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Sample records for retroperitoneal sarcoma results

  1. 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

  2. Spilled gallstones mimicking a retroperitoneal sarcoma following laparoscopic cholecystectomy.

    PubMed

    Kim, Bum-Soo; Joo, Sun-Hyung; Kim, Hyun-Cheol

    2016-05-01

    Laparoscopic cholecystectomy has become a standard treatment of symptomatic gallstone disease. Although spilled gallstones are considered harmless, unretrieved gallstones can result in intra-abdominal abscess. We report a case of abscess formation due to spilled gallstones after laparoscopic cholecystectomy mimicking a retroperitoneal sarcoma on radiologic imaging. A 59-year-old male with a surgical history of a laparoscopic cholecystectomy complicated by gallstones spillage presented with a 1 mo history of constant right-sided abdominal pain and tenderness. Computed tomography and magnetic resonance imaging demonstrated a retroperitoneal sarcoma at the sub-hepatic space. On open exploration a 5 cm × 5 cm retroperitoneal mass was excised. The mass contained purulent material and gallstones. Final pathology revealed abscess formation and foreign body granuloma. Vigilance concerning the possibility of lost gallstones during laparoscopic cholecystectomy is important. If possible, every spilled gallstone during surgery should be retrieved to prevent this rare complication. PMID:27158213

  3. Spilled gallstones mimicking a retroperitoneal sarcoma following laparoscopic cholecystectomy

    PubMed Central

    Kim, Bum-Soo; Joo, Sun-Hyung; Kim, Hyun-Cheol

    2016-01-01

    Laparoscopic cholecystectomy has become a standard treatment of symptomatic gallstone disease. Although spilled gallstones are considered harmless, unretrieved gallstones can result in intra-abdominal abscess. We report a case of abscess formation due to spilled gallstones after laparoscopic cholecystectomy mimicking a retroperitoneal sarcoma on radiologic imaging. A 59-year-old male with a surgical history of a laparoscopic cholecystectomy complicated by gallstones spillage presented with a 1 mo history of constant right-sided abdominal pain and tenderness. Computed tomography and magnetic resonance imaging demonstrated a retroperitoneal sarcoma at the sub-hepatic space. On open exploration a 5 cm × 5 cm retroperitoneal mass was excised. The mass contained purulent material and gallstones. Final pathology revealed abscess formation and foreign body granuloma. Vigilance concerning the possibility of lost gallstones during laparoscopic cholecystectomy is important. If possible, every spilled gallstone during surgery should be retrieved to prevent this rare complication. PMID:27158213

  4. Retroperitoneal Sarcoma: Fact, Opinion, and Controversy.

    PubMed

    Gladdy, Rebecca A; Gupta, Abha; Catton, Charles N

    2016-10-01

    After diagnosis of retroperitoneal sarcoma (RPS), detailed imaging and multidisciplinary discussion should guide treatment including surgical resection and in select cases, neoadjuvant therapy. Local recurrence is common in RPS and is associated with grade, histologic subtype, completeness of resection, and size. As guidelines to standardize RPS patient management emerge, expert pathologic assessment and management in centers of excellence are benchmarks of quality of care. The efficacy of current chemotherapy is limited and there is a critical need to understand the molecular basis of sarcoma so that new drug therapies are developed. Multicenter clinical trials are needed to limit opinion and controversy in this complex and challenging disease. PMID:27591493

  5. SURGICAL MANAGEMENT OF RETROPERITONEAL AND PELVIC SARCOMAS

    PubMed Central

    Tan, Marcus C. B.; Yoon, Sam S.

    2014-01-01

    Management of retroperitoneal sarcomas presents technical and oncological challenges. Imaging is crucial for diagnosis and to define local tumor extent. Complete gross resection at initial presentation is the best chance for cure, but there is controversy as to how this can be best achieved. There is a long-term risk of local recurrence, which is best treated with repeat resection if feasible. The roles of radiation and chemotherapy remain undefined. PMID:25482329

  6. Treatment outcome of postoperative radiotherapy for retroperitoneal sarcoma

    PubMed Central

    Lee, Hyun Jin; Kwon, Tae-Won; Yook, Jeong Hwan; Kim, Song-Cheol; Han, Duck-Jong; Kim, Choung-Soo; Ahn, Hanjong; Chang, Heung Moon; Ahn, Jin-Hee; Jwa, Eun Jin; Lee, Sang-Wook; Kim, Jong Hoon; Choi, Eun Kyung; Shin, Seong Soo; Ahn, Seung Do

    2011-01-01

    Purpose To evaluate the treatment outcome and prognostic factor after postoperative radiotherapy in retroperitoneal sarcoma. Materials and Methods Forty patients were treated with surgical resection and postoperative radiotherapy for retroperitoneal sarcoma from August 1990 to August 2008. Treatment volume was judged by the location of initial tumor and surgical field, and 45-50 Gy of radiation was basically delivered and additional dose was considered to the high-risk area. Results The median follow-up period was 41.4 months (range, 3.9 to 140.6 months). The 5-year overall survival (OS) was 51.8% and disease free survival was 31.5%. The 5-year locoregional recurrence free survival was 61.9% and distant metastasis free survival was 50.6%. In univariate analysis, histologic type (p = 0.006) was the strongest prognostic factor for the OS and histologic grade (p = 0.044) or resection margin (p = 0.032) had also effect on the OS. Histologic type (p = 0.004) was unique significant prognostic factor for the actuarial local control. Conclusion Retroperitoneal sarcoma still remains as a poor prognostic disease despite the combined modality treatment including surgery and postoperative radiotherapy. Selective dose-escalation of radiotherapy or combination of effective chemotherapeutic agent must be considered to improve the treatment result especially for the histopathologic type showing poor prognosis. PMID:22984679

  7. Contemporary Management of Retroperitoneal Soft Tissue Sarcomas.

    PubMed

    Olimpiadi, Yuliya; Song, Suisui; Hu, James S; Matcuk, George R; Chopra, Shefali; Eisenberg, Burton L; Sener, Stephen F; Tseng, William W

    2015-08-01

    Management of retroperitoneal soft tissue sarcomas (RP STS) can be very challenging. In contrast to the more common extremity STS, the two predominant histologic subtypes encountered in the retroperitoneum are well-differentiated/dedifferentiated liposarcoma and leiomyosarcoma. Surgery remains the mainstay of treatment for RP STS. Preoperative planning and anticipation of the need for resection of adjacent organs/structures are critical. The extent of surgery, including the role of compartmental resection, is still controversial. Radiation therapy may be an important adjunct to surgery to provide locoregional disease control; this is currently being evaluated in the preoperative setting in the EORTC STRASS trial. Systemic therapy, tailored to the specific histologic subtype, may also be of benefit for the management of RP STS. Further investigation of novel therapies (e.g., targeted therapies, immunotherapy) is needed. Overall, multi-institutional collaboration is important moving forward, to continue to better understand and optimize management of this disease. PMID:26092408

  8. Recent Progress in the Management of Retroperitoneal Sarcoma

    PubMed Central

    Cheifetz, Rona; Catton, Charles N.; Kandel, Rita; O'Sullivan, Brian; Couture, Jean

    2001-01-01

    Retroperitoneal sarcomas (RPS) are rare tumours that typically present late and carry a poor prognosis even following grossly complete resection. In an attempt to improve the outlook for patients with RPS, sarcoma specialists have employed various adjuvant therapies, including extermal beam radiation, intraoperative radiation, brachyradiation and systemic chemotherapy. This article reviews the presentation and prognosis of RPS, and focuses on the results of new treatment strategies compared with conventional management. A Medline search of the English literature was performed to identify all retrospective and prospective reports relating to the management of adult RPS published since 1980. Series that did not analyse RPS separately from other intra-abdominal or extra-abdominal sarcomas or other malignancies were excluded, and information on investigation, presentation, prognostic factors, treatment and outcome was extracted from the remaining reports. Survival and local control data were collected from reports that contained at least 30 cases of RPS (n = 31). While surgical resection remains the cornerstone of treatment for RPS, the majority of patients will relapse and die from sarcoma within 5 years of resection. Adjuvant radiation may improve these results, but further trials are required to definitively demonstrate its benefit. Possible reasons for the failure of conventional treatment are discussed, and alternative strategies designed to overcome these obstacles are presented. PMID:18521304

  9. 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.

  10. Intraoperative electron beam radiation therapy for retroperitoneal soft tissue sarcoma.

    PubMed

    Willett, C G; Suit, H D; Tepper, J E; Mankin, H J; Convery, K; Rosenberg, A L; Wood, W C

    1991-07-15

    From December 1981 to December 1989, 20 patients with primary or recurrent retroperitoneal sarcoma received 4000 to 5000 cGy of external beam radiation therapy (EBRT) in conjunction with surgical resection and intraoperative radiation therapy (IORT). Seventeen of 20 patients underwent complete (14 patients) or partial (3 patients) resection. Three patients had shown evidence of metastases after EBRT by the time of surgery. The 4-year actuarial local control and disease-free survival rates of the 17 patients undergoing resection were 81% and 64%, respectively. Twelve patients received IORT at the time of resection for microscopic disease (10 patients) or gross residual sarcoma (2 patients). Of the ten patients receiving IORT for microscopic tumor, one patient has died of local failure and peritoneal sarcomatosis and two patients have died of distant metastases only. The remaining seven patients are disease-free. One patient treated for gross residual sarcoma has experienced a local failure 1 year after IORT and is without disease 7 years after salvage chemotherapy. The other patient treated for gross residual sarcoma has died of local failure. Five patients did not receive IORT at the time of resection because of the extensive size of the tumor bed. Three of these patients are disease-free with one patient alive with lung metastases and one patient dying of hepatic metastases. Aggressive radiation and surgical procedures appear to provide satisfactory resectability and local control with acceptable tolerance. PMID:1906369

  11. Hepatitis B Reactivation After Ifosfamide Therapy for Retroperitoneal Sarcoma

    PubMed Central

    Chhibar, Purva; Zhu, Ziqiang; Cheedella, Naga K.S.; Chaudhry, Rashid; Wang, Jen Chin

    2016-01-01

    Patient: Male, 61 Final Diagnosis: Ifosfamide induced reactivation of hepatitis B Symptoms: — Medication: Ifosfamide Clinical Procedure: DC ifosfamide and added Tenofovir Specialty: Oncology Objective: Unusual clinical course Background: Patients receiving cancer treatment are at risk for hepatitis B virus (HBV) reactivation. Ifosfamide is an alkylating agent and is considered to be one of the important drugs for the treatment of metastatic sarcoma. No association of ifosfamide and HBV reactivation has been reported so far. Case Report: We report a case of a 61-year-old Asian man with metastatic retroperitoneal liposarcoma who was HBcAb positive and was treated with ifosfamide and dacarbazine, developed HBV reactivation secondary to ifosfamide requiring treatment with tenofovir. To the best of our knowledge, this is the first report describing HBV reactivation in a patient with positive HBcAb who was treated with ifosfamide. Conclusions: We recommend close surveillance of possible HBV reactivation while employing ifosfamide chemotherapy. PMID:27246626

  12. Hepatitis B Reactivation After Ifosfamide Therapy for Retroperitoneal Sarcoma.

    PubMed

    Chhibar, Purva; Zhu, Ziqiang; Cheedella, Naga K S; Chaudhry, Rashid; Wang, Jen Chin

    2016-01-01

    BACKGROUND Patients receiving cancer treatment are at risk for hepatitis B virus (HBV) reactivation. Ifosfamide is an alkylating agent and is considered to be one of the important drugs for the treatment of metastatic sarcoma. No association of ifosfamide and HBV reactivation has been reported so far. CASE REPORT We report a case of a 61-year-old Asian man with metastatic retroperitoneal liposarcoma who was HBcAb positive and was treated with ifosfamide and dacarbazine, developed HBV reactivation secondary to ifosfamide requiring treatment with tenofovir. To the best of our knowledge, this is the first report describing HBV reactivation in a patient with positive HBcAb who was treated with ifosfamide. CONCLUSIONS We recommend close surveillance of possible HBV reactivation while employing ifosfamide chemotherapy. PMID:27246626

  13. Preoperative intensity modulated radiation therapy for retroperitoneal sarcoma.

    PubMed

    El-Bared, Nancy; Taussky, Daniel; Mehiri, Selma; Patocskai, Erika; Roberge, David; Donath, David

    2014-06-01

    The use of intensity modulated radiation therapy (IMRT) has allowed for the administration of high doses to retroperitoneal sarcomas (RSTS) while limiting toxicity to adjacent organs. The purpose of our study is to assess the outcome and toxicities of patients with RSTS treated with neo-adjuvant external beam radiation (EBRT) therapy using IMRT. This is a retrospective study of 21 patients treated with preoperative IMRT for primary or recurrent RSTS between 2005 and 2011. Overall survival (OS) and local recurrence free survival (LRFS) were computed using the Kaplan-Meier method (log-rank test). Acute and chronic toxicities were assessed using the CTCAE v. 3 criteria. The actuarial 2 and 3-year OS was 66% for both and the 5-year OS was 51%. As for LRFS it was 57% at 2 and 3-year and 51% for the 5-year LRFS. Factors predictive for local control were microscopically negative margins (p = 0.022), a median tumor diameter <15 cm (p = 0.007) and pathology of liposarcoma (p = 0.021). Furthermore, patients treated for recurrent disease fared worse (p = 0.04) in local control than patients treated for primary disease. As for OS, patients treated for Grade 1 histology had a better outcome (p 5 0.05). EBRT was generally well tolerated. Acute gastrointestinal (GI) Grade 1 or 2 toxicities occurred in 33% of patients and one patient had unexplained post-radiation Grade 2 fever that resolved after tumor resection. As for chronic toxicities 24% of our patients presented Grade 1 GI toxicity and one patient presented Grade 3 small bowel stenosis not clearly due to radiation toxicity. Despite the location and volume of the tumors treated, preoperative IMRT was very well tolerated in our patients with retroperitoneal sarcoma. Unfortunately local recurrences remain common and dose escalation is to be considered. PMID:23919397

  14. Preoperative Intensity Modulated Radiation Therapy for Retroperitoneal Sarcoma

    PubMed Central

    El-Bared, Nancy; Taussky, Daniel; Mehiri, Selma; Patocskai, Erika; Roberge, David; Donath, David

    2014-01-01

    The use of intensity modulated radiation therapy (IMRT) has allowed for the administration of high doses to retroperitoneal sarcomas (RSTS) while limiting toxicity to adjacent organs. The purpose of our study is to assess the outcome and toxicities of patients with RSTS treated with neo-adjuvant external beam radiation (EBRT) therapy using IMRT. This is a retrospective study of 21 patients treated with preoperative IMRT for primary or recurrent RSTS between 2005 and 2011. Overall survival (OS) and local recurrence free survival (LRFS) were computed using the Kaplan-Meier method (log-rank test). Acute and chronic toxicities were assessed using the CTCAE v. 3 criteria. The actuarial 2 and 3-year OS was 66% for both and the 5-year OS was 51%. As for LRFS it was 57% at 2 and 3-year and 51% for the 5-year LRFS. Factors predictive for local control were microscopically negative margins (p = 0.022), a median tumor diameter <5 cm (p = 0.007) and pathology of liposarcoma (p = 0.021). Furthermore, patients treated for recurrent disease fared worse (p = 0.04) in local control than patients treated for primary disease. As for OS, patients treated for Grade 1 histology had a better outcome (p = 0.05). EBRT was generally well tolerated. Acute gastrointestinal (GI) Grade 1 or 2 toxicities occurred in 33% of patients and one patient had unexplained post-radiation Grade 2 fever that resolved after tumor resection. As for chronic toxicities 24% of our patients presented Grade 1 GI toxicity and one patient presented Grade 3 small bowel stenosis not clearly due to radiation toxicity. Despite the location and volume of the tumors treated, preoperative IMRT was very well tolerated in our patients with retroperitoneal sarcoma. Unfortunately local recurrences remain common and dose escalation is to be considered. PMID:23919397

  15. Surgical Management of Retroperitoneal Soft Tissue Sarcomas: Role of Curative Resection.

    PubMed

    Rosa, Fausto; Fiorillo, Claudio; Tortorelli, Antonio Pio; Sánchez, Alejandro Martin; Costamagna, Guido; Doglietto, Giovanni Battista; Alfieri, Sergio

    2016-02-01

    Retroperitoneal sarcomas are a rare group of malignant soft tissue tumors with a generally poor prognosis. However, factors affecting the recurrence and long-term survival are not well understood. The aim of this study was to assess clinical, pathological, and treatment-related factors affecting prognosis in patients with retroperitoneal sarcomas. The hospital records of 107 patients who underwent surgical exploration at our unit for primary or recurrent retroperitoneal sarcomas between 1984 and 2013 were reviewed. Of these patients, 92 had a primary tumor and 15 had a recurrent neoplasm. Study end points included factors affecting overall and recurrence-free survival for the 92 patients with primary disease. Mean follow-up was 79.7 ± 56.3 months. Only the patients undergoing surgery for primary sarcoma were included in this study. Overall 5-year survival was 71 per cent. Disease-free 5-year survival was 65 per cent. Only tumor grade affects overall and disease-free survival. This study confirmed the importance of an aggressive surgical management for retroperitoneal sarcomas to offer these patients the best chance of cure. In our series, only the tumor grade seems to be associated with worse outcome and higher rate of recurrence, regardless of the size of the tumor. PMID:26874134

  16. Intensity Modulated Radiation Therapy for Retroperitoneal Sarcoma: A Case for Dose Escalation and Organ at Risk Toxicity Reduction

    PubMed Central

    Koshy, Mary; Lawson, Joshua D.; Staley, Charles A.; Esiashvili, Natia; Howell, Rebecca; Ghavidel, Shahram; Davis, Lawrence W.

    2003-01-01

    Purpose: Radiation therapy for retroperitoneal sarcoma remains challenging because of proximity to surrounding organs at risk (OAR). We report the use of intensity modulated radiation therapy (IMRT) in the treatment of retroperitoneal sarcomas to minimize dose to OAR while concurrently optimizing tumor dose coverage. Patients and methods: From January 2000 to October 2002, 10 patients (average age 56 years) with retroperitoneal sarcoma and one with inguinal sarcoma were treated with radiation at Emory University. Prescription dose to the planning treatment volume (PTV) was commonly 50.4 at 1.8 Gy/fraction. CT simulation was used in each patient, three patients were treated with 3D-conformal treatment (3D-CRT), and the remaining eight received multi-leaf collimator-based (MLC) IMRT. IMRT treatment fields ranged from eight to 11 and average volume treated was 3498 cc. Optimal 3D-CRT plans were generated and compared with IMRT with respect to tumor coverage and OAR dose toxicity. Dose volume histograms were compared for both the 3D-CRT and IMRT plans. Results: Mean dose to small bowel decreased from 36 Gy with 3D-CRT to 27 Gy using IMRT, and tumor coverage (V95) increased from 95.3% with 3D-CRT to 98.6% using IMRT. Maximum and minimum doses delivered to the PTV were significantly increased by 6 and 22%, respectively (P = 0.011, P = 0.055). Volume of small bowel receiving > 30Gy was significantly decreased from 63.5 to 43.1% with IMRT compared with conventional treatment (P = 0.043). Seven patients developed grade 2 nausea, three developed grade 2 diarrhea, one had grade 2 skin toxicity, and one patient developed grade 3 liver toxicity (RTOG toxicity scale). No other delayed toxicities related to radiation were observed. At a median follow-up of 58 weeks, there were no local recurrences and only one patient developed disease progression with distant metastasis in the liver. Conclusions: IMRT for retroperitoneal sarcoma allowed enhanced tumor coverage and better sparing

  17. 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

  18. 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

  19. Combined modality management of retroperitoneal sarcomas: a single institution series of 121 patients

    PubMed Central

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

    2016-01-01

    Purpose To investigate local control, survival outcomes, and complication rates of patients treated with aggressive surgery and radiation therapy (RT) for retroperitoneal sarcomas (RPS). Methods 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 liposarcomas (n=42, 35%). The median follow-up was 100 months (range, 20–467 months). Eighty six (71%) patients 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 post-operative in 33 (27%) (median dose, 55 Gy). Results The 5-year LC and OS 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<0.001, HR 2.7 95% CI 1.6–4.8) and presenting with recurrent disease after previous resection (76% vs. 31% for de novo RPS, P<0.001, 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<0.001) and a RT dose ≥ 60 Gy (P<0.001). Conclusions Intra-abdominal RPS recurrences continue 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. PMID:26130233

  20. 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.

  1. Simultaneous Renal Cell Carcinoma and Giant Retroperitoneal Liposarcoma Involving Small Intestine.

    PubMed

    Reznichenko, Aleksandr A

    2016-01-01

    Background. The concomitant occurrence of a renal cell carcinoma and retroperitoneal sarcoma is extremely rare with only few cases being reported. Methods. We present a case of simultaneous renal cell carcinoma and exceptionally large size retroperitoneal sarcoma involving small intestine. Surgical resection of retroperitoneal sarcoma and simultaneous right nephrectomy were performed. Results. Patient developed recurrent and metastatic disease and underwent debulking surgery following by chemotherapy. Despite aggressive behavior of the retroperitoneal sarcomas, patient is currently (7 years after simultaneous resection and nephrectomy) recurrence-free. Conclusions. Complete surgical resection is the mainstay of therapy for both renal cell carcinoma and retroperitoneal sarcoma. We present a case of simultaneous renal cell carcinoma and exceptionally large size retroperitoneal sarcoma. Debulking surgery and chemotherapy were helpful in our case. PMID:27595033

  2. Simultaneous Renal Cell Carcinoma and Giant Retroperitoneal Liposarcoma Involving Small Intestine

    PubMed Central

    2016-01-01

    Background. The concomitant occurrence of a renal cell carcinoma and retroperitoneal sarcoma is extremely rare with only few cases being reported. Methods. We present a case of simultaneous renal cell carcinoma and exceptionally large size retroperitoneal sarcoma involving small intestine. Surgical resection of retroperitoneal sarcoma and simultaneous right nephrectomy were performed. Results. Patient developed recurrent and metastatic disease and underwent debulking surgery following by chemotherapy. Despite aggressive behavior of the retroperitoneal sarcomas, patient is currently (7 years after simultaneous resection and nephrectomy) recurrence-free. Conclusions. Complete surgical resection is the mainstay of therapy for both renal cell carcinoma and retroperitoneal sarcoma. We present a case of simultaneous renal cell carcinoma and exceptionally large size retroperitoneal sarcoma. Debulking surgery and chemotherapy were helpful in our case. PMID:27595033

  3. 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

  4. Long-Term Outcomes in Treatment of Retroperitoneal Sarcomas: A 15 Year Single-Institution Evaluation of Prognostic Features

    PubMed Central

    Abdelfatah, Eihab; Guzzetta, Angela A.; Nagarajan, Neeraja; Wolfgang, Christopher L.; Pawlik, Timothy M.; Choti, Michael A.; Schulick, Richard; Montgomery, Elizabeth A.; Meyer, Christian; Thornton, Katherine; Herman, Joseph; Terezakis, Stephanie; Frassica, Deborah; Ahuja, Nita

    2016-01-01

    Background Retroperitoneal sarcomas are connective tissue tumors arising in the retroperitoneum. Surgical resection is the mainstay of treatment. Debate has arisen over extent of resection, changes in histological classification/grading, and interest in incorporating radiotherapy. Therefore, we reviewed our institution’s experience to evaluate prognostic factors. Methods Retrospective chart review of all primary RPS patients at Johns Hopkins Hospital from 1994 – 2010. Histologic diagnosis and grading were re-evaluated with current criteria. Prognostic factors for survival, and recurrence were assessed. Results 131 primary RPS patients met inclusion criteria. Median survival for patients who undergo en-bloc resection to negative margins (R0/R1) is 81.7 months. Surgical margins and grade were the most important factors for survival along with age, gender, presence of metastases and resection of ≥5 organs. 5 year survival for R0/R1 resection was 60%, similar to compartmental resection. Radiotherapy significantly decreased local recurrence (p=0.026) on multivariate analysis. Grade in leiomyosarcomas and dedifferentiation in liposarcomas dictated patterns of local vs distal recurrence. Conclusions En bloc surgical resection to R0/R1 margins remains the cornerstone of therapy and provides comparable outcomes to compartmental resections. Grade remains important for prognosis, and histology dictates recurrence patterns. Radiotherapy appears promising for local control and warrants further investigation. PMID:27076350

  5. 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

  6. 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.

  7. Giant dedifferentiated retroperitoneal liposarcoma.

    PubMed

    Dominguez, Elias; Lopez de Cenarruzabeitia, Iñigo; Martinez, Manuel; Rueda, J C; Lede, A; Barreiro, Erica; Diz, Susana

    2008-01-01

    Liposarcoma tumors only represent 0.1% of all cancers, but they are the more common of retroperitoneal sarcomas. It has a great tendency for local recurrence, mainly the dedifferentiated variety, but its complete resection can provide a 5-year survival of 70%. In this report, we present a case of a giant dedifferentiated retroperitoneal liposarcoma that did not affect any neighboring organ and that was successfully treated by means of complete surgical resection. PMID:19731863

  8. 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. PMID:26350044

  9. 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. ...

  10. Sarcomas.

    PubMed

    HaDuong, Josephine H; Martin, Andrew A; Skapek, Stephen X; Mascarenhas, Leo

    2015-02-01

    Malignant bone tumors (osteosarcoma, Ewing sarcoma) and soft-tissue sarcomas (rhabdomyosarcoma, nonrhabdomyosarcoma) account for approximately 14% of childhood malignancies. Successful treatment of patients with sarcoma depends on a multidisciplinary approach to therapy, including oncology, surgery, radiation oncology, radiology, pathology, and physiatry. By combining systemic treatment with chemotherapy and primary tumor control using surgery and/or radiation, survival rates for localized disease range from 70% to 75%. However, children with metastatic or recurrent disease continue to have dismal outcomes. A better understanding of the biology underlying both bone and soft-tissue sarcomas is required to further improve outcomes for children with these tumors. PMID:25435119

  11. Retroperitoneal schwannomas

    PubMed Central

    Veliovits, Dousan; Fiska, Aliki; Zorbas, Georgios; Tentes, Antonios-Apostolos K.

    2012-01-01

    Summary Background: Retroperitoneal schwannomas are very rare and are usually found incidentally. Cases Report: Two rare cases of retroperitoneal schwannomas are reported. Both were incidentally found during US scans for non-specific epigastric pain and were initially diagnosed as non-secreting retroperitoneal tumors. The diagnosis was confirmed by CT scan. In both patients the tumors were resected. The definitive diagnosis was possible by histopathology. Conclusions: Although the preoperative assessment of a retroperitoneal tumor may be indicative of a retroperitoneal schwannoma, the definitive diagnosis is possible only by histopathology after surgical removal of the tumor. PMID:23569539

  12. The long-term results of laparoscopic retroperitoneal pyeloplasty in adults

    PubMed Central

    Gargouri, Mohamed M.; Nouira, Yassine; Kallel, Yousri; Sellami, Ahmed; Boulma, Rami; Mohamed, Chlif; Rhouma, Sami Ben

    2013-01-01

    Objectives To report the long-term outcome of laparoscopic retroperitoneal pyeloplasty (LRP) in adults. Patients and methods Thirty patients underwent LRP for primary pelvi-ureteric junction obstruction (PUJO). Anderson–Hynes dismembered pyeloplasty was used in 28 patients and a Foley Y–V pyeloplasty in two. A JJ stent was inserted antegradely during the procedure. Patients were reviewed at 1 month after LRP for stent removal, and then at 6 and 12 months routinely, using excretory urography. Results The mean patient age was 29.7 years, with a female predominance of 60%. Conversion to open surgery was mandated by dense adhesions secondary to previous pyelonephritis in three patients, and difficulty in suturing in one. The mean (range) operative duration was 228 (190–280) min. There was a crossing vessel in 11 patients and it was not transposed in any. The mean hospital stay after LRP was 4.2 days. The mean (range) follow-up was 60 (29–106) months. Of the 26 patients who had complete laparoscopic procedures, 23 had no evidence of obstruction on long-term postoperative intravenous urography and/or diuretic renography. Conclusion LRP combines the high functional success rate of open pyeloplasty in the long term and the minimally invasive morbidity of laparoscopy. PMID:26558113

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

    PubMed

    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

  14. 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

  15. [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. PMID:17679405

  16. Scrotal hematoma resulting from extracorporeal shock wave lithotripsy for a renal calculus: a sign of retroperitoneal hemorrhage

    PubMed Central

    Katz, Darren J.; Dodds, Lachlan J.

    2011-01-01

    We report a rare case of a patient presenting with scrotal hematoma associated with retroperitoneal hemorrhage after extracorporeal shock wave lithotripsy (ESWL). We propose a mechanism for the formation of scrotal hematoma and its importance as a sign of retroperitoneal hemorrhage. PMID:24578909

  17. [Retroperitoneal ganglioneurinoma].

    PubMed

    Prevljak, Sabina; Dalagija, Faruk; Sehović, Sanja; Kapidzić, Nedzib

    2003-01-01

    We present a case of 20-years-old female with nonspecific symptoms (intermittent abdominal pains) and blood and urine tests showed the changes connected with infection of the urinal tract. The patient was sent to further diagnostic imaging methods, because the repeated attacks of uroinfection after the applied antibiotic therapy. After the US we performed CT and MRI that indicated the existence of retroperitoneal mass which dislocated the surrounding structures (left kidney and the urether) but was not infiltrated them. US were initial method and CT and MRI have done as complementary methods providing the exact picture of the mass. After surgical treatment, ganglioneurinoma was histological diagnosed. PMID:14528723

  18. Secondary retroperitoneal teratoma.

    PubMed

    Grandjean, P; Danse, E; Thys, F; Cosyns, J P; Wese, F X

    2011-01-01

    Retroperitonal teratomas are rare. We report on a case of a retroperitoneal secondary localisation of a gonadal teratoma in a patient who had developed primary testicular teratoma 12 years previously. The retroperitoneal mass was detected with an abdominal CT requested for the management of a non-specific abdominal pain. CT and MRI examinations showed cystic retroperitoneal masses combined with calcifications and peripheral enhancement. Review of the literature is presented, including the common differential diagnoses to be considered. PMID:22338389

  19. Post-traumatic retroperitoneal colonic injury presenting as gluteal abscess.

    PubMed

    Sinha, D D; Sharma, Chetan; Gupta, Vipul; Chaturvedi, V; Sharma, Vinod

    2004-01-01

    We report an 8-year-old boy who sustained blunt retroperitoneal right colonic injury in a vehicular accident and presented with gluteal abscess. Surgical exploration revealed perforated posterior wall of ascending colon with surrounding retroperitoneal abscess communicating with the gluteal region. Right hemicolectomy with drainage of retroperitoneal and gluteal collections resulted in satisfactory recovery. PMID:15333978

  20. Retroperitoneal liposarcoma in two siblings.

    PubMed

    Narita, T; Yokoyama, S; Matsuda, K

    1996-12-01

    We report here on two cases of retroperitoneal liposarcoma which heterochronously occurred in two siblings. A huge myxoid liposarcoma, 20 x 14 cm size, was noticed in a 33-year-old female, who died with multiple liver metastasis in about half a year. Two years later after the death of the younger sister, pleomorphic liposarcoma, 8 x 8 cm in size, was noticed in a 39-year-old male who died with local recurrence and multiple metastasis one year after the operation. Their mother (69 years old) had had an operation for malignant fibrous histiocytoma of the right thigh at age 66. There was no evidence of multiple lipomatosis, an autosomal dominant trait, in the siblings or their family. Diverse soft-tissue sarcomas, but not liposarcoma, occur excessively in siblings with the syndrome of Li-Fraumeni. To our knowledge this is the first such report of its sibship occurrence. PMID:9182297

  1. Genetics Home Reference: retroperitoneal fibrosis

    MedlinePlus

    ... Understand Genetics Home Health Conditions retroperitoneal fibrosis retroperitoneal fibrosis Enable Javascript to view the expand/collapse boxes. Download PDF Open All Close All Description Retroperitoneal fibrosis is a disorder in which inflammation and extensive ...

  2. Retroperitoneal necrotizing fasciitis.

    PubMed

    Rush, B M; Hood, J S; McDonald, J C; Grafton, W D; Burton, G V

    1991-06-01

    We present a case of retroperitoneal necrotizing fasciitis treated by urgent abdomino-perineal resection of the rectum with permanent colostomy. We can find no prior case in the English literature treated in this manner. Retroperitoneal occurrence of this disease is unusual. The process originated from perianal sepsis following chemotherapy for head and neck cancer. PMID:2071998

  3. Surveillance Strategies for Sarcoma: Results of a Survey of Members of the Musculoskeletal Tumor Society

    PubMed Central

    2016-01-01

    Background. Surveillance is crucial to oncology, yet there is scant evidence to guide strategies. Purpose. This survey identified sarcoma surveillance strategies for Musculoskeletal Tumor Society (MSTS) members and rationales behind them. Understanding current practice should facilitate studies to generate evidence-based surveillance protocols. Methods. Permission was granted by the Research and Executive Committee of the MSTS to survey members on surveillance strategies. First, the questionnaire requested demographic and clinical practice information. Second, the survey focused on clinicians' specific surveillance soft tissue and bone sarcoma protocols. Results. 20 percent of MSTS members completed the survey. The primary rationale for protocols was training continuation, followed by published guidelines, and finally personal interpretation of the literature. 95% of the respondents believe that additional studies regarding appropriate surveillance protocols are needed. 87% reported patient concerns regarding radiation exposure from surveillance imaging. For soft tissue and bone sarcoma local recurrence, responders identified surgical margin, histologic grade, and tumor size as the most important factors. For metastases, important risk factors identified included histologic grade, tumor size, and histologic type. Protocols demonstrated wide variation. Conclusion. This survey demonstrates that surveillance strategies utilized by MSTS members are not evidence-based, providing rationale for multi-institutional studies. It also confirms the public health issue of excessive radiation exposure. PMID:27478404

  4. Kras activation in p53-deficient myoblasts results in high-grade sarcoma formation with impaired myogenic differentiation

    PubMed Central

    McKinnon, Timothy; Venier, Rosemarie; Dickson, Brendan C.; Kabaroff, Leah; Alkema, Manon; Chen, Li; Shern, Jack F.; Yohe, Marielle E.; Khan, Javed; Gladdy, Rebecca A.

    2015-01-01

    While genomic studies have improved our ability to classify sarcomas, the molecular mechanisms involved in the formation and progression of many sarcoma subtypes are unknown. To better understand developmental origins and genetic drivers involved in rhabdomyosarcomagenesis, we describe a novel sarcoma model system employing primary murine p53-deficient myoblasts that were isolated and lentivirally transduced with KrasG12D. Myoblast cell lines were characterized and subjected to proliferation, anchorage-independent growth and differentiation assays to assess the effects of transgenic KrasG12D expression. KrasG12D overexpression transformed p53−/− myoblasts as demonstrated by an increased anchorage-independent growth. Induction of differentiation in parental myoblasts resulted in activation of key myogenic regulators. In contrast, Kras-transduced myoblasts had impaired terminal differentiation. p53−/− myoblasts transformed by KrasG12D overexpression resulted in rapid, reproducible tumor formation following orthotopic injection into syngeneic host hindlimbs. Pathological analysis revealed high-grade sarcomas with myogenic differentiation based on the expression of muscle-specific markers, such as Myod1 and Myog. Gene expression patterns of murine sarcomas shared biological pathways with RMS gene sets as determined by gene set enrichment analysis (GSEA) and were 61% similar to human RMS as determined by metagene analysis. Thus, our novel model system is an effective means to model high-grade sarcomas along the RMS spectrum. PMID:25992772

  5. Subcutaneous Axillary and Scalp Metastases from Non-Gynecological Retroperitoneal Leiomyosarcoma: An Unusual Presentation After Surgical Resection

    PubMed Central

    Kaur, Simrandeep; Selhi, Pavneet; Singh, Aminder; Puri, Harpreet; Sood, Neena

    2015-01-01

    Retroperitoneal leiomyosarcomas are rare sarcomas, with an incidence of less than 2 per million population. Cutaneous metastases from sarcoma account for only 1-2.6% of metastatic skin lesions. Cutaneous and subcutaneous metastasis from retroperitoneal leiomyosarcoma is a very rare entity. We present a case of 72-year-old male with scalp nodule and subcutaneous swelling in left posterior axillary fold. Fine needle aspiration cytology from both these sites revealed a sarcoma, which was positive for Smooth Muscle Actin and negative for S100 on cell block immuno-histochemistry (IHC). The past history revealed surgical resection of a retroperitoneal mass in 2010 which was diagnosed on histopathology and IHC as leiomyosarcoma. A final diagnosis of metastatic deposits from leiomyosarcoma was made. Retroperitoneal leiomyosarcoma presenting as scalp and subcutaneous metastasis is an unusual presentation. Adequate clinical history and a high index of clinical suspicion is required to detect cutaneous and subcutaneous metastatic deposits occurring five years after surgical resection. PMID:26788272

  6. 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

  7. Adjuvant radiation for soft tissue sarcomas.

    PubMed

    Dickie, Colleen I; Haas, Rick; O'Sullivan, Brian

    2015-01-01

    Over recent decades, limb-preservation surgery in combination with radiotherapy achieves local control rates exceeding 90% for extremity soft tissue sarcoma (STS). Local control is not as successful for retroperitoneal sarcoma (approximately 60%) despite aggressive surgical approaches including en bloc resection of uninvolved adjacent organs combined with intensity modulated radiotherapy (IMRT). This review will discuss the indications for adjuvant radiation therapy (RT) for primary presentation of soft tissue sarcoma: "What," referring to the type and manner of planning and delivery of RT; "When," referring to the timing and scheduling of RT; and "Why," referring to the rationale for the use of RT will be addressed. From a practical stand point, this Educational Chapter on "adjuvant RT" will focus on pre- and postoperative RT in the context of gross total resection for extremity and retroperitoneal soft tissue sarcoma, the two most frequent paradigms for the use of adjuvant RT. PMID:25993234

  8. [Giant retroperitoneal ganglioneuroma].

    PubMed

    Sarf, Ismail; el Mejjad, Amine; Badre, Latifa; Mani, Ahmed; Aboutaieb, Rachid; Meziane, Fethi

    2003-06-01

    The authors report a new case of retroperitoneal ganglioneuroma in an 18-year-old girl presenting with abdominal mass and lumbosciatica. The diagnosis of retroperitoneal tumour was based on computed tomography and magnetic resonance imaging. Treatment consisted of complete resection of the tumour. The postoperative course was favourable with no recurrence after one year of follow-up. The authors discuss the diagnostic, therapeutic and prognostic aspects of this disease. PMID:12940207

  9. Primary Retroperitoneal Mucinous Cystadenoma

    PubMed Central

    Han, Weon Cheol

    2016-01-01

    Mucinous cystadenomas and cystadenocarcinomas of the ovary are clinically and histopathologically well-established common tumors. However, primary retroperitoneal mucinous cystic tumors are extremely rare, and although their histopathogenesis is still uncertain, several theories have been proposed. Most authors suggest that they develop through mucinous metaplasia in a preexisting mesothelium-lined cyst. An accurate preoperative diagnosis of these tumors is difficult because no effective diagnostic measures have been established. Delay in diagnosis and treatment of this tumor may be fatal for the patient because of complications such as rupture, infection, and malignant transformation. We describe the case of a 31-year-old woman with abdominal pain and a palpable mass. Computed tomography of the abdomen revealed a retroperitoneal cystic mass, which was resected successfully through laparoscopy. Histopathological examination of the resected mass confirmed the diagnosis of a primary retroperitoneal mucinous cystadenoma. The patient was discharged on postoperative day 5 without any complications. PMID:26962534

  10. [Retroperitoneal postoperative necrotizing fasciitis].

    PubMed

    Fichev, G; Poromanski, I; Marina, M

    2000-01-01

    This is a report on clinical experience had with 17 patients presenting necrotizing fasciitis--a complication ever more frequently encountered. The case material is distributed in two group differing by origin and clinical course of the complication. In group one (n = 11) it is a matter of postoperative development of postoperative complication, consistent with the classical "per continuitatem" and "per contiguitatem" mechanisms, while in group two (n = 6) the process originates, evolves and speads within the retroperitoneal space proper. Comprehensive microbiological examinations performed in 13 cases show that in either group different microorganisms are identified. In group one aerobic-anaerobic mixed infection is documented in all patients, with predominance of Enterobacteroidaceae among aerobic ones. In group two, anaerobic bacterial species, mainly Clostridium sp, prevail in all the isolates. The clinical study points to a substantial difference in the time of septic complication occurrence, as well as between the clinical picture of the two species. Accordingly, the final results are radically different--in group one survivorship amounts to 62.6%, whereas in group two--to 16.6% only. PMID:11692928

  11. Improved results in osteogenic sarcoma 1973-79 vs. 1980-86: analysis of results from a single center.

    PubMed

    Cohen, I J; Kaplinsky, C; Katz, K; Mor, C; Goshen, Y; Yaniv, Y; Avidor, I; Yosipovitch, Z; Zaizov, R

    1993-01-01

    Analysis of treatment results in osteogenic sarcoma patients with classical limb primary tumors and without metastasis at diagnosis or major protocol violations showed improved prognosis with a minimum follow-up of over 5.5 years when divided by years of treatment for all event-free survivors. Twelve patients treated in 1980-86 had a 5-year disease-free survival of 67% and 9 treated in 1973-79 had a 5-year disease-free survival of 33% (P = 0.0368). The improvement appeared to reflect the increase in the intensity of the chemotherapy utilized. Definitive surgery, amputation or limb salvage did not affect the outcome. With these surgical approaches the disease-free survival was 67% and 60% respectively. PMID:8454442

  12. 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.

  13. 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. PMID:27542645

  14. 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.

  15. Retroperitoneal Leiomyosarcoma Associated with an Elevated β-HCG Serum Level Mimicking Extragonadal Germ Cell Tumor

    PubMed Central

    Gaertner, Hans-Juergen; Manseck, Andreas; Oehlschlaeger, Sven; Wirth, Manfred P.

    2000-01-01

    Patient. A 65-year-old man was admitted with a large primary retroperitoneal tumor and an increased β-human chorionic gonadotropin (β-HCG) serum level. A germ cell tumor was suspected; however, a computed tomography-guided biopsy failed to enable tumor classification. After two courses of chemotherapy, the β-HCG serum level had returned to the normal level and a diagnostic laparotomy with incisional biopsy was performed. The immunohistochemical examination of the specimen identified the tumor as a retroperitoneal pleomorphic leiomyosarcoma. Discussion. Tumor markers play only a marginal role in the work-up of patients with soft tissue sarcomas. In men with suspected retroperitoneal sarcomas, however, the determination of germ cell tumor markers occasionally enables a preoperative distinguishing of primary retroperitoneal germ cell tumors with considerable consequences for management. In this setting, a retroperitoneal tumor associated with a moderately elevated β-HCG is a diagnostic dilemma, and surgeons should be aware of the pitfall of a β-HCG-producing leiomyosarcoma in the differential diagnosis. PMID:18521299

  16. Kaposi sarcoma

    MedlinePlus

    ... HIV, a weakened immune system, and the human herpesvirus-8 (HHV-8). Kaposi sarcoma has been linked ... on foot References Kaye KM. Kaposi's sarcoma-associated herpesvirus (human herpesvirus type 8). In: Mandell GL, Bennett ...

  17. Ewing sarcoma

    MedlinePlus

    Bone cancer - Ewing sarcoma; Ewing family of tumors; Primitive neuroectodermal tumors (PNET); Bone neoplasm - Ewing sarcoma ... NCCN clinical practice guidelines in oncology (NCCN guidelines): bone cancer. Updated 2016. www.nccn.org/professionals/physician_gls/ ...

  18. [Primary pulmonary sarcomas].

    PubMed

    Jakubcová, T; Jakubec, P

    2009-01-01

    Primary pulmonary sarcomas are rare diseases unlike lung carcinomas. The occurence of these sarcomas is between 0.013-0.40% of all malignant lung tumours. There are malignant mesenchymal tumours. They are flowing from the soft tissue of lung. The pulmonary sarcomas are heterogenic group with various biological behaviour. Their morfologic structure does not digger from the sarcomas of soft tissue. The primary pulmonary sarcomas occur more often in childhood and in young people unlike lung carcinomas. Radiation and some toxic substances are noted risk factors. Some gene mutations, infectious pathoghens and contraception have a possible impact on the origin of some types of the sarcomas. The current hypothesis is, that most of the sarcomas, if not all sarcomas, stem from primitive multipotent mesenchymal cell by malignant transformation in one or more lines. The diagnostic standard is biopsy from tumour with histologic and immunohistochemistry examination of a sample. The basic diagnostic problem is exclusion of a secondary origin of sarcomatic cells in the lung, because pulmonary metastasis of extrapulmonary sarcomas are more often than the primary pulmonary involvement.The optimal treatment is a resection of the tumour.The other therapeutic modalities are radiotherapy and chemotherapy, but results of these modalities are unsatisfactory. There are various chemotherapeutic regimes, monotherapy or combination regimes. The basic cytostatics are doxorubicine, iphosphamide, dacarbazine. Problems of the chemotherapy are high toxicity and relatively low curative effect about 20%.The first studies with biological treatment of the sarcomas of soft tissue have been published recently.This types of drugs could be a part of the complex management of these primary pulmonary tumours in the future. The primary pulmonary sarcomas have mostly aggresive course and often recur. Their prognosis is usually not very good. The survival median is 48 months and 5-years survival ranges

  19. Primary Retroperitoneal Myxoid Liposarcomas.

    PubMed

    Setsu, Nokitaka; Miyake, Mototaka; Wakai, Susumu; Nakatani, Fumihiko; Kobayashi, Eisuke; Chuman, Hirokazu; Hiraoka, Nobuyoshi; Kawai, Akira; Yoshida, Akihiko

    2016-09-01

    Myxoid liposarcomas (MLSs) are genetically defined by the presence of DDIT3 gene fusions and most commonly arise in the extremities of young adults. Whether MLSs develop primarily in the retroperitoneum is controversial, and a recent retrospective study found no molecularly confirmed examples. Because MLSs tend to metastasize to deep soft tissues, purported examples of primary retroperitoneal lesions might represent distant metastasis, most commonly from extremities. In addition, well-differentiated or dedifferentiated liposarcomas, which are characterized by MDM2 amplifications, may exhibit prominent myxoid changes and mimic MLSs. Here, we document 5 cases of MLSs that originated in the retroperitoneum that were identified through critical clinicopathologic reevaluation. These cases accounted for 2.3% of 214 primary retroperitoneal liposarcomas and 3.2% of 156 MLSs in our database. They occurred in 3 men and 2 women with a median age of 32 years. All tumors were localized to the retroperitoneum at presentation, and no patient developed extra-abdominal recurrences during the clinical course (median, 50 mo). All 5 cases exhibited at least focal classic histologic findings. All harbored DDIT3 gene rearrangements, and none harbored MDM2 amplifications according to fluorescence in situ hybridization. This study demonstrates that primary MLSs can occur in the retroperitoneum, albeit rarely, and can be accurately diagnosed through combined clinicopathologic and molecular analysis. PMID:27158758

  20. 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

  1. 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

  2. [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. PMID:18492425

  3. 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

  4. Retroperitoneal necrotizing fasciitis.

    PubMed

    Woodburn, K R; Ramsay, G; Gillespie, G; Miller, D F

    1992-04-01

    Necrotizing fasciitis is a mixed infection of the skin and subcutaneous tissues with a characteristic clinical and pathological appearance. Early radical surgical excision of all affected tissue is the treatment of choice. In a series of 19 patients with necrotizing fasciitis, bacteriological assessment in 15 confirmed the mixed nature of the infection, with Bacteroides sp. isolated from ten patients. All 12 patients who underwent radical surgical excision survived. A subgroup of patients was identified in whom the appearance of necrotizing fasciitis in the abdomen or perineum was indicative of more extensive disease in the retroperitoneal tissues. Surgical resection of all affected tissue was not feasible in these cases and the outcome was uniformly fatal, giving an overall mortality rate for the series of 37 per cent. PMID:1576505

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

    PubMed

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

    2016-04-01

    Ewing's sarcoma is a highly malignant tumour of osseous or non-osseous origin, tremed as extra-skeletal Ewings sarcoma if arising from soft tissue. It is rare occurrence tumor most commonly occurring in paravertebral area, chest wall, head & neck and retroperitoneum. Reporting an interesting case of retroperitoneal Ewing's sarcoma in 39 years old female. Patient had complains of abdominal discomfort & vague pain since 2 months, following weakness in lower limb and loss of weight. On detail history and examination she was further referred to detail pathological and radiological investigations. Haematological profile, renal function test and liver function test were in normal limits. USG abdomen was normal, MRI showed a mass in pelvis retroperitoneum measuring 10x10cms, bilateral ovaries and tubes were normal. Because of retroperitoneal nature of tumor and suspicion of uterine sarcoma, laparotomy was performed. The large retroperitoneal mass adherent to posterior of uterus was excised and send for histopathological diagnosis. On gross and microscopy examination the diagnosis of blue cell tumor with PAS positivity, possibility of extraskeletal Ewing's sarcoma/primitive neuro-ectodermal tumor was made which was further confirmed by immunohistochemistry, positive for S100, Vementin and CD99 and negative for desmin and CK. Confirmed diagnosis help in accurate management and improves survival rate. PMID:27190820

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

    PubMed Central

    Karigoudar, Mahesh H; Palur, Katyayani

    2016-01-01

    Ewing’s sarcoma is a highly malignant tumour of osseous or non-osseous origin, tremed as extra-skeletal Ewings sarcoma if arising from soft tissue. It is rare occurrence tumor most commonly occurring in paravertebral area, chest wall, head & neck and retroperitoneum. Reporting an interesting case of retroperitoneal Ewing’s sarcoma in 39 years old female. Patient had complains of abdominal discomfort & vague pain since 2 months, following weakness in lower limb and loss of weight. On detail history and examination she was further referred to detail pathological and radiological investigations. Haematological profile, renal function test and liver function test were in normal limits. USG abdomen was normal, MRI showed a mass in pelvis retroperitoneum measuring 10x10cms, bilateral ovaries and tubes were normal. Because of retroperitoneal nature of tumor and suspicion of uterine sarcoma, laparotomy was performed. The large retroperitoneal mass adherent to posterior of uterus was excised and send for histopathological diagnosis. On gross and microscopy examination the diagnosis of blue cell tumor with PAS positivity, possibility of extraskeletal Ewing’s sarcoma/primitive neuro-ectodermal tumor was made which was further confirmed by immunohistochemistry, positive for S100, Vementin and CD99 and negative for desmin and CK. Confirmed diagnosis help in accurate management and improves survival rate. PMID:27190820

  7. 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

  8. Idiopathic Retroperitoneal Fibrosis.

    PubMed

    Vaglio, Augusto; Maritati, Federica

    2016-07-01

    Idiopathic retroperitoneal fibrosis (RPF), reviewed herein, is a rare fibro-inflammatory disease that develops around the abdominal aorta and the iliac arteries, and spreads into the adjacent retroperitoneum, where it frequently causes ureteral obstruction and renal failure. The clinical phenotype of RPF is complex, because it can be associated with fibro-inflammatory disorders involving other organs, is considered part of the spectrum of IgG4-related disease, and often arises in patients with other autoimmune conditions. Obstructive uropathy is the most common complication, although other types of renal involvement may occur, including stenosis of the renal arteries and veins, renal atrophy, and different types of associated GN. Environmental and genetic factors contribute to disease susceptibility, whereas the immunopathogenesis of RPF is mediated by different immune cell types that eventually promote fibroblast activation. The diagnosis is made on the basis of computed tomography or magnetic resonance imaging, and positron emission tomography is a useful tool in disease staging and follow-up. Treatment of idiopathic RPF aims at relieving ureteral obstruction and inducing disease regression, and includes the use of glucocorticoids, combined or not with other traditional immunosuppressants. However, biologic therapies such as the B cell-depleting agent rituximab are emerging as potentially efficacious agents in difficult-to-treat cases. PMID:26860343

  9. Radiation Therapy for Soft Tissue Sarcoma: Indications and Controversies for Neoadjuvant Therapy, Adjuvant Therapy, Intraoperative Radiation Therapy, and Brachytherapy.

    PubMed

    Larrier, Nicole A; Czito, Brian G; Kirsch, David G

    2016-10-01

    Soft tissue sarcomas are rare mesenchymal cancers that pose a treatment challenge. Although small superficial soft tissue sarcomas can be managed by surgery alone, adjuvant radiotherapy in addition to limb-sparing surgery substantially increases local control of extremity sarcomas. Compared with postoperative radiotherapy, preoperative radiotherapy doubles the risk of a wound complication, but decreases the risk for late effects, which are generally irreversible. For retroperitoneal sarcomas, intraoperative radiotherapy can be used to safely escalate the radiation dose to the tumor bed. Patients with newly diagnosed sarcoma should be evaluated before surgery by a multidisciplinary team that includes a radiation oncologist. PMID:27591502

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

    PubMed Central

    2011-01-01

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

  11. A Primary Retroperitoneal Mucinous Tumor

    PubMed Central

    Heelan Gladden, Alicia A.; Wohlauer, Max; McManus, Martine C.; Gajdos, Csaba

    2015-01-01

    A twenty-five-year-old female presented with a large retroperitoneal mass. Workup included history and physical exam, imaging, biopsy, colonoscopy, and gynecologic exam. After surgical resection, the mass was determined to be a primary retroperitoneal mucinous tumor (PRMT). Clinically and histologically, these tumors are similar pancreatic and ovarian mucinous neoplasms. PRMTs are rare and few case reports have been published. PRMTs are divided into mucinous cystadenomas, mucinous borderline tumors of low malignant potential, and mucinous carcinoma. These tumors have malignant potential so resection is indicated and in some cases adjuvant chemotherapy and/or surveillance imaging. PMID:25874152

  12. [A case of retroperitoneal hemangiopericytoma].

