Science.gov

Sample records for retroperitoneal sarcoma results

  1. Retroperitoneal Sarcomas.

    PubMed

    Porpiglia, Andrea S; Reddy, Sanjay S; Farma, Jeffrey M

    2016-10-01

    Retroperitoneal sarcomas are rare tumors, representing only 15% of all sarcomas. The mainstay of therapy is surgical resection with negative margins. However, this is challenging because of the late presentation of many of these tumors and involvement with adjacent structures. Decisions on radiation therapy and chemotherapy should be made in a multidisciplinary setting at a tertiary referral center.

  2. 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

  3. 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

  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. 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.

  6. 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.

  7. 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

  8. Intraoperative electron-beam therapy for primary and recurrent retroperitoneal soft-tissue sarcoma

    SciTech Connect

    Krempien, Robert . E-mail: robert_krempien@med.uni-heidelberg.de; Roeder, Falk; Oertel, Susanne; Weitz, Juergen; Hensley, Frank W.; Timke, Carmen; Funk, Angela; Lindel, Katja; Harms, Wolfgang; Buchler, Markus W.; Debus, Juergen; Treiber, Martina

    2006-07-01

    Purpose: This study assesses the long-term outcome of patients with retroperitoneal soft-tissue sarcomas treated by maximal resection in combination with intraoperative electron-beam therapy (IOERT) and postoperative external-beam radiotherapy. Methods and Materials: From 1991 to 2004, 67 patients were treated with curative intent for primary (n = 26) or recurrent (n = 41) retroperitoneal soft-tissue sarcoma. All patients underwent maximal resection in combination with IOERT (mean dose, 15 Gy), 45 patients underwent additional postoperative EBRT, and 20 patients were previously irradiated. Results: The 5-year actuarial overall survival (OS), disease-free survival, local control (LC), and freedom from metastatic disease of all patients was 64%, 28%, 40%, and 50%, respectively. The 5-year LC inside the IOERT field was 72%. For patients who completed IOERT and EBRT after R0-resection 5-year and 10-year OS was 80%, and 5-year and 10-year LC was 100%. Only 1 of the 21 patients after R0-resection and only 8 of 34 patients after R1-resection compared with 9 of 12 patients after R2-resection experienced inside IOERT-field relapse. Grade II or higher late complications were seen in 21% of the patients, but only 2 patients required surgical intervention because of late complications. Conclusion: In selected patients, IOERT results in excellent local control and survival, with acceptable morbidity.

  9. 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

  10. Primary retroperitoneal mucinous cystadenoma with sarcoma-like mural nodule : a case report and review of the literature.

    PubMed

    Bakker, R F R; Stoot, J H M B; Blok, P; Merkus, J W S

    2007-10-01

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

  11. 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

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

    SciTech Connect

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

    2007-01-01

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

  13. 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

  14. 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.

  15. Effectiveness of Regional Hyperthermia With Chemotherapy for High-Risk Retroperitoneal and Abdominal Soft-Tissue Sarcoma After Complete Surgical Resection

    PubMed Central

    Angele, Martin K.; Albertsmeier, Markus; Prix, Niclas J.; Hohenberger, Peter; Abdel-Rahman, Sultan; Dieterle, Nelli; Schmidt, Michael; Mansmann, Ulrich; Bruns, Christiane J.; Issels, Rolf D.; Jauch, Karl-Walter; Lindner, Lars H.

    2015-01-01

    Objective To determine whether regional hyperthermia (RHT) in addition to chemotherapy improves local tumor control after macroscopically complete resection of abdominal or retroperitoneal high-risk sarcomas. Background Within the prospectively randomized EORTC 62961 phase- III trial, RHT and systemic chemotherapy significantly improved local progression-free survival (LPFS) and disease-free survival (DFS) in patients with abdominal and extremity sarcomas. That trial included macroscopically complete and R2 resections. Methods A subgroup analysis of the EORTC trial was performed and long-term survival determined. From 341 patients, 149 (median age 52 years, 18–69) were identified with macroscopic complete resection (R0, R1) of abdominal and retroperitoneal soft-tissue sarcomas (median diameter 10 cm, G2 48.3%, G3 51.7%). Seventy-six patients were treated with EIA (etoposide, ifosfamide, doxorubicin) + RHT (≥5 cycles: 69.7%) versus 73 patients receiving EIA alone (≥5 cycles: 52.1%, P = 0.027). LPFS and DFS as well as overall survival were determined. Results RHT and systemic chemotherapy significantly improved LPFS (56% vs 45% after 5 years, P = 0.044) and DFS (34% vs 27% after 5 years, P = 0.040). Overall survival was not significantly improved in the RHT group (57% vs 55% after 5 years, P = 0.82). Perioperative morbidity and mortality were not significantly different between groups. Conclusions In patients with macroscopically complete tumor resection, RHT in addition to chemotherapy resulted in significantly improved local tumor control and DFS without increasing surgical complications. Within a multimodal therapeutic concept for abdominal and retroperitoneal high-risk sarcomas, RHT is a treatment option beside radical surgery and should be further evaluated in future trials. PMID:25379845

  16. 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

  17. 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

  18. 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

  19. [The diagnosis and immediate treatment results in retroperitoneal tumors].

    PubMed

    Ivanov, S; Karanov, S; Kurtev, P

    1989-01-01

    Immediate results of treatment of 20 patients with primary retroperitoneal tumors treated over a period of 5 years (1980-1985) at the Research Institute of Oncology in Sofia are reported. Attempt is made for anamnestic analysis, aimed at elucidating the semiotics of the disease. Analysis of the diagnostic methods serves to give recommendations for "first-rate" diagnostic procedure--computer tomography when a retroperitoneal tumor is suspected. Complex therapeutic approach consisting of radical operation and adjuvant radiation treatment is advised.

  20. Primary retroperitoneal mucinous cystadenoma with a sarcoma-like mural nodule: an immunohistochemical study with histogenetic considerations and literature review.

    PubMed

    Demirel, Dilaver; Gun, Ismet; Kucukodaci, Zafer; Balta, Ahmet Ziya; Ramzy, Ibrahim

    2013-01-01

    Primary retroperitoneal mucinous cystadenomas (PRMCs) are extremely rare tumors and their association with sarcoma-like mural nodules (SLMNs) has not been described thoroughly. The aim of this study is to characterize the gross and microscopic features and the immunohistochemical profile of the first case of PRMC with SLMN and to discuss the differential diagnosis of SLMNs. The literature related to primary retroperitoneal mucinous tumors is reviewed in an attempt to clarify the histogenesis of the epithelial and sarcomatoid components of the associated mural nodules. A 34-yr-old woman presented with a 14-cm retroperitoneal cystic lesion with a 6-cm mural nodule. An immunohistochemical study with a panel of 19 antibodies and a histochemical study for mucin stains were performed. The epithelial component of the PRMC showed positive staining for cytokeratin (CK) 7, CK AE1/3, epithelial membrane antigen, carcinoembryonic antigen, and calretinin. The neoplasm was not immunoreactive for CK 20, CK 5/6, and the other antibodies used in this study. In addition, it stained positively for mucin by mucicarmine, periodic acid-Schiff, and Alcian blue. The stromal cells of the cyst showed estrogen receptor positivity. SLMN cells were negative for all CKs and other epithelial markers used in the study, but they showed diffuse positive staining for vimentin and CD68, and positive staining for Ki-67 was demonstrated in 25% of these cells. The immunohistochemical and histochemical profiles of PRMC were similar to those of ovarian mucinous neoplasms and the mesothelium. The formation of SLMNs seems to be related to subepithelial hemorrhage and some reactive epithelial changes near the mural nodules. The specific immunohistochemical and morphologic features of SLMNs are helpful in differentiating them from malignant mural nodules, including true sarcomas, osteoclast-rich undifferentiated carcinomas, and carcinosarcomas. Such a differentiation is critical in view of its significant

  1. 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.

  2. Retroperitoneal robotic renal surgery: technique and early results.

    PubMed

    Patel, Manish N; Kaul, Sanjeev A; Laungani, Rajesh; Eun, Daniel; Bhandari, Mahendra; Menon, Mani; Rogers, Craig G

    2009-03-01

    We describe a robotic retroperitoneal approach to renal surgery, optimized in porcine and cadaveric models, and applied to human patients. A retroperitoneal approach for robotic kidney surgery was developed in nonsurvival porcine and a fresh cadaver models, and then utilized in ten patients (three partial nephrectomy, three radical nephrectomy, two simple nephrectomy, one pyeloplasty, one cryoablation). Retroperitoneal access was successfully achieved for robotic renal procedures in six pigs and a human cadaver. Ten human patients (mean age 56 years, range 36-72 years) then underwent a successful retroperitoneal approach for robotic renal surgery. Mean console time was 166 (120-300) min. Mean blood loss was 82 (50-100) ml and average hospital stay was 2.6 (1-5) days. Pathology demonstrated clear cell renal cell carcinoma (four), papillary renal cell carcinoma (two), and xanthogranulomatous pyelonephritis (two). One patient with xanthogranulomatous pyelonephritis required open conversion for failure to progress due to dense adhesions. A retroperitoneal approach is a safe and feasible alternative to a transperitoneal approach for robotic renal surgery, including radical nephrectomy, partial nephrectomy, pyeloplasty, and cryoablation. PMID:27628446

  3. Comparison of perioperative radiation therapy and surgery versus surgery alone in 204 patients with primary retroperitoneal sarcoma: a retrospective two-institution study

    PubMed Central

    Kelly, Kaitlyn J.; Yoon, Sam S.; Kuk, Deborah; Qin, Li-Xuan; Dukleska, Katerina; Chang, Kevin K.; Chen, Yen-Lin; Delaney, Thomas F.; Brennan, Murray F.; Singer, Samuel

    2014-01-01

    Objective To compare outcomes of patients with retroperitoneal or pelvic sarcoma (RPPS) treated with versus without perioperative radiation therapy (RT). Summary Background Data Radiation therapy for RPPS is controversial, and few studies have compared outcomes with and without RT. Methods Prospectively-maintained databases were reviewed to retrospectively compare patients with primary RPPS treated during 2003-2011. Multivariate Cox regression models were used to assess associations with the primary endpoints: local recurrence-free survival (LRFS) and disease-specific survival (DSS). Results At one institution 172 patients were treated with surgery alone while at another institution 32 patients were treated with surgery and perioperative proton beam RT or intensity-modulated RT with or without intraoperative RT. The groups were similar in age, tumor size, grade, and margin status (all p>0.08). The RT group had a higher percentage of pelvic tumors (p=0.03) and a different distribution of histologies (p=0.04). Perioperative morbidity was higher in the RT group (44% vs 16% of patients; p=0.004). After a median follow-up of 39 months, 5-year LRFS was 91% (95% CI, 79-100%) in the RT group and 65% (57-74%) in the surgery only group (p=0.02). On multivariate analysis, RT was associated with better LRFS (hazard ratio 0.26; p=0.03). Five-year DSS was 93% (95% CI, 82-100%) in the RT group and 85% (78-92%) in the surgery-only group (p=0.3). Conclusions The addition of advanced-modality RT to surgery for primary RPPS was associated with improved LRFS, although this did not translate into significantly better DSS. This treatment strategy warrants further investigation in a randomized trial. PMID:26061213

  4. 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.

  5. Identification of two homologs of the Kaposi's sarcoma-associated herpesvirus (human herpesvirus 8) in retroperitoneal fibromatosis of different macaque species.

    PubMed Central

    Rose, T M; Strand, K B; Schultz, E R; Schaefer, G; Rankin, G W; Thouless, M E; Tsai, C C; Bosch, M L

    1997-01-01

    Simian retroperitoneal fibromatosis (RF) is a vascular fibroproliferative neoplasm which has many morphological and histological similarities to human Kaposi's sarcoma (KS). Like epidemic KS in AIDS patients, RF is highly associated with an immunodeficiency syndrome (simian acquired immunodeficiency syndrome [SAIDS]) caused by a retrovirus infection. Recently, a new gammaherpesvirus, called Kaposi's sarcoma-associated herpesvirus (KSHV) or human herpesvirus 8 (HHV8), has been identified in KS tumors, suggesting that KS has a viral etiology. Our previous experimental transmission studies and epidemiological data suggest that RF also has an infectious etiology. In order to determine whether a similar virus is also associated with RF, we have assayed for the presence of an unknown herpesvirus using degenerate PCR primers targeting the highly conserved DNA polymerase genes of the herpesvirus family. Here we provide DNA sequence evidence for two new herpesviruses closely related to KSHV from RF tissues of two macaque species, Macaca nemestrina and Macaca mulatta. Our data suggest that KSHV and the putative macaque herpesviruses define a new group within the subfamily Gammaherpesvirinae whose members are implicated in the pathogenesis of KS and KS-like neoplasms in different primate species. PMID:9094697

  6. 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

  7. 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.

  8. 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

  9. Retroperitoneal inoculation of murine neuroblastoma results in a reliable model for evaluation of the antitumor immune response.

    PubMed

    Katsanis, E; Blazar, B R; Bausero, M A; Gunther, R; Anderson, P M

    1994-04-01

    To more closely mimic the natural site of human neuroblastoma and the original spontaneous arising paraspinal murine tumor, the authors developed a new model system in which murine neuroblastoma cells (neuro-2a) are implanted directly into the retroperitoneal space. This method of administration resulted in an aggressive and reproducible neuroblastoma model, with death occurring at a median of 20.3 days after tumor implantation using 1 x 10(6) neuro-2a cells, compared with the intraperitoneal (median, 31 days) and subcutaneous routes (median, 35.1 days) (P < .001). Adoptive transfer of single cell suspensions from livers, spleens, and bone marrows of mice with retroperitoneal tumors into healthy hosts resulted in tumor growth, confirming the presence of metastatic foci in these organs. The retroperitoneal murine neuroblastoma model was used to assess the importance of natural killer (NK) and T cells in regulating the growth of neuro-2a in vivo. T cells played an equally protective role as NK cells; depletion of either T or NK populations significantly decreased survival as compared with undepleted mice. Elimination of both NK and T cells further accelerated mortality of neuro-2a-bearing mice as compared to those depleted of either T or NK populations. The retroperitoneal murine model is a highly relevant in vivo system for preclinical studies of new therapeutic approaches for neuroblastoma.

  10. Retroperitoneal schwannoma.

    PubMed

    Takatera, H; Takiuchi, H; Namiki, M; Takaha, M; Ohnishi, S; Sonoda, T

    1986-12-01

    A case of retroperitoneal benign schwannoma is presented; in addition, 133 cases of retroperitoneal schwannoma reported in Japanese literature are studied. In our series 66 per cent of the cases were found to be cystic. Therefore, the cystic change may be one of the preoperative features of schwannomas, because other types of retroperitoneal tumor do not frequently form cysts. Furthermore, the necessity of postoperative electron microscopy for accurate histologic diagnosis is discussed.

  11. Retroperitoneal decortication of simple renal cysts vs decortication with wadding using perirenal fat tissue: results of a prospective randomized trial.

    PubMed

    Porpiglia, Francesco; Fiori, Cristian; Billia, Michele; Renard, Julien; Di Stasio, Andrea; Vaccino, Davide; Bertolo, Riccardo; Scarpa, Roberto Mario

    2009-06-01

    OBJECTIVES To evaluate, in a pilot prospective randomized trial, the safety, effectiveness and radiological recurrence of retroperitoneal renal cyst decortication compared with retroperitoneal decortication with wadding using perirenal pedicled fat tissue. PATIENTS AND METHODS From March 2004 to December 2007, 40 patients with simple renal cysts were enrolled and randomized; 22 (group A) had a simple retroperitoneal decortication (SRD) and 18 (group B) a decortication with wadding of the cyst using perirenal fat tissue (RDCW). The following variables were recorded: age, gender, side, size on ultrasonography/computed tomography (CT), location, operative duration, blood loss, complications, pathology, presence or absence of flank pain, hypertension, urinary tract compression or urinary infection. The primary endpoint of this trial was to evaluate and compare the efficacy of both treatments. Secondary endpoints were safety and pain, hypertension and the resolution of urinary tract obstruction. RESULTS In all, 40 cysts were treated; there were no bilateral cysts. The mean (sd) size on CT was 11.9 (1.84) cm in group A and 12.8 (1.25) cm in group B (P = 0.1). All the procedures were completed laparoscopically and no conversion was necessary. There were no intraoperative complications. The mean (range) hospital stay was 3.4 (3-6) days. There was no statistically significant difference between the groups for all variables assessed. There was a radiological recurrence in three patients (14%) in group A, but none in group B (all successful). CONCLUSION To be completely successful, with maximum safety and to prevent recurrences in the treatment of renal cysts, RCDW is recommended when a retroperitoneal approach is chosen, especially if the cyst is located anteriorly. When symptom relief is considered, RCDW duplicates the results obtained with SRD.

  12. [Retroperitoneal pathology].

    PubMed

    Zuluaga Gómez, A; Jiménez Verdejo, A

    2002-01-01

    The retroperitoneum is one of the most complex regions of human anatomy as it contains a variety organs and structures from different systems, in particular those belonging to the urinary and digestive tracts and the vascular systems. The emergence of different diagnostic imaging techniques has made easier to study the retroperitoneum. Thanks to ultrasonography, computerised tomography and magnetic resonance the assessment of retroperitoneal conditions has taken a great leap forwards permitting the anatomical connections and characteristics of the structures in this region to be reliably established. Owing to the location in the retroperitoneal space of the different organs of the digestive tract and the vascular system, a multidisciplinary approach is required involving the different surgical specialties. The urologist performs a large proportion of his surgical activity in this region and must, therefore, have a good knowledge of the different retroperitoneal organs belonging to the urinary tract and also the connections between these and other organs and structures of the retroperitoneal region.

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

    PubMed

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

    2016-10-01

    Radiotherapy and surgery are the two main pillars of the locoregional treatment of cancer. The risk of second malignancy is better evaluated and constitutes a major issue regarding radioprotection of the patients. Among malignant disease observed in the surviving irradiated patients, the occurrence of sarcoma is a rare event but associated with a poor outcome since the 5 year overall survival is estimated at 10 to 35 %. The SARI protocol, written in 2011, included 120 patients and 240 controlled patients, irradiated in the same conditions but without sarcoma observed during the follow up. The main objective was to identify the clinical and biological factors associated with the occurrence of such a complication. The secondary objective was to identify the dosimetric characteristics of the treatment of the primary. Preliminary results will be presented during the 2016 meeting of the French radiation oncology society. PMID:27599685

  14. 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

  15. Retroperitoneal lymphangioma.

    PubMed

    Cherk, Martin; Nikfarjam, Mehrdad; Christophi, Christopher

    2006-01-01

    Lymphangiomas are rare cystic tumours that may present as cystic masses in the retroperitoneum. Retroperitoneal lymphangiomas account for approximately 1% of all lymphangiomas. Confusion with other cystic tumours of the retroperitoneum including those arising from the liver, kidney and pancreas is common. A case of a retroperitoneal cystic lymphangioma occurring in a 41-year-old woman raising interesting diagnostic and management issues is reported. The patient presented with vague abdominal pain and persistent nausea. Radiological imaging demonstrated a large multiloculated thin-walled cyst involving the upper retroperitoneum. Surgical resection was complete, revealing a benign cavernous lymphangioma. The patient made a complete recovery and was disease-free 30 months postoperatively.

  16. 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

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

    SciTech Connect

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

    1989-04-15

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

  18. Retroperitoneal foregut cyst.

    PubMed

    Kajiya, Y; Nakajo, M; Ichinari, N; Yamazumi, K; Otuji, T; Tanaka, T

    1997-01-01

    A foregut cyst is formed as a result of abnormal budding and pinching of the tracheobronchial tree when bronchial buds develop to form the primitive respiratory tree. Foregut cysts are clinically classified as bronchogenic, esophageal, enterogastric, or ciliated hepatic. We present a foregut cyst that occurred in the retroperitoneum and was difficult to distinguish from other retroperitoneal cystic mass lesions. Magnetic resonance imaging was useful in revealing the cyst's continuity to adjacent organs.

  19. Efficacy of adjuvant CYVADIC chemotherapy in early-stage uterine sarcomas: results of long-term follow-up.

    PubMed

    Odunsi, K; Moneke, V; Tammela, J; Ghamande, S; Seago, P; Driscoll, D; Marchetti, D; Baker, T; Lele, S

    2004-01-01

    Data on adjuvant chemotherapy in early-stage uterine sarcomas are conflicting and most often based on small patient groups with relatively short duration of follow-up. Approximately 60% of patients present with stage I disease with an overall 5-year survival of 30-50% when treated with surgery alone. This study examines the efficacy and results of long-term follow-up of a multiagent chemotherapy regimen of cyclophosphamide, vincristine, doxorubicin, and dacarbazine (CYVADIC) as adjuvant treatment for patients with stage I uterine sarcoma. Between 1982 and 1999, 24 evaluable patients with completely staged uterine sarcomas received adjuvant multiagent chemotherapy with vincristine sulfate (1mg /m(2)) on days 1 and 4, doxorubicin (40 mg /m(2)) and cyclophosphamide (400 mg /m(2)) on day 2, and dacarbazine (200 mg /m(2)) on days 1 through 4 for a total of nine monthly cycles or until recurrence of disease was documented. Survival distributions were calculated by the Kaplan-Meier method, and statistical significance was determined with the log-rank test. Factors significant on univariate analysis were analyzed in a multivariate fashion using Cox proportional hazards model. The histologic distribution of patients was 46% leiomyosarcoma, 33% mixed mullerian tumors, 13% stromal sarcomas, 4% adenosarcomas, and 4% hemangiosarcoma. The patients received 206 of a planned 216 cycles of chemotherapy. The median follow-up of the patient population was 93 months (range 11-213 months). Eight patients (33%) developed recurrent disease. The median time to recurrence was 19 months (range 7-184 months). The estimated survival for the entire group was 88, 75, and 69% at 2, 5, and 15 years, respectively. Factors that did not affect survival included age, histology, and tumor grade. Four patients required dose reductions secondary to grade 2-3 toxicities (hematologic). Grade 1 neurotoxicity was observed in six patients (25%) and grade 2 neurotoxicity in one patient (4%). Adjuvant CYVADIC

  20. 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

  1. 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.

  2. 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.

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

    SciTech Connect

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

    2006-02-01

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

  4. 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/ ...

  5. 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 ...

  6. Retroperitoneal laparoscopic pyelolithotomy.

    PubMed

    Gaur, D D; Agarwal, D K; Purohit, K C; Darshane, A S

    1994-04-01

    Retroperitoneal laparoscopic pyelolithotomy was successful in 5 of 8 patients using the recently described balloon technique of retroperitoneal laparoscopy. All patients were considered for this new minimally invasive procedure only on economic grounds. However, with improved technique and instrumentation, the retroperitoneal laparoscopic approach could become a practical alternative for the management of patients with medium sized pelvic stones not amenable to extracorporeal shock wave lithotripsy nor ideally suitable for percutaneous nephrolithotomy, or when both of these facilities are not available. PMID:8126827

  7. 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

  8. 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

  9. Retroperitoneal and mesenteric cysts.

    PubMed

    Alwan, M H; Eid, A S; Alsharif, I M

    1999-03-01

    Retroperitoneal and mesenteric cysts are rare abdominal tumours. This report is a presentation of three cases. One patient had large retroperitoneal cyst which was accidentally discovered, another patient had mesenteric cyst presenting with abdominal pain, and the third patient had emergency admission due to infection of a large mesenteric cyst. The literature on this condition is reviewed.

  10. Calcified retroperitoneal fibroma.

    PubMed

    Illuminati, G; Bertagni, A; Montesano, G; Soda, G; Baiocchini, A; Melis, M; Vietri, F

    1997-01-01

    A case of 31-year-old male with a retroperitoneal tumor is described. Abdominal ultrasound revealed a left para-aortic calcific mass, adjacent to the left lobe of the liver and to the upper pole of the left kidney. A CT-scan of the abdomen showed the mass to originate from the left adrenal gland. At operation, a large, retroperitoneal mass, adherent to the left kidney and the spleen, but not infiltrating, was excised. Histologically the tumor was diagnosed as a calcified osteo-producing fibroma. Benign retroperitoneal tumors represent about 25% of all retroperitoneal neoplasm. This reported case represents a retroperitoneal tumor of slow growth and benign clinical course whose characteristic consists of the heavy calcifications which are normally absent in a fibroma type mass.

  11. [Importance of tomodensitometry in the diagnosis of retroperitoneal tumors].

    PubMed

    Braedel, H U; Schindlec, E; Rzehak, L

    1983-01-01

    3 patients are presented who were admitted with the suspected urographic diagnosis of a "retroperitoneal tumor". A battery of additional investigations had not led to a definite diagnosis in the patients with an aortic aneurysm, a large dermoid cyst and a small bowel sarcoma infiltrating into the retroperitoneum. Among the 3 cases, only the dermoid cyst, could not be recognized angiographically. CT, however, provided the correct diagnosis in all 3 patients. Only one additional barium study in a patient with a small bowel sarcoma was necessary for further confirmation of the diagnosis.

  12. [Retroperitoneal diseases and geriatric-gynecologic laparatomy (author's transl)].

    PubMed

    Jaluvka, V

    1976-05-01

    24 cases are described in which retroperitoneal processes were operated on for primarily gynecologic reasons in women of at least 60 years of age. They include 6 sarcomas, 3 neurinomas, 1 cyst, 1 fibroma, 1 lipoma, 1 congenital sacciform kidney, 1 hydronephrosis, 3 pancreatic carcinomas, 2 renal carcinomas, 1 ureteral cyst and 4 metastases of various malignomas. These cases were gathered in 18 West Berlin Departments of Obstetrics and Gynecology during a 10-year period. The post-operative mortality rate was 37.5% (9 deaths); this is probably due to the relatively low incidence of symptoms and signs associated with retroperitoneal diseases and their anatomical localization. Finally, new procedures for the diagnosis of retroperitoneal tumors are indicated.

  13. Retroperitoneal malignant fibrous histiocytoma can mimic a hydatid cyst.

    PubMed

    Erbay, Gurcan; Ulusan, Serife; Koc, Zafer; Canpolat, Emine Tuba; Calıskan, Kenan

    2011-01-01

    Malignant fibrous histiocytoma is the second most common soft-tissue sarcoma in adults. After the extremities, the retroperitoneal space is the second most common site of this tumor. A 50-year-old man presented with a right retroperitoneal, thick-walled, cystic multilocular mass measuring 10 × 10 cm that was thought to be a type CE 5 hydatid cyst preoperatively. However, the postoperative histopathology did not agree with the radiological findings and instead showed a malignant fibrous histiocytoma. The computed tomography and ultrasound/Doppler ultrasound findings of this retroperitoneal mass mimicked a type CE 5 hydatid cyst. We present this case because the surgical management of these two lesions differs and misdiagnosis can be problematic.

  14. [Recent results of research on cancer of the colon, gastric cancer, sarcoma and bronchial carcinoma].

    PubMed

    Hacker, U T; Wolf, J; Wendtner, C-M

    2011-02-01

    In patients up to 70 years of age with colon carcinoma stage III adjuvant chemotherapy with infusions of fluorouracil (5-FU) or oral capecitabine combined with oxaliplatin should be the standard method. A new standard for the palliative treatment of Her2/newly positive advanced gastric cancer and cancer at the gastro-esophageal junction is the administration of trastuzumab combined with chemotherapy. Patients with high-risk soft tissue sarcoma can be helped, in addition to surgical resection and subsequent radiotherapy, by neoadjuvant chemotherapy combined with regional deep hyperthermia. For patients with lung cancer additional individualized treatment is about to become routine. In addition to the EGFR mutation status, all non-smokers should in future be tested for aberration in the anaplastic lymphoma kinase (ALK) gene.

  15. [Retroperitoneal chylous cyst].

    PubMed

    Klingenberg, C; Johansen, T E

    1993-11-10

    In pathology, cysts of the retroperitoneum are usually classified together with omental and mesenteric cysts. Because of risk of complications and malignancy these cysts should be extirpated. The frequency of complications and recurrence after surgery is significantly higher for retroperitoneal cysts than for the other types mentioned above. We describe a patient with a retroperitoneal chylous cyst of three litres. Correct diagnosis was made by CT-scan and chemical analysis of cyst fluid prior to surgery. The cyst was removed completely using an intercostal, retroperitoneal approach. The postoperative course was uneventful, and no evidence of recurrence was found after six months.

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

    SciTech Connect

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

    1983-08-01

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

  17. Mesenteric and retroperitoneal cysts.

    PubMed

    Kurtz, R J; Heimann, T M; Holt, J; Beck, A R

    1986-01-01

    Mesenteric and retroperitoneal cysts are rare intra-abdominal tumors. Ten new patients are presented as well as 152 other cases reported in the English literature. These 162 cases were then analyzed for significant trends. Patients under 10 years of age were significantly different from the older group with respect to a shorter duration of symptoms, a higher number of patients requiring an emergency operation, a lower number of recurrences and the location of the cyst. Patients with retroperitoneal cysts were more likely to have incomplete excision of the cyst and therefore had a higher incidence of recurrence. They also required marsupialization more often. Retroperitoneal cysts should be considered a different entity from mesenteric cysts even though they present clinically in a similar fashion. The outcome of surgical treatment is less satisfactory in patients with retroperitoneal cysts.

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

    SciTech Connect

    Kuten, A.; Sapir, D.; Cohen, Y.; Haim, N.; Borovik, R.; Robinson, E.

    1985-03-01

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

  19. [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.

  20. Primary retroperitoneal hydatid cyst.

    PubMed

    Çullu, N; Karakaş, Ö; Kiliçaslan, N; Konukoğlu, O; Karakaş, E

    2013-01-01

    Hydatid cyst is formed from parasitic infestation by Echinococcus granulosus, and may involve several bodily organs, primarily the liver and lungs. In endemic regions, hydatid cyst should be borne in mind in cases of retroperitoneal cystic masses. Diagnosis of hydatid cyst is made from radiological findings and serological test positivity. Hydatid cyst diagnosis can be made from typical radiological imaging findings. This paper presents the imaging findings of a 56-year old male with retroperitoneal hydatid cyst.

