our experience confirms trabectedin as an effective therapeutic option for metastatic lipo- and leiomyosarcoma and suggests promise in synovial

  2. Does the two-week rule pathway improve the diagnosis of soft tissue sarcoma? A retrospective review of referral patterns and outcomes over five years in a regional sarcoma centre

    PubMed Central

    Pencavel, Tim D; Strauss, Dirk C; Thomas, Greg P; Thomas, J Meirion; Hayes, Andrew J

    2010-01-01

    INTRODUCTION The NHS Cancer Plan was introduced in 2000 and included guidelines for the rapid assessment and referral of cases of suspected malignancy. We wished to assess the efficiency and appropriateness of patients referred under the Department of Health's general practitioner referral guidelines implemented for sarcomas in December 2000. PATIENTS AND METHODS A retrospective case-note review was performed of all patients referred to our regional soft tissue sarcoma unit between 1 January 2004 and 31 December 2008. Patients referred under the two-week guidelines and all patients referred routinely were analysed. The main outcome measures were the total number of patients referred on the basis of the two-week guidelines and the proportion they constitute of all referrals. The referring criteria were noted and compared to the observed criteria recorded. The final histo-logical diagnosis of patients referred on the basis of the two-week guidelines are documented. RESULTS A total of 2746 referrals for suspected sarcoma were made from January 2004 to December 2008. Of these, 154 referrals were made under the two-week rule of which 102 were referred purely on the clinical criteria for suspected soft tissue sarcoma. The remaining patients were referred after non-urgent special investigations indicated the possibility of sarcoma. Twelve patients referred under the two-week rule were proved to have sarcoma, nine after specific investigations including imaging or histological diagnosis. Of the 102 patients referred on clinical suspicion of a sarcoma, two patients had proven soft tissue sarcomas and one patient a cutaneous sarcoma. Between 2004 and 2008, the number of 2-week referrals rose 25-fold but accounted for an increase of less than 1% of the sarcomas treated in this unit. CONCLUSIONS The numbers of all referrals for suspected sarcoma are increasing; however, the rate of increase of 2-week referrals is increasing faster than routine referrals and will exceed it in

  3. Spontaneous diaphragmatic rupture related to local invasion by retroperitoneal liposarcoma

    PubMed Central

    Pehar, M; Vukoja, I; Rozić, D; Mišković, J

    2012-01-01

    We report a case of the female patient who was admitted to the hospital because of syncope experienced while climbing stairs. Diagnostic workup raised the suspicion of a right diaphragmatic rupture that was eventually confirmed by surgery (right-sided thoracotomy). Surgery also revealed tissue protruding through the rupture site from within the retroperitoneum that was proven subsequently to be a dedifferentiated liposarcoma. Second surgery was performed to completely remove the liposarcoma tissue and repair a coincident old right lumbar region hernia. The patient recovered fully. Spontaneous rupture of the diaphragm is rare and this is especially true for the right hemidiaphragm. We report the first case of diaphragmatic rupture caused by local infiltration by a retroperitoneal liposarcoma. This and similar reports emphasise that in cases with high clinical suspicion of diaphragmatic rupture, diagnosis should be pursued even in the absence of a preceding traumatic event. PMID:22524913

  4. Spontaneous diaphragmatic rupture related to local invasion by retroperitoneal liposarcoma.

    PubMed

    Pehar, M; Vukoja, I; Rozić, D; Mišković, J

    2012-01-01

    We report a case of the female patient who was admitted to the hospital because of syncope experienced while climbing stairs. Diagnostic workup raised the suspicion of a right diaphragmatic rupture that was eventually confirmed by surgery (right-sided thoracotomy). Surgery also revealed tissue protruding through the rupture site from within the retroperitoneum that was proven subsequently to be a dedifferentiated liposarcoma. Second surgery was performed to completely remove the liposarcoma tissue and repair a coincident old right lumbar region hernia. The patient recovered fully. Spontaneous rupture of the diaphragm is rare and this is especially true for the right hemidiaphragm. We report the first case of diaphragmatic rupture caused by local infiltration by a retroperitoneal liposarcoma. This and similar reports emphasise that in cases with high clinical suspicion of diaphragmatic rupture, diagnosis should be pursued even in the absence of a preceding traumatic event.