    PubMed

    Kura, N; Kihara, K; Masuda, H; Tsujii, T; Oshima, H

    1999-01-01

    We report a case of a huge retroperitoneal tumor in a 67-year-old woman. When the patient was taken to another hospital by ambulance, she had lost consciousness because of hypertension and hypoglycemia and abdominal CT revealed a huge retroperitoneal tumor with deviation of the right kidney and inferior vena cava. After further examinations including ultrasonography, MRI and angiography in our hospital, the tumor was extirpated. The tumor, 22 x 17 x 10 cm in size and 2,580 g in weight was diagnosed as hemangiopericytoma histologically. She has remained well with no evidence of recurrence for 9 months since the operation. PMID:10086266

  13. Uterine sarcoma

    MedlinePlus

    ... cancer called retinoblastoma also increases the risk of uterine sarcoma. Symptoms Fibroids in the uterus are a common problem in women. Common symptoms of fibroids include abnormal uterine bleeding, pelvic pain and pressure, and a pelvic ...

  14. Kaposi's Sarcoma

    MedlinePlus

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

  15. 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.

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

    PubMed Central

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

    2014-01-01

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

  17. The Evolution and Technique of Nerve-Sparing Retroperitoneal Lymphadenectomy.

    PubMed

    Masterson, Timothy A; Cary, Clint; Rice, Kevin R; Foster, Richard S

    2015-08-01

    The evolution of retroperitoneal lymph node dissection technique and associated template modifications for nonseminomatous germ cell tumors have resulted in significant improvement in the long-term morbidity. Through the preservation of sympathetic nerves via exclusion from or prospective identification within the boundaries of resection, maintenance and recovery of antegrade ejaculation are achieved. Nerve-sparing strategies in early-stage disease are feasible in most patients. Postchemotherapy, select patients can be considered for nerve preservation. This article describes the anatomic and physiologic basis for, indications and technical aspects of, and functional and oncologic outcomes reported after nerve-sparing retroperitoneal lymphadenectomy in testicular cancer. PMID:26216818

  18. 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

  19. 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.

  20. Adjuvant chemotherapy for soft-tissue sarcoma: review and meta-analysis of the published results of randomised clinical trials.

    PubMed Central

    Tierney, J. F.; Mosseri, V.; Stewart, L. A.; Souhami, R. L.; Parmar, M. K.

    1995-01-01

    Fifteen published randomised trials comparing adjuvant chemotherapy with no chemotherapy in soft-tissue sarcoma (STS) were identified (1546 patients). A qualitative review and a meta-analysis of this published literature were performed. With the qualitative review it was not possible to synthesise the apparently conflicting results of individual trials. The meta-analysis of the published data suggests an improvement in survival at 2 years (OR = 0.73, 95% CI = 0.53-0.99, P = 0.044) and at 5 years (OR = 0.59, 95% CI = 0.45-0.78, P = 0.0002) in favour of chemotherapy. However, the assumptions and approximations required to conduct this quantitative summary demand that the results are interpreted with caution. The only reliable means of assessing the current evidence on whether adjuvant chemotherapy has a role in the treatment of patients with STS, is to collect, check and reanalyse individual patients data (IPD) from each trial centrally, and formally combine the results in a stratified time-to-event analysis. Such an IPD analysis is currently being undertaken by an international collaborative group. PMID:7640234

  1. Benign Mesenchymal Stromal Cells in Human Sarcomas

    PubMed Central

    Morozov, Alexei; Downey, Robert J.; Healey, John; Moreira, Andre L.; Lou, Emil; Leung, Roland; Edgar, Mark; Singer, Samuel; LaQuaglia, Michael; Maki, Robert G.; Moore, Malcolm A.S.

    2010-01-01

    Purpose Recent evidence suggests that at least some sarcomas arise through aberrant differentiation of mesenchymal stromal cells (MSCs), but MSCs have never been isolated directly from human sarcoma specimens. Experimental Design We examined human sarcoma cell lines and primary adherent cultures derived from human sarcoma surgical samples for features of MSCs. We further characterized primary cultures as either benign or malignant by the presence of tumor-defining genetic lesions and tumor formation in immunocompromised mice. Results We show that a dedifferentiated liposarcoma cell line DDLS8817 demonstrates fat, bone and cartilage trilineage differentiation potential characteristic of MSCs. Primary sarcoma cultures have the morphology, surface immunophenotype and differentiation potential characteristic of MSCs. Surprisingly, many of these cultures are benign as they do not form tumors in mice and lack sarcoma-defining genetic lesions. Consistent with the recently proposed pericyte origin of MSCs in normal human tissues, sarcoma-derived benign MSCs express markers of pericytes and cooperate with endothelial cells in tube formation assays. In human sarcoma specimens, a subset of CD146-positive microvascular pericytes express CD105, an MSC marker, while malignant cells largely do not. In an in vitro co-culture model, sarcoma-derived benign MSCs as well as normal human pericytes markedly stimulate the growth of sarcoma cell lines. Conclusions Sarcoma-derived benign MSCs/pericytes represent a previously undescribed stromal cell type in sarcoma which may contribute to tumor formation. PMID:21138865

  2. [Morbus Ormond (idiopatic retroperitoneal fibrosis)].

    PubMed

    Michaligová, A; Plank, L; Jezíková, A; Manka, V; Makovický, P; Mokán, M

    2011-05-01

    Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by the development of fibrotic tissue around the abdominal aorta and iliac arteries and often involves structures as ureters and the inferior vena cava. The age at onset of signs and symptoms is between 40-60 years, males predominane over females. In most cases the clinical manifestation is presented as compressive syndrom of ureters, therefore the first known cases were described by urologists. In this report we present the case of 37-years old male examinated for persistent fever about 38 degrees C and high inflammatory activity in spite of empiric antibiotic therapy. Positron emission tomography (PET) showed locality of high metabolic activity of fluorodeoxyglucose with maximum paraaortal left. Microscopic examination of extracted mass showed presence of fibrous and inflammatory components. With clinical presentation, imaging and histological findings we made out the diagnosis of idiopathic retroperitoneal fibrosis--morbus Ormond. PMID:21695934

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

    PubMed Central

    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. PMID:23227427

  4. 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

  5. Retroperitoneal laparoscopic bilateral lumbar sympathectomy.

    PubMed

    Segers, B; Himpens, J; Barroy, J P

    2007-06-01

    The first retroperitoneal lumbar sympathectomy was performed in 1924 by Julio Diez. The classic procedure for sympathectomy is open surgery. We report a unilateral laparoscopic retroperitoneal approach to perform bilateral lumbar sympathectomy. This approach was performed for a 43-year-old man with distal arterial occlusive disease and no indication for direct revascularization. His predominant symptoms were intermittent claudication at 100 metres and cold legs. The patient was placed in a left lateral decubitus position. The optical system was placed first in an intra-abdominal position to check that the trocars were well positioned in the retroperitoneal space. The dissection of retroperitoneum was performed by CO2 insufflation. The inferior vena cava was reclined and the right sympathetic chain was individualized. Two ganglia (L3-L4) were removed by bipolar electro-coagulation. The aorta was isolated on a vessel loop and careful anterior traction allowed a retro-aortic pre-vertebral approach between the lumbar vessels. The left sympathetic chain was dissected. Two ganglia (L3-L4) were removed by bipolar electro-coagulation. PMID:17685269

  6. Oncolytic adenovirus and doxorubicin-based chemotherapy results in synergistic antitumor activity against soft-tissue sarcoma.

    PubMed

    Siurala, Mikko; Bramante, Simona; Vassilev, Lotta; Hirvinen, Mari; Parviainen, Suvi; Tähtinen, Siri; Guse, Kilian; Cerullo, Vincenzo; Kanerva, Anna; Kipar, Anja; Vähä-Koskela, Markus; Hemminki, Akseli

    2015-02-15

    Despite originating from several different tissues, soft-tissue sarcomas (STS) are often grouped together as they share mesenchymal origin and treatment guidelines. Also, with some exceptions, a common denominator is that when the tumor cannot be cured with surgery, the efficacy of current therapies is poor and new treatment modalities are thus needed. We have studied the combination of a capsid-modified oncolytic adenovirus CGTG-102 (Ad5/3-D24-GMCSF) with doxorubicin, with or without ifosfamide, the preferred first-line chemotherapeutic options for most types of STS. We show that CGTG-102 and doxorubicin plus ifosfamide together are able to increase cell killing of Syrian hamster STS cells over single agents, as well as upregulate immunogenic cell death markers. When tested in vivo against established STS tumors in fully immunocompetent Syrian hamsters, the combination was highly effective. CGTG-102 and doxorubicin (without ifosfamide) resulted in synergistic antitumor efficacy against human STS xenografts in comparison with single agent treatments. Doxorubicin increased adenoviral replication in human and hamster STS cells, potentially contributing to the observed therapeutic synergy. In conclusion, the preclinical data generated here support clinical translation of the combination of CGTG-102 and doxorubicin, or doxorubicin plus ifosfamide, for the treatment of STS, and provide clues on the mechanisms of synergy. PMID:24975392

  7. 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. PMID:24502401

  8. Solitary ganglioneuromatosis of the descending colon, presenting as giant retroperitoneal tumour.

    PubMed

    Mateş, In; Iosif, C; Dinu, D; Constantinoiu, S

    2013-01-01

    Ganglioneuroma (GN) is a benign neoplasia of the autonomous nervous system, colonic GN is uncommon in adults. There are three subgroups: polypoid GN, ganglioneuromatous polyposis and diffuse ganglioneuromatosis. Ganglioneuromatosis is highly-associated to neurofibromatosis type 1 (NF1) and multiple endocrine neoplasia type 2b (MEN2B). A 68-year-old female, with a discrete retarded emission of stools, was admitted for a large tumor in the left flank; CT scan, urography and barium enema demonstrated a large retroperitoneal mass, presumed as sarcoma. Open surgery discovered a 16 10 11 cm solid and encapsulated tumor, attached to the retroperitoneal descending colon, with no macroscopic mucosal involvement; the pathologic diagnosis of the resected specimen (en-bloc tumorectomy with limited colectomy) was intramural colonic ganglio-neuromatosis. Anamnesis, physical examination and complete endoscopic explorations showed no evidence of personal bearing or familial aggregation of genetic syndromes. In adults, association of transmural ganglioneuromatosis to NF1 or MEN2B is not mandatory; presentation often mimics obstructive carcinoma and positive diagnosis is provided by pathological examination of the resected specimen. In this peculiar case, the loose tissue of the retroperitoneal space favoured a slow development of intramural ganglioneuromatosis, presenting as a gigantic retroperitoneal mass with no radiological evidence of its colonic origin. PMID:23958108

  9. Laparoscopic Excision of Retroperitoneal Schwannoma

    PubMed Central

    Rajkumar, J S; Anirudh, J R; Akbar, S; Kishore, C M

    2015-01-01

    Schwannomas are tumours that arise from the myelin sheath of the nerves. A very unusual location for schwannoma is the retro peritoneal areas (less than 2%). We present herewith a patient who had a 4x5cm Schwannoma arising from the nerve root of L2 on the right side, which presented as a lump in the psoas major muscle. This was treated by total laparoscopic excision after splitting open the psoas major. In the published english medical literature we could find only 16 cases of laparoscopic resection of retroperitoneal schwannoma and we believe ours to be the first case that was done through a psoas muscle split technique. Technical and histopathological details are discussed elaborately in this article. PMID:26676094

  10. Retroperitoneal Cyst: An Uncommon Presentation of Filariasis

    PubMed Central

    Ganesan, Senthil; Galodha, Saurabh; Saxena, Rajan

    2015-01-01

    Primary retroperitoneal parasitic cysts are rare. Here we report about a middle aged male patient from rural north India with a recent onset of central abdominal retroperitoneal lump, pain, and fever. After surgical resection due to diagnostic uncertainty, at histopathology, it turned out be a filarial cyst. After receiving a course of diethylcarbamazine, the patient is asymptomatic at 4 months' follow-up. PMID:26664759

  11. Retroperitoneal well-differentiated inflammatory liposarcoma: a diagnostic dilemma.

    PubMed

    Kawano, Rinsaku; Nishie, Akihiro; Yoshimitsu, Kengo; Irie, Hiroyuki; Tajima, Tsuyoshi; Hirakawa, Masakazu; Ishigami, Kousei; Ushijima, Yasuhiro; Okamoto, Daisuke; Yabuuchi, Hidetake; Taketomi, Akinobu; Nishihara, Yunosuke; Fujita, Nobuhiro; Honda, Hiroshi

    2008-08-01

    We present a case of retroperitoneal well-differentiated inflammatory liposarcoma that was extremely difficult to diagnose preoperatively. Computed tomography and magnetic resonance images showed a 5-cm homogeneous soft-tissue mass with a decreased apparent diffusion coefficient and without fat component in the retroperitoneum. Minimal fat stranding was detected around the mass. The preoperative working diagnosis was malignant lymphoma or inflammatory pseudotumor, whereas the final diagnosis after surgery was well-differentiated inflammatory liposarcoma. As a result, only a large component of lymphoid infiltration was recognized as a tumor preoperatively, and minimal fat stranding represented a component of lipoma-like liposarcoma. In this entity, a lipomatous component could easily be missed on radiologic imaging because of the conspicuity of lymphoid infiltration. We should consider the possibility of this variant when we evaluate a retroperitoneal tumor. PMID:18770006

  12. [Cytogenetics of bone sarcomas].

    PubMed

    Vagner-Capodano, A M; Poitout, D

    There has been much progress in the cytogenesis, and molecular biology of bone tumours such as Ewing sarcoma and osteosarcomas, greatly improving diagnostic possibilities and prognosis. Ewing's sarcoma is an indifferentiated sarcoma with round cells which usually occurs in children or adolescents. Ewing's sarcoma corresponds to 6% of all bone tumours. Histologically Ewing's sarcoma belongs to a group of small round cell tumours including neuroblastoma, embryon and alveolar rhabdomyosarcoma and non-Hodgkin's lymphoma. Differential diagnosis is difficult. Cytogenetic examinations can now differentiate Ewing's sarcoma from other small round cell tumours. There is a specific 11:12 translocation (q24; q12) which can be used as a marker. PMID:8785922

  13. Non-metastatic unresected paediatric non-rhabdomyosarcoma soft tissue sarcomas: Results of a pooled analysis from United States and European groups

    PubMed Central

    Ferrari, Andrea; Miceli, Rosalba; Rey, Annie; Oberlin, Odile; Orbach, Daniel; Brennan, Bernadette; Mariani, Luigi; Carli, Modesto; Bisogno, Gianni; Cecchetto, Giovanni; De Salvo, Gian Luca; Casanova, Michela; Vannoesel, Max M.; Kelsey, Anna; Stevens, Michael C.; Devidas, Meenakshi; Pappo, Alberto S.; Spunt, Sheri L.

    2012-01-01

    Background Non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) with initially unresected tumours represent a particular subset of patients with a poor outcome. Various international research groups pooled their data in a joint study in order to investigate prognostic variables and treatment modalities. Methods The study population consisted of 304 patients <21 years old treated between 1980 and 2005 using a multimodality therapeutic strategy. Results Synovial sarcoma and malignant peripheral nerve sheath tumour (MPNST) were the most frequent histotypes. Most patients received initial chemotherapy: major responses were recorded in 41% and minor in 16% of cases. Overall survival (OS) was 60.0% and 51.5% at 5 and 10 years, respectively, and it was significantly associated with patient's age, histological subtype, tumour site and size, quality of delayed surgical resection, radiotherapy administration and response to induction chemotherapy. MPNST associated to neurofibromatosis type 1 was the tumour type with the worst rate of response to chemotherapy and the worst outcome. Conclusions In unresected NRSTS patients, radiotherapy and delayed surgery are of crucial importance. Patients who respond to chemotherapy have better chance of survival. However, given the relatively poor prognosis, research on intensive multimodal treatment approaches and novel strategies is warranted. PMID:21145727

  14. Retroperitoneal Laparoscopic Management of Paraganglioma: A Single Institute Experience

    PubMed Central

    Xu, Weifeng; Li, Hanzhong; Ji, Zhigang; Yan, Weigang; Zhang, Yushi; Zhang, Xuebin; Li, Qian

    2016-01-01

    Objectives To explore the feasibility and safety of retroperitoneal laparoscopic resection of paraganglioma (RLPG) in a large study population. Methods In a six-year period, 49 patients with primary retroperitoneal paragangliomas (PG) underwent retroperitoneal laparoscopic surgery in a single center. Medical records were reviewed, and collected the following data, which were clinical characteristics, perioperative data (operative time, estimated blood loss, intraoperative hemodynamic changes, intraoperative and postoperative complications, and open conversions), and follow-up data (recurrence or distant metastases). Results All PGs were removed with negative tumor margin confirmed by postoperative histopathology. The operative time of RLPG was 101.59±31.12 minutes, and the estimated blood loss was 169.78±176.70ml. Intraoperative hypertensive and hypotensive episodes occurred in 25 cases and 27 cases, respectively. Two open conversions occurred. Two intraoperative complications occurred but were successfully managed endoscopically. Postoperative complications were minor and unremarkable. No local recurrence or distant metastasis were observed during the follow-up period. Conclusions Our experience indicates the feasibility and safety of resection of PGs in a relatively large study population. PMID:26885838

  15. 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

  16. 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

  17. 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. PMID:26175874

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

    PubMed

    Karim, Lawen; Larkin, David; Sadat, Mohamed

    2016-01-01

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

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

    PubMed Central

    Karim, Lawen; Larkin, David; Sadat, Mohamed

    2016-01-01

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

  20. Pelvic Retroperitoneal Cellular Leiomyoma: A Case Report.

    PubMed

    Tantitamit, Tanitra; Hamontri, Suttha; Rangsiratanakul, Likit; Suksamarnwong, Maysita

    2015-10-01

    Leiomyomas are common benign gynecological tumors and usually arise in the uterus. The retroperitoneal cellular leiomyoma, one of the unusual manifestations, is a rare tumor. Diagnosis and treatment are challenges. We report a case of 65-year-old women presented with an asymptomatic mass beneath the right posterior vaginal mucosa. CT imaging revealed heterogeneous mass 6 cm in the pelvic cavity abutted lower segment of uterus, cervix, and vagina. The provisional diagnosis was subserosal cervical leiomyoma. She underwent exploratory laparotomy. Intra-operative, a normal size uterus was found separately from retroperitoneal pelvic mass at the level of internal os. Histological report confirmed cellular leiomyoma later Total hysterectomy, bilateral salpingoophorectomy and completely excision of tumor were achieved with good outcome. Our patient represents the rare case of retroperitoneal cellular leiomyoma, which is hardly identified from internal examination and preoperative imaging. Surgical removal is essential for pathological diagnosis and treatment. PMID:26817226

  1. Isolated retroperitoneal hydatid cyst invading splenic hilum.

    PubMed

    Ozturk, Safak; Unver, Mutlu; Kibar Ozturk, Burcin; Kebapci, Eyup; Bozbiyik, Osman; Erol, Varlık; Zalluhoglu, Nihat; Olmez, Mustafa

    2014-01-01

    Introduction. Hydatid disease (HD) is an infestation that is caused by the larval stage of Echinococcus granulosus. The liver is affected in approximately two-thirds of patients, the lungs in 25%, and other organs in a small proportion. Primary retroperitoneal hydatid cyst is extremely rare. The most common complaint is abdominal pain; however, the clinical features of HD may be generally dependent on the location of the cyst. Case Presentation. A 43-year-old female was admitted with the complaint of abdominal pain. Her physical examination was normal. Computed tomography (CT) revealed a 17 × 11 cm cystic lesion, with a thick and smooth wall that is located among the left liver lobe, diaphragm, spleen, tail of the pancreas, and transverse colon and invading the splenic hilum. Total cystectomy and splenectomy were performed. Pathological examination was reported as cyst hydatid. Discussion. Cysts in the peritoneal cavity are mainly the result of the spontaneous or traumatic rupture of concomitant hepatic cysts or surgical inoculation of a hepatic cyst. Serological tests contribute to diagnosis. In symptomatic and large hydatid peritoneal cysts, surgical resection is the only curative treatment. Total cystectomy is the gold standard. Albendazole or praziquantel is indicated for inoperable and disseminated cases. Percutaneous aspiration, injection, and reaspiration (PAIR) technique is another nonsurgical option. PMID:24790764

  2. Isolated Retroperitoneal Hydatid Cyst Invading Splenic Hilum

    PubMed Central

    Ozturk, Safak; Unver, Mutlu; Kibar Ozturk, Burcin; Kebapci, Eyup; Bozbiyik, Osman; Erol, Varlık; Zalluhoglu, Nihat; Olmez, Mustafa

    2014-01-01

    Introduction. Hydatid disease (HD) is an infestation that is caused by the larval stage of Echinococcus granulosus. The liver is affected in approximately two-thirds of patients, the lungs in 25%, and other organs in a small proportion. Primary retroperitoneal hydatid cyst is extremely rare. The most common complaint is abdominal pain; however, the clinical features of HD may be generally dependent on the location of the cyst. Case Presentation. A 43-year-old female was admitted with the complaint of abdominal pain. Her physical examination was normal. Computed tomography (CT) revealed a 17 × 11 cm cystic lesion, with a thick and smooth wall that is located among the left liver lobe, diaphragm, spleen, tail of the pancreas, and transverse colon and invading the splenic hilum. Total cystectomy and splenectomy were performed. Pathological examination was reported as cyst hydatid. Discussion. Cysts in the peritoneal cavity are mainly the result of the spontaneous or traumatic rupture of concomitant hepatic cysts or surgical inoculation of a hepatic cyst. Serological tests contribute to diagnosis. In symptomatic and large hydatid peritoneal cysts, surgical resection is the only curative treatment. Total cystectomy is the gold standard. Albendazole or praziquantel is indicated for inoperable and disseminated cases. Percutaneous aspiration, injection, and reaspiration (PAIR) technique is another nonsurgical option. PMID:24790764

  3. Diagnosis and surgical treatment of retroperitoneal tumours.

    PubMed

    Testini, M; Catalano, G; Macarini, L; Paccione, F

    1996-01-01

    The authors examine the various techniques for diagnosing Retroperitoneal Tumours (RPT) and analyse the results of the surgical treatment performed. Between March 1987 and February 1991, 20 patients with RPT (6 benign and 14 malignant forms) were observed in our Institution. CT and NMR revealed more diagnostic accuracy than other techniques (100%), while NMR had greater accuracy than CT in predictly resectability preoperatively (100% vs. 80.0%, respectively). A total of 26 laparotomies were performed: 20 for primary neoplasms and 6 for recurrent tumours. Exeresis of the mass was performed in 18/20 (90%) patients. Mean follow-up was 57.6 months (84-36). The benign forms had no recurrence. In malignant cases the disease recurred in 58.3% of the cases after an interval varying from 10 to 59 months. Overall mean survival of the 12 patients with malignant tumours subjected to resection was 58.3%. The 1- and 3-year survival rates were 91.7% and 58.3% respectively. Prognosis in malignant RPT is still very poor. PMID:8803715

  4. 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

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

    PubMed Central

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

    2013-01-01

    Background: Retroperitoneal tumors constitute a difficult diagnostic category as they are not easily accessible. The advent of image-guided fine-needle aspiration (FNA) has resolved this problem significantly. Aims: We present a short study based on guided aspiration of retroperitoneal tumors, in which we have tried to assess the role of image-guided fine-needle aspiration cytology as a tool for pre-operative diagnosis. Materials and Methods: The study was conducted on patients diagnosed with retroperitoneal masses. FNA was performed under image guidance with the help of ultrasonography and/or computed tomography; smears were prepared and meticulously screened according to a fixed protocol. The results were analyzed to determine sensitivity, specificity, and diagnostic efficacy of cytopathological diagnosis using image-guided FNA techniques. Results: We assessed 38 patients with retroperitoneal masses. In all cases, adequate cellular material was obtained. No major complications were encountered. Statistical analysis was carried out in 35 cases; sensitivity, specificity, and diagnostic accuracy were 100% in these cases. Conclusion: FNA under image guidance should be considered a first-line diagnostic approach for retroperitoneal and other abdominal tumors, although caution should be exercised in case selection. In areas where advanced tests are not available, the cytotechnologist and cytopathologist have a very important role to play in ensuring accurate diagnoses. PMID:23661939

  6. 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

  7. 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. PMID:21966332

  8. [Extrarenal Retroperitoneal Angiomyolipoma Masquerading as Retroperitoneal Liposarcoma : A Report of Two Cases].

    PubMed

    Yamamoto, Ryohei; Inoue, Takamitsu; Numakura, Kazuyuki; Tsuruta, Hiroshi; Maeno, Atsushi; Saito, Mitsuru; Narita, Shintaro; Tsuchiya, Norihiko; Satoh, Shigeru; Habuchi, Tomonori

    2016-06-01

    We report two patients with extrarenal retroperitoneal angiomyolipoma masquerading as perinephric liposarcoma. Patient 1 : A 66-year-old man was diagnosed with a retroperitoneal tumor near the right renal hilum on an abdominal computed tomography (CT) performed before surgery for gastric cancer. A diagnosis of extrarenal retroperitoneal angiomyolipoma was made on the basis of negative uptake of fluorine- 18 2-deoxy-2-fluoro-D-glucose positron emission tomography (18F-FDG PET)/CT. However, because the tumor was found to have gradually enlarged at 18 months afterward, he underwent resection of the extrarenal fat tissue together with the right kidney. Patient 2 : A 56-year-old man underwent abdominal ultrasound during a periodic medical examination, which revealed a right retroperitoneal tumor. Because of the findings in the contrast-enhanced CT and positive uptake of 18F-FDG PET/CT, he underwent resection of the extrarenal fat tissue together with the right kidney. The pathological examination of the two tumors confirmed extrarenal angiomyolipoma. The differential diagnosis of extrarenal retroperitoneal angiomyolipoma from retroperitoneal liposarcoma is difficult even with the use of 18F-FDG PET/CT. PMID:27452495

  9. A Giant Retroperitoneal Lymphangioma: A Case Report

    PubMed Central

    Hubli, Prabhu; Sachin, Basavaraju M

    2016-01-01

    Lymphangioma is a congenital lesion of childhood. They usually present in head and neck region. Intra-abdominal lymphangioma in an adult is a rare lesion which poses diagnostic difficulty. Although asymptomatic, they may present with an acute abdomen. Here, we report a rare case of huge asymptomatic retro-peritoneal lymphangioma who underwent complete surgical excision with an uneventful postoperative period.

  10. 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.

  11. Spontaneous retroperitoneal haemorrhage in a young adult

    PubMed Central

    Baksi, Aditya; Gupta, Shahana; Ray, Udipta; Ghosh, Shibajyoti

    2014-01-01

    We report a rare case of a primary adrenal cortical malignancy presenting with spontaneous retroperitoneal haemorrhage in a young adult. To the best of our knowledge, this is the thirteenth such case to be reported in the English literature. PMID:24658522

  12. [Retroperitoneal nodular fasciitis: analysis of a case].

    PubMed

    Fabbro, M A; Costa, L; Cimaglia, M L; Donadio, P; Spata, E; Dante, S

    1995-01-01

    A case of nodular fasciitis occurring in a 14-year-old girl is described and the clinical-pathological profile and the diagnostic problems are analyzed. The Authors believe this report useful to be reported for the relative rarity of the disease, especially in children, the very great dimension and the unusual retroperitoneal localization. PMID:8685002

  13. 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. PMID:26827688

  14. Can Kaposi Sarcoma Be Prevented?

    MedlinePlus

    ... early? Can Kaposi sarcoma be prevented? Kaposi sarcoma (KS) is caused by the Kaposi sarcoma associated herpesvirus ( ... to protect people against KSHV. For now, preventing KS depends on reducing the chance of becoming infected ...

  15. 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.

  16. 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.

  17. 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

  18. Retroperitoneal Castleman's disease mimicking soft tissue tumour.

    PubMed

    Pandya, B; Ghosh, S K; Chude, G; Rajmohan, M V; Narang, R

    2007-08-01

    Castleman's disease is a type of non-neoplastic lymphoproliferative disease having lymph nodal hyperplasia. It has two distinct microscopic types: hyaline-vascular type and plasma cell type. Clinically, it may present either as a solitary mass, most commonly in the mediastinum, or as a multicentric form whose features are generalized lymph-adenopathy, splenomegaly and involvement of other organs like the lungs and kidneys. Here we report a case of isolated retroperitoneal Castleman's disease, which presented as a lump in the iliac fossa in a young female. A clinico-radio-logical diagnosis of retroperitoneal soft tissue tumour was made and the patient underwent complete surgical excision. The exact diagnosis was only obtained at histopathology and there is no evidence of recurrence at six months follow-up. PMID:23132970

  19. A randomized, double-blind, placebo-controlled, Phase III study of pazopanib in patients with soft tissue sarcoma: results from the Japanese subgroup

    PubMed Central

    Kawai, Akira; Araki, Nobuhito; Hiraga, Hiroaki; Sugiura, Hideshi; Matsumine, Akihiko; Ozaki, Toshifumi; Ueda, Takafumi; Ishii, Takeshi; Esaki, Taito; Machida, Michiko; Fukasawa, Nobuaki

    2016-01-01

    Objective This analysis of the Japanese subpopulation of the PALETTE Phase III, randomized, placebo-controlled study investigated efficacy and safety of pazopanib in patients with metastatic soft tissue sarcoma after failure of standard chemotherapy. Methods Patients were randomly assigned in a 2:1 ratio to receive either pazopanib 800 mg once daily or placebo, with no subsequent cross-over. Primary endpoint was progression-free survival. Secondary endpoints included overall survival and overall response rate. Efficacy analysis was by intent-to-treat. Safety was also investigated. Results Forty-seven patients received either pazopanib (n = 31) or placebo (n = 16). Median progression-free survival was 7.0 weeks (95% confidence interval: 4.0–11.7) for placebo and 24.7 weeks (95% confidence interval: 8.6–28.1) for pazopanib (hazard ratio = 0.41 [95% confidence interval: 0.19–0.90]; P = 0.002). Median overall survival was 14.9 months (95% confidence interval: 6.8—not calculable) for placebo and 15.4 months (95% confidence interval: 7.9–28.8) for pazopanib (hazard ratio = 0.87 [95% confidence interval: 0.41–1.83]; P = 0.687). More patients receiving pazopanib experienced best response of stable disease versus placebo. Adverse events were similar to the global population; those leading to dose reduction were more common and mean daily dose was lower in the Japanese population versus the global population (45 vs. 32% and 624.4 vs. 700.4 mg, respectively). Conclusions The efficacy and safety of pazopanib observed in the Japanese subpopulation of PALETTE were similar to those in the global population. Pazopanib is a new treatment option for Japanese patients with metastatic non-adipocytic soft tissue sarcoma after chemotherapy. Clinical trial Registration number NCT00753688; GSK study ID: VEG110727; http://www.gsk-clinicalstudyregister.com/study/VEG110727#ps. PMID:26864131

  20. Infected retroperitoneal cystic lymphangioma masquerading as psoas abscess.

    PubMed

    Pratap, Akshay; Tiwari, Awadhesh; Sah, Birendra Prasad; Sinha, Arvind Kumar; Shakya, Vikal Chandra; Niels, Koopmans G

    2008-01-01

    A 2-year-old male is described who presented with fever, fixed flexion deformity of the right hip and a tender mass in the right iliac fossa. A clinical diagnosis of psoas abscess was made. Abdominal ultrasound was suggestive of an echogenic retroperitoneal cyst but could not accurately determine the nature of the cyst. A contrast CT scan showed a retroperitoneal cyst. At laparotomy, a large retroperitoneal cyst containing turbid fluid was found and was excised completely. Histopathological examination was suggestive of cystic lymphangioma. Clinical presentation of retroperitoneal lymphangioma may be variable and misleading, therefore complex imaging studies are necessary in the evaluation of this condition. PMID:18480641

  1. Preliminary Results From a Prospective Study Using Limited Margin Radiotherapy in Pediatric and Young Adult Patients With High-Grade Nonrhabdomyosarcoma Soft-Tissue Sarcoma

    SciTech Connect

    Krasin, Matthew J.; Davidoff, Andrew M.; Xiong Xiaoping; Wu Shengjie; Hua, C.-H.; Navid, Fariba; Rodriguez-Galindo, Carlos; Rao, Bhaskar N.; Hoth, Kelly A.; Neel, Michael D.; Merchant, Thomas E.; Kun, Larry E.; Spunt, Sheri L.

    2010-03-01

    Purpose: To demonstrate the safety and efficacy of limited margin radiotherapy in the local control of pediatric and young adult patients with high-grade nonrhabdomyosarcoma soft tissue sarcoma (NRSTS). Methods and Materials: Pediatric patients with high-grade NRSTS requiring radiation were treated on an institutional review board approved prospective institutional study of conformal/intensity-modulated/interstitial brachytherapy using a 2-cm anatomically constrained margin. Results: A total of 32 patients (median age, 15.3 years; range, 2-22 years) received adjuvant (27 patients) or definitive (5 patients) irradiation. With a median follow-up of 32 months, the 3-year cumulative incidence of local failure was 3.7% for patients undergoing irradiation after surgical resection. Four patients experienced local failure; the mean dose to the volume of recurrence was >=97% of the prescribed dose. Conclusions: Delivery of limited margin radiotherapy using external beam or brachytherapy provides a high rate of local tumor control without marginal failure. Further follow-up is required to determine whether normal tissue effects are minimized using this approach.

  2. Trial of Dasatinib in Advanced Sarcomas

    ClinicalTrials.gov

    2014-12-17

    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)

  3. Retroperitoneal fluid collection following anterior spine surgery--differential and management.

    PubMed

    VanValkenburg, Scott; Trussell, J C; Lavelle, William F

    2016-04-01

    Iatrogenic ureteral injuries are rare and must be accurately identified to minimizing the risk for additional complications. Anterior lumbar interbody fusion (ALIF) is a valuable technique utilized in spine surgery, with its own unique set of complications. For example, retroperitoneal fluid collections, following ALIF surgery are rare and may result in back pain, radicular pain, nausea, and even death. It is important to rapidly identify the nature of the fluid collection to clarify appropriate management options. The purpose of this case report is to present a differential diagnosis for a delayed presentation of an extremely large retroperitoneal fluid collection following anterior lumbar surgery, as well as to provide discussion on this rare complication. Specifically, a 51-year-old female with a history of numerous previous abdominal surgeries underwent an L3-S1 ALIF through a paramedian retroperitoneal approach. Postoperatively, she developed a large retroperitoneal fluid collection heralded by unilateral left lower extremity swelling and paresthesias. Fluid aspiration suggested a urine leak, but no specific injury was identified on retrograde pyelogram, most likely due to hardware obscuration in the area of presumed injury. A presumptive ureteral injury resulted in a ureteral stent placement, with resolution of the fluid collection and hydronephrosis. A high index of suspicion allowed for proper treatment, healing, and ultimately, a satisfactory outcome. PMID:27085832

  4. Application of Proteomics to Soft Tissue Sarcomas

    PubMed Central

    Kondo, Tadashi; Kubota, Daisuke; Kawai, Akira

    2012-01-01

    Soft tissue sarcomas are rare and account for less than 1% of all malignant cancers. Other than development of intensive therapies, the clinical outcome of patients with soft tissue sarcoma remains very poor, particularly when diagnosed at a late stage. Unique mutations have been associated with certain soft tissue sarcomas, but their etiologies remain unknown. The proteome is a functional translation of a genome, which directly regulates the malignant features of tumors. Thus, proteomics is a promising approach for investigating soft tissue sarcomas. Various proteomic approaches and clinical materials have been used to address clinical and biological issues, including biomarker development, molecular target identification, and study of disease mechanisms. Several cancer-associated proteins have been identified using conventional technologies such as 2D-PAGE, mass spectrometry, and array technology. The functional backgrounds of proteins identified were assessed extensively using in vitro experiments, thus supporting expression analysis. These observations demonstrate the applicability of proteomics to soft tissue sarcoma studies. However, the sample size in each study was insufficient to allow conclusive results. Given the low frequency of soft tissue sarcomas, multi-institutional collaborations are required to validate the results of proteomic approaches. PMID:22778956

  5. Retroperitoneal Laparoscopic Nephroureterectomy for Tuberculous Nonfunctioning Kidneys: a single-center experience

    PubMed Central

    Tian, Xiquan; Wang, Mingshuai; Niu, Yinong; Zhang, Junhui; Song, Liming; Xing, Nianzeng

    2015-01-01

    Purpose To present our surgical techniques and experiences of retroperitoneal laparoscopic nephroureterectomy for the treatment of tuberculous nonfunctioning kidneys. Materials and Methods From March 2005 to March 2013, a total of 51 patients with tuberculous nonfunctioning kidney underwent retroperitoneal laparoscopic nephroureterectomy at our medical center. The techniques included early control of renal vessels and dissection of the diseased kidney along the underlying layer outside the Gerato’s fascia. The distal ureter was dissected through a Gibson incision and the entire specimen was removed en bloc from the incision. Patient demographics, perioperative characteristics and laboratory parameters as well as postoperative outcome were retrospectively reviewed. Results Retroperitoneal laparoscopic nephroureterectomy was successfully performed in 50 patients, whereas one case required conversion to open surgery due to non-progression of dissection. The mean operating time was 123.0 minutes (107-160 minutes) and the mean estimated blood loss was 134 mL (80-650 mL).The mean postoperative hospital stay was 3.6 days (3-5days) and the mean return to normal activity was 11.6 days (10-14days). Most intra-operative and post-operative complications were minor complications and can be managed conservatively. After 68 months (12-96 months) follow-up, the outcome was satisfactory, and ureteral stump syndrome did not occur. Conclusions Retroperitoneal laparoscopic nephroureterectomy as a minimally invasive treatment option is feasible for treatment of tuberculous nonfunctioning kidneys. PMID:26005971

  6. Retroperitoneal laparoendoscopic single-site surgery: preliminary experience in kidney and ureteral indications.

    PubMed

    Micali, Salvatore; Isgrò, Gianmarco; De Stefani, Stefano; Pini, Giovannalberto; Sighinolfi, Maria Chiara; Bianchi, Giampaolo

    2011-01-01

    The advantages of retroperitoneoscopic technique are well known. We decided to combine this access with the emerging laparoendoscopic single-site surgery (LESS) technique. We present our preliminary data on 11 renoureteral procedures and describe our retroperitoneoscopic LESS technique. As of March 2009, 10 patients were submitted to retroperitoneal LESS and divided into three groups: Group A, 3 patients underwent ureterolithotomy; Group B, 4 patients underwent renal cyst ablation; Group C, 4 patients underwent renal biopsy. Retroperitoneal access was obtained with an optical trocar. After retroperitoneal space blunt dissection, a multichannel port was placed. Standard and bent 5-mm instruments were used; we also used a 5-mm flexible laparoscope as a single procedure in group A. Ten of 11 procedures were completed without conversion; a single case in group A was converted to open surgery. Retroperitoneoscopic LESS is a safe and feasible procedure for renal biopsy and renal cyst ablation, with shorter convalescence time, less postoperative pain, and better cosmetic outcomes. LESS ureterolithotomy was more challenging for the lack of triangulation, resulting in a prolonged convalescence period. In addition, bent laparoscopic instruments are not suitable for retroperitoneal space; the multichannel port leaks carbon dioxide due to the flank position. Therefore LESS pelvic trainer practice is imperative in this case. PMID:20727667

  7. Association of retroperitoneal lymphangioleiomyomatosis with endosalpingiosis: a case report.

    PubMed

    Herce, José Manuel Lorente; Luque, Virgilio Ruiz; Luque, José Aguilar; García, Pablo Martínez; Gómez, Daniel Díaz

    2009-01-01

    Lymphangioleiomyomatosis is a rare disorder of unknown origin that usually presents pulmonary symptoms. Retroperitoneal lymphangioleiomyomatosis without lung involvement has rarely been reported. We present a 38-year-old woman, the fourth case reported of retroperitoneal lymphangioleiomyomatosis with endosalpingiosis in the literature. PMID:19918577

  8. Association of retroperitoneal lymphangioleiomyomatosis with endosalpingiosis: a case report

    PubMed Central

    Luque, Virgilio Ruiz; Luque, José Aguilar; García, Pablo Martínez; Gómez, Daniel Díaz

    2009-01-01

    Lymphangioleiomyomatosis is a rare disorder of unknown origin that usually presents pulmonary symptoms. Retroperitoneal lymphangioleiomyomatosis without lung involvement has rarely been reported. We present a 38-year-old woman, the fourth case reported of retroperitoneal lymphangioleiomyomatosis with endosalpingiosis in the literature. PMID:19918577

  9. Necrotizing fasciitis as the clinical presentation of a retroperitoneal abscess.

    PubMed

    Amitai, Allon; Sinert, Richard

    2008-01-01

    Infections of the retroperitoneal space may present with insidiously vague symptoms and non-specific clinical signs. We report a case of a retroperitoneal abscess presenting as necrotizing fasciitis of the thigh from direct spread over the iliac crest. In cases of necrotizing fasciitis of the thigh with no obvious source, an intra-abdominal nidus of infection may be considered. PMID:17976803

  10. 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

  11. 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. PMID:24712182

  12. Recurrent Thymoma in the Retroperitoneal Space: A Rare Case Report

    PubMed Central

    Yang, Jun; Li, Qin-qing; Ding, Ying-ying; Cheng-de, Liao

    2015-01-01

    Thymoma is an epithelial neoplasm of the thymus, which commonly lies in the anterior mediastinum and recurrences of thymoma generally are locally, and retroperitoneal recurrence is considered to be rare. A 46-year old Asian woman with invasive thymoma had undergone thymectomy 10 years ago. Computed tomography demonstrated a well-circumscribed mass in the left retroperitoneal space. The patient had not any symptom including myasthenia gravis. Because on the anterior mediastinum area shows no sign of tumor recurrence and the mass adjacent to the vertebral body, neurogenic tumor was suspected. Surgical resection was performed using a retroperitoneal approach, which revealed the tumor adhering neighboring diaphragm. The tumor was histologically diagnosed to be type B1 thymoma according to the World Health Organization classification. The retroperitoneal mass was an unusual local recurrence after thymectomy. The patients whose had under invasive thymectomy should be evaluated carefully when finding retroperitoneal mass during follow-up. PMID:26236455

  13. 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

  14. Image-Guided Fine Needle Cytology with Aspiration Versus Non-Aspiration in Retroperitoneal Masses: Is Aspiration Necessary?

    PubMed Central

    Misra, Rajiv Kumar; Mitra, Shaila; Jain, Rishav Kumar; Vahikar, Shilpa; Bundela, Archana; Misra, Purak

    2015-01-01

    Background: Although using fine needle cytology with aspiration (FNC-A) for establishing diagnoses in the retroperitoneal region has shown promise, there is scant literature supporting a role of non-aspiration cytology (FNC-NA) for this region. We assessed the accuracy and reliability of FNC-A and FNC-NA as tools for preoperative diagnosis of retroperitoneal masses and compared the results of both techniques with each other and with histopathology. Methods: Fifty-seven patients with retroperitoneal masses were subjected to FNC-A and FNC-NA. Smears were stained with May-Grunwald Giemsa and hematoxylin and eosin stain. An individual slide was objectively analysed using a point scoring system to enable comparison between FNC-A and FNC-NA. Results: By FNC-A, 91.7% accuracy was obtained in cases of retroperitoneal lymph node lesions followed by renal masses (83.3%). The diagnostic accuracy of other sites by FNC-A varied from 75.0%–81.9%. By FNC-NA, 93.4% diagnostically accurate results were obtained in the kidney, followed by 75.0% in adrenal masses. The diagnostic accuracy of other sites by FNC-NA varied from 66.7%–72.8%. Conclusions: Although both techniques have their own advantages and disadvantages, FNC-NA may be a more efficient adjuvant method of sampling in retroperitoneal lesions. PMID:25812734

  15. What Is Uterine Sarcoma?

    MedlinePlus

    ... supporting tissues of the uterus (womb). About the uterus The uterus is a hollow organ, about the ... a baby out during childbirth. Cancers of the uterus and endometrium Sarcomas are cancers that start from ...

  16. Epidemic Kaposi Sarcoma

    MedlinePlus

    ... therapy are used to treat Kaposi sarcoma lesions . Photon radiation therapy treats lesions with high-energy light. ... complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information ...

  17. Classic Kaposi Sarcoma

    MedlinePlus

    ... therapy are used to treat Kaposi sarcoma lesions . Photon radiation therapy treats lesions with high-energy light. ... complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information ...

  18. 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.

  19. Diagnosis and management of retroperitoneal ancient schwannomas

    PubMed Central

    Choudry, Haroon A; Nikfarjam, Mehrdad; Liang, John J; Kimchi, Eric T; Conter, Robert; Gusani, Niraj J; Staveley-O'Carroll, Kevin F

    2009-01-01

    Background Ancient schwannomas are degenerate peripheral nerve sheath tumors that very rarely occur in the retroperitoneum. They generally reach large proportions before producing symptoms due to mass effect. We describe three cases of retroperitoneal ancient schwannomas and discuss the diagnosis and management of these tumors. Case presentations Three female patients with retroperitoneal ancient schwannomas were reviewed. One patient presented with several weeks of upper abdominal pain and lower chest discomfort, whereas back pain and leg pain with associated weakness were predominant symptoms in the remaining two. Abdominal imaging findings demonstrated heterogeneous masses in the retroperitoneum with demarcated margins, concerning for malignancy. The patients successfully had radical excision of their tumors. Histological examination showed encapsulated tumors that displayed alternating areas of dense cellularity and areas of myxoid matrix consistent with a diagnosis of ancient schwannoma. Conclusion A diagnosis of ancient schwannoma should be entertained for any heterogeneous, well encapsulated mass in the retroperitoneum. In these cases less radical surgical resection should be considered as malignant transformation of these tumors is extremely rare and recurrence is uncommon following excision. PMID:19187535

  20. Excellent local control with IOERT and postoperative EBRT in high grade extremity sarcoma: results from a subgroup analysis of a prospective trial

    PubMed Central

    2014-01-01

    Background To report the results of a subgroup analysis of a prospective phase II trial focussing on radiation therapy and outcome in patients with extremity soft tissue sarcomas (STS). Methods Between 2005 and 2010, 50 patients (pts) with high risk STS (size ≥ 5 cm, deep/extracompartimental location, grade II-III (FNCLCC)) were enrolled. The protocol comprised 4 cycles of neoadjuvant chemotherapy with EIA (etoposide, ifosfamide and doxorubicin), definitive surgery with IOERT, postoperative EBRT and 4 adjuvant cycles of EIA. 34 pts, who suffered from extremity tumors and received radiation therapy after limb-sparing surgery, formed the basis of this subgroup analysis. Results Median follow-up from inclusion was 48 months in survivors. Margin status was R0 in 30 pts (88%) and R1 in 4 pts (12%). IOERT was performed as planned in 31 pts (91%) with a median dose of 15 Gy, a median electron energy of 6 MeV and a median cone size of 9 cm. All patients received postoperative EBRT with a median dose of 46 Gy after IOERT or 60 Gy without IOERT. Median time from surgery to EBRT and median EBRT duration was 36 days, respectively. One patient developed a local recurrence while 11 patients showed nodal or distant failures. The estimated 5-year rates of local control, distant control and overall survival were 97%, 66% and 79%, respectively. Postoperative wound complications were found in 7 pts (20%), resulting in delayed EBRT (>60 day interval) in 3 pts. Acute radiation toxicity mainly consisted of radiation dermatitis (grade II: 24%, no grade III reactions). 4 pts developed grade I/II radiation recall dermatitis during adjuvant chemotherapy, which resolved during the following cycles. Severe late toxicity was observed in 6 pts (18%). Long-term limb preservation was achieved in 32 pts (94%) with good functional outcome in 81%. Conclusion Multimodal therapy including IOERT and postoperative EBRT resulted in excellent local control and good overall survival in

  1. 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.

  2. 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. PMID:27111283

  3. Adult human sarcomas. II. Medical oncology.

    PubMed

    Sinkovics, Joseph G

    2007-02-01

    Human sarcoma cells can be killed by radio- and chemotherapy, but tumor cells acquiring resistance frequently kill the patient. A keen understanding of the intracellular course of oncogenic cascades leads to the discovery of small molecular inhibitors of the involved phosphorylated kinases. Targeted therapy complements chemotherapy. Oncogene silencing is feasible by small interfering RNA. The restoration of some of the mutated or deleted tumor-suppressor genes (p53, Rb, PTEN, hSNF, INK/ARF and WT) by demethylation or reacetylation of their histones has been accomplished. Genetically engineered or naturally oncolytic viruses selectively lyse tumors and leave healthy tissues intact. Adeno- or retroviral vectors deliver genes of immunological costimulators, tumor antigens, chemo- or cytokines and/or tumor-suppressor proteins into tumor (sarcoma) cells. Suicide gene delivery results in apoptosis induction. Genes of enzymes that target prodrugs as their substrates render tumor cells highly susceptible to chemotherapy, with the prodrug to be targeted intracellularly. It will be combinations of sophisticated surgical removal of the nonencapsulated and locally invasive primary sarcomas, advanced forms of radiotherapy to the involved sites and immunotherapy with sarcoma vaccines that will cure primary sarcomas. Adoptive immunotherapy with immune lymphocytes will be operational in metastatic disease only when populations of regulatory T cells are controlled. Targeted therapy with small molecular inhibitors of oncogene cascades, the driving forces of sarcoma cells, alteration of the tumor stroma from a supportive to a tumor-hostile environment, reactivation or replacement of wild-type tumor-suppressor genes, and radio-chemotherapy (with much reduced toxicity) will eventually accomplish the cure of metastatic sarcomas. PMID:17288529

  4. General Information about Ewing Sarcoma

    MedlinePlus

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

  5. General Information about Kaposi Sarcoma

    MedlinePlus

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

  6. General Information About Uterine Sarcoma

    MedlinePlus

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

  7. Sarcoma-associated sarcoid reaction: Report of cutaneous sarcoid reaction in a patient with liposarcoma.

    PubMed

    Beutler, Bryce D; Cohen, Philip R

    2015-12-16

    Sarcoidosis is a systemic inflammatory condition in which noncaseating epithelioid cell granulomas appear within one or several body sites. Sarcoid reaction (also referred to as sarcoidal or sarcoid-like reaction) occurs in patients who do not fulfill the diagnostic criteria for systemic sarcoidosis but present with similar clinical and histological features. As sarcoma-associated sarcoid reactions are rare, we describe the features of sarcoid reaction that developed in a man with liposarcoma and summarize reports of other oncology patients with sarcoma-associated sarcoid reactions. A 68-year-old man with retroperitoneal liposarcoma presented for evaluation of erythematous dermal plaques on his left leg. Microscopic examination of a tissue specimen revealed multiple epithelioid granulomas in the superficial and mid-reticular dermis. Correlation of the clinical presentation and histopathologic findings established a diagnosis of liposarcoma-associated cutaneous sarcoid reaction. Sarcoid reactions have been described in only seven individuals with sarcoma, including two patients with leiomyosarcoma and one patient with either carcinosarcoma, Kaposi sarcoma, liposarcoma, malignant peripheral nerve sheath tumor, rhabdosarcoma, or synovial sarcoma. Sarcoidal granulomas most commonly develop within the locoregional draining lymph nodes. Sarcoid reactions may also affect other organs, such as the lungs, skin, and spleen. PMID:26677448

  8. Sarcoma-associated sarcoid reaction: Report of cutaneous sarcoid reaction in a patient with liposarcoma

    PubMed Central

    Beutler, Bryce D; Cohen, Philip R

    2015-01-01

    Sarcoidosis is a systemic inflammatory condition in which noncaseating epithelioid cell granulomas appear within one or several body sites. Sarcoid reaction (also referred to as sarcoidal or sarcoid-like reaction) occurs in patients who do not fulfill the diagnostic criteria for systemic sarcoidosis but present with similar clinical and histological features. As sarcoma-associated sarcoid reactions are rare, we describe the features of sarcoid reaction that developed in a man with liposarcoma and summarize reports of other oncology patients with sarcoma-associated sarcoid reactions. A 68-year-old man with retroperitoneal liposarcoma presented for evaluation of erythematous dermal plaques on his left leg. Microscopic examination of a tissue specimen revealed multiple epithelioid granulomas in the superficial and mid-reticular dermis. Correlation of the clinical presentation and histopathologic findings established a diagnosis of liposarcoma-associated cutaneous sarcoid reaction. Sarcoid reactions have been described in only seven individuals with sarcoma, including two patients with leiomyosarcoma and one patient with either carcinosarcoma, Kaposi sarcoma, liposarcoma, malignant peripheral nerve sheath tumor, rhabdosarcoma, or synovial sarcoma. Sarcoidal granulomas most commonly develop within the locoregional draining lymph nodes. Sarcoid reactions may also affect other organs, such as the lungs, skin, and spleen. PMID:26677448

  9. Laparoscopic retroperitoneal lymph node dissection for testicular cancer

    PubMed Central

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

    2012-01-01

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

  10. 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

  11. Spectrum of cytopathologic features of epithelioid sarcoma in a series of 7 uncommon cases with immunohistochemical results, including loss of INI1/SMARCB1 in two test cases.