  1. [Retroperitoneal enteroid cyst].

    PubMed

    Patel, J C; Monneins, F; Louvel, A

    1975-04-01

    The author report a case of enteroid retroperitoneal cyst treated by enucleation. Such a lesion raises mainly the problem of histological recognition and embryological interpretation for which there are two possible theories: (1) Uptake, before sealing off of the embryo, of a cell group of endoblastic origin which develops some distance from the digestive tract. (2) Retroperitoneal course of a simple teratoma of enteroid type. Such a possibility of teratoma leaves open the possibility of malignant transformation.

  2. Laparoscopic retroperitoneal renal cystectomy.

    PubMed

    Munch, L C; Gill, I S; McRoberts, J W

    1994-01-01

    Laparoscopic manipulation of retroperitoneal organs is usually performed by the transperitoneal approach primarily because of the ease of access by way of the pneumoperitoneum. However, difficulty in adequately accessing structures that are surrounded by bowel, liver, spleen or postoperative adhesions makes this approach suboptimal in certain cases. We describe the use of the retroperitoneal laparoscopic approach to the upper pole of a kidney for marsupialization of a symptomatic, recurrent, complex renal cyst. An algorithm for current management of symptomatic renal cysts is discussed.

  3. 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

  4. Retroperitoneal lymphocele formation after selective laparoscopic retroperitoneal lymph node sampling.

    PubMed

    Goh, M; Kantoff, P; Kavoussi, L R

    1994-06-01

    Laparoscopic retroperitoneal lymph node sampling is being evaluated at several institutions as a minimally invasive alternative to evaluate pathologically retroperitoneal lymph nodes in cases of clinical stage A testis tumor. We report on the development of a symptomatic lymphocele after selective laparoscopic retroperitoneal lymph node sampling.

  5. 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 ( ...

  6. 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

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

    PubMed Central

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

    2016-01-01

    Dedifferentiated liposarcoma (DDL) is a histologically pleomorphic sarcoma, traditionally defined as well-differentiated liposarcoma with abrupt transition to high grade, non-lipogenic sarcoma. It can occur as part of recurrent well-differentiated liposarcoma, or may arise de novo. DDL most frequently occurs within the retroperitoneum, and while it is prone to local recurrence, it usually has a lower rate of metastasis than other pleomorphic sarcomas. We describe a case of retroperitoneal dedifferentiated liposarcoma in a 63-year-old male, who showed MDM2 amplification with fluorescence in situ hybridization, which displayed unusually aggressive behavior, with brain, lung and subcutaneous soft tissue metastases. As previous reports of metastatic liposarcoma have largely grouped DDL in with other (genetically and clinically distinct) liposarcoma subtypes, we highlight and discuss the rare occurrence of brain metastasis in MDM2-amplified retroperitoneal liposarcoma. PMID:27746879

  8. [Primary Retroperitoneal Hydatid Cyst].

    PubMed

    Tali, Servet; Aksu, Ali; Bozdağ, Pınar Gündoğan; Bozdağ, Ahmet

    2015-09-01

    Hydatid disease is a parasitosis which is created by Echinococcus granulosus. Hydatid cysts most of ten settled in the liver and lungs. Hydatid cyst is rarely seen in retroperitoneal. Sixty-three year-old female patient was admitted to our hospital with complaints of abdominal distention and with back pain in the Abdominal ultrasonography and computed tomography images, on the posterior of the left kidney, in paravertebral area approximately 15 x 10 cm in size septal cystic lesion was observed retroperitoneally. At laparotomy, partial excision of the retroperitoneal cyst was performed and drainage of the cyst pouch was provided by suction drain. Suction drain was removed 5 days after surgery. Histopathological diagnosis is was reported as hydatid cyst. Hydatid disease is a endemic disease in our country and it should be known that has a typical placements.

  9. Primary Retroperitoneal Mucinous Cystadenoma.

    PubMed

    Lee, Seok Youn; Han, Weon Cheol

    2016-02-01

    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.

  10. Retroperitoneal enteric duplication cyst.

    PubMed

    Lo, Yu-Shing; Wang, Jyh-Seng; Yu, Chia-Cheng; Chou, Chung-Ping; Chen, Chia-Jung; Lin, Shong-Ling; Lee, Mang-Gang; Kuo, Yau-Chang; Tseng, Hui-Hwa

    2004-09-01

    Enteric duplication cysts (EDCs) can occur in any portion of the alimentary tract, but are most commonly associated with the small bowel and esophagus. Retroperitoneal location is really unusual. This 19-year-old female was in excellent health, but a week's abdominal pain made her search for a doctor's help. After the detailed examination, surgical intervention was performed under the impression of cystic tumor of the retroperitoneum. A retroperitoneal cystic tumor, 13.0 x 8.0 x 3.5 cm in size, without any communication with the alimentary tract was noted during the operation. Finally, EDC was diagnosed after the pathologic examination of this resected cystic lesion. To our knowledge, there have been only 6 reported cases of EDC of the retroperitoneum in the English literature. This report concerns the seventh case of retroperitoneal EDC, in an adolescent, with different clinical presentation and histopathologic findings from the previous ones.

  11. Primary retroperitoneal mucinous cystadenoma.

    PubMed

    Abedalthagafi, Malak; Jackson, Patrick G; Ozdemirli, Metin

    2009-01-01

    Primary mucinous neoplasms of the retroperitoneum, including mucinous cystadenocarcinomas, mucinous borderline tumors, and mucinous cystadenomas are uncommon tumors found exclusively in women. Since the retroperitoneum does not contain mucinous epithelium, the origin, and histogenesis of these tumors remain unclear. It is speculated that these tumors can arise from teratomas, supernumerary ovaries, or mucinous metaplasia of the retroperitoneal mesothelium. We describe a case of a primary mucinous cystadenoma of the retroperitoneum in a 44 year-old female that presented as a palpable abdominal mass. There was no evidence of recurrence 16 months after complete laparoscopic excision of the tumor. The morphology and immunohistochemical analysis in this case support the hypothesis that mucinous metaplasia of the retroperitoneal mesothelium overlying a preceding inclusion cyst can give rise to retroperitoneal mucinous tumors.

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

    PubMed

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

    2015-01-01

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

  13. [Retroperitoneal germ cell tumor].

    PubMed

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

    1999-03-01

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

  14. [Retroperitoneal bronchogenic cyst].

    PubMed

    Piton, Nicolas; Gobet, Françoise; Werquin, Claire; Landréat, Antoine; Lefebvre, Hervé; Pfister, Christian; Sabourin, Jean-Christophe

    2012-08-01

    Bronchogenic cysts are benign lesions, which are usually described at the chest level. We present here a case report of a retroperitoneal bronchogenic cyst. A 77-year-old man presented with a left retroperitoneal tumor discovered by scanner. There was no endocrine disruption. Excision of the lesion was performed and final diagnosis was a bronchogenic cyst. Current widespread use of modern radiology enables increased discovery of such "incidentalomes". In the future, pathologists will be routinely faced with this type of diagnosis, which up to now has been described as exceptional.

  15. Retroperitoneal cystic lymphangioma.

    PubMed

    Nuzzo, G; Lemmo, G; Marrocco-Trischitta, M M; Boldrini, G; Giovannini, I

    1996-03-01

    Two cases of retroperitoneal cystic lymphangioma (CL) are presented; the current literature on this rare, benign neoplasm of the lymphatic system is reviewed. This tumor consists of various numbers of cyst-like cavities filled with a serous, serosanguineous or chylous fluid. The histogenesis of CL is still uncertain. Most commonly CL occurs in the neck and in the axillary region, whereas it is rare in the retroperitoneum. Although retroperitoneal CL is a benign lesion, it may cause significant morbidity due to its large size, and its often invasive character with a strong tendency to secondary infection. The treatment of choice is surgical excision.

  16. Primary retroperitoneal mucinous cystadenoma.

    PubMed

    Rifki Jai, S; Bouffetal, R; Chehab, F; Khaiz, D; Bouzidi, A

    2009-09-01

    Primary retroperitoneal mucinous cystic tumors are extremely rare, and although their histopathogenesis is still uncertain, several theories have been proposed. Traditionally, transabdominal laparotomy and enucleation of the cyst is the treatment of choice. The anatomopathological examination of the mass is imperative in the fact to eliminate malignant lesions. We report the case of a 43-year-old woman, with primary retroperitoneal mucinous cystic tumor, revealed by an abdomino-pelvic mass. During laparotomy, a totality of the tumor was removed. The anatomopathologic study permitted the diagnosis.

  17. 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

  18. 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.

  19. Retroperitoneal lymphocele after abdominal aortic surgery.

    PubMed

    Garrett, H E; Richardson, J W; Howard, H S; Garrett, H E

    1989-09-01

    Lymphoceles may occur as a result of lymphatic injury during abdominal aortic surgery. These lymphatic collections may occur as a retroperitoneal mass or as groin lymphoceles. Four cases are presented in which persistent retroperitoneal lymphoceles were discovered 2 to 8 years after surgery. Reexploration of the groin and repeated aspirations of lymphatic fluid failed to control the drainage. Reexploration of the retroperitoneum documented lymphatic injury, which was controlled by ligation of the lymphatics with suture. A review of the literature discloses five similar reported cases of retroperitoneal lymphocele and 12 cases of chylous ascites after abdominal aortic surgery. Clearly, avoiding lymphatic injury or immediate repair of any lymphatic injuries will prevent this problem. Once a persistent lymphocele has developed, aspiration will establish the diagnosis. Our experience would suggest that reexploration of the retroperitoneum is required to control the drainage and to prevent possible graft infection.

  20. Retroperitoneal fibrosis and scleroderma.

    PubMed Central

    Mansell, M. A.; Watts, R. W.

    1980-01-01

    The association of retroperitoneal fibrosis and systemic sclerosis is reported from a patient positive for the HLA-B27 antigen. This appears to be the first report of such an association. Pathological features common to the 2 syndromes are discussed and the literature is reviewed. PMID:7220411

  1. [Pediatric retroperitoneal tumors].

    PubMed

    Benicio dos Santos, I; Benicio dos Santos, M

    1980-01-01

    The author has based his work "Retroperitoneals tumors in infancy and childhood" in 65 cases observed at "Hospital Martagao Gesteira", Salvador, Bahia, Brasil. 32 of the retroperitoneals tumors, either intrarenals or extrarenals, observed in infancy and childhood were Wilm's tumor, 22 neuroblastoma, 5 hydronephrosis, 2 multicystic kidney, 1 policystic kidney, 2 pancreatic cyst and 1 biliar cyst. Wilm's tumor had the highest incidence - 32 cases (49,2%); neuroblastoma was in the second place in incidence - 22 (33,8%) of the 65 cases of retroperitoneals tumors studied, were neuroblastoma. As registered by the author in previous paper, the neuroblastoma, on contrary of what is established in the specialized literature, not was: the most frequent abdominal tumors, in infancy and childhood, neither it was also the abdominal pediatric tumor which could match Wilm's tumor in incidence. The plain X ray film of the abdomen, the Excretory Urography, the Cavography and Arteriography, the Radiological Examination of the Stomach and Duodenum, of the Small Intestine and the Colons, contribute in a very important way to establish the topography (retro or intraperitoneal) of the pediatric abdominal tumors. The author emphasizes that the plain X ray film of the abdomen supply important elements for the conclusion concerning the localization of abdominal tumors, from the observation of a simple criterion - the retroperitoneals tumors obliterate the border of kidney, because they are placed in the same plan of the kidney, data which is not pointed out sufficiently by the authors who have studied the subject.

  2. [Primary retroperitoneal hydatidosis].

    PubMed

    Benejam Gual, J M; Rioja Sanz, C; Cuesta Presedo, J M; Gil Martínez, P; Bono Ariño, A; Rioja Sanz, L A

    1997-05-01

    Genitourinary involvement by the Echinococcus granulosus larvae (urinary hydatidosis) ranks third in order of frequency after liver and lung involvement. The finding of a primary hydatidic cyst with retroperitoneal location is an uncommon fact. This paper presents once case of this infrequent disease. A revision of the different etiopathogenic mechanisms, as well as diagnostic and therapeutic approaches is made.

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

    PubMed

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

    2010-03-01

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

  4. 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.

  5. Histiocytic sarcoma

    PubMed Central

    Machado, Eduardo Silva; de Miranda, Ana Carolina; Escopelli, Ticiane; Caron, Ruggero; Escopelli, Alessandra Cristhina

    2011-01-01

    A 59-year-old white woman, SC, after being treated for pneumonia, presented with an increase in the size of lymph nodes. The immunohistochemical examination diagnosed histiocytic sarcoma. Relapse occurred 12 months after starting chemotherapy. The patient evolved with febrile neutropenia, septic shock and death. PMID:23284265

  6. 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

  7. 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.

  8. [Primary retroperitoneal hydatid cyst].

    PubMed

    Gündeş, Ebubekir; Küçükkartallar, Tevfik; Çakır, Murat

    2014-01-01

    Hydatid cysts cause diseases most frequently by localizing in the liver and the lungs. Hydatid cysts with retroperitoneal localization are very rare. A 45-year-old female patient presented to our hospital with complaints of back pain, weight loss, and fatigue. The computerized tomography (CT) revealed that the patient had a septated cystic lesion of about 8x7x6 cm localized in the posterior of the left kidney, in the paravertebral site causing destruction of the neighboring costa. During laparotomy, the wall of the cyst with retroperitoneal localization was partially excised and the remaining cavity was drained. The drain was removed on post-op day 5. The histopathological diagnosis was reported to be a hydatid cyst. No problems were seen during the follow-ups of the patient who was administered post-op albendazole. Hydatid cysts are an endemic disease in our country and it should be kept in mind that they also have atypical localizations.

  9. [Retroperitoneal and mesenteric cysts].

    PubMed

    Vasilev, N; Kirov, G; Avramov, T

    1990-01-01

    Experience is recorded with the diagnosis and successful surgical treatment of 5 patients with retroperitoneal and mesenterial cysts. According to available data in the literature, one patients with retroperitoneal or mesenteric cyst falls among an average of 34,000-105,000 in-lying patients. Basic symptom on physical examination was a palpable tumor mass in the abdomen. In recent years correct preoperative diagnosis of these diseases has been established in 85-95 per cent of the cases with the help of echography and computer tomography. The surgical approach was determined from the possibility for total or partial removal of the cyst, which was sometimes accompanied by partial or total removal of abdominal organs involved in the pathologic process.

  10. Retroperitoneal unilateral ileofemoral thromboendarterectomy.

    PubMed

    Brown, L L; Kartchner, M M

    1986-04-01

    This clinical series of 145 operative procedures is presented to demonstrate that an aggressive approach in the management of ileofemoral arterial insufficiency utilizing a retroperitoneal approach for thromboendarterectomy is useful and appropriate. Considering the overall risk factors associated with this patient population, we have demonstrated that this method is well tolerated as supported by the low morbidity and mortality figures presented. The native vascular tree has been preserved, and excellent early and long-term revascularization goals have been obtained.

  11. Retroperitoneal neurogenous choristoma.

    PubMed

    Wan, J; Ritchey, M L; Muraszko, K; Bloom, D A

    1992-12-01

    A 6-year-old girl with urinary complaints underwent ultrasonography, which revealed a retroperitoneal cystic mass adjacent to the left kidney. Computerized tomography did not demonstrate whether the mass was an adrenal cyst or a duplicated upper pole segment. Exploration revealed a neurogenous choristoma, which is a collection of histologically normal tissue that is ectopically situated. We believe that this is the first report of this rare entity occurring in the retroperitoneum.

  12. [A retroperitoneal bronchogenic cyst].

    PubMed

    Colović, R; Radovanović, N; Micev, M; Colović, N; Stojković, M

    2001-01-01

    Bronchogenic cyst is a rare congenital (developmental) anomaly. It is usually asymptomatic but its enlargement and localization may cause serious symptoms. Exact preoperative diagnosis is rarely established. As a rule, it is established during histological examination of the resected specimen. We present a patient with a subdiaphragmatic retroperitoneal bronchogenic cyst in whom exact diagnosis was documented by histological examination of excised cyst. To our knowledge this is the third such case ever reported.

  13. [Retroperitoneal liposarcoma. Two cases].

    PubMed

    Bennani, S; Debbagh, A; Louahlia, S; el Mrini, M; Benjelloun, S

    1995-01-01

    Concerning two cases of liposarcomas developing in the retroperitoneal space, the characteristics of these rare tumors are reviewed. They are the most frequent mesenchymal tumors. Often, they are uncovered by palpable abdominal mass or when they compress adjoining organs. Computed tomography has proved valuable in the diagnosis of these lesions. Surgical removal is the treatment of choice. Recurrences are frequent and prognosis is poor. PMID:7486852

  14. Retroperitoneal Laparoscopic Pyelolithotomy in an Ectopic Pelvic Kidney

    PubMed Central

    Cirakoglu, Abdullah; Ozer, Serafettin

    2012-01-01

    Background and Objectives: Retroperitoneal laparoscopic pyelolithotomy was performed in an ectopic pelvic kidney with renal pelvis calculi. Methods and Results: Laparoscopic pyelolithotomy was successfully performed in an ectopic pelvic kidney by using the retroperitoneal route. The total operation time was 130 minutes, and the estimated blood loss was <50mL. The patient was discharged on the second postoperative day without any complications. Conclusion: Laparoscopic pyelolithotomy is an effective treatment option for management of stones in the pelvis of an ectopic pelvic kidney. The retroperitoneal route may help to avoid intraoperative and postoperative complications. PMID:23477189

  15. 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

  16. Bronchogenic cysts in retroperitoneal region.

    PubMed

    Cai, Yunnan; Guo, Zonghua; Cai, Qiliang; Dai, Shipeng; Gao, Weimin; Niu, Yuanjie; Li, Gang; Zhang, Ye

    2013-02-01

    Bronchogenic cyst is a relatively rare abnormality that develop from the accessory lung buds of the foregut. The cyst is regarded as a congenital developmental abnormality. Occurence in the retroperitoneal presentation is rare. Here, we present two patients who had an incidentally discovered retroperitoneal mass which were revealed to be bronchogenic cysts after surgical extirpation.

  17. Neurocutaneous syndromes and retroperitoneal tumors.

    PubMed

    Rossi, R; Libertino, J A; Dowd, J B; Braasch, J W

    1979-03-01

    A patient with multiple basal cell carcinoma syndrome, a symptom complex characteristized by nevoid basal cell carcinomas of the skin, jaw cysts, skeletal abnormalities, and hyporesponsiveness to parathormone is presented. In addition, the patient had a retroperitoneal lymphagiomyoma, a hamartomatous lesion, causing ureteral obstruction. The association of neuroectodermic syndromes and retroperitoneal and intra-abdominal tumors is reviewed.

  18. An egg shell-like retroperitoneal pseudocyst.

    PubMed

    Li, Zhao-Lun; Li, Hong-Liang; Chen, Hai-Weng; Gan, Wei-Ming

    2011-09-01

    Retroperitoneal pseudocysts are rare entities. Egg shell-like calcified retroperitoneal pseudocyst is rarer. We report a 75-year-old woman with an egg shell-like calcified retroperitoneal pseudocyst. Subsequently, the calcified pseudocyst was dissected and excised completely through laparoscopy via a retroperitoneal approach.

  19. Primary retroperitoneal echinococcal cyst.

    PubMed

    Gavriilidis, Paschalis; Ananiadis, Ananias; Theodoulidis, Vasilios; Barbanis, Sotirios

    2012-01-01

    A 74-year-old man was admitted with right flank pain and discomfort lasting for 2 months. CT scanning revealed a large retroperitoneal cystic mass. There were no cysts elsewhere. Serological testing revealed hydatid disease. Preoperatively he was treated by Albendazole 400 mg for 1 month and then underwent laparotomy. The entire mass was excised en bloc and intact and right hemicolectomy was simultaneously performed for excision of the adhered ascending colon. He recovered uneventfully and was discharged on the eighth day, Albendazole was given and follow-up visits were arranged for every 6 months. Total cystectomy in case of active echinococcal cysts remains the treatment of choice.

  20. Retroperitoneal mesenteric cysts.

    PubMed

    Katsohis, C D; Papapolichroniadis, C; Nenopoulou, H; Aletras, H A

    1989-01-01

    Retroperitoneal cysts are most commonly found in relation to the small bowel but may be found in the mesentery of the colon or in the omentum. Only a few hundred cases have been reported. In the present analysis four more cases are reported and the pertinent literature is reviewed. The diagnosis is made by clinical suspicion, barium meal and/or enema, sonography and axial computerized tomography. Treatment of choice is by enucleation of the cyst, if possible. Morbidity and mortality should be very low, because of recent advances in surgery and intensive care of the patient.

  1. Primary retroperitoneal mucinous cystadenocarcinoma.

    PubMed

    Nelson, H; Benjamin, B; Alberty, R

    1988-05-15

    A case of primary retroperitoneal cystadenocarcinoma is presented as the fourth reported case in the world literature to date. The cyst was removed intact and demonstrated an infiltrating malignant process with nuclear pleomorphism and mitotic activity. No ovarian tissue was identified and a cancer antigen 125 (CA 125) test was normal. The patient underwent a staging procedure subsequently that included peritoneal washings, hysterectomy, bilateral salpingoophorectomy, and iliac node dissection. No metastases were found and the patient is without recurrence 22 months postoperative. The literature is reviewed to better define the origin and prognosis of these tumors.

  2. Hemoperitoneum in a peritoneal dialysis patient from a retroperitoneal source.

    PubMed

    Balsera, Cristina; Guest, Steven

    2013-01-01

    Hemoperitoneum in peritoneal dialysis patients is a known but infrequent complication. Hemoperitoneum is more frequent in women because of its association with a variety of gynecologic presentations such as reflux menstruation, ovulation, endometrial tissue implants within the peritoneal cavity (endometriosis), and bleeding follicular cysts. Other intraperitoneal causes of hemoperitoneum include minor catheter or abdominal trauma, vascular anomalies, or hepatic or splenic cysts. Less frequently encountered is a presentation of hemoperitoneum from a retroperitoneal source. These presentations result either from peritoneal inflammation and subsequent peritoneal bleeding or retroperitoneal cavity pathology rupturing into the peritoneal cavity. Here, we present the case of a peritoneal dialysis patient presenting with hemoperitoneum several days after undergoing cardiac catheterization. The catheterization was complicated by a large retroperitoneal hematoma. Details of the case are reported, and other retroperitoneal causes of hemoperitoneum are reviewed.

  3. Primary Retroperitoneal Myxoid Liposarcomas

    PubMed Central

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

    2016-01-01

    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

  4. [Retroperitoneal cystic lymphangioma].

    PubMed

    Waisberg, J; Pezzolo, S; Henrique, A C; Kerr, L M; Speranzini, M B

    1999-01-01

    The lymphangioma is a rare disease, more frequently reported in children and just occasionally in the adult patient. The lymphangioma is considered a benign neoplasm of embryonic origin of the lymphatic vessels. Its habitual location is in the cervical and axillary area; it is rarely found in the abdominal cavity and exceptionally in the retroperitonio. In this latter location, the lesion habitually is asymptomatic. The clinical diagnosis of the retroperitoneal cystic lymphangioma is not often due to its rarity and the absence of clinical expression. The size of the lesion is more important than its location to the symptomatology development. The findings of the abdominal ultrasonography and computerized tomography of the abdomen usually show a cystic lesion and its location. The treatment is surgical and it consists of the resection of the cyst or group of cysts once the liquid accumulation in its interior may be responsible for the development of some important complications of this disease. The cure is obtained when the lesion is completely resected also with the resection of eventual adhesive structures. The relapse may take place when the resection is incomplete. A case of retroperitoneal lymphangioma in a female adult patient as incidental finding of abdominal ultra-sonography is described. It is discussed the clinical picture, the radiologic diagnosis, the treatment and the prognostic of this unusual disease.

  5. Ewing sarcoma: a chronicle of molecular pathogenesis.

    PubMed

    Kim, Sang Kyum; Park, Yong-Koo

    2016-09-01

    Sarcomas have traditionally been classified according to their chromosomal alterations regardless of whether they accompany simple or complex genetic changes. Ewing sarcoma, a classic small round cell bone tumor, is a well-known mesenchymal malignancy that results from simple sarcoma-specific genetic alterations. The genetic alterations are translocations between genes of the TET/FET family (TLS/FUS, EWSR1, and TAF15) and genes of the E26 transformation-specific (ETS) family. In this review, we intend to summarize a chronicle of molecular findings of Ewing sarcoma including recent advances and explain resultant molecular pathogenesis. PMID:27246176

  6. Measurement of Visceral Fat: Should We Include Retroperitoneal Fat?

    PubMed Central

    Yang, Chung-Yi; Hsieh, Hung-Ren; Ma, Wen-Ya; Lin, Mao-Shin; Liu, Pi-Hua; Shih, Shyang-Rong; Liou, Jyh-Ming; Chuang, Lee-Ming; Chen, Ming-Fong; Lin, Jou-Wei; Wei, Jung-Nan; Li, Hung-Yuan

    2014-01-01

    Objective Whether retroperitoneal fat should be included in the measurement of visceral fat remains controversial. We compared the relationships of fat areas in peritoneal, retroperitoneal, and subcutaneous compartments to metabolic syndrome, adipokines, and incident hypertension and diabetes. Methods We enrolled 432 adult participants (153 men and 279 women) in a community-based cohort study. Computed tomography at the umbilicus level was used to measure the fat areas. Results Retroperitoneal fat correlated significantly with metabolic syndrome (adjusted odds ratio (OR), 5.651, p<0.05) and the number of metabolic abnormalities (p<0.05). Retroperitoneal fat area was significantly associated with blood pressure, plasma glycemic indices, lipid profile, C-reactive protein, adiponectin (r = −0.244, P<0.05), and leptin (r = 0.323, p<0.05), but not plasma renin or aldosterone concentrations. During the 2.94±0.84 years of follow-up, 32 participants developed incident hypertension. Retroperitoneal fat area (hazard ration (HR) 1.62, p = 0.003) and peritoneal fat area (HR 1.62, p = 0.009), but not subcutaneous fat area (p = 0.14) were associated with incident hypertension. Neither retroperitoneal fat area, peritoneal fat area, nor subcutaneous fat areas was associated with incident diabetes after adjustment. Conclusions Retroperitoneal fat is similar to peritoneal fat, but differs from subcutaneous fat, in terms of its relationship with metabolic syndrome and incident hypertension. Retroperitoneal fat area should be included in the measurement of visceral fat for cardio-metabolic studies in human. PMID:25401949

  7. Successful enucleation of retroperitoneal cyst.

    PubMed

    Singal, Rikki; Gupta, Samita; Singh, Bir

    2012-01-01

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

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

    PubMed

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

    2011-01-01

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

  9. Imaging of uncommon retroperitoneal masses.

    PubMed

    Rajiah, Prabhakar; Sinha, Rakesh; Cuevas, Carlos; Dubinsky, Theodore J; Bush, William H; Kolokythas, Orpheus

    2011-01-01

    Retroperitoneal masses not arising from major solid organs are uncommon. Although there is no simple method of classifying retroperitoneal masses, a reasonable approach is to consider the masses as predominantly solid or cystic and to subdivide these into neoplastic and nonneoplastic masses. Because the treatment options vary, it is useful to be able to differentiate these masses by using imaging criteria. Although the differential diagnosis of retroperitoneal masses can be narrowed down to a certain extent on the basis of imaging characteristics, patterns of involvement, and demographics, there is still a considerable overlap of imaging findings for these masses, and histologic examination is often required for definitive diagnosis. Computed tomography (CT) and magnetic resonance (MR) imaging play an important role in characterization and in the assessment of the extent of the disease and involvement of adjacent and distant structures. Familiarity with the CT and MR imaging features of various retroperitoneal masses will facilitate accurate diagnosis and staging for aggressive lesions.

  10. Primary retroperitoneal pelvic echinococcal cyst.

    PubMed

    Kalogeris, K G; Christoforidis, L I; Milioudis, N M

    1986-06-01

    We report a case of a single primary retroperitoneal echinococcal cyst located in the pelvis. There were no other hydatid cysts in any other organ. The cyst was excised and serological tests returned to normal.

  11. Primary retroperitoneal tumours and cysts.

    PubMed

    Bors, G; Polyák, L; Frang, D

    1986-01-01

    The authors give a summarizing report on retroperitoneal tumours and cysts. They review the origin and classification of tumours and cysts, their diagnostic and differential diagnostic possibilities as well as the therapeutic measures. Finally, their own 3 cases are reported.

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

    PubMed Central

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

    2016-01-01

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

  13. [Retroperitoneal cystic mesothelioma and lymphangioma].

    PubMed

    Segura Martín, M; Lorenzo Romero, J G; Hernández Millán, I; Pastor Guzmán, J M; Salinas Sánchez, A S; Ruiz Mondéjar, R; Virseda Rodríguez, J A

    1998-03-01

    Retroperitoneal cysts are uncommon entities of difficult diagnosis because of their insidious symptomatology. Urinary apparatus involvement is quite often the mode of presentation and the reason for calling on the urologist. The origin of many of these retroperitoneal cysts remains practically unknown. Surgery with exeresis is the choice management method. Follow-up is necessary for cystic mesothelioma because of the highly frequent relapses. The outlook of hormonal conservative therapy for relapses appears as a future alternative to treatment.

  14. A retroperitoneal bronchogenic cyst.

    PubMed

    Brient, C; Muller, C; Cassagneau, P; Taieb, D; Sebag, F; Henry, J-F

    2012-10-01

    Bronchogenic cysts are benign congenital malformations usually. A retroperitoneal location is extremely uncommon. We reported a case of bronchogenic cyst occurred in the retroperitoneum in a 60-year-old patient. An abdominal CT for a prostatic adenocarcinoma staging has reported this incidental lesion. Biological, radiological and histological assessment confirmed the bronchogenic cyst diagnosis. The treatment of choice for most authors is surgical excision. However, because of a low malignant transformation risk, we have chosen a follow-up with abdominal computerized tomography every 6 months. During 3 years, biological and radiological assessments have shown a stable lesion. Therefore, it seems reasonable to propose a simple radiological monitoring for bronchogenic cysts in selected patients.