  5. Post-Chemotherapy Robotic Retroperitoneal Lymph Node Dissection: Institutional Experience.

    PubMed

    Kamel, Mohamed H; Littlejohn, Nathan; Cox, Michelle; Eltahawy, Ehab A; Davis, Rodney

    2016-05-01

    There is little literature on robotic retroperitoneal lymph node dissection (RRPLND) in the difficult post-chemotherapy (PC) setting. We report on the outcome of RRPLND in patients with PC-residual masses. Between 2011 and 2015, we performed 12 PC-RRPLND. Mean patient age was 37.8 years. Mean body mass index was 30.78. Nine (75%) patients had nonseminomatus germ cell tumor (NSGCT) and three (25%) patients had seminoma tumors. Cancer stage was III in six (50%), II-C in three (25%), II-B in two (16.7%), and II-A in one (8.03%). International Germ Cell Cancer Collaborative Group (IGCCCG) prognostic classification in the NSGCT was good in six, intermediate in two, and poor in one, and was good in the three seminoma patients. The procedure was completed effectively in 11 (91.7%) patients. Mean operative time was 312 minutes. The mean estimated blood loss was 475 mL. Mean hospital stay was 3.2 days. Mean number of lymph node excised was 12. Six of the excised masses were ≥5 cm (N3), largest was 7.5 cm. Pathology showed teratoma in five (45.5%), benign/necrosis in five (45.5%), and viable germ cells in one (9%). Major complication (Clavien ≥3) occurred in one patient and minor (Clavien ≤2) in two. Antegrade ejaculation was preserved in eight patients and in one could not be assessed. At a median follow-up of 31 months, no infield or outfield relapses occurred. Robotic PC-retroperitoneal lymph node dissection (RPLND) is technically feasible and with acceptable morbidity. It is associated with low blood loss and short hospital stay. More research is needed to assess the long-term outcome and to compare standard open RPLND.

  6. Differentiation of Lymphoma Presenting as Retroperitoneal Mass and Retroperitoneal Fibrosis: Evaluation with Multidetector-row Computed Tomography.

    PubMed

    Zhang, Shuai; Chen, Min; Li, Chun-Mei; Song, Guo-Dong; Liu, Ying

    2017-03-20

    Retroperitoneal fibrosis (RPF) and lymphoma presenting as retroperitoneal mass may closely resemble each other and misdiagnosis may occur. This study investigated the differential imaging features of RPF and lymphoma which presented as a retroperitoneal soft tissue using multidetector-row computed tomography (MDCT). The 42 consecutive patients were included in this retrospective review, including 19 RPF patients (45.2%; including 13 males and 6 females; mean age: 56.7 ± 6.2 years) and 23 patients with lymphoma (54.8%; including 14 males and 9 females; mean age: 57.4 ± 12.3 years). An array of qualitative computed tomography (CT) features of lesions in 42 consecutive patients with newly diagnosed untreated RPF and lymphoma were retrospectively analyzed. The quantitative size of the lesion at the para-aortic region and attenuation in the precontrast, arterial, and portal phases were calculated in regions of interest and compared between the patients with newly diagnosed untreated RPF and with lymphoma. Receiver operating characteristic curve analysis was used to assess the potential diagnostic value of each quantitative parameter. Inter-reader concordance was also calculated. Mean ages between patients with RPF and lymphoma were not significantly different (56.7 ± 6.2 years vs. 57.4 ± 12.3 years P = 0.595). Compared to those in patients with lymphoma, homogeneous enhancement (65.2% vs. 94.7%, P = 0.027) and pelvic extension (52.2% vs. 89.5%, P = 0.017) were significantly more common while the involvement of additional nodes (78.3% vs. 5.3%, P < 0.001), suprarenal extension (60.9% vs. 15.8%, P = 0.004), and aortic displacement (43.5% vs. 5.3%, P = 0.006) were significantly less common in patients with RPF. Lesion size at the para-aorta was significantly greater in patients with lymphoma, compared with RPF patients (3.9 ± 1.2 cm vs. 1.8 ± 0.6 cm; P < 0.001). The attenuation values in three phases were not significantly different between patients with RPF and

  7. AZD0530 in Treating Patients With Recurrent Locally Advanced or Metastatic Soft Tissue Sarcoma

    ClinicalTrials.gov

    2015-07-02

    Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Rhabdomyosarcoma; Dermatofibrosarcoma Protuberans; Endometrial Stromal 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

  8. Thoraco-abdominal wall reconstruction after surgical debulking of a giant retroperitoneal liposarcoma: a case report.

    PubMed

    Colebunders, B; Colpaert, S D M; Mertens, M; Willemsen, P

    2011-01-01

    A case of a patient with a recurrent dedifferentiated retroperitoneal liposarcoma with extensive invasion of the thoraco-abdominal wall including the skin, requiring reconstructive surgery after debulking of the tumor is reported.