    PubMed

    Rekhi, Bharat; Singh, Neha

    2016-07-01

    Diagnosis of an epithelioid sarcoma (ES) is challenging on fine needle aspiration cytology (FNAC) smears. There are few documented series describing cytopathologic features and immunostaining results of ESs. The present study describes cytopathologic features of seven cases of ES. All seven tumors occurred in males within age-range of 22-61 years; in sites, such as forearm (n = 3), hand (n = 2), thigh (n = 1), and inguinal region (n = 1). FNAC was performed for metastatic lesions (n = 5), recurrent lesions (n = 4), as well as for a primary diagnosis (n = 1). FNAC smears in most cases were moderate to hypercellular, composed of polygonal cells(seven cases) and spindle cells(three cases), arranged in loosely cohesive groups, non-overlapping clusters, and scattered singly, containing moderate to abundant cytoplasm, defined cell borders, vesicular nuclei, and discernible nucleoli. Variable cytopathologic features identified in certain cases were "rhabdoid-like" intracytoplasmic inclusions (n = 5), giant cells (n = 3), and interspersed scanty, metachromatic stroma (n = 4). Histopathologic examination revealed two cases of conventional-type ES, three of proximal/large cell-type ES, and two cases of mixed-type ES, displaying features of conventional and proximal subtypes. By immunohistochemistry (IHC), tumor cells were positive for cytokeratin (CK)(4/5), epithelial membrane antigen (EMA) (6/6), panCK (1/1), vimentin (3/3), and CD34 (7/7). Tumor cells were completely negative for INI1/SMARCB1 (0/2) and CD31 (0/5). In our settings, FNAC was mostly performed in recurrent and/or metastatic cases of ES, and rarely for a primary diagnosis of ES. Important cytopathologic features of ESs include loosely cohesive, non-overlapping clusters of polygonal cells with variable "rhabdoid-like" and spindle cells. Optimal diagnostic IHC markers in such cases include CK, EMA, AE1AE3, CD34, and INI1/SMARCB1. Clinical correlation is imperative in all

  12. Targeted Therapies in Sarcomas: Challenging the Challenge

    PubMed Central

    Martín Liberal, Juan; Lagares-Tena, Laura; Sáinz-Jaspeado, Miguel; Mateo-Lozano, Silvia; García del Muro, Xavier; Tirado, Oscar M.

    2012-01-01

    Sarcomas are a heterogeneous group of mesenchymal malignancies that very often lead to death. Nowadays, chemotherapy is the only available treatment for most sarcomas but there are few active drugs and clinical results still remain very poor. Thus, there is an imperious need to find new therapeutic alternatives in order to improve sarcoma patient's outcome. During the last years, there have been described a number of new molecular pathways that have allowed us to know more about cancer biology and tumorigenesis. Sarcomas are one of the tumors in which more advances have been made. Identification of specific chromosomal translocations, some important pathways characterization such as mTOR pathway or the insulin-like growth factor pathway, the stunning development in angiogenesis knowledge, and brand new agents like viruses have lead to the development of new therapeutic options with promising results. This paper makes an exhaustive review of preclinical and clinical evidence of the most recent targeted therapies in sarcomas and provides a future view of treatments that may lead to improve prognosis of patients affected with this disease. PMID:22701332

  13. Primary Intracranial Synovial Sarcoma.

    PubMed

    Patel, Mohit; Li, Luyuan; Nguyen, Ha Son; Doan, Ninh; Sinson, Grant; Mueller, Wade

    2016-01-01

    Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma. We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature. Case Description. A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia. CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression. Histopathology revealed synovial sarcoma. FISH analysis noted the existence of the t(X;18)(p11.2;q11.2) chromosomal translocation. PET scan did not show other metastatic disease. He underwent stereotactic radiotherapy and adjuvant chemotherapy. At 2-year follow-up, he remained nonfocal without recurrence. Conclusion. We report the first known case of primary intracranial synovial sarcoma. Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression. PMID:27247811

  14. Primary Intracranial Synovial Sarcoma

    PubMed Central

    Li, Luyuan; Sinson, Grant; Mueller, Wade

    2016-01-01

    Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma. We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature. Case Description. A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia. CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression. Histopathology revealed synovial sarcoma. FISH analysis noted the existence of the t(X;18)(p11.2;q11.2) chromosomal translocation. PET scan did not show other metastatic disease. He underwent stereotactic radiotherapy and adjuvant chemotherapy. At 2-year follow-up, he remained nonfocal without recurrence. Conclusion. We report the first known case of primary intracranial synovial sarcoma. Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression. PMID:27247811

  15. 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.

  16. Retroperitoneal Mature Cystic Teratoma in a Neonate: A Case Report

    PubMed Central

    Patil, Prashant Sadashiv; Kothari, Paras; Gupta, Abhaya; Dikshit, Vishesh; Kamble, Ravi; Kekre, Geeta; Deshmukh, Shahaji

    2016-01-01

    We report a case of retroperitoneal mature cystic teratoma in a 2-day-old neonate. Diagnostic and surgical procedure including its complexity and relevant literature review has been discussed. PMID:27123399

  17. Retroperitoneal Mature Cystic Teratoma in a Neonate: A Case Report.

    PubMed

    Patil, Prashant Sadashiv; Kothari, Paras; Gupta, Abhaya; Dikshit, Vishesh; Kamble, Ravi; Kekre, Geeta; Deshmukh, Shahaji

    2016-01-01

    We report a case of retroperitoneal mature cystic teratoma in a 2-day-old neonate. Diagnostic and surgical procedure including its complexity and relevant literature review has been discussed. PMID:27123399

  18. Giant Primary Retroperitoneal Teratoma in an Adult: A Case Report

    PubMed Central

    Mathur, Poonam; Lopez-Viego, Miguel A.; Howell, Myron

    2010-01-01

    Teratomas are bizarre neoplasms derived from embryonic tissues that are typically found only in the gonadal and sacrococcygeal regions of adults. Retroperitoneal teratomas are rare and present challenging management options. We report here the case of a histologically unusual retroperitoneal tumor detected on computed tomography during the workup of abdominal pain in a 32-year-old male. The evaluation and treatment of this condition and a review of the literature are included in this paper. PMID:20862380

  19. Retroperitoneal necrotizing fasciitis in a 4-year-old girl.

    PubMed

    Paya, K; Hayek, B F; Rebhandl, W; Pollak, A; Horcher, E

    1998-05-01

    Necrotizing fasciitis is a rare but serious condition with a poor prognosis both in adults and in children. Retroperitoneal localization is mostly associated with fatal outcome. Early diagnosis, extensive and repeated surgical debridement, and use of antibiotics are necessary. Herein the authors report on a 4-year-old girl in whom retroperitoneal necrotizing fasciitis developed after she suffered from pyelonephritis. In this case, the outcome was favorable because of early surgical intervention, confirming the diagnosis. PMID:9607500

  20. Management of Truncal Sarcoma.

    PubMed

    Mullinax, John E; Gonzalez, Ricardo J

    2016-10-01

    The management of truncal sarcoma presents unique challenges to the treating physician for reasons specific to this tumor location. First, the reconstruction options after resection of the abdominal or chest wall require a balance between cosmesis and structural integrity due to the multiplanar forces exerted on this region. Second, the histologies that commonly arise in this region are often associated with high local recurrence rates, which often require complex decision making due to prior therapy. Finally, sarcomas of the trunk in the inguinal region involve those organs in the genitourinary system, of which resection can have significant psychosocial implications. PMID:27542639

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

    PubMed

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

    2016-06-01

    Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for Soft Tissue Sarcoma (available at NCCN.org) provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumor, desmoid tumors, and rhabdomyosarcoma. This manuscript discusses guiding principles for the diagnosis and staging of STS and evidence for treatment modalities that include surgery, radiation, chemoradiation, chemotherapy, and targeted therapy. PMID:27283169

  3. [Adjuvant chemotherapy of adults soft tissue sarcomas].

    PubMed

    Bui-Nguyen, B; Italiano, A; Delva, F; Toulmond, M

    2010-06-01

    The main progress in the management of soft tissue sarcomas have been obtained in the field of local control. Although the main evolutive, vital, risk of these diseases is metastatic dissemination, efficacy of adjuvant chemotherapy remains a controversial issue. Thus, adjuvant chemotherapy cannot be considered as a standard for any situation. The last results of clinical trials, meta-analysis and population studies are presented and discussed in this article. New therapeutic strategies are to be developed to prevent metastases in soft tissue sarcomas. This needs a better understanding of the biology of those tumors, of metastases risk factors and of the determinants of systemic therapies efficacy in these tumors. PMID:20547481

  4. 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

  5. Adult soft tissue sarcoma

    MedlinePlus

    ... free at 5 years. Most people who survive 5 years can expect to be cancer-free at 10 years. ... most soft tissue sarcomas, and there is no way to prevent it. ... them can increase your chance of surviving this type of cancer.

  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. 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. PMID:27542644

  8. Leukosis/Sarcoma Group

    Technology Transfer Automated Retrieval System (TEKTRAN)

    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 ...

  9. Leukosis/Sarcoma Group

    Technology Transfer Automated Retrieval System (TEKTRAN)

    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. Lymphoid leukosis has been the most common form of L/S group of diseases seen in field flocks, although myeloid leuk...

  10. Immunotherapy of Childhood Sarcomas.

    PubMed

    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. 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

  12. Drugs Approved for Soft Tissue Sarcoma

    MedlinePlus

    ... Professionals Questions to Ask about Your Treatment Research Drugs Approved for Soft Tissue Sarcoma This page lists ... soft tissue sarcoma that are not listed here. Drugs Approved for Soft Tissue Sarcoma Cosmegen (Dactinomycin) Dactinomycin ...

  13. Innovative radiotherapy of sarcoma: Proton beam radiation.

    PubMed

    DeLaney, Thomas F; Haas, Rick L M

    2016-07-01

    This review on proton beam radiotherapy (PBT) focusses on an historical overview, cost-effectiveness, techniques, acute and late toxicities and clinical results of PBT for sarcoma patients. PBT has gained its place among the armamentarium of modern radiotherapy techniques. For selected patients, it can be cost-effective. PMID:27258968

  14. Retroperitoneal fibrosis with pancreatic involvement – radiological appearance

    PubMed Central

    Zielonko, Joanna; Obołończyk, Łukasz

    2011-01-01

    Summary Background: Retroperitoneal fibrosis or Ormond’s disease is an uncommon process characterized by fibrous tissue proliferation in the retroperitoneum, usually involving the aorta, inferior vena cava and iliac vessels. Obstructive hydronephrosis is often observed due to ureteral entrapment. This report presents a case of the peripancreatic location of the disease. The role of CT and MRI in establishing diagnosis of retroperitoneal fibrosis in an atypical site is discussed. Case Report: A 52-year-old woman with Hashimoto’s thyroiditis was admitted to hospital because of pain suggesting renal colic. The patient was subjected to ultrasound, CT, and MRI which did not confirm urolithiasis but revealed pancreatic infiltration. Partial pancreatectomy, left-sided adrenalectomy and splenectomy were performed. Retroperitoneal fibrosis was diagnosed in the histopathological examination. A few weeks after surgery, a complication such as pancreatitis developed. Repeat CT confirmed it and showed right hydronephrosis secondary to ureteral involvement by a mass adjacent to the common iliac artery (defined as a typical manifestation of retroperitoneal fibrosis). Nephrostomy and conservative treatment improved the clinical state of the patient. No progression of the process was observed in the follow-up examinations. Conclusions: Atypical retroperitoneal fibrosis remains a diagnostic challenge. Imaging techniques CT and MRI are useful tools for evaluating the extent of Ormond’s disease. An unusual distribution of the process (e.g. peripancreatic location reported in this study) requires histopathological assessment to establish the final diagnosis. PMID:22802859

  15. Retroperitoneal cavernous hemangioma resected by a pylorus preserving pancreaticoduodenectomy

    PubMed Central

    Hanaoka, Marie; Hashimoto, Masaji; Sasaki, Kazunari; Matsuda, Masamichi; Fujii, Takeshi; Ohashi, Kenichi; Watanabe, Goro

    2013-01-01

    A retroperitoneal hemangioma is a rare disease. We report on the diagnosis and treatment of a retroperitoneal hemangioma which had uncommonly invaded into both the pancreas and duodenum, thus requiring a pylorus preserving pancreaticoduodenectomy (PpPD). A 36-year-old man presented to our hospital with abdominal pain. An enhanced computed tomography scan without contrast enhancement revealed a 12 cm × 9 cm mass between the pancreas head and right kidney. Given the high rate of malignancy associated with retroperitoneal tumors, surgical resection was performed. Intraoperatively, the tumor was inseparable from both the duodenum and pancreas and PpPD was performed due to the invasive behavior. Although malignancy was suspected, pathological diagnosis identified the tumor as a retroperitoneal cavernous hemangioma for which surgical resection was the proper diagnostic and therapeutic procedure. Reteoperitoneal cavernous hemangioma is unique in that it is typically separated from the surrounding organs. However, clinicians need to be aware of the possibility of a case, such as this, which has invaded into the surrounding organs despite its benign etiology. From this case, we recommend that combined resection of inseparable organs should be performed if the mass has invaded into other tissues due to the hazardous nature of local recurrence. In summary, this report is the first to describe a case of retroperitoneal hemangioma that had uniquely invaded into surrounding organs and was treated with PpPD. PMID:23901241

  16. Doxorubicin With Upfront Dexrazoxane for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma

    ClinicalTrials.gov

    2016-07-11

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

  17. Synovial sarcoma: laryngopharynx a challenge.

    PubMed

    Verma, Ravinder; Verma, Ravneet Ravinder; Verma, Rohan Ravinder; Sardana, N K

    2014-06-01

    Synovial sarcoma is a rare malignant tumor. It derives from a mesenchymal precursor stem cell that is unrelated to mature synovial tissue. Synovial sarcoma classically affects lower limbs between the ages of 15 and 40 years and the proportion of male-to-female patients is 3:2. It is very rare in the head and neck region especially in laryngopharynx. Till date, only six cases of synovial sarcoma involving laryngopharynx have been reported in the English literature. Painless mass, hoarseness, upper respiratory distress, and dysphagia characterize the original complaints in laryngopharyngeal synovial sarcoma. Because head and neck synovial sarcoma in clinical practice is so uncommon, early diagnosis is difficult and the treatment protocol is unclear. Therefore, every case report should include complete information on presentation and management. Also, long-term prognostic indices need to be evaluated. We hereby report a case of large laryngopharyngeal synovial sarcoma confirmed by histopathology and immunohistochemistry with review of literature. PMID:24822167

  18. Completely resected follicular dendritic cell sarcoma of the posterior mediastinum: report of a case.

    PubMed

    Miyoshi, Ryo; Sonobe, Makoto; Miyamoto, Ei; Date, Hiroshi

    2016-12-01

    Follicular dendritic cell sarcoma is a rare malignant neoplasm originating from follicular dendritic cells, and most of them develop in lymph nodes of the head and neck. One third of follicular dendritic cell sarcomas occur in the extranodal sites such as the tonsils, mesentery, and retroperitoneal organs, but those of mediastinal origin are rare. Here, we present the case of a 16-year-old female with a large follicular dendritic cell sarcoma of posterior mediastinal origin. The tumor was found by a chest X-ray mass examination at her high school, and she had no subjective symptoms or significant past medical history. The tumor was diagnosed as a follicular dendritic cell sarcoma by computed tomography-guided needle biopsy. Although the tumor compressed the mediastinal organs and showed moderate uptake in 18-fluorodeoxyglucose positron emission tomography imaging, it was completely resected through posterolateral incision. Histological examination revealed that spindle-shaped tumor cells formed fascicular or storiform pattern with cellular pleomorphism. By immunohistochemical examination, the tumor cells were found to be positive for CD21 and follicular dendritic cell antigen. Two years after surgery, the patient remains alive with no signs of tumor recurrence. PMID:27001632

  19. A Giant Retroperitoneal Liposarcoma Encasing the Entire Left Kidney and Adherent to Adjacent Structures: A Case Report

    PubMed Central

    Oh, Sung Don; Oh, Sung Jin; Suh, Byoung Jo; Shin, Jin Yong; Oh, Cheol Kyu; Park, Jong Kwon; Kim, Yeon Mee; Kim, Bo Mi

    2016-01-01

    Retroperitoneal liposarcoma is a rare tumor. The dimension and weight of liposarcoma are variable; those over 20 kg are called ‘giant liposarcoma’. Herein, we report giant retroperitoneal liposarcoma measuring 45 cm in diameter and 25 kg in weight encasing the entire left kidney and adherent to adjacent structures. A 71-year-old woman presented for a regular checkup. Image study revealed a huge mass probably indicative of retroperitoneal liposarcoma encasing the entire left kidney and adherent to adjacent structures. We performed an organ-preserving surgical removal. The pathologic report was liposarcoma. At postoperative month 16, a follow-up CT revealed a locally recurrent tumor. The patient underwent surgical removal of the newly discovered mass. After the second surgery, the patient underwent regular follow-up CT for approximately 12 months, and to date, there has been no evidence of tumor recurrence. High-grade liposarcoma shows sensitivity to radiation therapy. However, the toxic effect of radiation therapy limits this option by treatment modality. The use of chemotherapy is also controversial. As a result, complete resection is the gold standard treatment. Here, we report a giant retroperitoneal liposarcoma encasing the entire left kidney and adherent to adjacent structures, describe successful organ-preserving surgical removal and discuss prognosis. PMID:27462239

  20. 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. PMID:25494288

  1. Harvey Sarcoma Virus: A Second Murine Type C Sarcoma Virus with Rat Genetic Information

    PubMed Central

    Scolnick, Edward M.; Parks, Wade P.

    1974-01-01

    The nucleic acid sequences found in the Harvey strain of murine sarcoma virus have been analyzed by RNA·[3H]DNA and [3H]RNA·DNA hybridization techniques. The Harvey strain of murine sarcoma virus has been found to possess at least two sets of nucleic acid sequences. One set of sequences is contained in the Moloney strain of mouse type-C virus, and the other set is contained in DNA transcripts synthesized in endogenous reactions containing rat type-C virus(es). The nucleic acid sequences that are detected in the Harvey sarcoma virus with the DNA probes synthesized from the rat type-C virus(es) are related to the rat sequences detected in the Kirsten strain of murine sarcoma virus. The results support the model that both Kirsten and Harvey sarcoma viruses arose through a process of recombination or reassortment between mouse type-C viruses and sequences in rat cells and suggest that the information for transformation of fibroblasts may be contained in the rat type-C or cellular genome. PMID:4364897

  2. 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.

  3. 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.

  4. Retroperitoneal amyloidosis as the presenting manifestation of Waldenstrom's macroglobulinaemia

    PubMed Central

    Franco-Palacios, Domingo; Tama, Maher; Samaddar, Suprotim; Yang, Jay

    2013-01-01

    Retroperitoneal amyloidosis has been described in a few case reports and is typically a secondary manifestation of inflammation or malignancy. We present the case of a 69-year-old man who presented with a large pleural effusion and an incidental retroperitoneal mass in the CT imaging. Further investigation confirmed the diagnosis of amyloid amyloidosis involving the retroperitoneum as well as a concurrent Waldenstrom's macroglobulinaemia. Chemotherapy consistent of drugs active against both the lymphoid and plasma cell components of the disease is the proposed therapy for Waldenstrom's macroglobulinaemia in those patients amenable to receive chemotherapy and can make a difference in the survival. PMID:23616327

  5. Laparoscopic resection of a retroperitoneal hemangioma arising from ovarian vessels.

    PubMed

    Choi, Youn Seok; Oh, Hoon Kyu

    2009-01-01

    Hemangiomas are known to be common benign tumors. However, hemangiomas of female genital organs are very rare. Furthermore, a retroperitoneal hemangioma arising from ovarian vessels has never been reported. Here we report a case of a 29-year-old woman with a retroperitoneal cavernous hemangioma arising from the ovarian vessels of infundibulopelvic ligament, which was treated with laparoscopic resection. The operating time was 30 minutes, and resection was carried out with minimal blood loss. The postoperative period was uneventful, and the patient was discharged on postoperative day 3. Laparoscopic resection of this type of hemangioma is feasible. PMID:19896610

  6. Giant Pelvic Retroperitoneal Epidermoid Cyst: A Rare Case Report

    PubMed Central

    Fdili Alaoui, F. Z.; Oussaden, A.; Bouguern, H.; El Fatemi, H.; Melhouf, M. A.; Amarti, A.; Ait Taleb, K.

    2012-01-01

    Epidermoid cyst is a frequent benign cutaneous tumor. The pelvic localization does not occur very often. The literature that taps into such cases is very limited in scope. Here is a report of a 27-year-old woman with a giant pelvic retroperitoneal epidermoid cyst. The use of ultrasound exploration and computed tomography has indicated ovarian origins. The surgery also revealed a retroperitoneal epidermoid cyst, uterus and ovaries were all intact. The evacuation of a cyst was found to contain lamellas of keratin. Histology permitted us to confirm the diagnosis. The patient was faring well after two years of followup. PMID:23150734

  7. Giant pelvic retroperitoneal epidermoid cyst: a rare case report.

    PubMed

    Fdili Alaoui, F Z; Oussaden, A; Bouguern, H; El Fatemi, H; Melhouf, M A; Amarti, A; Ait Taleb, K

    2012-01-01

    Epidermoid cyst is a frequent benign cutaneous tumor. The pelvic localization does not occur very often. The literature that taps into such cases is very limited in scope. Here is a report of a 27-year-old woman with a giant pelvic retroperitoneal epidermoid cyst. The use of ultrasound exploration and computed tomography has indicated ovarian origins. The surgery also revealed a retroperitoneal epidermoid cyst, uterus and ovaries were all intact. The evacuation of a cyst was found to contain lamellas of keratin. Histology permitted us to confirm the diagnosis. The patient was faring well after two years of followup. PMID:23150734

  8. Extensive cytogenetic heterogeneity in a benign retroperitoneal schwannoma.

    PubMed

    Gorunova, L; Dawiskiba, S; Andrén-Sandberg, A; Höglund, M; Johansson, B

    2001-06-01

    A benign retroperitoneal schwannoma from a patient without prior exposure to radiotherapy or chemotherapy was analyzed by chromosome banding after short-term culture. An extensive intratumor heterogeneity in the form of 29 karyotypically related as well as unrelated clones was found. The aberrant clones were diploid or near-diploid and displayed both numerical and structural changes. All chromosomes, except 11, 16, and 20, were affected. Numerical changes included trisomies X, 7, 9, 17, and 18, and monosomies 13 and 18. No clonal loss of chromosome 22, the most characteristic abnormality in schwannomas of other locations, was, however, detected. The structural aberrations resulted in a total of 58 chromosomal breakpoints, with chromosomes 18, 1, and 15 participating in rearrangements most frequently, followed by chromosomes 14, 2, and 22. A striking finding was the clonal involvement of 18p11 in eight rearrangements affecting different chromosomes, suggesting alteration of telomeric function. The molecular mechanisms underlying the observed massive polyclonality in the schwannoma, particularly the presence of cytogenetically unrelated clones, are unknown and probably heterogeneous. PMID:11425455

  9. Single-port retroperitoneal renal biopsy using standard urological instruments

    PubMed Central

    Guerra, Rodrigo; Ordones, Flávio Vasconcelos; Yamamoto, Hamilto Akihissa; Kawano, Paulo Roberto; Amaro, João Luiz

    2015-01-01

    Objective To describe the surgical technique and initial experience with a single-port retroperitoneal renal biopsy (SPRRB). Materials and Methods Between January and April 2013, five children underwent SPRRB in our hospital. A single 1.5 cm incision was performed under the 12th rib at mid-axillary line, and an 11 mm trocar was inserted. A nephroscope was used to identify the kidney and dissect the perirenal fat. After lower pole exposure, a laparoscopic biopsy forceps was introduced through the nephroscope working channel to collect a renal tissue sample. Results SPRRB was successfully performed in five children. The mean operative time was 32 minutes, and mean estimated blood loss was less than 10 mL. The hospital stay of all patients was two days because they were discharged in the second postoperative day, after remaining at strict bed rest for 24 hours after the procedure. The average number of glomeruli present in the specimen was 31. Conclusion SPRRB is a simple, safe and reliable alternative to open and videolaparoscopic approaches to surgical renal biopsy. PMID:25928523

  10. Therapeutic effectiveness of selected protoporphyrin derivatives in treatment of lung sarcoma (sarcoma L1)

    NASA Astrophysics Data System (ADS)

    Pirozynska, E.; Kalczak, M.; Stanowski, Edward; Kupsc, M.; Graczyk, Alfreda; Konarski, Jerzy

    1995-03-01

    The effectiveness of two preparations used as photosensitizers in the photodynamic method for neoplasm treatment was examined. The material consisted of Balb/c strain mice with transplanted lung sarcoma, Sarcoma L1. Arginine derivative HpDArg2 (hematoporphyrin derivatives -- arginine) and PP(Phen2Arg2, i.e., 1-arginine di(N- phenylalanyl) protoporphyrinate, were used as photosensitizers. Animals were administered the photosensitizer in a dose of 10 mg/kg body weight. After 24 hrs they were irradiated with He-Ne laser; total energy of 150 J/cm2 was applied in three portions of 50 J/cm2 each, at 24-h intervals. The results showed that PP(Phen)2Arg2 destroyed Sarcoma L1 much more effectively than the conventionally used mixture of hematoporphyrin derivatives, customarily named HpD.

  11. 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. PMID:27315524

  12. 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

  13. Diagnostic triage for sarcoma: an effective model for reducing referrals to the sarcoma multidisciplinary team

    PubMed Central

    Botchu, R; Ashford, R U; Rennie, W J

    2015-01-01

    Objective: Soft-tissue lesions are common and often benign. Owing to the rarity of soft-tissue sarcomas (STSs), evidence has shown that patients are increasingly referred urgently onto the 2-week wait pathway, which may have a detrimental impact on the management of patients with a proven STS. Imaging plays a vital role in lesion characterization and can be used to triage referrals to reduce the caseload of a sarcoma multidisciplinary team (MDT). In our institution, we established a sarcoma diagnostic triage meeting (SDTM). This study aimed to determine the effectiveness of the SDTM in reducing non-sarcomatous referrals to the main sarcoma MDT. Methods: A retrospective review of the SDTM minutes from July 2011 to June 2012 was performed. Data collected for each case included details of referrer, referral modality and referral outcome. Results: 165 cases were reviewed. 58% of referrals underwent a core biopsy or surgical excision with 85% benign pathology, the commonest being lipoma. 15% of referrals were sarcomatous lesions and were referred onwards to the main MDT. Conclusion: A total of 82% of the patients referred urgently with a suspicious soft-tissue mass was managed by the SDTM and hence not referred onwards to the East Midlands Sarcoma Service MDT. A diagnostic triage is effective in reducing the caseload burden of the main MDT and allowing it to be more effective. Advances in knowledge: Referrals based on imaging can be prioritized by diagnostic triage. Diagnostic triage established in our institution reduced 82% of MDT referrals allowing a more focused MDT discussion on patients with a proven sarcoma. PMID:25697295

  14. 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

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

    ClinicalTrials.gov

    2016-05-16

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

  16. Deregulation of the Hippo pathway in soft-tissue sarcoma promotes FOXM1 expression and tumorigenesis

    PubMed Central

    Eisinger-Mathason, T. S. Karin; Mucaj, Vera; Biju, Kevin M.; Nakazawa, Michael S.; Gohil, Mercy; Cash, Timothy P.; Yoon, Sam S.; Skuli, Nicolas; Park, Kyung Min; Gerecht, Sharon; Simon, M. Celeste

    2015-01-01

    Genetic aberrations responsible for soft-tissue sarcoma formation in adults are largely unknown, with targeted therapies sorely needed for this complex and heterogeneous family of diseases. Here we report that that the Hippo pathway is deregulated in many soft-tissue sarcomas, resulting in elevated expression of the effector molecule Yes-Associated Protein (YAP). Based on data gathered from human sarcoma patients, a novel autochthonous mouse model, and mechanistic analyses, we determined that YAP-dependent expression of the transcription factor forkhead box M1 (FOXM1) is necessary for cell proliferation/tumorigenesis in a subset of soft-tissue sarcomas. Notably, FOXM1 directly interacts with the YAP transcriptional complex via TEAD1, resulting in coregulation of numerous critical pro-proliferation targets that enhance sarcoma progression. Finally, pharmacologic inhibition of FOXM1 decreases tumor size in vivo, making FOXM1 an attractive therapeutic target for the treatment of some sarcoma subtypes. PMID:26080399

  17. 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

    2016-01-01

    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. PMID:26987019

  18. 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

  19. Orthopaedic patterns of retroperitoneal tumors in pediatric population.

    PubMed

    Brdar, R; Petronic, I; Abramovic, D; Nikolic, D; Cirovic, D

    2009-01-01

    The paper presents three children of various ages with tumors of different histology localized in the retroperitoneum. The children underwent investigation as orthopedic cases at the Orthopedic Department of the Belgrade University Childrens' Hospital. All children had orthopedic symptoms and several similar clinical findings: high or increased red blood cell (RBC) sedimentation, increased lactate dehydrogenase (LDH) and hypochromic anemia. Retroperitoneal tumors were diagnosed by echosonography. Further investigations were targeted towards histological verification and treatment protocol for retroperitoneal tumor. Since the children were presented chronologically to the deparmtent, diagnosis was reached more rapidly. It is our aim to draw attention to the possibility that various retroperitoneal tumors can be presented as orthopedic diseases. If symptomatology of retroperitoneal tumors is suspected and particularly in insufficiently clear cases, one should always perform echsonography of the retroperitoneum as a non-agressive, simple, readily available and reliable diagnostic method. This reduces examination time, direction of patients to further treatment according to pathology and also in reduction of risk both for patient and orthopedic surgeon who normally are presented with such diseases (Fig. 2, Ref. 10). Full Text (Free, PDF) www.bmj.sk. PMID:19507640

  20. [Synovial sarcoma. Case report].

    PubMed

    Deme, Dániel; Abdulfatah, Bishr; Telekes, András

    2016-02-01

    In 2013 there were 94,770 new cancer patients reported in Hungary. Synovial sarcoma accounts for 0.05-0.1% of all cancers and, therefore its incidence is predicted to be 47-94 patients/year in Hungary. The authors report the history of a 18-year-old man who was operated on a right upper abdominal wall tumor with R1 resection. During the next 5 months the tumor grew up to 8 cm in largest diameter. Histology revealed monophasic synovial sarcoma. Immunohistochemistry showed bcl2, focal CD99 and high molecular weight cytokeratin positivity, while smooth muscle actin, S100 and CD34 immunostainings were negative. Becose of this reoperation was not possible, curative six cycles of doxorubicine and ifosfamide with granulocyte colony stimulating factor support and 60 Gy radiotherapy was given to the tumor bed. After these treatments computed tomography scan was negative and the patient attended regular imaging every 3 months. At the age of 20 years the patient developed two neoplastic lesions in the surgical scar measuring 10 mm and 45 × 10 mm in size. R0 resection, partial rib resection and abdominal wall reconstruction were performed. Histology confirmed residual monophasic synovial sarcoma. Radiotherapy was not given because of a risk of intestinal wall perforation. Staging positron emission tomography-computed tomography proved to be negative. At the age of 22 years magnetic resonance imaging scans indicated no tumor recurrence, but after one month a rapidly growing tumorous lesion was found on ultrasound in the surgical scar measuring 20 × 20 × 12 mm in size. Cytology confirmed local recurrence and fluorescence in situ hibridization indicated t(x;18). R0 exstirpation and partial mesh resection were performed and histology showed the same monophasic synovial sarcoma. Because of the presence of vascular invasion and a close resection margin (1 mm) the patient underwent 3 cycles of adjuvant chemotherapy (doxorubicine and ifosfamide) with granulocyte colony stimulating

  1. Robot-assisted laparoscopic excision of a retroperitoneal paracaval tumor.

    PubMed

    Wei, Tzu-Chun; Chung, Hsiao-Jen; Lin, Alex T L; Chen, Kuang-Kuo

    2013-12-01

    During the past few years, robotic surgical systems have been rapidly developed. The progress and advantages of these systems include three-dimensional vision and enhanced ergonomics. These advantages have helped a new generation of minimally invasive surgery to evolve. The da Vinci Surgical System seems to greatly resolve problems (e.g., wide exposure and retraction of peritoneal organs) that are confronted by traditional laparoscopic surgeries for retroperitoneal tumors that are near great vessels. There have been few reported cases concerning laparoscopic excision of retroperitoneal tumors situated between the inferior vena cava, the right renal vessel, and the kidney. We report the use of a robotic surgical system for this type of treatment. A 54-year-old female patient had a hypoechoic lesion near the inferior vena cava and superior to the right renal vessels. It was incidentally found by ultrasound during a health check-up examination. The computed tomography (CT) scan revealed a heterogeneous contrast-enhanced retroperitoneal mass approximately 4.4 cm medial to the right kidney with the inferior vena cava slightly deviated to the left. Robot-assisted laparoscopic excision of the retroperitoneal tumor was performed on October 15, 2010 with an operation time of 135 minutes and an estimated blood loss of less than 30 mL. The J-Vac drainage tube was removed on postoperative Day 3, and the patient was discharged in a stable condition the following day. The pathology of the tumor was retroperitoneal schwannoma. A re-evaluation was arranged postoperatively for the 15-month ambulatory visit. No local recurrence or distal metastasis was present. PMID:24079977

  2. Follicular Dendritic Cell Sarcoma

    PubMed Central

    Udayakumar, Achandira M.; Al-Bahri, Maiya; Burney, Ikram A.; Al-Haddabi, Ibrahim

    2015-01-01

    Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm with a non-specific and insidious presentation further complicated by the difficult diagnostic and therapeutic assessment. It has a low to intermediate risk of recurrence and metastasis. Unlike other soft tissue sarcomas or histiocytic and dendritic cell neoplasms, cytogenetic studies are very limited in FDCS cases. Although no specific chromosomal marker has yet been established, complex aberrations and different ploidy types have been documented. We report the case of a 39-year-old woman with FDCS who presented to the Sultan Qaboos University Hospital in Muscat, Oman, in February 2013. Ultrastructural, immunophenotypical and histological findings are reported. In addition, karyotypic findings showed deletions of the chromosomes 1p, 3q, 6q, 7q, 8q and 11q. To the best of the authors’ knowledge, these have not been reported previously in this tumour. Techniques such as spectral karyotyping may help to better characterise chromosomal abnormalities in this type of tumour. PMID:26355964

  3. [Diagnostic possibilities of magnetic resonance tomography (MRT) in the retroperitoneal space].

    PubMed

    Zilch, H G; Held, P

    1989-08-01

    The diagnostic value of MR-imaging is tested on 130 patients with pathological retroperitoneal processes. Improved imaging techniques resulted in a more accurate delineation of tumor extension than seen with CT-imaging. Using a ratio of signal intensity from the adrenal tumors and the liver on T2-weighted images, it is usually possible to differentiate adenomas from metastases, carcinomas or phaeochromocytomas. New indications for renal MR-imaging would be parenchymal renal disease and transplant rejections. Moreover, MR allows visualization of lumbar lymph node-chains in the coronal view corresponding to the situs during surgery, thus, facilitating preoperative orientation for the surgeon. PMID:2807972

  4. 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

  5. Endosialin expression in soft tissue sarcoma as a potential marker of undifferentiated mesenchymal cells

    PubMed Central

    Thway, Khin; Robertson, David; Jones, Robin L; Selfe, Joanna; Shipley, Janet; Fisher, Cyril; Isacke, Clare M

    2016-01-01

    Background: Soft tissue sarcomas are a group of neoplasms with differentiation towards mesenchymal tissue, many of which are aggressive and chemotherapy resistant. Histology and immunoprofiles often overlap with neoplasms of other lineages, and establishing an accurate histopathological diagnosis is crucial for correct management, and therapeutic stratification. The endosialin cell surface glycoprotein is predominantly expressed by stromal fibroblasts and pericytes in epithelial neoplasms; however, tumour cell expression has been reported in small series of sarcomas. Methods: We assessed endosialin expression by immunohistochemistry in a large set of 514 human soft tissue sarcomas. Results: Tumour cell endosialin expression was seen in 89% of undifferentiated pleomorphic sarcomas (104/117), 77% adult fibrosarcomas/spindle cell sarcomas (20/26), 62% synovial sarcomas (37/60), 51% leiomyosarcomas (94/185) and 31% rhabdomyosarcomas (39/126). Conclusions: Endosialin immunohistochemistry has potential to distinguish undifferentiated and poorly differentiated sarcomas from other poorly differentiated, non-mesenchymal neoplasms. A Phase II trial randomising patients with advanced sarcomas to receive chemotherapy with/without an endosialin therapeutic antibody has recently completed enrolment. Endosialin expression could be used to select patients for such clinical trials. Based on our results, patients with undifferentiated pleomorphic sarcoma may be particularly suitable for such a therapeutic approach. PMID:27434038

  6. What Should You Ask Your Doctor about Uterine Sarcoma?

    MedlinePlus

    ... for uterine sarcoma? What should you ask your doctor about uterine sarcoma? It is important for you ... and Staging Treating Uterine Sarcoma Talking With Your Doctor After Treatment What`s New in Uterine Sarcoma Research? ...

  7. Alisertib in Treating Patients With Advanced or Metastatic Sarcoma

    ClinicalTrials.gov

    2016-02-02

    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

  8. Laparoscopic ureterolithotomy; which is better: Transperitoneal or retroperitoneal approach?

    PubMed Central

    Khalil, Mostafa; Omar, Rabea; Abdel-baky, Shabieb; Mohey, Ahmed; Sebaey, Ahmed

    2015-01-01

    Objective This was a prospective study to compare the outcome of laparoscopic transperitoneal ureterolithotomy (LTU) with laparoscopic retroperitoneal ureterolithotomy (LRU) as a primary treatment for a large stone in the proximal ureter. Material and methods A total of 24 patients with a solitary, large (>1.5 cm), and impacted stone in the proximal ureter was selected and randomly divided into two groups. The first group included 13 patients who were treated by LTU, and the second group included 11 patients who were treated by LRU. Patient demographics and stone characteristics as well as the operative and postoperative data of both groups were compared and statistically analyzed. Results There was no significant difference between the two groups regarding patient demographics and stone characteristics. The mean operative time was significantly shorter in the LTU group than in the LRU group [116.2±21.8 min vs 137.3±17.9 min, respectively (p=0.02)]. The mean time to oral intake was significantly longer in the LTU group than in the LRU group [21.2±4.9 h vs 15.5±2.8 h, respectively (p=0.002)]. There was significant higher rate (27.3%) of changing to open surgery in LRU (p=0.04). The stone-free rate was significantly higher in the LTU group than in the LRU group [100% vs. 72.8%, respectively (p=0.03)]. There was no statistically significant difference between the two groups regarding the mean blood loss, mean hospital stay, mean analgesia dose, blood transfusion rate, postoperative fever, and stone migration during surgery. Conclusion Both approaches of laparoscopic ureterolithotomy are effective in treating large impacted stones in the proximal ureter. LTU has significantly shorter operative time and lower rate of open conversion but has a significantly longer time to oral intake. PMID:26623147

  9. 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. PMID:20226998

  10. The Role of Mirk Kinase in Sarcomas

    PubMed Central

    Friedman, Eileen

    2011-01-01

    Targeting the tyrosine kinase KIT in gastrointestinal stromal tumors has led to improved treatment. Other kinases might serve as therapeutic targets in the more common forms of sarcoma. The kinase Mirk/dyrk1B is highly expressed in the vast majority of osteosarcomas and rhabdomyosarcomas and mediates their growth, as depletion of Mirk led to tumor cell apoptosis. Mirk is known to increase the expression of a series of antioxidant genes, which scavenge reactive oxygen species (ROS) within various tumor cells, mediating their survival. As a result, depleting Mirk led to increased levels of damaging ROS. Tumor cells depleted of Mirk were also sensitized to low levels of chemotherapeutic drugs that increase ROS levels. In contrast, Mirk expression is quite low in most normal cells, and Mirk depletion or embryonic knockout of Mirk did not detectably affect cell survival. Thus targeting Mirk for intervention in sarcomas might spare most normal tissues. PMID:21559261

  11. 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.

  12. Treatment Options for Kaposi Sarcoma

    MedlinePlus

    ... therapy are used to treat Kaposi sarcoma lesions . Photon radiation therapy treats lesions with high-energy light. ... complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information ...

  13. Treatment Option Overview (Kaposi Sarcoma)

    MedlinePlus

    ... therapy are used to treat Kaposi sarcoma lesions . Photon radiation therapy treats lesions with high-energy light. ... complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information ...

  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. Retroperitoneal calcifying fibrous tumor mimicking an adrenal tumor.

    PubMed

    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

  16. 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

  17. Nonrhabdomyosarcomatous abdominopelvic sarcomas: Analysis of prognostic factors

    PubMed Central

    Iqbal, Nida; Shukla, Nootan K.; Deo, S. V. S.; Agarwala, Sandeep; Sharma, D. N.; Sharma, Meher C.; Bakhshi, Sameer

    2016-01-01

    Background: Data concerning treatment outcome and prognostic factors in sarcomas of abdomen and pelvis are sparse in literature. Methods and Results: Of 696 patients with nonrhabdomyosarcomatous soft tissue sarcoma registered at our center between June 2003 and December 2012, 112 (16%) patients of sarcomas arising from abdomen and pelvis were identified, of which 88 patients were analyzed for treatment outcome and prognostic factors. The median age was 40 years (range: 1–78 years) with a male: female ratio of 0.7:1. Twenty-one (24%) patients were metastatic at baseline. The most common tumor sites were retroperitoneum in 70% patients and abdominal wall in 18% patients. Leiomyosarcoma was the most common histological subtype in 36% patients followed by liposarcoma in 17% patients. Thirty-five (40%) patients had Grade III tumors. Forty-six (52%) patients underwent surgical resection. At a median follow-up of 43 months (range: 2–94 months), the 5-year event-free survival (EFS) and overall survival (OS) were 35% and 42%, with a median of 22 months and 43 months, respectively. Multivariate analysis identified male gender (P - 0.03, hazard ratio [HR] - 0.46, 95% confidence interval [CI] - 0.23–0.92), baseline metastatic disease (P - 0.01, HR - 2.98, 95% CI - 1.27–6.98) and Grade III tumors (P - 0.02, HR - 1.84, 95% CI - 1.08–3.13) as factors associated with poor EFS, whereas baseline metastatic disease (P < 0.001, HR - 5.45, 95% CI - 2.31–12.87) and unresectability (P - 0.01, HR - 2.72, 95% CI - 1.27–5.83) were associated with poor OS. Conclusion: This is a single-institutional study of patients with abdominopelvic sarcomas where gender was identified as a new factor affecting survival apart from baseline presentation, histologic grade, and surgical resection. PMID:27168708

  18. A pelvic retroperitoneal Schwannoma presenting as an adnexal mass.

    PubMed

    Khatib, R A; Khalil, A M; Saba, M I; Aswad, N K; Mroueh, A M

    1994-05-01

    Solitary nerve sheath tumors such as benign schwannomas arising in the pelvic retroperitoneum are infrequently reported. We report a case of a benign retroperitoneal pelvic schwannoma that presented with pelvic pain and an adnexal mass. Complete surgical excision was achieved only after transection of the S1 nerve root on the left side. The adjacent vascular and urinary channels sustained no injuries and the patient had minimal neurologic deficit. PMID:8188087

  19. Retroperitoneal necrotizing fasciitis - when a simple procedure turns ugly.

    PubMed

    Alvi, Abdul; Shamsi, Ghina

    2009-01-01

    We present a case series of patients who underwent simple perianal procedures outside this hospital but developed postoperative and life-threatening retroperitoneal necrotizing fasciitis. With a high index of clinical suspicion and radiological imaging, we were able to establish earlier diagnosis. Resuscitation, the use of intravenous broad-spectrum antibiotics, aggressive surgical debridement and continuous organ support were essential in achieving a positive outcome in both of these patients. PMID:19211434

  20. Juxta-adrenal Ancient Schwannoma: A Rare Retroperitoneal Tumor

    PubMed Central

    Wollin, Daniel A; Sivarajan, Ganesh; Shukla, Pratibha; Melamed, Jonathan; Huang, William C; Lepor, Herbert

    2015-01-01

    Retroperitoneal schwannoma is a rare tumor that is often misdiagnosed as malignancy due to a concerning appearance on cross-sectional imaging. Pathology and immunohistochemistry form the gold standard for diagnosis; as such, local excision is the treatment of choice for this disease. We present two cases of juxta-adrenal ancient schwannoma that were treated with adrenalectomy and discuss the current literature regarding this entity.

  1. Retroperitoneal Endodermal Sinus Tumor Patient with Palliative Care Needs

    PubMed Central

    Kashyap, Surbhi

    2016-01-01

    This article is a case reflection of a personal encounter on the palliative care treatment required after the removal of a complicated case of a primary extra-gonadal retro-peritoneal endodermal sinus tumor (yolk sac tumor). This reflection is from the perspective of a recently graduated MD student who spent one month with an Indian pain management and palliative care team at the Institute Rotary Cancer Hospital (IRCH), All India Institute of Medical Sciences (AIIMS), New Delhi PMID:26962288

  2. 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.

  3. 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

  4. Minimally invasive retroperitoneal pancreatic necrosectomy in necrotising pancreatitis.

    PubMed

    Shelat, V G; Diddapur, R K

    2007-08-01

    With the marriage of surgery and technology, applications of minimal access surgery are increasing exponentially. Pancreatic diseases are no exception. Minimally invasive retroperitoneal pancreatic necrosectomy (MIRP), or percutaneous video-assisted necrosectomy, is a new technique to debride the necrotic pancreas. We report a 51- year-old male patient who successfully underwent MIRP for infected pancreatic necrosis, and briefly review of literature. PMID:17657370

  5. Retroperitoneal mass presenting as recurrent inguinal hernia: A case report

    PubMed Central

    Tardu, Ali; Yagci, Mehmet Ali; Karagul, Servet; Ertugrul, Ismail; Kayaalp, Cuneyt

    2016-01-01

    Introduction Retroperitoneal masses presenting as an inguinal hernia are rare conditions. Presentation of case A 53 year old male admitted with the symptoms of weight loss, abdominal discomfort and left sided recurrent inguinal hernia. Physical examination demonstrated an abdominal mass in the left flank and an irreducible, painless scrotal mass. He had a history of left sided inguinal hernia surgery six years ago. Computed tomography revealed a large enhancing left sided retroperitoneal mass invading the colon, pancreas and kidney and it was going down towards the left scrotum. Unblock tumor resection including the neighboring organs (left kidney, left colon, distal pancreas with spleen) was performed. Scrotal extension of the tumor was also excised and the inguinal canal was repaired primarily. Histopathology of the mass was myxoid-liposarcoma. The patient has disease free, without hernia recurrence but poor in renal function after twenty months follow-up. Discussion Large retroperitoneal tumors may grow towards the inguinal region and they can mimic an inguinal hernia. An irreducible, painless and hard scrotal mass should be considered from this perspective. PMID:26812669

  6. Surgical management of isolated retroperitoneal Castleman's disease: A case report

    PubMed Central

    XU, JUN; ZHOU, BO; CAO, HUA-LI; WANG, BO; YAN, SHENG; ZHENG, SHU-SEN

    2016-01-01

    Castleman's disease (CD) is an uncommon, poorly understood lymphoproliferative disease. Retroperitoneal forms may present as either a unicentric or multicentric disease. The present study reports the case of a 36-year-old man who was referred to the First Affiliated Hospital, School of Medicine, Zhejiang University (Hangzhou, China), for a detailed examination of an abdominal mass. The abdominal ultrasound and computed tomography scans revealed a solid mass localized in the region between segment 1 of the liver and the pancreas. An endosonography-guided fine-needle aspiration biopsy revealed chronic inflammation and lymphadenosis. The present study reports a rare case, in which the patient was treated with an exploratory laparotomy and resection. The retroperitoneal mass was pathologically diagnosed as CD of the hyaline vascular type. The patient was closely followed-up for 11 months and is presently free of disease. In conclusion, the possibility of unicentric CD should be considered when facing a solid hypervascular retroperitoneal mass. A complete surgical resection may successfully treat the disease without an unnecessarily extensive resection for the unicentric type. PMID:26998133

  7. [Clinical value of testicular lymphangiography in diagnosis of retroperitoneal metastases].

    PubMed

    Takasaki, N; Matsuse, K; Okada, S; Ra, S; Ueda, H; Ogita, T

    1984-11-01

    Testicular lymphangiography was performed before retroperitoneal lymph node dissection in 20 patients with testicular tumor. The clinical value of testicular lymphangiography in the diagnosis of retroperitoneal metastases was evaluated retrospectively in comparison with the findings obtained by retroperitoneal lymph node dissection. In 12 patients who had no metastasis in the primary lymph nodes of the testis, testicular lymphangiography showed the lymph vessels to be diverged into 2 to 6 vessels (mean: 3.5) at the level between L2 and L4, and 4 to 10 lymph nodes (mean: 6.2) at the level between L1 and L4 were filled with contrast medium. On the other hand, in 8 patients who had metastases in the primary lymph nodes, several abnormal findings were observed in both lymph vessels and nodes, i.e., discontinuity, extravasation of contrast medium, dilatation, displacement and reflux to the distal side in the lymph vessels, and decrease in number (less than 2), non-visualization, filling defect, displacement and contrastfilling in the contralateral side in lymph nodes. Three to 5 of these abnormal findings were usually found in each case. The extravasation of contrast medium was not a finding specific to cases with lymph node metastases, because it was also found in a few cases without metastases. Testicular lymphangiography is a valuable method to detect primary lymph node metastases from testicular tumor. However, the combination of testicular and foot lymphangiography is imperative to demonstrate wide spread lymph node involvement in the retroperitoneum. PMID:6528843

  8. 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

  9. Immediate versus Delayed Sarcoma Reconstruction: Impact on Outcomes

    PubMed Central

    Sanniec, Kyle J.; Zhang, Nan; III, William J. Casey

    2016-01-01

    Background. Sarcoma is a rare malignancy, and more recent management algorithms emphasize a multidisciplinary approach and limb salvage, which has resulted in an increase in overall survival and limb preservation. However, limb salvage has resulted in a higher rate of wound complications. Objective. To compare the complications between immediate and delayed (>three weeks) reconstruction in the multidisciplinary limb salvage sarcoma patient population. Methods. A ten-year retrospective review of patients who underwent sarcoma resection was performed. The outcome of interest was wound complication in the postoperative period based on timing of reconstruction. We defined infection as any infection requiring intravenous antibiotics, partial flap failure as any flap requiring a debridement or revision, hematoma/seroma as any hematoma/seroma requiring drainage, and wound dehiscence as a wound that was not completely intact by three weeks postoperatively. Results. 70 (17 delayed, 53 immediate) patients who underwent sarcoma resection and reconstruction met the inclusion criteria. Delayed reconstruction significantly increased the incidence of postoperative wound infection and wound dehiscence. There was no difference in partial or total flap loss, hematoma, or seroma between the two groups. Discussion and Conclusion. Immediate reconstruction results in decreased wound complications may reduce the morbidity associated with multidisciplinary treatment in the limb salvage sarcoma patient. PMID:27478403

  10. 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

  11. Pericardial Synovial Sarcoma: A Rare Clinical Entity.

    PubMed

    Goldblatt, Joshua; Saxena, Pankaj; McGiffin, David C; Zimmet, Adam

    2015-11-01

    Synovial sarcoma is an extremely rare form of primary malignancy of the pericardium. We present a case of primary synovial sarcoma of the pericardium followed by a review of the literature. PMID:26347295

  12. General Information about Adult Soft Tissue Sarcoma

    MedlinePlus

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

  13. General Information about Childhood Soft Tissue Sarcoma

    MedlinePlus

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

  14. 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 ...

  15. Survival by Stage of Soft Tissue Sarcoma

    MedlinePlus

    ... Next Topic How are soft tissue sarcomas treated? Survival by stage of soft tissue sarcoma Survival rates ... observed, not relative survival): Stage 5-year observed survival rate I 90% II 81% III 56% IV ...