  15. [Giant retroperitoneal hydatid cyst].

    PubMed

    Massoud, W; Saheb, N; Iliescu, B; Kreitmann, L; Chabenne, J; Campeggi, A; Molinie, V; Baumert, H

    2009-06-01

    Hydatidosis is an endemic, widely distributed anthropozoonosis, which involves the liver, lung and other organs [Int J Urol 13 (2006) 76-9]. We reported the case of a large retroperitoneal hydatid cyst, which is a rare situation [Hepatogastroenterology 48 (2001) 1037-9; Int Urol Nephrol 32 (2000) 41-6; J Urol (Paris) 94 (1988) 445-8]. Diagnosis was suspected with blood tests and radiological examinations. A wide incision in the right iliac fossa (such in renal graft) was performed. The cyst wall was excised partially. Before, during and following the operation, the patient was given albendazole tablets (15mg/kg per day) for 3 weeks (1 week before and 2 weeks after the surgery) with blood count and liver enzyme monitorization. Especially in the endemic areas, hydatid cyst should be remembered when evaluating cystic masses in the retroperitoneum. It can be treated successfully with surgery.

  16. [Retroperitoneal bronchogenic cyst: Report of one case].

    PubMed

    Orellana, Franco; Cárdenas, Rubén; Manríquez, María Eugenia; Ríos, Horacio; Suárez, Leopoldo; Videla, Domingo

    2007-07-01

    Retroperitoneal cystic tumors are uncommon. More than two thirds are malignant. Benign lesions include lipomas and leiomyomas, among others. Bronchogenic cysts are congenital anomalies that result from an abnormal budding of the primitive foregut, and are most commonly found in the mediastinum. Occasionally they can be seen in the skin, subcutaneous tissue, in the pre-sternal and pericardial areas, but those located under the diaphragm are extremely rare. We report a 24 year-old female who consulted for abdominal pain. An abdominal ultrasound disclosed a retroperitoneal cystic lesion, that was confirmed with a CAT scan. The patient was operated, finding a cyst that was adhered to the pancreas. A distal pancreatectomy and cyst excision were done. The pathological examination of the lesion showed a bronchogenic cyst. The patient was discharged ten days after surgery.

  17. Sarcoma Foundation of America

    MedlinePlus

    ... Make a Donation Matching Gifts Sarcoma Dedication Page Stocks and Securities Workplace Giving Year-End Giving FAQ's ... Make a Donation Matching Gifts Sarcoma Dedication Page Stocks and Securities Workplace Giving Year-End Giving

  18. Endoscopic Retroperitoneal Adrenalectomy for Adrenal Metastases

    PubMed Central

    Simutis, Gintaras; Lengvenis, Givi; Beiša, Virgilijus; Strupas, Kęstutis

    2014-01-01

    Objectives. To evaluate whether retroperitoneal approach for adrenalectomy is a safe and effective treatment for adrenal metastases (AM). Methods. From June 2004 to January 2014, nine consecutive patients with AM were treated with endoscopic retroperitoneal adrenalectomy (ERA). A retrospective study was conducted, and clinical data, tumor characteristics, and oncologic outcomes were acquired and analyzed. Results. Renal cancer was the primary site of malignancy in 44.4% of cases. The mean operative time was 132 ± 10.4 min. There were 5 synchronous and 4 metachronous AM. One patient required conversion to transperitoneal laparoscopic procedure. No mortality or perioperative complications were observed. The median overall survival was 11 months (range: 2–42 months). Survival rates of 50% and 25% were identified at 1 and 3 years, respectively. At the end of the study, 4 patients were alive with a mean observed follow-up of 20 months. No patients presented with local tumor relapse or port-site metastases. Conclusions. This study shows that ERA is a safe and effective procedure for resection of AM and advances the surgical treatment of adrenal disease. The use of the retroperitoneal approach for adrenal tumors less than 6 cm can provide very favorable surgical outcomes. PMID:25276132

  19. A retroperitoneal bronchogenic cyst: laparoscopic treatment.

    PubMed

    Ishizuka, O; Misawa, K; Nakazawa, M; Nishizawa, O

    2004-01-01

    Subdiaphragmatic bronchogenic cysts are rare, and those located retroperitoneally are exceptional. We describe a retroperitoneal cyst presenting as an asymptomatic adrenal mass which was treated with laparoscopic surgery with three trocars.

  20. Symptomatic retroperitoneal cyst: a diagnostic challenge.

    PubMed

    Renzulli, Pietro; Candinas, Daniel

    2009-03-01

    Retroperitoneal cystic masses pose an important diagnostic and therapeutic challenge. Simple drainage, internal or external, is usually not sufficient. We report a case of a large symptomatic retroperitoneal cyst and its management.

  1. Computed tomography of pseudocysts in retroperitoneal fibrosis.

    PubMed

    Feldberg, M A; van Waes, P F

    1987-01-01

    Two cases of pseudocysts associated with retroperitoneal fibrosis and ureteral obstruction are described. Chemical analysis in the first case identified the retroperitoneal cyst as chylous in origin. In the second case the cystic area within the retroperitoneal fibrosis disappeared following treatment with steroids.

  2. 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

  3. Retroperitoneal, mesenteric, and omental cysts.

    PubMed

    Vanek, V W; Phillips, A K

    1984-07-01

    Retroperitoneal, mesenteric, and omental cysts are rare abdominal tumors occurring in approximately one of 105,000 hospitalized patients. These cysts have a similar pathogenesis that primarily may be ectopic lymphatic tissue. Retroperitoneal and mesenteric cysts can occur anywhere in the area between the duodenum and rectum but are most common in the small-bowel mesentery, especially the ileum. They can appear as chronic abdominal pain, a painless abdominal mass, or acute abdomen. The most common physical finding of a retroperitoneal or mesenteric cyst is a compressible abdominal mass, movable transversely but not longitudinally; omental cysts are freely movable. Diagnostic aids include abdominal computed tomography and ultrasound. The upper gastro-intestinal (GI) tract series, barium enema examination, and intravenous pyelogram exclude GI and genitourinary cysts and tumors. Treatment of choice is enucleation; resection of the adjacent bowel may occasionally be necessary. Morbidity and mortality should be very low because of modern surgical techniques and follow-up procedures.

  4. Mast cell sarcoma: clinical management.

    PubMed

    Weiler, Catherine R; Butterfield, Joseph

    2014-05-01

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

  5. Retroperitoneal cysts: a case report.

    PubMed

    Pace, Gianna; Galatioto Paradiso, Giuseppe; Galassi, Paolo; Vicentini, Carlo

    2006-03-01

    Retroperitoneal cysts are rare lesions, variable from asymptomatic cases with incidental discovery to case with acute or chronic abdominal discomfort. A 50-year-old female after a car crash refered chronic abdominal pain. An X-ray revealed the presence of sternal and multiple costal fractures. Abdominal ultrasonography (US) and computed tomography (CT) lead to the discovery of a retroperitoneal cyst too. As the patient was well after fractures solution, we decided to control the cyst in the time. In symptomatic cases surgery is the treatment of choice. The advent of laparoscopic surgery allows resection of these cysts to be achieved without full laparotomy.

  6. 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

  7. Laparoscopic management of large retroperitoneal lymphoceles complicating aortic surgery.

    PubMed

    Blessios, G A; Rokkas, C K; Neulander, O; Panoussopoulos, D

    2002-01-01

    Retroperitoneal lymphocele is a rare but debilitating complication of aortic replacement with synthetic graft. The only effective treatment reported to date is surgical reexploration and ligation of leaking lymphatics. This report illustrates the successful management of two patients with large retroperitoneal lymphoceles formed after aortic surgery using laparoscopic techniques. The available literature is reviewed. Laparoscopic fenestration of the lymphocele and laparoscopically assisted ligation of the leaking lymphatics combined with internal drainage resulted in long-term relief of compression symptoms, as observed, respectively, over the 5-year and 3-month follow-up periods. Percutaneous catheter drainage before laparoscopic management was unsuccessful in both cases. In addition, the unique presentation of a large retroperitoneal lymphocele with intestinal obstruction is reported, and currently available treatment options are discussed.

  8. 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 ...

  9. Combined mediastinal and retroperitoneal fibrosis

    PubMed Central

    Salmon, H. W.

    1968-01-01

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

  10. Retroperitoneal malignant cyst. Case report.

    PubMed

    Melén, K; Sandermann, J; Stubberöd, A; Boiesen, P

    1991-10-01

    Forty years after removal of a benign mucinous cyst from the left retroperitoneal space, a malignant cyst with both sarcomatous and carcinomatous components was removed from the same site in a 75-year-old woman. Within 6 months the lesion recurred as a carcinoma penetrating the left colon and the abdominal wall.

  11. Retroperitoneal lymphangioma. A case report.

    PubMed

    Wang, H H; Li, S G; Chang, S Y; Ma, C P

    1989-06-01

    Retroperitoneal cystic lymphangiomas are rare benign tumours. Ultrasonography, computed tomography, lymphography or fine-needle percutaneous aspiration may be used to make the diagnosis pre-operatively. Complete excision of the cyst without rupture is the preferred treatment. However, in complicated cases a conservative surgical approach is mandatory.

  12. 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

  13. [Retroperitoneal hematoma secondary to traumatic rupture of the psoas muscle].

    PubMed

    Arrizabalaga, M; Benítez, J; Gallardo, C; Garrido, R; Casanueva, T

    1990-01-01

    Rupturing of the psoas muscle by closed injury as a result of the formation of a retroperitoneal hematoma is a very uncommon pathological entity, which may rise problems of differential diagnosis with kidney lesions. Supplementary explorations do not always clear up diagnostic doubts, and it is surgery, if indicated, that confirms the picture. We present a case of retroperitoneal hematoma of traumatic origin in a one-kidney ipsilateral patient, in whom the abdominal CAT revealed affection of the renal capsule associated with rupture of teh psoas. In the surgery carried out due to formation of an abscess in the hematoma we verified that the kidney was undamaged.

  14. Retroperitoneal perirenal pseudocyst in a Massese breed ram.

    PubMed

    Mutinelli, Franco; Vascellari, Marta; Schiavon, Eliana

    2005-05-01

    The macroscopic and microscopic features of a retroperitoneal perirenal pseudocyst in a 12-month-old ram without impairment of renal function are described. In humans and animals, uriniferous pseudocysts may be of traumatic origin, resulting from rupture of kidney, renal pelvis, or ureter, or congenital. Lymphatic pseudocysts may develop secondary to inflammatory obstruction of the hilar lymphatics after perinephritis or renal transplantation. In this case, histologic characteristics of the pseudocyst wall were suggestive of development from the parietal peritoneal layer encapsulating the kidney. This is the first case of retroperitoneal perirenal pseudocyst in a sheep.

  15. 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

  16. 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.

  17. Large retroperitoneal cysts in children and adolescents.

    PubMed

    McClellan, D S; Brasch, J; Rifkin, H

    1981-12-01

    Retroperitoneal cysts may be divided into those of urogenital origin: pronephric, mesonephric, metanephric and müllerian; mesocolic; teratoma; lymphatic; parasitic, and traumatic blood cysts. Müllerian cysts are thought to originate from the specialized mesothelial cells of the genital ridge and present as fimbrial cysts or broad ligament wolffian cysts. Symptoms may be absent, or the result of pressure or displacement of an organ. Diagnosis is made by x-ray studies, sonographic evaluation and, frequently, at operation. We report a case of a 14-pound cyst arising from the right broad ligament and partially obstructing the right ureter that was removed surgically.

  18. Spontaneous retroperitoneal hemorrhage from a ruptured hypernephroma.

    PubMed

    Trivedi, R; Levy, M; Ibrahim, I; Bonacarti, A

    1979-01-01

    The clinical presentation and roentgenographic findings of renal cell carcinoma have been consistently variable. These patients can appear with flank pain mimicking ureteral colic, flank tumors, or symptomatic metastasis [1]. Systemic cardiac manifestations including cardiomegaly with congestive heart failure due to arteriovenous fistula formation have been reported [2] Roentgenographic findings may show the tumor to be either vascular or avascular. It may present as a spontaneous perforation of the pelvic ureteral system which is demonstrated by intravenous pyelography (3). In this article, we describe a case of hypernephroma in a cyst wall causing severe spontaneous hemorrhage in the retroperitoneal space resulting in a state of hypovolemic shock.

  19. 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

  20. 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.

  1. Identification of HHV8 in early Kaposi's sarcoma: implications for Kaposi's sarcoma pathogenesis.

    PubMed Central

    Kennedy, M M; Cooper, K; Howells, D D; Picton, S; Biddolph, S; Lucas, S B; McGee, J O; O'Leary, J J

    1998-01-01

    AIMS: Kaposi's sarcoma is a vascular tumour of uncertain pathogenesis possibly caused by an infectious agent, identified in high risk groups. Accumulating solution phase polymerase chain reaction (PCR) and seroepidemiological data suggest that a previously undescribed herpes DNA virus (human herpesvirus 8 (HHV8)) is the causative agent. Using a unique cohort of early Kaposi's sarcoma, the precise cell type infected with HHV8 in such lesions was identified to elucidate further the role of HHV8 in the pathobiology of Kaposi's sarcoma. METHODS: Sixteen cases of early Kaposi's sarcoma (derived from skin and lymph node) were assessed for the presence of HHV8 using both standard solution phase PCR and TaqMan PCR to the KS330 Bam region of HHV8. In situ amplification was also performed on a selected group in an attempt to identify the candidate infected cells. RESULTS: Using both conventional solution phase and TaqMan PCR, 87% of cases were positive. In addition, HHV8 amplicons were localised in situ to endothelial and spindle cell proliferations in early Kaposi's sarcoma. The HHV8 viral load varied from lesion to lesion. CONCLUSIONS: The presence of HHV8 in early lesions supports a role for HHV8 in the pathogenesis of Kaposi's sarcoma. Coupled with recent seroepidemiological studies, these results suggest that HHV8 is the aetiological agent of Kaposi's sarcoma. Its precise interaction with other factors known to be involved in the development of Kaposi's sarcoma, including cytokines and anti-apoptosis genes, requires elucidation. PMID:9624414

  2. 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

  3. Giant Retroperitoneal Lipoma in an Infant

    PubMed Central

    2014-01-01

    Lipomas can occur almost anywhere in the body, but retroperitoneal lipomas are extremely rare. They are slowly growing benign tumors and can attain an enormous size due to silent course of the disease. Total excision of the mass is the treatment of choice and is curative for benign retroperitoneal lipomas. We treated an 11-month-old female patient with giant retroperitoneal lipomas by surgical excision. Histopathology confirmed it as fibrolipoma. PMID:25374800

  4. Retroperitoneal cystic lymphangioma: a diagnostic and surgical challenge.

    PubMed

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

    2013-01-01

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

  5. Sonographic features of retroperitoneal neurilemoma.

    PubMed

    Kuo, C H; Changchien, C S

    1993-06-01

    Seven cases of retroperitoneal neurilemoma confirmed by histology had an ultrasound examination. There were 5 women and 2 men with a mean age of 55. Most of the cases (5/7) had a palpable mass clinically. Of the 7 cases, 4 had a mass with cystic degeneration and internal echoes on sonogram, consistent with the macroscopic findings of tumors. The internal echoes represented blood clots and residual tissue (or septa) in cyst. Of the remaining 3 cases, one showed a confluent lobulated mass, the second had a solitary homogeneous mass with small cysts, and the third one, which was the smallest (4 cm x 4 cm x 6 cm), had a hypoechoic and homogeneous pattern. The sonograms of all 7 cases were consistent with the macroscopic features of the tumors. The sonographic pattern of tumor parenchyma is either homogenously hyperechoic or hypoechoic. Because of the cystic degeneration, hemorrhage, and residual tissue of retroperitoneal neurilemoma, the sonographic pattern was variable.

  6. Intra-retroperitoneal duplication cyst.

    PubMed

    Ma, Juine-Yih; Lin, Yu-Cheng; Tseng, Sheng-Hong; Lai, Tsung-Hsein; Chen, Yun

    2004-11-01

    Duplication cyst occupying the retro- and intra-peritoneal space is a rare condition. We describe a case of duplication cyst in a 13-year-old girl who presented with abdominal pain, vomiting, and a lower abdominal mass. Plain abdominal X-ray films revealed local ileus over the lower abdomen. Ultrasonography revealed 2 double-layered cystic masses over the lower abdomen with a suspicious communicating tract. Mild hydropelvis of the right kidney was also noted. Abdominal computed tomography revealed 2 cystic lesions. One was located at the pelvic cavity just above the urinary bladder and the other was in the left retroperitoneal space. Laparotomy revealed a dumbbell-shaped intra-retroperitoneal duplication cyst with a small communicating tract. The cyst was excised without disturbing bowel continuity and the vascular supply. The patient was doing well at 1-year follow-up.

  7. Retroperitoneal bronchogenic cyst: MRI findings.

    PubMed

    Castro, R; Oliveira, M I; Fernandes, T; Madureira, A J

    2013-01-01

    The authors describe a case of a retroperitoneal bronchogenic cyst in a 36-year-old female. She presented with abdominal pain, nausea, and vomiting. An MRI scan revealed an 8 cm cystic lesion in the left upper retroperitoneum, with intermediate signal on T2-weighted images, high signal on T1 weighted images, and lack of internal enhancement after gadolinium. After laparoscopic excision, the histology findings were compatible with a bronchogenic cyst, which is extremely uncommon in the retroperitoneum.

  8. Ultrasound findings in retroperitoneal lymphangiomyomatosis.

    PubMed

    Di Matteo, A M; Luisetti, M; Brunetti, E; Filice, C

    1990-01-01

    A 44-year old female patient with pulmonary lymphangiomyomatosis was examined by standard abdominal sonography. A wide involvement of the retroperitoneal lymph nodes and vessels was found. The relevant sonographic features were enlarged lymph nodes, and multiple anechoic structures, irregular in shape, thin-walled, with intraluminal septa. The findings, which resembled active cysts or liquid-filled bowels, were diagnosed as multiple ectasic lymphatic vessels.

  9. 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

  10. 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.

  11. Retroperitoneal lymphangioleiomyomatosis associated with endosalpingiosis.

    PubMed

    Fukunaga, Masaharu; Mistuda, Aki; Shibuya, Kazuhiro; Honda, Yoshiko; Shimada, Nagato; Koike, Junichi; Sugimoto, Motonobu

    2007-12-01

    A case of retroperitoneal lymphangioleiomyomatosis (LAM) arising from endosalpingiosis is described. A 25-year-old woman with no history of tuberous sclerosis or hormonal therapy presented with a painless, palpable abdominal mass. Computed tomographic and magnetic resonance imaging studies of the abdomen demonstrated a 4 cm cystic mass in the retroperitoneum. Macroscopically, the excised retroperitoneal cyst was multilocular and measured 4.0 x 3.5 x 3.5 cm. Histologically, the lesion demonstrated three components. The first comprised multiple cysts or glands lined by columnar epithelial cells with cilia. The second component was a condensation of small stromal cells immediately subjacent to the cystic epithelium or glands. The third component was a thick exterior wall composed of plump spindle cells with clear to palely eosinophilic cytoplasm in a fascicular pattern, and slit-like vascular spaces, resembling LAM. Immunohistochemically, the epithelium and glands were positive for cytokeratin 7. The stromal cells were positive for vimentin and CD10. The cells of the LMA-like component showed positive staining for HMB45, alpha-smooth muscle actin, muscle actin and h-caldesmon. The lesion, LAM arising from endosalpingiosis, represents a distinctive pathologic entity that should be recognized and studied further. This type of lesion should be included in the differential diagnosis of retroperitoneal cystic lesions.

  12. CT evaluation of primary benign retroperitoneal tumor.

    PubMed

    Hayasaka, K; Yamada, T; Saitoh, Y; Yoshikawa, D; Aburano, T; Hashimoto, H; Yachiku, S

    1994-01-01

    We studied CT patterns in 21 cases of primary benign retroperitoneal tumor including teratoma in nine cases, schwannoma in six, leiomyoma in three, lipoma in one, lymphangioma in one, and neurofibroma in one. The tumors were analyzed for size, internal homogeneity, CT density, calcification, border with neighboring organs, and contrast enhancement (CE). The mean diameter of the tumors was 10.2 (+/- 4.8) cm. Internal homogeneous distribution was observed in 33%, calcification in 43%, and well-defined border in 86%. The CT density and calcification were compared according to histology, and the results were as follows: teratoma showed fat density in 78%, water density in 100%, and calcification in 89%; schwannoma showed water density in 100% and septal CE in 33%; leiomyoma showed soft tissue density in 100%, CE in 100%, and water density in 33%; lipoma showed fat density and calcification; and lymphangioma and neurofibroma showed water density. Internal homogeneity, fat density, cyst formation, and calcification are considered to be important predictors of primary benign retroperitoneal tumor on CT.

  13. Trial of Dasatinib in Advanced Sarcomas

    ClinicalTrials.gov

    2016-10-12

    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)

  14. [Magnetic resonance imaging in the diagnosis of knee joint sarcomas].

    PubMed

    Shubkin, V N; Gunicheva, N V; Akhadov, T A; Puzhitskiĭ, L B; Keshishian, R A

    2007-01-01

    The purpose of the investigation was to study the potentialities of magnetic resonance imaging (MRI) in the diagnosis of knee joint sarcomas. The paper presents the results of examining 13 patients of different age, shows the potentialities of the technique in the identification of knee joint sarcomas, and describes the MRI semiotics of sarcomas in both the routine study and that using contrast enhancement in lesions of bone and soft tissue elements in the presence of regional metastases.

  15. Management of Breast Sarcoma.

    PubMed

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

    2016-10-01

    Breast sarcomas are exceptionally rare mesenchymal neoplasms composed of many histologic subtypes. Therapy is guided by principles established in the management of extremity sarcomas. The anatomic site does influence treatment decisions, particularly the surgical management. Surgery should be undertaken with the aim of achieving a widely negative margin. Selected patients can be managed with breast-conserving surgery. Breast reconstruction is increasingly being undertaken for selected patients. Radiation therapy and chemotherapy are used selectively for large, high-grade sarcomas for which there is significant concern for local and distant recurrence. PMID:27542642

  16. Retroperitoneal Cyst: An Uncommon Presentation of Filariasis.

    PubMed

    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.

  17. Retroperitoneal bronchogenic cyst: CT and MR imaging.

    PubMed

    Murakami, R; Machida, M; Kobayashi, Y; Ogura, J; Ichikawa, T; Kumazaki, T

    2000-01-01

    Retroperitoneal bronchogenic cysts are extremely rare congenital anomalies that represent malformations of the embryonic foregut and are morphologically expressed as maldevelopments of the respiratory system. Because of the low prevalence of these tumors, their imaging features have seldom been described. We present the computed tomographic and magnetic resonance imaging findings of a case of retroperitoneal bronchogenic cyst.

  18. Thymidine phosphorylase expression in Kaposi sarcoma.

    PubMed Central

    Dada, M A; Boshoff, C H; Comley, M A; Turley, H; Schneider, J W; Chetty, R; Gatter, K C

    1996-01-01

    AIMS: To examine the immunohistochemical distribution of thymidine phosphorylase (TP) in all clinicopathological subtypes of Kaposi sarcoma. METHODS: Thirty two biopsy specimens of Kaposi sarcoma (29 patients) were studied. Six of these patients represented classic, six endemic, eight HIV associated, seven post-immunosuppression/transplant related, and two unclassified variants of Kaposi sarcoma. The average age was 49 years (range 22-83 years) and the male: female ratio 24:5. Four samples of angiosarcoma and one of spindle cell haemangio-endothelioma were stained in parallel. All specimens were fixed in formalin, embedded in paraffin wax and processed routinely. Immunohistochemistry was carried out using an antibody directed against CD31 (JC70) and the monoclonal antibody P-GF.44C against TP. RESULTS: All biopsy specimens showed immunoexpression for TP. The spindle cell component stained more strongly than newly formed endothelium lined vessels and normal, resident vessels at a distance from the lesions. CONCLUSIONS: The strong immunoexpression of TP suggests up-regulation of TP and a role for TP in angiogensis in Kaposi sarcoma. The mechanism for the up-regulation of TP remains unknown, but viral infections may trigger it. The differential staining of the various cell components of Kaposi sarcoma also suggest that TP either plays a role in the differentiation and maturation of Kaposi sarcoma or is a reflection of such changes. Images PMID:8707955

  19. DNA-binding and transcriptional activation properties of the EWS-FLI-1 fusion protein resulting from the t(11;22) translocation in Ewing sarcoma.

    PubMed Central

    Bailly, R A; Bosselut, R; Zucman, J; Cormier, F; Delattre, O; Roussel, M; Thomas, G; Ghysdael, J

    1994-01-01

    The 5' half of the EWS gene has recently been described to be fused to the 3' regions of genes encoding the DNA-binding domain of several transcriptional regulators, including ATF1, FLI-1, and ERG, in several human tumors. The most frequent occurrence of this situation results from the t(11;22)(q24;q12) chromosome translocation specific for Ewing sarcoma (ES) and related tumors which joins EWS sequences to the 3' half of FLI-1, which encodes a member of the Ets family of transcriptional regulators. We show here that this chimeric gene encodes an EWS-FLI-1 nuclear protein which binds DNA with the same sequence specificity as the wild-type parental FLI-1 protein. We further show that EWS-FLI-1 is an efficient sequence-specific transcriptional activator of model promoters containing FLI-1 (Ets)-binding sites, a property which is strictly dependent on the presence of its EWS domain. Comparison of the properties of the N-terminal activation domain of FLI-1 to those of the EWS domain of the fusion protein indicates that EWS-FLI-1 has altered transcriptional activation properties compared with FLI-1. These results suggest that EWS-FLI-1 contributes to the transformed phenotype of ES tumor cells by inducing the deregulated and/or unscheduled activation of genes normally responsive to FLI-1 or to other close members of the Ets family. ES and related tumors are characterized by an elevated level of c-myc expression. We show that EWS-FLI-1 is a transactivator of the c-myc promoter, suggesting that upregulation of c-myc expression is under control of EWS-FLI-1. Images PMID:8164678

  20. Retroperitoneal fat necrosis producing ureteral obstruction.

    PubMed

    Ross, J S; Prout, G R

    1976-05-01

    We present 3 cases of a rare syndrome of extrinsic ureteral obstruction produced by retroperitoneal fat necrosis and compare the medical records and histopathology to 19 cases of classic idiopathic retroperitoneal fibrosis. Clinical similarities include average age of onset in the fourth and fifth decades, male preponderance, abdominal or back pain as the presenting symptom, elevation of the blood urea concentration prior to diagnosis and tendency toward bilateral involvement. Histologically, the 2 conditions were different. Retroperitoneal fat necrosis is characterized by coalescence of fat cells into fat cysts bordered by foreign body giant cell granulomas, foam cells, light chronic inflammatory infiltrate and unimpressive fibrosis. The microscopic pathology of idiopathic retroperitoneal fibrosis is dominated by densely collagenized fibrous tissue of varying cellularity without evidence of fat necrosis. Of the 3 cases of retroperitoneal fat necrosis 2 featured prior ischiorectal abscesses and this is considered in a discussion of the etiology of this unusual cause of ureteral compression.

  1. Retroperitoneal bronchogenic cyst mimicking pancreatic cystic lesion.

    PubMed

    Wang, Shin-E; Tsai, Yi-Fang; Su, Cheng-Hsi; Shyr, Yi-Ming; Lee, Rheun-Chuan; Tsai, Wan-Chen; Li, Fen-Yau; Chen, Tien-Hua; Wu, Chew-Wun; Lui, Wing-Yiu

    2006-11-01

    Retroperitoneal bronchogenic cyst is detected extremely rarely and often masquerades as other diseases. Here, we report 2 cases of retroperitoneal bronchogenic cyst mimicking pancreatic mucinous tumor. Histologically, both cysts were composed of ciliated respiratory-like epithelium with abundant mucin content, smooth muscle bundles and mature cartilage, compatible with the diagnosis of retroperitoneal bronchogenic cyst. In addition to these 2 cases, another 42 retroperitoneal bronchogenic cysts reported in the English literature were collected for review and analysis. Twelve (28%) were located over the peripancreatic area. Just over half (51%) of them were asymptomatic. No accurate preoperative diagnosis could be made for any of the lesions. About a third (33.3%) of the peripancreatic retroperitoneal bronchogenic cysts masqueraded as pancreatic cystic lesions.

  2. Current Immunotherapies for Sarcoma: Clinical Trials and Rationale

    PubMed Central

    Mitsis, Demytra; Francescutti, Valerie

    2016-01-01

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

  3. Results of a phase I dose escalation study of eltrombopag in patients with advanced soft tissue sarcoma receiving doxorubicin and ifosfamide

    PubMed Central

    2013-01-01

    Background The objective of this Phase I dose escalation study was to explore the safety and tolerability of eltrombopag, an oral, nonpeptide, thrombopoietin receptor agonist, in patients with advanced soft tissue sarcoma (STS) and thrombocytopenia due to treatment with doxorubicin and ifosfamide (AI) combination chemotherapy. Methods Patients aged 18 or older with histologically confirmed, locally advanced or metastatic STS were treated with 1 cycle of AI followed by AI with eltrombopag starting at Cycle 2, using 2 different dosing schedules. The study design included an eltrombopag dose escalation phase starting at 75 mg daily to determine the optimal biological dose (OBD). Results Eighteen patients were enrolled and 15 received at least 1 dose of chemotherapy; 3 patients withdrew prior to receiving eltrombopag. Seven, 4, and 1 patients received 75 mg, 100 mg, and 150 mg eltrombopag daily, respectively. No dose-limiting toxicities were reported. Due to slow recruitment, the study was closed prior to identifying an OBD. The most common hematologic adverse events (AEs) were thrombocytopenia (80%), neutropenia (73%), and anemia (67%). The most common nonhematologic AEs were fatigue (53%), alanine aminotransferase increased, constipation, and nausea (47% each). Eleven of 12 patients who received eltrombopag completed at least 2 chemotherapy cycles; all had increased platelet counts on Day 1 of Cycle 2 (cycle with eltrombopag) compared to Day 1 of Cycle 1 (cycle without eltrombopag). Conclusions Although data are limited, safety data were consistent with the known toxicities of AI combination chemotherapy or the side effect profile of eltrombopag seen in other studies. Available data suggest a potential pre- and post-chemotherapy dosing scheme for eltrombopag when administered with AI chemotherapy, and support further investigation of eltrombopag treatment in patients with chemotherapy-induced thrombocytopenia. PMID:23497336

  4. 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 ...