  9. A case of disseminated recurrent retroperitoneal plasmacytoma with left obstructive hydronephrosis.

    PubMed

    Mainali, Naba Raj; Jalota, Leena; Pathak, Ranjan; Jain, Shashank

    2014-07-03

    Retroperitoneal extramedullary plasmacytoma (EMP) is a rare condition that often poses a diagnostic challenge. This is mainly due to its unusual location and non-specific symptoms especially in its early stages. Retroperitoneal plasmacytoma with renal involvement is an extremely rare entity and to date, less than 25 cases have been reported in the literature. We describe a case of a 65-year-old woman with primary retroperitoneal plasmacytoma, confirmed by ultrasound-guided biopsy and histopathological examination, with unilateral left obstructive hydronephrosis and metastasis to the lungs. Our patient was started on chemotherapy to which she did not respond. Subsequently, she resorted to palliative measures. Our case highlights key aspects of clinical presentation, diagnosis and treatment options available for management of retroperitoneal plasmacytoma. 2014 BMJ Publishing Group Ltd.

  10. A case of disseminated recurrent retroperitoneal plasmacytoma with left obstructive hydronephrosis

    PubMed Central

    Mainali, Naba Raj; Jalota, Leena; Pathak, Ranjan; Jain, Shashank

    2014-01-01

    Retroperitoneal extramedullary plasmacytoma (EMP) is a rare condition that often poses a diagnostic challenge. This is mainly due to its unusual location and non-specific symptoms especially in its early stages. Retroperitoneal plasmacytoma with renal involvement is an extremely rare entity and to date, less than 25 cases have been reported in the literature. We describe a case of a 65-year-old woman with primary retroperitoneal plasmacytoma, confirmed by ultrasound-guided biopsy and histopathological examination, with unilateral left obstructive hydronephrosis and metastasis to the lungs. Our patient was started on chemotherapy to which she did not respond. Subsequently, she resorted to palliative measures. Our case highlights key aspects of clinical presentation, diagnosis and treatment options available for management of retroperitoneal plasmacytoma. PMID:24994764

  11. Organ Preservation in a Case of Retroperitoneal Ganglioneuroma: A Case Report and Review of Literature

    PubMed Central

    2016-01-01

    The retroperitoneum is a closed space harbouring vital organs including the great vessels, kidneys and adrenal glands, ureters, and the ascending and descending colon. Surgical management of retroperitoneal pathologies may need multiorgan resection in order to achieve complete surgical resection while preservation of surrounding organs should be attempted, especially in case of benign tumors. We present a case of 15-year-old girl with an 11 × 6 × 5 cm retroperitoneal ganglioneuroma displacing the right kidney, renal vein, and ureter and abutting the IVC which was excised in toto preserving the right kidney and ureter with careful dissection around the great vessels. We also attempt to review the various surgical options available while dealing with these benign retroperitoneal tumors which are often detected incidentally and usually surround important retroperitoneal organs and vessels. PMID:27668117

  12. Diffuse abdominal pain, nausea and vomiting due to retroperitoneal fibrosis: a rare but often missed diagnosis.

    PubMed

    Netzer, P; Binek, J; Hammer, B

    1997-10-01

    Retroperitoneal fibrosis is a rare chronic inflammatory disease usually involving the ureters, retroperitoneal vessels and nerves; however, any intestinal organ may also be involved. In recent years, a few successful immunosuppressive treatments of this disease have been described and surgery can, therefore, probably be avoided in most cases. We report here on a case of secondary retroperitoneal fibrosis with compression and midline deviation of the ureters and impaired renal function which was probably caused by ergotamine abuse because of migraine. The patient complained of diffuse abdominal pain, nausea and vomiting. After immunosuppressive treatment with azathioprine and prednisone for a year, we observed a complete resolution of the retroperitoneal mass on computed tomography, although renal function remained impaired. Eleven months after the cessation of treatment, the patient had not relapsed.

  13. Laparoscopic excision of a large retroperitoneal lymphovascular malformation in an adult

    PubMed Central

    Suryawanshi, Pravin R.; Agrawal, Mohit M.; Rathod, Mukesh D.; Mandhane, Anirudha M.