  16. [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. PMID:27523415

  17. A case of pulmonary lymphangioleiomyomatosis complicated with uterine and retroperitoneal tumors.

    PubMed

    Numata, Takanori; Araya, Jun; Mikami, Jiro; Hara, Hiromichi; Harada, Tohru; Takahashi, Hiroyuki; Nakayama, Katsutoshi; Kuwano, Kazuyoshi

    2015-01-01

    A 39-year-old female experienced dyspnea on exertion for eight months. Chest CT demonstrated findings of Lymphangioleiomyomatosis (LAM), including diffuse thin-walled cystic lesions. A surgical lung biopsy revealed human melanoma black-45-positive cell infiltration and aggregation, resulting in a diagnosis of sporadic LAM without tuberous sclerosis complex. Pelvic MRI showed two large tumors, one of which was in the myometrium and the other was in the retroperitoneal space. Because we were not able to exclude the presence of malignant tumors using MR imaging, the tumors were surgically resected. The histopathology demonstrated the resected tumors to be composed of LAM cells. The patient's symptoms worsened, and sirolimus was administered, which improved the dyspnea and pulmonary function. The adverse effect was mild liver damage. Following the initiation of treatment with sirolimus, transient elevation of the serum KL-6 level was detected without interstitial pneumonia. This LAM case complicated with large uterine and retroperitoneal tumors was successfully treated with surgical resection and sirolimus. PMID:26236608

  18. 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

  19. Magnetic resonance imaging of retroperitoneal interfascial plane involvement in acute pancreatitis

    PubMed Central

    Chi, Xiao-Xiao; Chen, Tian-Wu; Huang, Xiao-Hua; Yang, Lin; Tang, Wei; Wáng, Yì-Xiáng J.; Xiao, Bo

    2016-01-01

    Background To study the magnetic resonance imaging (MRI) features of acute pancreatitis (AP) involving the retroperitoneal interfascial planes and to analyze the correlations of interfascial plane involvement with the magnetic resonance severity index (MRSI) and the Acute Physiology and Chronic Health Evaluation (APACHE II) scoring system. Methods A total of 316 consecutive patients with AP between November 2011 and August 2013 were retrospectively analyzed. The extension and path of the inflammation spreading along the retroperitoneal interfascial plane on MRI were graded from grade 0 to grade 5. The relationships between interfascial plane involvement and MRSI and APACHE II were analyzed. Results Of the 316 patients with AP, 293 patients (92.7%) had interfascial plane involvement, which appeared as interfascial plane edema, thickening and effusion. There were 60, 105, 78, 25, and 25 patients in grades 1, 2, 3, 4, and 5, respectively. Interfascial plane involvement strongly correlated with the MRSI score (r=0.703), but was only weakly correlated with the APACHE-II score (r=0.291). Conclusions MRI depicts the characteristics of interfascial plane involvement in AP. The value of interfascial plane involvement for determining the disease severity is likely to be limited. PMID:27429909

  20. Should We Place Ureteral Stents in Retroperitoneal Laparoscopic Ureterolithotomy?: Consideration of Surgical Techniques and Complications

    PubMed Central

    You, Jae Hyung; Kim, Young Gon

    2014-01-01

    Purpose There is some debate over the necessity of ureteral stenting after laparoscopic ureterolithotomy. We evaluated the need for ureteral stenting after retroperitoneal laparoscopic ureterolithotomy (RLU). Materials and Methods Between January 2009 and January 2013, 41 patients underwent RLU to remove upper ureteral stones. The retroperitoneal approach was used in all patients by a single surgeon. A double J (D-J) stent was placed in the first 17 patients after the procedure but not in any of the next 24 patients. Results The mean patient age, serum creatinine levels, and stone size were not significantly different between the two groups. The stone-free rate was 100%. The mean operative time was significantly shorter in the stentless group than in the stent group (59.48 minutes vs. 77.88 minutes, p<0.001). Parenteral analgesic use and anticholinergic medication use were observed in the stent group only. The blood loss, drain removal day, and hospital stay were not significantly different between the two groups. No other significant complications occurred during or after the operation in any patients. Conclusions RLU is a safe and effective treatment modality for large impacted ureteral stones. In this study, D-J stent placement was not necessary after RLU. In the future, large-scale studies of RLU without D-J stenting, especially on the frequency of the development of complications according to the surgical technique, may be needed. PMID:25132944

  1. Uterine Sarcoma: The Indian Scenario.

    PubMed

    Sivakumari, S; Rajaraman, R; Subbiah, S

    2015-09-01

    Uterine sarcomas are rare, highly malignant tumours comprising < 1 % of all gynaecologic malignancies. To evaluate clinical presentation, histolopathologic pattern and outcome of uterine sarcomas presenting to a tertiary referral centre over an 8 year period (2004-2012). All histologically proven uterine sarcomas were retrospectively analysed. Clinical presentation, histology, treatment and outcome were analysed. Mean age was 42 years. Predominant histopathology was endometrial stromal sarcoma (n = 13); 9 were low grade, carcinosarcoma (n = 8) and leiomyosarcoma (n = 2). Fourteen patients had Stage I disease, 3 Stage II, 4 Stage III and 2 were Stage IV at presentation. Patients with disease confined to uterus received no adjuvant treatment (61 %). Of these, 11 were endometrial stromal sarcoma (7 were low grade) and 3 were carcinosarcomas. Four patients received adjuvant EBRT following hysterectomy (17 %). Two patients who presented with metastases received palliative chemotherapy. Mean follow-up period was 46 months (0-86 months). Eleven patients (47 %) developed disease recurrence. Seven (30 %) had local recurrence, while 4 (17 %) developed pulmonary metastases. A total of eight patients died and all deaths were within 1 year of recurrence. The only prognostic factor that correlated with survival was the stage of disease at diagnosis. PMID:27217670

  2. 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. PMID:27106779

  3. 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.

  4. 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

  5. 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

  6. CIC-rearranged Sarcomas: A Study of 20 Cases and Comparisons With Ewing Sarcomas.

    PubMed

    Yoshida, Akihiko; Goto, Keisuke; Kodaira, Makoto; Kobayashi, Eisuke; Kawamoto, Hiroshi; Mori, Taisuke; Yoshimoto, Seiichi; Endo, Otone; Kodama, Narihito; Kushima, Ryoji; Hiraoka, Nobuyoshi; Motoi, Toru; Kawai, Akira

    2016-03-01

    The CIC gene rearrangement exists in a subset of small round cell sarcomas. As the nosologic relationship of these sarcomas to Ewing sarcomas remains undetermined, we examined 20 CIC-rearranged sarcomas to compare their clinicopathologic features with those of Ewing sarcomas. The CIC-rearranged sarcomas were from a group of 14 men and 6 women with a median age of 24.5 years. The primary tumor sites included the limbs, trunk wall, internal trunk, lung, cerebrum, and pharynx. A comparison of the demographic and clinical characteristics of the 20 patients with CIC-rearranged sarcomas with those of the 53 near-consecutive patients with EWSR1-rarranged Ewing sarcomas showed that there were no differences with respect to their ages and sexes. Although none of the CIC-rearranged sarcomas arose in the bone, 40% of the Ewing sarcomas primarily affected the skeleton. The overall survival of patients with Ewing sarcomas was significantly better than that for patients with CIC-rearranged sarcomas. A histologic comparison of the CIC-rearranged sarcomas with 20 EWSR1-rearranged Ewing sarcomas showed significantly higher degrees of lobulation, nuclear pleomorphism, the prominence of the nucleoli, spindle cell elements, and myxoid changes in the CIC-rearranged sarcomas. Distinguishing immunohistochemical features included heterogenous CD99 reactivity, nuclear WT1 expression, and calretinin expression in the CIC-rearranged sarcomas and NKX2.2 expression in the Ewing sarcomas. CIC-rearranged sarcomas are distinct from Ewing sarcomas clinically, morphologically, and immunohistochemically, and they should be considered a separate entity rather than being grouped within the same family of tumors. PMID:26685084

  7. Complete and safe resection of challenging retroperitoneal tumors: anticipation of multi-organ and major vascular resection and use of adjunct procedures

    PubMed Central

    2011-01-01

    Background Retroperitoneal tumors are often massive and can involve adjacent organs and/or vital structures, making them difficult to resect. Completeness of resection is within the surgeon's control and critical for long-term survival, particularly for malignant disease. Few studies directly address strategies for complete and safe resection of challenging retroperitoneal tumors. Methods Fifty-six patients representing 63 cases of primary or recurrent retroperitoneal tumor resection between 2004-2009 were identified and a retrospective chart review was performed. Rates of complete resection, use of adjunct procedures, and perioperative complications were recorded. Results In 95% of cases, complete resection was achieved. Fifty-eight percent of these cases required en bloc multi-organ resection, and 8% required major vascular resection. Complete resection rates were higher for primary versus recurrent disease. Adjunct procedures (ureteral stents, femoral nerve monitoring, posterior laminotomy, etc.) were used in 54% of cases. Major postoperative complications occurred in 16% of cases, and one patient died (2% mortality). Conclusions Complete resection of challenging retroperitoneal tumors is feasible and can be done safely with important pre- and intraoperative considerations in mind. PMID:22054416

  8. 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

  9. [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. PMID:26004546

  10. Synovial sarcoma specific translocation associated with both epithelial and spindle cell components.

    PubMed

    Birdsall, S; Osin, P; Lu, Y J; Fisher, C; Shipley, J

    1999-08-12

    The biphasic subtype of synovial sarcoma consists of both epithelial and spindle cell components. To address the relationship between the different cellular components found in synovial sarcoma, we deduced the presence of the synovial sarcoma-specific der(X)t(X;18)(p11.2;q11.2), and involvement of the SSX1 gene in both the epithelial/glandular and spindle cell components of 3 biphasic synovial sarcomas with wide ranging proportions of each of the 2 elements. This has been achieved using a 2-color fluorescence in situ hybridization (FISH) methodology that we had developed recently for analysis paraffin-embedded material. The presence of the rearrangement could be deduced in histologically defined regions and the results showed clearly that the rearrangement was present in both cellular components. This finding provides direct genetic evidence for biphasic synovial sarcomas being clonal and truly biphasic. PMID:10404078

  11. An increased incidence of Hodgkin's lymphoma in patients with adult-onset sarcoma

    PubMed Central

    2012-01-01

    Background Sarcomas are rare, often fatal malignancies of connective tissues that can occur in genetic predisposition syndromes or result from carcinogen exposure. Hodgkin's lymphoma (HL) is not known to contribute to any recognised familial cancer syndrome comprising sarcomas, but is known to be associated with a variety of second cancers, including sarcomas. This study describes the prevalence of HL in families affected by sarcoma. Methods The International Sarcoma Kindred Study (ISKS) is a prospective cohort of 561 families ascertained via a proband with adult-onset sarcoma. Cancer-specific standardised incidence ratios (SIR) for multiple primary malignancies in probands were estimated. Clinical characteristics of individuals reporting both sarcoma and HL were described. Standardised incidence ratios for the occurrence of cancer in ISKS families were also estimated. Results Multiple primary cancers were reported in 16% of probands, significantly higher than in the general population. The risk of HL in probands was increased 15.8-fold (95%CI 7.9-31.6) and increased risks were also seen for breast cancer (SIR 2.9, 95%CI 1.9-4.4) and thyroid cancer (SIR 8.4, 95%CI 4.2-16.8). In 8 probands with both HL and sarcoma, the diagnosis of HL preceded that of sarcoma in 7 cases, and occurred synchronously in one case. Only 3 cases of sarcoma occurred in or close to prior radiotherapy fields. The overall incidence of HL in the ISKS cohort was not significantly increased by comparison with age- and gender-specific population estimates (SIR 1.63, 95%CI 1.05-2.43), suggesting that the association between HL and sarcomas did not extend to other family members. The age of onset of non-sarcoma, non-HL cancers in families affected by both HL and sarcoma was younger than the general population (56.2 y vs 65.6 y, P < 0.0001). Conclusions The basis for the association between HL and sarcomas may include the carcinogenic effects of therapy combined with excellent survival rates for HL

  12. 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

  13. Intraneural Extension of Synovial Sarcoma: Exceptional, or Simply Underrecognized?

    PubMed

    Ng, Wen; Thway, Khin

    2015-12-01

    Intraneural extension of soft tissue sarcomas is uncommon; it is most frequently seen in malignant peripheral nerve sheath tumor, but its occurrence is exceptional in synovial sarcoma. We describe a case arising extraneurally within the deep soft tissues of the forearm, which recurred and resulted in above-elbow amputation, revealing an unexpected finding of diffuse intraneural extension of tumor within a macroscopically normal major nerve. Despite macroscopic and microscopically clear soft tissue margins, the neoplasm had "traveled" a significant distance intraneurally to involve the neural resection margin. This feature does not appear to have been described before; it highlights the issue of whether intraneural spread of synovial sarcoma might have been previously underrecognized, and we discuss briefly some practical implications. PMID:26215219

  14. Extraosseous Ewing Sarcoma: Diagnosis, Prognosis and Optimal Management.

    PubMed

    Galyfos, George; Karantzikos, Georgios A; Kavouras, Nikolaos; Sianou, Argiri; Palogos, Konstantinos; Filis, Konstantinos

    2016-02-01

    Extraosseous Ewing sarcomas (EESs) are rare tumours originating from soft tissues. Their clinical picture depends mainly on the primary site of the sarcoma. Patient characteristics and outcomes seem to be different in EES compared to patients with skeletal Ewing sarcoma, with implications for patient care and prognosis. However, multimodality therapeutic strategies are recommended for all types of the Ewing tumour family. The available diagnostic tools include ultrasonographic evaluation and computed tomography (CT) or magnetic resonance imaging as well as histopathologic and immunohistochemical tissue examination. Several histologic and genetic biomarkers have been established, although their utilization needs to be further tested by larger prospective studies. Regarding localized disease, the recommended treatment remains surgery. However, chemotherapy can be added to achieve improved survival, with neoadjuvant regimens showing more promising results than adjuvant regimens. Radiotherapy is an option to obtain local control, although its complications have reduced its utilization. In metastatic or recurrent disease, systematic chemotherapy improves survival. PMID:27186040

  15. Epithelioid sarcoma of the spine: case report and literature review.

    PubMed

    Chamadoira, Clara; Pereira, Paulo; Silva, Pedro S; Castro, Ligia; Vaz, Rui

    2014-01-01

    Epithelioid sarcomas are rare mesenchymal neoplasms mainly arising in the limbs of young adults. We report the case of a 24-year-old male presenting low back pain radiating to both lower limbs, constipation and urinary retention. The MRI scan showed an intraspinal lesion extending from L4 to S2. Surgery resulted in gross total removal of the extradural lesion and partial removal of the intradural component. The immunohistological study of the lesion was consistent with an epithelioid sarcoma. The patient was submitted to radiotherapy and chemotherapy, but a local recurrence of the lesion and dissemination along the neuraxis were observed 3 months after surgery. Despite treatment, the patient died 4 months after the surgical procedure due to multiorgan failure. Despite there being isolated reports of epithelioid sarcomas appearing in the spine, this is, to our knowledge, the first case with intradural extension. PMID:24674671

  16. [Chylous ascites following radical nephrectomy and retroperitoneal lymphadenectomy].

    PubMed

    Rodríguez Alonso, Andrés; González Blanco, Alfonso; Barbagelata López, Alfonso; Bonelli Martín, Carlos; Fernández López, María; Cuerpo Pérez, Miguel A

    2009-02-01

    Chylous ascites consists of the accumulation of chyle in the abdominal cavity. Postoperative presentation develops as a consequence of unrecognized injury of cisterna chyli or one of its major lumbar tributaries. It usually present as abdominal distention and pain or drainage of milky fluid from surgical wound or abdominal drain. Diagnosis is established by cytochemical analysis of fluid and staining with Sudan III, that shows fat globules, leukocytes with lymphocytic predominance and a high triglyceride content. We present a case of postoperative chylous ascites following radical nephrectomy and retroperitoneal lymphadenectomy for renal cancer, which successfully respond to conservative measures: total parenteral nutrition and octreotide. PMID:19418845

  17. Retroperitoneal nodular fasciitis: magnetic resonance imaging (MRI) and pathological features.

    PubMed

    Meduri; Zuiani; Del Frate C; Bazzocchi

    1998-07-01

    A case of pelvic nodular fasciitis, with particular reference to its peculiar radiological and pathological features is described. Only a few cases of pelvic nodular fasciitis are reported in the English literature and at the best of our knowledge, this is the first case of retroperitoneal origin. This report discusses the role of MRI in the characterization of soft tissue masses. No specific MRI findings of nodular fasciitis were identified and MRI doesn't add any contribution to the differential diagnosis between benign and malignant lesions. As a consequence, the histopathological examination is necessary for a definitive diagnosis. PMID:10358366

  18. [A case of cap polyposis complicated with idiopathic retroperitoneal fibrosis].

    PubMed

    Song, Limhwa; Jhun, Byung Woo; Park, Jihyeon; Kim, Damin; Chang, Dong Kyung; Kim, Young Ho; Kim, Jae Jun; Kim, Jin Yong

    2011-11-25

    An optimal treatment for cap polyposis has not been established. Several treatment approaches, including anti-inflammatory agents, antibiotics, immunomodulators, and endoscopic therapy have been described. Surgical resection of the affected colon and rectum may be indicated for patients with persistent disease. Repeat surgery is indicated in cases of recurrence after surgery. However, symptomatic polyposis may still recur, and spontaneous resolution of cap polyposis is possible. We report a case of recurrent cap polyposis complicated with retroperitoneal fibrosis after inadequate low anterior resection with a positive resection margin. Surgical approaches for the treatment of cap polyposis should be carefully considered before treatment. PMID:22113045

  19. Immunotherapy in a rare case of primary pelvic retroperitoneal melanoma.

    PubMed

    Talag, Maria Monica; Alsharedi, Mohamed; Bou Zgheib, Nadim; Lebowicz, Yehuda

    2016-01-01

    Recent advances in novel immunotherapeutic and targeted therapeutic agents have increased treatment options in patients with advanced metastatic melanoma. However, evidence in the literature on whether or not extracutaneous melanoma will acquire an equivalent advantage from these therapies is very scarce. In general, extracutaneous melanomas are rare and aggressive melanomas, which are clinically and biologically unique, with higher incidence of metastatic disease and poor prognosis. In this case report, we present a very rare case of a 54-year-old woman with primary pelvic retroperitoneal melanoma treated with an anti-PD-1 antibody, pembrolizumab. Furthermore, we explore the role of novel immunotherapies in the treatment of advanced melanoma. PMID:27624447

  20. New data on diagnosis and medical treatment of retroperitoneal fibrosis.

    PubMed

    Oosterlinck, W; Derie, A

    1997-06-01

    A review of the literature of the last 5 years on retroperitoneal fibrosis is given. MRT seems to add to the exact extend of the disease and galliumscintigraphy can give new information on the activity of the process and the usefulness of medical therapy. The use of corticosteroid or other immunosuppressive drugs such a azathioprine, again is confirmed. Other drugs mentioned in the medical therapy are methotrexate, cyclophosfamide and penicillamine. A few cases responded well to tamoxifen, a drug which was already used in therapy of desmoid tumours. Medical treatment is anyhow superior to surgery alone. PMID:9287431

  1. 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

  2. Head and Neck Sarcomas: Analysis of the SEER Database

    PubMed Central

    Peng, Kevin A.; Grogan, Tristan; Wang, Marilene B.

    2015-01-01

    Objective To summarize the epidemiology of sarcomas occurring in the head and neck and identify prognostic factors for patient survival. Study Design and Setting Cross-sectional analysis of the National Cancer Institute’s Surveillance, Epidemiology and End Results (SEER) program. Methods The SEER 18 registries, comprising sarcoma diagnoses made from 1973 to 2010, were queried for sarcomas arising in the head and neck. Pediatric and adult patients were analyzed separately, and multivariate and propensity-matched analyses were performed to identify predictors of disease-specific survival. Results In all, 11,481 adult cases and 1244 pediatric cases were identified. In adults, the most common histologic subtypes were malignant fibrous histiocytoma (MFH), Kaposi sarcoma, and hemangiosarcoma, while in the pediatric cohort, the most common histologic subtypes were rhabdomyosar-coma, MFH, and osteosarcoma. Cause-specific 2-, 5-, and 10-year survival rates were 76%, 66%, and 61% for adults and 84%, 73%, and 71% for pediatric patients. Multivariate analysis performed for adults revealed that male gender, absence of radiation therapy, and stage I disease were associated with improved cause-specific survival reaching statistical significance. However, a propensity-matched model demonstrated no significant difference in cause-specific survival between patients who received radiation and those who did not. Conclusion Sarcomas, a heterogeneous group of malignant mesenchymal tumors, are uncommonly found in the head and neck. This study represents the largest analysis of patients with head and neck sarcomas in the literature and demonstrates the impact of age, gender, primary site, histology, and radiation status on overall prognosis. PMID:25135525

  3. A Case of Clear Cell Sarcoma Occurring on the Abdomen

    PubMed Central

    Jung, Suck Joon; Seung, Na Reu; Park, Eun Ju; Kim, Chul Woo; Kim, Kwang Ho

    2008-01-01

    Clear cell sarcoma is a rare malignant tumor representing about 1% of soft tissue tumors. It usually presents in the distal extremities of young adults, frequently attached to tendons or aponeuroses. This slowly progressive tumor tends to recur and results in eventual development of metastatic growth. Early recognition of the disease and prompt wide excision of tumor are essential to get a favorable outcome. We report a rare case of clear cell sarcoma in a 57 year-old female who presented with an erythematous hard nodule on her abdomen. PMID:27303159

  4. Evaluation of Costimulatory Molecules in Peripheral Blood Lymphocytes of Canine Patients with Histiocytic Sarcoma

    PubMed Central

    Tagawa, Michihito; Maekawa, Naoya; Konnai, Satoru; Takagi, Satoshi

    2016-01-01

    Histiocytic sarcoma is a rapidly progressive and fatal neoplastic disease in dogs. It is unclear whether costimulatory molecules, including CD28, cytotoxic T-lymphocyte-associated antigen-4 (CTLA-4), and programmed death-1 (PD-1), are expressed on peripheral blood lymphocytes (PBLs) of canine patients with histiocytic sarcoma. The objective of this study was to evaluate the expression of CD28, CTLA-4, and PD-1 molecules on PBLs of patients with histiocytic sarcoma, patients with other tumors, and healthy controls. Twenty-six dogs were included in the study, with eight, ten, and eight dogs in the histiocytic sarcoma, other tumor, and healthy control groups, respectively. PBLs and serum were prospectively obtained from patients diagnosed histopathologically with histiocytic sarcoma, other tumors and healthy controls. The surface expression of CTLA-4, CD28, and PD-1 on T lymphocytes was examined using flow cytometric analysis. Serum samples were frozen at −30°C until serum interferon-γ (IFN-γ) was measured by enzyme-linked immunosorbent assay. The expression level of CTLA-4 on CD4+ lymphocytes was significantly higher in the histiocytic sarcoma group than in the control group. The expression of CTLA-4 on CD8+ lymphocytes was significantly higher in the histiocytic sarcoma group than in the other two groups. In addition, the expression of PD-1 on CD8+ lymphocytes was significantly higher in the histiocytic sarcoma group than in the control group. However, no significant differences in CD28 expressions and serum IFN-γ levels were observed. The present results provided evidence showing that the expression levels of CTLA-4 on both CD4+ and CD8+ lymphocytes and PD-1 on CD8+ lymphocytes in peripheral blood obtained from dogs with histiocytic sarcoma were upregulated. The overexpressions of CTLA 4 and PD-1 suggested that antitumor immunity may be suppressed in dogs with histiocytic sarcoma. PMID:26901565

  5. Management of Sarcoma Metastases to the Lung.

    PubMed

    Digesu, Christopher S; Wiesel, Ory; Vaporciyan, Ara A; Colson, Yolonda L

    2016-10-01

    For decades, surgical resection of pulmonary metastases has been performed; despite limited randomized data, surgery is increasingly accepted as an integral part in the management of metastatic disease. Long-term results indicate resection is potentially curative with significantly improved survival following complete resection. Recurrence, however, is not uncommon with many patients undergoing repeat resection. With advancing surgical technique and adjuvant therapies, patients with high or recurrent tumor burden are increasingly afforded disease control and potential cure. In this review, the prognostic characteristics of pulmonary metastases from sarcoma, preoperative evaluation, operative technique, long-term outcomes, and management of complex patients are highlighted. PMID:27591495

  6. 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. PMID:19635748

  7. Immunotherapy in Sarcoma: Future Horizons.

    PubMed

    Burgess, Melissa; Gorantla, Vikram; Weiss, Kurt; Tawbi, Hussein

    2015-11-01

    Immunologic approaches to cancer are over a century old. Over the years, the strategy has been fine-tuned from inciting infections in subjects to inhibiting negative regulatory signals from the innate immune system. Sarcomas are among the first tumors to be considered for immune interventions. From Coley's toxin to cytokine-based therapies to adoptive cell therapy, there have been numerous immunotherapeutic investigations in this patient population. A promising strategy includes adoptive T cell therapy which has been studied in small cohorts of synovial sarcoma, a subtype that is known to widely express the cancer testis antigen, NY-ESO-1. Additionally, recent data in metastatic melanoma and renal cell carcinoma demonstrate the utility and tremendous efficacy of immune checkpoint blockade with increased rates of durable responses compared to standard therapies. Responses in traditionally "non-immunogenic" tumors, such as lung and bladder cancers, provide ample rationale for the study of immune checkpoint inhibitors in sarcoma. While immunotherapy has induced some responses in sarcomas, further research will help clarify optimal patient selection for future clinical trials and new combinatorial immunotherapeutic strategies. PMID:26423769

  8. Kaposi's sarcoma in South Africa.

    PubMed

    Sitas, F; Newton, R

    2001-01-01

    Kaposi's sarcoma was endemic in South Africa even before the advent of the human immunodeficiency virus (HIV). Between 1988 and 1996, the incidence of Kaposi's sarcoma in South Africa has risen at least threefold and continues to increase as the HIV epidemic grows. Research from South Africa has shown that infection with human herpesvirus 8 (HHV8) is associated with Kaposi's sarcoma but not with any other major cancer site or type. In addition, the risk of Kaposi's sarcoma increases with increasing antibody titer to HHV8, but, for a given titer, the risk is greater in HIV-seropositive compared with HIV-seronegative individuals. The age- and sex-standardized seroprevalence of HHV8 in black South African hospital patients was found to be slightly more than 30%; the seroprevalence of HHV8 increased with age and was similar in men and in women. The modes of transmission of HHV8 are yet to be fully elucidated. Limited evidence exists for sexual transmission in black South African adults, but mother-to-child and person-to-person transmission in childhood is also likely. Furthermore, the seroprevalence of HHV8 decreases with increasing levels of education and is lower in whites than in blacks, suggesting that factors associated with poverty may be important determinants of transmission. Future research should focus on risk factors for Kaposi's sarcoma in HHV8-infected individuals, on determinants and mode of transmission of HHV8, and on the elucidation of the effect of primary HHV8 infection in adults and in children. PMID:11158199

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

    ClinicalTrials.gov

    2016-09-09

    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

  10. 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

  11. Spontaneous appendico-cutaneous fistula: an unusual presentation of retroperitoneal appendicular perforation associated with psoas abscess.

    PubMed

    Singh, Mahendra Pratap; Samaiya, Atul; Sainia, Tarun Kumar; Saxena, Agamya

    2016-01-01

    Spontaneous appendico-cutaneous fistula is an extremely rare complication of appendicitis and only a few cases are reported in literature. We present one such case in a 65-year-old diabetic male who had recurrent atypical abdominal pain for 32 years. The patient also had recurrent right psoas abscess, which had failed to respond to a repeated surgical drainage, antibiotics and anti-tubercular treatment. Patient presented to us with recurrent discharging sinus in right lumbar region since 6 months. Multi-detector computed tomography (MDCT) suspected the appendicitis as a possible cause. On laparotomy, retroperitoneal perforated appendix tip was found communicating with the fistulous tract. Appendectomy with excision of fistulous tract resulted in cure. To prevent inappropriate treatment and complication in such atypical presentation of appendicitis, high index of suspicion is required and MDCT is a very useful tool for making correct diagnosis. PMID:27340228

  12. Spontaneous appendico-cutaneous fistula: an unusual presentation of retroperitoneal appendicular perforation associated with psoas abscess

    PubMed Central

    Singh, Mahendra Pratap; Samaiya, Atul; Sainia, Tarun Kumar; Saxena, Agamya

    2016-01-01

    Spontaneous appendico-cutaneous fistula is an extremely rare complication of appendicitis and only a few cases are reported in literature. We present one such case in a 65-year-old diabetic male who had recurrent atypical abdominal pain for 32 years. The patient also had recurrent right psoas abscess, which had failed to respond to a repeated surgical drainage, antibiotics and anti-tubercular treatment. Patient presented to us with recurrent discharging sinus in right lumbar region since 6 months. Multi-detector computed tomography (MDCT) suspected the appendicitis as a possible cause. On laparotomy, retroperitoneal perforated appendix tip was found communicating with the fistulous tract. Appendectomy with excision of fistulous tract resulted in cure. To prevent inappropriate treatment and complication in such atypical presentation of appendicitis, high index of suspicion is required and MDCT is a very useful tool for making correct diagnosis. PMID:27340228

  13. 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

  14. What's New in Soft Tissue Sarcomas Research and Treatment?

    MedlinePlus

    ... Topic Additional resources for soft tissue sarcoma What`s new in soft tissue sarcoma research and treatment? Research ... develop. This information is already being applied to new tests to diagnose and classify sarcomas. This is ...

  15. 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

  16. 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

  17. Diagnosis and Management of Hereditary Sarcoma.

    PubMed

    Thomas, David M; Ballinger, Mandy L

    2016-01-01

    Sarcomas are rare and heterogeneous diseases that affect a younger population than most epithelial cancers. Epidemiologic studies suggest a strong genetic component to sarcomas, and many familial cancer syndromes have been described, in which sarcomas are a feature. The best known of these are the Li-Fraumeni and retinoblastoma syndromes, study of which has been pivotal to elucidating the molecular basis for the cell response to DNA damage and the cell division. Although much has been learnt about cancer biology from the study of sarcoma families, in general clinical management of increased sarcoma risk has lagged behind other cancer predisposition syndromes. With the advent of genomic tools for genetic testing, it is likely that a substantial fraction of sarcoma patients will be identified as carriers of known risk alleles. The translation of this knowledge into effective risk management programs and cancer treatments will be essential to changes in routine clinical practice. PMID:27075354

  18. MRI, enhanced CT, and FDG PET/CT in primary retroperitoneal mucinous cystadenocarcinoma.

    PubMed

    Dong, Aisheng; Zhai, Zhijun; Wang, Yang; Zuo, Changjing

    2015-01-01

    Primary retroperitoneal mucinous cystadenocarcinoma (PRMC) is an extremely rare neoplasm. We present a case of PRMC with MRI, enhanced CT, and FDG PET/CT findings. Abdominal MRI showed a cystic lesion in the retroperitoneum with a mural nodule. The mural nodule showed progressive enhancement on enhanced CT and intense FDG uptake on early PET/CT with increased SUVmax on delayed PET/CT. Laparoscopy was performed. Retroperitoneal mucinous cystadenocarcinoma was confirmed histopathologically. Metastasis from gastrointestinal tract or ovary was excluded. This case indicates, although rare, PRMC should be considered when a hypermetabolic retroperitoneal cystic lesion with bilateral normal ovaries is found on FDG PET/CT. PMID:24445275

  19. Retroperitoneal and pleural fibrosis in an insulator working in power plants.

    PubMed

    Uibu, Toomas; Jäntti, Matti; Järvenpää, Ritva; Oksa, Panu; Tossavainen, Antti; Vanhala, Esa; Roto, Pekka

    2009-01-01

    We describe a case history of a former insulator who developed concomitant retroperitoneal and pleural fibrosis. In his work, the patient had been exposed on a daily basis to asbestos dust while demolishing and installing pipeline insulations. The heavy asbestos exposure was confirmed by a high level of asbestos content in his autopsy lung sample. We propose that both retroperitoneal fibrosis and diffuse pleural thickening were induced in our patient by an abundant amount of amphibole asbestos fibres found in his lung and retroperitoneal tissues. PMID:21686706

  20. 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.

  1. 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

  2. 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.

  3. Pancreaticoduodenectomy With or Without Distal Gastrectomy and Extended Retroperitoneal Lymphadenectomy for Periampullary Adenocarcinoma, Part 2

    PubMed Central

    Yeo, Charles J.; Cameron, John L.; Lillemoe, Keith D.; Sohn, Taylor A.; Campbell, Kurtis A.; Sauter, Patricia K.; Coleman, JoAnn; Abrams, Ross A.; Hruban, Ralph H.

    2002-01-01

    Objective To evaluate, in a prospective, randomized single-institution trial, the end points of operative morbidity, operative mortality, and survival in patients undergoing standard versus radical (extended) pancreaticoduodenectomy. Summary Background Data Numerous retrospective reports and a few prospective randomized trials have suggested that the performance of an extended lymphadenectomy in association with a pancreaticoduodenal resection may improve survival for patients with pancreatic and other periampullary adenocarcinomas. Methods Between April 1996 and June 2001, 299 patients with periampullary adenocarcinoma were enrolled in a prospective, randomized single-institution trial. After intraoperative verification (by frozen section) of margin-negative resected periampullary adenocarcinoma, patients were randomized to either a standard pancreaticoduodenectomy (removing only the peripancreatic lymph nodes en bloc with the specimen) or a radical (extended) pancreaticoduodenectomy (standard resection plus distal gastrectomy and retroperitoneal lymphadenectomy). All pathology specimens were reviewed, fully categorized, and staged. The postoperative morbidity, mortality, and survival data were analyzed. Results Of the 299 patients randomized, 5 (1.7%) were subsequently excluded because their final pathology failed to reveal periampullary adenocarcinoma, leaving 294 patients for analysis (146 standard vs. 148 radical). The two groups were statistically similar with regard to age (median 67 years) and gender (54% male). All the patients in the radical group underwent distal gastric resection, while 86% of the patients in the standard group underwent pylorus preservation (P < .0001). The mean operative time in the radical group was 6.4 hours, compared to 5.9 hours in the standard group (P = .002). There were no significant differences between the two groups with respect to intraoperative blood loss, transfusion requirements (median zero units), location of primary

  4. Clinical and pathological characteristics of adrenal lymphangioma treated by laparoscopy via a retroperitoneal approach: experience and analysis of 7 cases

    PubMed Central

    Gao, Liang; Zhang, Shu; Wang, Huan; Qiu, Yan; Yang, Lu; Yuan, Jiuhong; Wei, Qiang; Han, Ping

    2015-01-01

    To describe the clinical and pathological characteristics of adrenal lymphangioma (AL) and share our experiences of the treatment of AL with retroperitoneal laparoscopic surgery. All patients pathologically diagnosed with AL were examined. The clinical and pathological characteristics, process of diagnosis, and preparation and treatment of all patients, especially patients treated with laparoscopic surgery, were summarized and retrospectively analyzed. From January 2008 to May 2014, 8 patients underwent adrenalectomies and were diagnosed with AL in our hospital. The median age was 45.5 years. All of these patients experienced a smooth adrenalectomy: 7 performed by laparoscopy via a retroperitoneal approach and 1 performed by open surgery. Five were female and the other 3 were male. These patients had unilateral adrenal lesions. Four were located on the right which to be same as the contralateral. In addition, 1 specimen was assayed by immunohistochemistry (IHC), which revealed positive results for CD31, CD34, Factor VIII-related antigen and D2-40, and negative results for cytokeratin AE1/AE3. During a brief follow up, all patients exhibited favorable results without discomfort. AL is a benign lesion with mild bio-behavior and patients are generally asymptomatic. The use of computerized tomography (CT) combined with enhanced CT has a superior advantage in diagnosis. Laparoscopic adrenalectomies that are performed via a retroperitoneal approach would be a very safe and efficient choice for AL treatment. D2-40 can be considered as a specific IHC marker in the pathological diagnosis of AL. However, pheochromocytoma and adrenal tuberculosis should be ruled out before and during the operation. PMID:26064332

  5. Gemcitabine-based chemotherapy in sarcomas: A systematic review of published trials.

    PubMed

    Ducoulombier, Agnès; Cousin, Sophie; Kotecki, Nuria; Penel, Nicolas

    2016-02-01

    Gemcitabine is largely used in the management of sarcomas. We have systematically reviewed all of the fully published trials that investigated a gemcitabine-based regimen in the management of sarcomas and then provided a grade of recommendations and a level of evidence for every recommendation. Because of conflicting results from successive non-randomized phase II trials, gemcitabine activity alone in unselected pretreated soft tissue sarcomas could not be properly assessed. Gemcitabine alone and gemcitabine-docetaxel appeared to both be active in pretreated uterine and non-uterine leiomyosarcoma (1B;I). Gemcitabine-dacarbazine appeared to be active in pretreated unselected soft tissue sarcomas (1B;I). According the GeDDIS phase III trial (not yet fully published), gemcitabine-docetaxel appeared slightly less active than doxorubicine and more toxic than doxorubicine in chemo-naïve metastatic soft tissue sarcoma patients. Because of the absence of controlled randomized trials, the benefit of gemcitabine-docetaxel as an adjuvant treatment in high-grade uterine leiomyosarcoma could not be appropriately assessed. The level of activity of gemcitabine/docetaxel in bone sarcomas cannot be ascertained with the available data. The level of evidence supporting the use of gemcitabine-based regimens in sarcoma management is limited. Confirmatory phase III trials are warranted when phase II trials suggest some preliminary activity. PMID:26555460

  6. 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. PMID:19555393

  7. Reliable Establishment of Human Sarcoma Xenografts in the Nude Rat

    PubMed Central

    Tinkey, Peggy T.; Milas, Mira

    1999-01-01

    Purpose. The ability to establish consistent human tumor xenografts in experimental animals is a crucial part of preclinical investigations.The goal of this study was to develop a method of establishing a human tumor xenograft in the leg of a nude rat for evaluation of new surgical and molecular methods of treatments of human extremity sarcoma. Methods and results. Initial attempts to produce sarcoma nodules by subcutaneous injection of a human leiomyosarcoma tumor cell suspension (SKLMS-1) resulted in tumor nodule formation in only four of 10 sites (40%).The xenograft method was modified to include younger nude rats of a different source and substrain (HSD:rnu/rnu, 5–9 weeks old), treated with 500 cGy whole-body irradiation, and the transplantation of tumor cells or small tumor fragments which had been embedded in Matrigel.These changes improved the tumor take rate per site to 52/52 (100%).Tumor nodules demonstrated rapid and progressive growth and histological features consistent with the original human sarcoma. Discussion. Successful human leiomyosarcoma establishment in these nude rats permits the investigation of sarcoma biology and treatment with surgical procedures for which a mouse model would be inadequate. In this study we identified modifications in technique which enhanced the xenografting of a leiomyosarcoma cell line in nude rats; these techniques may increase tumor take rates for other tumor types as well. PMID:18521275

  8. Sorafenib in advanced, heavily pretreated patients with soft tissue sarcomas.

    PubMed

    Brämswig, Kira; Ploner, Ferdinand; Martel, Alexandra; Bauernhofer, Thomas; Hilbe, Wolfgang; Kühr, Thomas; Leitgeb, Clemens; Mlineritsch, Brigitte; Petzer, Andreas; Seebacher, Veronika; Stöger, Herbert; Girschikofsky, Michael; Hochreiner, Gerhard; Ressler, Sigrun; Romeder, Franz; Wöll, Ewald; Brodowicz, Thomas

    2014-08-01

    Therapeutic options for patients with advanced pretreated soft tissue sarcomas are limited. However, in this setting, sorafenib has shown promising results. We reviewed the data of 33 patients with soft tissue sarcoma treated with sorafenib within a named patient program in Austria. Twelve physicians from eight different hospitals provided records for the analysis of data. Among the 33 patients, the predominant histological subtype of sarcoma was leiomyosarcoma (n=18, 55%). Other subtypes were represented by only one or two cases. Fifteen patients presented with metastases at the time of diagnosis. Another 17 patients developed metastases later in the course of the disease (data on one patient are missing). Most of the 33 patients had undergone resection of the primary (n=29, 88%) and half of the patients had received radiotherapy (n=17, 52%). Chemotherapy for metastatic disease had been administered to 30 patients (91%). The majority had received two or more regimens of chemotherapy (n=25, 76%) before sorafenib treatment. The use of sorafenib resulted in a median time to treatment failure of 92 days in patients with leiomyosarcoma and 45 days in patients with other histological subtypes. One-third of the patients derived benefits from treatment: four patients were documented with partial response and six with stabilized disease. In terms of treatment-related toxicity, skin problems of various degrees and gastrointestinal disturbances were frequently reported. In this retrospective analysis of heavily pretreated patients with advanced soft tissue sarcomas, sorafenib was associated with some antitumor activity and an acceptable toxicity profile. PMID:24667659

  9. Penile Sarcoma: Report of a Rare Malignancy

    PubMed Central

    Kumar, Vijay; Chaturvedi, Arun; Vishnoi, Jeevan Ram; Dontula, Prashant

    2016-01-01

    Penile cancer is an uncommon malignancy. Squamous cell carcinoma constitutes approximately 95% of all histology. Non-squamous malignancies are rare in penis. Sarcomas of penis are rarer among them. Spindle cell sarcoma is one of the extremely rare sarcoma of penis. To best of our knowledge, only two cases have been reported so far, one in English literature and other in Japanese. We are presenting this uncommon case of spindle cell sarcoma of penis, which was diagnosed with microscopy with its characteristic immunohistochemistry. The disease had an aggressive course with multiple recurrences in a short duration despite margin negative resection. Disease responded poorly with the chemotherapy and patient succumbed to the disease.

  10. Pleomorphic malignant fibrous histiocytoma/undifferentiated pleomorphic sarcoma of the glans penis

    PubMed Central

    Seo, Ill Young; Oh, Tae Hoon; Chuluun, Sarantulga; Choi, Keum Ha

    2013-01-01

    Primary sarcoma of the penis has an extremely low incidence, and its diagnosis and treatment are unclear. A 55-year-old man presented with an oval-shaped mass of the glans penis, which was treated by a wide excision. The pathologic result revealed an undifferentiated high-grade sarcoma, which was diagnosed as a pleomorphic malignant fibrous histiocytoma/undifferentiated pleomorphic sarcoma based on the World Health Organization classification. There was no recurrence at the12-months postoperative follow-up. PMID:24475005

  11. 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. PMID:24167396

  12. 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

  13. [Sarcomas of the small intestine].

    PubMed

    Beyrouti, M L; Abid, M; Beyrouti, R; Ben Amar, M; Gargouri, F; Frikha, F; Affes, N; Boujelbene, S; Ghorbel, A

    2005-03-12

    Sarcomas of the small intestine are rare, clearly differentiated, malignant, mesenchymatous tumours that can be of smooth muscle, Schwann cell or fibroblastic origin. From a clinical point of view, the pain and abdominal mass are the 2 types of symptoms that frequently reveal the disease. In rare cases, sarcomas of the small intestine are manifested by an acute complication. No imaging method can clearly confirm the diagnosis. Before immunohistochemistry, differential diagnosis was made on undifferentiated mesenchymatous "stromal" tumours, which are also rare. Exeresis must be complete and without perforation of the tumour because of the risk of locoregional relapse. The benefits provided by chemotherapy and radiotherapy are limited because of the low mitotic activity of the tumour cells and its weak vascularisation. Long-term survival is limited by poor prognosis criteria: high grade malignancy, size greater than 5 cm, tumour extension, perforation of the tumour, quality of surgical resection and histological type. PMID:15859576

  14. Molecular abnormalities in Ewing's sarcoma.

    PubMed

    Burchill, Susan Ann

    2008-10-01

    Ewing's sarcoma is one of the few solid tumors for which the underlying molecular genetic abnormality has been described: rearrangement of the EWS gene on chromosome 22q12 with an ETS gene family member. These translocations define the Ewing's sarcoma family of tumors (ESFT) and provide a valuable tool for their accurate and unequivocal diagnosis. They also represent ideal targets for the development of tumor-specific therapeutics. Although secondary abnormalities occur in over 80% of primary ESFT the clinical utility of these is currently unclear. However, abnormalities in genes that regulate the G(1)/S checkpoint are frequently described and may be important in predicting outcome and response. Increased understanding of the molecular events that arise in ESFT and their role in the development and maintenance of the malignant phenotype will inform the improved stratification of patients for therapy and identify targets and pathways for the design of more effective cancer therapeutics. PMID:18925858

  15. The role of multidetector CT in local staging and evaluation of retroperitoneal surgical margin involvement in colon cancer

    PubMed Central

    Elibol, Funda Dinç; Obuz, Funda; Sökmen, Selman; Terzi, Cem; Canda, Aras Emre; Sağol, Özgül; Sarıoğlu, Sülen

    2016-01-01

    PURPOSE We aimed to evaluate preoperative T and N staging and retroperitoneal surgical margin (RSM) involvement in colon cancer using multidetector computed tomography (MDCT). METHODS In this retrospective study, preoperative MDCTs of 141 patients with colon adenocarcinoma were evaluated in terms of T and N staging and retroperitoneal surgical margin involvement by two observers. Results were compared with histopathology. RESULTS In determining extramural invasion, sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of MDCT were 81%, 50%, 95%, 26%, and 81% for observer 1 and 87%, 75%, 97%, 27%, and 84% for observer 2, respectively. Moderate interobserver agreement was observed (κ=0.425). In determining T stage of the tumor, accuracy of MDCT was 55% for observer 1 and 51% for observer 2. In the detection of lymph node metastasis, sensitivity, specificity, PPV, NPV, and accuracy of MDCT were 84%, 46%, 60%, 74% and 64% for observer 1 and 84%, 56%, 65%, 78%, and 70% for observer 2, respectively. Interobserver agreement was substantial (κ=0.650). RSM was involved in six cases (4.7%). When only retroperitoneal colon segments were considered, 1.6% of subjects demonstrated RSM involvement. Four of the six RSM-positive tumors were located on sigmoid colon and one tumor was on transverse colon and caecum. Considering all colon tumors, in the detection of RSM involvement, sensitivity and specificity of MDCT were 33% and 81% for observer 1 and 50% and 80% for observer 2. Interobserver agreement was moderate (κ=0.518). CONCLUSION MDCT is a promising technique with moderate interobserver agreement in detection of extramural invasion, lymph node metastases, and RSM involvement in colon carcinomas. PMID:26611110

  16. Synovial sarcoma of the foot.

    PubMed

    Bekarev, Mikhail; Elsinger, Elisabeth C; Villanueva-Siles, Esperanza; Borzykowski, Ross M; Geller, David S

    2013-01-01

    We report the case of a 75-year-old male who underwent lung lobectomy for presumed lung cancer. Thereafter, he presented with a painful mass between the third and fourth metatarsal heads in the foot that was assumed to be Morton's neuroma. After extensive oncologic evaluation, the foot mass was diagnosed as a synovial sarcoma. In retrospect, his lung lesion was understood to be metastatic disease. PMID:23632071

  17. Surgical management of pelvic Ewing’s sarcoma

    PubMed Central

    Natarajan, Mayil Vahanan; Sameer, M Mohamed; Bose, Jagdish Chandra; Dheep, Kunal

    2010-01-01

    Background: Despite advances in adjuvant therapy, Ewing’s sarcoma of the pelvis remains an anatomic site with a poor prognosis due to its relative inaccessibility, complex anatomy, and limited reconstructive options available. This study evaluates the role of surgery in the management of patients with pelvic Ewing’s sarcoma who also have received conventional radiation therapy and chemotherapy. Materials and Methods: From July 1990 to July 2006, we received 10 patients with Ewing’s sarcoma of pelvis at our center. Nine patients were in stage II B and one in Stage III at the time of presentation to us. All patients underwent surgical resection after preoperative chemotherapy with or without radiotherapy, which was given at the discretion of the referral center. Reconstruction was attempted using plate osteosynthesis in four patients, SS wires and screws in three patients, free fibular strut graft in one patient, and none was done in two patients. Results: Functional outcome assessed by Enneking’s criteria revealed excellent outcome in two patients, good outcome in five patients, and poor outcome in two patients. At a mean followup of 10.3 years, seven patients remained free from the disease, and three patients died. The 5- and 10-year cumulative survival (Kaplan Meier method) was 63% and 34%, respectively. Conclusion: This study demonstrates that surgery plus chemotherapy and radiation therapy is helpful for treating patients with pelvic Ewing’s sarcoma, particularly in achieving local control. PMID:20924480

  18. Myeloid sarcoma of the Gingiva with myelodysplastic syndrome

    PubMed Central

    Jin, Seong-Ho; Park, Gyeongsin; Ko, Youngkyung; Park, Jun-Beom

    2016-01-01

    Abstract The purpose of this report is to present a case of myeloid sarcoma of the gingiva with myelodysplastic syndrome. A 52-year-old male diagnosed with myelodysplastic syndrome with skin lesions presented with gingival swelling and gingival redness involving the maxillary left second premolar and the maxillary left first molar. The patient was referred from the Department of Hematology for a biopsy of the lesion. Full-thickness flaps were elevated and inflamed, and neoplastic soft tissue was removed from a lesion and the samples sent for histopathologic analysis. Histopathologic results showed leukemic cell infiltration beneath the oral epithelium, and the specimen was positive for the leukocyte marker. The diagnosis was myeloid sarcoma. Uneventful healing was observed at 2-week follow-up, but relapse of the lesions with the hyperplastic and neoplastic tissue was noted at 4-week follow-up. Further follow-up or treatment could not be performed because the patient did not visit at the next follow-up. In conclusion, myeloid sarcoma should be a diagnosis option for gingival growth because it can involve intraoral lesion. In this report, a biopsy was performed due to referral considering the patient's medical history. Although myeloid sarcoma in the oral cavity is extremely rare, a small biopsy and consultation with a hematologist may be beneficial for patients and may provide a differential diagnosis. PMID:27310987

  19. Incidence of sarcoma in patients treated with fast neutrons

    SciTech Connect

    MacDougall, R. Hugh . E-mail: medical.dean@st-andrews.ac.uk; Kerr, Gillian R.; Duncan, William

    2006-11-01

    Purpose: The aim of this study is to report the incidence of soft tissue sarcoma in a large group of patients treated with fast neutrons. Methods: A systematic review was conducted of long-term follow-up after trials of fast neutron therapy for cancers at various sites. The study took place at Edinburgh Cancer Centre, Western General Hospital, Edinburgh, Scotland, United Kingdom. From 1977 to 1984, 620 patients were treated using fast neutrons in the MRC cyclotron unit in Edinburgh. Most of these were treated within randomized controlled trials. Follow-up was maintained in all except 2 patients, who left the area to return abroad. The main outcome measure was the incidence of new soft-tissue sarcomas during long-term follow-up. Results: Three cases of sarcoma, developing within the treatment volume, were observed in a small group of patients treated some years earlier using fast neutrons. This incidence was 111 times what would have been expected in the normal population and 15 times the incidence in a comparable photon-treated group of patients. Conclusion: The long-term incidence of sarcomas in patients previously treated with fast neutrons is significant.

  20. A retroperitoneal enteric duplication cyst communicating with the right upper ureter in an infant

    PubMed Central

    Bal, Harshjeet Singh; Kisku, Sundeep; Sen, Sudipta; Masih, Dipti

    2014-01-01

    We report an extremely rare case of isolated retroperitoneal enteric duplication cyst with gastric mucosa causing haematuria and dysuria by communicating with the urinary system. A 9-month-old male child was admitted to our hospital with persistent haematuria, dysuria and anaemia. Investigations revealed a retroperitoneal cyst abutting the hydronephrotic non-functioning right kidney. At surgery an isolated retroperitoneal cyst communicating with the right pelviureteric junction was found. The kidney and associated cyst were excised. Histology of the cystic lesion revealed an enteric duplication cyst lined by ectopic gastric mucosa. Isolated retroperitoneal enteric duplication cyst communicating with the urinary tract has not been previously reported in the English literature. We propose that acid secretion into the right renal system was the cause of the haematuria–dysuria syndrome which promptly resolved postoperatively. PMID:24813198

  1. Retroperitoneal fibrosis due to B-cell non-Hodgkin lymphoma: Responding to rituximab!

    PubMed

    Alvarez Argote, Juliana; Bauer, Frank A; Posteraro, Anthony F; Dasanu, Constantin A

    2016-02-01

    Retroperitoneal fibrosis is a rare disease manifesting as chronic soft tissue fibrosis in the retroperitoneum, with potential anatomic and/or functional compromise of adjacent organs. It can be primary (idiopathic) or secondary to other conditions such as cancers, autoimmune disorders, or drugs. We report herein a 66-year-old patient with symptomatic retroperitoneal fibrosis leading to bilateral hydronephrosis and renal failure, in whom, after a complex diagnostic work-up and protracted clinical course, a B-cell non-Hodgkin lymphoma in the retroperitoneal space and several vertebral bodies was identified. The patient was treated with radiation therapy and weekly rituximab infusions, with resolution of hydronephrosis and lower back pain. We include a thorough literature review on etiopathogenesis, diagnosis, therapy, and prognosis of retroperitoneal fibrosis. A meticulous search for malignancy is necessary in this rare condition that, if positive, may have significant therapeutic and prognostic implications. PMID:25013186

  2. Radiation-induced Sarcomas Occurring in Desmoid-type Fibromatosis Are Not Always Derived From the Primary Tumor.

    PubMed

    Verschoor, Arie J; Cleton-Jansen, Anne-Marie; Wijers-Koster, Pauline; Coffin, Cheryl M; Lazar, Alexander J; Nout, Remi A; Rubin, Brian P; Gelderblom, Hans; Bovée, Judith V M G

    2015-12-01

    Desmoid-type fibromatosis is a rare, highly infiltrative, locally destructive neoplasm that does not metastasize, but recurs often after primary surgery. Activation of the Wnt/β-catenin pathway is the pathogenic mechanism, caused by an activating mutation in exon 3 of CTNNB1 (85% of the sporadic patients). Radiotherapy is a frequent treatment modality with a local control rate of approximately 80%. In very rare cases, this may result in the development of radiation-induced sarcoma. It is unclear whether these sarcomas develop from the primary tumor or arise de novo in normal tissue. In 4 tertiary referral centers for sarcoma, 6 cases of desmoid-type fibromatosis that subsequently developed sarcoma after radiotherapy were collected. The DNA sequence of CTNNB1 exon 3 in the desmoid-type fibromatosis and the subsequent postradiation sarcoma was determined. Sarcomas developed 5 to 21 years after the diagnosis of desmoid-type fibromatosis and included 2 osteosarcomas, 2 high-grade undifferentiated pleomorphic sarcomas, 1 fibrosarcoma, and 1 undifferentiated spindle cell sarcoma. Three patients showed a CTNNB1 hotspot mutation (T41A, S45F, or S45N) in both the desmoid-type fibromatosis and the radiation-induced sarcoma. The other 3 patients showed a CTNNB1 mutation in the original desmoid-type fibromatosis (2 with a T41A and 1 with an S45F mutation), which was absent in the sarcoma. In conclusion, postradiation sarcomas that occur in the treatment area of desmoid-type fibromatosis are extremely rare and can arise through malignant transformation of CTNNB1-mutated desmoid fibromatosis cells, but may also originate from CTNNB1 wild-type normal cells lying in the radiation field. PMID:26414222

  3. Retroperitoneal Haematom due to Spontaneous Rupture and Haemorrhage of Adrenal Cyst Presenting with Grey Turner's Sign.