  5. 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 ...

  6. 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 ...

  7. Primary retroperitoneal mucinous tumors: a clinicopathologic study of 18 cases.

    PubMed

    Roma, Andres Anibal; Malpica, Anais

    2009-04-01

    Primary retroperitoneal mucinous tumors (PRMTs) are uncommon neoplasms occurring almost exclusively in women. PRMTs are divided into mucinous cystadenoma (MC), mucinous borderline tumors or tumors of low malignant potential (MLMP), and mucinous carcinomas (MCas). In this retrospective study, we present the clinicopathologic features of 18 such cases, the largest series to date. All patients were women, ranging in age from 20 to 63 years (mean 38.6 y). All except 2 patients presented with an enlarged mass during a routine examination or by self-palpation. All tumors were located exclusively in the retroperitoneum, with histologic or clinical confirmation of the lack of ovarian involvement. The tumors ranged from 7 to 26 cm (mean 13.2 cm). The gross appearance was variable: unilocular cyst with a thin wall (4 cases), predominantly cystic with papillary areas or nodule(s) (8 cases), multiloculated cyst with or without nodules (1 case each), and predominantly solid with cystic areas (4 cases). Histologically, there were 2 cases of MC, 7 of MLMP (7 cases; 3 of them with intraepithelial carcinoma and 1 with microinvasion), and 9 of MCas (9 cases, 5 of them associated with MLMP and 1 associated with MC). Three of the MCas had areas of anaplastic or sarcomatoid carcinoma whereas 1 had an associated sarcoma. Immunohistochemical studies were performed in 6 cases. Cytokeratin 7 was diffusely positive in all cases studied, whereas cytokeratin 20 and cytokeratin 17 were focally positive in 4 and 2 cases, respectively. All patients underwent surgical resection of the entire tumor. Two patients with MCa and sarcoma or sarcomatoid carcinoma received chemotherapy. Follow-up was available in 16 cases, ranging from 1 to 148 months (mean 40 mo, median 22 mo). Two patients died of disease at 5 and 9 months; both had MCa with anaplastic carcinoma or sarcoma. Three patients with MCa were alive with disease at 14, 26, and 58 months. The remaining patients were alive with no evidence of

  8. 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.

  9. [Retroperitoneal solitary fibrous tumor: a case report].

    PubMed

    Nukui, Akinori; Ochi, Masanori; Suzuki, Kazumi; Yuzawa, Masayuki; Morita, Tatsuo

    2009-08-01

    A 63-year-old man with a retroperitoneal tumor found incidentally was referred to our hospital. Computed tomography showed a tumor ventrally adjacent to urinary bladder and prostate. Pathological examination of retroperitoneal tumor specimens obtained by percutaneous needle biopsy revealed hypercellularity of spindle cells positive for CD 34. Under the suspicion of solitary fibrous tumor (SFT) or stromal tumors of uncertain malignant potential (STUMP), we performed en bloc resection of tumor, urinary bladder and prostate because tumor was firmly fixed to urinary bladder and prostate. The final diagnosis of retroperitoneal tumor was SFT because pathological findings of the surgical specimen were the same as those of the biopsy specimens.

  10. Benign retroperitoneal cyst of Mullerian type.

    PubMed

    Yohendran, Jay; Dias, Maxwell M; Eckstein, Robert; Wilson, Tim

    2004-10-01

    Retroperitoneal cysts are rare, usually asymptomatic, lesions. We present the case of a 42-year-old woman with an asymptomatic abdominal mass. The mass excised at our district hospital was found in the retroperitoneum. Histologically, the cyst was lined with benign Mullerian-type epithelium. The classification, aetiology and clinical manifestations of retroperitoneal cysts are discussed. Retroperitoneal cysts present as an acute abdomen in only a minority of cases. Ultrasound is the most reliable test for their detection, usually followed by computed tomography and/or an upper gastrointestinal series. Optimally, these cysts should be completely excised.

  11. [A case report of retroperitoneal malignant schwannoma].

    PubMed

    Okamura, K; Ito, K; Aota, Y; Suzuki, Y; Shimoji, T

    1984-08-01

    A case of retroperitoneal malignant schwannoma is reported. The patient was a 66-years-old man who was referred to our clinic because of an abdominal lump. CT scan and renal arteriography pointed out right renal cyst, but the tumor was separate from the right kidney at operation. Pathological diagnosis of this retroperitoneal tumor was malignant schwannoma. After 10 months he developed local recurrence and had transabdominal excision. He has been doing well 12 months after the second operation. Thirty-one cases of retroperitoneal malignant schwannoma in Japan are reviewed.

  12. Retroperitoneal bronchogenic cyst: a case report.

    PubMed

    Mirsadeghi, Ali; Farrokhi, Farid; Fazli-Shahri, Azadeh; Gholipour, Bahareh

    2014-01-01

    Bronchogenic cysts are among developmental disorders of the primitive foregut which are typically found above the diaphragm. Bronchial cysts discovered in the abdominal cavity or retroperitoneum are extremely rare. We present a rare case of a retroperitoneal bronchogenic cyst which was incidentally detected after a wrestling injury in a 23-year-old man who had a negative medical history. Although initial imaging studies suggested an adrenal tumor, histopathological analysis provided a definite diagnosis of bronchogenic cyst. Though rare, bronchogenic cysts must be considered in the differential diagnosis of retroperitoneal cystic lesions. This is the first case of a retroperitoneal bronchogenic cyst reported in Iran.

  13. 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 ...

  14. 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 ...

  15. 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 ...

  16. Explant culture of sarcoma patients' tissue.

    PubMed

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

    2016-07-01

    Human sarcomas comprise a heterogeneous group of rare tumors that affect soft tissues and bone. Due to the scarcity and heterogeneity of these diseases, patient-derived cells that can be used for preclinical research are limited. In this study, we investigated whether the tissue explant technique can be used to obtain sarcoma cell lines from fresh as well as viable frozen tissue obtained from 8 out of 12 soft tissue and 9 out of 13 bone tumor entities as defined by the World Health Organization. The success rate, defined as the percent of samples that yielded sufficient numbers of outgrowing cells to be frozen, and the time to freeze were determined for a total of 734 sarcoma tissue specimens. In 552 cases (75%) enough cells were obtained to be frozen at early passage. Success rates were higher in bone tumors (82%) compared with soft tissue tumors (68%), and the mean time to freezing was lower in bone tumors (65 days) compared with soft tissue tumors (84 days). Overall, from 40% of the tissues cells could be frozen at early passage within <2 month after tissue removal. Comparable results as with fresh tissue were obtained after explant of viable frozen patient-derived material. In a selected number of bone and soft tissue sarcoma entities, conventional karyotyping and/or FISH (fluorescence in situ hybridization) analysis revealed a high amount (>60%) of abnormal cells in 41% of analyzed samples, especially in bone sarcomas (osteosarcoma and Ewing sarcoma). In conclusion, the explant technique is well suited to establish patient-derived cell lines for a large majority of bone and soft tissue sarcoma entities with adequate speed. This procedure thus opens the possibility for molecular analysis and drug testing for therapeutic decision making even during patient treatment. PMID:27111283

  17. Sarcomas and pharmacogenetics.

    PubMed

    Biason, Paola; Toffoli, Giuseppe

    2005-09-01

    Sarcomas are a heterogeneous group of tumors, requiring different chemotherapeutic approaches. Recently, several regimens for metastatic tumors were evaluated with respect to the different responses to conventional chemotherapy of the various histologic subtypes of sarcomas. The impact of pharmacogenetics in the progress of chemotherapy appears to be crucial in defining the clinical response to many drugs, such as anthracycline or alkylating agents, that are widely used in treatment regimens for soft tissue sarcomas (STS) or sarcomas of the bone. Polymorphisms of metabolizing enzymes (e.g., cytochrome P450 and glutathione-S-transferase), transporter proteins (reduced folate carrier and P-glycoprotein) or target proteins (thymidylate synthase, methylenetetrahydrofolate reductase, dihydrofolate reductase, and c-KIT) may be responsible for an altered clinical outcome, in terms of both response and toxicity. The administration of new chemotherapeutic agents, such as imatinib for gastrointestinal tumors (GIST), requires the study of genetic polymorphisms possibly affecting the integrity of the target (c-KIT), which may provide valid information regarding possible developments of therapy. For STS and sarcoma of the bone, the genetic markers, which could be unambiguously predictive of the phenotypic profile of patients, are as yet undetermined.

  18. Primary pleuropulmonary synovial sarcoma.

    PubMed

    Mirzoyan, Michael; Muslimani, Ala'a; Setrakian, Sebouh; Swedeh, Mohamed; Daw, Hamed A

    2008-09-01

    Pleuropulmonary synovial sarcoma (PPSS) is increasingly recognized as a subtype of sarcoma because of the recent identification of a distinctive chromosomal translocation specific to synovial sarcoma. Soft-tissue synovial sarcoma is far more common than PPSS and typically develops in para-articular locations of the extremities, affects young and middle-aged adults, with no difference in distribution between the sexes, and has well-documented radiologic manifestations. Pleuropulmonary synovial sarcoma can arise in the chest wall, heart, mediastinum, pleura, or lung, and it shares patient demographics and several imaging features with its soft-tissue counterpart. Patients present with a cough, chest pain, or dyspnea. On chest radiographs, PPSS typically appears as a sharply marginated mass with uniform opacity, based in the pleura or in the lung, and often accompanied by an ipsilateral pleural effusion. Computed tomographic images show a well-circumscribed, heterogeneously enhanced lesion without associated involvement of bone and without calcifications (except in the case of a chest wall primary tumor). Magnetic resonance imaging provides superior demonstration of nodular soft tissue and multilocular fluid-filled internal components of PPSS, in addition to peripheral rim enhancement after the intravenous administration of a gadoliniumbased contrast material such as gadopentetate dimeglumine. Current treatment consists of surgical resection followed by chemotherapy, radiation therapy, or both. PMID:18824448

  19. 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

  20. 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

  1. Sonographic appearance of primary retroperitoneal cysts.

    PubMed

    Derchi, L E; Rizzatto, G; Banderali, A; Sala, P; Larghero, G C; Solbiati, L

    1989-07-01

    The sonographic findings observed in four patients with primary retroperitoneal cysts were reviewed. All lesions presented as fluid-filled masses with anechoic content and regular margins; all increased through transmission. Preoperative location of the cysts within the retroperitoneal space was possible in all cases by either real-time ultrasound (US) analysis of the different relationships of the kidneys and the cysts during respiratory movements or the anterior displacement of surrounding structures. A differential diagnosis among retroperitoneal lesions containing fluid is difficult and cannot be obtained with imaging methods alone. However, preoperative knowledge of both the retroperitoneal location of the disease process and its fluid content may help to narrow the diagnostic possibilities, thus allowing the surgeon to more accurately plan the therapeutic approach to the patient.

  2. Retroperitoneal bronchogenic cyst in a young adult.

    PubMed

    Resl, M; Navrátil, P; Krajina, A

    1996-01-01

    Abdominal sonography performed in a 21-year-old man for suspected hepatopathy revealed a pathological lesion within the retroperitoneal space. Morphological examination showed an unusual mode of presentation of an asymptomatic bronchogenic cyst.

  3. Computed tomography of retroperitoneal cystic lymphangiomas.

    PubMed

    Munechika, H; Honda, M; Kushihashi, T; Koizumi, K; Gokan, T

    1987-01-01

    The CT features of retroperitoneal lymphangiomas are unilocular or multilocular cysts in the retroperitoneum and slightly and uniformly thickened wall that enhances following contrast medium administration. Three cases are reported.

  4. [Lymphatic cyst of the retroperitoneal space (author's transl)].

    PubMed

    De Blasi, P; Scavelli, V; Martino, D

    1979-10-01

    The authors describe an enormous lymphatic cyst, occupying cephalad and of the retroperitoneal space on the left side, half of it filled with a liquid collection and the other half being solid, displacing and compressing the stomach, spleen, and left kidney and adrenal gland (the later showing evidence of athrophy as a result of compression), Witlial, the patient had no subjective complaints and gave no objective signs of illness beyond the mere evidence of a space-occupying mass.

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

    PubMed

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

    2014-10-01

    BCOR-CCNB3 fusion transcripts resulting from an X-chromosomal paracentric inversion were recently identified in a series of unclassifiable soft tissue and bone sarcomas with Ewing sarcoma-like morphology. The morphologic and clinical features of these sarcomas are, as yet, not well characterized. Here we describe the clinicopathologic features of 10 cases of BCOR-CCNB3 sarcoma and compare their clinical course with typical Ewing sarcoma. Nine of 10 patients were male, and all were 11 to 18 years of age. Seven tumors were located in the bone and 3 in the deep soft tissues. The histomorphologic spectrum was quite wide, with 7 tumors predominately showing small primitive cell morphology with angulated nuclei simulating so-called atypical Ewing sarcoma and 3 predominately showing spindle cell morphology. Recurrent and metastatic lesions showed increased cellularity and marked pleomorphism. Immunohistochemistry showed expression of CCNB3 (100%), bcl2 (90%), CD99 (60%), and CD117 (60%). Reverse transcription polymerase chain reaction for BCOR-CCNB3 fusion transcripts was positive in all 9 cases, which yielded sufficient extracted RNA. Five- and 10-year survival rates were 75% and 56%, respectively. BCOR-CCNB3 sarcomas located in axial skeleton and soft tissues showed a significantly shorter survival. The Ewing sarcoma overall survival was not statistically different, although there was a trend for longer survival of patients with BCOR-CCNB3 sarcomas in the extremities. In conclusion, this study provides a detailed description of the histologic spectrum, immunohistochemical features, and clinical characteristic of BCOR-CCNB3 sarcoma justifying distinction from Ewing sarcoma with its typical EWS/FUS-ETS translocations. Ideally immunohistochemistry is used in combination with reverse transcription polymerase chain reaction for definitive diagnosis. PMID:24805859

  6. Retroperitoneal liposarcoma: the role of adjuvant radiation therapy and the prognostic factors

    PubMed Central

    Lee, Hong Seok; Yu, Jeong Il; Lim, Do Hoon; Kim, Sung Joo

    2016-01-01

    Purpose To evaluate the benefit of adjuvant radiation therapy (RT) for retroperitoneal liposarcoma (RPLS) following gross tumor removal. Materials and Methods We reviewed 77 patients with primary RPLS surgically treated between January 2000 and December 2013. Cases with gross residual disease were excluded. Tumor grade was evaluated according to the French Federation of Cancer Centers Sarcoma Group (FNCLCC) system. Adjuvant RT was delivered to 32 patients (42%) using external beam RT alone. Median follow-up time was 36 months (range, 5 to 169). Results Among 77 patients, 33 (43%) presented with well-differentiated, 31 (40%) with de-differentiated, 8 (10%) with myxoid/round and 4 (5%) with pleomorphic morphology. The RT group included less well-differentiated subtype than surgery group (28% vs. 53%). During follow up, 34 patients (44%) showed local recurrence. Local recurrence rate was lower in the RT group (38%) compared to the surgery group (49%). The 3-year local control rate (LC) was 55.6%, and the 3-year overall survival (OS) was 82.1%. Tumor histology and FNCLCC grade were significantly associated with local recurrence. There was no statistical significance of adding adjuvant RT in LC (p = 0.312). However, patients with tumor histology other than well-differentiated subtype showed marginally decreased local recurrence rate after adjuvant RT (3-year LC, RT 43.9% vs. no RT 35.3%; p = 0.087). Conclusion RPLS patients receiving RT experienced less local recurrence. We suggest that the addition of adjuvant RT may be related to improvement of LCs, especially in patients with non-favorable histologic subtypes. PMID:27730802

  7. Multicentric myofibroblastic sarcoma

    PubMed Central

    Wechalekar, Mihir Dilip; Ayres, Oliver; Farshid, Gelareh; Clayer, Mark; Cleland, Leslie G

    2014-01-01

    We report a case of synchronous, multicentric low-grade myofibroblastic sarcoma presenting in a 62-year-old man. He initially presented with inflammatory symmetric polyarthritis and adhesive capsulitis of his shoulder and hips bilaterally and did not respond to a trial of disease modifying antirheumatic drugs. Over a period of several years he developed progressive restriction of both knees and nodules on his hands, both knees and back. A biopsy of the nodule on his back was inconclusive and subsequent biopsies on his left and then right knee revealed a spindle cell neoplasm with an infiltrative growth pattern, mitotic figures, positive immunostaining for smooth muscle actin and focal myxoid change consistent with myofibroblastic sarcoma. While myofibroblastic sarcoma has been known to metastasise, to our knowledge, a multifocal presentation of this tumour has not been described previously. PMID:25368122

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

    PubMed Central

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

    1973-01-01

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

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

    PubMed

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

    2007-03-15

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

  10. [Extraskeletal Ewing's sarcoma].

    PubMed

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

    1992-01-01

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

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

    PubMed

    Neuville, Agnes; Coindre, Jean-Michel

    2013-12-01

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

  12. Retroperitoneal bronchogenic cyst presenting as adrenal tumor in adult successfully treated with retroperitoneal laparoscopic surgery.

    PubMed

    Chung, Jae Min; Jung, Min Jung; Lee, Wan; Choi, Seong

    2009-02-01

    A bronchogenic cyst in the retroperitoneum is rare in adults. A 41-year-old woman presented with an incidental left retroperitoneal mass that was suspicious for an adrenal tumor. Retroperitoneal laparoscopic excision and complete resection were performed. The pathologic examination confirmed a bronchogenic cyst in the retroperitoneum. We present the case with review of the relevant published data.

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

    PubMed Central

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

    2012-01-01

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

  14. 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

  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. 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

  17. 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 ...

  18. 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...

  19. 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

  20. 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

  1. 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

  2. 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.

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

    PubMed Central

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

    2016-01-01

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

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

    PubMed

    Mirilas, Petros; Skandalakis, John E

    2010-03-01

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

  5. 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.

  6. Primary retroperitoneal hydatid cyst: a rare case report.

    PubMed

    Yusuf Sherwani, Afak; Sarmast, ArifHussain; Malik, AjazAhmad; Shah, Mubashir; Q-Parray, Fazl; A-Wani, Muneer; Dangroo, Sajad

    2015-07-01

    Hydatid disease is a parasitic tapeworm infestation that usually involves liver and lungs. Primary retroperitoneal hydatid without liver and lung involvement is very rare. Surgery is the principle method of treatment for large retroperitoneal hydatid, whenever feasible. We report a case of primary retroperitoneal hydatid cyst in 29-year-old male patient.

  7. [A case of retroperitoneal venous aneurysm].

    PubMed

    Tsujimura, A; Nishimura, K; Matsumiya, K; Oka, T; Takaha, M; Arima, R; Kurata, A

    1992-09-01

    A case of retroperitoneal venous aneurysm is reported. A 73-year-old woman was referred to us with the chief complaint of left abdominal mass. A giant abdominal mass was palpable and diagnostic imaging examination including ultrasound tomography, excretory pyelography, computed tomography, magnetic resonance imaging and angiography revealed a giant cystic mass encircled by calcification in the left retroperitoneal space. Operation for this cystic mass was performed under the preoperative diagnosis of a giant left renal cyst. During operation the mass was located between the left kidney and the left adrenal gland. Because it was difficult to separate the mass from the left kidney the mass was removed with the left kidney. The extirpated tumor measured 15.5 x 15.0 x 9.5 cm and contained old blood clots and red-yellow colored fluid. A histological examination revealed that the tumor wall was composed of smooth muscle and elastic fibers. Therefore, pathological diagnosis was retroperitoneal venous aneurysm. Retroperitoneal venous aneurysm is very rare. To our knowledge, this is the 8th case of retroperitoneal venous aneurysm reported in Japan.

  8. Production of Virus by Mammalian Cells Transformed by Rous Sarcoma and Murine Sarcoma Viruses

    PubMed Central

    Valentine, Artrice F.; Bader, John P.

    1968-01-01

    Cultured cells of mammalian tumors induced by ribonucleic acid (RNA)-containing oncogenic viruses were examined for production of virus. The cell lines were established from tumors induced in rats and hamsters with either Rous sarcoma virus (Schmidt-Ruppin or Bryan strains) or murine sarcoma virus (Moloney strain). When culture fluids from each of the cell lines were examined for transforming activity or production of progeny virus, none of the cell lines was found to be infectious. However, electron microscopic examination of the various cell lines revealed the presence of particles in the rat cells transformed by either Rous sarcoma virus or murine sarcoma virus. These particles, morphologically similar to those associated with murine leukemias, were found both in the extracellular fluid concentrates and in whole-cell preparations. In the latter, they were seen budding from the cell membranes or lying in the intercellular spaces. No viruslike particles were seen in preparations from hamster tumors. Exposure of the rat cells to 3H-uridine resulted in the appearance of labeled particles with densities in sucrose gradients typical of virus (1.16 g/ml.). RNA of high molecular weight was extracted from these particles, and double-labeling experiments showed that this RNA sedimented at the same rate as RNA extracted from Rous sarcoma virus. None of the hamster cell lines gave radioactive peaks in the virus density range, and no extractable high molecular weight RNA was found. These studies suggest that the murine sarcoma virus produces an infection analogous to certain “defective” strains of Rous sarcoma virus, in that particles produced by infected cells have a low efficiency of infection. The control of the host cell over the production and properties of the RNA-containing tumorigenic viruses is discussed. Images PMID:4316021

  9. 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 ...

  10. Case report of primary retroperitoneal hydatid cyst.

    PubMed

    Yang, Gang; Wang, Xuejun; Mao, Yu; Liu, Wenying

    2011-09-01

    Hydatid cyst develops in retroperitoneal space without accompanied lesion in other organs is defined as primary retroperitoneal hydatid cyst. It is extremely rare though hydatid cyst may affect every organ in human body. A 15 years old boy presented with a giant retroperitoneal cyst and hydatid cyst was suspected. Thorough examination did not revealed lesions in liver, lung, kidney or other organs. The diagnosis was confirmed through laparotomy. The diagnosis is difficult, but the possibility should be considered before operation to prevent spillage or fatal anaphylactic shock. Living in an endemic area, ultrasonography, CT or serology test is helpful in the diagnosis of this disease. Total cystectomy is the ideal surgery, if not possible or at high risk, partial pericystectomy is acceptable.

  11. 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

  12. [Retroperitoneal bronchogenic cyst: a case report].

    PubMed

    Takahashi, Norio; Murakami, Husao; Umeda, Hiroyuki; Haga, Nobuhiro; Kameoka, Hiroshi; Shishido, Keiichi; Yamaguchi, Osamu

    2002-05-01

    A rare case of retroperitoneal bronchogenic cyst is presented. A 50-year-old man was referred to our hospital for an investigation of a retroperitoneal mass. Computed tomography and magnetic resonance imaging confirmed a retroperitoneal mass, about 4 cm in diameter, adjacent to the left kidney and spleen. On the basis of these imaging modalities, a definitive diagnosis of this mass could not be made. So, resection of the mass was performed. The removed mass was an unilocular cyst and filled with mucinous fluid. Histopathologically, the cyst wall was composed of a fibrovascular connective tissue containing mucus-secreting glands and cartilages and was lined by ciliated pseudostratified columnar epitherium. Thus, a diagnosis of bronchogenic cyst was made. Bronchogenic cysts are rare congenital anomalies of the primitive foregut and usually develop in the mediastinum and lung.

  13. Retroperitoneal bronchogenic cyst: a case report.

    PubMed

    Hsieh, S P; Tseng, H H; Huang, J K

    1997-05-01

    Only a few subdiaphragmatic bronchogenic cysts are described, and their occurrence in the retroperitoneum is extremely rare. So far, only a few cases have been reported in the English-language literature. The pathogenesis is caused by the pinching off of irregular lung budding of the primitive ventral foregut, with aberrant migration into the abdomen before fusion of the diaphragm during embryonal development. A unique case with clinical, radiographic, surgical finding is presented. Final pathological findings confirmed the diagnosis of retroperitoneal bronchogenic cyst without other associated congenital anomalies. Retroperitoneal bronchogenic cyst, although rare, should be considered in the differential diagnosis in the retroperitoneal mass. This rare case is described and the relevant literature, reviewed.

  14. 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

  15. 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

  16. 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.

  17. Spontaneous retroperitoneal hemorrhage secondary to subcapsular renal hematoma: MRI findings.

    PubMed

    Balci, N C; Sirvanci, M; Tüfek, I; Onat, L; Duran, C

    2001-10-01

    Spontaneous retroperitoneal hemorrhage is a rare intraabdominal bleeding. In this report we present a case of a nontraumatic retroperitoneal hemorrhage secondary to spontaneous subcapsular renal hematoma. A 54-year-old patient who was under warfarin therapy, developed subcapsular right renal hematoma. Subcapsular and retroperitoneal hemorrhage were low signal on T1- and T2-weighted images consistent with acute stage of blood. The source of subcapsular hematoma was shown to be the rupture of hemorrhagic renal cyst on MRI. Extension of hemorrhage into the retroperitoneal space anterior to right psoas muscle was also successfully shown on MRI. Patient underwent nephrectomy and retroperitoneal blood was evacuated.

  18. 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.

  19. Fibromatosis--a rare retroperitoneal tumour.

    PubMed

    Uranüs, S; Beham, A; Stenzl, W

    1990-01-01

    A case of retroperitoneal fibromatosis in a 28-year-old white male is presented. The soft tissue tumor, with a weight of 8000 g, was resected by laparotomy. Because of adhesions to the ascending colon and the right ureter, a hemicolectomy and partial resection of the right ureter had to be performed additionally. Intraabdominal fibromatoses are very infrequent tumorous lesions of the connective tissue, occurring retroperitoneally only in isolated cases. Their etiology is presumed to be a hereditary or gene-associated defect in the regulation of connective tissue growth. In addition, trauma and hormonal influences often appear as inductive cofactors.

  20. Cystic retroperitoneal lymphangioma in infants and children.

    PubMed

    Leonidas, J C; Brill, P W; Bhan, I; Smith, T H

    1978-04-01

    Retroperitoneal lymphangiomas are uncommon benign tumors usually presenting in early life. They may cause significant pressure on adjacent structures, most often with superior and medial displacement of the kidney and medial deviation of the ureter. Relative radiolucency on total body opacification during excretory urography, and features of a cystic structure on ultrasonography and computed tomography should further suggest the possibility of a retroperitoneal lymphangioma. Vascular stretching and displacement without neovascularity on angiography and contrast entry into the cysts on lower extremity lymphography are additional features, but such procedures are usually unnecessary.

  1. [A case of retroperitoneal venous aneurysm].

    PubMed

    Maeda, S; Tamaki, M; Yamamoto, N; Takeda, A; Kuriyama, M; Kawada, Y; Mizoguchi, Y; Kasahara, M

    1991-01-01

    Venous aneurysm is a rare entity and the disease occurring in the retroperitoneal space has been reported in only 4 cases. Therefore, the fifth case of retroperitoneal venous aneurysm on the literature was described. A 59-year-old male was referred to our clinic because of painless large mass in the left abdomen. Computed tomography, ultrasonography, and magnetic resonance imaging revealed a cystic mass at the perinephric space. The resected cyst contained yellow-grayish fluid. The cyst wall was microscopically formed of 4 layers; blood and cholesterin, hyaline, muscle and collagen from the inner to outer side. He is well without any trouble after the operation.

  2. Ovarian and retroperitoneal teratomas in a dog.

    PubMed

    Nagashima, Y; Hoshi, K; Tanaka, R; Shibazaki, A; Fujiwara, K; Konno, K; Machida, N; Yamane, Y

    2000-07-01

    A 2-year-old female Labrador retriever was presented with anorexia and abdominal distention. Laparotomy revealed the presence of a 31-cm ovoid mass in the portion of the left ovary and an 11-cm round mass in the left retroperitoneal region. Both masses were surgically removed. Histopathological examination of the masses revealed a mixture of tissues characteristic of teratomas, such as multiple bronchial and intestinal cysts, hair follicles, sebaceous and apocrine sweat glands, and neuron tissue with intervening cartilage, bone and fat tissue. This paper appears to be the first report of primary retroperitoneal teratoma in the dog.

  3. [Splenoportography in pancreatic tumors and retroperitoneal neoplasms].

    PubMed

    Roshchektaev, N V

    1975-01-01

    Along with other methods of investigation splenoportography was performed in 29 patients with tumors and in 2 patients with cystic affection of the pancreas. Moreover, in 5 cases splenoportography was performed due to a suspicion to pancreatic tumor, and also in 6 patients with retroperitoneal tumors and in 7-with renal tumors. In all these patients with pancreatic tumors splenoportograms have shown changes on the part of a splenoportal trunk, which corresponded to the localization and degree of spread of a tumor in most cases. Splenoportography is felt to be rational in retroperitoneal tumors. In such cases splenoportography would contribute to a detailed determination of tumor localization and its relation with the liver.

  4. Retroperitoneal Pyelolithotomy for Management of Renal Calculi

    PubMed Central

    Suryavanshi, Manav; Lal, Pawan; Singh, Lakvinder; Ramteke, V. K.