    2017-01-01

    Retroperitoneal lymphangioma is a rare benign tumour of the retroperitoneal lymphatics that usually manifests in infancy. It is worth reporting of an unexpected presentation, especially in an adult. They frequently affect the neck (75%) and the axilla (20%). Intra-abdominal lymphangiomas (<5%) have been reported in the mesentery, gastrointestinal tract, spleen, liver and pancreas. Retroperitoneal lymphangiomas account for nearly 1% of all lymphangiomas and are uncommon incidental findings usually at surgery, autopsy or lymphography. Differentiating cystic lymphangiomas from other cystic growths by imaging studies alone is often inconclusive, and surgery is frequently required for definitive diagnosis. An interesting and rare case of a retroperitoneal lymphangioma in an adult patient which was removed laparoscopically is described here. PMID:27251838

  14. Targeting epigenetic misregulation in synovial sarcoma.

    PubMed

    Waterfall, Joshua J; Meltzer, Paul S

    2012-03-20

    Like many sarcomas, synovial sarcoma is driven by a characteristic oncogenic transcription factor fusion, SS18-SSX. In this issue of Cancer Cell, Su et al. elucidate the protein partners necessary for target gene misregulation and demonstrate a direct effect of histone deacetylase inhibitors on the SS18-SSX complex composition, expression misregulation, and apoptosis.

  15. 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

    2016-01-14

    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.

  16. Ewing sarcoma of the oral cavity. A review

    PubMed Central

    Bagán, José; Poveda-Roda, Rafael

    2017-01-01

    Objectives A review is made of the clinical, diagnostic, therapeutic and survival characteristics of Ewing sarcoma (ES) of the oral cavity. Material and Methods A systematic literature search was carried out, with restrictions referred to time (1960-2014), language (English and Spanish) and type of study (case reports, letters, datasets, reviews). The following MeSH terms and boolean operators were used: Ewing AND Sarcoma AND [tongue, jaw, maxilla, cheek, condyle OR temporomandibular, floor AND mouth, gum OR gingiva, palate OR palatal, lip, uvula, head AND neck]. Results Seventy-one cases of ES of the oral cavity were documented from 53 articles. The main differences versus ES of other locations were a younger age at manifestation, a shorter time from symptoms onset to diagnosis, and swelling as the most frequent clinical manifestation versus swelling and pain in the rest of disease locations. The way in which ES manifests in the oral cavity is varied and comprises dental displacement (19.7%), dental mobility (7%), root reabsorption (5.6%), destruction of the dental follicle (4.2%), premature exfoliation (4.2%) and paresthesia of the chin (2.8%). Metastatic neck adenopathies appear in 11.3% of the cases. Significant differences in survival are observed between patients with a complete diagnosis of ES (hematoxylin-eosin staining, PAS positivity, CD99 positivity) and those with an incomplete diagnosis. Conclusions Ewing sarcoma of the oral cavity presents a series of specific features that distinguish it from ES of other locations. Key words:Primitive neuroectodermal tumor, PNET, Ewing sarcoma, Ewing tumor, sarcoma, oral cavity. PMID:28210452

  17. Adjuvant chemotherapy following surgery in the management of uterine sarcomas.

    PubMed

    Szánthó, A; Bálega, J; Szabó, I; Demeter, A; Sipos, N; Csapó, Z; Papp, Z

    2003-01-01

    The aim of this study was to investigate the use of imaging tools in the diagnosis of uterine sarcomas, and to evaluate the effect of the adjuvant chemotherapy for uterine sarcomas. The data of 29 patients with uterine sarcomas who received cytostatic polychemotherapy between 1990 and 2000 at the Oncological Division of the Ist Department of Obstetrics and Gynecology, Semmelweis University were evaluated by the authors. Symptoms leading to diagnosis and methods of diagnosis were examined. Vascular changes shown by two-dimensional, color and pulsed Doppler ultrasonography were observed. For staging the currently accepted FIGO method was adopted. Most of the patients underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAH & BSO). In each case we administered adjuvant combination chemotherapy according to the CYVADIC-protocol. The effect of adjuvant chemotherapy was evaluated. Six patients had Stage I, ten had Stage II, 11 had Stage III, and two had Stage IV disease. The mean age of the patients was 53.6 years with a range of 22 to 77 years. Histopathologic distribution included nine leiomyosarcomas (LMS), 13 mixed mesodermal sarcomas (MMS), and seven endometrial stromal sarcomas (ESS). Although most patients experienced neutropenia following cytotoxic chemotherapy, other non-hematologic adverse effects were easy to control. The average progression-free interval was 22.14 months, in which no significant difference was found between the histologic types. Different stages showed highly varied responses: surprisingly, patients in Stage IV with lung metastases were documented to have the longest progression-free survival. The three-year survival rate for all stages was demonstrated in 34.4% of cases. Patients with progressive disease had an average survival period of 4.4 months. These findings suggest that adjuvant cytostatic therapy for patients with distant metastasis confined to a single organ may produce better results than expected.