    PubMed

    Sonmez, Bedriye Muge; Yilmaz, Fevzi; Özkan, Fevzi Bircan; Ongar, Murat; Özturk, Derya; Cesur, Fatma

    2015-07-01

    Spontaneous retroperitoneal haemorrhage is a rare entity and a potentially life-threatening condition. A 41-year-old woman presented to our emergency department with left flank pain and dysuria. Her physical examination disclosed left abdominal and costovertebral angle tenderness, left flank ecchymosis (Grey Turner sign). Abdominal computerised tomography revealed spontaneous retroperitoneal haemorrhage. She was discharged after 10 days with recommendation of urology follow-up. PMID:26160093

  4. Primary Retroperitoneal Müllerian Adenocarcinoma: A Case Report and Literature Review

    PubMed Central

    Spinelli, C.; Strambi, S.; Tartaglia, D.; Di Franco, G.; Pucci, V.; Faviana, P.; Lencioni, M.

    2013-01-01

    Primary retroperitoneal müllerian adenocarcinoma (PRMA) is an extremely rare clinical entity. We report the case of a 54-year-old woman who presented with a mass in the right lower retroperitoneum, identified during an ultrasound exam. Computed tomography confirmed a retroperitoneal mass measuring 11 cm. The patient underwent laparotomy and the mass was completely excised. The histopathological exam revealed PRMA. PMID:24474926

  5. Primary retroperitoneal müllerian adenocarcinoma: a case report and literature review.

    PubMed

    Spinelli, C; Strambi, S; Tartaglia, D; Di Franco, G; Pucci, V; Faviana, P; Lencioni, M

    2013-09-01

    Primary retroperitoneal müllerian adenocarcinoma (PRMA) is an extremely rare clinical entity. We report the case of a 54-year-old woman who presented with a mass in the right lower retroperitoneum, identified during an ultrasound exam. Computed tomography confirmed a retroperitoneal mass measuring 11 cm. The patient underwent laparotomy and the mass was completely excised. The histopathological exam revealed PRMA. PMID:24474926

  6. 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

  7. 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

  8. The Role of Insulin-Like Growth Factor System in Soft Tissue Sarcomas: From Physiopathology to Targeted Therapeutic Approaches

    PubMed Central

    1998-01-01

    Purpose/Results. Although surgical, chemo- and radiotherapeutic treatment regimens in patients with soft tissue sarcomas have constantly been refined over the past two decades, the survival rate for these patients is rather low. Discussion. There is a great need to investigate the mechanisms for oncogenesis and to identify the factors involved in malignant transformation in sarcomas. Among these factors, IGFs are thought to play a pivotal role as progression factors in various types of sarcomas. The dysregulation of the IGF-II synthesis, e.g. by loss of imprinting which occurs in most types of sarcomas, is a permissive effect through the suppression of cell death. In addition, cells that overexpress the type I IGF receptors are more susceptible to transformation by oncogenes. As TP53 suppresses the activity of IGF-II P3 and P4, as well as the type I IGF receptor promoter, mutations of TP53 in sarcomas may alternatively lead to the activation of these factors. Finally, the phenomenon of non-islet cell tumour hypoglycaemia that occurs in patients with sarcomas, and which is related to the secretion of IGF-II prohormones, is discussed. Future therapeutic strategies may be based upon the application of antibodies or antisense oligonucleotides directed against the type I IGF receptors, with the common goal of inducing apoptosis in sarcoma cells. Ultimately, these and other therapeutic approaches may lead to a better outcome in patients suffering from sarcoma. PMID:18521237

  9. Therapeutic Trial for Patients With Ewing Sarcoma Family of Tumor and Desmoplastic Small Round Cell Tumors

    ClinicalTrials.gov

    2015-12-01

    Desmoplastic Small Round Cell Tumor; Ewing Sarcoma of Bone or Soft Tissue; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  10. Sequencing Overview of Ewing Sarcoma: A Journey across Genomic, Epigenomic and Transcriptomic Landscapes

    PubMed Central

    Sand, Laurens G. L.; Szuhai, Karoly; Hogendoorn, Pancras C. W.

    2015-01-01

    Ewing sarcoma is an aggressive neoplasm occurring predominantly in adolescent Caucasians. At the genome level, a pathognomonic EWSR1-ETS translocation is present. The resulting fusion protein acts as a molecular driver in the tumor development and interferes, amongst others, with endogenous transcription and splicing. The Ewing sarcoma cell shows a poorly differentiated, stem-cell like phenotype. Consequently, the cellular origin of Ewing sarcoma is still a hot discussed topic. To further characterize Ewing sarcoma and to further elucidate the role of EWSR1-ETS fusion protein multiple genome, epigenome and transcriptome level studies were performed. In this review, the data from these studies were combined into a comprehensive overview. Presently, classical morphological predictive markers are used in the clinic and the therapy is dominantly based on systemic chemotherapy in combination with surgical interventions. Using sequencing, novel predictive markers and candidates for immuno- and targeted therapy were identified which were summarized in this review. PMID:26193259

  11. Laparoscopic surgery on broken points for uterine sarcoma in the early stage decrease prognosis

    PubMed Central

    Liu, Hong; Zhu, Yi; Zhang, Guo-Nan; Wang, Chang; Li, Chao; Shi, Yu

    2016-01-01

    Uterine sarcoma, a rare solid tumor in uterus, is difficult to identify in the early stage from some benign uterine tumors, such as uterine fibroids. Hence, uterine sarcoma may be treated in the same way as uterine fibroids; and this may not be found until pathological diagnosis. Consequently, this can lead to tumor’s abdominal spread, planting and local invasive growth, resulting in an early uterine sarcoma, an increased relapse rate after surgery and a decreased survival. Therefore, it’s important to avoid these unintended and iatrogenic complications through an accurate diagnosis and an appropriate surgical approach. The surgical staging and a complete resection of the tumor are both important for patients’ prognosis. In this review, we will discuss the laparoscopic surgery for uterine sarcoma in the early stage and patients’ prognosis. PMID:27503773

  12. Laparoscopic surgery on broken points for uterine sarcoma in the early stage decrease prognosis.

    PubMed

    Liu, Hong; Zhu, Yi; Zhang, Guo-Nan; Wang, Chang; Li, Chao; Shi, Yu

    2016-01-01

    Uterine sarcoma, a rare solid tumor in uterus, is difficult to identify in the early stage from some benign uterine tumors, such as uterine fibroids. Hence, uterine sarcoma may be treated in the same way as uterine fibroids; and this may not be found until pathological diagnosis. Consequently, this can lead to tumor's abdominal spread, planting and local invasive growth, resulting in an early uterine sarcoma, an increased relapse rate after surgery and a decreased survival. Therefore, it's important to avoid these unintended and iatrogenic complications through an accurate diagnosis and an appropriate surgical approach. The surgical staging and a complete resection of the tumor are both important for patients' prognosis. In this review, we will discuss the laparoscopic surgery for uterine sarcoma in the early stage and patients' prognosis. PMID:27503773

  13. Origins of Injection-Site Sarcomas in Cats: The Possible Role of Chronic Inflammation—A Review

    PubMed Central

    Woodward, Kevin N.

    2011-01-01

    The etiology of feline injection-site sarcomas remains obscure. Sarcomas and other tumors are known to be associated with viral infections in humans and other animals, including cats. However, the available evidence suggests that this is not the case with feline injection-site sarcomas. These tumors have more in common with sarcomas noted in experimental studies with laboratory animals where foreign materials such as glass, plastics, and metal are the causal agent. Tumors arising with these agents are associated with chronic inflammation at the injection or implantation sites. Similar tumors have been observed, albeit infrequently, at microchip implantation sites, and these also are associated with chronic inflammation. It is suggested that injection-site sarcomas in cats may arise at the administration site as a result of chronic inflammation, possibly provoked by adjuvant materials, with subsequent DNA damage, cellular transformation, and clonal expansion. However, more fundamental research is required to elucidate the mechanisms involved. PMID:23738095

  14. Synovial Sarcoma Mimicking Myositis Ossificans

    PubMed Central

    Erkut, Adem; Guvercin, Yılmaz; Bedir, Recep

    2016-01-01

    A calcification mass was incidentally found in the soft tissue of a patient who had a history of trauma to the extremity during examination. The patient had no symptom. The pathological analysis of the mass revealed it was an early-phase synovial sarcoma (SS). The diagnosis was made before the onset of symptoms and proper surgical intervention was performed. Therefore, in case of a <1 cm lesion clinically suspicious of myositis ossificans, SS should be taken into consideration as a possible diagnosis. PMID:27595081

  15. Synovial Sarcoma Mimicking Myositis Ossificans.

    PubMed

    Balik, Mehmet Sabri; Erkut, Adem; Guvercin, Yılmaz; Bedir, Recep

    2016-09-01

    A calcification mass was incidentally found in the soft tissue of a patient who had a history of trauma to the extremity during examination. The patient had no symptom. The pathological analysis of the mass revealed it was an early-phase synovial sarcoma (SS). The diagnosis was made before the onset of symptoms and proper surgical intervention was performed. Therefore, in case of a <1 cm lesion clinically suspicious of myositis ossificans, SS should be taken into consideration as a possible diagnosis. PMID:27595081

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

    SciTech Connect

    Vreeze, Ronald S.A. de Jong, Daphne de; Haas, Rick L.; Stewart, Fiona; Coevorden, Frits van

    2008-12-01

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

  17. Ewing Sarcoma of the External Ear Canal.

    PubMed

    Binnetoglu, Adem; Baglam, Tekin; Tokuc, Gulnur; Kecelioglu Binnetoglu, Kiymet; Gerin, Fatma; Sari, Murat

    2016-01-01

    Background. Ewing sarcoma (ES) is a high-grade malignant tumor that has skeletal and extraskeletal forms and consists of small round cells. In the head and neck region, reported localization of extraskeletal ES includes the larynx, thyroid gland, submandibular gland, nasal fossa, pharynx, skin, and parotid gland, but not the external ear canal. Methods. We present the unique case of a 2-year-old boy with extraskeletal ES arising from the external ear canal, mimicking auricular hematoma. Results. Surgery was performed and a VAC/IE (vincristine, adriamycin, cyclophosphamide alternating with ifosfamide, and etoposide) regimen was used for adjuvant chemotherapy for 12 months. Conclusion. The clinician should consider extraskeletal ES when diagnosing tumors localized in the head and neck region because it may be manifested by a nonspecific clinical picture mimicking common otorhinolaryngologic disorders. PMID:27313930

  18. Ewing Sarcoma of the External Ear Canal

    PubMed Central

    Kecelioglu Binnetoglu, Kiymet; Gerin, Fatma; Sari, Murat

    2016-01-01

    Background. Ewing sarcoma (ES) is a high-grade malignant tumor that has skeletal and extraskeletal forms and consists of small round cells. In the head and neck region, reported localization of extraskeletal ES includes the larynx, thyroid gland, submandibular gland, nasal fossa, pharynx, skin, and parotid gland, but not the external ear canal. Methods. We present the unique case of a 2-year-old boy with extraskeletal ES arising from the external ear canal, mimicking auricular hematoma. Results. Surgery was performed and a VAC/IE (vincristine, adriamycin, cyclophosphamide alternating with ifosfamide, and etoposide) regimen was used for adjuvant chemotherapy for 12 months. Conclusion. The clinician should consider extraskeletal ES when diagnosing tumors localized in the head and neck region because it may be manifested by a nonspecific clinical picture mimicking common otorhinolaryngologic disorders. PMID:27313930

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

    PubMed Central

    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

    2016-01-01

    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. DOI: http://dx.doi.org/10.7554/eLife.12116.001 PMID:26987019

  20. Soft tissue sarcoma and occupational exposures

    SciTech Connect

    Wingren, G.; Fredrikson, M.; Brage, H.N.; Nordenskjoeld, B.A.; Axelson, O. )

    1990-08-15

    The associations between soft tissue sarcoma (STS) and occupational exposures were studied in a case-referent study in the southeast of Sweden. Exposure information was obtained through mailed questionnaires to 96 cases, 450 randomly selected population referents, and 200 cancer referents. Odds ratios (OR), were calculated for various occupational groups, and particularly, for occupations with potential exposure to chlorinated phenoxy herbicides and chlorophenols. In the analyses based on population referents, increased risks for soft tissue sarcoma were seen for especially gardeners (OR = 4.1), but also railroad workers (OR = 3.1); construction workers with exposure to impregnating agents (OR = 2.3), asbestos (OR = 1.8), or pressure impregnating agents (OR = 1.7); and unspecified chemical workers with potential exposure to phenoxy herbicides and/or chlorophenols (OR = 1.6). A similar pattern appeared when cancer referents were used although the numerical values of the odds ratios became different. A grouping of jobs resulted in Mantel-Haensel OR from 1.5 to 1.9 for farmers and forestry workers, dependent on referents used and even more increased OR for railroad workers and unspecified chemical workers with potential exposure to phenoxy herbicides and chlorophenols. The results of the study confirm rather than refute that phenoxy herbicides and chlorophenols could be of etiologic importance for STS; the high risk for gardeners, although based on a small number of individuals, was unexpected and remains unclear. Also, since other cancers were used as referents, no definite problems of recall bias should obtain in this material. None of the exposed groups had a higher proportion of smokers than the unexposed group.

  1. Age-Stratified Risk of Unexpected Uterine Sarcoma Following Surgery for Presumed Benign Leiomyoma

    PubMed Central

    Li, Li; Andikyan, Vaagn; Običan, Sarah G.; Cioffi, Angela; Hao, Ke; Dudley, Joel T.; Ascher-Walsh, Charles; Kasarskis, Andrew; Maki, Robert G.

    2015-01-01

    Background. Estimates of unexpected uterine sarcoma following surgery for presumed benign leiomyoma that use age-stratification are lacking. Patients and Methods. A retrospective cohort of 2,075 patients that had undergone myomectomy was evaluated to determine the case incidence of unexpected uterine sarcoma. An aggregate risk estimate was generated using a meta-analysis of similar studies plus our data. Database-derived age distributions of the incidence rates of uterine sarcoma and uterine leiomyoma surgery were used to stratify risk by age. Results. Of 2,075 patients in our retrospective cohort, 6 were diagnosed with uterine sarcoma. Our meta-analysis revealed 8 studies from 1980 to 2014. Combined with our study, 18 cases of leiomyosarcoma are reported in 10,120 patients, for an aggregate risk of 1.78 per 1,000 (95% confidence interval [CI]: 1.1–2.8) or 1 in 562. Eight cases of other uterine sarcomas were reported in 6,889 patients, for an aggregate risk of 1.16 per 1,000 (95% CI: 0.5–4.9) or 1 in 861. The summation of these risks gives an overall risk of uterine sarcoma of 2.94 per 1,000 (95% CI: 1.8–4.1) or 1 in 340. After stratification by age, we predict the risk of uterine sarcoma to range from a peak of 10.1 cases per 1,000, or 1 in 98, for patients aged 75–79 years to <1 case per 500 for patients aged <30 years. Conclusion. The risk of unexpected uterine sarcoma varies significantly across age groups. Our age-stratified predictive model should be incorporated to more accurately counsel patients and to assist in providing guidelines for the surgical technique for leiomyoma. PMID:25765878

  2. Preclinical activity of selinexor, an inhibitor of XPO1, in sarcoma.

    PubMed

    Nakayama, Robert; Zhang, Yi-Xiang; Czaplinski, Jeffrey T; Anatone, Alex J; Sicinska, Ewa T; Fletcher, Jonathan A; Demetri, George D; Wagner, Andrew J

    2016-03-29

    Selinexor is an orally bioavailable selective inhibitor of nuclear export that has been demonstrated to have preclinical activity in various cancer types and that is currently in Phase I and II clinical trials for advanced cancers. In this study, we evaluated the effects of selinexor in several preclinical models of various sarcoma subtypes. The efficacy of selinexor was investigated in vitro and in vivo using 17 cell lines and 9 sarcoma xenograft models including gastrointestinal stromal tumor (GIST), liposarcoma (LPS), leiomyosarcoma, rhabdomyosarcoma, undifferentiated sarcomas, and alveolar soft part sarcoma (ASPS). Most sarcoma cell lines were sensitive to selinexor with IC50s ranging from 28.8 nM to 218.2 nM (median: 66.1 nM). Selinexor suppressed sarcoma tumor xenograft growth, including models of ASPS that were resistant in vitro. In GIST cells with KIT mutations, selinexor induced G1- arrest without attenuation of phosphorylation of KIT, AKT, or MAPK, in contrast to imatinib. In LPS cell lines with MDM2 and CDK4 amplification, selinexor induced G1-arrest and apoptosis irrespective of p53 expression or mutation and irrespective of RB expression. Selinexor increased p53 and p21 expression at the protein but not RNA level, indicating a post-transcriptional effect. These results indicate that selinexor has potent in vitro and in vivo activity against a wide variety of sarcoma models by inducing G1-arrest independent of known molecular mechanisms in GIST and LPS. These studies further justify the exploration of selinexor in clinical trials targeting various sarcoma subtypes. PMID:26918731

  3. Preclinical activity of selinexor, an inhibitor of XPO1, in sarcoma

    PubMed Central

    Nakayama, Robert; Zhang, Yi-Xiang; Czaplinski, Jeffrey T.; Anatone, Alex J.; Sicinska, Ewa T.; Fletcher, Jonathan A.; Demetri, George D.; Wagner, Andrew J.

    2016-01-01

    Selinexor is an orally bioavailable selective inhibitor of nuclear export that has been demonstrated to have preclinical activity in various cancer types and that is currently in Phase I and II clinical trials for advanced cancers. In this study, we evaluated the effects of selinexor in several preclinical models of various sarcoma subtypes. The efficacy of selinexor was investigated in vitro and in vivo using 17 cell lines and 9 sarcoma xenograft models including gastrointestinal stromal tumor (GIST), liposarcoma (LPS), leiomyosarcoma, rhabdomyosarcoma, undifferentiated sarcomas, and alveolar soft part sarcoma (ASPS). Most sarcoma cell lines were sensitive to selinexor with IC50s ranging from 28.8 nM to 218.2 nM (median: 66.1 nM). Selinexor suppressed sarcoma tumor xenograft growth, including models of ASPS that were resistant in vitro. In GIST cells with KIT mutations, selinexor induced G1- arrest without attenuation of phosphorylation of KIT, AKT, or MAPK, in contrast to imatinib. In LPS cell lines with MDM2 and CDK4 amplification, selinexor induced G1-arrest and apoptosis irrespective of p53 expression or mutation and irrespective of RB expression. Selinexor increased p53 and p21 expression at the protein but not RNA level, indicating a post-transcriptional effect. These results indicate that selinexor has potent in vitro and in vivo activity against a wide variety of sarcoma models by inducing G1-arrest independent of known molecular mechanisms in GIST and LPS. These studies further justify the exploration of selinexor in clinical trials targeting various sarcoma subtypes. PMID:26918731

  4. Proteasome Addiction Defined in Ewing Sarcoma Is Effectively Targeted by a Novel Class of 19S Proteasome Inhibitors.

    PubMed

    Shukla, Neerav; Somwar, Romel; Smith, Roger S; Ambati, Sri; Munoz, Stanley; Merchant, Melinda; D'Arcy, Padraig; Wang, Xin; Kobos, Rachel; Antczak, Christophe; Bhinder, Bhavneet; Shum, David; Radu, Constantin; Yang, Guangbin; Taylor, Barry S; Ng, Charlotte K Y; Weigelt, Britta; Khodos, Inna; de Stanchina, Elisa; Reis-Filho, Jorge S; Ouerfelli, Ouathek; Linder, Stig; Djaballah, Hakim; Ladanyi, Marc

    2016-08-01

    Ewing sarcoma is a primitive round cell sarcoma with a peak incidence in adolescence that is driven by a chimeric oncogene created from the fusion of the EWSR1 gene with a member of the ETS family of genes. Patients with metastatic and recurrent disease have dismal outcomes and need better therapeutic options. We screened a library of 309,989 chemical compounds for growth inhibition of Ewing sarcoma cells to provide the basis for the development of novel therapies and to discover vulnerable pathways that might broaden our understanding of the pathobiology of this aggressive sarcoma. This screening campaign identified a class of benzyl-4-piperidone compounds that selectively inhibit the growth of Ewing sarcoma cell lines by inducing apoptosis. These agents disrupt 19S proteasome function through inhibition of the deubiquitinating enzymes USP14 and UCHL5. Functional genomic data from a genome-wide shRNA screen in Ewing sarcoma cells also identified the proteasome as a node of vulnerability in Ewing sarcoma cells, providing orthologous confirmation of the chemical screen findings. Furthermore, shRNA-mediated silencing of USP14 or UCHL5 in Ewing sarcoma cells produced significant growth inhibition. Finally, treatment of a xenograft mouse model of Ewing sarcoma with VLX1570, a benzyl-4-piperidone compound derivative currently in clinical trials for relapsed multiple myeloma, significantly inhibited in vivo tumor growth. Overall, our results offer a preclinical proof of concept for the use of 19S proteasome inhibitors as a novel therapeutic strategy for Ewing sarcoma. Cancer Res; 76(15); 4525-34. ©2016 AACR. PMID:27256563

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

    PubMed

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

    2016-04-01

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

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

    ClinicalTrials.gov

    2013-12-10

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

  7. Desmoplastic small round cell tumor: postoperative retroperitoneal mass.

    PubMed

    Shen, Colette J; Loeb, David M; Terezakis, Stephanie A

    2016-09-01

    We describe the case of a 14-year-old boy who presented with a large, 17.6-cm retroperitoneal mass, along with multiple metastases, and was diagnosed with desmoplastic small round cell tumor. After initial chemotherapy, he underwent gross total resection with a positive margin. On postoperative radiation planning computed tomography, a 6.8-cm heterogeneous mass was noted in the surgical bed. Given the tumor's aggressive nature and positive surgical margins, there was real concern for recurrent disease. Further evaluation with magnetic resonance imaging elucidated that the mass consisted of simple fluid and fat, without contrast enhancement, suggesting a postoperative fluid collection. He was able to continue with adjuvant treatment as planned. This case example illustrates that even large postoperative heterogeneous masses may still be related to postoperative fluid collection in patients with aggressive tumor. However, it is important to rule out recurrent disease before starting adjuvant therapy given improved outcomes with gross total resection in desmoplastic small round cell tumor. PMID:27594960

  8. Asymptomatic Isolated Retroperitoneal Castleman's Disease: A Case Report.

    PubMed

    Rajabiani, Afsaneh; Abdollahi, Alireza; Farahani, Zahra

    2015-09-01

    Castleman's disease, giant lymph node hyperplasia, is a kind of benign lymphoproliferative disease with gentle behavior. Its etiology and prevalence are unclear. This rare disease is usually found in mediastinal area asymptomatically and incidentally. It is also rare to see this tumor in the retroperitoneum. In this study, we have introduced a 34-year-old woman who referred just with occasional abdominal pain caused by compressive symptoms. Laboratory findings only reported microcytic anemia (MCH: 18.5, MCV: 63, Hemoglobin 10.2 g/dl). Chest and abdominal X-ray imaging showed no remarkable point. In abdominal ultrasonography, a solid and firm tumor with 12.2×5.3×6.6 cm was reported in patient's retroperitoneum. Patient's surgery was done and the tumor (covered by a fibrous thick capsule, with no bizarre appearance and bleeding) was completely removed. Pathologic examination indicated a Castleman's tumor, type of unicentric and hyaline-vascular. This item had been one of the rare reported items of Castleman's disease in the retroperitoneal space. PMID:26379356

  9. Postpartum retroperitoneal fasciitis: a case report and review of literature.

    PubMed

    Yagi, Hiroshi; Fukushima, Kotaro; Satoh, Shoji; Nakashima, Yasuharu; Nozaki, Masahiro; Nakano, Hitoo

    2005-02-01

    We present a case of a postpartum female with iliopsoas fasciitis in the puerperium. Two days after a spontaneous vaginal delivery at 38 weeks without any complications, the patient complained of pain in the left thigh and hip, associated with a temperature of 38 degrees C. Consequently, she could not walk 4 days after delivery and her body temperature had increased to 39 degrees C. Extreme left flank pain and tenderness in the left pelvic wall were prominent, whereas the tenderness in the pelvis was moderate. Magnetic resonance imaging led us to diagnose iliopsoas fasciitis. This was complicated by sepsis but improved after a protracted antibiotic treatment without any surgical intervention. A literature review revealed that serious complications, including sepsis or permanent functional disturbance, could arise although retroperitoneal fasciitis and/or abscesses are very rare after vaginal delivery. Iliopsoas fasciitis could be considered when patients complain of extreme pain in the pelvic wall, sacroiliac joint region, or thigh-symptoms that are uncommon in uncomplicated endometritis. PMID:15731991

  10. Endovascular Embolization of Spontaneous Retroperitoneal Hemorrhage Secondary to Anticoagulant Treatment

    SciTech Connect

    Isokangas, Juha-Matti Peraelae, Jukka M.

    2004-11-15

    The purpose of this study was to report a single hospital's experience of endovascular treatment of patients with retroperitoneal hemorrhage (RPH) secondary to anticoagulant treatment. Ten consecutive patients treated in an intensive care unit and needing blood transfusions due to RPH secondary to anticoagulation were referred for digital subtraction angiography (DSA) to detect the bleeding site(s) and to evaluate the possibilities of treating them by transcatheter embolization. DSA revealed bleeding site(s) in all 10 patients: 1 lumbar artery in 4 patients, 1 branch of internal iliac artery in 3 patients and multiple bleeding sites in 3 patients. Embolization could be performed in 9 of them. Coils, gelatin and/or polyvinyl alcohol were used as embolic agents. Bleeding stopped or markedly decreased after embolization in 8 of the 9 (89%) patients. Four patients were operated on prior to embolization, but surgery failed to control the bleeding in any of these cases. Abdominal compartment syndrome requiring surgical or radiological intervention after embolization developed in 5 patients. One patient died, and 2 had sequelae due to RPH. All 7 patients whose bleeding stopped after embolization had a good clinical outcome. Embolization seems to be an effective and safe method to control the bleeding in patients with RPH secondary to anticoagulant treatment when conservative treatment is insufficient.

  11. Langerhans cell sarcoma: an unusual microscopic presentation.

    PubMed

    Gagnon, A L; Daniel, S; Greer, K; Patterson, J W; Tchernev, G; Chokoeva, A A; Wollina, U; Lotti, T; Fioranelli, M; Roccia, M G; Guarneri, C; Aguilera, N

    2016-01-01

    A 70-year-old Caucasian male presented to our clinic for a pruritic eruption progressing over several months. He complained of fatigue with a 20-pound weight loss over the past year. On presentation, the patient had browny-yellow to violaceous, purpuric, macular and papular lesions on the legs, arms, lower abdomen and back. Initial biopsy showed an angiocentric infiltrate with a suggestion of intraluminal proliferation; CD31 and Fli-1 positivity suggested either reactive angioendotheliomatosis or an unusual intravascular histiocytosis. Further excisional biopsies demonstrated perivascular collections of cells with ample cytoplasm, prominent nuclear pleomorphism and mitotic activity. The nuclei demonstrated nuclear folding, grooves and indentations. The atypical cells were S100, CD1a and CD56 positive with immunohistochemistry. A diagnosis of Langerhans cell sarcoma (LCS) was made. LCS is a rare, aggressive malignancy that can involve multiple organs including the skin, lymph nodes, lung, bone marrow, spleen, heart, and brain. The skin and lymph nodes are commonly involved, and the cutaneous presentation varies greatly. Immunohistochemistry characteristically shows CD1a and S100 positivity. CD56 expression is uncommon and often portends a poor prognosis. There is no established treatment of LCS due to its rarity. Surgery, radiation, and chemotherapy have been used with varied outcomes. Our patient was treated with prednisone with improvement of cutaneous disease. He did not develop systemic involvement, but died 1.5 years later from complications associated with heart failure. Langerhans cell sarcoma should be considered when faced with an unusual angiocentric infiltrate in which initial immunohistochemical staining results may be misleading. PMID:27373133

  12. Kaposi Sarcoma in a Non HIV Patient

    PubMed Central

    Jan, Rafi A; Koul, Parvaiz A; Ahmed, Manzoor; Shah, Sonaullah; Mufti, Showkat A; War, Fayaz A

    2008-01-01

    We report a case of a 45 year old non HIV infected female, who presented with multiple painful, livid reddish brown plaques, papules and nodules on both lower limbs and left index finger. The cutaneous nodular lesions on biopsy showed characteristic features of Kaposi’s sarcoma. This case is reported due to paucity of Kapsi’s sarcoma in non HIV Persons. It is typically a disease of older men from European and Mediterranean region. Here we present a case report of classic Kaposi’s Sarcoma in a young Indian female. PMID:21475497

  13. Ewing's Sarcoma of the Adrenal Gland.

    PubMed

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

    2016-01-01

    Ewing's sarcoma (ES) or primitive neuro-ectodermal tumor (PNET) typically occurs in long or flat bones, the chest wall, extra-skeletal soft tissue, and rarely in solid organs. Incidence of adrenal Ewing's sarcoma is very rare. Here we report a case of Ewing's sarcoma of the right adrenal gland in an 8-year-old girl who presented with an abdominal mass. The huge tumor was managed by preoperative neo-adjuvant chemotherapy followed by surgical resection. She died due to metastasis after five months of surgery. PMID:27398321

  14. Ziv-aflibercept in Treating Patients With Locally Advanced, Unresectable, or Metastatic Gynecologic Soft Tissue Sarcoma

    ClinicalTrials.gov

    2015-12-03

    Fallopian Tube Cancer; Female Reproductive Cancer; Ovarian Carcinosarcoma; Ovarian Sarcoma; Recurrent Ovarian Epithelial Cancer; Recurrent Uterine Sarcoma; Stage III Ovarian Epithelial Cancer; Stage III Uterine Sarcoma; Stage IV Ovarian Epithelial Cancer; Stage IV Uterine Sarcoma; Uterine Carcinosarcoma; Uterine Leiomyosarcoma

  15. Non-pancreatic retroperitoneal mucinous neoplasms and a discussion of the differential diagnosis

    PubMed Central

    Cobb, Camilla; Raza, Anwar Sultana

    2016-01-01

    Retroperitoneal mucinous neoplasms are common and have a wide range of differential diagnoses to consider. It is of utmost importance to maintain a multidisciplinary approach when evaluating these lesions. Clinical history, surgical impression and radiographic studies should be obtained and used in conjunction with morphology and immunohistochemistry to help guide the pathologist to the correct diagnosis. We recently encountered a case of a 51-year-old man who presented with an 11.5 cm left-sided retroperitoneal mucinous neoplasm. This lesion was initially diagnosed as a low-grade mucinous carcinoma of probable pancreatic origin at an outside facility, and he was subsequently treated with chemotherapy. One year later he presented to our institution and underwent retroperitoneal en bloc resection of the 7-cm residual multiloculated mucinous neoplasm. When discussed at tumor board additional medical history was obtained, and review of the patient’s chart revealed a remote history of left orchiectomy for a mixed malignant germ cell tumor and metastatic embryonal carcinoma in 2 of 34 retroperitoneal/para-aortic lymph nodes. With no clinical evidence of tumor in the pancreas or extension from it, the predominately para-aortic location of the tumor favors a mucinous carcinoma arising from a rest of mature metastatic/proliferating teratoma that persisted after chemotherapy. This case illustrates the importance of a thorough history, however remote, and correlation with imaging in the development of differential diagnoses, as well as, the need to consider non-pancreaticobiliary sources of retroperitoneal mucinous tumors. PMID:27034817

  16. Effect of Cross-Sex Hormonal Replacement on Antioxidant Enzymes in Rat Retroperitoneal Fat Adipocytes

    PubMed Central

    Velázquez Espejel, Rodrigo; Cabrera-Orefice, Alfredo; Uribe-Carvajal, Salvador; Pavón, Natalia

    2016-01-01

    We report the effect of cross-sex hormonal replacement on antioxidant enzymes from rat retroperitoneal fat adipocytes. Eight rats of each gender were assigned to each of the following groups: control groups were intact female or male (F and M, resp.). Experimental groups were ovariectomized F (OvxF), castrated M (CasM), OvxF plus testosterone (OvxF + T), and CasM plus estradiol (CasM + E2) groups. After sacrifice, retroperitoneal fat was dissected and processed for histology. Adipocytes were isolated and the following enzymatic activities were determined: Cu-Zn superoxide dismutase (SOD), catalase (CAT), glutathione peroxidase (GPx), glutathione-S-transferase (GST), and glutathione reductase (GR). Also, glutathione (GSH) and lipid peroxidation (LPO) were measured. In OvxF, retroperitoneal fat increased and adipocytes were enlarged, while in CasM rats a decrease in retroperitoneal fat and small adipocytes are observed. The cross-sex hormonal replacement in F rats was associated with larger adipocytes and a further decreased activity of Cu-Zn SOD, CAT, GPx, GST, GR, and GSH, in addition to an increase in LPO. CasM + E2 exhibited the opposite effects showing further activation antioxidant enzymes and decreases in LPO. In conclusion, E2 deficiency favors an increase in retroperitoneal fat and large adipocytes. Cross-sex hormonal replacement in F rats aggravates the condition by inhibiting antioxidant enzymes.

  17. [Retroperitoneal fibrosis and arthritis--a manifestation of the same illness].

    PubMed

    Thiele, A; Störkel, S; Stierle, H E

    1998-10-01

    Retroperitoneal fibrosis is a disorder in which the retroperitoneal fat is the site of a subacute and chronic inflammatory reaction and is subsequently replaced by dense fibrotic tissue. Rheumatoid nodules are chronic granulomata occurring at sites of pressure and movement, both near the body surface and internally. A 55-year-old sales-manager was admitted to radiation synovectomy after a 5 year history of excessive right and left knee effusions. There were no other clinical or laboratory abnormalities. The patient did not respond to either radioisotope synoviorthesis using radioactive Yttrium (90 Y), or to open synovectomy and prostetic surgery of the right knee. One year later, surgery of left ureter was necessary. Histological findings revealed the diagnosis of Ormond's disease. Comparative histological studies of synovial membrane of knee and retroperitoneal tissues showed local necrosis, fibrin deposition, lining cell proliferation, and infiltration by lymphocytes. Diagnosis of arthritis complicated by retroperitoneal rheumatoid nodules and retroperitoneal fibrosis was made. Serum rheumatoid factor has been negative. For the last 3 years, the patient has been on successful therapy with azathioprine. Rheumatoid nodules of the retroperitoneum have vanished completely and frequency of knee effusions decreased. PMID:9864832

  18. Primary Ewing's sarcoma of the ribs. A report from the intergroup Ewing's sarcoma study.

    PubMed

    Thomas, P R; Foulkes, M A; Gilula, L A; Burgert, E O; Evans, R G; Kissane, J; Nesbit, M E; Pritchard, D J; Tefft, M; Vietti, T J

    1983-03-15

    Thirty-six patients with primary Ewing's sarcoma of the ribs have been reviewed. Of these, 21 had clinically localized disease at diagnosis and were entered on protocol IESS 7299, eight had regional and seven metastatic disease at diagnosis and were entered on protocol 7450. The 21 with localized disease were treated with surgical excision or biopsy, followed by local radiotherapy (in all but one patient) and randomization to one of three chemotherapy regimens. Eleven patients (52%) remain disease-free for periods ranging from 18 to 64 months, respectively. Seven of eight patients who underwent complete surgical excision of the primary lesion remain disease-free, compared with four of 12 (excluding one patient who died disease-free) who remain disease-free after partial excision or biopsy. However, analysis of size of tumor at diagnosis reveals that smaller primary tumors have a better prognosis irrespective of extent of surgery. Protocol IESS 7450, consisted of radiotherapy to all areas of known disease and four drug chemotherapy. Four (50%) with regional disease but none with metastatic disease have remained alive and continuously disease-free. It is concluded that an aggressive approach to Ewing's sarcoma of the ribs is justified by the results, as even regional disease may be curable. The apparent prognostic advantage for those patients undergoing surgical excision may be explained by patient selection. PMID:6821866

  19. Initial Experience with Retroperitoneal Laparoendoscopic Single-Site Surgery for Upper Urinary Tract Surgery

    PubMed Central

    Pak, Chul-Ho; Baik, Seung

    2011-01-01

    Purpose To report our initial clinical experience and perioperative outcomes of retroperitoneal laparoendoscopic single-site surgery (RLESS) for upper urinary tract surgery. Materials and Methods Between June 2009 and October 2010, we performed RLESS in 23 patients for various indications including radical nephrectomy (n=4), nephroureterectomy (n=2), simple nephrectomy (n=10), and renal cyst ablation (n=7). RLESS was performed with a homemade single-port device with a conventional rigid laparoscopic instrument and laparoscope. The parameters analyzed were age, body mass index, operative time, estimated blood loss, transfusion, time of oral intake, visual analogue pain scale score (VAPS), length of hospital stay, and complications. Results One case of simple nephrectomy was converted to open nephrectomy because of severe adhesion and inadequate surgical exposure. RLESS was completed in 23 patients. Mean operative time was 168.7±29.2, 227.5±50.0, 230.0±56.5, and 70.5±8.9 minutes for simple nephrectomy, radical nephrectomy, nephroureterectomy, and renal cyst ablation, respectively. Estimated blood loss was 113.0±149.8, 170.0±156.8, 400.0±141.4, and 22.8±16.0 ml. The time to oral intake after surgery was 1.4±0.5, 1.2±0.5, 1.5±0.7, and 1.1±0.3 days. The mean VAPS score was 1.1±0.2, 2.1±0.5, 2.0±0.5, and 1.0±0.0 of 10 (range, 0.8 to 2.6). The hospital stay was 4.6±1.5, 3.7±0.5, 6.0±1.4, and 3.2±1.7 days. No major perioperative complications were observed. Conclusions The initial outcomes of our experience suggest that RLESS is a technically feasible and safe procedure for upper urinary tract surgery. Prospective comparative studies with conventional retroperitoneal laparoscopic surgery are needed to confirm the potential benefits of RLESS. PMID:22216397

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

    ClinicalTrials.gov

    2014-08-26

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

  1. Retroperitoneal Castleman's disease: benign in the midst of malignancy

    PubMed Central

    Jayant, Kumar; Agrawal, Swati; Agrawal, Rajendra

    2014-01-01

    A 32-year-old asymptomatic woman presented with localised mass on the left side of the abdomen for 8 months. An abdominal ultrasound and CT scan revealed a solid heterogenous mass located just below the left kidney in the retroperitoneum. Provisional diagnosis of soft tissue sarcoma was made. Following this, surgery was planned and open biopsy (frozen section) was performed, which demonstrated localised hyaline-vascular type of Castleman's disease, which was then excised completely. Thus, intraoperative diagnosis avoided extensive resection and radiation therapy, which reduced the patient's morbidity and expenses. Postoperative period was uneventful and no recurrence was seen even after 3 years of follow-up. PMID:24700042

  2. Granulocytic sarcoma: an atypical presentation in the oral cavity.

    PubMed

    Stoopler, Eric T; Pinto, Andres; Alawi, Faizan; Raghavendra, Sree; Boyce, Ricardo; Porter, David; Sollecito, Thomas P

    2004-01-01

    Acute myelogenous leukemia (AML) is a hematologic disorder that is characterized by an abnormal proliferation of immature myeloid cells. Granulocytic sarcomas are clusters of leukemic myeloid cells that may develop as a result of AML. Oral manifestations of AML are common and often involve enlargements of the gingiva and/or mucosal tissue from direct leukemia cell infiltration. We describe the case history of a 50-year-old man who had an ulcerative lesion of the oral mucosa that was determined to be a granulocytic sarcoma of AML-MO subtype. The combination of both the subtype and clinical presentation of the leukemia makes this presentation unusual, and to the best of our knowledge, of a type that has not been previously reported in the literature. PMID:15200230

  3. [Lymph drainage with secondary lymphedema caused by Kaposi sarcoma].

    PubMed

    Einfeldt, H

    1992-12-01

    For reasons not yet known HIV infected patients in the final state of their aids disease often tend to develop Kaposi's sarcoma. These tumours result in secondary lymphatic edema which is found on both sides of the sarcoma up to the regional lymphatic nodes, transferred by the tumour cells. Depending on the state of the edema, a lymph drainage treatment is indicated palliatively; the patients can thus be relieved. A fundamental deterioration of the prognosis is not to be expected, the more as all patients are in the final state of this not yet curable disease. Differing from treatment of other lymphatic edema, it is of special importance to the therapist--apart from the difficult and specifically psychic burden--to pay attention to a protection from infection by gloves as a precaution for each single treatment. PMID:1288026

  4. [Lymph drainage therapy in secondary lymphedema caused by Kaposi sarcoma].

    PubMed

    Einfeldt, H

    1989-07-01

    For reasons not yet known HIV infected patients in the final state of their aids disease often tend to develop Kaposi's sarcoma. These tumours result in secondary lymphatic edemas which are found on both sides of the sarcoma. They reach up to the regional lymphatic nodes blocked by the tumour cells. Depending on the state of the edema a lymphaticdrainage treatment is indicated palliatively; the patients can thus be relieved. A fundamental deterioration of prognosis is not to be expected, as all patients are anyway in the final stage of this not yet curable disease. Differing from treatment of other lymphatic edema, it is of special importance to the therapist - apart from the particular and difficult psychic burden - to pay attention to infection protection by using gloves for each single treatment. PMID:2672661

  5. Kaposi Sarcoma-associated Herpesvirus: mechanisms of oncogenesis.

    PubMed

    Schulz, Thomas F; Cesarman, Ethel

    2015-10-01

    Kaposi Sarcoma-associated Herpesvirus (KSHV, HHV8) causes three human malignancies, Kaposi Sarcoma (KS), an endothelial tumor, as well as Primary Effusion Lymphoma (PEL) and the plasma cell variant of Multicentric Castleman's Disease (MCD), two B-cell lymphoproliferative diseases. All three cancers occur primarily in the context of immune deficiency and/or HIV infection, but their pathogenesis differs. KS most likely results from the combined effects of an endotheliotropic virus with angiogenic properties and inflammatory stimuli and thus represents an interesting example of a cancer that arises in an inflammatory context. Viral and cellular angiogenic and inflammatory factors also play an important role in the pathogenesis of MCD. In contrast, PEL represents an autonomously growing malignancy that is, however, still dependent on the continuous presence of KSHV and the action of several KSHV proteins. PMID:26431609

  6. Exomic Analysis of Myxoid Liposarcomas, Synovial Sarcomas, and Osteosarcomas

    PubMed Central

    Jiao, Yuchen; Wood, Laura D.; Kinde, Isaac; Wu, Jian; Mandahl, Nils; Luo, Jinyong; Hruban, Ralph H.; Diaz, Luis A.; He, Tong-Chuan; Vogelstein, Bert; Kinzler, Kenneth W.; Mertens, Fredrik; Papadopoulos, Nickolas

    2014-01-01

    Bone and soft tissue sarcomas are a group of histologically heterogeneous and relatively uncommon tumors. To explore their genetic origins, we sequenced the exomes of 13 osteosarcomas, eight myxoid liposarcomas (MLPS), and seven synovial sarcomas (SYN). These tumors had few genetic alterations (median of 10.8). Nevertheless, clear examples of driver gene mutations were observed, including canonical mutations in TP53, PIK3CA, SETD2, AKT1, and subclonal mutation in FBXW7. Of particular interest were mutations in H3F3A, encoding the variant histone H3.3. Mutations in this gene have only been previously observed in gliomas. Loss of heterozygosity of exomic regions was extensive in osteosarcomas but rare in SYN and MLPS. These results provide intriguing nucleotide-level information on these relatively uncommon neoplasms and highlight pathways that help explain their pathogenesis. PMID:24190505

  7. Bone sarcoma in humans induced by radium: A threshold response?

    SciTech Connect

    Rowland, R.E.

    1996-08-01

    The radium 226 and radium 228 have induced malignancies in the skeleton (primarily bone sarcomas) of humans. They have also induced carcinomas in the paranasal sinuses and mastoid air cells. There is no evidence that any leukemias or any other solid cancers have been induced by internally deposited radium. This paper discuses a study conducted on the dial painter population. This study made a concerted effort to verify, for each of the measured radium cases, the published values of the skeletal dose and the initial intake of radium. These were derived from body content measurements made some 40 years after the radium intake. Corrections to the assumed radium retention function resulted in a considerable number of dose changes. These changes have changed the shape of the dose response function. It now appears that the induction of bone sarcomas is a threshold process.

  8. High-Grade Sarcomas Mimicking Traumatic Intramuscular Hematomas

    PubMed Central

    Gomez, Pablo; Morcuende, Jose

    2004-01-01

    We reported on three patients with high-grade soft-tissue sarcomas mimicking traumatic intramuscular hematomas. Patients had an episode of trauma to the extremity, and after initial clinical and imaging evaluations they were considered to have muscular hematomas. The lesions increased in size over time, leading to further evaluations that demonstrated the actual diagnosis. We conducted a retrospective review of the clinical findings, magnetic resonance images, and computed tomography scans to assess characteristics that will help in the differential diagnosis. We conclude that intramuscular hematomas following trauma should be approached with a high degree of clinical suspicion. MRI analysis can be used as an important diagnostic tool, but the results must be seen in the context of the clinical history. MRI is not sensitive or specific enough to rule out malignancy. The diagnosis of a high-grade sarcoma must be considered in these patients and any doubt should be resolved with a biopsy. PMID:15296215

  9. Synovial Sarcoma Microvesicles Harbor the SYT-SSX Fusion Gene Transcript: Comparison of Different Methods of Detection and Implications in Biomarker Research.

    PubMed

    Fricke, A; Ullrich, P V; Cimniak, A F V; Follo, M; Nestel, S; Heimrich, B; Nazarenko, I; Stark, G B; Bannasch, H; Braig, D; Eisenhardt, S U

    2016-01-01

    Background. Synovial sarcoma is an aggressive soft-tissue malignancy. This study examines the presence of the SYT-SSX fusion transcript in synovial sarcoma microvesicles as well as its potential role as a biomarker for synovial sarcoma. Patients and Methods. Microvesicle release of synovial sarcoma cells was examined by transmission electron microscopy. RNA-content was analyzed by qPCR, nested PCR, nested qPCR, and droplet digital PCR to compare their sensitivity for detection of the SYT-SSX fusion gene transcript. Whole blood RNA, RNA of mononuclear cells, and microvesicle RNA of synovial sarcoma patients were analyzed for the presence of the fusion gene transcripts. Results. Electron microscopic analysis revealed synovial sarcoma cells releasing membrane-enclosed microvesicles. In vitro, the SYT-SSX fusion gene transcript was detected in both synovial sarcoma cells and microvesicles. Nested qPCR proved to be the most sensitive in detecting the SYT-SSX fusion gene mRNA. In contrast, the fusion gene transcript was not detected in peripheral blood cells and microvesicles of synovial sarcoma patients. Conclusion. Synovial sarcoma cells release microvesicles harboring the SYT-SSX fusion transcript. Nested qPCR proved to be the most sensitive in detecting the SYT-SSX fusion gene mRNA; however, more sensitive assays are needed to detect cancer-specific microvesicles in the peripheral blood of cancer patients. PMID:27069481

  10. Synovial Sarcoma Microvesicles Harbor the SYT-SSX Fusion Gene Transcript: Comparison of Different Methods of Detection and Implications in Biomarker Research

    PubMed Central

    Fricke, A.; Ullrich, P. V.; Cimniak, A. F. V.; Follo, M.; Nestel, S.; Heimrich, B.; Nazarenko, I.; Stark, G. B.; Bannasch, H.; Braig, D.; Eisenhardt, S. U.

    2016-01-01

    Background. Synovial sarcoma is an aggressive soft-tissue malignancy. This study examines the presence of the SYT-SSX fusion transcript in synovial sarcoma microvesicles as well as its potential role as a biomarker for synovial sarcoma. Patients and Methods. Microvesicle release of synovial sarcoma cells was examined by transmission electron microscopy. RNA-content was analyzed by qPCR, nested PCR, nested qPCR, and droplet digital PCR to compare their sensitivity for detection of the SYT-SSX fusion gene transcript. Whole blood RNA, RNA of mononuclear cells, and microvesicle RNA of synovial sarcoma patients were analyzed for the presence of the fusion gene transcripts. Results. Electron microscopic analysis revealed synovial sarcoma cells releasing membrane-enclosed microvesicles. In vitro, the SYT-SSX fusion gene transcript was detected in both synovial sarcoma cells and microvesicles. Nested qPCR proved to be the most sensitive in detecting the SYT-SSX fusion gene mRNA. In contrast, the fusion gene transcript was not detected in peripheral blood cells and microvesicles of synovial sarcoma patients. Conclusion. Synovial sarcoma cells release microvesicles harboring the SYT-SSX fusion transcript. Nested qPCR proved to be the most sensitive in detecting the SYT-SSX fusion gene mRNA; however, more sensitive assays are needed to detect cancer-specific microvesicles in the peripheral blood of cancer patients. PMID:27069481

  11. Functional, chemical genomic, and super-enhancer screening identify sensitivity to cyclin D1/CDK4 pathway inhibition in Ewing sarcoma

    PubMed Central

    Crompton, Brian; Cowley, Glenn; Vazquez, Francisca; Weir, Barbara A.; Tsherniak, Aviad; Parasuraman, Sudha; Kim, Sunkyu; Alexe, Gabriela; Stegmaier, Kimberly

    2015-01-01

    Ewing sarcoma is an aggressive bone and soft tissue tumor in children and adolescents, with treatment remaining a clinical challenge. This disease is mediated by somatic chromosomal translocations of the EWS gene and a gene encoding an ETS transcription factor, most commonly, FLI1. While direct targeting of aberrant transcription factors remains a pharmacological challenge, identification of dependencies incurred by EWS/FLI1 expression would offer a new therapeutic avenue. We used a combination of super-enhancer profiling, near-whole genome shRNA-based and small-molecule screening to identify cyclin D1 and CDK4 as Ewing sarcoma-selective dependencies. We revealed that super-enhancers mark Ewing sarcoma specific expression signatures and EWS/FLI1 target genes in human Ewing sarcoma cell lines. Particularly, a super-enhancer regulates cyclin D1 and promotes its expression in Ewing sarcoma. We demonstrated that Ewing sarcoma cells require CDK4 and cyclin D1 for survival and anchorage-independent growth. Additionally, pharmacologic inhibition of CDK4 with selective CDK4/6 inhibitors led to cytostasis and cell death of Ewing sarcoma cell lines in vitro and growth delay in an in vivo Ewing sarcoma xenograft model. These results demonstrated a dependency in Ewing sarcoma on CDK4 and cyclin D1 and support exploration of CDK4/6 inhibitors as a therapeutic approach for patients with this disease. PMID:26337082

  12. Do We Know What Causes Kaposi Sarcoma?

    MedlinePlus

    ... escape to close saved articles window. My Saved Articles » My ACS » Kaposi Sarcoma + - Text Size Download Printable Version [PDF] » Causes, Risk Factors, and Prevention TOPICS Document Topics ...

  13. Postirradiation sarcomas of the head and neck

    SciTech Connect

    Maisel, R.H.; Manivel, J.C.; Porto, D.P.; Stanley, M.

    1989-09-01

    We discuss four cases of postirradiation sarcomas of the head and neck. Two cases were metachronous sarcomas that appeared after operation and irradiation for primary sarcomas, 1 case was a mandibular malignant fibrous histiocytoma that developed on the opposite side of the jaw from a malignant histiocytic neoplasm that was irradiated 8 years previously, and 1 case was a laryngeal tumor that appeared 5 years after combined operative and radiation therapy for a laryngeal squamous carcinoma. Immunohistochemical studies more precisely defined and classified these tumors, and assisted in determining a therapeutic protocol. The therapy for postirradiation sarcomas includes extirpative operation when possible, but the role of chemotherapy is uncertain. The aggressive behavior of these neoplasms was attested to by the death of three patients within 18 months of their operations.

  14. [Grading of soft tissue and bone sarcomas].

    PubMed

    Petersen, I; Wardelmann, E

    2016-07-01

    Malignancy grading is an essential element in the classification of sarcomas. It correlates with the prognosis of the disease and the risk of metastasis. This article presents the grading schemes for soft tissue, bone and pediatric sarcomas. It summarizes the histological criteria of the Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC) system and the Pediatric Oncology Group as well as the grading of bone tumors by the College of American Pathologists (CAP). Furthermore, the potential relevance of gene expression signatures, the complexity index in sarcoma (CINSARC) and single genetic alterations (p53, MDM2, p16, SWI/SNF, EWSR1 fusions and PAX3/PAX7-FOXO1 fusions) for the prognosis of sarcomas are discussed. PMID:27384333

  15. Pulmonary Artery Intimal Sarcoma: A Case Report

    PubMed Central

    Kriz, Joseph P.; Munfakh, Nabil A.; King, Gregory S.; Carden, Juan O.

    2016-01-01

    Pulmonary artery intimal sarcomas are rare and lethal malignant tumors that typically affect larger vessels: the aorta, inferior vena cava, and pulmonary arteries. Since symptoms and imaging of pulmonary arterial intimal sarcomas mimic pulmonary thromboembolism, the differential diagnosis of a patient presenting with chest pain, dyspnea, and filling defect within the pulmonary arteries should include intimal sarcoma. Often right ventricular failure is observed due to pulmonary hypertension caused by the obstructive effect of the tumor and concomitant chronic thromboembolism. We report the case of a 72-year-old African-American male with arterial intimal sarcoma of the left and right pulmonary artery with extension through the right artery into the bronchus and right lung. PMID:27239183

  16. Soft Tissue Sarcomas and Agent Orange

    MedlinePlus

    ... survivors' benefits . Research on soft tissue sarcoma and herbicides The Health and Medicine Division (formally known as ... report " Veterans and Agent Orange: Health Effects of Herbicides Used in Vietnam " and other updates that there ...