    2005-01-01

    Objective: We evaluated the role of retroperitoneoscopic pyelolithotomy in the management of renal calculi. Methods: Fifty-six cases (male, 27; female, 29) of solitary or multiple renal calculi were evaluated in the study. There were 46 patients with a single calculus, 4 patients with a staghorn calculus, and 6 with a caliceal calculus. Retroperitoneoscopic pyelolithotomy was carried out after creating a retroperitoneal space with the balloon dissection method. Pneumoretroperitoneum was maintained by carbon dioxide insufflation. Results: Stone clearance was achieved in all cases barring 2 cases of caliceal calculi that were converted to the open procedure. The postoperative hospital stay averaged 4 days. Patients were ambulatory within 24 hours and back to work within 7 days on average. Complications encountered were peritoneal rent, subcutaneous emphysema, and superficial wound infection. The postoperative analgesic requirement averaged 100 mg of diclofenac (2 tablets). Conclusions: Retroperitoneoscopic pyelolithotomy is a safe, simple, and effective minimally invasive procedure and is a feasible option that can be recommended for management of renal calculi. PMID:15791981

  5. Mini-access for retroperitoneal laparoscopy.

    PubMed

    Gaur, D D; Gopichand, M; Dubey, M; Jhunjhunwala, V

    2002-10-01

    The purpose of this study was to establish the feasibility of primary mini-access to the retroperitoneal space to perform certain retroperitoneoscopic procedures in which an enlarged port is not required for extraction of the specimen. Mini-laparoscopy or needlescopic laparoscopy has been performed in the past via the transperitoneal route, but this has not yet been reported for a retroperitoneoscopic procedure. Through a 5-mm primary access and 3- or 5-mm secondary ports, retroperitoneoscopic surgery was performed in seven adults for ureterolithotomy, renal biopsy, simple nephrectomy, nephropexy, cyst decortication, or drainage of a subphrenic abscess. The access technique used was a mini-version of a previously described percutaneous access technique with some modifications. The retroperitoneoscopic procedure was successful in all seven patients, and no complications developed. The operative time, blood loss, analgesic intake, and length of hospital stay were comparable with those of a standard retroperitoneoscopic procedure. However, the cosmetic results were better. Mini-retroperitoneoscopy is feasible and is a better alternative for patients in whom the whole procedure can be performed through 5-mm or smaller ports.

  6. 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.

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

    PubMed

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

    2015-05-01

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

  8. Laparoscopic retroperitoneal lymph node dissection after prior open retroperitoneal lymphadenectomy and chemotherapy.

    PubMed

    Lima, Guilherme C; Kohanim, Sahar; Rais-Bahrami, Soroush; Kavoussi, Louis R

    2005-12-01

    Laparoscopic retroperitoneal lymphadenectomy for testicular cancer is a challenging surgical procedure. Several factors can increase the difficulty, including prior chemotherapy or open surgery. We present a case of a laparoscopic "redo" postchemotherapy nodal dissection to treat a residual retroperitoneal mass in a patient with non-seminomatous germ cell tumor. This approach allowed rapid recovery, and at 2.5 years after surgery no evidence of tumor recurrence was seen.

  9. Retroperitoneal laparoscopic pyelolithotomy: how does it compare with percutaneous nephrolithotomy for larger stones?

    PubMed

    2001-03-01

    Our 10-year experience of retroperitoneal laparoscopic pyelolithotomy, a rarely performed minimally invasive operative procedure, is presented. The results are compared with our own experience of percutaneous nephrolithotomy for larger renal stones. Forty-two patients with a mean age of 39.12 years underwent 43 retroperitoneal laparoscopic pyelolithotomies using Gaur's balloon technique. The total number of stones was 65 (44 pelvic and 21 calyceal) and they ranged in size between 5 and 48 mm. Two patients had chronic renal failure due to bilateral impacted renal calculi. Forty-eight percutaneous nephrolithotomies performed in the same unit during the last 2 years in patients with non-staghorn calculi > 2 cm were included for a comparative study. The open conversion rate and the drainage period for retroperitoneal laparoscopic pyelolithotomy were much higher. However, the operative time, blood loss, analgesic intake, hospital stay, residual stone rate, re-treatment rate and major complication rates were lower, compared with percutaneous nephrolithotomy. PMID:16753999

  10. [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

  11. Renal metastases from osteogenic sarcoma

    SciTech Connect

    Ayres, R.; Curry, N.S.; Gordon, L.; Bradford, B.F.

    1985-01-01

    A clinically and radiographically unsuspected ossified renal metastasis from a primary osteogenic sarcoma was identified by computed tomography (CT) and radionuclide bone scan. These imaging modalities play an important adjunctive role in the evaluation and follow-up of patients with primary osteogenic sarcoma.

  12. 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.

  13. A Giant Retroperitoneal Lymphangioma: A Case Report.

    PubMed

    Hubli, Prabhu; Rohith, Muddasetty; Sachin, Basavaraju M

    2016-07-01

    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. PMID:27630902

  14. 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. PMID:27630902

  15. Spontaneous Retroperitoneal Hemorrhage from Adrenal Artery Aneurysm

    SciTech Connect

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

    2007-04-15

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

  16. Giant retroperitoneal cyst in an adult male.

    PubMed

    Egawa, S; Satoh, T; Suyama, K; Uchida, T; Iwabuchi, K; Koshiba, K

    1996-07-01

    This paper presents a case of a symptomatic giant retroperitoneal cyst in an adult male. The unilocular cyst was excised successfully with resolution of the attendant symptoms. Histological findings of the cyst wall suggested a lymphangiomatous etiology. Any good risk patient found to harbor such a cyst should undergo complete excision in view of the potential for the development of symptoms and complications.

  17. [Computer tomography of retroperitoneal trauma (author's transl)].

    PubMed

    Fischedick, A R; Müller, R P; Kramps, H; Cramer, B

    1982-01-01

    Computer tomography changes after retroperitoneal trauma are described on the basis of 19 patients seen by the authors. It appears that this method is superior to conventional techniques, both as a screening method and for carrying out follow-ups. The indications for angiography are thereby reduced.

  18. A Giant Retroperitoneal Lymphangioma: A Case Report.

    PubMed

    Hubli, Prabhu; Rohith, Muddasetty; Sachin, Basavaraju M

    2016-07-01

    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.

  19. [A case of retroperitoneal cystic lymphangioma].

    PubMed

    Perini, F; Metelli, F; Bazzana, M

    1979-06-30

    A case of retroperitoneal cystic lymphangioma in a 62-yr-old woman presented as symptom-free abdominal swelling. The intraoperative diagnosis was confirmed histologically. Radical management consisted of stripping of the cyst from its adjacent structures. The postoperative course was uneventful.

  20. [Lymphangioadenography in the diagnosis of retroperitoneal tumors].

    PubMed

    Galaĭko, G M

    1975-10-01

    A method of direct lymphangioadenography was employed in 18 patients with abdominal tumors of obscure origin. These were as follows: lymphogranuloma, splenic lymphosarcoma, tumors of the stomach, kidney, pancreas both benign and malignant, retroperitoneal cysts, fibroma, malignant periganglioma. Roentgenological symptoms of abdominal tumors of various localization are reported.

  1. [A case of retroperitoneal bronchogenic cyst].

    PubMed

    Kondo, Hideaki; Fujimoto, Kiyohide; Aoki, Katsuya; Cho, Masaki; Hirao, Yoshihiko; Natsume, Osamu

    2005-01-01

    A 59-year-old hypertensive man was referred to our hospital with a retroperitoneal cystic tumor, measuring 6 cm in diameter that was detected by an ultrasound examination during routine check-up 2 years before coming to our department. During the 2-year follow-up, the cystic tumor gradually increased in size. The patient also became hypertensive with slightly elevated urine levels of noradrenaline and dopamine, while the plasma catecholamines and their metabolites in the urine were within the normal range. Computed tomographic scanning and magnetic resonance imaging revealed a dumbbell-shaped retroperitoneal cyst with dense fluid, measuring 7 x 3.5 x 3 cm in diameter, in the left supra-adrenal and sub-diaphragmatic regions. He underwent extirpation of the cystic tumor with suspicion of adrenal endocrine cystic tumor. The histopathological diagnosis was a bronchogenic cyst, which is an extremely rare developmental anomaly in the retroperitoneal space. We herein report this rare case of retroperitoneal bronchogenic cyst and present a brief review of the previously reported 30 Japanese cases.

  2. 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

  3. A retroperitoneal bronchogenic cyst mimicking adrenal tumour in an adult: is differential diagnosis truly possible?

    PubMed

    Onol, F F; Baytekin, F; Dikbas, O; Ergönenç, T; Tanidir, Y

    2009-02-01

    Bronchogenic cysts are developmental abnormalities of the primitive foregut resulting from aberrant budding from the ventral diverticulum. A retroperitoneal position for a bronchial cyst is extremely unusual and should be differentiated from other neoplastic lesions. Although histologically they can be differentiated from other lesions, bronchial cysts may show various pathological patterns of differentiation that may cause confusion in differentiating them from cystic teratomas. This is a case of a retroperitoneal bronchogenic cyst featuring uncommon histological findings, which raises the question whether these benign lesions can always be accurately differentiated from teratomatoid cystic neoplasms.

  4. Identification and surgical management of cystic retroperitoneal lymphangioma in children.

    PubMed

    Waldhausen, J H; Holterman, M J; Tapper, D

    1996-04-01

    Retroperitoneal lymphangiomas are rare, benign cystic structures that are best evaluated with computed tomography and ultrasound. Preoperative diagnosis is often difficult, in part because there is little to distinguish them from other cystic masses and because the lesion is often not considered on the differential diagnosis. Surgery may be required as both a diagnostic and therapeutic measure. The cysts may be asymptomatic for years and then present because of compression of surrounding structures or pain. The treatment is as complete surgical excision as is possible. Bowel cleansing should be done preoperatively. The long-term results are excellent when total excision or near-total excision with marsupialization is accomplished.

  5. 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

  6. 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

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

    PubMed Central

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

    2016-01-01

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

  8. Primary retroperitoneal hydatid cyst: report of 2 cases and review of 41 published cases.

    PubMed

    Akbulut, Sami; Senol, Ayhan; Ekin, Abdulselam; Bakir, Sule; Bayan, Kadim; Dursun, Mehmet

    2010-01-01

    This paper gives an overview of the literature between 2000 and 2010 on primary retroperitoneal hydatid cyst. We reported 2 cases of primary retroperitoneal hydatid cyst, and studies published in English literature on hydatid cyst developing in the retroperitoneal space were accessed via Pubmed and Google Scholar databases. Forty-one published primary retroperitoneal hydatid cyst cases were evaluated, and 2 patients (1 man, 78 years old; 1 woman, 75 years old) who presented with abdominal mass caused by retroperitoneal hydatid cyst were reported. Twenty-five of the patients were men (including our patient), and 18 were women; patients ranged in age from 3 to 80 years, and the median +/- standard deviation age was 41.37 +/- 20.4 years. On presentation, 72% of the patients complained of back or abdominal pain; 13.9% had urinary tract symptoms, and 65.1% were determined as having a palpable mass. Ultrasonography was performed on 93% of the patients, computed tomography was performed on 81.4%, magnetic resonance imaging was performed on 18.6%, and intravenous pyelography test was performed on 13.9%. The results of these tests showed a cystoid mass located on the left in 32.5% of the patients, on the right in 37.2%, and in the retrovesical area in 16.2%. Serologic tests determined 67.8% of the patients were indirect hemagglutination positive, and 71.4% were positive on enzyme-linked immunosorbent assay. As a surgical approach, total exision was performed on 55.8% of patients, partial cystectomy was performed on 39.5%, and 4.6% of patients underwent unroofing. If a cystic lesion is determined in the retroperitoneal area in a patient living in an area of endemic hydatid disease, a differential diagnosis of hydatid cyst should be considered. Clinical, radiologic, serologic, and histopathologic evaluations should be made for a differential diagnosis.

  9. 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

  10. 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

  11. Sarcomas of the fallopian tube: disentangling a rare entity.

    PubMed

    Zagouri, Flora; Dimopoulos, Meletios-Athanassios; Thomakos, Nikolaos; Chrysikos, Dimosthenis; Papadimitriou, Christos A

    2011-01-01

    Sarcomas of the fallopian tube are exceedingly rare malignancies. They have been considered the most lethal of all gynaecological malignancies with high metastatic potential, frequent recurrences and cancer-related deaths. The reported pathological types of the fallopian tube sarcomas are malignant mixed mullerian (mesodermal) tumours or carcinosarcomas, leiomyosarcomas, rhabdomyosarcomas, liposarcomas, and synovial sarcomas. The rarity of these sarcomas and their often aggressive clinical course has resulted in a relatively limited amount of literature. Thus a single hospital or specialist cannot gain sufficient experience with these tumours. This review article tries to elucidate this uncommon malignancy, in a systematic way, focusing on the different pathological types, epidemiology, risk factors, diagnosis, survival, and different therapeutic modalities (surgery, chemotherapy, and radiotherapy).

  12. Sarcoma of the breast: an update on a rare entity.

    PubMed

    Lim, Sue Zann; Ong, Kong Wee; Tan, Benita Kiat Tee; Selvarajan, Sathiyamoorthy; Tan, Puay Hoon

    2016-05-01

    Breast sarcoma is a rare condition. It consists of a heterogeneous group of non-epithelial tumours arising from the mesenchymal tissue of the breast. It has a distinctly different natural history, treatment response and prognosis as compared with carcinoma of the breast. A different diagnostic approach and treatment strategy have to be defined for this group of tumours. Due to its rarity, the current understanding on breast sarcoma is limited and is mostly based on small retrospective case series or case reports. Hence, the management generally follows the algorithms derived from randomised control trials of soft tissue sarcomas in the extremities and chest wall. Through this review, we discuss the results of major retrospective studies on breast sarcomas including data on epidemiology, aetiology, diagnostic approach, treatment strategies and outcomes of this challenging and potentially aggressive condition.

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

    PubMed

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

    2016-03-17

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

  14. 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

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

    PubMed Central

    Varshney, Jyotika; Subramanian, Subbaya

    2015-01-01

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

  16. 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

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

    PubMed

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

    2013-01-01

    Antecedentes: el sarcoma fibromixoide de bajo grado o tumor de Evans es una variedad de sarcoma de partes blandas que representa el 1% de todos los tumores malignos. Más frecuente en extremidades y tronco (50%), presentamos tres nuevos casos de localización retroperitoneal. Se revisan sus características y se realiza actualización bibliográfica. Casos clínicos: en su localización retroperitoneal destaca lo inespecífico de su sintomatología clínica, se manifiesta en función de su crecimiento y tamaño, como una tumoración abdominal, habitualmente indolora, o por la compresión y/o invasión de estructuras próximas. Los casos presentados se manifestaron, por la presencia de tumoración abdominal, sin otra sintomatología; a pesar de infiltrar a otros órganos. Conclusiones: el diagnóstico preoperatorio es difícil, y debe tenerse la presunción clínica para solicitar el estudio histológico, con tipificación inmuno-histo-química para su dentificación precisa.

  18. [Isolated retroperitoneal hydatid cyst. CT study. Apropos of a case].

    PubMed

    Ménor Serrano, F; Marti-Bonmati, L; Garcia Aguayo, F; Gordo Roman, G; Ballesta Cunat, A

    1987-03-01

    A 30 year old patient with isolated retroperitoneal hydatid cyst was the first case of this type to be studied by CT scanning. Isolated retroperitoneal hydatid cyst is defined as any zone of hydatidosis occurring in the fatty tissue of the spaces lying behind posterior parietal peritoneum, without any parasitic foci in other organs. Differential diagnosis from other primary retroperitoneal cysts has been improved by the availability of modern imaging procedures: ultrasonography and CT scan.

  19. Laparoscopic removal of a benign pelvic retroperitoneal dermoid cyst.

    PubMed

    Sharpe, L A; Van Oppen, D J

    1995-02-01

    Retroperitoneal tumors are rare, often malignant, and difficult to diagnose. Surgical removal is associated with significant morbidity and mortality. We believe this to be the first report of laparoscopic removal of a benign pelvic retroperitoneal dermoid cyst from the right pararectal and presacral areas. Iatrogenic enterocele was prevented by laparoscopic suturing and knot tying to perform a modified McCall culdoplasty. The excellent outcome of this case suggests that laparoscopic management should be considered for carefully selected benign retroperitoneal tumors.

  20. Cyclin D1 expression and HHV8 in Kaposi sarcoma.

    PubMed Central

    Kennedy, M M; Biddolph, S; Lucas, S B; Howells, D D; Picton, S; McGee, J O; Silva, I; Uhlmann, V; Luttich, K; O'Leary, J J

    1999-01-01

    BACKGROUND: Human herpesvirus 8 (HHV8) appears to be the agent responsible for Kaposi sarcoma. The mechanism remains undetermined but may involve cell cycle regulating genes including D type cyclins which are pivotal in cell cycle progression. Recent HHV8 genetic analysis has revealed the presence of a v-cyclin which is homologous to D type cyclins. AIMS: First, to assess whether there is an independent relation between endogenous cyclin D1 expression in Kaposi sarcoma and HHV8 status; second to determine whether v-cyclin mRNA expression varies with Kaposi sarcoma stage. METHODS: Cyclin D1 immunohistochemistry was performed on 17 paraffin embedded Kaposi sarcoma samples from 16 patients. HHV8 status was assessed in 15 of these using nested polymerase chain reaction (PCR) to ORF 26 and the newly described technique of TaqMan PCR. An additional 10 fresh Kaposi sarcoma samples (early and nodular) were examined for HHV8 v-cyclin RNA. RESULTS: One case, which did not contain amplifiable HHV8, showed strong cyclin D1 staining. The remaining cases were negative or weakly staining; v-cyclin transcript load was higher in early Kaposi sarcoma. CONCLUSIONS: While endogenous cyclin D1 expression is independent of HHV8 status, v-cyclin transcription is higher in early lesions, supporting the "viral hit" hypothesis. Images PMID:10645225

  1. [A case of bronchogenic cyst treated as retroperitoneal tumor].

    PubMed

    Matsuzaki, Kyosuke; Okumi, Masayoshi; Yoshida, Yasuyuki; Yoshioka, Iwao; Tsujimura, Akira; Nonomura, Norio

    2013-11-01

    A 66-year-old man presented with a retroperitoneal mass found in a routine medical examination. He had no complaints and no medical history. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a cystic mass in the retroperitoneal space, attached to the left crus of the diaphragm, 5.5 cm in size. Retroperitoneal cystic tumor was diagnosed, and transperitoneal tumor resection was performed. Pathological findings revealed a cyst wall lined with ciliated epithelium and cartilage, diagnosed as a retroperitoneal bronchogenic cyst. The patient was in good health at 12 months after the surgery with no evidence of recurrence.

  2. Laparoscopic excision of an infected "egg-shelled" retroperitoneal pseudocyst.

    PubMed

    Palanivelu, Chinnusamy; Rangarajan, Muthukumaran; Senthilkumar, Rangaswamy; Madhankumar, Madhupalayam Velusamy; Annapoorni, Shankar

    2008-12-01

    Primary retroperitoneal pseudocysts are rare entities. Though laparoscopic approach has been described in their treatment, open surgical excision is still the mainstay of treatment for these lesions. We present a case of infected retroperitoneal pseudocyst and its successful laparoscopic excision. The patient was an 80-year old female. Contrast enhanced CT scan of the abdomen and ultrasonography confirmed a large retroperitoneal cyst. Laparoscopic resection was accomplished after puncturing and decompressing the cyst. There were no complications or conversion. The operating time was 176 minutes. The patient was discharged 3 days after surgery. Histopathology revealed a pseudocyst. Retroperitoneal pseudocysts can be resected laparoscopically with careful and meticulous laparoscopic dissection, utilizing the advantages of laparoscopy.

  3. 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.

  4. 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

  5. Case of retroperitoneal solitary fibrous tumor.

    PubMed

    Yamashita, Shinichi; Tochigi, Tatsuo; Kawamura, Sadafumi; Aoki, Hiroshi; Tateno, Hiroo; Kuwahara, Masaaki

    2007-07-01

    Solitary fibrous tumor (SFT) of the retroperitoneal space is rare. We report a case of retroperitoneal tumor, diagnosed as SFT. A 69-year-old woman presented with right lower abdominal swelling, and was referred to our hospital with suspicion of right renal tumor. Abdominal ultrasound and computerized tomography (CT) showed a mass (about 15 x 14 x 10 cm) in the right abdomen. The tumor was thought to be right renal rumor, and right radical nephrectomy was performed. In the excised specimen the tumor was not connected to gastrointestinal tract, peritoneum, or right kidney. The histological and immunohistochemical examination of the specimen revealed SFT. The tumor has malignant potential with partially increased mitotic activity and cellularity in the histological examination. The patient is healthy and without evidence of recurrence or metastasis 26 months from surgery.

  6. Isolated renal and retroperitoneal hydatid cysts.

    PubMed

    Tan, M O; Emir, L; Germiyanoğlu, C; Uygur, C; Altuğ, U; Erol, D

    2000-01-01

    Four patients (3 male, 1 female) with isolated renal and 1 female patient with isolated retroperitoneal cysts were reviewed. The mean age of the patients was 46 (25-64). The most common presenting symptom was pain. Two cases were discovered incidentally by the observance of renal calcification on abdominal x-ray. Indirect hemagglutination test was positive in all cases but eosinophilia was present only in 1 (20%) case. Nephrectomy was performed to 1 patient who presented with hydaturia and had a large communicating cyst involving most of the kidney. Total cystectomy was performed in other renal cysts. Total cystectomy with wide excision of the involved muscle was performed to the retroperitoneal hydatid cyst. Patients were followed by an average of 23.8 (9-50) months with indirect hemagglutination test and USG. No evidence for recurrence was found up to date.

  7. Retroperitoneal Ancient Schwannoma: A Case Report.

    PubMed

    Çalişkan, Selahattin; Gümrükçü, Gülistan; Kaya, Cevdet

    2015-01-01

    Schwannomas are extremely rare tumors that are composed of Schwann cells. Retroperitoneal localization comprises 0.7% to 2.6% of all schwannomas. Patients usually present with nonspecific symptoms. There are no pathognomonic features on radiologic evaluation. Preoperative biopsy is not recommended because of complication risks; however, surgery is necessary for diagnosis and treatment. Although most schwannomas are benign tumors, those that are associated with von Recklinghausen disease are malignant. Schwannomas exhibit regions of high and low cellularity, termed Antoni A and Antoni B areas, with a diffuse positivity of S100 protein on pathologic evaluation. If there are degenerative changes, such as cyst formation, hemorrhage, calcification, and hyalinization, these tumors are termed ancient schwannomas. We present a case of retroperitoneal ancient schwannoma.

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

    PubMed

    Cariati, A

    1993-12-01

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

  9. Endoscopic management of a retroperitoneal hydatid cyst.

    PubMed

    Chowbey, Pradeep K; Wadhwa, Atul; Shah, Sumeet; Sharma, Anil; Khullar, Rajesh; Soni, Vandana; Baijal, Manish

    2004-08-01

    Hydatid cyst is a significant health problem in endemic regions. Although progress has been made in medical treatment and interventional radiology techniques, surgery is currently the treatment of choice. The hydatid cysts are known to occur at several unusual sites in the body. With increasing experience in laparoscopy and retroperitoneoscopy, attempts have been made to offer the advantages of minimal access procedures to hydatid disease patients. We present a case report of such a patient whose retroperitoneal hydatid cyst was removed endoscopically.

  10. Pseudocyst formation in retroperitoneal fibrosis relapse.

    PubMed

    Jansen, I; Hendriksz, T R; Van Bommel, E F H

    2010-06-01

    We describe a 45-year-old male patient with recurrent retroperitoneal fibrosis (RPF), in whom a pseudocyst in the peri-aortic fibrotic mantle was diagnosed. Without any intervention other than oral treatment with tamoxifen, the pseudocyst showed significant regression. Although rare, pseudocyst formation may sometimes appear in RPF and may mimic other benign and malignant conditions. This is the first paper to describe pseudocyst formation in an RPF relapse.

  11. Retroperitoneal endoscopic debridement for infected peripancreatic necrosis.

    PubMed

    Seifert, H; Wehrmann, T; Schmitt, T; Zeuzem, S; Caspary, W F

    2000-08-19

    Standard management of infected peripancreatic necrosis consists of open surgical debridement and lavage--a traumatic intervention with substantial morbidity and mortality. As an alternative and novel approach with minimum invasiveness, we present fenestration of the gastric wall and debridement of infected necrosis by direct retroperitoneal endoscopy. In three patients, this strategy led to rapid clinical improvement and no serious complications. Transgastric endoscopic therapy may be a less traumatic alternative to surgery and should be further assessed in prospective studies.

  12. Pulmonary lymphangioleiomyomatosis associated with retroperitoneal lymphangioleiomyomas.

    PubMed

    Oikonomou, Anastasia; Tzouvelekis, Argyris; Zezos, Petros; Bouros, Demostehenes; Prassopoulos, Panos

    2012-01-01

    A 36-year-old woman presented with worsening symptoms of abdominal pain, diarrhoea, low fever, dyspnoea on exertion and bilateral leg during the last 2 months. Chest radiograph showed mild diffuse reticular pattern with preserved lung volumes. Lung high resolution CT disclosed multiple thin-walled lung cysts throughout the lungs bilaterally with no zonal predominance and with normal intervening parenchyma and abdominal CT revealed bilateral retrocrural and retroperitoneal hypodense masses. Imaging findings were consistent with lymphangioleiomyomatosis.

  13. [Giant retroperitoneal mesenteric cyst presenting as dyspepsia].

    PubMed

    Rosón Rodríguez, Pedro J; Asensio, Antonio Del Fresno; Quintero Barranco, Belén

    2010-01-01

    Dyspepsia is a frequent cause of referral to gastroenterology units. After appropriate investigations, many patients receive a diagnosis of functional disorders, although dyspepsia can have an organic basis. We present the case of a woman with typical symptoms of dyspepsia, which were initially mild. After appropriate investigations, the patient was diagnosed with a giant retroperitoneal tumor. We discuss the diagnostic approach to this entity and review the literature on the topic.

  14. [Primary retroperitoneal enteric cyst in an adult].

    PubMed

    Kondor, Ariella; Hajdu, Gábor; Zöllei, István; Bende, Mariann

    2005-06-01

    The posterior mediastinum is the usual location of enteric cysts. They can be located at any part of the posterior mediastinum adjacent to the oesophagus, but communication with that structure is rare. Approximately 60 percent of the cysts occur in infants less than one year of age, and symptoms of tracheal or oesophageal obstruction are common. The cysts usually contain clear, colorless mucoid fluid. The authors describe here a successfully treated enteric cyst with an unusual, retroperitoneal location, which presented in an adult.

  15. 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

  16. 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.

  17. 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 ...

  18. 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 ...

  19. [Spontaneous retroperitoneal hematoma: a case report].

    PubMed

    Tosato, F; Palermo, S; Carnevale, L; Paolini, A

    2000-01-01

    The A.A. present a case report of retroperitoneal spontaneous hematoma and put in evidence the difficulties in making an etiopathogenetic diagnosis of spontaneous renal cyst rupture. The patient was a 72 years old man admitted to the hospital in emergency for a painful ipogastric syntomatology and biliary vomit. The ecography showed a distanced gallbladder and the endoscopy evidenced a duodenal stenosis with compression of the gastric posterior wall, so that we were initially directed towards a pancreatic tumor even if the modest increase of the serum bilirubinemy and mostly the fact that direct component was almost normal, gave us strong doubts. The spiral TC gave finally the diagnosis of retroperitoneal hematoma and the presence of a bilateral polycystic kidney with several subcapsular cyst, let us suggest, as well as the anamnestic report of a physical stress, a renal origin of the bleeding. A wild literature review did not evidence other similar cases, but the ecclusion of other possible causes of retroperitoneal hematoma, suggested this conclusion.

  20. [Laparoscopic extirpation of retroperitoneal bronchogenic cyst].

    PubMed

    Akos, Molnár Béla; Péter, Kaliszky; Edina, Nagy; János, Horányi; Eszter, Székely

    2006-02-01

    The retroperitoneal bronchogenic cyst is an extremely rare anomaly. During the examination of an 18-year-old female patient due to her extreme thinness, the abdominal ultrasound and later the CT indicated as an accidental finding--a left side adrenal/suprarenal adenoma, which turned out to be hormonally inactive. But while we were doing a laparoscopic surgical intervention we found a 6-8-cm cyst in the retroperitoneum, between the greater curvature and the spleen. It contained light grey, mucinous liquid. The left suprarenal gland had normal size and appearance. During the microscopic examination the removed terime showed cystic structures, which were covered inside with multilayer ciliated columnar epithelium, mucous excretory glands, a whole layer of connective/interstitial and smooth muscle tissue. Neither cartilage, nor gastrointestinal epithelium was found. Only a few publications have reported about retroperitoneal bronchogenic cyst so far. These lesions occur very rarely at the greater curvature. Although this alteration is really unusual, bronchogenic cysts have to be expected in the differential diagnostics of the retroperitoneal tumors.

  1. A retroperitoneal bronchogenic cyst with malignant change.

    PubMed

    Sullivan, S M; Okada, S; Kudo, M; Ebihara, Y

    1999-04-01

    A unique case of adenocarcinoma arising in a retroperitoneal bronchogenic cyst is presented. A 55-year-old woman presented with lower abdominal discomfort. Computed tomography revealed a retroperitoneal cystic mass attached to the ascending colon. The resected cyst was unilocular and filled with milky white mucus and hemorrhagic debris. Histologically, most of the cyst wall was of well-differentiated papillary adenocarcinoma with no cyst wall invasion. Other small areas of the cyst were lined with variably atypical dysplastic/metaplastic cuboidal to pseudostratified columnar epithelium. The cyst wall was mostly hyalinized, but there was apparent thickened subepithelial basement membrane, elastosis, and a single layer of smooth muscle that suggested bronchial wall structures. A mucin staining study with O-acylated sialic acid, which is used for the demonstration of gastrointestinal, cholecystic and uterine cervical mucins, was negative for the mucin-producing epithelial cells of the cyst. Thus, to our knowledge, this is the first reported case of adenocarcinoma arising in a retroperitoneal bronchogenic cyst.