  18. The ganglioside antigen GD2 is surface-expressed in Ewing sarcoma and allows for MHC-independent immune targeting

    PubMed Central

    Kailayangiri, S; Altvater, B; Meltzer, J; Pscherer, S; Luecke, A; Dierkes, C; Titze, U; Leuchte, K; Landmeier, S; Hotfilder, M; Dirksen, U; Hardes, J; Gosheger, G; Juergens, H; Rossig, C

    2012-01-01

    Background: Novel treatment strategies are needed to cure disseminated Ewing sarcoma. Primitive neuroectodermal features and a mesenchymal stem cell origin are both compatible with aberrant expression of the ganglioside antigen GD2 and led us to explore GD2 immune targeting in this cancer. Methods: We investigated GD2 expression in Ewing sarcoma by immunofluorescence staining. We then assessed the antitumour activity of T cells expressing a chimeric antigen receptor specific for GD2 against Ewing sarcoma in vitro and in vivo. Results: Surface GD2 was detected in 10 out of 10 Ewing sarcoma cell lines and 3 out of 3 primary cell cultures. Moreover, diagnostic biopsies from 12 of 14 patients had uniform GD2 expression. T cells specifically modified to express the GD2-specific chimeric receptor 14. G2a-28ζ efficiently interacted with Ewing sarcoma cells, resulting in antigen-specific secretion of cytokines. Moreover, chimeric receptor gene-modified T cells from healthy donors and from a patient exerted potent, GD2-specific cytolytic responses to allogeneic and autologous Ewing sarcoma, including tumour cells grown as multicellular, anchorage-independent spheres. GD2-specific T cells further had activity against Ewing sarcoma xenografts. Conclusion: GD2 surface expression is a characteristic of Ewing sarcomas and provides a suitable target antigen for immunotherapeutic strategies to eradicate micrometastatic cells and prevent relapse in high-risk disease. PMID:22374462

  19. A Comprehensive Single Institutional Review of 2 Years in a Designated Fast-Track Sarcoma Diagnostic Clinic Linked with a Sarcoma Specialist Advisory Group: Meeting the Target but Failing the Task?

    PubMed Central

    Davidson, Dochka; Wong, Han Hsi; Horan, Gail; Bearcroft, Philip W. P.; Grant, Ian; Grimer, Robert; Hopper, Melanie A.; Hatcher, Helen; Earl, Helena

    2016-01-01

    Background. National guidelines prompted the implementation of a designated two-week wait referral pathway to facilitate the early diagnosis of sarcomas, to improve treatment outcomes. Methods. Patients referred to the Cambridge Sarcoma Diagnostic Clinic between January 2013 and December 2014 were identified through the electronic appointments system. Information was retrospectively retrieved about patient characteristics and details of the diagnostic pathway. Results. 17.3% of patients referred (69/397) were diagnosed with a malignancy. Of these, 59.3% (41/69) had primary sarcomas, 17.4% (12/69) had metastatic cancer, and 23.2% (16/69) had a different primary malignancy. 15% of the 41 sarcomas were <5 cm, 34% in the 5–10 cm range, and 51% >10 cm. Sarcomas diagnosed through this clinic represented 13% (41/315) of sarcomas managed at the centre during the same 2 years. Conclusion. While we achieved the target of 10% (41/397) sarcoma diagnosis rate in the rapid access clinic, only 15% of these were <5 cm better prognosis lesions. This calls into question the “real world” impact of such diagnostic clinics on early diagnosis of sarcomas. In order to enhance generic cancer diagnostic skills, training in these diagnostic clinics could be usefully integrated into national training curricula for both surgical and nonsurgical oncologists. PMID:27340367

  20. Ewing Sarcoma in the Right Ventricle

    PubMed Central

    Zhao, Bihong; Thangam, Manoj; Loyalka, Pranav; Buja, L. Maximilian; Kar, Biswajit; Gregoric, Igor D.

    2016-01-01

    Ewing sarcoma is the second most prevalent malignant primary bone tumor but constitutes only a small proportion of cardiac metastases. We present a case of asymptomatic Ewing sarcoma metastatic to the right ventricle. A 36-year-old man presented for evaluation and resection of a pedunculated right ventricular cardiac tumor. Three years before, he had been diagnosed with translocation-negative Ewing sarcoma, for which he had undergone chemotherapy and amputation of the left leg below the knee. We resected the right ventricular tumor. Analysis of the resected mass supported the diagnosis of metastatic Ewing sarcoma. Postoperative transthoracic echocardiograms showed normal biventricular size and function. One year later, the patient had no recurrence of the sarcoma. In addition to discussing this case, we review the relevant medical literature. PMID:27777536