  17. Sarcomas of Soft Tissue and Bone.

    PubMed

    Ferrari, Andrea; Dirksen, Uta; Bielack, Stefan

    2016-01-01

    The definition of soft tissue and bone sarcomas include a large group of several heterogeneous subtypes of mesenchymal origin that may occur at any age. Among the different sarcomas, rhabdomyosarcoma, synovial sarcoma, Ewing sarcoma and osteosarcoma are aggressive high-grade malignancies that often arise in adolescents and young adults. Managing these malignancies in patients in this age bracket poses various clinical problems, also because different therapeutic approaches are sometimes adopted by pediatric and adult oncologists, even though they are dealing with the same condition. Cooperation between pediatric oncologists and adult medical oncologists is a key step in order to assure the best treatment to these patients, preferably through their inclusion into international clinical trials. PMID:27595362

  18. Immunosuppressive Therapy-Related Kaposi Sarcoma

    MedlinePlus

    ... therapy are used to treat Kaposi sarcoma lesions . Photon radiation therapy treats lesions with high-energy light. ... complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information ...

  19. Isolated Limb Perfusion of Melphalan With or Without Tumor Necrosis Factor in Treating Patients With Soft Tissue Sarcoma of the Arm or Leg

    ClinicalTrials.gov

    2012-03-14

    Stage IVB Adult Soft Tissue Sarcoma; Stage IIB Adult Soft Tissue Sarcoma; Stage IIC Adult Soft Tissue Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Stage IVA Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma

  20. Bone sarcoma diagnosed at the time of reconstructive hip surgery

    PubMed Central

    Dowdy, Paul A.; Griffin, Anthony M.; White, Lawrence M.; Wunder, Jay S.; Bell, Robert S.

    1998-01-01

    Objective To describe the clinical course of a group of patients in whom sarcoma of the proximal femur was diagnosed at the time of reconstructive hip surgery. Design A retrospective case series. Setting Final management of all patients took place at a tertiary care centre. Patients and Interventions Six consecutive patients with sarcoma of the proximal femur diagnosed at the time of reconstructive hip surgery. The mistaken diagnoses made before surgery were benign tumour (2 patients), avascular necrosis (2 patients), subtrochanteric fracture due to metastasis (1 patient) and granuloma from a loose hip implant (1 patient). The final diagnosis was osteosarcoma in 3 patients and chondrosarcoma in 3. Three patients with high-grade sarcoma received neoadjuvant chemotherapy followed by femoral or pelvic resection, or both, and reconstruction. Two patients with chondrosarcoma underwent wide excision of the tumour with allograft or modular implant reconstruction. One patient with widespread metastasis received only palliative chemotherapy. Main outcome measures Overall survival with respect to oncologic and functional results of treatment. Results Two patients (1 who received only palliative chemotherapy) died after 5 and 21 months’ follow-up, respectively. Average follow-up for the remaining 4 patients was 65.2 months (range from 51 to 75 months). They were disease free at latest follow-up. One patient required amputation for septic complications related to the reconstruction. Conclusions Limb salvage surgery for sarcoma of the proximal femur is challenging when the diagnosis is made at the time of reconstructive surgery rather than through an appropriately planned biopsy. However, this series suggests that limb preservation is feasible and that hindquarter amputation is not the only solution. PMID:9711160

  1. Imaging in Soft Tissue Sarcomas: Current Updates.

    PubMed

    Jagannathan, Jyothi P; Tirumani, Sree Harsha; Ramaiya, Nikhil H

    2016-10-01

    Soft tissue sarcomas (STS) are heterogeneous malignant tumors that have nonspecific imaging features. A combination of clinical, demographic, and imaging characteristics can aid in the diagnosis. Imaging provides important information regarding the tumor extent, pretreatment planning, and surveillance of patients with STS. In this article, we illustrate the pertinent imaging characteristics of the commonly occurring STS and some uncommon sarcomas with unique imaging characteristics. PMID:27591491

  2. Surgical management of soft tissue sarcomas

    SciTech Connect

    Arlen, M.; Marcove, R.C.

    1987-01-01

    This volume reflects the latest thinking in surgical and adjuvant forms of therapy that can be offered to the sarcoma patient. Based on their analysis of sarcoma patients, the authors stress management based on site of origin, and discuss tumors on and about the shoulder girdle, hip joint, extremity, retroperitoneum, etc. Coverage includes methods for limb preservation; techniques for regional node resection; indications and methods for arterial perfusion, cryosurgery and isotope implantation; pre- and post-operative immunotherapy chemotherapy and radiation therapy.

  3. Local Recurrence of Extremity Soft Tissue Sarcoma.

    PubMed

    Guerrero, Whitney M; Deneve, Jeremiah L

    2016-10-01

    The management of recurrent soft tissue sarcoma is a challenging problem for clinicians and has a significant physical, mental, emotional, and oncologic impact for the patient. Despite excellent limb-preservation therapies, approximately one-quarter of patients may eventually develop recurrence of disease. How to most appropriately manage these patients is a matter of debate. Several treatment options exist, including surgical resection, irradiation, systemic chemotherapy, amputation, and regional therapies. This article highlights the management of recurrent extremity soft tissue sarcoma. PMID:27542648

  4. Midline extraperitoneal approach for bilateral widespread retroperitoneal abscess originating from anorectal infection

    PubMed Central

    Okuda, Koji; Oshima, Yuka; Saito, Kentaro; Uesaka, Takahiro; Terasaki, Yasunobu; Kasai, Hironori; Minagawa, Nozomi; Oshima, Takahiro; Okawa, Yumi; Misawa, Kazuhito

    2015-01-01

    Introduction Anorectal abscess is one of the most common anorectal conditions encountered in practice. However, such abscesses may rarely extend upward and cause life-threatening medical conditions. Presentation of case A 53-year-old woman presented with symptoms of anorectal abscess and evidence of severe inflammatory response and acute kidney injury. Computed tomography revealed a widespread abscess extending to the bilateral retroperitoneal spaces. Surgical drainage was performed via a totally extraperitoneal approach through a lower midline abdominal incision, and the patient had a rapid and uncomplicated recovery. Discussion Although retroperitoneal abscesses originating from the anorectal region are rare, they are life-threating events that require immediate treatment. Percutaneous abscess drainage has been recently evolved; however, surgical drainage is required sometimes that may be challenging, particularly in the case of widespread abscesses, as in our case. Conclusion The midline extraperitoneal approach reported here might be an effective surgical option for patients with bilateral widespread retroperitoneal abscesses. PMID:26701843

  5. Massive Retroperitoneal Hemorrhage as an Initial Presentation of a Rare and Aggressive Form of Multiple Myeloma

    PubMed Central

    Alawadhi, Aydah; Leb, Laszlo

    2016-01-01

    Multiple myeloma, a plasma cell neoplasm, presents most commonly with anemia, hypercalcemia, renal failure, and bone pain. Only few cases of clinical aggressive presentation associated with bleeding were reported in the medical literature. The reported cases included gastrointestinal bleeding and cardiac tamponade. Spontaneous retroperitoneal haemorrhage as initial presentation has not been so far reported. We hereby report a case of a 64-year-old female who was found to have catastrophic hemorrhage in the retroperitoneal region that extended into intrathecal space causing cord compression. The case posed a significant diagnostic and management dilemma. This case emphasizes the need to think broadly and include multiple myeloma in the diagnosis of unexplained massive retroperitoneal bleeding. PMID:26885415

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

    ClinicalTrials.gov

    2016-05-13

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

  7. Undifferentiated Embryonal Sarcoma of Liver

    PubMed Central

    Kallam, Avyakta; Krishnamurthy, Jairam; Kozel, Jessica

    2015-01-01

    Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant hepatic tumor. A 47 year old male presented with symptoms of sour taste in his mouth, occasional nausea, indigestion and 15-pound weight loss over two months. He had an unremarkable upper gastrointestinal endoscopy. Imaging showed a large liver mass in the left hepatic lobe that was resected and then reported as UESL. He went on to develop lung metastases and was initially treated with doxorubicin and ifosfamide followed by switching of therapy to gemcitabine and docetaxel due to progression of disease. He had a good response after two cycles and went on to receive four more cycles, achieving stable disease. We can therefore conclude that the combination of gemcitabine and docetaxel is a potential therapeutic option for patients with UESL. PMID:26788276

  8. Acroangiodermatitis (Pseudo-Kaposi sarcoma)

    PubMed Central

    Singh, Satyendra Kumar; Manchanda, Kajal

    2014-01-01

    Acroangiodermatitis or Pseudo-Kaposi sarcoma is a rare angioproliferative entity, related to chronic venous insufficiency or certain other vascular anomalies. It is often associated with chronic venous insufficiency, arteriovenous malformation of the legs, chronic renal failure treated with dialysis, paralyzed legs and amputation stumps. We hereby describe a case of 45 year old female presenting with pitting pedal edema, multiple ulcers over bilateral lower limbs with irregular margins with erythema and hyperpigmentation of the surrounding skin. Color Doppler study of bilateral lower limbs was normal. Histopathological examination from one of the lesions showed hyperplastic epidermis, proliferation of capillaries in dermis, hemosiderin deposits and lymphocytic infiltrate. These features thus confirmed the diagnosis of Acroangiodermatitis. PMID:25165656

  9. Undifferentiated Embryonal Sarcoma of Liver.

    PubMed

    Kallam, Avyakta; Krishnamurthy, Jairam; Kozel, Jessica; Shonka, Nicole

    2015-12-29

    Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant hepatic tumor. A 47 year old male presented with symptoms of sour taste in his mouth, occasional nausea, indigestion and 15-pound weight loss over two months. He had an unremarkable upper gastrointestinal endoscopy. Imaging showed a large liver mass in the left hepatic lobe that was resected and then reported as UESL. He went on to develop lung metastases and was initially treated with doxorubicin and ifosfamide followed by switching of therapy to gemcitabine and docetaxel due to progression of disease. He had a good response after two cycles and went on to receive four more cycles, achieving stable disease. We can therefore conclude that the combination of gemcitabine and docetaxel is a potential therapeutic option for patients with UESL. PMID:26788276

  10. Imaging sarcomas of the great vessels and heart.

    PubMed

    Bendel, Emily C; Maleszewski, Joseph J; Araoz, Philip A

    2011-10-01

    Primary sarcomas of the aorta, pulmonary artery, superior vena cava, inferior vena cava, and the heart are rare neoplasms. Aortic sarcomas are broadly categorized as either primarily luminal or primarily mural, with luminal sarcomas more likely to be misdiagnosed as thrombus. Pulmonary artery sarcomas are often mistaken for pulmonary embolism both clinically and at imaging. Vena caval sarcomas appear as intraluminal or extraluminal masses connecting to or filling the veins. The most common are leiomyosarcomas of the inferior vena cava. Primary sarcomas of the heart are rare and usually appear as heterogeneous aggressive masses. PMID:21963161

  11. Kaposi's Sarcoma Herpesvirus Genome Persistence.

    PubMed

    Juillard, Franceline; Tan, Min; Li, Shijun; Kaye, Kenneth M

    2016-01-01

    Kaposi's sarcoma-associated herpesvirus (KSHV) has an etiologic role in Kaposi's sarcoma, primary effusion lymphoma, and multicentric Castleman's disease. These diseases are most common in immunocompromised individuals, especially those with AIDS. Similar to all herpesviruses, KSHV infection is lifelong. KSHV infection in tumor cells is primarily latent, with only a small subset of cells undergoing lytic infection. During latency, the KSHV genome persists as a multiple copy, extrachromosomal episome in the nucleus. In order to persist in proliferating tumor cells, the viral genome replicates once per cell cycle and then segregates to daughter cell nuclei. KSHV only expresses several genes during latent infection. Prominent among these genes, is the latency-associated nuclear antigen (LANA). LANA is responsible for KSHV genome persistence and also exerts transcriptional regulatory effects. LANA mediates KSHV DNA replication and in addition, is responsible for segregation of replicated genomes to daughter nuclei. LANA serves as a molecular tether, bridging the viral genome to mitotic chromosomes to ensure that KSHV DNA reaches progeny nuclei. N-terminal LANA attaches to mitotic chromosomes by binding histones H2A/H2B at the surface of the nucleosome. C-terminal LANA binds specific KSHV DNA sequence and also has a role in chromosome attachment. In addition to the essential roles of N- and C-terminal LANA in genome persistence, internal LANA sequence is also critical for efficient episome maintenance. LANA's role as an essential mediator of virus persistence makes it an attractive target for inhibition in order to prevent or treat KSHV infection and disease. PMID:27570517

  12. The Role of Adjuvant Radiation in Uterine Sarcomas

    SciTech Connect

    Sampath, Sagus; Schultheiss, Timothy E.; Ryu, Janice K.; Wong, Jeffrey Y.C.

    2010-03-01

    Purpose: To determine clinical and pathological factors significant for overall survival (OS) and local-regional failure-free survival (LRFFS) in uterine sarcoma as they relate to adjuvant radiotherapy (AR). Methods and Materials: A retrospective analysis of 3,650 patients with uterine sarcoma was conducted using the National Oncology Database, a proprietary database of aggregated tumor registries owned by Impac Medical Systems (Sunnyvale, CA). Adjuvant radiotherapy was defined as postoperative external beam radiation to the pelvis, with or without brachytherapy. Prognostic factors were identified by multivariate analysis (MVA) using the Cox proportional hazards model. The Kaplan-Meier method was used to estimate survival, with significant differences (p < 0.05) determined using the log-rank test. Results: The median follow-up time was 59 months, with a 5-year OS of 37%. Significant prognostic factors for OS were stage, race/ethnicity, grade, age, histology, lymph node status, and surgical treatment (p < 0.01 for all factors). Use of AR was not predictive for OS. For nonmetastatic cancer patients receiving definitive surgery (n = 2,206), the 5-year LRFFS was 87%. In this group, stage, grade, histology, and AR were prognostic for LRFFS (p < 0.05), with AR associated with improved outcome compared with surgery alone (hazard ratio = 0.4, p < 0.001). Patients with carcinosarcoma, endometrial stromal sarcoma, leiomyosarcoma, poorly differentiated tumors, and negative lymph nodes had reduced local-regional failure (LRF) with AR (log-rank, p < 0.05 for all). Conclusion: In the largest retrospective analysis of uterine sarcoma published thus far, AR conferred a 53% reduction in the risk of LRF at 5 years. Use of AR may have broader indications than what are currently accepted in clinical practice.

  13. Extra-adrenal retroperitoneal paraganglioma associated with duplication of inferior vena cava

    PubMed Central

    Chatterjee, Parangama; Singh, Ashish; Ayyappan, Anoop; Nair, Aravindan

    2008-01-01

    Retroperitoneal paragangliomas arise from specialized neural crest cells symmetrically distributed along the aorta in association with the sympathetic chain. If this tissue aggregates in the adrenal medulla pheochromocytoma may arise. When it remains in the paraaortic sites it could develop into extra-adrenal, retroperitoneal paraganglioma. We report a case of extra-adrenal paraganglioma in the renal hilum intimately related to the left side of a double inferior vena cava. To the best of our knowledge such an association has never been described before. The clinical significance of this venous anomaly is reviewed. PMID:22470594

  14. Challenges in the management of a rare case of extensive retroperitoneal haemangioma in a pregnant woman

    PubMed Central

    Tan, Shu-Qi; Lim, Jason Shau Khng; Tan, Yin Ru; Tan, Hak Koon

    2014-01-01

    Haemangioma of the retroperitoneal space is a rare benign capillary malformation, which can grow significantly in pregnancy due to the multiple associated cardiovascular changes. We herein describe the case of a pregnant woman with an extensive right retroperitoneal haemangioma extending from the level of the renal hilum, across the lateral anterior abdominal wall and into the thigh. We also highlight the challenges faced in the management of the patient’s delivery process. To the best of our knowledge, this is the first case of such nature and severity described in the English literature. PMID:25631977

  15. A retroperitoneal cyst with unusual urinary histogenesis: clinical and immunomorphological characteristics

    PubMed Central

    Branca, Giovanni; Ieni, Antonio; Barresi, Valeria; Versaci, Antonino

    2010-01-01

    Primary retroperitoneal cysts are rare benign lesions which often present as an incidental radiological finding and also cause abdominal symptoms. A 47-year-old woman was admitted to the surgery unit because of right-sided abdominal pain. Computed tomographic scan investigation confirmed a circumscribed, oval, dishomogeneous mass in the right anterior pararenal space. The final diagnosis was obtained after laparotomy excision and pathological evaluation. Immunohistochemical profile was characterized by positive expression of epithelial cystic cells for CKAE1/AE3, CK7, CK8, and CK18, with EMA sustaining the diagnosis of a benign retroperitoneal cyst of urinary system origin. PMID:23754895

  16. The histone demethylase KDM3A is a microRNA-22-regulated tumor promoter in Ewing Sarcoma.

    PubMed

    Parrish, J K; Sechler, M; Winn, R A; Jedlicka, P

    2015-01-01

    Ewing Sarcoma is a biologically aggressive bone and soft tissue malignancy affecting children and young adults. Ewing Sarcoma pathogenesis is driven by EWS/Ets fusion oncoproteins, of which EWS/Fli1 is the most common. We have previously shown that microRNAs (miRs) regulated by EWS/Fli1 contribute to the pro-oncogenic program in Ewing Sarcoma. Here we show that miR-22, an EWS/Fli1-repressed miR, is inhibitory to Ewing Sarcoma clonogenic and anchorage-independent cell growth, even at modest overexpression levels. Our studies further identify the H3K9me1/2 histone demethylase KDM3A (JMJD1A/JHDM2A) as a new miR-22-regulated gene. We show that KDM3A is overexpressed in Ewing Sarcoma, and that its depletion inhibits clonogenic and anchorage-independent growth in multiple patient-derived cell lines, and tumorigenesis in a xenograft model. KDM3A depletion further results in augmentation of the levels of the repressive H3K9me2 histone mark, and downregulation of pro-oncogenic factors in Ewing Sarcoma. Together, our studies identify the histone demethylase KDM3A as a new, miR-regulated, tumor promoter in Ewing Sarcoma. PMID:24362521

  17. Ewing sarcoma dissemination and response to T-cell therapy in mice assessed by whole-body magnetic resonance imaging

    PubMed Central

    Liebsch, L; Kailayangiri, S; Beck, L; Altvater, B; Koch, R; Dierkes, C; Hotfilder, M; Nagelmann, N; Faber, C; Kooijman, H; Ring, J; Vieth, V; Rossig, C

    2013-01-01

    Background: Novel treatment strategies in Ewing sarcoma include targeted cellular therapies. Preclinical in vivo models are needed that reflect their activity against systemic (micro)metastatic disease. Methods: Whole-body magnetic resonance imaging (WB-MRI) was used to monitor the engraftment and dissemination of human Ewing sarcoma xenografts in mice. In this model, we evaluated the therapeutic efficacy of T cells redirected against the Ewing sarcoma-associated antigen GD2 by chimeric receptor engineering. Results: Of 18 mice receiving intravenous injections of VH-64 Ewing sarcoma cells, all developed disseminated tumour growth detectable by WB-MRI. All mice had lung tumours, and the majority had additional manifestations in the bone, soft tissues, and/or kidney. Sequential scans revealed in vivo growth of tumours. Diffusion-weighted whole-body imaging with background signal suppression effectively visualised Ewing sarcoma growth in extrapulmonary sites. Animals receiving GD2-targeted T-cell therapy had lower numbers of pulmonary tumours than controls, and the median volume of soft tissue tumours at first detection was lower, with a tumour growth delay over time. Conclusion: Magnetic resonance imaging reliably visualises disseminated Ewing sarcoma growth in mice. GD2-retargeted T cells can noticeably delay tumour growth and reduce pulmonary Ewing sarcoma manifestations in this aggressive disease model. PMID:23839490

  18. Of Mice and Men: Opportunities to Use Genetically Engineered Mouse Models of Synovial Sarcoma for Preclinical Cancer Therapeutic Evaluation

    PubMed Central

    Jones, Kevin B.; Haldar, Malay; Schiffman, Joshua D.; Cannon-Albright, Lisa; Lessnick, Stephen L.; Sharma, Sunil; Capecchi, Mario R.; Randall, R. Lor

    2016-01-01

    Background Synovial sarcoma is a soft tissue malignancy with a predilection for adolescents and young adults. Despite recent improvements in the understanding of its character and etiology, few therapeutic advances have been made. The mortality rate is high among the young population it affects. The low incidence of most subtypes of sarcoma, such as synovial sarcoma, makes disease-specific trials difficult to organize. The biological differences between sarcoma subtypes make inclusion of multiple types in general trials unsatisfactory as well. Methods A review of the literature regarding targetable pathways in synovial sarcoma was undertaken. A strategy has been devised to utilize available technologies in order to prioritize drug trial planning. Results Cell culture and xenograft research with synovial sarcoma cell lines have identified some critical pathways that may be targetable. Promising therapeutic strategies include newer cytotoxic chemotherapies, antiangiogenic agents, anti-IGF1R pathway agents, anti-Bcl-2/proapoptotic agents, and histone deacetylase complex inhibitors. Conclusions We propose to prioritize potential therapeutic strategies via preclinical testing of agents in a genetic mouse model of synovial sarcoma. Preclinical optimization of treatment regimens can guide the development of more focused patient trials. PMID:21666582

  19. Experience with fast neutron therapy for locally advanced sarcomas

    SciTech Connect

    Salinas, R.; Hussey, D.H.; Fletcher, G.H.; Lindberg, R.D.; Martin, R.G.; Peters, L.J.; Sinkovics, J.G.

    1980-03-01

    Between October 1972 and April 1978, 34 patients with locally advanced sarcomas were treated with fast neutrons using the Texas A and M variable energy cyclotron. The clinical material included 29 patients with soft tissue sarcomas, 4 with chondrosarcomas, and one with an osteosarcoma. The best results were achieved for patients with soft tissue sarcomas; 69% (20/29) had local control of their tumor. Only one of 4 patients with chondrosarcomas was classified as having local tumor control, and one patient with osteosarcoma had persistent disease. With most fractionation schedules, local tumor control was superior for patients who received doses greater than 6500 rad/sub eq/ (2100 rad/sub n..gamma../ with 50 MeV/sub d ..-->.. Be/ neutrons). The incidence of major complications was notably increased when maximum radiation doses of 7500 rad/sub eq/ or greater were administered (2400 rad/sub n..gamma../ with 50 MeV/sub d ..-->.. Be/ neutrons). In patients who underwent subsequent surgery, healing was satisfactory if the maximum radiation dose was limited to 4500 to 5500 rad/sub eq/(1450 to 1775 rad/sub n..gamma../ with 50 MeV/sub d ..-->.. Be/ neutrons).

  20. Retroperitoneal lymphangioma: A report of 2 cases and a review of the literature regarding the differential diagnoses of retroperitoneal cystic masses

    PubMed Central

    DI MARCO, MARIACRISTINA; GRASSI, ELISA; VECCHIARELLI, SILVIA; DURANTE, SANDRA; MACCHINI, MARINA; BIASCO, GUIDO

    2016-01-01

    Cystic lymphangioma is a type of benign tumor originating from the lymph vessels. The tumor commonly occurs in childhood, in the head or neck regions, and retroperitoneal localization and presentations in adulthood are rare. Determining a pre-operative diagnosis is often challenging, and in the majority of cases, a diagnosis is only possible subsequent to the histological examination of the surgical specimen. A radical resection is the recommended treatment for cystic lymphangioma, and recurrence is usually due to an incomplete excision of the mass. The present study reports 2 cases of cystic lymphangioma, localized in the pancreatic gland and duodenal wall respectively, which were treated with surgical resection. The study also briefly reviews the literature regarding the differential diagnosis of retroperitoneal cystic masses. PMID:27123082

  1. Risk Factors Associated With Secondary Sarcomas in Childhood Cancer Survivors: A Report From the Childhood Cancer Survivor Study

    SciTech Connect

    Henderson, Tara O.; Rajaraman, Preetha; Stovall, Marilyn; Constine, Louis S.; Olive, Aliza; Smith, Susan A.; Mertens, Ann; Meadows, Anna; Neglia, Joseph P.; Hammond, Sue; Whitton, John; Inskip, Peter D.; Robison, Leslie L.; Diller, Lisa

    2012-09-01

    Purpose: Childhood cancer survivors have an increased risk of secondary sarcomas. To better identify those at risk, the relationship between therapeutic dose of chemotherapy and radiation and secondary sarcoma should be quantified. Methods and Materials: We conducted a nested case-control study of secondary sarcomas (105 cases, 422 matched controls) in a cohort of 14,372 childhood cancer survivors. Radiation dose at the second malignant neoplasm (SMN) site and use of chemotherapy were estimated from detailed review of medical records. Odds ratios (ORs) and 95% confidence intervals were estimated by conditional logistic regression. Excess odds ratio (EOR) was modeled as a function of radiation dose, chemotherapy, and host factors. Results: Sarcomas occurred a median of 11.8 years (range, 5.3-31.3 years) from original diagnosis. Any exposure to radiation was associated with increased risk of secondary sarcoma (OR = 4.1, 95% CI = 1.8-9.5). A dose-response relation was observed, with elevated risks at doses between 10 and 29.9 Gy (OR = 15.6, 95% CI = 4.5-53.9), 30-49.9 Gy (OR = 16.0, 95% CI 3.8-67.8) and >50 Gy (OR = 114.1, 95% CI 13.5-964.8). Anthracycline exposure was associated with sarcoma risk (OR = 3.5, 95% CI = 1.6-7.7) adjusting for radiation dose, other chemotherapy, and primary cancer. Adjusting for treatment, survivors with a first diagnosis of Hodgkin lymphoma (OR = 10.7, 95% CI = 3.1-37.4) or primary sarcoma (OR = 8.4, 95% CI = 3.2-22.3) were more likely to develop a sarcoma. Conclusions: Of the risk factors evaluated, radiation exposure was the most important for secondary sarcoma development in childhood cancer survivors; anthracycline chemotherapy exposure was also associated with increased risk.

  2. What Are the Key Statistics about Uterine Sarcoma?

    MedlinePlus

    ... factors for 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. Last Medical Review: ...

  3. What Are the Key Statistics about Soft Tissue Sarcoma?

    MedlinePlus

    ... for soft tissue sarcomas? What are the key statistics about soft tissue sarcomas? The American Cancer Society's ... in the United States for 2016 are (these statistics include both adults and children): About 12,310 ...

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

    MedlinePlus

    ... for Kaposi sarcoma? What should you ask your doctor about Kaposi sarcoma? As you cope with Kaposi ... need to have honest, open discussions with your doctor. You should ask any question on your mind ...

  5. What's New in Kaposi Sarcoma Research and Treatment?

    MedlinePlus

    ... Next Topic Additional resources for 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) ...

  6. What's New in Uterine Sarcoma Research and Treatment?

    MedlinePlus

    ... Next Topic Additional resources for 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 ...

  7. What Are the Key Statistics about Kaposi Sarcoma?

    MedlinePlus

    ... key statistics about Kaposi sarcoma? Before the AIDS epidemic, Kaposi sarcoma (KS) was rare in the United ... were classic and transplant-related. With the AIDS epidemic, the rate of KS in this country increased ...

  8. Intratumoral oxygen gradients mediate sarcoma cell invasion.

    PubMed

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

    2016-08-16

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

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

    ClinicalTrials.gov

    2016-06-09

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

  10. Human Sarcoma growth is sensitive to small-molecule mediated AXIN stabilization.

    PubMed

    De Robertis, Alessandra; Mennillo, Federica; Rossi, Marco; Valensin, Silvia; Tunici, Patrizia; Mori, Elisa; Caradonna, Nicola; Varrone, Maurizio; Salerno, Massimiliano

    2014-01-01

    Sarcomas are mesenchymal tumors showing high molecular heterogeneity, reflected at the histological level by the existence of more than fifty different subtypes. Genetic and epigenetic evidences link aberrant activation of the Wnt signaling to growth and progression of human sarcomas. This phenomenon, mainly accomplished by autocrine loop activity, is sustained by gene amplification, over-expression of Wnt ligands and co-receptors or epigenetic silencing of endogenous Wnt antagonists. We previously showed that pharmacological inhibition of Wnt signaling mediated by Axin stabilization produced in vitro and in vivo antitumor activity in glioblastoma tumors. Here, we report that targeting different sarcoma cell lines with the Wnt inhibitor/Axin stabilizer SEN461 produces a less transformed phenotype, as supported by modulation of anchorage-independent growth in vitro. At the molecular level, SEN461 treatment enhanced the stability of the scaffold protein Axin1, a key negative regulator of the Wnt signaling with tumor suppressor function, resulting in downstream effects coherent with inhibition of canonical Wnt signaling. Genetic phenocopy of small molecule Axin stabilization, through Axin1 over-expression, coherently resulted in strong impairment of soft-agar growth. Importantly, sarcoma growth inhibition through pharmacological Axin stabilization was also observed in a xenograft model in vivo in female CD-1 nude mice. Our findings suggest the usefulness of Wnt inhibitors with Axin stabilization activity as a potentialyl clinical relevant strategy for certain types of sarcomas. PMID:24842792

  11. Human Sarcoma Growth Is Sensitive to Small-Molecule Mediated AXIN Stabilization

    PubMed Central

    Rossi, Marco; Valensin, Silvia; Tunici, Patrizia; Mori, Elisa; Caradonna, Nicola; Varrone, Maurizio; Salerno, Massimiliano

    2014-01-01

    Sarcomas are mesenchymal tumors showing high molecular heterogeneity, reflected at the histological level by the existence of more than fifty different subtypes. Genetic and epigenetic evidences link aberrant activation of the Wnt signaling to growth and progression of human sarcomas. This phenomenon, mainly accomplished by autocrine loop activity, is sustained by gene amplification, over-expression of Wnt ligands and co-receptors or epigenetic silencing of endogenous Wnt antagonists. We previously showed that pharmacological inhibition of Wnt signaling mediated by Axin stabilization produced in vitro and in vivo antitumor activity in glioblastoma tumors. Here, we report that targeting different sarcoma cell lines with the Wnt inhibitor/Axin stabilizer SEN461 produces a less transformed phenotype, as supported by modulation of anchorage-independent growth in vitro. At the molecular level, SEN461 treatment enhanced the stability of the scaffold protein Axin1, a key negative regulator of the Wnt signaling with tumor suppressor function, resulting in downstream effects coherent with inhibition of canonical Wnt signaling. Genetic phenocopy of small molecule Axin stabilization, through Axin1 over-expression, coherently resulted in strong impairment of soft-agar growth. Importantly, sarcoma growth inhibition through pharmacological Axin stabilization was also observed in a xenograft model in vivo in female CD-1 nude mice. Our findings suggest the usefulness of Wnt inhibitors with Axin stabilization activity as a potentialyl clinical relevant strategy for certain types of sarcomas. PMID:24842792

  12. The gamut of primary retroperitoneal masses: multimodality evaluation with pathologic correlation.

    PubMed

    Sangster, Guillermo P; Migliaro, Matias; Heldmann, Maureen G; Bhargava, Peeyush; Hamidian, Alireza; Thomas-Ogunniyi, Jaiyeola

    2016-07-01

    The retroperitoneum is a large space where primary and metastatic tumors grow silently before clinical signs appear. Neoplastic retroperitoneal diseases may be solid or cystic, primary or secondary and range from benign to aggressive in behavior. Retroperitoneal neoplasms are notable for their widely disparate histologies. The solid primary retroperitoneal neoplasms are extremely uncommon and can be classified based on their tissue of origin into three main categories: mesodermal tumors, neurogenic tumors, and extragonadal germ cell tumors. These tumors can grow to a large size before clinical symptoms occur or become palpable. When symptoms do occur, they are nonspecific. The majority of these masses are malignant and imaging plays a pivotal role in the detection, staging, and pre-operative planning. Benign and malignant masses should be distinguished whenever possible to avoid unnecessary surgical procedures. Macroscopic fat, calcification, necrosis, vascularity, and neural foraminal widening are common imaging features helping for tumor differentiation. Meticulous cross-sectional imaging can triage the patient to the most appropriate therapy. Tumor morphology dictates imaging character, and biologic activity is reflected by positron emission tomography (PET). Complete surgical excision with tumor free margins is essential for long-term survival. Biopsy should be performed in consultation with surgical oncology to avoid complicating curative surgery. This pictorial essay illustrates the spectrum of multidetector computed tomography (MDCT) imaging findings in common and uncommon primary retroperitoneal masses, with an emphasis on cross-sectional imaging features for an adequate tumor characterization and staging. PMID:27271217

  13. A malignant retroperitoneal mass--a rare presentation of recurrent thymoma.

    PubMed

    van Geffen, Wouter H; Sietsma, Johanna; Roelofs, Pieter M M; Hiltermann, Thijo J N

    2011-01-01

    A 60-year-old man presented with a suspected retroperitoneal mass, after primarily resected thymoma (type B1/B2, Masaoke stage 1). A germ cell tumour was excluded and a diagnostic biopsy was performed. The mass appeared to be a local recurrence of the primary thymoma, for example, a droplet metastasis, progressed to type B3. PMID:22674945

  14. A malignant retroperitoneal mass – A rare presentation of recurrent thymoma

    PubMed Central

    van Geffen, Wouter H; Sietsma, Johanna; Roelofs, Pieter MM; Hiltermann, Thijo JN

    2011-01-01

    A 60-year-old man presented with a suspected retroperitoneal mass, after primarily resected thymoma (type B1/B2, Masaoke stage 1). A germ cell tumour was excluded and a diagnostic biopsy was performed. The mass appeared to be a local recurrence of the primary thymoma, for example, a droplet metastasis, progressed to type B3. PMID:22674945

  15. Massive retroperitoneal lymphadenopathy as a terminal event in hairy cell leukaemia.

    PubMed

    Mehta, A B; Catovsky, D; O'Brien, C J; Lott, M; Bowley, N; Hemmingway, A

    1983-01-01

    A case of hairy cell leukaemia complicated as a terminal event by massive retroperitoneal lymphadenopathy is described. The patient had recently been treated with lithium carbonate and had previously been demonstrated to suffer from a systemic vasculitis, either or both of which may have contributed to the development of this rare complication. PMID:6360496

  16. Chyloperitoneum and chylothorax: a combined rare occurrence after retroperitoneal lymphadenectomy and radiotherapy for testis tumor.

    PubMed

    Dharman, K; Temes, S P; Wetherell, F E; Kendrick, M J

    1984-02-01

    We report a case of chyloperitoneum and chylothorax 5 weeks after retroperitoneal lymphadenectomy and radiotherapy for embryonal cell carcinoma of the testis. To our knowledge, this is the second reported case of this rare combined complication. The patient was treated successfully with an alimental dietary regimen (medium chain triglycerides and Vivonex), coupled with thoracenteses and paracenteses. PMID:6699970

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

    PubMed Central

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

    2015-01-01

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

  18. Major Types of Soft Tissue Sarcomas in Children

    MedlinePlus

    ... 253-8690 info@curesarcoma.org Follow @CureSarcoma on Twitter 11h Way to go, Damon and @happy2badad! #CureSarcoma ... to the extent permitted by law. Facebook Google+ Twitter LinkedIn YouTube © 2016 Sarcoma Foundation of America | All ...

  19. Primary Synovial Sarcoma of Lung: A Rare Tumor

    PubMed Central

    Kumar, Parveen; Sarin, Yogesh Kumar

    2016-01-01

    Synovial sarcoma of lung is a rare tumor with few case reports in literature. Though named synovial sarcoma due to its resemblance to synovium on light microscopy, it arises from mesenchymal tissue. Here, we present a case of synovial sarcoma of lung in a 7-year old boy, with main emphasis on difficulty faced in the management. PMID:27170917

  20. Primary Synovial Sarcoma of Lung: A Rare Tumor.

    PubMed

    Raj, Prince; Kumar, Parveen; Sarin, Yogesh Kumar

    2016-01-01

    Synovial sarcoma of lung is a rare tumor with few case reports in literature. Though named synovial sarcoma due to its resemblance to synovium on light microscopy, it arises from mesenchymal tissue. Here, we present a case of synovial sarcoma of lung in a 7-year old boy, with main emphasis on difficulty faced in the management. PMID:27170917

  1. Monophasic synovial sarcoma of the pharynx: a case report

    PubMed Central

    Betal, Dibendu; Babu, Ramesh; Mehmet, Veysi

    2009-01-01

    Synovial sarcomas are a rare form of soft tissue sarcomas. We present a case of a 62 year-old male presenting with a left thyroid lump initially though to be a thyroid adenoma but subsequently diagnosed as a monophasic synovial sarcoma of the pharynx. We discuss the diagnosis and treatment of this case. PMID:19335917

  2. The role of inflammation in sarcoma.

    PubMed

    Radons, Jürgen

    2014-01-01

    Sarcomas encompass a heterogenous group of tumors with diverse pathologically and clinically overlapping features. It is a rarely curable disease, and their management requires a multidisciplinary team approach. Chronic inflammation has emerged as one of the hallmarks of tumors including sarcomas. Classical inflammation-associated sarcomas comprise the inflammatory malignant fibrous histiocytoma and Kaposi sarcoma. The identification of specific chromosomal translocations and important intracellular signaling pathways such as Ras/Raf/MAPK, insulin-like growth factor, PI3K/AKT/mTOR, sonic hedgehog and Notch together with the increasing knowledge of angiogenesis has led to development of targeted therapies that aim to interrupt these pathways. Innovative agents like oncolytic viruses opened the way to design new therapeutic options with encouraging findings. Preclinical evidence also highlights the therapeutic potential of anti-inflammatory nutraceuticals as they can inhibit multiple pathways while being less toxic. This chapter gives an overview of actual therapeutic standards, newest evidence-based studies and exciting options for targeted therapies in sarcomas. PMID:24818727

  3. Primary osteogenic sarcoma of the mediastinum

    PubMed Central

    Ikeda, Takaaki; Ishihara, Tsuneo; Yoshimatsu, Hiroshi; Kikuchi, Keiichi; Murakami, Masaru; Kobayashi, Koichi; Inoue, Hiroshi; Kasahara, Masao

    1974-01-01

    Ikeda, T., Ishihara, T., Yoshimatsu, H., Kikuchi, K., Murakami, M., Kobayashi, K., Inoue, H., and Kasahara, M. (1974). Thorax, 582-588. Primary osteogenic sarcoma of the mediastinum. A 22-year-old man with primary osteogenic sarcoma of the superior mediastinum is reported. This case is the second instance of primary osteogenic sarcoma of the mediastinum and the first case of superior mediastinal origin to be reported. The patient had local recurrence one year after the first operation. After resection of the recurrent tumour with left upper lobectomy and partial pericardectomy followed by radiation, he has been well for more than five years without recurrence. Extraosseous osteogenic sarcoma of soft tissue is very rare and has been reported in 103 patients. The five-year survival of patients with extraosseous osteogenic sarcoma is 22·4%. The distribution and prognosis of this tumour are similar to those of rhabdomyosarcoma in soft tissue. Primary amputation or wide excision is the treatment of choice. Images PMID:4530480

  4. Uterine sarcoma Part II-Uterine endometrial stromal sarcoma: The TAG systematic review.

    PubMed

    Horng, Huann-Cheng; Wen, Kuo-Chang; Wang, Peng-Hui; Chen, Yi-Jen; Yen, Ming-Shyen; Ng, Heung-Tat

    2016-08-01

    Endometrial stromal tumors are rare uterine tumors (<1%). Four main categories include endometrial stromal nodule, low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and uterine undifferentiated sarcoma (UUS). This review is a series of articles discussing the uterine sarcomas. LG-ESS, a hormone-dependent tumor harboring chromosomal rearrangement, is an indolent tumor with a favorable prognosis, but characterized by late recurrences even in patients with Stage I disease, suggesting the requirement of a long-term follow-up. Patients with HG-ESS, based on the identification of YWHAE-NUTM2A/B (YWHAE-FAM22A/B) gene fusion, typically present with advanced stage diseases and frequently have recurrences, usually within a few years after initial surgery. UUS is, a high-grade sarcoma, extremely rare, lacking a specific line of differentiation, which is a diagnosis of exclusion (the wastebasket category, which fails to fulfill the morphological and immunohistochemical criteria of translocation-positive ESS). Surgery is the main strategy in the management of uterine sarcoma. Due to rarity, complex biological characteristics, and unknown etiology and risk factors of uterine sarcomas, the role of adjuvant therapy is not clear. Only LG-ESS might respond to progestins or aromatase inhibitors. PMID:27590366

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

    ClinicalTrials.gov

    2016-04-05

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

  6. [Soft tissue sarcomas and gastrointestinal stromal tumors].

    PubMed

    Reichardt, P

    2016-03-01

    Soft tissue sarcomas are rare tumors that represent a major challenge due to varying clinical presentations and often interdisciplinary treatment concepts. Gold standard for the treatment of localized resectable soft tissue sarcomas is complete surgical removal. In metastatic soft tissue sarcoma, systemic therapy is the treatment of choice. The most active drugs are anthracyclines and ifosfamide. Combination chemotherapy has improved both response rate and progression-free survival at the cost of increased toxicity. Imatinib at a dose of 400 mg/day is the gold standard for patients with advanced or metastatic gastrointestinal stromal tumors (GIST). In patients with a mutation in KIT exon 9, 800 mg/day is the recommended dose. In imatinib refractory or intolerant patients, sunitinib is recommended. Regorafenib has been approved for third-line therapy. PMID:26907871

  7. Primary pulmonary synovial sarcoma: a rare neoplasm

    PubMed Central

    Ramos, Montserrat Blanco; Fontán, Eva María García; Carretero, Miguel Ángel Cańizares; Pińeiro, Ana González

    2016-01-01

    Primary pulmonary synovial sarcoma is an extremely rare tumor with an unknown cause. The diagnosis is established after other primary lung malignancies or metastatic extrathoracic sarcoma have been excluded. We report the case of a 69-year-old man who presented with a well-defined mass in the right upper lobe on a chest X-ray. A video-assisted thoracoscopic surgery (VATS) right upper lobectomy was performed. Immunohistochemically, neoplastic cells were positive for vimentin, CD56 and Bcl-2, and focally positive for CD99, epithelial membrane antigen and cytokeratin 7 and 19. The cytogenetic study revealed a SYT genetic reassortment. So, the final pathological diagnosis was primary pulmonary synovial sarcoma. PMID:27516790

  8. Primary cardiac sarcoma after breast cancer.

    PubMed

    Ramalho, Joana; Nunes, Sandra; Marques, Irene; Marques, Franklim

    2013-01-01

    Primary cardiac sarcomas are rare tumours carrying poor prognosis. Postradiation sarcoma has been reported in patients with breast, cervical and head and neck cancers. We report a case of a 56-year-old woman with stage IIA breast cancer diagnosed in 1997, submitted to mastectomy, adjuvant chemotherapy, radiotherapy and hormonotherapy. Pulmonary metastasis were detected in 2008 and treated with chemotherapy and hormonotherapy, being in complete remission since August 2009. She was admitted in December 2009 with a 3-week history of fever, dyspnoea, polyarthralgias and leg oedema. An echocardiography showed a mass in the left atrium. She was submitted to a surgical tumour resection and the histology revealed a sarcoma of intermediate degree of differentiation. Chemoradiation therapy was started and she remains alive after 3 years, without tumour regrowth or metastasis. This case is a therapeutic challenge, because the previous therapies for breast cancer hampered the options for extra chemoradiation therapy. PMID:23608855

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

    ClinicalTrials.gov

    2014-09-08

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

  10. Human herpes virus 8 (Kaposi's sarcoma herpesvirus).

    PubMed

    Porter, S R; Di Alberti, L; Kumar, N

    1998-01-01

    Human herpes virus 8 (HHV-8) is a recently discovered herpesvirus related to Herpesvirus saimiri and Epstein-Barr virus (EBV). It has been assigned to the Rhadinovirus genus (gamma-2 herpesvirus) on the basis of its genomic sequence and structure. HHV-8 is the first member of this genus known to infect humans and it is now evident that it is the likely cause of Kaposi's sarcoma (KS). The virus is present in endothelial and spindle cells of KS, and in HIV disease the presence of HHV-8 in peripheral blood, and/or serum IgG antibodies to HHV-8, predicts the development of AIDS-related KS. HHV-8 can also infect CD19 + B cells and is of aetiological significance in the development of body cavity B cell lymphomas of AIDS. Of note, the translation products of viral open reading frames (ORFs) reveal HHV-8 to be a molecular pirate, capable of producing homologues of several human gene products that may result in alterations in cell cycle arrest, inhibit apoptosis and cell-mediated immune responses, and thus provide the potential for tumour production. PMID:9659514

  11. Use of powerful infrared pulsed Nd-YAG laser for treating osteogenic sarcoma

    NASA Astrophysics Data System (ADS)

    Biser, Vladimir A.; Kaplan, Michael A.; Kursova, Larisa V.; Neborak, Yuri T.

    1996-01-01

    Powerful infra-red laser radiation may induce necrosis of a malignant tumor located in a human bone without destructing skin cover. A superficial irradiation of the osteogenic sarcoma with an Nd-YAG laser (pulse power no less than 10 MW, average power 100 - 300 mW, time of exposure 10 - 90 min) has resulted in a severe damage of the tumor (more than 90% of the tumor mass) in 57% of cases. A combined laser/gamma irradiation showed a severe damage in 83% of cases. The results obtained suggest that laser radiation with the above parameters combined with gamma radiation may be used in treatment of osteogenic sarcoma.

  12. Synovial sarcoma of kidney in a child: A rare presentation.

    PubMed

    Radhakrishnan, Venkatraman; Dhanushkodi, Manikandan; Narayanswamy, Kathiresan; Raja, Anand; Sundersingh, Shirley; Sagar, Tenali

    2016-01-01

    There are no reported cases in the literature of primary renal synovial sarcoma in pediatric patients. The management of renal synovial sarcoma has been extrapolated from the management of soft tissue sarcomas at other sites. We present a 4-year-old female who was suspected to have Wilms' tumor. The patient underwent guided biopsy as she did not respond to neoadjuvant chemotherapy for Wilms' tumor. The biopsy was consistent with primary renal synovial sarcoma. The child was treated with change in her neoadjuvant chemotherapy regimen and surgery. The diagnosis of synovial sarcoma was confirmed by demonstrating the t (X, 18) translocation using polymerase chain reaction. PMID:27046979

  13. Synovial sarcoma of kidney in a child: A rare presentation

    PubMed Central

    Radhakrishnan, Venkatraman; Dhanushkodi, Manikandan; Narayanswamy, Kathiresan; Raja, Anand; Sundersingh, Shirley; Sagar, Tenali

    2016-01-01

    There are no reported cases in the literature of primary renal synovial sarcoma in pediatric patients. The management of renal synovial sarcoma has been extrapolated from the management of soft tissue sarcomas at other sites. We present a 4-year-old female who was suspected to have Wilms’ tumor. The patient underwent guided biopsy as she did not respond to neoadjuvant chemotherapy for Wilms’ tumor. The biopsy was consistent with primary renal synovial sarcoma. The child was treated with change in her neoadjuvant chemotherapy regimen and surgery. The diagnosis of synovial sarcoma was confirmed by demonstrating the t (X, 18) translocation using polymerase chain reaction. PMID:27046979

  14. Primary pulmonary monophasic synovial sarcoma: Evading diagnosis.

    PubMed

    Taylor, Marcus; Srinivasan, Lakshmi; Abid, Qamar

    2016-02-01

    Primary pulmonary synovial sarcoma is a very rare tumor, thus there is no consensus as to the most appropriate management. A 78-year-old man presented with nonspecific symptoms of weight loss and shortness of breath. Imaging confirmed a large right-sided mass and accompanying pleural effusion. Strong 18F-fluorodeoxyglucose uptake was found on positron-emission tomography. The preoperative work-up and intraoperative frozen section were inconclusive. Immunohistochemistry and molecular analysis confirmed the diagnosis of primary pulmonary monophasic synovial sarcoma. PMID:26612959

  15. Systemic Therapy for Advanced Soft Tissue Sarcoma.

    PubMed

    Sheng, Jennifer Y; Movva, Sujana

    2016-10-01

    Soft tissue sarcomas are rare tumors that present with distant metastasis in up to 10% of patients. Survival has improved significantly because of advancements in histologic classification and improved management approaches. Older agents such as doxorubicin, ifosfamide, gemcitabine, and paclitaxel continue to demonstrate objective response rates from 18% to 25%. Newer agents such as trabectedin, eribulin, aldoxorubicin, and olaratumab have demonstrated improvements in progression-free survival, overall survival, or toxicity profiles. Future studies on treatment of advanced soft tissue sarcoma will continue to concentrate on reducing toxicity, personalization of therapy, and targeting novel pathways. PMID:27542647

  16. Osteosarcoma With Apparent Ewing Sarcoma Gene Rearrangement.

    PubMed

    Mathias, Melissa D; Chou, Alexander J; Meyers, Paul; Shukla, Neerav; Hameed, Meera; Agaram, Narasimhan; Wang, Lu; Berger, Michael F; Walsh, Michael; Kentsis, Alex

    2016-07-01

    Poorly differentiated round cell sarcomas present diagnostic challenges because of their variable morphology and lack of specific immunophenotypic markers. We present a case of a 15-year-old female with a tibial tumor that exhibited features of Ewing-like sarcoma, including apparent rearrangement of the EWSR1 gene. Hybridization capture-based next-generation DNA sequencing showed evidence of complex genomic rearrangements, absence of known pathogenic Ewing-like chromosome translocations, and deletions RB1, PTCH1, and ATRX, supporting the diagnosis of osteosarcoma. This illustrates the potential of clinical genomic profiling to improve diagnosis and enable specifically targeted therapies for cancers with complex pathologies. PMID:27352193

  17. Molecular piracy of Kaposi's sarcoma associated herpesvirus.

    PubMed

    Choi, J; Means, R E; Damania, B; Jung, J U

    2001-01-01

    Kaposi's Sarcoma associated Herpesvirus (KSHV) is the most recently discovered human tumor virus and is associated with the pathogenesis of Kaposi's sarcoma, primary effusion lymphoma, and Multicentric Casttleman's disease. KSHV contains numerous open reading frames with striking homology to cellular genes. These viral gene products play a variety of roles in KSHV-associated pathogenesis by disrupting cellular signal transduction pathways, which include interferon-mediated anti-viral responses, cytokine-regulated cell growth, apoptosis, and cell cycle control. In this review, we will attempt to cover our understanding of how viral proteins deregulate cellular signaling pathways, which ultimately contribute to the conversion of normal cells to cancerous cells. PMID:11325605

  18. Nonmetastatic Ewing's Sarcoma of the Lumbar Spine in an Adult Patient

    PubMed Central

    Iacoangeli, Maurizio; Dobran, Mauro; Di Rienzo, Alessandro; di Somma, Lucia Giovanna Maria; Alvaro, Lorenzo; Moriconi, Elisa; Nocchi, Niccolò; Gladi, Maurizio; Scerrati, Massimo

    2012-01-01

    Although the spine is frequently involved in metastatic Ewing's sarcoma, primary involvement of the spine, beside sacrum, is much less frequent, especially in adult patients. Because of the low incidence of these tumors, there are currently no clinical guidelines outlining their management and a multitude of therapeutic strategies have been employed with varying success. The definitive management of Ewing's sarcoma of the spine, as in other locations, could include the combination of three main modalities: aggressive surgery, radiotherapy, and combined chemotherapy. Whenever possible, en bloc spondylectomy or extralesional resection is preferable, providing a better oncological result with a longer survival and a better preservation of the spine biomechanics. This is the lesson we learned about the case, we present here, of nonmetastatic lumbar localization by Ewing's sarcoma in as adult patient. PMID:23133768

  19. Pulmonary Surveillance Strategies Following Sarcoma Excision Vary Among Orthopedic Oncologists: A Survey of the Musculoskeletal Tumor Society

    PubMed Central

    Ries, Zachary; Gibbs, C. Parker; Scarborough, Mark T.; Miller, Benjamin J.

    2016-01-01

    Background Pulmonary surveillance protocols following sarcoma excision based on clinical evidence and outcomes are limited in current literature. The purpose of this study was to determine the method, frequency, and reasoning behind pulmonary surveillance strategies in patients treated for sarcoma among members of the Musculoskeletal Tumor Society (MSTS). Methods SurveyMonkey, an online survey tool, was used to create and distribute a questionnaire to 211 members of the MSTS in 2011. The 16 questions focused on current pulmonary surveillance algorithms and their reasoning. Results Of the surveyed members of the MSTS, 65% follow high-grade sarcoma with routine chest CT scans. Most disagreement involved low-grade sarcomas, where radiographs (34%), routine CT (33%), or selective CT scans (31%) were evenly distributed. Selective CT scans in low-grade lesions were warranted with an indeterminate nodule on prior CT (81%), local recurrence (40%), or large/ deep tumor characteristics (31%). Most protocols were based on continuation of training protocols (46%), clinician’s interpretation of the current literature (23%), or personal experience (14%). Conclusions Significant clinician variability exists in terms of pulmonary surveillance of sarcomas, most notably in low-grade lesions. The results of this study represent an area in need of further study to develop an evidence-based protocol for sarcoma pulmonary surveillance. PMID:27528846

  20. Detection and molecular cytogenetic characterization of a novel ring chromosome in a histological variant of Ewing sarcoma.