  2. Heterotopic gastrointestinal mucosa and pancreatic tissue in a retroperitoneal tumor.

    PubMed

    Hashimoto, Naoki; Hakamada, Kenichi; Narumi, Shunji; Totsuka, Eishi; Aoki, Kazunori; Kamata, Yoshimasa; Sasaki, Mutsuo

    2006-01-01

    We believe that this is the first report of a retroperitoneal tumor consisting of heterotopic gastrointestinal mucosa and pancreatic tissue. The patient was a 19-year-old woman with the chief complaint being occasional back pain. Abdominal computerized tomography demonstrated a 3.1 x 2.5 x 3.2-cm low-density solid and cystic lesion adjoining the left renal vein between the aorta and inferior vena cava. Angiography revealed that the inferior vena cava was displaced by the hypovascular tumor. The retroperitoneal lesion was diagnosed preoperatively as a benign tumor such as a neurogenic neoplasm or lymphangioma. At laparotomy, a cystic tumor was found, which existed behind the inferior vena cava and renal vessels, and contained reddish-brown fluid, suggesting hemorrhage in the past. The cut surface of the tumor showed a unilocular cyst with partially hypertrophic wall. Histopathological examination revealed a cystic tumor lined with heterotopic gastric and duodenal mucosa, with pancreatic tissue in the muscularis propria. In addition, evidence of bleeding from the gastric mucosa was observed in the cystic tumor. External secretion from these tissues could have triggered the hemorrhage and expanded the tumor, possibly resulting in the back pain.

  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. 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.

  5. 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

  6. 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.

  7. [A case of serous cyst of the retroperitoneal space].

    PubMed

    Nishizawa, K; Murakami, Y; Okada, K; Matsushita, K; Kawamura, N

    1983-03-01

    There have been very few reports on retroperitoneal serous cysts and only 23 cases have been reported in Japan. We report a case of a 47-year-old woman who presented with the complaint of dull pain in her lower abdomen. After careful examination, she was diagnosed to have retroperitoneal serous cyst.

  8. [Benign non-organ-related diseases of the retroperitoneal space].

    PubMed

    Szolar, D H; Uggowitzer, M M; Kammerhuber, F H; Schreyer, H H

    1997-08-01

    Because of the anatomic localisation of the retroperitoneal space, the detection and elucidation of pathology in the retroperitoneum calls for clinical acumen and the utilisation of imaging techniques. During the past two decades, efforts spearheaded by the work of M. A. Meyers led to an enhanced understanding of retroperitoneal anatomy and pathology. Conventional radiographic techniques are often incapable of detecting and/or characterising retroperitoneal abnormalities. Sonography may be limited by patient-dependent-factors. CT is unaffected by bowel gas and provides discrete cross-sectional images of the organs, fascial planes and retroperitoneal compartments, making it an ideal tool for assessment of retroperitoneal disease. In clinically stable patients MRT may be a useful modality for providing helpful and additional information in characterising retroperitoneal abnormalities. In this review article the diagnostic possibilities of benign not organ-related diseases of the retroperitoneum are described. This is intended to give the reader an insight into the etiology and distribution patterns of retroperitoneal fluid and gas collections as well as into diagnosis and differential diagnosis of benign retroperitoneal diseases. The diagnostic impact of the different imaging modalities is discussed.

  9. Case report: prenatally detected dumdbell-shaped retroperitoneal duplication cyst.

    PubMed

    May, D A; Spottswood, S E; Ridick-Young, M; Nwomeh, B C

    2000-10-01

    Enteric duplication cysts are infrequently located in the retroperitoneum. Such cysts are typically spherical or ovoid. We report a retroperitoneal duplication cyst with extension across the abdominal midline in a previously unreported dumbbell configuration. This is the third reported case of prenatally detected retroperitoneal enteric duplication cyst.

  10. Massive retroperitoneal hemorrhage owing to a ruptured adrenal cyst.

    PubMed

    Pasciak, R M; Cook, W A

    1988-01-01

    We report a rare case of massive retroperitoneal hemorrhage owing to rupture of an adrenal cyst after blunt abdominal trauma. A large retroperitoneal hematoma was evacuated and subtotal adrenalectomy was performed. Convalescence was uneventful. Histology revealed an endothelial-lined adrenal cyst suggesting a vascular or lymphatic etiology.

  11. A bronchogenic cyst, presenting as a retroperitoneal cystic mass.

    PubMed

    Govaerts, Kim; Van Eyken, Peter; Verswijvel, Geert; Van der Speeten, Kurt

    2012-01-01

    Bronchogenic cysts are mostly benign, congenital abnormalities originating from the remnants of the primitive foregut. A retroperitoneal location is rare. Due to the mostly asymptomatic behavior and the historical confusion regarding histology, an exact prevalence is not known. We present here a case report of a retroperitoneal bronchogenic cyst. A literature review was performed for cases of retroperitoneal bronchogenic cysts written in English. Anatomopathological criteria for inclusion were pseudo stratified, ciliated, columnar epithelium together with the presence of at least one of the following: cartilage, smooth muscle or seromucous glands. In addition, the embryology, pathogenesis, radiological, clinical and suggested treatment modalities are reviewed. We report the surgical excision of a retroperitoneal bronchogenic cyst that presented as a non-functioning left adrenal mass. Our review of literature revealed only 62 potential cases of retroperitoneal bronchogenic cysts. After applying the strict anatomopathological criteria, only 30 cases of true retroperitoneal bronchogenic cysts could be identified. Retroperitoneal location of a bronchogenic cyst is rare. Despite the rarity of this pathologic entity, bronchogenic cysts should be considered in the differential diagnosis of retroperitoneal cystic lesions. Only histology can confirm definitive diagnosis. Surgery remains the recommended treatment of choice.

  12. Retroperitoneal bronchogenic cyst presenting as an adrenal mass.

    PubMed

    Foerster, H M; Sengupta, E E; Montag, A G; Kaplan, E L

    1991-10-01

    Subdiaphragmatic bronchogenic cysts are rare, and those located retroperitoneally are exceptional. A review of the English-language literature revealed only three reported cases. We describe an additional case of a retroperitoneal bronchogenic cyst that presented uniquely as a symptomatic adrenal mass and discuss the cases of subdiaphragmatic bronchogenic cysts reported in the English-language literature.

  13. A laparoscopic method for excision of large retroperitoneal paraovarian cysts.

    PubMed

    Herbert, C M; Segars, J H; Hill, G A

    1990-01-01

    Large retroperitoneal paraovarian cysts often present in reproductive-age women as an adnexal mass, with the appearance of a simple cyst on ultrasound. This report describes a laparoscopic method for removal of large paraovarian retroperitoneal cysts. Use of this technique permits successful removal of these cysts while avoiding laparotomy.

  14. Bronchogenic cyst. A rare differential diagnosis of retroperitoneal tumors.

    PubMed

    Reichelt, O; Grieser, T; Wunderlich, H; Möller, A; Schubert, J

    2000-01-01

    Subdiaphragmatic bronchogenic cysts are rare, and those located retroperitoneally are exceptional. A review of the English-language literature revealed only 16 reported cases. We describe an additional case of a retroperitoneal cyst presenting as an asymptomatic adrenal mass and discuss clinical, radiographic, surgical and pathological findings as well as its embryological background.

  15. A bronchogenic cyst, presenting as a retroperitoneal cystic mass

    PubMed Central

    Govaerts, Kim; Van Eyken, Peter; Verswijvel, Geert; Van der Speeten, Kurt

    2012-01-01

    Bronchogenic cysts are mostly benign, congenital abnormalities originating from the remnants of the primitive foregut. A retroperitoneal location is rare. Due to the mostly asymptomatic behavior and the historical confusion regarding histology, an exact prevalence is not known. We present here a case report of a retroperitoneal bronchogenic cyst. A literature review was performed for cases of retroperitoneal bronchogenic cysts written in English. Anatomopathological criteria for inclusion were pseudo stratified, ciliated, columnar epithelium together with the presence of at least one of the following: cartilage, smooth muscle or seromucous glands. In addition, the embryology, pathogenesis, radiological, clinical and suggested treatment modalities are reviewed. We report the surgical excision of a retroperitoneal bronchogenic cyst that presented as a non-functioning left adrenal mass. Our review of literature revealed only 62 potential cases of retroperitoneal bronchogenic cysts. After applying the strict anatomopathological criteria, only 30 cases of true retroperitoneal bronchogenic cysts could be identified. Retroperitoneal location of a bronchogenic cyst is rare. Despite the rarity of this pathologic entity, bronchogenic cysts should be considered in the differential diagnosis of retroperitoneal cystic lesions. Only histology can confirm definitive diagnosis. Surgery remains the recommended treatment of choice. PMID:22532911

  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.

  17. 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 ...

  18. 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 ...

  19. 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 ...

  20. [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

  1. 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

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

    ClinicalTrials.gov

    2014-05-07

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

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

    ClinicalTrials.gov

    2016-10-04

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

  4. [Isolated retroperitoneal hydatid cyst. Apropos of 4 cases].

    PubMed

    el Ouakdi, M; Ben Fadhel, S; Ayed, M; Zmerli, S

    1988-01-01

    The isolated retroperitoneal cyst is rare. The hydatid cyst grow behind the posterior parietal peritoneum and have no connection with organs normally located within that space and lying next to them. Out of the 31 retroperitoneal tumors files collected over a five-year period, 25 are authentic primitive retroperitoneal tumors and 6 retroperitoneal hydatid cysts; 4 of which are isolated retroperitoneal localisations (neither pelvic subperitoneal localisation, nor multiple hydatic localisation are covered in this work). The abdominal mass is the most frequent sign, unfrequently concomitant with compressive syndrome. Diagnosis is based on urographic and echographic data. These data enabled a diagnosis to be reached in two cases and helped the diagnosis in one case. Surgery is the only treatment.

  5. 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

  6. [Unusual lung localization of histiocytic sarcoma].

    PubMed

    Aichaouia, C; Daboussi, S; Haddaoui, A; Moatemri, Z; Farah, S; Khadraoui, M; Bouzaiene, A; Cheikh, R

    2012-10-01

    Histiocytic sarcoma, proliferation araising from immunoregulatory effector system cells, is a very rare and recently recognised tumor. Diagnosis is based on immunohistochemistry and molecular genetic techniques, which allow to distinguish histiocytic sarcoma from lymphocytic proliferation, such as non-Hodgkin's. We report this rare case of multivisceral histiocytic sarcoma revealed by lung localization and for which the evolution was fatal.

  7. Granulocytic sarcoma masquerading as Ewing's sarcoma: a diagnostic dilemma.

    PubMed

    Haresh, Kunhi Parambath; Joshi, Nikhil; Gupta, Chaitali; Prabhakar, Ramachandran; Sharma, Daya Nand; Julka, Pramod Kumar; Rath, Goura Kishor

    2008-01-01

    An eleven-year-old boy presented with a swelling in his left elbow. Radiologically the features were that of an Ewing's sarcoma involving the ulna. Histopathology showed small round cell tumor strongly positive for Monoclonal Imperial Cancer research fund 2 (MIC2) antigen. Similar cells in the bone marrow were involved with MIC2 positivity. The patient developed skin lesions, which on biopsy were found to be chloromas. The initial biopsies were reevaluated with special stains revealing granulocytic sarcomas in acute myeloid leukemia masquerading as Ewing's due to its MIC2 positivity. The possibility of myeloid neoplasms should be considered routinely with known MIC2 positive round cell tumors. PMID:18923208

  8. Retroperitoneal pulmonary choristoma in a newborn calf.

    PubMed

    Bassi, Patrizia; Gentile, Arcangelo; Militerno, Gianfranco

    2010-11-01

    A newborn male Romagnola calf, who had died a few hours after birth, was submitted for postmortem examination. Necropsy revealed a 23 cm × 22 cm × 5 cm, pale pink, lobulated, elastic, partially fluctuant mass that protruded from the dorsal retroperitoneal space into the abdominal cavity, extending from the diaphragm to the left kidney. The mass consisted of mature pulmonary tissue and was consistent with a pulmonary choristoma. The gross and microscopic appearance of this rare tumor-like congenital lesion and the possible pathogenesis are discussed.

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

    PubMed

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

    2004-09-01

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

  10. Retroperitoneal cystic neuroendocrine tumor. A case report.

    PubMed

    Scapinello, A; D'Amore, E S; Cavazzana, A O; Gramegna, V; Ninfo, V

    1995-10-01

    A 21 cm retroperitoneal cystic mass was excised from a 71 year old woman. The cyst was filled with a hemorrhagic fluid and contained a 5 cm parietal hemorrhagic nodule. On histology, the nodule was composed of a uniform population of round cells arranged in trabeculae and nests. The neoplastic cells were immunoreactive to cytokeratin, EMA, NSE, chromogranin A, pancreatic polypeptide (PP) and Gastrin (G). Ultrastructural observation of neurosecretory granules confirmed the neuroendocrine nature of the tumor. No other lesions were detected and a diagnosis of primary epithelial neuroendocrine tumor was rendered. The histogenesis of the tumor including the possibility of a paraganglionic origin is discussed.

  11. A case of primary retroperitoneal mucinous cystadenocarcinoma.

    PubMed

    Gotoh, K; Konaga, E; Arata, A; Takeuchi, H; Mano, S

    1992-02-01

    A rare case of primary retroperitoneal mucinous cystadenocarcinoma in a 44-year-old woman is reported. The cystic tumor was delineated by CT and echography. The tumor was removed intact in the presence of bilateral normal ovaries and demonstrated an infiltrating malignant process. This neoplasm may have arisen from a supernumerary ovary. The patient died of recurrence 4 months after surgery. A comparison of the known cases indicates that aggressive treatment by hysterectomy with bilateral salpingo-oophorectomy in addition to cyst extirpation may improve prognosis.

  12. Retroperitoneal alimentary tract duplications detected in utero.

    PubMed

    Duncan, B W; Adzick, N S; Eraklis, A

    1992-09-01

    The prenatal diagnosis of numerous congenital anomalies has become routine. The prenatal diagnosis of cystic lesions of the retroperitoneum can be due to a variety of renal, gastrointestinal, or adrenal lesions. This finding demands aggressive postnatal follow-up to rule out the possibility of cystic adrenal neuroblastoma. We report the first cases of retroperitoneal cystic masses diagnosed in utero that ultimately proved to be enteric duplications. Therefore, the differential diagnosis of cystic masses of the retroperitoneum found prenatally should be expanded to include enteric duplication cysts.

  13. SYNOVIAL SARCOMA OF THE LARYNX.

    PubMed

    Javed, Nabila; Iqbal, Javed

    2015-01-01

    Synovial sarcoma is a mesenchymal spindle cell tumour that displays variable epithelial differentiation. It most commonly occurs in lower extremities. Head and neck is a rare site for synovial sarcoma accounting for less than 10%. Larynx is an extremely rare site and only 16 cases with laryngeal location have been reported. Immunohistochemistry is important for correct diagnosis. Surgical excision of the tumour with clear margins and local radiotherapy is effective in local control. Chemotherapy is indicated in the presence of distant metastasis. Case of a 16 years old female is presented with hoarseness of voice and mass in supraglottic region. Lateral pharangotomy and excision of mass revealed synovial sarcoma. She had been treated with adjuvant radiotherapy in September 2012. She was fine and coming for regular follow up.

  14. Testicular myeloid sarcoma: case report

    PubMed Central

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

    2013-01-01

    Myeloid sarcomas are extramedullary solid tumors composed of immature granulocytic precursor cells. In association with acute myeloid leukemia and other myeloproliferative disorders, they may arise concurrently with compromised bone marrow related to acute myeloid leukemia, as a relapsed presentation, or occur as the first manifestation. The testicles are considered to be an uncommon site for myeloid sarcomas. No therapeutic strategy has been defined as best but may include chemotherapy, radiotherapy and/or hematopoietic stem cell transplantation. This study reports the evolution of a patient with testicular myeloid sarcoma as the first manifestation of acute myeloid leukemia. The patient initially refused medical treatment and died five months after the clinical condition started. PMID:23580888

  15. Unusual uptake of radioiodine in a retroperitoneal bronchogenic cyst in a patient with thyroid carcinoma.

    PubMed

    Jiang, Xue; Zeng, Hao; Gong, Jing; Huang, Rui

    2015-05-01

    The incidence of retroperitoneal bronchial cyst is rare and is unusual to be visualized on I scan. We report a 52-year-old man who received I therapy for papillary thyroid cancer. The postablation whole-body scan revealed increased radioiodine activity in the left abdomen. SPECT/CT localized this activity from a soft tissue mass in the retroperitoneal space. A retroperitoneal tumor was considered, and retroperitoneal laparoscopic tumor resection was performed. Pathologic examination confirmed a retroperitoneal bronchogenic cyst.

  16. Spontaneous Retroperitoneal Haematoma due to Polyarteritis Nodosa: Report of a Case and Literature Review

    PubMed Central

    Simsek, Emrah; Yilmaz, Hasan; Teke, Kerem; Uslubas, Ali Kemal; Yuksekkaya, Mustafa

    2016-01-01

    Retroperitoneal haematoma is a rare clinical entity with variable etiology. It can happen spontaneously, without any obvious precipitating factors, the so-called spontaneous retroperitoneal haematoma. There is no general consensus as to the best management plan for patients with retroperitoneal haematoma. Polyarteritis nodosa (PAN) is a rare cause of retroperitoneal haematoma. Here we report relationship between PAN and retroperitoneal haematoma and treatment approaches. However, an accepted and clearly defined treatment has not been established due to its rarity. PMID:26885433

  17. 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.

  18. 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

  19. 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

  20. Targeted Therapy of Ewing's Sarcoma

    PubMed Central

    Subbiah, Vivek; Anderson, Pete

    2011-01-01

    Refractory and/or recurrent Ewing's sarcoma (EWS) remains a clinical challenge because the disease's resistance to therapy makes it difficult to achieve durable results with standard treatments that include chemotherapy, radiation, and surgery. Recently, insulin-like-growth-factor-1-receptor (IGF1R) antibodies have been shown to have a modest single-agent activity in EWS. Patient selection using biomarkers and understanding response and resistance mechanisms in relation to IGF1R and mammalian target of rapamycin pathways are areas of active research. Since EWS has a unique tumor-specific EWS-FLI1 t(11;22) translocation and oncogenic fusion protein, inhibition of EWS-FLI1 transcription, translation, and/or protein function may be key to eradicating EWS at the stem-cell level. Recently, a small molecule that blocks the protein-protein interaction of EWS-FLI1 with RNA helicase A has been shown in preclinical models to inhibit EWS growth. The successful application of this first-in-class protein-protein inhibitor in the clinic could become a model system for translocation-associated cancers such as EWS. PMID:21052545

  1. Extra osseous primary Ewing's sarcoma.

    PubMed

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

    2010-01-01

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

  2. 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.

  3. 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

  4. [Pelvic retroperitoneal teratoma. A case report].

    PubMed

    Becerril-González, Ana Norma Gricelda; Pérez-Martínez, Andrés; Sereno-Coló, José Antonio

    2013-12-01

    Teratomas are congenital tumors comprising of tissues arising from pluripotent embryonic cells. Their common sites of occurrences are ovary, testis, mediastinum and retroperitoneum. Retroperitoneal teratomas are extremely rare in adults but occur in 3% of children. The presence of a cystic or complex adnexal mass during pregnancy is common with mature teratoma reported in approximately 30-50% of pregnancies. The ultrasound is the number one method of detection. The use of tumor markers is limited because it alters its value during pregnancy. We report a case of a 18 year old female patient who came to the General Hospital Dr. Miguel Silva in Morelia, Michoacán with a gestation age of 10.2 weeks and a recent diagnosis of an asymptomatic adnexal mass. Using the transvaginal approach, the ultrasound reported an irregular gestational sac with the absence of cardiac activity in the embryo. A diffusely echogenic adnexal mass with oval appearance, wall regularity and homogeneous internal echos of 12.96 x 8.99 cm. Exploratory laparotomy was performed finding normal uterus and ovaries, with the posterior retroperitoneal resection of the 15 cm multi-lobed tumor. The surgical specimen measured 13 x 12 x 8 cm, had a brownish color, wall irregularity, soft consistency, multi-lobed, and a yellowish interior greasy material. Microscopically the tumor is delimited with a firm capsule composed of a hyaline-fibrous tissue, lined by squamous keratinized epithelium, with multiple layers of keratin. This anato-pathologic finding is diagnostic of retroperitoneum mature teratoma.

  5. A large retroperitoneal malignant solitary fibrous tumor.

    PubMed

    Yoh, Tomoaki; Sata, Ritsuko; Kobayashi, Atsushi; Wada, Seidai; Nakamura, Yuya; Kato, Tatsushi; Nakayama, Hiroyuki; Okamura, Ryuji

    2014-01-01

    We report on a large, retroperitoneal, malignant, solitary fibrous tumor (SFT) with high proliferation activity. A 43-year-old man was admitted to our department complaining of a palpable mass. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a large retroperitoneal tumor occupying the entire abdominal cavity. A laparotomy was performed for diagnosis and treatment, which revealed a tumor in the retroperitoneum but with no invasion to the surrounding organs, thereby allowing safe macroscopic excision. Histologically, the tumor was composed of spindle-shaped cells with patternless pattern and a hemangiopericytomatous appearance. Moreover, immunohistochemical staining was positive for CD34, vimentin, Bcl-2, and CD99 and negative for desmin, S-100p, and smooth muscle actin (AMA). The tumor exhibited high cellularity, moderate mitotic activity, pleomorphism, necrosis, and hemorrhagic changes. In addition, the Ki-67 labeling index was 37%. These findings confirmed the diagnosis of malignant SFT with high proliferation activity. Subsequently, adjuvant doxorubicin plus ifosfamide chemotherapy was performed. No signs of recurrence were observed 12 months after the surgery.

  6. [A case of retroperitoneal solitary fibrous tumor].

    PubMed

    Anchi, Takashi; Tamura, Kenji; Inoue, Keiji; Fukata, Satoshi; Nishikawa, Hiroshi; Moriki, Toshiaki; Shuin, Taro

    2009-07-01

    Solitary fibrous tumor (SFT) is a common neoplasm of the pleura and rarely arises from the retroperitoneal space. We report a case of retroperitoneal SFT. A 54-year-old woman was admitted to the department of dermatology in our hospital for examination and treatment of malignant melanoma of the right lower leg. Computerized tomography (CT) showed the left adrenal mass measuring 6.0 x 4.5 x 10 cm. Because the tumor was thought to be left adrenal tumor or left adrenal metastasis of the malignant melanoma, she admitted to our department. Left radical adrenalectomy was performed. The histological examination of the specimen revealed that the spindle or oval cells proliferated patternless with hyalinized collagen cells and there were few mitotic figures. The tumor was not connected to the adrenal gland. The immunohistochemical examination showed that the tumor cells were diffusely positive for CD34, bcl-2 and vimentin. According to these findings, we diagnosed SFT arising from the retroperitoneum. At 5 years follow up she had no evidence of local recurrence and no distant metastasis.

  7. 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

  8. 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

  9. Stewart-Treves syndrome following idiopathic leg lymphoedema: remember sarcoma.

    PubMed

    Harrison, W D; Chandrasekar, C R

    2015-06-01

    Patients with skin complaints secondary to oedema are commonly encountered and can be a diagnostic challenge. Here, we present the case of a 78-year-old lady with Stewart-Treves syndrome, a rare cutaneous angiosarcoma. The histology showed angiocutaneous sarcoma with poorly defined margins. The prognosis remains extremely poor. A successful transfemoral amputation reduced the massive tumour burden and cleared a source of sepsis from this fungating tumour. Our aim is to highlight this rare but high-grade sarcoma which results from chronic lymphoedema. The red flag signs of sarcoma are masses greater than 5 cm, tumours which are rapidly growing, deep to fascia or painful, and tumours that recur after previous surgery. Non-healing or progressive ulceration in the background of chronic lymphoedema should raise the possibility of underlying malignancy. We need a low threshold for biopsy and early referral to an appropriate multidisciplinary team for the optimum management of the patient. PMID:26075513

  10. A rare cause of mechanical obstruction: Intestinal myeloid sarcoma

    PubMed Central

    Yoldaş, Tayfun; Erol, Varlık; Demir, Batuhan; Hoşcoşkun, Cüneyt

    2014-01-01

    Myeloid sarcoma is a rare aggressive tumour that originates from immature extramedullary myeloid cells. It can be seen as a relapse in patients with acute myeloid leukaemia. Sometimes it can be seen in the form of a solid tumour without any evidence of leukaemia. A case of a 44-year-old male patient who was admitted with symptoms and signs of mechanical intestinal bowel obstruction was operated on. The operation findings showed small bowel obstruction due to a mass. The mass was then resected with end-to-end intestinal anastomosis. The resected mass pathology results were consistent with myeloid sarcoma. The post-operative period was uneventful and adjuvant therapy was applied. In this case report we aimed to evaluate the clinical signs and treatment modalities of small intestinal myeloid sarcoma. PMID:25931908

  11. 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

  12. 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.

  13. Prognostic features of renal sarcomas (Review)

    PubMed Central

    ÖZTÜRK, HAKAN

    2015-01-01

    The aim of the present review was to evaluate the prognostic features of primary sarcomas of the kidney. A literature review was conducted using a number of databases, including Medline (PubMed) and Scopus, for studies published between January 1992 and December 2013. Of the studies published in English, those describing the prognostic features of primary sarcomas of the kidney were recorded. The electronic search was limited to the following keywords: Sarcoma, renal sarcoma, prognosis, diagnosis, immunohistochemistry, genetic and survey. Subsequent to the search, no review articles and/or meta-analyses associated with the prognosis of primary sarcomas of the kidney were identified. In total, 31 studies, which consisted of case studies, case series and studies concerned with the overall prognosis of urological soft-tissue sarcomas, were reviewed. Primary sarcoma of the kidney has a poor prognosis compared with other sarcomas of the urogenital system. In addition to the surgical excision of renal sarcomas, pathological, molecular and genetic prognostic factors are also considered. Due to the small number of cases, previous studies have not randomized the prognostic features of primary sarcomas of the kidney. The elucidation of the so-called ‘chaotic’ genetic and molecular basis of renal sarcomas will help to predict patient prognoses. Surgical excision is the most significant parameter for determining the prognosis of sarcomas of the kidney. However, sarcomas also exhibit prognostic features that are based upon pathological, genetic and molecular factors. The present review suggests that additional factors may be important in predicting the prognosis of patients with renal sarcomas, and that clinicians should plan treatment and follow-up regimens according to these factors. PMID:25663853

  14. 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

  15. 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

  16. Kaposi's sarcoma of the penis.

    PubMed

    Houston, W; Pontin, A; Kuhn, T; Mambo, N

    1975-06-01

    The 11th case of Kaposi's sarcoma of the glans penis is reported. Aetiology and treatment are discussed and evidence adduced to support a conservative surgical approach by local excision, supplemented if necessary by low dosage irradiation and possibly methotrexate infusion.

  17. 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

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

    PubMed

    Mirilas, Petros; Skandalakis, John E

    2010-02-01

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

  19. 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

  20. Surgical considerations of the renal fascia and the retroperitoneal space around the kidney.

    PubMed

    Takahashi, Ryosuke; Furubayashi, Nobuki; Nakamura, Motonobu; Hasegawa, Yoshihiro

    2012-07-01

    Surgical anatomy around the kidney remains controversial. We therefore examined the constitutions of renal fascia and retroperitoneal space around the kidney through the close observations of intraoperative views. The surface of the removed kidney was covered with a smooth membrane, which is the so-called renal fascia. However, such a smooth membrane could not be observed at the dissection site around the kidney during surgery. Only an intricate connective tissue could be observed. On the other hand, using an operative procedure such as dissection or pulling tissue in some direction, an intricate connective tissue changed to a membranous structure. These results suggest that the retroperitoneal area around the kidney would be filled with connective tissue including some fat, which is arranged in a chaotic manner without any specific alignment tridimentionally. As a result of operative procedures, such connective tissue would be grouped, which would be recognized as renal fascia.

  1. 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.

  2. 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

  3. [Retroperitoneal cyst: a case report].

    PubMed

    Yamaguchi, K; Okasho, A; Hisazumi, H

    1984-12-01

    A 71-year-old woman who had an abdominal mass was admitted to our hospital in May, 1983. Physical examination revealed the presence of a soft mass without tenderness in the right upper abdomen. The results of laboratory tests were within the normal range. An excretory urogram showed a slight lateral displacement of the upper position of the right ureter and a slight dilatation of the right pyelocalyceal system. The urogram for the left kidney and ureter was normal. Abdominal CT scan revealed a cystic homogeneous mass anterior to the right kidney, 12 X 11 cm in size. Ultrasonography showed a cystic mass corresponding to the CT scan finding. Under ultrasonographic guidance, percutaneous puncture of the cyst was performed, and 800 ml clear straw-yellow fluid containing 457 ng/ml alpha-fetoprotein was drained. A repeat CT scan 9 months later showed no evidence of fluid reaccumulation and the patient remained asymptomatic.

  4. A Massive Intratumoral Aneurysmal Vessel in a Retroperitoneal Lipoblastoma.

    PubMed

    Moon, Suk-Bae

    2015-12-01

    Lipoblastoma is a benign tumor and usually does not require radical operation for complete excision. We describe here a case of a retroperitoneal lipoblastoma with a massive intratumoral aneurysmal vessel.

  5. 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.

  6. A patient with combined mediastinal, mesenteric and retroperitoneal fibrosis.

    PubMed

    Graal, M B; Lustermans, F A

    1994-06-01

    We report a patient with combined mediastinal, mesenteric and retroperitoneal fibrosis who first presented with signs of a superior vena cava syndrome. She was successfully treated with corticosteroids. The aetiology, clinical picture, and possible therapy of idiopathic fibrosclerosis are discussed.

  7. [A case of retroperitoneal schwannoma of the vagus nerve].