    PubMed

    Szuhai, Károly; IJszenga, Marije; Tanke, Hans J; Taminiau, Antonie H M; de Schepper, Arthur; van Duinen, Sjoerd G; Rosenberg, Carla; Hogendoorn, Pancras C W

    2007-01-01

    Ewing sarcoma/peripheral primitive neuro-ectodermal tumor (PNET) is a round-cell sarcoma that may show varying degrees of neuro-ectodermal differentiation. These tumors are identified by a characteristic round-cell morphology and immunohistochemical profile, as well as by specific translocations involving the EWS gene on chromosome 22 and the 3' portion of the E26 transformation-specific family of transcription factors. These translocations result in fusion proteins that act as aberrant transcription factors. The majority of Ewing sarcoma cases are characterized by a balanced t(11;22). Specific chromosomal abnormalities often correlate with distinct morphologic or phenotypic subtypes of tumors and play an important role in prognosis. Here we describe the molecular cytogenetic investigation of a case of Ewing sarcoma in the proximal humerus of a 39-year-old male using COBRA (combined binary ratio labelling) fluorescent in situ hybridization karyotyping, array comparative genomic hybridization, and EWS-gene specific fluorescence in situ hybridization. Multiple chromosomal aberrations were identified, including a der(22)r(20;22), resulting in an amplification of the proximal region of the EWS gene. This is the first time that both translocation and amplification involving the EWS gene and an unidentified gene are described. This case adds to the spectrum of both morphology and genetic rearrangements in Ewing sarcoma, and shows the importance of combined molecular cytogenetic approaches in identifying uncommon rearrangements in sarcomas. PMID:17175374

  1. Opposing Effects of Omega-3 and Omega-6 Long Chain Polyunsaturated Fatty Acids on the Expression of Lipogenic Genes in Omental and Retroperitoneal Adipose Depots in the Rat

    PubMed Central

    Muhlhausler, B. S.; Cook-Johnson, R.; James, M.; Miljkovic, D.; Duthoit, E.; Gibson, R.

    2010-01-01

    This study aimed to determine the effect of varying dietary intake of the major n-3 PUFA in human diets, α-linolenic acid (ALA; 18 : 3n-3), on expression of lipogenic genes in adipose tissue. Rats were fed diets containing from 0.095%en to 6.3%en ALA and a constant n-6 PUFA level for 3 weeks. Samples from distinct adipose depots (omental and retroperitoneal) were collected and mRNA expression of the pro-lipogenic transcription factors Sterol-Retinoid-Element-Binding-Protein1c (SREBP1c) and Peroxisome Proliferator Activated Receptor-γ (PPARγ), lipogenic enzymes Sterol-coenzyme Desaturase1 (SCD-1), Fatty Acid Synthase (FAS), lipoprotein lipase (LPL) and glycerol-3-phosphate dehydrogenase (G3PDH) and adipokines leptin and adiponectin determined by qRT-PCR. Increasing dietary ALA content resulted in altered expression of SREBP1c, FAS and G3PDH mRNA in both adipose depots. SREBP1c mRNA expression was related directly to n-6 PUFA concentrations (omental, r2 = .71; P < .001; Retroperitoneal, r2 = .20; P < .002), and inversely to n-3 PUFA concentrations (omental, r2 = .59; P < .001; Retroperitoneal, r2 = .19; P < .005) independent of diet. The relationship between total n-6 PUFA and SREBP1c mRNA expression persisted when the effects of n-3 PUFA were controlled for. Altering red blood cell concentrations of n-3 PUFA is thus associated with altered expression of lipogenic genes in a depot-specific manner and this effect is modulated by prevailing n-6 PUFA concentrations. PMID:20814437

  2. Risk factor analysis for massive lymphatic ascites after laparoscopic retroperitonal lymphadenectomy in gynecologic cancers and treatment using intranodal lymphangiography with glue embolization

    PubMed Central

    2016-01-01

    Objective To evaluate risk factors for massive lymphatic ascites after laparoscopic retroperitoneal lymphadenectomy in gynecologic cancer and the feasibility of treatments using intranodal lymphangiography (INLAG) with glue embolization. Methods A retrospective analysis of 234 patients with gynecologic cancer who received laparoscopic retroperitonal lymphadenectomy between April 2006 and November 2015 was done. In June 2014, INLAG with glue embolization was initiated to manage massive lymphatic ascites. All possible clinicopathologic factors related to massive lymphatic ascites were determined in the pre-INLAG group (n=163). Clinical courses between pre-INLAG group and post-INLAG group (n=71) were compared. Results In the pre-INLAG group (n=163), four patients (2.5%) developed massive lymphatic ascites postoperatively. Postoperative lymphatic ascites was associated with liver cirrhosis (three cirrhotic patients, p<0.001). In the post-INLAG group, one patient with massive lymphatic ascites had a congestive heart failure and first received INLAG with glue embolization. She had pelvic drain removed within 7 days after INLAG. The mean duration of pelvic drain and hospital stay decreased after the introduction of INLAG (13.2 days vs. 10.9 days, p=0.001; 15.2 days vs. 12.6 days, p=0.001). There was no evidence of recurrence after this procedure. Conclusion Underlying medical conditions related to the reduced effective circulating volume, such as liver cirrhosis and heart failure, may be associated with massive lymphatic ascites after retroperitoneal lymphadenectomy. INLAG with glue embolization can be an alternative treatment options to treat leaking lymphatic channels in patients with massive lymphatic leakage. PMID:27171674

  3. Intensity modulated radiation-therapy for preoperative posterior abdominal wall irradiation of retroperitoneal liposarcomas

    SciTech Connect

    Bossi, Alberto . E-mail: alberto.bossi@uz.kuleuven.ac.be; De Wever, Ivo; Van Limbergen, Erik; Vanstraelen, Bianca

    2007-01-01

    Purpose: Preoperative external-beam radiation therapy (preop RT) in the management of Retroperitoneal Liposarcomas (RPLS) typically involves the delivery of radiation to the entire tumor mass: yet this may not be necessary. The purpose of this study is to evaluate a new strategy of preop RT for RPLS in which the target volume is limited to the contact area between the tumoral mass and the posterior abdominal wall. Methods and Materials: Between June 2000 and Jan 2005, 18 patients with the diagnosis of RPLS have been treated following a pilot protocol of pre-op RT, 50 Gy in 25 fractions of 2 Gy/day. The Clinical Target Volume (CTV) has been limited to the posterior abdominal wall, region at higher risk for local relapse. A Three-Dimensional conformal (3D-CRT) and an Intensity Modulated (IMRT) plan were generated and compared; toxicity was reported following the National Cancer Institute (NCI) Common Terminology Criteria for Adverse Events v3.0. Results: All patients completed the planned treatment and the acute toxicity was tolerable: 2 patients experienced Grade 3 and 1 Grade 2 anorexia while 2 patients developed Grade 2 nausea. IMRT allows a better sparing of the ipsilateral and the contralateral kidney. All tumors were successfully resected without major complications. At a median follow-up of 27 months 2 patients developed a local relapse and 1 lung metastasis. Conclusions: Our strategy of preop RT is feasible and well tolerated: the rate of resectability is not compromised by limiting the preop CTV to the posterior abdominal wall and a better critical-structures sparing is obtained with IMRT.

  4. Relationship between impaired adipogenesis of retroperitoneal adipose tissue and hypertrophic obesity: role of endogenous glucocorticoid excess.

    PubMed

    Zubiría, María G; Vidal-Bravo, Juana; Spinedi, Eduardo; Giovambattista, Andrés

    2014-08-01

    Although the pro-adipogenic effect of glucocorticoid (GC) on adipose tissue (AT) precursor cell differentiation is openly accepted, the effect of chronically high peripheral levels of GC on AT mass expansion is not fully understood. In the present study, we aim to assess the in vitro adipogenic capacity of AT precursor cells isolated from retroperitoneal (RP) AT pads of the hypercorticosteronaemic, adult neonatally treated monosodium L-glutamate (MSG) male rat. To ascertain this issue, we explored the in vitro adipogenic process of stromal-vascular fraction (SVF) cells isolated from RPAT pads of 60-day-old MSG rats. The data recorded indicated that RPAT-SVF cells from hypercorticosteronaemic MSG rats, although displaying an enhanced proliferation capacity, differentiated slower than normal cells. This dysfunction was associated with a reduction in key parameters indicative of precursor cell commitment, differentiation capacity and the percentage of fully differentiated adipocytes, with a retarded maturation process. The distorted adipogenic capacity was highly conditioned by RPAT-SVF cells displaying a low committed population and both excessive and reduced expression of anti- (Pref-1 and Wnt-10b) and pro-adipogenic (mineralocorticoid receptor) signals respectively. Notably, the normalization of peripheral corticosterone levels in MSG rats, as a result of bilateral adrenalectomy combined with GC replacement therapy, fully prevented reduced RPAT precursor cell commitment and overall impaired adipogenesis. Our study strongly supports that the impaired adipogenic process observed in the adult hypertrophic obese MSG male rat is a GC-dependent mechanism, thus explaining the unhealthy RPAT expansion observed in human hypertrophic obese phenotypes, such as in the Cushing's syndrome. PMID:24913911

  5. Complete response after rechallenge with trabectedin in a patient with previously responding high-grade undifferentiated sarcoma

    PubMed Central

    Di Donato, Samantha; Fargnoli, Rossana; Dona, Manjola; Bertulli, Rossella; Parisi, Elisabetta; Fantini, Lorenzo; Sbaraglia, Marta; Panella, Mauro

    2016-01-01

    Evidence supporting rechallenge in patients responding to first exposure to trabectedin is limited. We report on a 39-year-old woman with advanced high-grade undifferentiated sarcoma (US) retreated twice with trabectedin after first response. The patient presented in June 2006 with an abdominal mass originating from the rear fascia of the rectus abdominis. Staging examinations did not indicate metastases and she underwent surgery; pathology showed a high-grade (FNCLCC G3) US. Subsequently, the patient received five cycles of adjuvant chemotherapy with epirubicin and ifosfamide. In February 2009 a computed tomography (CT) scan showed an abdominal mass involving the transverse mesocolon. R0 surgery was performed. In September 2009, peritoneal lesions appeared. Trabectedin was initiated at a dose of 1.5 mg/m2 by a 24 h intravenous infusion every 3 weeks, without relevant toxicity. After six cycles (March 2010), CT and PET-CT scans showed complete disappearance of metastases. In February 2012, new secondary lesions in the subdiaphragmatic region and a peritoneal lesion appeared. We rechallenged the patient with the same schedule of trabectedin; a complete response was achieved after two cycles. In October 2013, new secondary lesions in the subdiaphragmatic region and a retroperitoneal lesion were found. We rechallenged with the same schedule of trabectedin; PET-CT scans after two cycles showed complete response on the subdiaphragmatic lesion. Radiotherapy on the retroperitoneal lesion was performed. The patient underwent a total of 18 cycles and remains free from radiologically detectable disease. We report complete radiological remission after two rechallenges with trabectedin in a patient with previously responding high-grade US. PMID:27348763

  6. Wee1 inhibition by MK-1775 leads to tumor inhibition and enhances efficacy of gemcitabine in human sarcomas.

    PubMed

    Kreahling, Jenny M; Foroutan, Parastou; Reed, Damon; Martinez, Gary; Razabdouski, Tiffany; Bui, Marilyn M; Raghavan, Meera; Letson, Douglas; Gillies, Robert J; Altiok, Soner

    2013-01-01

    Sarcomas are rare and heterogeneous mesenchymal tumors affecting both pediatric and adult populations with more than 70 recognized histologies. Doxorubicin and ifosfamide have been the main course of therapy for treatment of sarcomas; however, the response rate to these therapies is about 10-20% in metastatic setting. Toxicity with the drug combination is high, response rates remain low, and improvement in overall survival, especially in the metastatic disease, remains negligible and new agents are needed. Wee1 is a critical component of the G2/M cell cycle checkpoint control and mediates cell cycle arrest by regulating the phosphorylation of CDC2. Inhibition of Wee1 by MK1775 has been reported to enhance the cytotoxic effect of DNA damaging agents in different types of carcinomas. In this study we investigated the therapeutic efficacy of MK1775 in various sarcoma cell lines, patient-derived tumor explants ex vivo and in vivo both alone and in combination with gemcitabine, which is frequently used in the treatment of sarcomas. Our data demonstrate that MK1775 treatment as a single agent at clinically relevant concentrations leads to unscheduled entry into mitosis and initiation of apoptotic cell death in all sarcomas tested. Additionally, MK1775 significantly enhances the cytotoxic effect of gemcitabine in sarcoma cells lines with different p53 mutational status. In patient-derived bone and soft tissue sarcoma samples we showed that MK1775 alone and in combination with gemcitabine causes significant apoptotic cell death. Magnetic resonance imaging (MRI) and histopathologic studies showed that MK1775 induces significant cell death and terminal differentiation in a patient-derived xenograft mouse model of osteosarcoma in vivo. Our results together with the high safety profile of MK1775 strongly suggest that this drug can be used as a potential therapeutic agent in the treatment of both adult as well as pediatric sarcoma patients. PMID:23520471

  7. Wee1 Inhibition by MK-1775 Leads to Tumor Inhibition and Enhances Efficacy of Gemcitabine in Human Sarcomas

    PubMed Central

    Kreahling, Jenny M.; Foroutan, Parastou; Reed, Damon; Martinez, Gary; Razabdouski, Tiffany; Bui, Marilyn M.; Raghavan, Meera; Letson, Douglas; Gillies, Robert J.; Altiok, Soner

    2013-01-01

    Sarcomas are rare and heterogeneous mesenchymal tumors affecting both pediatric and adult populations with more than 70 recognized histologies. Doxorubicin and ifosfamide have been the main course of therapy for treatment of sarcomas; however, the response rate to these therapies is about 10–20% in metastatic setting. Toxicity with the drug combination is high, response rates remain low, and improvement in overall survival, especially in the metastatic disease, remains negligible and new agents are needed. Wee1 is a critical component of the G2/M cell cycle checkpoint control and mediates cell cycle arrest by regulating the phosphorylation of CDC2. Inhibition of Wee1 by MK1775 has been reported to enhance the cytotoxic effect of DNA damaging agents in different types of carcinomas. In this study we investigated the therapeutic efficacy of MK1775 in various sarcoma cell lines, patient-derived tumor explants ex vivo and in vivo both alone and in combination with gemcitabine, which is frequently used in the treatment of sarcomas. Our data demonstrate that MK1775 treatment as a single agent at clinically relevant concentrations leads to unscheduled entry into mitosis and initiation of apoptotic cell death in all sarcomas tested. Additionally, MK1775 significantly enhances the cytotoxic effect of gemcitabine in sarcoma cells lines with different p53 mutational status. In patient-derived bone and soft tissue sarcoma samples we showed that MK1775 alone and in combination with gemcitabine causes significant apoptotic cell death. Magnetic resonance imaging (MRI) and histopathologic studies showed that MK1775 induces significant cell death and terminal differentiation in a patient-derived xenograft mouse model of osteosarcoma in vivo. Our results together with the high safety profile of MK1775 strongly suggest that this drug can be used as a potential therapeutic agent in the treatment of both adult as well as pediatric sarcoma patients. PMID:23520471

  8. Surgical advances in bone and soft tissue sarcoma: 50 years of progress.

    PubMed

    Henshaw, Robert M

    2014-01-01

    As the American Society of Clinical Oncology celebrates its 50th anniversary, physicians can appreciate the significant advances made in the treatment of patients with sarcoma. Historically, these rare tumors have garnered great interest in the medical profession, due to their ability to reach extraordinary size, resulting in substantial deformities and disabilities. Fortunately, advances in surgical management, which have occurred concurrently with advances in imaging, diagnostic techniques, and both local and systemic adjuvant treatments, offer patients diagnosed with sarcoma significant hope for successful treatment and the expectation of a meaningful quality of life. PMID:24857083

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

    ClinicalTrials.gov

    2016-05-26

    Childhood Alveolar Soft-part Sarcoma; Childhood Angiosarcoma; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Gliosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Neurofibrosarcoma; Childhood Synovial Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Osteosarcoma

  10. Granulocytic sarcoma of the rectum: a rare complication of myelodysplasia.

    PubMed Central

    Dabbagh, V; Browne, G; Parapia, L A; Price, J J; Batman, P A

    1999-01-01

    A 67 year old man with myelodysplasia was admitted as an emergency with a six week history of rectal bleeding and diarrhoea. Barium enema showed an irregular polypoid filling defect in the lateral wall of the proximal rectum near the rectosigmoid junction. Histology showed this to be a granulocytic sarcoma (extramedullary granulocytic leukaemia; chloroma) infiltrating the bowel. A low index of suspicion of this lesion results in an incorrect diagnosis in many such cases. A chloroacetate esterase immunoperoxidase stain will confirm the granulocytic nature of the tumour cells. Images PMID:10690184

  11. Targeting EZH2-mediated methylation of H3K27 inhibits proliferation and migration of Synovial Sarcoma in vitro.

    PubMed

    Shen, Jacson K; Cote, Gregory M; Gao, Yan; Choy, Edwin; Mankin, Henry J; Hornicek, Francis J; Duan, Zhenfeng

    2016-01-01

    Synovial sarcoma is an aggressive soft tissue sarcoma genetically defined by the fusion oncogene SS18-SSX. It is hypothesized that either SS18-SSX disrupts SWI/SNF complex inhibition of the polycomb complex 2 (PRC2) methyltransferase Enhancer of Zeste Homologue 2 (EZH2), or that SS18-SSX is able to directly recruit PRC2 to aberrantly silence target genes. This is of potential therapeutic value as several EZH2 small molecule inhibitors are entering early phase clinical trials. In this study, we first confirmed EZH2 expression in the 76% of human synovial sarcoma samples. We subsequently investigated EZH2 as a therapeutic target in synovial sarcoma in vitro. Knockdown of EZH2 by shRNA or siRNA resulted in inhibition of cell growth and migration across a series of synovial sarcoma cell lines. The EZH2 selective small-molecule inhibitor EPZ005687 similarly suppressed cell proliferation and migration. These data support the hypothesis that targeting EZH2 may be a promising therapeutic strategy in the treatment of synovial sarcoma; clinical trials are initiating enrollment currently. PMID:27125524

  12. Targeting EZH2-mediated methylation of H3K27 inhibits proliferation and migration of Synovial Sarcoma in vitro

    PubMed Central

    Shen, Jacson K.; Cote, Gregory M.; Gao, Yan; Choy, Edwin; Mankin, Henry J.; Hornicek, Francis J.; Duan, Zhenfeng

    2016-01-01

    Synovial sarcoma is an aggressive soft tissue sarcoma genetically defined by the fusion oncogene SS18-SSX. It is hypothesized that either SS18-SSX disrupts SWI/SNF complex inhibition of the polycomb complex 2 (PRC2) methyltransferase Enhancer of Zeste Homologue 2 (EZH2), or that SS18-SSX is able to directly recruit PRC2 to aberrantly silence target genes. This is of potential therapeutic value as several EZH2 small molecule inhibitors are entering early phase clinical trials. In this study, we first confirmed EZH2 expression in the 76% of human synovial sarcoma samples. We subsequently investigated EZH2 as a therapeutic target in synovial sarcoma in vitro. Knockdown of EZH2 by shRNA or siRNA resulted in inhibition of cell growth and migration across a series of synovial sarcoma cell lines. The EZH2 selective small-molecule inhibitor EPZ005687 similarly suppressed cell proliferation and migration. These data support the hypothesis that targeting EZH2 may be a promising therapeutic strategy in the treatment of synovial sarcoma; clinical trials are initiating enrollment currently. PMID:27125524

  13. Not All Acute Abdomen Cases in Early Pregnancy Are Ectopic; Expect the Unexpected: Renal Angiomyolipoma Causing Massive Retroperitoneal Haemorrhage.

    PubMed

    Rana, Muhammad Asim; Mady, Ahmed F; Jakaraddi, Nagesh; Mumtaz, Shahzad A; Ahmad, Habib; Naser, Kamal

    2016-01-01

    Retroperitoneal haemorrhage (or retroperitoneal haematoma) refers to an accumulation of blood found in the retroperitoneal space. It is a rare clinical entity with variable aetiology including anticoagulation, ruptured aortic aneurysm, acute pancreatitis, malignancy, and bleeding from renal aneurysm. Diagnosis of retroperitoneal bleed is sometimes missed or delayed as presentation is often nonspecific. Multislice CT and arteriography are important for diagnosis. There is no consensus about the best management plan for patients with retroperitoneal haematoma. Stable patients can be managed with fluid resuscitation, correction of coagulopathy if any, and blood transfusion. Endovascular options involving selective intra-arterial embolisation or stent-grafts are clearly getting more and more popularity. Open repair is usually reserved for cases when there is failure of conservative or endovascular measures to control the bleeding or expertise is unavailable and in cases where the patient is unstable. Mortality of patients with retroperitoneal haematoma remains high if appropriate and timely measures are not taken. Haemorrhage from a benign renal tumour is a rarer entity which is described in this case report which emphasizes that physicians should have a wide index of suspicion when dealing with patients presenting with significant groin, flank, abdominal, or back pain, or haemodynamic instability of unclear cause. Our patient presented with features of acute abdomen and, being pregnant, was thought of having a ruptured ectopic pregnancy. PMID:27429809

  14. Not All Acute Abdomen Cases in Early Pregnancy Are Ectopic; Expect the Unexpected: Renal Angiomyolipoma Causing Massive Retroperitoneal Haemorrhage

    PubMed Central

    Mady, Ahmed F.; Jakaraddi, Nagesh; Naser, Kamal

    2016-01-01

    Retroperitoneal haemorrhage (or retroperitoneal haematoma) refers to an accumulation of blood found in the retroperitoneal space. It is a rare clinical entity with variable aetiology including anticoagulation, ruptured aortic aneurysm, acute pancreatitis, malignancy, and bleeding from renal aneurysm. Diagnosis of retroperitoneal bleed is sometimes missed or delayed as presentation is often nonspecific. Multislice CT and arteriography are important for diagnosis. There is no consensus about the best management plan for patients with retroperitoneal haematoma. Stable patients can be managed with fluid resuscitation, correction of coagulopathy if any, and blood transfusion. Endovascular options involving selective intra-arterial embolisation or stent-grafts are clearly getting more and more popularity. Open repair is usually reserved for cases when there is failure of conservative or endovascular measures to control the bleeding or expertise is unavailable and in cases where the patient is unstable. Mortality of patients with retroperitoneal haematoma remains high if appropriate and timely measures are not taken. Haemorrhage from a benign renal tumour is a rarer entity which is described in this case report which emphasizes that physicians should have a wide index of suspicion when dealing with patients presenting with significant groin, flank, abdominal, or back pain, or haemodynamic instability of unclear cause. Our patient presented with features of acute abdomen and, being pregnant, was thought of having a ruptured ectopic pregnancy. PMID:27429809

  15. Combination Therapy for Advanced Kaposi Sarcoma

    Cancer.gov

    In this clinical trial, adult patients with any form of advanced Kaposi sarcoma will be treated with liposomal doxorubicin and bevacizumab every 3 weeks for a maximum of six treatments.  Patients who respond to this therapy or have stable disease will rec

  16. Instantánea del sarcoma

    Cancer.gov

    Información sobre las tendencias de incidencia, mortalidad y financiamiento del NCI sobre el sarcoma; así como ejemplos de actividades del NCI y adelantos en la investigación de este tipo de cáncer.

  17. Dual Pten/Tp53 Suppression Promotes Sarcoma Progression by Activating Notch Signaling

    PubMed Central

    Guijarro, Maria V.; Dahiya, Sonika; Danielson, Laura S.; Segura, Miguel F.; Vales-Lara, Frances M.; Menendez, Silvia; Popiolek, Dorota; Mittal, Khushbakhat; Wei, Jian Jun; Zavadil, Jiri; Cordon-Cardo, Carlos; Pandolfi, Pier Paolo; Hernando, Eva

    2014-01-01

    Soft tissue sarcomas are a heterogeneous group of tumors associated with poor clinical outcome. Although a subset of soft tissue sarcomas is characterized by simple karyotypes and recurrent chromosomal translocations, the mechanisms driving cytogenetically complex sarcomas are largely unknown. Clinical evidence led us to partially inactivate Pten and Tp53 in the smooth muscle lineage of mice, which developed high-grade undifferentiated pleomorphic sarcomas, leiomyosarcomas, and carcinosarcomas that widely recapitulate the human disease, including the aberrant karyotype and metastatic behavior. Pten was found haploinsufficient, whereas the wild-type allele of Tp53 invariably gained point mutations. Gene expression profiles showed up-regulated Notch signaling in PtenΔ/+Tp53Δ/+ tumors compared with Pten+/+Tp53Δ/+ tumors. Consistently, Pten silencing exacerbated the clonogenic and invasive potential of Tp53-deficient bone marrow–derived mouse mesenchymal stem cells and tumor cells and activated the Notch pathway. Moreover, the increased oncogenic behavior of PtenΔ/+Tp53Δ/+ and shPten-transduced Pten+/+Tp53Δ/+ tumor cells was counteracted by treatment with a γ-secretase inhibitor, suggesting that the aggressiveness of those tumors can be attributed, at least in part, to enhanced Notch signaling. This study demonstrates a cooperative role for Pten and Tp53 suppression in complex karyotype sarcomas while establishing Notch as an important functional player in the cross talk of these pathways during tumor progression. Our results highlight the importance of molecularly subclassifying patients with high-grade sarcoma for targeted treatments. PMID:23708211

  18. Current management of pediatric soft tissue sarcomas.

    PubMed

    Sangkhathat, Surasak

    2015-11-01

    Pediatric soft tissue sarcomas are a group of malignant neoplasms arising within embryonic mesenchymal tissues during the process of differentiation into muscle, fascia and fat. The tumors have a biphasic peak for age of incidence. Rhabdomyosarcoma (RMS) is diagnosed more frequently in younger children, whereas adult-type non-RMS soft tissue sarcoma is predominately observed in adolescents. The latter group comprises a variety of rare tumors for which diagnosis can be difficult and typically requires special studies, including immunohistochemistry and molecular genetic analysis. Current management for the majority of pediatric sarcomas is based on the data from large multi-institutional trials, which has led to great improvements in outcomes over recent decades. Although surgery remains the mainstay of treatment, the curative aim cannot be achieved without adjuvant treatment. Pre-treatment staging and risk classification are of prime importance in selecting an effective treatment protocol. Tumor resectability, the response to induction chemotherapy, and radiation generally determine the risk-group, and these factors are functions of tumor site, size and biology. Surgery provides the best choice of local control of small resectable tumors in a favorable site. Radiation therapy is added when surgery leaves residual disease or there is evidence of regional spread. Chemotherapy aims to reduce the risk of relapse and improve overall survival. In addition, upfront chemotherapy reduces the aggressiveness of the required surgery and helps preserve organ function in a number of cases. Long-term survival in low-risk sarcomas is feasible, and the intensity of treatment can be reduced. In high-risk sarcoma, current research is allowing more effective disease control. PMID:26566481

  19. Current management of pediatric soft tissue sarcomas

    PubMed Central

    Sangkhathat, Surasak

    2015-01-01

    Pediatric soft tissue sarcomas are a group of malignant neoplasms arising within embryonic mesenchymal tissues during the process of differentiation into muscle, fascia and fat. The tumors have a biphasic peak for age of incidence. Rhabdomyosarcoma (RMS) is diagnosed more frequently in younger children, whereas adult-type non-RMS soft tissue sarcoma is predominately observed in adolescents. The latter group comprises a variety of rare tumors for which diagnosis can be difficult and typically requires special studies, including immunohistochemistry and molecular genetic analysis. Current management for the majority of pediatric sarcomas is based on the data from large multi-institutional trials, which has led to great improvements in outcomes over recent decades. Although surgery remains the mainstay of treatment, the curative aim cannot be achieved without adjuvant treatment. Pre-treatment staging and risk classification are of prime importance in selecting an effective treatment protocol. Tumor resectability, the response to induction chemotherapy, and radiation generally determine the risk-group, and these factors are functions of tumor site, size and biology. Surgery provides the best choice of local control of small resectable tumors in a favorable site. Radiation therapy is added when surgery leaves residual disease or there is evidence of regional spread. Chemotherapy aims to reduce the risk of relapse and improve overall survival. In addition, upfront chemotherapy reduces the aggressiveness of the required surgery and helps preserve organ function in a number of cases. Long-term survival in low-risk sarcomas is feasible, and the intensity of treatment can be reduced. In high-risk sarcoma, current research is allowing more effective disease control. PMID:26566481

  20. [Synovial sarcoma in children and adolescents].

    PubMed

    Mansuy, Ludovic; Bernier, Valérie; Ranchère-Vince, Dominique; Mainard, Laurence; Orbach, Daniel; Corradini, Nadège

    2016-02-01

    Synovial sarcoma (SS) is a rare high-grade malignant mesenchymal tumor affecting children, adolescents, and young adults. Cytogenetically, more than 90% of SS is characterized by the t(X;18)(p11.2;q11.2), translocation resulting in two chimeric fusion genes SYT-SSX1 and SYT-SSX2, confirming histological diagnosis. Pediatric SS arises most often in soft tissues of the extremities (66% of cases), and is a localized tumor without spreading to regional lymph nodes (96% of cases) nor to metastatic sites (94% of cases). Although clinical and radiologic presentation, histologic analysis and tumor biology appear similar in pediatric and adolescent SS, outcome seems better in children than in adolescents, respectively 84% vs 60% of 5years overall survival (OS). If complete resection is the gold standard in SS, other therapeutic modalities differ between pediatric and adult populations, considering SS as an intermediate chemosensitive tumor more frequently by pediatric oncologists. Prognostic factors evaluation (tumor size, site of primary and IRS group) is necessary to establish optimal treatment strategies, with multimodal therapeutic approach in children and adolescents. Thus, recent results about the European prospective EpSSG NRSTS 05 study for children and adolescent patients with localized SS showed a 5years OS >90%. Moreover, recent somatic genetic data about SS open the debate on an appropriate strategy based and stratified on tumor genomic. Multinational prospective pediatric, adolescent and young adult study is necessary to improve optimal and appropriate approach in this rare tumor. PMID:26774699

  1. Radiation Therapy for Chloroma (Granulocytic Sarcoma)

    SciTech Connect

    Bakst, Richard; Wolden, Suzanne; Yahalom, Joachim

    2012-04-01

    Objectives: Chloroma (granulocytic sarcoma) is a rare, extramedullary tumor of immature myeloid cells related to acute nonlymphocytic leukemia or myelodysplastic syndrome. Radiation therapy (RT) is often used in the treatment of chloromas; however, modern studies of RT are lacking. We reviewed our experience to analyze treatment response, disease control, and toxicity associated with RT to develop treatment algorithm recommendations for patients with chloroma. Patients and Methods: Thirty-eight patients who underwent treatment for chloromas at our institution between February 1990 and June 2010 were identified and their medical records were reviewed and analyzed. Results: The majority of patients that presented with chloroma at the time of initial leukemia diagnosis (78%) have not received RT because it regressed after initial chemotherapy. Yet most patients that relapsed or remained with chloroma after chemotherapy are in the RT cohort (90%). Thirty-three courses of RT were administered to 22 patients. Radiation subsite breakdown was: 39% head and neck, 24% extremity, 9% spine, 9% brain, 6% genitourinary, 6% breast, 3% pelvis, and 3% genitourinary. Median dose was 20 (6-36) Gy. Kaplan-Meier estimates of progression-free survival and overall survival in the RT cohort were 39% and 43%, respectively, at 5 years. At a median follow-up of 11 months since RT, only 1 patient developed progressive disease at the irradiated site and 4 patients developed chloromas at other sites. RT was well tolerated without significant acute or late effects and provided symptom relief in 95% of cases. Conclusions: The majority of patients with chloromas were referred for RT when there was extramedullary progression, marrow relapse, or rapid symptom relief required. RT resulted in excellent local disease control and palliation of symptoms without significant toxicity. We recommend irradiating chloromas to at least 20 Gy, and propose 24 Gy in 12 fractions as an appropriate regimen.

  2. Clinicopathological implications of GNAS in Ewing sarcoma

    PubMed Central

    NOH, BYEONG-JOO; SUNG, JI-YOUN; KIM, YOUN WHA; ARAUJO, EDUARDO SANTINI; KALIL, RICARDO KARAM; JUNG, WOON-WON; KIM, HYUN-SOOK; PARK, YONG-KOO

    2016-01-01

    The objective of the present study was to determine whether guanine nucleotide-binding protein α stimulating (GNAS) gene expression correlates with pathognomonic signs by analyzing the mutations, methylation status and G-protein α subunit (Gsα) expression of GNAS in Ewing sarcoma (ES). Formalin-fixed paraffin-embedded tissue samples from 77 patients with primary ES were obtained in South Korea, Argentina and Brazil, and were studied via methylation chip assay and direct sequencing of the GNAS gene and immunohistochemical analysis of Gsα. The mutation and methylation statuses of the GNAS gene were examined. Immunohistochemical results were measured with respect to proportion and staining intensity. The results revealed that GNAS genes in ES tumor samples were less methylated compared with normal controls. No mutations were detected at exons 8 or 9 of the GNAS locus complex on chromosome 20q13.3, indicating that the pathogenesis of ES was not associated with GNAS mutation. Gsα expression correlated well with the methylation status of the GNAS gene. Notably, high Gsα expression was detected more frequently in samples from living patients than from decedents, although this was not statistically significant (P=0.055). In conclusion, GNAS mutation is not associated with the pathogenesis of ES tumors. This finding may be used to differentiate ES tumors from metastatic bone lesions with morphological similarity to ES tumors. Analysis of the methylation status of the GNAS gene and immunohistochemical Gsα expression suggests that hypermethylated GNAS (low Gsα expression) in ES may be associated with unfavorable progression with a non-significant trend. PMID:27313744

  3. Retroperitoneal abscess and acute acalculous cholecystitis after iatrogenic colon injury: report of a case

    PubMed Central

    Dong, Chengwei; Wang, Yuxu; Hu, Sanyuan; Du, Futian; Ding, Wei

    2015-01-01

    Acute acalculous cholecystitis has a high mortality rate due to the difficulties in early diagnosis and high rate of complications like empyema, gangrene and perforation. We report a case of 20-year-old male with acute severe pancreatitis, acute renal failure and acute peripancreatic fluid collection who was transferred to our department after blood filtration treatment in ICU. After percutaneous catheter drainage for 20 hours, the patient got a high fever. Computed tomography revealed retroperitoneal colon injury. In this case, percutaneous catheter drainage was performed again and the pus cavity was flushed regularly, after which the patient’s state gradually improved. Unpredictably, septic shock appeared on the 51st day. Repeated computed tomography revealed acute acalculous cholecystitis and abscess formation. After percutaneous transhepatic gallbladder catheterization and drainage, the patient got better gradually. Three months later the retroperitoneal catheter was removed. Four months later, ultrasound examination showed normal gallbladder and the catheter was removed. PMID:26131252

  4. Retroperitoneal lymphatic malformation and transverse testicular ectopia: a unique clinical presentation.

    PubMed

    Morris, Michael W; Cauthen, William; Bofill, James A; Blewett, Christopher J; Liechty, Kenneth W

    2013-04-01

    This case report presents a fetal patient diagnosed in utero with a retroperitoneal lymphatic malformation by ultrasound and followed through gestation. At birth the child was noted to have a right inguinal hernia with two palpable testicles. Plan for partial resection and hernia repair with postoperative sclerotherapy was made. At the time of hernia repair, transverse testicular ectopia was diagnosed, and subsequent extraperitoneal transposition orchiopexy was performed following partial resection of the lymphatic malformation. Delayed sclerotherapy in combination with partial resection afforded definitive treatment of the residual lymphatic malformation as the patient demonstrates no recurrence over one year later. This is the first reported case to suggest a direct relationship between transverse testicular ectopia and a retroperitoneal lymphatic malformation. PMID:23583159

  5. [Secondary retroperitoneal fibrosis in a 39-year-old man after rectal cancer].

    PubMed

    Jarosch, A; Tiller, M; Rohrbach, H; Leimbach, T; Schepp, W

    2016-05-01

    A 39-year-old man had been treated for rectal cancer 6 years ago by lower anterior resection of the rectum and perioperative radiochemotherapy. Since then follow-up had been unremarkable but now the patient presented with unspecific lower abdominal pain. The cause of the pain was identified as paraneoplastic retroperitoneal fibrosis secondary to metachronous pulmonary metastases of the rectal cancer. PMID:26895316

  6. The Retroperitoneal Laparoscopic Renal Capsulectomy for Spontaneous Renal Subcapsular Fluid Collection

    PubMed Central

    Zhu, Guodong; Wu, Dapeng; Wu, Kaijie; Song, Wenbin; Yang, Zhishang; Zhang, Yue; Zhang, Linlin; He, Dalin

    2016-01-01

    Abstract Spontaneous renal subcapsular fluid collection may occur as a rare presentation of nephritic syndrome, and distension of the renal capsula and Gerota fascia due to massive fluid accumulation may cause pain. In addition, hypertension secondary to renal ischemia and activation of renin–angiotensin–aldosterone system may also occur. The objective of this study is to evaluate the surgical outcome of retroperitoneal laparoscopic renal capsulectomy for patients with this disease. We retrospectively analyzed the clinical data of 10 female patients with spontaneous renal subcapsular fluid collection, diagnosed with B ultrasound and enhanced computed tomography (CT) scan. Eight patients first underwent percutaneous renal subcapsular drainage, which seemed to be less effective, and then all patients underwent retroperitoneal laparoscopic renal capsulectomy. The volume of renal subcapsular fluid was documented, the fluid was examined by routine biochemical tests, and the excised renal capsules underwent pathological examination individually. The postoperative drainage time for each patient was documented, and follow-up was conducted 1, 3, 6, 12 months, and 2 years postoperatively. Retroperitoneal laparoscopic renal capsulectomy was successfully performed in all patients with no major complications. The average volume of renal subcapsular fluid was 436 milliliter (mL, 180–880 mL) in light yellow color, and the concentration of creatinine and urea nitrogen was quite similar to that of serum. The pathological findings revealed fibrous dysplasia of the renal capsule with chronic infiltration of inflammatory cells. The average drainage time was 11.5 days (5–30 days) postoperatively. All patients recovered 1 month after the operation and there were no recurrences with a mean follow-up period of 12 months (6–24 months). The reason for spontaneous renal subcapsular fluid collection is unknown, and the aim of treatment is mainly to alleviate symptoms. In our

  7. Retroperitoneal nodular fasciitis: a benign etiology on the differential diagnosis of malignant gastric outlet obstruction

    PubMed Central

    Jiang, Wei; Coben, Robert M.

    2015-01-01

    Nodular fasciitis is a relatively rare, benign and proliferative lesion that is not typically found in the retroperitoneal (RP) space and has not been previously reported as a cause of gastric outlet obstruction (GOO). GOOs are frequently associated with malignancies, however, benign etiologies should be considered as well. We report the first case of GOO secondary to nodular fasciitis in the form of a spontaneously regressing RP mass that was initially concerning for malignancy. PMID:25830048

  8. Retroperitoneal nodular fasciitis: a benign etiology on the differential diagnosis of malignant gastric outlet obstruction.

    PubMed

    Kistler, C Andrew; Jiang, Wei; Coben, Robert M

    2015-04-01

    Nodular fasciitis is a relatively rare, benign and proliferative lesion that is not typically found in the retroperitoneal (RP) space and has not been previously reported as a cause of gastric outlet obstruction (GOO). GOOs are frequently associated with malignancies, however, benign etiologies should be considered as well. We report the first case of GOO secondary to nodular fasciitis in the form of a spontaneously regressing RP mass that was initially concerning for malignancy. PMID:25830048

  9. Primary squamous cell carcinoma of the stomach presenting as a huge retroperitoneal tumor: a case report.

    PubMed

    Wu, Xu-Dong; Zhou, Yong; Fan, Ren-Gen; Zhou, Bin; Shi, Quan; Jia, Jing

    2016-05-01

    A man complained of upper abdominal pain and early satiety for one month. An upper gastrointestinal endoscopy showed nothing specia. A CT scan of the abdomen was perfromed, which demonstrated a huge heterogeneous retroperitoneal mass close to the dorsal wall of the stomach and surrounding the abdominal aortic and celiac trunk. The resected specimen suggested that an un-regular tumor invaded to the dorsal wall of the stomach. Postoperative histological examination confirmed that it was a gastric squamous cell carcinoma. PMID:26181433

  10. Extra-Adrenal Retroperitoneal Paraganglioma with Extensive Duodenal Invasion and Inferior Vena Cava Tumor Thrombus.

    PubMed

    Sadamori, Hiroshi; Monden, Kazuteru; Hioki, Masayoshi; Yoshimoto, Masashi; Ueki, Toru; Hyodo, Tsuyoshi; Omonishi, Kunihiro; Kioka, Yukio; Kuriyama, Mitsuhito; Ohno, Satoshi; Sakaguchi, Kohsaku; Matsuda, Tadakazu; Takakura, Norihisa

    2016-08-01

    We report a case of extra-adrenal retroperitoneal paraganglioma (RP) with extensive duodenal invasion and tumor thromboses both in the right testicular vein and in the inferior vena cava (IVC). Because there was rigid adherence between the RP and the abdominal aorta, pancreatoduodenectomy with replacement of the IVC and aorta was performed for complete surgical resection. In the present case, both the mode of progression of the RP and the surgical approach were extremely rare. PMID:26921027

  11. Retroperitoneal Biloma due to Spontaneous Perforation of the Left Hepatic Duct

    PubMed Central

    Ishii, Kenjiro; Matsuo, Kazuhiro; Seki, Hiroaki; Yasui, Nobutaka; Sakata, Michio; Shimada, Akihiko; Matsumoto, Hidetoshi

    2016-01-01

    Patient: Male, 82 Final Diagnosis: Retroperitoneal biloma due to spontaneous perforation of the left hepatic duct Symptoms: Abdominal pain • high fever Medication: — Clinical Procedure: Emergent operation Specialty: Gastroenterology and Hepatology Objective: Rare disease Background: Spontaneous perforation of the bile duct in adults is very rare, particularly in cases accompanied by retroperitoneal biloma. We report a patient with retroperitoneal biloma due to a spontaneous perforation of the left hepatic duct. Case Report: An 82-year-old man was admitted to our institution with abdominal pain and a high fever. He had tenderness at the epi-mesogastrium. Computed tomography showed several stones in the gall bladder and common bile duct (CBD) and a few ascites. A substantial amount of fluid had collected from the dorsal stratum of the duodenum and pancreas head to the right paracolic gutter and anterior side of the right iliopsoas. Laboratory examination revealed a high inflammation score. He underwent emergent laparotomy. Biliary fluid was revealed after the mobilization of the pancreas head, duodenum, and right side of the colon. Bile duct perforation was suspected. Therefore, we exfoliated the dorsal side of the CBD to the cranial side, and intraoperative cholangiography was performed. However, the perforation site could not be detected. Cholecystectomy and choledocholithotomy were performed. A retrograde transhepatic biliary drainage tube was inserted, and primary closure of the CBD incision site was achieved. Postoperative cholangiography revealed leakage from the left hepatic duct near the caudate branch. Conclusions: There are a few reports of spontaneous bile duct perforation cases in the literature, particularly on infants or children with congenital anomalies, but it is rare in adults. It usually causes bile peritonitis, although bile duct perforation should be considered in the differential diagnosis of spontaneous retroperitoneal fluid collection in

  12. Fat-containing Retroperitoneal Lesions: Imaging Characteristics, Localization, and Differential Diagnosis.

    PubMed

    Shaaban, Akram M; Rezvani, Maryam; Tubay, Marc; Elsayes, Khaled M; Woodward, Paula J; Menias, Christine O

    2016-01-01

    The complex anatomy of the retroperitoneum is reflected in the spectrum of neoplastic and nonneoplastic conditions that can occur in the retroperitoneum and appear as soft-tissue masses. The presence of fat within a retroperitoneal lesion is helpful in refining the differential diagnosis. Fat is easily recognized because of its characteristic imaging appearance. It typically is hyperechoic at ultrasonography and demonstrates low attenuation at computed tomography (-10 to -100 HU). Magnetic resonance imaging is a more ideal imaging modality because it has better soft-tissue image contrast and higher sensitivity for depicting (a) microscopic fat by using chemical shift imaging and (b) macroscopic fat by using fat-suppression techniques. Whether a lesion arises from a retroperitoneal organ or from the soft tissues of the retroperitoneal space (primary lesion) is determined by examining the relationship between the lesion and its surrounding structures. Multiple imaging signs help to determine the organ of origin, including the "beak sign," the "embedded organ sign," the "phantom (invisible) organ sign," and the "prominent feeding artery sign." Adrenal adenoma is the most common adrenal mass that contains microscopic fat, while myelolipoma is the most common adrenal mass that contains macroscopic fat. Other adrenal masses, such as pheochromocytoma and adrenocortical carcinoma, rarely contain fat. Renal angiomyolipoma is the most common fat-containing renal mass. Other fat-containing renal lesions, such as lipoma and liposarcoma, are rare. Fatty replacement of the pancreas and pancreatic lipomas are relatively common, whereas pancreatic teratomas are rare. Of the primary retroperitoneal fat-containing lesions, lipoma and liposarcoma are common, while other lesions are relatively rare. (©)RSNA, 2016. PMID:27163589

  13. Collecting and Storing Tissue, Blood, and Bone Marrow Samples From Patients With Rhabdomyosarcoma or Other Soft Tissue Sarcoma

    ClinicalTrials.gov

    2016-03-18

    Adult Rhabdomyosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Chordoma; Desmoid Tumor; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Previously Untreated Childhood Rhabdomyosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  14. Examining the aetiopathogenesis of varicoceles: the relationship between retroperitoneal adipose tissue and testicular venous drainage.

    PubMed

    Umul, M; Değirmenci, B; Umul, A; Uçar, M; Yılmaz, Ö; Altok, M; Güneş, M; Orhan, H; Serel, T A

    2016-04-01

    This study evaluated the effect of retroperitoneal adipose tissue on testicular venous drainage and tested the nutcracker phenomenon by clinical and imaging findings. A total of 95 patients were included. The patients were evaluated with a detailed medical history and physical examination for varicocele. Their weight, height and waist circumference were also recorded. Body mass index was calculated as weight (kg)/height squared (m(2) ). Pampiniform plexus diameters were measured by scrotal colour Doppler ultrasonography, and retroperitoneal adipose tissue was evaluated by noncontrast abdominal computed tomography. We determined an almost significant correlation between BMI and varicocele presence by physical examination (P = 0.06). However, there was a significant relationship between WC and varicocele identified by physical examination (P = 0.021). There was a positive and significant relationship between BMI and pampiniform plexus diameters. Furthermore, we detected a negative correlation between retroperitoneal adipose tissue measurements and CDU findings. Additionally, there was a significant correlation between WC, pampiniform plexus diameters and CT findings. It is concluded that increasing BMI and increasing WC may play a protective role in the development of varicocele. There is a need for further studies to verify the effect of obesity on varicocele formation. PMID:26085083

  15. Fatal Retroperitoneal Bleeding Caused by Neurofibromatosis: A Case Report and Review of the Literature

    PubMed Central

    Moerbeek, Patrick R.; van Buijtenen, Jesse M.; van den Heuvel, Baukje; Hoksbergen, Arjan W. J.

    2015-01-01

    A young female was brought into the emergency department with pulseless electrical activity (PEA) after local resection of neurofibromateous lesions. Chest ultrasonography was normal. Abdominal ultrasonography was not performed. After successful resuscitation a total body CT-scan was performed to rule out potential bleeding sources. However, haemodynamic instability reoccurred and the scan had to be aborted at the thoracoabdominal level. No thoracic abnormalities were found. Resuscitation was reinitiated and abdominal ultrasonography was performed, showing a large amount of abdominal fluid. A progressive fall in haemoglobin was noted. Emergency laparotomy was performed, revealing a large retroperitoneal haematoma. Despite ligation and packing, bleeding continued. Postoperative angiography showed active bleeding from a branch of the left internal iliac artery, which could be successfully coiled. Unfortunately, the patient died five days later due to irreversible brain damage. Revision of an MRI scan made one year earlier showed a 10 cm large retroperitoneal neurofibromatous lesion exactly at the location of the current bleeding. This case shows that patients with neurofibromatosis might develop spontaneous life-threatening bleeding from retroperitoneal located lesions. Furthermore, it points out the necessity of focused assessment with ultrasonography of the abdomen in all patients with PEA of unknown origin. PMID:25688270

  16. Surgical management of retroperitoneal necrotising fasciitis by planned repeat laparotomy and debridement.

    PubMed

    Mokoena, T; Luvuno, F M; Marivate, M

    1993-06-01

    Mortality remains high in patients with necrotising fasciitis despite use of modern powerful antimicrobial drug therapy and advances in the care of the critically ill. This is particularly so in patients with intra-abdominal retroperitoneal lesions. While necrotising fasciitis of the integument has been well described, its retroperitoneal (extraperitoneal) location has not been highlighted. Planned repeated laparotomies and debridement have been used in 10 recent patients with only 2 deaths. The initiating incident was caesarean section in 3 patients; perineal sepsis, trauma or intra-uterine death in 2 patients each; and uterine instrumentation to induce early abortion in the remaining patient. All patients received empirical antimicrobial therapy, which was changed when microbial isolates and their sensitivity indicated. Mechanical ventilation support was given to 60% of the patients early on during the illness. All received nutritional supplementation either parenterally or enterally throughout their inhospital treatment. This report focuses on intra-abdominal extraperitoneal necrotising fasciitis and the use of planned repeated laparotomy and debridement in the treatment of retroperitoneal (extraperitoneal) necrotising fasciitis per se and we recommend it as an essential part of the management of this condition. PMID:8211410

  17. Retroperitoneal disorders associated with IgG4-related autoimmune pancreatitis.

    PubMed

    Hara, Noboru; Kawaguchi, Makoto; Takeda, Keisuke; Zen, Yoh

    2014-11-28

    IgG4-related autoimmune pancreatitis is frequently accompanied by relevant lesions in the genitourinary tract and retroperitoneal organs, which cause various clinical problems, ranging from non-specific back pain or bladder outlet obstruction to renal failure. The diagnosis of IgG4-related retroperitoneal fibrosis requires a multidisciplinary approach, including serological tests, histological examination, imaging analysis, and susceptibility to steroid therapy. Radiological examinations are helpful to diagnose this condition, but surgical resection is occasionally unavoidable to exclude malignancy, particularly for patients with isolated retroperitoneal involvement. Steroid therapy is the treatment of choice for this condition, the same as for other manifestations of IgG4-related disease. For patients with severe ureteral obstruction, additional ureteral stenting needs to be considered prior to steroid therapy to preserve the renal function. Some papers have suggested that IgG4-related disease can affect male reproductive organs including the prostate and testis. IgG4-related prostatitis usually causes lower urinary tract symptoms, such as dysuria and pollakisuria. Patients sometimes state that corticosteroids given for IgG4-related disease at other sites relieve their lower urinary tract symptoms, which leads us to suspect prostatic involvement in this condition. Because of the limited number of publications available, further studies are warranted to better characterize IgG4-related disease in male reproductive organs. PMID:25469023

  18. Lymphangiectatic Kaposi's sarcoma in a patient with AIDS.

    PubMed

    Santos, Mônica; Vilasboas, Virginia; Mendes, Luciana; Talhari, Carolina; Talhari, Sinésio

    2013-01-01

    Kaposi's sarcoma is a malignant disease that originates in the lymphatic endothelium. It has a broad spectrum of clinical manifestations. Its four distinct clinical forms are: classic, endemic, iatrogenic and epidemic Kaposi's sarcoma. In non-HIV-associated Kaposi's sarcoma, the disease is typically limited to the lower extremities, but in immunodeficient patients, it is a multifocal systemic disease. The clinical course of the disease differs among patients, ranging from a single or a few indolent lesions to an aggressive diffuse disease. Advanced Kaposi's sarcoma lesions, typically those on the lower extremities, are often associated with lymphedema. In this paper, we report a case of a patient with a rare form of AIDS-associated Kaposi sarcoma called lymphangiectatic Kaposis's sarcoma. PMID:23739700

  19. Proton Radiotherapy for Pediatric Ewing's Sarcoma: Initial Clinical Outcomes

    SciTech Connect

    Rombi, Barbara; DeLaney, Thomas F.; MacDonald, Shannon M.; Huang, Mary S.; Ebb, David H.; Liebsch, Norbert J.; Raskin, Kevin A.; Yeap, Beow Y.; Marcus, Karen J.; Tarbell, Nancy J.; Yock, Torunn I.

    2012-03-01

    Purpose: Proton radiotherapy (PT) has been prescribed similarly to photon radiotherapy to achieve comparable disease control rates at comparable doses. The chief advantage of protons in this setting is to reduce acute and late toxicities by decreasing the amount of normal tissue irradiated. We report the preliminary clinical outcomes including late effects on our pediatric Ewing's sarcoma patients treated with PT at the Francis H. Burr Proton Therapy Center at Massachusetts General Hospital (Boston, MA). Methods and Materials: This was a retrospective review of the medical records of 30 children with Ewing's sarcoma who were treated with PT between April 2003 and April 2009. Results: A total of 14 male and 16 female patients with tumors in several anatomic sites were treated with PT at a median age of 10 years. The median dose was 54 Gy (relative biological effectiveness) with a median follow-up of 38.4 months. The 3-year actuarial rates of event-free survival, local control, and overall survival were 60%, 86%, and 89%, respectively. PT was acutely well tolerated, with mostly mild-to-moderate skin reactions. At the time of writing, the only serious late effects have been four hematologic malignancies, which are known risks of topoisomerase and anthracyline exposure. Conclusions: Proton radiotherapy was well tolerated, with few adverse events. Longer follow-up is needed to more fully assess tumor control and late effects, but the preliminary results are encouraging.

  20. The First European Interdisciplinary Ewing Sarcoma Research Summit

    PubMed Central

    Kovar, Heinrich; Alonso, Javier; Aman, Pierre; Aryee, Dave N. T.; Ban, Jozef; Burchill, Sue A.; Burdach, Stefan; De Alava, Enrique; Delattre, Olivier; Dirksen, Uta; Fourtouna, Argyro; Fulda, Simone; Helman, Lee J.; Herrero-Martin, David; Hogendoorn, Pancras C. W.; Kontny, Udo; Lawlor, Elizabeth R.; Lessnick, Stephen L.; Llombart-Bosch, Antonio; Metzler, Markus; Moriggl, Richard; Niedan, Stephan; Potratz, Jenny; Redini, Françoise; Richter, Günther H. S.; Riedmann, Lucia T.; Rossig, Claudia; Schäfer, Beat W.; Schwentner, Raphaela; Scotlandi, Katia; Sorensen, Poul H.; Staege, Martin S.; Tirode, Franck; Toretsky, Jeffrey; Ventura, Selena; Eggert, Angelika; Ladenstein, Ruth

    2012-01-01

    The European Network for Cancer Research in Children and Adolescents (ENCCA) provides an interaction platform for stakeholders in research and care of children with cancer. Among ENCCA objectives is the establishment of biology-based prioritization mechanisms for the selection of innovative targets, drugs, and prognostic markers for validation in clinical trials. Specifically for sarcomas, there is a burning need for novel treatment options, since current chemotherapeutic treatment protocols have met their limits. This is most obvious for metastatic Ewing sarcoma (ES), where long term survival rates are still below 20%. Despite significant progress in our understanding of ES biology, clinical translation of promising laboratory results has not yet taken place due to fragmentation of research and lack of an institutionalized discussion forum. To fill this gap, ENCCA assembled 30 European expert scientists and five North American opinion leaders in December 2011 to exchange thoughts and discuss the state of the art in ES research and latest results from the bench, and to propose biological studies and novel promising therapeutics for the upcoming European EWING2008 and EWING2012 clinical trials. PMID:22662320

  1. Radiation-induced prostatic sarcoma: A case report

    SciTech Connect

    Scully, J.M.; Uno, J.M.; McIntyre, M.; Mosely, S. )

    1990-09-01

    A 64-year-old man had a prostatic sarcoma 8 years after transurethral prostatectomy and radical bilateral pelvic lymph node dissection with insertion of 125-iodine implants for stage B1N carcinoma of the prostate. Therapy for the sarcoma consisted of isolated pelvic perfusion and then pelvic exenteration with creation of an ileal conduit and colostomy. The pathology report showed well encapsulated grade 2 spindle cell sarcoma of the prostate. Multiple small metallic particles were embedded in the tumor specimen.

  2. Undifferentiated embryonal sarcoma of the liver in an adult patient.

    PubMed

    Lee, Kyu Ho; Maratovich, Mussin Nadiar; Lee, Kyoung-Bun

    2016-06-01

    Undifferentiated embryonal sarcoma of the liver (UESL) is rare primary hepatic sarcoma and is known to occur in pediatric patients. This case is the UESL occurred in a 51-year old male patient. Multilocular cystic lesion was composed of primitive spindle cells without specific differentiation. This rare case would help to review differential diagnosis of primary sarcoma in liver and cystic neoplasm of the liver. PMID:27377912

  3. Undifferentiated embryonal sarcoma of the liver in an adult patient

    PubMed Central

    Lee, Kyu Ho; Maratovich, Mussin Nadiar; Lee, Kyoung-Bun

    2016-01-01

    Undifferentiated embryonal sarcoma of the liver (UESL) is rare primary hepatic sarcoma and is known to occur in pediatric patients. This case is the UESL occurred in a 51-year old male patient. Multilocular cystic lesion was composed of primitive spindle cells without specific differentiation. This rare case would help to review differential diagnosis of primary sarcoma in liver and cystic neoplasm of the liver. PMID:27377912

  4. Comparison of MMF with prednisone in terms of rejection and duration of activity of transplant in rabbits that underwent retroperitoneal heterotopic heart transplantation

    PubMed Central

    Aygün, Fatih; Efe, Duran; Durgut, Kadir

    2015-01-01

    Summary Aim In this study, mycophenolate mofetil (MMF) and methylprednisolone (MP) were compared in terms of rejection and duration of activity of the transplant in New Zealand rabbits that underwent retroperitoneal heart transplantation. Methods Retroperitoneal heart transplantation was performed in New Zealand white rabbits. The animals were divided into two groups. MMF group (group 1) (10 donors, 10 recipients): 12.5 mg/kg MMF was administered orally for two days prior to the surgery; MP group (group 2) (nine donors, nine recipients): 2 mg/kg MP was administered intramuscularly for two days prior to the surgery. After the operation, we waited until all motor activity in the transplanted heart had stopped. The transplant was then removed and the recipient was sacrificed. A donor in the MP group was excluded since it died before the motor activity had stopped. Results No statistically significant difference was found between the groups in terms of rejection score (p = 0.865). However, duration of motor activity was found to be statistically significantly longer in the MMF group, compared to the MP group (p = 0.013). Conclusion In this experimental study, MMF was similar to MP in terms of rejection but had better efficacy in terms of duration of motor activity of the transplant. PMID:26592904

  5. Transanal natural orifice transluminal endoscopic surgery total mesorectal excision in animal models: endoscopic inferior mesenteric artery dissection made easier by a retroperitoneal approach

    PubMed Central

    Park, Sun Jin; Chang, Tae Young; Jung, Yunho; Kim, Hyung Jin; Kim, Young Ill; Chun, Ho-Kyung

    2014-01-01

    Purpose We report the performance of natural orifice transluminal endoscopic surgery (NOTES) low anterior resection in animals using transanal total mesorectal excision (TME) with laparoscopic assistance and endoscopic inferior mesenteric artery (IMA) dissection. Methods Four pigs weighing 45 kg each, and one dog weighing 25 kg, underwent surgery via a transanal approach. The rectum was occluded transanally using a purse-string suture, approximately 3-4 cm from the anal verge. The rectal mucosa was incised circumferentially just distal to the purse-string. A SILS or GelPOINT port was inserted transanally. Transanal TME was assisted by laparoscopy and proceeded up to the peritoneal reflection. More proximal dissection, including IMA dissection, was performed along the retroperitoneal avascular plane by endoscopy alone and facilitated by CO2 insufflation. The IMA was clipped and divided endoscopically. The mobilized rectosigmoid were exteriorized transanally and transected. A colorectal anastomosis was performed using a circular stapler with a single stapling technique. Results Endoscopic dissection of the IMA was successful in all five animals. The mean operation time was 125 minutes (range, 90-170 minutes). There were no intraoperative complications or hemodynamic instability. The mean length of the resected specimen was 14.4 cm (range, 12-16 cm). Conclusion A NOTES retroperitoneal approach to the IMA with CO2 insufflation and intact peritoneal covering overcame the difficulties of retraction and exposure of endoscopic dissection in animals. PMID:25025019

  6. Therapeutic opportunities in Ewing sarcoma: EWS-FLI inhibition via LSD1 targeting.

    PubMed

    Theisen, Emily R; Pishas, Kathleen I; Saund, Ranajeet S; Lessnick, Stephen L

    2016-04-01

    Ewing sarcoma is an aggressive primary pediatric bone tumor, often diagnosed in adolescents and young adults. A pathognomonic reciprocal chromosomal translocation results in a fusion gene coding for a protein which derives its N-terminus from a FUS/EWS/TAF15 (FET) protein family member, commonly EWS, and C-terminus containing the DNA-binding domain of an ETS transcription factor, commonly FLI1. Nearly 85% of cases express the EWS-FLI protein which functions as a transcription factor and drives oncogenesis. As the primary genomic lesion and a protein which is not expressed in normal cells, disrupting EWS-FLI function is an attractive therapeutic strategy for Ewing sarcoma. However, transcription factors are notoriously difficult targets for the development of small molecules. Improved understanding of the oncogenic mechanisms employed by EWS-FLI to hijack normal cellular programming has uncovered potential novel approaches to pharmacologically block EWS-FLI function. In this review we examine targeting the chromatin regulatory enzymes recruited to conspire in oncogenesis with a focus on the histone lysine specific demethylase 1 (LSD1). LSD1 inhibitors are being aggressively investigated in acute myeloid leukemia and the results of early clinical trials will help inform the future use of LSD1 inhibitors in sarcoma. High LSD1 expression is observed in Ewing sarcoma patient samples and mechanistic and preclinical data suggest LSD1 inhibition globally disrupts the function of EWS-ETS proteins. PMID:26848860

  7. Adjuvant Chemotherapy Following Complete Resection of Soft Tissue Sarcoma in Adults: A Clinical Practice Guideline

    PubMed Central

    Bramwell, Vivien H. C.; Bell, Robert; Davis, Aileen M.; Charette, Manya L.; The members of the cancer care Ontario practice guidelines initiative sarcoma disease site group

    2002-01-01

    Purpose. To review the literature and make recommendations for the use of anthracycline-based adjuvant chemotherapy in adult patients with soft tissue sarcoma (STS). Patients. The recommendations apply to patients >15 years old with completely resected STS. Methods. A systematic overview of the published literature was combined with a consensus process around the interpretation of the evidence in the context of conventional practice to develop an evidence-based practice guideline. Results. Four meta-analyses and 17 randomized clinical trials comparing anthracycline-based adjuvant chemotherapy versus observation were reviewed. The Sarcoma Meta-Analysis Collaboration (SMAC) was the best analysis because it assessed individual patient data and had the longest follow-up. The results of the SMAC meta-analysis together with data from more recently published randomized trials, as well as our analysis of the toxicity and compliance data, are incorporated in this systematic review. Discussion. It is reasonable to consider anthracycline-based adjuvant chemotherapy in patients who have had removal of a sarcoma with features predicting a high likelihood of relapse (deep location, size >5 cm, high histological grade). Although the benefits of adjuvant chemotherapy are most apparent in patients with extremity sarcomas, patients with high-risk tumours at other sites should also be considered for such therapy. PMID:18521341

  8. An Australasian perspective on sarcoma research, translational biology and clinical trials: the Australasian Sarcoma Study Group (ASSG).

    PubMed

    Bae, Susie; Caruso, Denise; Desai, Jayesh

    2014-02-01

    Each year approximately 800 Australians are diagnosed with sarcoma, accounting for less than 1% of cancer diagnoses overall. A significant proportion of these sarcoma cases are in children and adolescents. The rarity and heterogeneity of this group of tumours, coupled with Australasia's relative geographical isolation, pose significant challenges in developing locoregional basic, translational and clinical research. The Australasian Sarcoma Study Group (ASSG) was established in 2008 as a Cooperative Cancer Clinical Research Group and is now the peak body for sarcoma research in Australasia, providing a mechanism to drive and coordinate collaborative research, promote education and assist with advocating for sarcoma within the region. This paper describes the development of ASSG and examines the current state of play with regard to sarcoma research in Australasia. PMID:24378392

  9. Granulocytic Sarcoma in MLL-Positive Infant Acute Myelogenous Leukemia

    PubMed Central

    Park, Kyoung Un; Lee, Dong Soon; Lee, Hye Seung; Kim, Chong Jai; Cho, Han Ik

    2001-01-01

    Granulocytic sarcoma is considered to be rare and its frequent occurrence is associated with specific genetic changes such as t(8;21). To investigate an association between MLL (mixed lineage leukemia or myeloid-lymphoid leukemia) rearrangement and granulocytic sarcoma, we applied fluorescence in situ hybridization for detection of the 11q23/MLL rearrangements on the bone marrow cells of 40 patients with childhood acute myelogenous leukemia (AML). Nine (22.5%) of 40 patients exhibited MLL rearrangements. Three (33.3%) of these nine patients had granulocytic sarcoma and were younger than 12 months of age. Of these three patients one presented as granulocytic sarcoma of both testes with cerebrospinal fluid involvement, the second case presented in the form of an abdominal mass, and the third as a periorbital granulocytic sarcoma. On the other hand, no granulocytic sarcomas were found among MLL-negative patients. It is likely that MLL-positive infant AML may predispose granulocytic sarcoma. Regarding the findings of our study and those of other reports, we would guess that the incidence of granulocytic sarcoma in pediatric MLL-positive AML may be equal to or greater than the 18 to 24% described in AML with t(8;21). Further investigations designed to identify 11q23/MLL abnormalities of leukemic cells or extramedullary tumor may be helpful for the precise diagnosis of granulocytic sarcoma. PMID:11733351

  10. Refractory ulcerative colitis and iatrogenic colorectal Kaposi's sarcoma.

    PubMed

    Girelli, C M; Serio, G; Rocca, E; Rocca, F

    2009-02-01

    Colorectal Kaposi's sarcoma, a human herpes virus-8 associated mesenchymal tumour, is exceedingly rare in human immunodeficiency virus-negative subjects and almost always reported in association with severe, refractory, inflammatory bowel disease. In this paper we report a case--the second from Italy--of a colorectal Kaposi's sarcoma in a human immunodeficiency virus-negative, heterosexual man with severe refractory ulcerative colitis. Kaposi's sarcoma developed after starting glucocorticosteroid therapy, supporting the theory that colorectal Kaposi's sarcoma associated with ulcerative colitis is iatrogenic. PMID:18054849

  11. Recent advances in targeted therapy for Ewing sarcoma

    PubMed Central

    Pishas, Kathleen I.; Lessnick, Stephen L.

    2016-01-01

    Ewing sarcoma is an aggressive, poorly differentiated neoplasm of solid bone that disproportionally afflicts the young. Despite intensive multi-modal therapy and valiant efforts, 70% of patients with relapsed and metastatic Ewing sarcoma will succumb to their disease. The persistent failure to improve overall survival for this subset of patients highlights the urgent need for rapid translation of novel therapeutic strategies. As Ewing sarcoma is associated with a paucity of mutations in readily targetable signal transduction pathways, targeting the key genetic aberration and master regulator of Ewing sarcoma, the EWS/ETS fusion, remains an important goal.

  12. [Synovial sarcoma of the nasal cavity. A case report].

    PubMed

    Acosta Díaz, Hander; Trinidad Ruiz, Gabriel; Rejas, Eladio; Pando, Jesús; Cabrera, J J

    2011-01-01

    Synovial sarcoma is a rare tumour found in soft tissue; it is a mesenchymal spindle cell tumour that is not related to the synovial membrane. This tumour has a low incidence, the most frequent place of occurrence being the lower extremities in young adults. Synovial sarcoma of the head and neck accounts for 3-5% of sarcomas in this anatomical region. The treatment of choice for synovial sarcoma of the head and neck is complete surgical excision of the tumour mass followed by adjuvant radiotherapy. PMID:20466354

  13. Osteogenic Sarcoma of the Maxilla: Neutron Therapy for Unresectable Disease

    DOE PAGESBeta

    Smoron, Geoffrey L.; Lennox, Arlene J.; Mcgee, James L.

    1999-01-01

    Purpose. To present a case study involving the use of fast neutron therapy to treat an extensive unresectable osteogenic sarcoma arising from the left maxilla. Patient. A 14-year-old male presented with a massive tumor producing severe distortion of his facial structures. He had already received six courses of chemotherapy, which had reduced his pain, but had not measurably reduced the tumor. Methods. The patient was treated with 66 MeV fast neutrons to a dose of 20.4 Gy in 13 fractions over 35 days. Results. CT assessments indicate gradually increasing calcification and noticeable reduction of soft-tissue disease in the frontalmore » sinus, orbit and maxillary antrum.There has been some recontouring of the facial structures.The boy conducts an active life, has no pain, and feels well. He was 17 years old at the last follow-up. Discussion. Fast neutrons have a greater biological effectiveness than conventional photon beams. Their use has been associated with improved chance for local control of unresectable disease.This case illustrates their effectiveness in controlling an unusual and aggressive osteogenic sarcoma of the facial bone and sinuses.« less

  14. Undifferentiated embryonal liver sarcoma in childhood: A case report

    PubMed Central

    HE, BIN; XU, KEYU; HUANG, KATE; HUANG, YUEHAN; LI, PENG; CHEN, ZHENKUN; SHI, HONGQI; ZHANG, QIYU; SHAN, YUNFENG

    2014-01-01

    In order to improve the diagnosis and therapy of undifferentiated embryonal liver sarcoma (UELS), the present study presents the case of a 9-year-old female with UELS and discusses UELS in childhood. The patient presented with abdominal pain and fever. The laboratory tests, radiographic examination and pathological features presented by the female were similar to those of typical cases of UELS reported in childhood. The patient initially received surgical treatment and the immunohistochemical findings suggested that the patient had UELS. The patient’s parents refused adjuvant chemotherapy and demonstrated a right prerenal mass 6 months post-surgery. Microscopic examination revealed that the tumor was evidence of undifferentiated embryonal sarcoma recurrence. However, the patient was comfortable and physical examination revealed no abnormal conditions. In addition, the laboratory results were normal. Abdominal computed tomography scan and ultrasound were performed every 3 months to monitor the tumor recurrence. At the time of writing, it has been 6 months after the second surgical procedure and there has been no appearence of abnormalities. Previous studies have shown that patients who receive combined therapy with complete tumor resection and adjuvant chemotherapy have a longer survival time than those who undergo surgical therapy alone. Complete tumor resection combined with adjuvant chemotherapy may reduce the risk of recurrence and enhance the survival time in patients with UELS. PMID:25120671

  15. Stathmin Activity Influences Sarcoma Cell Shape, Motility, and Metastatic Potential

    PubMed Central

    Belletti, Barbara; Nicoloso, Milena S.; Schiappacassi, Monica; Berton, Stefania; Lovat, Francesca; Wolf, Katarina; Canzonieri, Vincenzo; D'Andrea, Sara; Zucchetto, Antonella; Friedl, Peter; Colombatti, Alfonso

    2008-01-01

    The balanced activity of microtubule-stabilizing and -destabilizing proteins determines the extent of microtubule dynamics, which is implicated in many cellular processes, including adhesion, migration, and morphology. Among the destabilizing proteins, stathmin is overexpressed in different human malignancies and has been recently linked to the regulation of cell motility. The observation that stathmin was overexpressed in human recurrent and metastatic sarcomas prompted us to investigate stathmin contribution to tumor local invasiveness and distant dissemination. We found that stathmin stimulated cell motility in and through the extracellular matrix (ECM) in vitro and increased the metastatic potential of sarcoma cells in vivo. On contact with the ECM, stathmin was negatively regulated by phosphorylation. Accordingly, a less phosphorylable stathmin point mutant impaired ECM-induced microtubule stabilization and conferred a higher invasive potential, inducing a rounded cell shape coupled with amoeboid-like motility in three-dimensional matrices. Our results indicate that stathmin plays a significant role in tumor metastasis formation, a finding that could lead to exploitation of stathmin as a target of new antimetastatic drugs. PMID:18305103

  16. Undifferentiated embryonal liver sarcoma in childhood: A case report.

    PubMed

    He, Bin; Xu, Keyu; Huang, Kate; Huang, Yuehan; Li, Peng; Chen, Zhenkun; Shi, Hongqi; Zhang, Qiyu; Shan, Yunfeng

    2014-09-01

    In order to improve the diagnosis and therapy of undifferentiated embryonal liver sarcoma (UELS), the present study presents the case of a 9-year-old female with UELS and discusses UELS in childhood. The patient presented with abdominal pain and fever. The laboratory tests, radiographic examination and pathological features presented by the female were similar to those of typical cases of UELS reported in childhood. The patient initially received surgical treatment and the immunohistochemical findings suggested that the patient had UELS. The patient's parents refused adjuvant chemotherapy and demonstrated a right prerenal mass 6 months post-surgery. Microscopic examination revealed that the tumor was evidence of undifferentiated embryonal sarcoma recurrence. However, the patient was comfortable and physical examination revealed no abnormal conditions. In addition, the laboratory results were normal. Abdominal computed tomography scan and ultrasound were performed every 3 months to monitor the tumor recurrence. At the time of writing, it has been 6 months after the second surgical procedure and there has been no appearence of abnormalities. Previous studies have shown that patients who receive combined therapy with complete tumor resection and adjuvant chemotherapy have a longer survival time than those who undergo surgical therapy alone. Complete tumor resection combined with adjuvant chemotherapy may reduce the risk of recurrence and enhance the survival time in patients with UELS. PMID:25120671

  17. Aortic intimal sarcoma with embolic metastases.

    PubMed

    Wright, E P; Glick, A D; Virmani, R; Page, D L

    1985-12-01

    A 46-year-old woman died from massive bowel infarction. At autopsy, a primary sarcoma was found growing along the intimal surface of the aorta at the level of the celiac axis. Tumor emboli were found in distal aortic branches and most abdominal organs. Immunoperoxidase for Factor VIII and electron microscopy (EM) did not support an endothelial origin. EM showed myofibroblastic differentiation. Review of the literature yields an array of diagnostic histologic terms for these tumors, hampering case comparison. The literature does suggest, however, that the clinical presentation of these rare neoplasms correlates nicely with the location and gross morphology of the lesion. We therefore propose a clinicopathologic classification, categorizing the lesions as intimal (obstructive and nonobstructive) and mural. The former are typically pleomorphic sarcomas and are probably of myofibroblastic origin, whereas the latter are usually leiomyosarcomas or fibrosarcomas that probably originate in the media or adventitia. PMID:3000205

  18. Thyroid carcino-sarcoma in a dog.

    PubMed

    Giuliano, Antonio; Grant, Jessica; Benoit, Jerome

    2013-01-01

    An adult male greyhound was diagnosed with a thyroid carcino-sarcoma by means of histopathology and positive immuno-histochemistry staining for cytokeratin and vimentin. Surgery and radiotherapy of the area were successful in local tumour control. Adjuvant chemotherapy was recommended to treat and prevent further metastasis. The use of carboplatin, metronomic cyclophosphamide chemotherapy and toceranib failed to control the progression of distant metastasis. The survival time was seven months from the time of diagnosis. This is the eighth case of carcino-sarcoma of the thyroid documented in veterinary medicine and the first one treated with a multimodal approach based on surgery, radiotherapy and chemotherapy. As documented in human medicine, chemotherapy appeared to be ineffective to prevent or delay the progression of the metastatic disease in this case. PMID:23718201

  19. Bone Sarcomas: From Biology to Targeted Therapies

    PubMed Central

    Gaspar, Nathalie; Di Giannatale, Angela; Geoerger, Birgit; Redini, Françoise; Corradini, Nadège; Enz-Werle, Natacha; Tirode, Franck; Marec-Berard, Perrine; Gentet, Jean-Claude; Laurence, Valérie; Piperno-Neumann, Sophie; Oberlin, Odile; Brugieres, Laurence

    2012-01-01

    Primary malignant bone tumours, osteosarcomas, and Ewing sarcomas are rare diseases which occur mainly in adolescents and young adults. With the current therapies, some patients remain very difficult to treat, such as tumour with poor histological response to preoperative CT (or large initial tumour volume for Ewing sarcomas not operated), patients with multiple metastases at or those who relapsed. In order to develop new therapies against these rare tumours, we need to unveil the key driving factors and molecular abnormalities behind the malignant characteristics and to broaden our understanding of the phenomena sustaining the metastatic phenotype and treatment resistance in these tumours. In this paper, starting with the biology of these tumours, we will discuss potential therapeutic targets aimed at increasing local tumour control, limiting metastatic spread, and finally improving patient survival. PMID:23226965

  20. Soft tissue sarcoma of the extremity.

    PubMed Central

    Cooper, T. M.; Sheehan, M.; Collins, D.; O'Connor, T. P.

    1996-01-01

    A retrospective review of 33 cases of soft tissue sarcoma of the extremity presenting over a 10 year period was undertaken. The history, patterns of referral, diagnostic investigations, procedures undertaken and outcomes were studied. We found there was a frequent delay in diagnosis and sometimes misinterpretation of biopsy specimens. Patients were seen by a variety of specialists from disciplines such as general surgery, plastic surgery, orthopaedic surgery and rheumatology. Considerable progress has been made in the treatment of soft tissue sarcomas, often allowing local control of the tumour without amputation. We believe there should be early referral of patients having these tumours to a centre where a combined multidisciplinary approach can be undertaken. PMID:8881731

  1. Primary Thyroid Sarcoma: A Systematic Review.

    PubMed

    Surov, Alexey; Gottschling, Sebastian; Wienke, Andreas; Meyer, Hans Jonas; Spielmann, Rolf Peter; Dralle, Henning

    2015-10-01

    Different types of malignant tumors can occur within the thyroid. Primary cancer is the most common type of thyroid malignancy. Non-epithelial malignancies can also arise within the thyroid. The aim of the present study was to analyze clinical and radiological characteristics of reported primary thyroid sarcomas (PTS), based on a large sample of cases. The PubMed database was screened for articles from between 1990 and 2014. Overall, 86 articles with 142 patients were identified. Ultrasound evaluation was reported for 36 patients. Data regarding computed tomography of the neck were available for 29 cases. Magnetic resonance imaging was performed for eight patients. The following data were retrieved for the identified sarcomas: localization, size, homogeneity, internal texture, and margin characteristics. In most cases, PTS occurred in patients over 40 years of age, with a peak incidence for the group aged 60-79 years. Angiosarcoma was diagnosed in 29 cases (20.4%), followed by malignant hemangioendothelioma (n=23, 16.3%), malignant fibrous histiocytoma (n=20, 14.1%), leiomyosarcoma (n=16, 11.3%), and fibrosarcoma (n=13, 9.2%). In most patients (n=113, 79.6%), PTS manifested clinically as a painless goiter. On ultrasound, PTS were predominantly mixed hypo-to-hyperechoic in comparison to the normal thyroid tissue. On non-contrast computed tomography, most sarcomas were inhomogeneous hypo-to-hyperdense. On post-contrast magnetic resonance images, most sarcomas showed marked non-homogenous enhancement. In 26.8%, infiltration of the adjacent organs was seen. The trachea or esophagus was affected more frequently in patients with malignant histiocytoma and liposarcoma. Different strategies were used in the treatment of PTS. Our analysis provides clinical and radiological characteristics of PTS. The described features should be taken into consideration in the differential diagnosis of thyroid tumors. PMID:26408676

  2. Management of soft tissue and bone sarcomas

    SciTech Connect

    Van Oosterom, A.T.; Van Unnik, J.A.M.

    1986-01-01

    This book contains 32 papers. Some of the titles are: Adjuvant Treatment for Osteosarcoma of the Limbs; Trial 20781 of the SIOP and the EORTC Radiotherapy/Chemotherapy; Application of Magnetic Resonance Imaging (MRI) in Diagnosis and Follow-up During Treatment of Bone Tumors; Radiological Assessment of Local Involvement in Bone Sarcomas; and Prevention of Lung Metastases by Irradiation Alone or Combined with Chemotherapy in an Animal Model.

  3. In vivo radiolocalization of antiosteogenic sarcoma monoclonal antibodies in osteogenic sarcoma xenografts

    SciTech Connect

    Nakamura, T.; Sakahara, H.; Hosoi, S.; Yamamuro, T.; Higashi, S.; Mikawa, H.; Endo, K.; Toyama, S.

    1984-05-01

    Monoclonal antibodies Ost6 and Ost7 (mouse Immunoglobulin G1) to human osteogenic sarcoma were isolated from ascitic fluid and labeled with radioiodine. After injection into athymic nu/nu mice with s.c. xenografts of human osteogenic sarcoma, the uptake of radioactivity in tumors, visceral organs, and blood was determined. Five days after injection, Ost6 and Ost7 showed preferential accumulation in tumors (tumor:blood ratio, 4.3). Furthermore, with testicular and bladder tumors, both unreactive with Ost7, there was no localization of radiolabeled Ost7 in xenograft growths. When Ost7 was labeled with /sup 131/I, its accumulation into human osteogenic sarcoma could be clearly visualized by whole-body gamma-scintigraphy without computer-assisted data processing.

  4. [Primary sarcomas and sarcoma metastases in the liver: morphological and molecular aspects].

    PubMed

    Mechtersheimer, G; Penzel, R; Hofmann, W J; Schirmacher, P

    2006-07-01

    The considerable progress made in radiology, in surgical management with curative intent, and in the identification of molecularly targeted small molecules, such as the tyrosine kinase inhibitor imatinib mesylate, in the treatment of gastrointestinal stromal tumors has greatly influenced the treatment of sarcoma manifestations within the liver. This requires not only the unequivocal pathomorphological differentiation of sarcomas from other tumor entities, e. g. spindle cell dedifferentiated/pleomorphic carcinomas, aggressive non-Hodgkin lymphomas or amelanotic malignant melanomas, but also an accurate subtyping of this complex group of tumors. Additionally to macroscopic and histological findings, the recognition of characteristic immunophenotypic constellations and, at least in some types of sarcoma, the identification of molecular signatures, have greatly expanded the diagnostic tools in pathology. PMID:16773310

  5. Phosphoproteomics in translational research: a sarcoma perspective.

    PubMed

    Noujaim, J; Payne, L S; Judson, I; Jones, R L; Huang, P H

    2016-05-01

    Phosphoproteomics has been extensively used as a preclinical research tool to characterize the phosphorylated components of the cancer proteome. Advances in the field have yielded insights into new drug targets, mechanisms of disease progression and drug resistance, and biomarker discovery. However, application of this technology to clinical research has been challenging because of practical issues relating to specimen integrity and tumour heterogeneity. Beyond these limitations, phosphoproteomics has the potential to play a pivotal role in translational studies and contribute to advances in different tumour groups, including rare disease sites like sarcoma. In this review, we propose that deploying phosphoproteomic technologies in translational research may facilitate the identification of better defined predictive biomarkers for patient stratification, inform drug selection in umbrella trials and identify new combinations to overcome drug resistance. We provide an overview of current phosphoproteomic technologies, such as affinity-based assays and mass spectrometry-based approaches, and discuss their advantages and limitations. We use sarcoma as an example to illustrate the current challenges in evaluating targeted kinase therapies in clinical trials. We then highlight useful lessons from preclinical studies in sarcoma biology to demonstrate how phosphoproteomics may address some of these challenges. Finally, we conclude by offering a perspective and list the key measures required to translate and benchmark a largely preclinical technology into a useful tool for translational research. PMID:26802162

  6. Cutaneous myeloid sarcoma of the penile foreskin.

    PubMed

    Afrose, Ruquiya; Nebhnani, Deepa; Wadhwa, Neelam

    2015-01-01

    Myeloid sarcoma, considered to herald the onset of a blast crisis in the setting of chronic myeloproliferative neoplasm/dysplasia, typically presents during the course of the disorder. Cutaneous involvement is uncommon and lesions on genital skin are seldom seen. We present a case of a well-differentiated myeloid sarcoma in the penile foreskin in an apparently healthy 29-year-old male presenting with phimosis. The unusual composition of the inflammatory cell infiltrate, and characteristic sparing of dermal blood vessels, nerves and smooth muscle fibres led to the correct diagnosis. Absence of commonly observed changes in the circumcision skin like those of balanitis xerotica was also helpful. Detailed hematological work up revealed a previously undiagnosed chronic myeloid leukemia in chronic phase. The patient also had simultaneous priapism, another rare presentation of chronic myeloid leukemia. One year hence, the patient is in hematological remission with no evidence of extramedullary disease. Although priapism has been described as a rare presenting symptom in chronic myeloid leukemia, the present case is unique as this is the first time a cutaneous myeloid sarcoma has been documented in the penile foreskin. PMID:24913300

  7. Sarcoma Immunotherapy: Past Approaches and Future Directions

    PubMed Central

    D'Angelo, S. P.; Tap, W. D.; Schwartz, G. K.; Carvajal, R. D.

    2014-01-01

    Sarcomas are heterogeneous malignant tumors of mesenchymal origin characterized by more than 100 distinct subtypes. Unfortunately, 25–50% of patients treated with initial curative intent will develop metastatic disease. In the metastatic setting, chemotherapy rarely leads to complete and durable responses; therefore, there is a dire need for more effective therapies. Exploring immunotherapeutic strategies may be warranted. In the past, agents that stimulate the immune system such as interferon and interleukin-2 have been explored and there has been evidence of some clinical activity in selected patients. In addition, many cancer vaccines have been explored with suggestion of benefit in some patients. Building on the advancements made in other solid tumors as well as a better understanding of cancer immunology provides hope for the development of new and exciting therapies in the treatment of sarcoma. There remains promise with immunologic checkpoint blockade antibodies. Further, building on the success of autologous cell transfer in hematologic malignancies, designing chimeric antigen receptors that target antigens that are over-expressed in sarcoma provides a great deal of optimism. Exploring these avenues has the potential to make immunotherapy a real therapeutic option in this orphan disease. PMID:24778572

  8. Caveolin-1 in sarcomas: friend or foe?

    PubMed Central

    Sáinz-Jaspeado, Miguel; Martin-Liberal, Juan; Lagares-Tena, Laura; Mateo-Lozano, Silvia; del Muro, Xavier Garcia; Tirado, Oscar M

    2011-01-01

    Sarcomas represent a heterogeneous group of tumors with a complex and difficult reproducible classification. Their pathogenesis is poorly understood and there are few effective treatment options for advanced disease. Caveolin-1 is a multifunctional scaffolding protein with multiple binding partners that regulates multiple cancer-associated processes including cellular transformation, tumor growth, cell death and survival, multidrug resistance, angiogenesis, cell migration and metastasis. However, ambiguous roles have been ascribed to caveolin-1 in signal transduction and cancer, including sarcomas. In particular, evidence indicating that caveolin-1 function is cell context dependent has been repeatedly reported. Caveolin-1 appears to act as a tumor suppressor protein at early stages of cancer progression. In contrast, a growing body of evidence indicates that caveolin-1 is up-regulated in several multidrug-resistant and metastatic cancer cell lines and human tumor specimens. This review is focused on the role of caveolin-1 in several soft tissue and bone sarcomas and discusses the use of this protein as a potential diagnostic and prognostic marker and as a therapeutic target. PMID:21471610

  9. Potential molecular targets for Ewing's sarcoma therapy.

    PubMed

    Jully, Babu; Rajkumar, Thangarajan

    2012-10-01

    Ewing's sarcoma (ES) is a highly malignant tumor of children and young adults. Modern therapy for Ewing's sarcoma combines high-dose chemotherapy for systemic control of disease, with advanced surgical and/or radiation therapeutic approaches for local control. Despite optimal management, the cure rate for localized disease is only approximately 70%, whereas the cure rate for metastatic disease at presentation is less than 30%. Patients who experience long-term disease-free survival are at risk for significant side-effects of therapy, including infertility, limb dysfunction and an increased risk for second malignancies. The identification of new targets for innovative therapeutic approaches is, therefore, strongly needed for its treatment. Many new pharmaceutical agents have been tested in early phases of clinical trials in ES patients who have recurrent disease. While some agents led to partial response or stable disease, the percentages of drugs eliciting responses or causing an overall effect have been minimal. Furthermore, of the new pharmaceuticals being introduced to clinical practice, the most effective agents also have dose-limiting toxicities. Novel approaches are needed to minimize non-specific toxicity, both for patients with recurrence and at diagnosis. This report presents an overview of the potential molecular targets in ES and highlights the possibility that they may serve as therapeutic targets for the disease. Although additional investigations are required before most of these approaches can be assessed in the clinic, they provide a great deal of hope for patients with Ewing's sarcoma. PMID:23580819

  10. Metachronous Bilateral Extremity Soft Tissue Sarcomas

    PubMed Central

    Nowrasteh, Ghodratollah; Aziz, Tanim; Assas, Mohammed Al; Nuaimi, Lateefa Al; Marzouqi, Saeeda; Quadri, Asif A.M.; Alrawi, Sadir

    2016-01-01

    Case series Patient: Male, 44 • Male, 58 Final Diagnosis: Soft tissue sarcomas Symptoms: Discomfort • swelling Medication: — Clinical Procedure: Image guided biopsy • metastatic work up • neoadjuvant radiotherapy • radical resection Specialty: Surgery Objective: Rare disease Background: Soft tissue sarcomas (STS) account for approximately 1% of adult malignancies, with 50 to 60% occurring in the extremities. Liposarcoma is the most common type of STS and represent about 20% of total adult sarcomas. There are rare syndromes associated with increased risk of developing STS. Further, chemical compounds such as chlorinated phenols and a few chemotherapeutic drugs have been linked to STS, along with ionizing radiation. Nevertheless, the etiology is uncertain for most of these lesions. Case Report: This report details 2 cases of metachronous bilateral STS of the lower extremities. The first of these presented as a local recurrence of a previously resected right thigh liposarcoma and a new liposarcoma in the left thigh. As mentioned above, among the different subtypes of STS, liposarcoma has the highest tendency for multifocality. The second patient had multifocal metachronous leiomyosarcoma with lung metastases occurring simultaneously with the second presentation. Leiomyosarcoma is another subtype reported to present with multi-focal disease. Conclusions: Despite the rarity of bilateral lesions, their occurrence should not be overlooked in the initial diagnosis and follow-up of the initially detected tumor. Early detection can affect patient survival because their presence predicts unfavorable outcomes. PMID:26744032

  11. MicroRNA-16 suppresses metastasis in an orthotopic, but not autochthonous, mouse model of soft tissue sarcoma

    PubMed Central

    Sachdeva, Mohit; Whitley, Melody J.; Mito, Jeffrey K.; Ma, Yan; Lev, Dina C.; Cardona, Diana M.; Kirsch, David G.

    2015-01-01

    ABSTRACT MicroRNAs (miRNAs) can regulate tumor cell invasion and metastasis in a tumor-specific manner. We recently demonstrated that global downregulation of miRNAs after deleting dicer can promote development of distant metastases in a mouse model of primary soft tissue sarcoma (STS). In this study, we identified miRNAs that are differentially downregulated in metastatic STS in both human and mouse, and investigated the role of these miRNAs in metastasis. miRNA- TaqMan PCR arrays showed a global downregulation of miRNAs in metastatic human sarcomas. Similar analysis in mouse metastatic sarcomas revealed overlap for several downregulated miRNAs including miR-16, miR-103, miR-146a, miR-223, miR-342 and miR-511. Restoration of these downregulated miRNAs in mouse primary sarcoma cell lines showed that miR-16, but not other downregulated miRNAs, was able to significantly suppress both migration and invasion in vitro, without altering cell proliferation. In addition, orthotopic transplantation of a sarcoma cell line stably expressing miR-16 into the muscle of immunocompromised mice revealed that restoration of miR-16 can significantly decrease lung metastasis in vivo. However, no change in the rate of lung metastasis was observed when miR-16 was deleted in mouse primary sarcomas at sarcoma initiation. Taken together, these results indicate that miR-16 can have metastasis-suppressing properties both in vitro and in vivo. However, the loss-of-function experiments in autochthonous tumors indicate that loss of miR-16 is not sufficient to promote metastasis in vivo. PMID:26044957

  12. Intraoperative detection and removal of microscopic residual sarcoma using wide-field imaging

    PubMed Central

    Mito, Jeffrey K; Ferrer, Jorge M; Brigman, Brian E; Lee, Chang-Lung; Dodd, Rebecca D; Eward, William C; Marshall, Lisa F; Cuneo, Kyle C; Carter, Jessica E; Ramasunder, Shalini; Kim, Yongbaek; Lee, W David; Griffith, Linda G; Bawendi, Moungi G; Kirsch, David G

    2012-01-01

    Abstract BACKGROUND: The goal of limb-sparing surgery for a soft tissue sarcoma of the extremity is to remove all malignant cells while preserving limb function. After initial surgery, microscopic residual disease in the tumor bed will cause a local recurrence in approximately 33% of patients with sarcoma. To help identify these patients, the authors developed an in vivo imaging system to investigate the suitability of molecular imaging for intraoperative visualization. METHODS: A primary mouse model of soft tissue sarcoma and a wide field-of-view imaging device were used to investigate a series of exogenously administered, near-infrared (NIR) fluorescent probes activated by cathepsin proteases for real-time intraoperative imaging. RESULTS: The authors demonstrated that exogenously administered cathepsin-activated probes can be used for image-guided surgery to identify microscopic residual NIR fluorescence in the tumor beds of mice. The presence of residual NIR fluorescence was correlated with microscopic residual sarcoma and local recurrence. The removal of residual NIR fluorescence improved local control. CONCLUSIONS: The authors concluded that their technique has the potential to be used for intraoperative image-guided surgery to identify microscopic residual disease in patients with cancer. Cancer 2012. © 2012 American Cancer Society. PMID:22437667

  13. Targeting the Insulin-Like Growth Factor 1 Receptor in Ewing's Sarcoma: Reality and Expectations

    PubMed Central

    Olmos, David; Martins, Ana Sofia; Jones, Robin L.; Alam, Salma; Scurr, Michelle; Judson, Ian R.

    2011-01-01

    Ewing's sarcoma family of tumours comprises a group of very aggressive diseases that are potentially curable with multimodality treatment. Despite the undoubted success of current treatment, approximately 30% of patients will relapse and ultimately die of disease. The insulin-like growth factor 1 receptor (IGF-1R) has been implicated in the genesis, growth, proliferation, and the development of metastatic disease in Ewing's sarcoma. In addition, IGF1-R has been validated, both in vitro and in vivo, as a potential therapeutic target in Ewing's sarcoma. Phase I studies of IGF-1R monoclonal antibodies reported several radiological and clinical responses in Ewing's sarcoma patients, and initial reports of several Phase II studies suggest that about a fourth of the patients would benefit from IGF-1R monoclonal antibodies as single therapy, with approximately 10% of patients achieving objective responses. Furthermore, these therapies are well tolerated, and thus far severe toxicity has been rare. Other studies assessing IGF-1R monoclonal antibodies in combination with traditional cytotoxics or other targeted therapies are expected. Despite, the initial promising results, not all patients benefit from IGF-1R inhibition, and consequently, there is an urgent need for the identification of predictive markers of response. PMID:21647361

  14. Myeloid sarcoma of the Gingiva with myelodysplastic syndrome: A Case Report.

    PubMed

    Jin, Seong-Ho; Park, Gyeongsin; Ko, Youngkyung; Park, Jun-Beom

    2016-06-01

    The purpose of this report is to present a case of myeloid sarcoma of the gingiva with myelodysplastic syndrome.A 52-year-old male diagnosed with myelodysplastic syndrome with skin lesions presented with gingival swelling and gingival redness involving the maxillary left second premolar and the maxillary left first molar. The patient was referred from the Department of Hematology for a biopsy of the lesion. Full-thickness flaps were elevated and inflamed, and neoplastic soft tissue was removed from a lesion and the samples sent for histopathologic analysis.Histopathologic results showed leukemic cell infiltration beneath the oral epithelium, and the specimen was positive for the leukocyte marker. The diagnosis was myeloid sarcoma. Uneventful healing was observed at 2-week follow-up, but relapse of the lesions with the hyperplastic and neoplastic tissue was noted at 4-week follow-up. Further follow-up or treatment could not be performed because the patient did not visit at the next follow-up.In conclusion, myeloid sarcoma should be a diagnosis option for gingival growth because it can involve intraoral lesion. In this report, a biopsy was performed due to referral considering the patient's medical history. Although myeloid sarcoma in the oral cavity is extremely rare, a small biopsy and consultation with a hematologist may be beneficial for patients and may provide a differential diagnosis. PMID:27310987

  15. Gene Expression Music Algorithm-Based Characterization of the Ewing Sarcoma Stem Cell Signature

    PubMed Central

    2016-01-01

    Gene Expression Music Algorithm (GEMusicA) is a method for the transformation of DNA microarray data into melodies that can be used for the characterization of differentially expressed genes. Using this method we compared gene expression profiles from endothelial cells (EC), hematopoietic stem cells, neuronal stem cells, embryonic stem cells (ESC), and mesenchymal stem cells (MSC) and defined a set of genes that can discriminate between the different stem cell types. We analyzed the behavior of public microarray data sets from Ewing sarcoma (“Ewing family tumors,” EFT) cell lines and biopsies in GEMusicA after prefiltering DNA microarray data for the probe sets from the stem cell signature. Our results demonstrate that individual Ewing sarcoma cell lines have a high similarity to ESC or EC. Ewing sarcoma cell lines with inhibited Ewing sarcoma breakpoint region 1-Friend leukemia virus integration 1 (EWSR1-FLI1) oncogene retained the similarity to ESC and EC. However, correlation coefficients between GEMusicA-processed expression data between EFT and ESC decreased whereas correlation coefficients between EFT and EC as well as between EFT and MSC increased after knockdown of EWSR1-FLI1. Our data support the concept of EFT being derived from cells with features of embryonic and endothelial cells. PMID:27446218

  16. Imaging Primary Mouse Sarcomas After Radiation Therapy Using Cathepsin-Activatable Fluorescent Imaging Agents

    SciTech Connect

    Cuneo, Kyle C.; Mito, Jeffrey K.; Javid, Melodi P.; Ferrer, Jorge M.; Kim, Yongbaek; Lee, W. David; Bawendi, Moungi G.; Brigman, Brian E.; Kirsch, David G.

    2013-05-01

    Purpose: Cathepsin-activated fluorescent probes can detect tumors in mice and in canine patients. We previously showed that these probes can detect microscopic residual sarcoma in the tumor bed of mice during gross total resection. Many patients with soft tissue sarcoma (STS) and other tumors undergo radiation therapy (RT) before surgery. This study assesses the effect of RT on the ability of cathepsin-activated probes to differentiate between normal and cancerous tissue. Methods and Materials: A genetically engineered mouse model of STS was used to generate primary hind limb sarcomas that were treated with hypofractionated RT. Mice were injected intravenously with cathepsin-activated fluorescent probes, and various tissues, including the tumor, were imaged using a hand-held imaging device. Resected tumor and normal muscle samples were harvested to assess cathepsin expression by Western blot. Uptake of activated probe was analyzed by flow cytometry and confocal microscopy. Parallel in vitro studies using mouse sarcoma cells were performed. Results: RT of primary STS in mice and mouse sarcoma cell lines caused no change in probe activation or cathepsin protease expression. Increasing radiation dose resulted in an upward trend in probe activation. Flow cytometry and immunofluorescence showed that a substantial proportion of probe-labeled cells were CD11b-positive tumor-associated immune cells. Conclusions: In this primary murine model of STS, RT did not affect the ability of cathepsin-activated probes to differentiate between tumor and normal muscle. Cathepsin-activated probes labeled tumor cells and tumor-associated macrophages. Our results suggest that it would be feasible to include patients who have received preoperative RT in clinical studies evaluating cathepsin-activated imaging probes.

  17. Outcome in patients with idiopathic retroperitoneal fibrosis treated with corticosteroid or tamoxifen monotherapy

    PubMed Central

    van der Bilt, Floor E.; Hendriksz, Tadek R.; van der Meijden, Wilbert A.G.; Brilman, Lisette G.; van Bommel, Eric F.H.

    2016-01-01

    Background Although corticosteroids (CS) are used primarily in idiopathic retroperitoneal fibrosis (iRPF), tamoxifen (TMX) may be a suitable alternative. We compared outcome with CS or TMX monotherapy for first presentation in a large group of patients with iRPF disease. Methods Of all patients with iRPF disease who were seen at our tertiary care referral centre from February 1999 to December 2011, 118 patients were eligible for this retrospective study. Treatment success was defined as the composite of (i) amelioration of symptoms, (ii) computed tomography (CT)-documented mass regression and, if applicable, (iii) definitive removal of ureteral stent or nephrostomy tube. Recurrence was defined as recurrence of signs and symptoms and/or CT-documented mass increase after initial treatment success with primary treatment. Results Presenting signs and symptoms did not differ between patients treated with CS (n = 50) or TMX (n = 68). Time to amelioration of symptoms after treatment initiation was shorter in CS-treated patients [CS, 2.0 (0.8–3.8) weeks versus TMX, 4.0 (2.0–6.0) weeks; P < 0.01]. Short-term percentual decrease in acute-phase reactant levels (P < 0.001 for both erythrocyte sedimentation rate and C-reactive protein) and serum creatinine level (P < 0.01) following treatment initiation was greater in CS-treated patients compared with that in TMX-treated patients. Mass regression at first follow-up CT scan was observed more frequently in CS-treated patients (CS, 84.0% versus TMX, 68.3%; P = 0.05) with no difference in time interval from treatment initiation to first follow-up CT between groups [CS, 5 (2–7) months versus TMX, 4 (4–5) months; P = 0.34]. Definite treatment success was non-significantly higher in CS-treated patients (CS, 72.7% versus TMX, 58.3%; P = 0.15). In patients with initial treatment success with primary treatment, recurrence rate was lower in TMX-treated patients (CS, 62.5% versus TMX, 21.4%; P < 0.01). Conclusions CS are superior

  18. Soft-tissue sarcomas of the extremities in patients of pediatric age

    PubMed Central

    Casanova, Michela; Meazza, Cristina; Gronchi, Alessandro; Fiore, Marco; Zaffignani, Elena; Podda, Marta; Collini, Paola; Gandola, Lorenza

    2007-01-01

    Purpose The extremity site is a peculiar location for soft-tissue sarcomas (STS) of children and adolescents. Here we report the experience of the Pediatric Oncology Unit of the Istituto Nazionale Tumori of Milan, Italy, concerning 204 patients with STS of the limbs treated between 1977 and 2006. Methods The study series included 52 patients with rhabdomyosarcoma (RMS)(65% of which were of the alveolar subtype), nine with extraosseous Ewing sarcoma and 143 with non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS), 38% of which were synovial sarcoma. Patients were treated with a multimodality approach including surgery, chemotherapy, and radiotherapy. Results For the RMS patients, the 5-year event-free survival (EFS) rate was 37.1%, with distant metastases being the main cause of treatment failure. The outcome was particularly poor for patients with large invasive tumors, hand/foot involvement and/or nodal or distant metastases, and for patients who were not given radiotherapy. For the extraosseous Ewing sarcoma cases, 5-year EFS was 74%. For the NRSTS cases, the 5-year EFS was 72.6%: tumor size and local invasiveness, tumor grade, malignant peripheral nerve sheath tumor (MPNST) histology, and distant metastases were the main prognostic factors. Discussion While the limbs are the most common sites of NRSTS and are often characterized by a more favorable prognosis than for axial tumors, the clinical features of extremity RMS often differ from those of RMS of other sites, with a higher incidence of unfavorable prognostic factors (e.g., alveolar subtype) and consequently unsatisfactory treatment results. The treatment of these patients is complex and necessarily multidisciplinary, and it demands not only adequate experience of treating children and adolescents in clinical trials, but also particular skills in the field of orthopedic surgery. PMID:19308495

  19. Percutaneous Hepatic Perfusion with Melphalan for Unresectable Metastatic Melanoma or Sarcoma to the Liver: A Single Institution Experience

    PubMed Central

    Forster, Meghan R.; Rashid, Omar M.; Perez, Matthew; Choi, Junsung; Chaudhry, Tariq; Zager, Jonathan S.

    2015-01-01

    Background Patients with unresectable melanoma or sarcoma hepatic metastasis have a poor prognosis with few therapeutic options. Percutaneous hepatic perfusion (PHP), isolating and perfusing the liver with chemotherapy, provides a promising minimally invasive management option. We reviewed our institutional experience with PHP. Methods We retrospectively reviewed patients with unresectable melanoma or sarcoma hepatic metastasis treated with PHP from 2008 to 2013 and evaluated therapeutic response, morbidity, hepatic progression free survival (hPFS), and overall survival (OS). Results Ten patients were treated with 27 PHPs (median 3). Diagnoses were ocular melanoma (n=5), cutaneous melanoma (n=3), unknown primary melanoma (n=1), and sarcoma (n=1). Median hPFS was 240 days, 9 of 10 patients (90%) demonstrated stable disease or partial response to treatment. At a median follow up of 11.5 months, 4 of 10 (40%) remain alive. There were no perioperative mortalities. Myelosuppresion was the most common morbidity, managed on an outpatient basis with growth factors. The median hospital stay was 3 days. Conclusions Patients with metastatic melanoma and sarcoma to the liver have limited treatment options. Our experience with PHP demonstrates promising results with minimal morbidity and should be considered (pending FDA approval) as a management option for unresectable melanoma or sarcoma hepatic metastasis. PMID:24249545

  20. Integrative DNA methylation and gene expression analysis in high-grade soft tissue sarcomas

    PubMed Central

    2013-01-01

    Background High-grade soft tissue sarcomas are a heterogeneous, complex group of aggressive malignant tumors showing mesenchymal differentiation. Recently, soft tissue sarcomas have increasingly been classified on the basis of underlying genetic alterations; however, the role of aberrant DNA methylation in these tumors is not well understood and, consequently, the usefulness of methylation-based classification is unclear. Results We used the Infinium HumanMethylation27 platform to profile DNA methylation in 80 primary, untreated high-grade soft tissue sarcomas, representing eight relevant subtypes, two non-neoplastic fat samples and 14 representative sarcoma cell lines. The primary samples were partitioned into seven stable clusters. A classification algorithm identified 216 CpG sites, mapping to 246 genes, showing different degrees of DNA methylation between these seven groups. The differences between the clusters were best represented by a set of eight CpG sites located in the genes SPEG, NNAT, FBLN2, PYROXD2, ZNF217, COL14A1, DMRT2 and CDKN2A. By integrating DNA methylation and mRNA expression data, we identified 27 genes showing negative and three genes showing positive correlation. Compared with non-neoplastic fat, NNAT showed DNA hypomethylation and inverse gene expression in myxoid liposarcomas, and DNA hypermethylation and inverse gene expression in dedifferentiated and pleomorphic liposarcomas. Recovery of NNAT in a hypermethylated myxoid liposarcoma cell line decreased cell migration and viability. Conclusions Our analysis represents the first comprehensive integration of DNA methylation and transcriptional data in primary high-grade soft tissue sarcomas. We propose novel biomarkers and genes relevant for pathogenesis, including NNAT as a potential tumor suppressor in myxoid liposarcomas. PMID:24345474