    PubMed

    Yoo, Byoung Kwan; Yoo, Kyo Sang; Park, Chul Sung; Lee, Jung Wha; Yoo, Ji Youn; Moon, Joon Ho; Jung, Jae One; Kim, Jong Pyo; Kim, Kyoung Oh; Park, Cheol Hee; Hahn, Tae Ho; Park, Sang Hoon; Kim, Jong Hyeok; Min, Soo Kee; Yang, Dae Hyun; Park, Choong Kee

    2005-10-01

    Schwannomas are benign nerve sheath tumors that originate from any anatomical site. Most schwannomas occur in the head, neck or limbs, but rarely occur in the retroperitoneal space. Furthermore, the schwannoma originating from the vagus nerve of retroperitoneal space is much rare. We experienced a case of retroperitoneal schwannoma of the vagus nerve. A 34-year-old male was referred to our hospital for the evaluation of abdominal mass on ultrasonography. Endoscopic examination revealed submucosal tumor-like lesion on high body of the stomach. Computed tomography (CT) revealed that the stomach was compressed by a solid tumor in the retroperitoneum. On exploratory laparotomy, this mass turned out to be a baseball sized mass in the retroperitoneal space. The mass was excised in an encapsulated state. Histological examination with immunohistochemical stains revealed a schwannoma of the vagus nerve.

  8. Primary hydatid cyst in the retroperitoneal space: role of CT.

    PubMed

    Yilmaz, Sevda; Baydar, Senol; Guney, Sefik; Hoser, Abdurrahman; Celik, Betül

    2002-09-01

    Localization of hydatid disease in the retroperitoneal space is extremely rare. We present the CT findings in a patient with echinococcal disease (E. Granulosus) in whom the cyst was localized to the retroperitoneum.

  9. [Primary retroperitoneal cyst in a 21-year-old woman].

    PubMed

    Thunedborg, L P; Rudnicki, P M

    1990-08-20

    A case of primary retroperitoneal cyst in a woman aged 21 years is presented. The cyst, which contained 6.5 litres, was treated by total excision. The diagnosis, treatment and classification are briefly described.

  10. [Retroperitoneal cystic lymphangioma. Preoperative diagnosis. Apropos of a case].

    PubMed

    Guérin, E; Babin, C; Moulle, P; Barret, F

    1987-11-01

    Retroperitoneal cystic lymphangioma is an uncommon tumor. One case is reported, with surgical confirmation. Authors point out the diagnostic value of ultrasonography and computed tomography in the pre-operative diagnosis.

  11. [A case of retroperitoneal serous cyst with ossification].

    PubMed

    Chihara, Yoshitomo; Horikawa, Naoki; Hayashi, Yoshiki; Fujimoto, Kiyohide; Hosokawa, Yukinari; Hirao, Yoshihiko

    2002-05-01

    A 36-year-old woman came to our hospital complaining of right flank pain. Computed tomographic (CT) scanning showed a cystic mass, 6 x 9 cm in size, including homogeneous low-density fluid contents, in the right retroperitoneal space. The cyst wall showed partly high-density epithelium, but there was no contrast enhancement. A 7.5 x 12 cm retroperitoneal cyst was easily removed with yellow serous fluid in it. Cytological examination showed no malignant cells in this fluid. The origin of the cyst was unknown. The histopathological diagnosis was retroperitoneal serous cyst with focal ossification in the lining epithelium. Here we report this rare case of retroperitoneal serous cyst and briefly discuss 57 cases reported in Japan.

  12. [Primary echinococcal retroperitoneal cysts. (Observation of a case)].

    PubMed

    Signorelli, I; Capelli, G; Andreoni, G M; Molinari, M; Maurizio, S; Porsio, G

    1995-01-01

    The Authors, after a careful review of the international literature, report one case of retroperitoneal primitive echinococcus cyst. They describe the anatomo-pathological and clinically specific qualities of this rare condition and emphasize the importance of radical surgical therapy.

  13. [Cysts and cyst-like formations of the abdominal cavity and retroperitoneal space in children].

    PubMed

    Fuchko, V I; Bibliuk, I I; Martyniuk, N A; Koturbash, R I; Matiiash, Ia V

    1992-01-01

    The results of surgical treatment of 29 children with cysts, and 10--with cyst-like formations of the abdominal cavity and retroperitoneal space are described. In majority of the patients, a cyst at the terminal stage was revealed. In presence of complications, they were operated on with tentative diagnosis of acute appendicitis, or ileus. The operation consisted of cyst enucleation, its elimination with resection, or removal of the organ, creation of cystodigestive anastomosis. Postoperative lethality was 12.8%.

  14. [Sonographic study of the spleen and retroperitoneal space-occupying masses].

    PubMed

    Kathrein, H; Judmaier, G

    1983-10-31

    Sonography of the spleen allows exact measurements of this organ. There are typical pictures in cysts or hematomas. Diffuse changes of the spleen however are hardly distinguishable in regard to differential diagnosis. Retroperitoneal masses can easily be recognized. Lymph-nodes can be identified and the results of therapeutic interventions can be judged. Hematomas or abscesses can be seen easily. Guided needle biopsy offers the possibility for histologic examinations.

  15. Essential steps in the performance of safe retroperitoneal aortic surgery.

    PubMed

    Conway, K; Williams, I M

    2016-10-01

    Endovascular stent grafting has become the primary modality when assessing patients for treatment of abdominal aortic aneurysms (AAA). The traditional open approach is transperitoneal (TP) but many suggest the retroperitoneal method (RP) has significant benefits. Retroperitoneal aortic surgery may be unfamiliar to many surgeons as they have been trained in the TP approach. This paper provides specific tips for the critical steps of this approach enabling it to be performed with ease and minimal morbidity. PMID:26211699

  16. Essential steps in the performance of safe retroperitoneal aortic surgery.

    PubMed

    Conway, K; Williams, I M

    2016-10-01

    Endovascular stent grafting has become the primary modality when assessing patients for treatment of abdominal aortic aneurysms (AAA). The traditional open approach is transperitoneal (TP) but many suggest the retroperitoneal method (RP) has significant benefits. Retroperitoneal aortic surgery may be unfamiliar to many surgeons as they have been trained in the TP approach. This paper provides specific tips for the critical steps of this approach enabling it to be performed with ease and minimal morbidity.

  17. Retroperitoneal gastric duplication cyst: a case report and literature review.

    PubMed

    Pachl, Max; Patel, Kamlesh; Bowen, Claire; Parikh, Dakshesh

    2012-01-01

    A rare case of retroperitoneal gastric duplication is reported and discussed. An intra-abdominal cyst was detected at 31 weeks gestation and was followed up prenatally as a left sided duplex kidney. Post-natal ultrasound however, showed a normal kidney, but a cyst with features of enteric duplication in the left upper quadrant adjacent and compressing the kidney. Surgery was carried out during infancy and a retroperitoneal cyst was excised that contained heterotrophic gastric mucosa.

  18. Adrenal and pancreatic presentation of subdiaphragmatic retroperitoneal bronchogenic cysts.

    PubMed

    El Youssef, Raphael; Fleseriu, Maria; Sheppard, Brett C

    2010-03-01

    Bronchogenic cysts are aberrations of normal development throughout the embryonic foregut; abdominal or retroperitoneal presentations are rare. They will often present a diagnostic dilemma because their appearance can mimic other, more common diagnoses. The initial work-up, differential diagnosis, management, and follow-up may present clinical challenges. We present 1 case of an adrenal lesion and 1 case of a pancreatic lesion that were revealed to be retroperitoneal bronchogenic cysts after surgical extirpation.

  19. Retroperitoneal bronchogenic cyst masquerading clinically and radiologically as a phaeochromocytoma.

    PubMed

    Doggett, R S; Carty, S E; Clarke, M R

    1997-07-01

    Bronchogenic cysts are relatively rare congenital anomalies that represent malformations of the embryonic foregut and are morphologically expressed as maldevelopments of the respiratory system. Anatomically, they can be positioned at any location along the central axis of the respiratory system, but are more commonly discovered in the thorax. Infradiaphragmatic bronchogenic cysts are rare and retroperitoneal ones distinctly unusual. We report a retroperitoneal bronchogenic cyst clinically masquerading as a phaeochromocytoma.

  20. Developmental retroperitoneal cysts of the pelvis. A review.

    PubMed

    Ravo, B; Metwally, N; Pai, P B; Ger, R

    1987-07-01

    Retroperitoneal cysts have been classified into traumatic, infective, degenerative, neoplastic, and developmental, according to their origin. This paper focuses on the developmental variety of retroperitoneal pelvic cysts, particularly those of mesothelial, mesonephric, or paramesonephric origin. Their clinical presentations depend on their location; they may be mesenteric, parovarian, or vaginal. The pathogenesis and embryologic, diagnostic, and therapeutic aspects are reviewed and a case report is presented.

  1. Primary retroperitoneal hydatid cyst with unusual clinical manifestation.

    PubMed

    Gürdal, Mesut; Kireççi, Sinan; Yücebaş, Ergin; Karaman, M Ihsan

    2004-01-01

    Although hydatid disease may affect any organ of the body, there are only a limited number of cases where the primary lesion is in the retroperitoneal region. Since the clinical and laboratory findings are nonspecific, the correct preoperative diagnosis may be difficult. A primary retroperitoneal hydatid cyst that was given a preoperative diagnosis of adrenal mass and treated by adrenalectomy and partial nephrectomy is presented and discussed with the relevant literature.

  2. Chronic Low Back Pain due to Retroperitoneal Cystic Lymphangioma.

    PubMed

    Fattahi, Asieh Sadat; Maddah, Godratollah; Motamedolshariati, Mohammad; Ghiasi-Moghadam, Taghi

    2014-03-01

    Abdominal cystic lymphangioma is a rare benign neoplasm. Less than 1% of lymphangiomas is in the retroperitoneum. Lymphangioma is mostly asymptomatic. Chronic symptoms were reported in retroperitoneal type more than others. Acute symptoms due to complications like infection, cyst rupture or hemorrhage may occur. We report an 18-years-old girl with low back pain from 6 months ago with huge pelvic mass and diagnosis of retroperitoneal cystic lymphangioma.

  3. An unusual presentation of a retroperitoneal cyst.

    PubMed

    Sarkar, Debashis; Gulur, Dev; Patel, Snehal; Nambirajan, Thiagarajan

    2014-01-01

    A 34-year-old woman presented to the surgical assessment unit with severe right loin to groin pain. An ultrasound scan of the abdomen revealed a complex cyst in the right iliac fossa and a subsequent CT scan revealed a 7.5 cm retroperitoneal cystic lesion below the lower pole of the right kidney. The patient also had MRI of the kidneys, which confirmed the finding. The image showed the cyst was not attached to the kidneys and was clearly separate. She underwent a laparoscopic excision of the cyst. Histopathology revealed a cyst lined by a single layer of mucinous epithelium of endocervical type with foci of calcification and hyalinisation on the wall. The cyst was thought to be a benign cyst of Mullerian origin.

  4. [Retroperitoneal hydatidosis secondary to hepatic hydatid cyst].

    PubMed

    Vizcaychipi, Katherina A; Sosa, Sonia; Camicia, Federico; Santillán, Graciela; Casalins, María; Nigro, María Del Carmen

    2012-01-01

    Hydatid disease is a worldwide zoonosis. It is caused by a parasitic platyhelminth of the genus Echinococcus. We present a patient with a fluctuating lumbar tumor in the retroperitoneal space, secondary to a hepatic cyst. the initial diagnosis was made by identification of rostellar hooks from protoscoleces in the fluid aspirated from the abscess. We herein describe the clinical manifestations, diagnosis and medical and surgical treatment of this unusual case and conclude that the development of an accurate diagnosis requires a proper analysis of the patient's epidemiological history, clinical manifestations, imaging studies and laboratory tests. a multidisciplinary approach and differential diagnosis is paramount to be able to establish a cause of the disease to deliver appropriate treatment.

  5. Retroperitoneal Bronchogenic Cyst Originating from Diaphragmatic Crura.

    PubMed

    Herek, Duygu; Erbiş, Halil; Kocyigit, Ali; Yagci, Ahmet Baki

    2015-12-01

    Bronchogenic cyst is a benign lesion which is commonly seen in the posterior mediastinum. Diaphragmatic origin in retroperitoneum is an unusual location for a bronchogenic cyst. Cross-sectional imaging modalities describe the origin and content of the cyst evidently. Magnetic resonance (MR) images of a 42-year-old male patient who attended ER with back pain revealed a huge retroperitoneal complicated bronchogenic cyst arising from the diaphragm and surrounding the abdominal aorta anteriorly. Bronchogenic cysts in the retroperitoneum rarely originate from the diaphragm and should be kept in mind in the differential diagnoses of abdominal cystic lesions. MR imaging (MRI) is superior to other imaging techniques such as computerized tomography (CT) in detecting the origin and content of these cystic lesions.

  6. An unusual presentation of a retroperitoneal cyst

    PubMed Central

    Sarkar, Debashis; Gulur, Dev; Patel, Snehal; Nambirajan, Thiagarajan

    2014-01-01

    A 34-year-old woman presented to the surgical assessment unit with severe right loin to groin pain. An ultrasound scan of the abdomen revealed a complex cyst in the right iliac fossa and a subsequent CT scan revealed a 7.5 cm retroperitoneal cystic lesion below the lower pole of the right kidney. The patient also had MRI of the kidneys, which confirmed the finding. The image showed the cyst was not attached to the kidneys and was clearly separate. She underwent a laparoscopic excision of the cyst. Histopathology revealed a cyst lined by a single layer of mucinous epithelium of endocervical type with foci of calcification and hyalinisation on the wall. The cyst was thought to be a benign cyst of Mullerian origin. PMID:25368127

  7. Retroperitoneoscopic resection of retroperitoneal bronchogenic cysts.

    PubMed

    McCrystal, David J; Borzi, Peter A J

    2002-09-01

    Two children aged 8 and 15 years underwent excision of retroperitoneal bronchogenic cysts (RBC) via a posterior retroperitoneoscopic approach (RPA). The operative technique and efficacy of this approach are reviewed. One lesion was symptomatic, while the other was an incidental finding on abdominal ultrasound. Operating times were 45 and 55 min. One patient was discharged on the day of surgery, the other on the 1st postoperative day. Both returned to normal physical activities within 1 week of surgery. No complication occurred in either case. To our knowledge, this is the first description in the literature of excision of this rare lesion via a RPA. We conclude that this is a safe method for resection of RBCs that offers several potential advantages compared with the open technique.

  8. Laparoscopic observation of retroperitoneal cystic lymphangioma.

    PubMed

    Takeuchi, Y; Fujinami, S; Kitagawa, S; Shiro, T; Fujii, T; Fukui, Y; Kawashima, Y; Kubota, Y; Shiozaki, Y; Inoue, K

    1994-01-01

    A case of retroperitoneal cystic lymphangioma observed laparoscopically is reported. In a 60 year old asymptomatic male patient, an 8 cm multi-loculated cystic lesion was detected incidentally near the splenic hilum with ultrasonography. Endosonography, computed tomography and magnetic resonance imaging revealed the lesion with thin wall and clear fluid. Laparoscopy showed a thin-walled cyst with smooth surface, and straw-coloured clear fluid was observed through the wall. These findings suggested benign aetiology, and seemed to be characteristic of cystic lymphangioma. The tumour was resected, and microscopic examination showed proliferated lymph channels intespersed by lymph follicles. Diagnosis of cystic lymphangioma was established. Laparoscopy seems a useful pre-operative method.

  9. Retroperitoneal malignant fibrous histiocytoma without histological atypias.

    PubMed

    Laky, D; Penciu, M; Rădulescu, D

    1993-01-01

    A case of a 36-year-old woman suspected of hepatic hydatid cyst with dyseptic symptoms, hepatomegalia, profound pains in the right hypochondrium is presented. During the operation a 15/12/17 cm well-incapsulated tumour is extirpated. The tumour stretched from the upper right renal pole behind the right hepatic lobe as far as the diaphragm and while sectioning it was grey and orange. Histopathologically we found a fibrous histiocytoma with zones of storiform fascicular pattern alternating with areas of histocytic cells with vacuolar-foamy cytoplasm, rich in lipids and without atypias or mitoses. We report the frequency, histological aspects of the fibrous histiocytomas as well as some particularities of their retroperitoneal location.

  10. Retroperitoneal bronchogenic cyst: a case report.

    PubMed

    Ingu, Akira; Watanabe, Atsushi; Ichimiya, Yasunori; Saito, Tatsuya; Abe, Tomio

    2002-04-01

    A 46-year-old woman presented with a 1-year history of progressive left-arm numbness. A cyst below the left hemidiaphragm was discovered incidentally when a CT scan was performed to examine the thymus for a suspected tumor. A thymic mass was found. MRI indicated that the cyst contained proteinaceous fluid. The thymoma was approached through a median sternotomy and resected, but the cyst was found to be infradiaphragmatic. A separate, left paraspinal incision was made to access the retroperitoneum, and the cyst was resected. Histologic examination showed that the cyst was bronchogenic in origin. Retroperitoneal bronchogenic cysts are very rare, and only four cases have been reported in the English-language literature.

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

    ClinicalTrials.gov

    2016-10-31

    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

  12. [Prenatal sonographic diagnosis of a cystic coccygeal teratoma with retroperitoneal extension].

    PubMed

    Goldhofer, W; Merz, E; Bauer, H; Koltai, I L

    1986-02-01

    The most common teratoma of th newborn is the sacrococcygeal, which contains tissues of all three germinal layers. It is located over and under the sacrum or the coccyx and may extend in front of the sacrum into the retroperitoneal region. The teratoma can lead to compression of the ureters with resultant hydronephrosis or to obstruction of the rectum. The most important complication is the malignant change which usually occurs between the fourth month and the third year. Because of the complications early diagnosis of the teratoma is necessary and extirpation should be carried out as soon as possible. The present paper reports on the sonographic findings in a sacrococcygeal teratoma with retroperitoneal extension at 37 weeks gestation. The aetiology and pathogenesis of this malformation and the clinical findings are discussed.

  13. Hyperthermia in soft tissue sarcoma.

    PubMed

    Lindner, Lars H; Issels, Rolf D

    2011-03-01

    Patients with high-risk soft tissue sarcomas (STS)-FNCLCC grade 2-3, size >5 cm, deep to the fascia-are at risk for developing local recurrence and distant metastasis despite surgical tumor resection. Therefore, the management of high-risk STS requires a multidisciplinary approach. Besides surgery, radiotherapy, and chemotherapy, regional hyperthermia (RHT) has the potential to become the fourth standard treatment modality for the treatment of these patients. RHT means non-invasive selective heating of the tumor area to temperatures within the range of 40-43°C for 60 min by the use of an electromagnetic heating device. Thereby RHT is always applied in addition to radiotherapy or chemotherapy or both but is not effective as a single treatment. Beside direct cytotoxicity, RHT in combination with chemotherapy enhances the drug cytotoxicity mainly by increased chemical reaction and intratumoral drug accumulation. For the neoadjuvant setting, RHT in combination with a doxorubicin- and ifosfamide-based chemotherapy has been shown to dramatically improve the tumor response rate but also prevents from early disease progression as compared to chemotherapy alone. The addition of RHT to a multimodal treatment of high-risk STS consisting of surgery, radiotherapy, and chemotherapy either in the neoadjuvant setting but also after incomplete or marginal tumor resection has been shown to significantly improve local recurrence- and disease-free survival. Based on these results and in conjunction with the low RHT-related toxicity, RHT combined with preoperative or postoperative chemotherapy should be considered as an additional standard treatment option for the multidisciplinary treatment of locally advanced high-grade STS.

  14. Report of a case: Retroperitoneal mucinous cystadenocarcinoma with rapid progression

    PubMed Central

    Kamiyama, Hirohiko; Shimazu, Ai; Makino, Yurika; Ichikawa, Ryosuke; Hobo, Takahiro; Arima, Shuei; Nohara, Shigeo; Sugiyama, Yuji; Okumura, Masafumi; Takei, Masahiko; Miura, Hiroyoshi; Namekata, Koji; Tsumura, Hidenori; Okada, Motoi; Takase, Masaru; Matsumoto, Fumio

    2015-01-01

    Introduction Retroperitoneal mucinous cystic neoplasms are uncommon, and little is known about the etiology of the disease. Malignant forms of these are extremely rare. Here, we report a case of primary retroperitoneal mucinous cystadenocarcinoma (PRMC), which demonstrated unexpectedly aggressive progression despite finding only a limited area of adenocarcinoma. Presentation of case A 62-year-old woman with a complaint of abdominal discomfort was admitted to the hospital. Abdominal CT and MRI showed multiple large retroperitoneal cysts dislocating the right kidney nearly to the center of the abdomen. Transabdominal resection of the cysts was performed. Those cysts contained 1100 ml of mucinous fluids in total. Cytological examination of those fluids revealed no malignant cells. The cyst wall was lined with mucinous epithelial cells, and contained some ovarian-type stroma. Also, there was a focal area of adenocarcinoma in the cyst wall, and the lesion was diagnosed as primary retroperitoneal mucinous cystadenocarcinoma. Eight months later, the patient developed lumbar bone metastasis. Chemotherapy with S-1, an oral fluoropyrimidine, and docetaxel had been begun immediately; however, the disease had rapidly spread in the retroperitoneum. Eventually, the patient died of the disease 15 months after surgery. Discussion Retroperitoneal mucinous cystic neoplasms are considered to be metaplasia of embryonal coelomic epithelium. Complete excision without rupture is essential. However, variance of biological aggressiveness might exist in PRMCs. Conclusion Retroperitoneal mucinous cystadenocarcinoma is a rare tumor, and it is urgently necessary to elucidate the etiology of an effective therapy for the disease. PMID:25884614

  15. 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 ...

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

    PubMed Central

    Alishahi, Batoul; Kargahi, Neda; Homayouni, Solmaz

    2015-01-01

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

  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. Hand-Assisted Retroperitoneoscopic Nephroureterectomy With Bladder Cuffing After Preperitoneal and Retroperitoneal Perivesical Ballooning

    PubMed Central

    Kim, Chang Hee; Kim, Kwang Taek; Kim, Khae Hawn

    2014-01-01

    Purpose We aimed to describe the surgical technique of hand-assisted retroperitoneoscopic nephroureterectomy (HARNU) with bladder cuffing after preperitoneal and retroperitoneal perivesical ballooning. Materials and Methods From March 2008 to September 2012, we performed HARNU and open bladder cuffing in 28 consecutive series of patients with upper urinary tract urothelial carcinoma. We performed HARNU according to the following procedure: (1) a camera port incision was made on the posterior axillary line; (2) multiple, repeated, preperitoneal and retroperitoneal ballooning was performed on both the posterior axillary line and in the umbilicus; (3) a 7.0 cm skin incision was made from the suprapubic to the lower inguinal with the balloon present in the extraperitoneal area; (4) hand-assisted laparoscopic retroperitoneal nephroureterectomy; (5) cessation of gas insufflation; and (6) extravesical cuffing as an open surgical procedure. Results The mean estimated blood loss was 250 mL. The mean operation time was 240 minutes. The mean time to oral intake and ambulation was 1.0 day and two days, respectively. As for postoperative complications due to the hand-assisted device, one patient developed febrile urinary tract infection within three weeks postoperatively and was hospitalized again to receive parenteral antibiotics. Conclusions We made a low Gibson incision for a route for the hand-assisted procedure as well as a window for open surgery in dissecting the distal ureter and extracting the surgical specimens. Thus, our results indicate that the HARNU might be a feasible surgical modality. PMID:24466394

  19. Kaposi Sarcoma Incidence and Survival Among HIV-Infected Homosexual Men After HIV Seroconversion

    PubMed Central

    Guiguet, Marguerite; Costagliola, Dominique; Fisher, Martin; de Luca, Andrea; Porter, Kholoud

    2010-01-01

    Background Despite the success of combination antiretroviral therapy (cART) in reducing the incidence of Kaposi sarcoma, HIV-infected individuals who have responded to treatment continue to be diagnosed with Kaposi sarcoma. We examine factors associated with the incidence of Kaposi sarcoma among cART-treated HIV-infected homosexual men and changes in their survival after its diagnosis over calendar time. Methods Data were from HIV-infected homosexual men with well-estimated dates of HIV seroconversion (ie, change in status from being HIV negative to having HIV antibodies detected). Incidence of Kaposi sarcoma was calculated. We used Kaplan–Meier methods to determine survival after Kaposi sarcoma diagnosis in three calendar periods: before 1996, 1996–2000, and 2001–2006. Poisson models were used to examine the effect of risk factors such as current and nadir CD4 cell count (ie, the lowest CD4 cell count ever recorded for a person), duration of infection, and age at diagnosis for Kaposi sarcoma incidence in cART-treated men. All statistical tests were two-sided. Results Among the 9473 men, 555 were diagnosed with Kaposi sarcoma in the period 1986–2006, of whom 319 died. The percentage surviving 24 months after Kaposi sarcoma diagnosis rose statistically significantly during the study period from 35% (95% confidence interval [CI] = 29% to 42%) before 1996 to 84% (95% CI = 76% to 90%) in 1996–2000 and to 81% (95% CI = 70% to 88%) in 2001–2006 (P < .001). Seventy men were diagnosed with Kaposi sarcoma after starting cART. Current (ie, within 6 months) CD4 cell count was associated with incidence of Kaposi sarcoma among cART-treated men (rate ratios [RRs] = 18.91, 95% CI = 8.50 to 42.09, for CD4 level category <200 cells per cubic millimeter; RR = 3.55, 95% CI = 1.40 to 9.00, for 200–349 cells per cubic millimeter; and RR = 4.11, 95% CI = 1.74 to 9.70, for 350–499 cells per cubic millimeter; all compared with ≥500 cells per cubic millimeter). After

  20. Uncommon primary pelvic retroperitoneal masses in adults: a pattern-based imaging approach.

    PubMed

    Shanbhogue, Alampady K; Fasih, Najla; Macdonald, David B; Sheikh, Adnan M; Menias, Christine O; Prasad, Srinivasa R

    2012-01-01

    There is a broad spectrum of primary pelvic retroperitoneal masses in adults that demonstrate characteristic epidemiologic and histopathologic features and natural histories. These masses may be classified into five distinct subgroups using a pattern-based approach that takes anatomic distribution and certain imaging characteristics into account, allowing greater accuracy in their detection and characterization and helping to optimize patient management. The five groups are cystic (serous and mucinous epithelial neoplasms, pelvic lymphangioma, tailgut cyst, ancient schwannoma), vascular or hypervascular (solitary fibrous tumor, paraganglioma, pelvic arteriovenous malformation, Klippel-Trénaunay-Weber syndrome, extraintestinal GIST [gastrointestinal stromal tumor]), fat-containing (lipoma, liposarcoma, myelolipoma, presacral teratoma), calcified (calcified lymphocele, calcified rejected transplant kidney, rare sarcomas), and myxoid (schwannoma, plexiform neurofibroma, myxoma).Cross-sectional imaging modalities help differentiate the more common gynecologic neoplasms from more unusual masses. In particular, the tissue-specific multiplanar capability of high-resolution magnetic resonance imaging permits better tumor localization and internal characterization, thereby serving as a road map for surgery.

  1. 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.

  2. 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

  3. [Breast hematoma masking a sarcoma].

    PubMed

    Relea Sarabia, A; Bahamonde Cabria, S; González Rebollo, M; Encinas Gaspar, M B

    2015-01-01

    Sarcomas account for less than 1% of malignant breast tumors. We present the case of a sarcoma (malignant fibrous histiocytoma) of the breast that debuted as a lump. The patient associated the lump with trauma, and the clinical, sonographic, and cytological findings were suggestive of a hematoma. The lump grew rapidly, and the follow-up sonogram one month later clearly revealed hypervascular solid poles. This atypical presentation is useful for reviewing the management of lesions suggestive of hematomas, which are often associated with nonspecific findings and occasionally with signs that raise suspicion of malignancy. The emergence of what appears to be a hematoma in the breast, even when accompanied by a history of trauma or a tendency toward bleeding, calls for prudence: very short-term follow-up and biopsy, even excisional biopsy, are recommended if the lesion does not evolve like a hematoma. PMID:26160042

  4. Ewing's sarcoma of proximal humeral epiphysis.

    PubMed

    Esmaili, Heydar Ali; Niknejad, Mohammad Taghi; Mohajeri, Shiva

    2015-02-01

    Ewing's sarcoma is one of the most common primary bone tumors of childhood. The tumor is almost always metaphyseal or diaphyseal, within long bones. In children, lesions of the epiphysis are often benign, with the most common diagnosis being chondroblastoma. Rarely, 1%-2% of Ewing sarcomas may involve epiphysis. We present a case of Ewing's sarcoma of the proximal humeral epiphysis in a 13-year-old boy. This case adds to previously reported cases of epiphyseal Ewing sarcoma and suggests that the diagnosis should be considered for pediatric epiphyseal lesions. PMID:25644805

  5. Primary pleuropulmonary synovial sarcoma: a case report.

    PubMed

    Yuan, Lianfang; Guan, Zhiyu; Dai, Xuan; Xu, Jie

    2015-01-01

    Pleuropulmonary synovial sarcoma (PPSS) is an extremely rare malignant tumor, which is increasingly recognized as a subtype of sarcoma with a distinctive chromosomal translocation specific to synovial sarcoma. It is often presents like any thoracic tumor with symptoms such as chest pain or cough. Here we report a case of PPSS in a 49-year-old woman presenting with cough, shortness of breath and chest pain. And who were found upon histologic examination of the resection specimen to have cystic primary pleuropulmonary synovial sarcoma. PMID:26823907

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

    PubMed

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

    2015-01-01

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

  7. Diagnostic Dilemma of a Rare, Giant Retroperitoneal Schwannoma: A Case Report and Review of Literature

    PubMed Central

    Kumar, Lovekesh; Chejara, Rajkumar; Prasad, Om Prakash; Kolhe, Yuvraj

    2014-01-01

    Schwannoma is a benign tumour of peripheral nerve sheath. It usually arises from head, neck, and trunk. Retroperitoneal schwannoma is a rare entity, accounting for only 0.3–3% of total schwannomas. Majority of retroperitoneal schwannomas reported in literature have a diameter of 5 to 15 cm. Preoperative diagnosis is difficult due to low frequency, nonspecific clinical course, and nonspecific imaging features. Histology usually provides definitive diagnosis. Schwannomas are solitary, well-circumscribed, and noninvasive, so complete surgical excision provides good result. We report a case of a 23-year-old male, who presented with progressive abdominal distension and intermittent episodes of intestinal obstruction. CECT was suggestive of huge solid-cystic mass in abdominopelvic region. Image guided percutaneous aspiration revealed around 1 litre of frank pus and FNAC was suggestive of abscess. Exploratory laparotomy revealed a large 32 × 28 × 26 cm mass with solid and cystic components containing 1 litre of pus. Histological features of tumour were suggestive of benign schwannoma and immunohistochemistry for S-100 was positive. Postoperative recovery was uneventful. We report this case of a retroperitoneal schwannoma because of giant size, rare location, unusual presentation, and diagnostic dilemma. PMID:25247101

  8. [Complex radiation diagnosis of retroperitoneal tumors and tumor-like states].

    PubMed

    Vlasov, P V; Kotliarov, P M

    1998-01-01

    The paper presents the results of complex radiation diagnosis of 118 cases of retroperitoneal tumors and tumor-like states, including 71 patients with tumors of mesenchymal origin, 7 with those of neurogenic origin, 6 with cysts, 9 with lymphogranulomatosis, 8 with retroperitoneal fibrosis, 12 with metastases, and 5 with hematomas. A wide complex of routine and up-to-date high-technology radiation diagnostic techniques was used to study the patients. The authors show the merits and dismerits of each technique. Greatest attention is given to the assessment of current techniques, primarily to ultrasonography (US) and computed tomography (CT). The studies have indicated that despite some specific features, many retroperitoneal tumors and tumor-like states bear similarities when various radiation methods are employed. The authors describe some syndromes which can be used to make a diagnosis. Despite the high capacities of US and CT in studying the normal and pathological structures of the human body, there are still great difficulties in differentiating different abnormalities when even the latest techniques are applied, these cases need paracentic aspiration or open biopsy.

  9. 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.

  10. 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.

  11. 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

  12. Prognostic relevance of CCN3 in Ewing sarcoma.

    PubMed

    Perbal, Bernard; Lazar, Noureddine; Zambelli, Diana; Lopez-Guerrero, Jose Antonio; Llombart-Bosch, Antonio; Scotlandi, Katia; Picci, Piero

    2009-10-01

    Ewing sarcoma is a highly aggressive malignant bone tumor occurring preferentially in children and young adults. At present, only clinical features, such as patient age, presence of clinically evident metastases at diagnosis, and poor response to neoadjuvant chemotherapy, are widely accepted as prognostic indicators in Ewing sarcoma. In this study, we assessed the prognostic value of CCN3 (Nov), a matricellular protein that play crucial roles in bone formation. Polyclonal antibodies directed against each of the different CCN3 modules were used to identify variant CCN3 proteins in tumors and to draw potential relationships between the expression of these variants and the outcome of patients with Ewing sarcoma. Our results confirmed that expression of the full-length CCN3 in Ewing sarcoma is associated to a worse prognostic. Furthermore, we report a possible relationship between the expression of a CCN3 protein lacking an internal module (von Willebrand factor type C) and sensitivity to radiotherapy. We hypothesize that the increased level of variant CCN3 in the tumor cells reduces their tumorigenic potential and results in better outcome.

  13. 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

  14. A new subtype of bone sarcoma defined by BCOR-CCNB3 gene fusion.

    PubMed

    Pierron, Gaëlle; Tirode, Franck; Lucchesi, Carlo; Reynaud, Stéphanie; Ballet, Stelly; Cohen-Gogo, Sarah; Perrin, Virginie; Coindre, Jean-Michel; Delattre, Olivier

    2012-04-01

    The identification of subtype-specific translocations has revolutionized the diagnostics of sarcoma and has provided new insight into oncogenesis. We used RNA-seq to investigate samples from individuals diagnosed with small round cell tumors of bone, possibly Ewing sarcoma, but which lacked the canonical EWSR1-ETS translocation. A new fusion was observed between BCOR (encoding the BCL6 co-repressor) and CCNB3 (encoding the testis-specific cyclin B3) on the X chromosome. RNA-seq results were confirmed by RT-PCR and through cloning of the tumor-specific genomic translocation breakpoints. In total, 24 BCOR-CCNB3-positive tumors were identified among a series of 594 sarcoma cases. Gene profiling experiments indicated that BCOR-CCNB3-positive cases are biologically distinct from other sarcomas, particularly Ewing sarcoma. Finally, we show that CCNB3 immunohistochemistry is a powerful diagnostic marker for this subgroup of sarcoma and that overexpression of BCOR-CCNB3 or of truncated CCNB3 activates S phase in NIH3T3 cells. Thus, the intrachromosomal X-chromosome fusion described here represents a new subtype of bone sarcoma caused by a newly identified gene fusion mechanism. PMID:22387997

  15. Postirradiation Sarcoma: Clinicopathologic Features and Role of Chemotherapy in the Treatment Strategy

    PubMed Central

    des Guetz, Gaetan; Chapelier, Alain; Mosseri, Véronique; Dorval, Thierry; Asselain, Bernard; Pouillart, Pierre

    2009-01-01

    Purpose. An analysis of the clinicopathologic features and treatment of patients was performed to guide evaluation and management of postirradiation sarcoma. Patients and Methods. Between 1994 and 2001, 25 patients with postirradiation sarcoma were treated in one center with different chemotherapy, mainly in neoadjuvant setting (19). Tumors for which these patients received radiotherapy initially were mainly breast carcinoma (for 15 patients). The postirradiation sarcomas were of different histopathologic forms, most frequently osteosarcoma, leiomyosarcoma, and angiosarcoma. Results. Of the 25 patients, 19 were initially treated with chemotherapy. Nine of 19 pretreated patients achieved clinical partial response (RP = 47%). Leiomyosarcomas were good responders (3/4) and undifferentiated sarcoma (3/5). Responders were more often treated with MAID (6/8). Eight of the 9 responders underwent surgery. Two patients achieved complete histological response. Seven of the 9 good responders are alive with a median follow up of 24 months. For all treated patients, median follow up 24 months (6–84 months), overall survival and disease free survival were, respectively, 17/25 (68%), and 14/25 (56%). Conclusion. From our data, postirradiation sarcoma should not be managed differently from primary sarcoma. Chemotherapy has to be included in the treatment plan of postirradiation sarcoma, in future studies. PMID:20011664

  16. Spontaneous regression of a cystic retroperitoneal tumour in young women postpartum. Report of two cases.

    PubMed

    de Zeeuw, Sharonne; Schouten van der Velden, Arjan P; Eggink, Alex J; Strijk, Simon; Wobbes, Theo

    2011-01-01

    Retroperitoneal cystic tumours are rarely found, and of these, the most common lesion is a cystic lymphangioma. We present two postpartum patients with a cystic retroperitoneal tumour which showed spontaneous regression and a review of the literature.

  17. 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

  18. Premonitory Pain Preceding Swelling: A Distinctive Clinical Presentation of Synovial Sarcoma which may Prompt Early Detection

    PubMed Central

    De Silva, M. V. Chandu; Barrett, Ann

    2003-01-01

    Purpose: The aim of this paper is to document the unusual presentation of long-standing pain at the tumour site before development of a swelling in patients with synovial sarcoma. Patients/methods and results: The clinical presentation of 53 patients with synovial sarcoma was compared with 56 randomly selected patients with other sarcomas of the trunk and extremities. The two groups were similar with regard to age (P = 0.980), sex (P = 0.784) duration of symptoms (P = 0.697), size (P = 0.931) and site of tumour (P = 0.288). Sixteen (30.2%) patients with synovial sarcoma had pain before development of a swelling compared to two (3.6%) patients with other sarcomas (P < 0.001, odds ratio = 11.68, 95% confidence interval 2.53, 53.83). The mean duration of such pain was 37 months (median 24, range 6–120 months). The nature of the pain was variable. Eight patients had sharply localised tenderness. Calcification seen in the X-rays of four patients was initially misdiagnosed as benign lesions. A swelling was ultimately detected by MRI, CT, ultrasound or at physical examination. The mean duration from first presentation with pain till diagnosis of synovial sarcoma was 20 months. In three patients, at explorative surgery there was friable, vascular or necrotic tissue in the absence of a well-defined tumour mass. Discussion: The occurrence of long-standing pain at the tumour site prior to development of a swelling is significantly more common with synovial sarcomas than with other sarcomas. Awareness of this unusual presentation and appropriate investigation may enable detection of synovial sarcoma at a prognostically favourable early stage. PMID:18521377

  19. Population-based Aarhus Sarcoma Registry: validity, completeness of registration, and incidence of bone and soft tissue sarcomas in western Denmark

    PubMed Central

    Maretty-Nielsen, Katja; Aggerholm-Pedersen, Ninna; Keller, Johnny; Safwat, Akmal; Baerentzen, Steen; Pedersen, Alma B

    2013-01-01

    Background: The aim of the present study was to validate the data in the Aarhus Sarcoma Registry (ASR), to determine if this registry is population-based for western Denmark, and to examine the incidence of sarcomas using validated, population-based registry data. Methods: This study was based on patients with bone and soft tissue sarcoma treated at the Sarcoma Centre of Aarhus University Hospital between January 1, 1979 and December 31, 2008. The validation process included a review of all medical files by two researchers using a standardized form. The Danish Cancer Registry was used as a reference to assess the completeness of registration of patients in the ASR. Crude and World Health Organization age-standardized incidence, as well as age-, gender-, and year-specific incidences were estimated. Results: The validation process added 385 to the 1442 patients who were registered in the ASR. Before validation, on average, 70.5% of the data for the variables was correct. Validation improved the average completeness of the registered variables from 83.7% to 99.3%. The 1827 patients in the ASR after validation include 85.3% of the patients registered in the Danish Cancer Registry. The overall World Health Organization age-standardized incidence of sarcoma in the trunk or extremities in western Denmark in the period 1979–2008 was 2.2 per 100,000, being 0.8 for bone sarcomas and 1.4 for soft tissue sarcomas. Conclusion: The validation process significantly improved the completeness of the variables and the quality of the ASR data. ASR is now a valuable population-based tool for epidemiological research and quality improvement in the treatment of sarcoma. It is our recommendation that documented validation of registries should be a prerequisite for publishing studies derived from them. PMID:23687450

  20. 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

  1. Targeting epigenetic misregulation in synovial sarcoma.

    PubMed

    Waterfall, Joshua J; Meltzer, Paul S

    2012-03-20

    Like many sarcomas, synovial sarcoma is driven by a characteristic oncogenic transcription factor fusion, SS18-SSX. In this issue of Cancer Cell, Su et al. elucidate the protein partners necessary for target gene misregulation and demonstrate a direct effect of histone deacetylase inhibitors on the SS18-SSX complex composition, expression misregulation, and apoptosis.

  2. AZD0530 in Treating Patients With Recurrent Locally Advanced or Metastatic Soft Tissue Sarcoma

    ClinicalTrials.gov

    2015-07-02

    Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Rhabdomyosarcoma; Dermatofibrosarcoma Protuberans; Endometrial Stromal Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Uterine Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma; Uterine Carcinosarcoma; Uterine Leiomyosarcoma

  3. [Minimally invasive adrenalectomy: transperitoneal vs. retroperitoneal approach].

    PubMed

    Ramacciato, Giovanni; Nigri, Giuseppe; Di Santo, Vincenzo; Piccoli, Michaela; Pansadoro, Vito; Buniva, Paolo; Bellagamba, Riccardo; Cescon, Matteo; Ercolani, Giorgio; Cucchetti, Alessandro; Lauro, Augusto; Del Gaudio, Massimo; Ravaioli, Matteo; Valabrega, Stefano; D'Angelo, Francesco; Aurello, Paolo; Stigliano, Antonio; Toscano, Vincenzo; Melotti, Gianluigi

    2008-01-01

    Laparoscopic adrenalectomy is now regarded as the procedure of choice for most adrenal glands presenting surgical pathology. The primary adrenal-specific contraindication to laparoscopic adrenalectomy today is the presence of a large adrenal mass with evidence of local infiltration or venous invasion. We used our multicentre experience to compare the transperitoneal (TLA) and retroperitoneal (RLA) minimally invasive approaches. In our study we found statistically significant differences between RLA and TLA in terms of duration of surgery (148 minuti vs. 112; p < 0.005), intra-operative blood loss (439 cc vs 333 p < 0.005; p < 0.005) and time of first oral intake (1.2 +/- 0.5 days vs 1.8 +/- 1.08 days; p < 0.005). The RLA approach is preferable in cases of previous abdominal surgery, but its learning curve is extremely steep. TLA access needs a less demanding learning curve and tends to be faster than RLA, where the working area is penalised by limited manoeuvring space. There is no clear preference between TLA and RLA in the literature. However, the experience of the surgeon still remains the most important variable when choosing between the two approaches.

  4. [Giant retroperitoneal cystic mass: appendiceal mucocele].

    PubMed

    Rodríguez Alonso, A; Suárez Pascual, G; Bonelli Martín, C; González Blanco, A; Lorenzo Franco, J; Cuerpo Pérez, M A; Nogueira Carballedo, C; Alvarez Fernández, J C; Nieto García, J

    2004-04-01

    Appendiceal mucocele is a term used to describe the dilatation of the vermiform appendix produced by an intraluminal accumulation of mucus. Four pathological processes have been described that may lead to an appendiceal mucocele: obturation of cecoappendiceal communication, mucosal hyperplasia, mucinous cystadenoma and mucinous cystoadenocarcinoma. The most frequent is mucinous cystadenoma, seen in 50% of appendiceal mucoceles. 6% of patients with appendiceal mucocele develop peritoneal pseudomixoma, possibly through dissemination of the epithelial cells into the abdominal cavity. Preoperative diagnosis of the lesion is particularly important in order to deal with it carefully during surgery. CT scan is the most precise radiological exploration, although there are no pathognomonic signs of mucocele. Typical CT finding of a mucocele is a well-defined cystic mass that compresses the cecum without any peripheral inflammatory reaction, and with low levels of attenuation that vary between water and soft tissue density. We present a case of an appendiceal mucocele caused by a mucinous cystadenoma clinically presented as a giant retroperitoneal mass. Diagnosis was postoperatively made, after pathological study of the surgical sample.

  5. Ewing Sarcoma in the Right Ventricle

    PubMed Central

    Zhao, Bihong; Thangam, Manoj; Loyalka, Pranav; Buja, L. Maximilian; Kar, Biswajit; Gregoric, Igor D.

    2016-01-01

    Ewing sarcoma is the second most prevalent malignant primary bone tumor but constitutes only a small proportion of cardiac metastases. We present a case of asymptomatic Ewing sarcoma metastatic to the right ventricle. A 36-year-old man presented for evaluation and resection of a pedunculated right ventricular cardiac tumor. Three years before, he had been diagnosed with translocation-negative Ewing sarcoma, for which he had undergone chemotherapy and amputation of the left leg below the knee. We resected the right ventricular tumor. Analysis of the resected mass supported the diagnosis of metastatic Ewing sarcoma. Postoperative transthoracic echocardiograms showed normal biventricular size and function. One year later, the patient had no recurrence of the sarcoma. In addition to discussing this case, we review the relevant medical literature. PMID:27777536

  6. [Alveolar sarcoma. Report of a case].

    PubMed

    Devisme, L; Mensier, E; Bisiau, S; Bloget, F; Gosselin, B

    1996-01-01

    Alveolar soft part sarcoma occurs mostly in the deep soft tissues. An unusual case of primary pulmonary alveolar soft part sarcoma is reported. A 39-year-old woman presented with thoracic pain revealing the tumor. The left lower lobe was surgically resected. The microscopic features of this tumor, including characteristic alveolar pattern and the PAS-positive crystals were typical of alveolar soft part sarcoma. Immunohistochemically, granular cytoplasmic reactivities were observed with antibodies against vimentin, myoglobin, methionine-enkephalin, S100 protein and neuron-specific-enolase. Electron microscopic study demonstrated numerous crystallized structures in the tumor cell cytoplasm. This is the third case of pulmonary alveolar soft part sarcoma, one arising from the pulmonary vein. The histogenesis of alveolar soft part sarcoma is still debated. Our case does not allow distinction between myogenic or neural origin of this tumor.

  7. 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

  8. The synthesis and activity of lipoprotein lipase in the subcutaneous adipose tissue of patients with musculoskeletal sarcomas.

    PubMed

    Sakayama, Kenshi; Kidani, Teruki; Tanji, Nozomu; Yamamoto, Haruyasu; Masuno, Hiroshi

    2008-01-01

    The purpose of this study was to explore the triacylglycerol (TG) deposition and lipoprotein lipase (LPL) activity in the adipose tissue of patients with muculoskeletal sarcoma. Subcutaneous adipose tissue was obtained from the thighs of 19 patients with musculoskeletal sarcomas (sarcoma group) and 20 patients with osteoarthritis of the hip joint (control group) at surgery. The adipose tissue was homogenized and aliquots of the homogenate were used to measure the TG content and to prepare an acetone/ether powder to measure the LPL activity. The TG content was higher, but not significantly, in the sarcoma group than in the control group. The LPL activity of the sarcoma group was significantly higher than that of the control group. The TG content of the sarcoma group correlated positively with the LPL activity. [35S]Methionine incorporation investigation showed that the rate of LPL synthesis was significantly higher in the sarcoma group than in the control group. These results indicated that LPL was up-regulated at the transcriptional/translational level, thus resulting in an increased TG deposition in the adipose tissue of patients with muculoskeletal sarcoma.

  9. 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

  10. 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.

  11. 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.

  12. Retroperitoneal teratoma in a domestic rabbit (Oryctolagus cuniculus).

    PubMed

    Mutinelli, Franco; Carminato, Antonio; Bozzato, Elisa; Marchioro, Wendy; Trevisan, Laura; Vascellari, Marta

    2009-03-01

    Teratomas are histologically complex tumors arising from germ cells of two or three embryonic layers either in the gonads or occasionally from germ cells that mistakenly migrated to ectopic sites. A case of retroperitoneal teratoma in a regularly slaughtered 4-month-old, male domestic rabbit is reported. A pale reddish, 3 x 4 cm, fibrous mass was detected, loosely connected to the parietal wall in the retroperitoneal region cranial to the kidney. Histologically, the neoplasm contained tissues from ectodermal, endodermal and mesodermal germ layers: nerves and neurons simulating ganglia, cysts lined by squamous epithelium containing keratin and hair, skin adnexa; respiratory glandular epithelium, serous and salivary glands; collagenous connective tissue, muscle fibers, mature cartilage and bone, bone marrow, teeth and fat. To the authors' knowledge this is the first report of a retroperitoneal teratoma in the rabbit.

  13. A retroperitoneal bronchogenic cyst mimicking a pancreatic or adrenal mass.

    PubMed

    Runge, Tina; Blank, Annika; Schäfer, Stephan C; Candinas, Daniel; Gloor, Beat; Angst, Eliane

    2013-01-01

    Retroperitoneal location of bronchogenic cysts is extremely rare. Most commonly they are encountered in the posterior mediastinum. Bronchogenic cysts arise from developmental aberrations of the tracheobronchial tree in the early embryologic period. We report a 42-year-old female patient with a retroperitoneal bronchogenic cyst in the left adrenal region. She was admitted to our hospital with epigastric pain and subsequently underwent CT of the abdomen. The examination revealed a mass related to the left adrenal gland. Endocrine tests for adrenal hypersecretion were negative. Because of the uncertain entity, laparoscopic adrenalectomy was performed. Pathological examination revealed a bronchogenic cyst in proximity to an inconspicuous left adrenal gland. Although very rare, bronchogenic cysts should be considered in the differential diagnosis of retroperitoneal cystic lesions and surgical resection pursued for symptom resolution and to establish a definitive histology.

  14. [Bronchogenic cyst as a rare differential diagnosis of retroperitoneal tumor].

    PubMed

    Wirbel, R J; Uhlig, U; Kiffner, E M; Berger, K

    1993-12-01

    A retroperitoneal tumor originating from a bronchogenic cyst is an extreme rarity. We report the sixth case of a retroperitoneal bronchogenic cyst in an asymptomatic 38-year-old man who was initially admitted to hospital due to elective herniotomy of a right-sided inguinal hernia. Excluding symptomatic hernia by preoperative, routine-ultrasound we detected left-sided adrenal tumor mass. Computerized tomography and inconspicuous hormone-analysis completed preoperative evaluation. A tumor with 3 cm in diameter above the left adrenal gland, partly solid, partly cystic could be removed by laparotomy incision without complications. Final pathology confirmed diagnosis of a bronchogenic, dysontogenic cyst. Although rare, these cysts should be considered in differential-diagnosis of retroperitoneal tumors.

  15. [A case of retroperitoneal serous cyst resected by laparoscopic surgery].

    PubMed

    Izaki, Hirofumi; Takahashi, Masayuki; Yuasa, Akihito; Fukawa, Tomoya; Koizumi, Takahiro; Yamaguchi, Kunihisa; Yamamoto, Yasuyo; Taue, Ryuichi; Nakatsuji, Hiroyoshi; Kishimoto, Tomoteru; Fukumori, Tomoharu; Kanayama, Hiro-Omi; Bando, Yoshimi; Kusuhara, Yoshito

    2009-11-01

    A 40-year-old woman was referred to our hospital because of pain extending from the left lateral abdomen to the left inferior limb. The abdominal computed tomography (CT) revealed an 8x7x12 cm retroperitoneal serous cystic mass. The serum carcinoembryogenic antigen (CEA) level was slightly elevated to 2.7 ng/ml. Therefore, we suspected it to be malignant, and we performed laparoscopic resection carefully. The retroperitoneal cyst was not adherent to the surrounding tissues and was easily dissected and removed under laparoscopy. Carbohydrale antigen (CA)19-9, CA125 and CEA levels in the fluid were elevated, but a cytology of the fluid was negative and no malignant sign was seen in the cyst wall. To our knowledge, this is the second reported case of retroperitoneal serous cyst resected by laparoscopic surgery in the Japanese literature.

  16. A Retroperitoneal Bronchogenic Cyst Mimicking a Pancreatic or Adrenal Mass

    PubMed Central

    Runge, Tina; Blank, Annika; Schäfer, Stephan C.; Candinas, Daniel; Gloor, Beat; Angst, Eliane

    2013-01-01

    Retroperitoneal location of bronchogenic cysts is extremely rare. Most commonly they are encountered in the posterior mediastinum. Bronchogenic cysts arise from developmental aberrations of the tracheobronchial tree in the early embryologic period. We report a 42-year-old female patient with a retroperitoneal bronchogenic cyst in the left adrenal region. She was admitted to our hospital with epigastric pain and subsequently underwent CT of the abdomen. The examination revealed a mass related to the left adrenal gland. Endocrine tests for adrenal hypersecretion were negative. Because of the uncertain entity, laparoscopic adrenalectomy was performed. Pathological examination revealed a bronchogenic cyst in proximity to an inconspicuous left adrenal gland. Although very rare, bronchogenic cysts should be considered in the differential diagnosis of retroperitoneal cystic lesions and surgical resection pursued for symptom resolution and to establish a definitive histology. PMID:24403880

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

    PubMed Central

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

    2015-01-01

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

  18. 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

  19. 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

  20. 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

  1. 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

  2. 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

  3. 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

  4. 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

  5. [A case of retroperitoneal lymphangioma successfully treated by pancreatoduodenectomy].

    PubMed

    Kurokawa, Y; Kamiya, J; Kirioka, T; Kajita, M; Maeda, M; Nakamura, T; Akiyama, S

    1987-02-01

    A rare case of retroperitoneal lymphangioma in 44-year-old male complaining of high fever and right hypochondralgia is reported. Retroperitoneal malignant tumor was suspected preoperatively, and pancreatoduodenectomy was performed because the tumor infiltrated to duodenum and head of the pancreas. Histopathological examination revealed that the tumor was lymphangioma infiltrating to the duodenum. There are two types of lymphangioma, solitary lymphatic cyst and true proliferating lymphangioma and in this case, the tumor is diagnosed as true proliferating lymphangioma which is cavernous and infiltrating.

  6. Laparoscopic resection of a retroperitoneal cystic lymphangioma in an adult.

    PubMed

    Kasza, Jason; Brody, Fredrick J; Khambaty, Fatima; Vaziri, Khashayar; Wallace, Brian

    2010-06-01

    Cystic lymphangiomas are rare, benign anomalies of the lymphatic system. More than 95% of cystic lymphangiomas occur in the head, neck, and axilla with only 1% in the retroperitoneum. Most of these cases are diagnosed by the second year of life with only a handful of adult cases. Once a symptomatic cystic lesion of the abdomen or retroperitoneum is diagnosed, treatment usually consists of surgical excision. Traditionally, surgery requires a laparotomy. This paper describes a patient with a retroperitoneal cyst who underwent a successful laparoscopic resection. The etiology and management of adult retroperitoneal cysts are reviewed as well.

  7. [A case of hamartoma of the retroperitoneal space].

    PubMed

    Gobara, M; Suzuki, Y; Okano, M; Muranaka, K; Shimizu, Y; Kawada, Y

    1988-02-01

    A case of hamartoma of the retroperitoneal space in a 59-year-old woman is reported. Excretory urography, computed tomography and adrenal angiography revealed a tumor with multiple calcification in the left retroperitoneal space. The tumor measuring 6.5 by 3.5 cm, and weighing 30 gm, was removed operatively. Removed specimen consisted of many cysts of various sizes containing mucous fluid and multiple small stones. Microscopically, the tumor was composed of a tubular structure lined by ciliated, epithelium-like tissue of the respiratory system or that of Fallopian tube. These tubules were occasionally dilated cystically. No atypical cells were found. The postoperative course was uneventful.

  8. Laparoscopic excision of a retroperitoneal lymphatic malformation in a newborn.

    PubMed

    Solari, Valeria; Mullassery, Dhanya; Lansdale, Nick; Jesudason, Edwin C

    2011-02-01

    Abdominal lymphatic malformations may be challenging to eradicate. Retroperitoneal lesions may more difficult to resect than mesenteric ones; however, the latter may predispose to intestinal volvulus, leading to calls for their prompt excision. Such lesions identified perinatally may pose particular challenges: in one case, respiratory failure caused by abdominal distension required emergency drainage followed by later laparoscopic excision; laparoscopy has also been used promptly to diagnose and resect neonatal mesenteric lymphatic malformations with their inherent volvulus risk. We illustrate that even if neonatal laparoscopy identifies a retroperitoneal rather than mesenteric lymphatic malformation, curative endosurgical excision remains feasible.

  9. Rupture of retroperitoneal hydatid cyst into the abdominal aorta.

    PubMed

    Hadjiat, N; Graba, A; Mansouri, H

    1987-05-01

    We report the case of a retroperitoneal hydatid cyst ruptured into the infrarenal abdominal aorta. Initial treatment consisted of marsupialization of the cyst with patch angioplasty of the aorta. Formation of a false aneurysm led to reoperation for placement of an aortobifemoral bypass, but the patient died postoperatively. In spite of the exceptional character of this location, diagnosis should be suggested routinely in endemic areas when a retroperitoneal mass is discovered. Treatment should be either in situ prosthetic replacement or extra-anatomic exclusion bypass.

  10. Retroperitoneal approach for lumbar lateral meningocele--case report.

    PubMed

    Miyata, M; Yamasaki, S; Yoshida, T; Matsubara, M; Okamura, M; Hirayama, A; Tamaki, N

    1995-12-01

    A 29-year-old female with neurofibromatosis presented with a right lumbar lateral meningocele. Abdominal computed tomography (CT) showed a huge right retroperitoneal cyst expanding anterolaterally and displacing the right kidney. CT following myelography disclosed the cyst expanding through a wide defect of the right pedicles of the T-12 and L-1 vertebrae. The cyst was resected through a retroperitoneal approach with right flank oblique incision. Postoperatively, cerebrospinal fluid leakage occurred, which improved after lumboperitoneal shunt. Careful screening for lateral meningocele, including the lumbar region, should be undertaken in a patient with neurofibromatosis who presents with vertebral anomalies.

  11. Simultaneous right retroperitoneal schwannoma and left renal hydatid cyst.

    PubMed

    Kamalati, Ali; Tabrizchi, Hamid

    2013-01-01

    Retroperitoneal schwannomas are quite rare tumors. Isolated renal hydatid cyst is also rare, and it forms 2-4% of hydatid disease. Because of their infrequent occurrence, nonspecific signs and symptoms, and lack of distinguishing radiologic features, we report herein a case of right retroperitoneal mass in a 26-year-old woman which was found to be benign schwannoma following a percutaneous core needle biopsy and a large cortical cyst in the lower pole of the left kidney which was diagnosed as isolated renal hydatid cyst following exploration.

  12. 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.

  13. Pulmonary sequestration presenting as retroperitoneal cyst: case report.

    PubMed

    Armatys, Sandra A; Cheng, Liang; Gardner, Thomas A; Sundaram, Chandru P

    2005-10-01

    A 21-year-old woman presented with flank pain, and an abdominal and pelvic CT scan demonstrated a left 7.4-cm simple cyst superior to the left kidney. She underwent laparoscopic cyst decortication complicated by a diaphragmatic injury and pneumothorax. The final pathology report described a retroperitoneal pulmonary sequestration (RPS). Urologists need to consider RPS in the differential diagnosis of a retroperitoneal cyst because of the associated morbidity of hemorrhage during surgical excision. With the widespread use abdominal imaging techniques, more cases of RPS are likely to be identified and referred for laparoscopic management.

  14. Large solitary retroperitoneal echinococcal cyst: a rare case report.

    PubMed

    Tepetes, Konstantinos; Christodoulidis, Gregory; Spryridakis, Michael; Hatzitheofilou, Konstantinos

    2007-12-01

    Echinococcal disease remains a problem within some endemic areas. Echinococcal cysts usually involve the liver and lungs, but any other organ can potentially be involved. Extrahepatic localization is reported in 14%-19% of all cases of abdominal hydatid disease. We report the case of a large echinococcal cyst localized in the lower pelvis. A 28-year-old woman was admitted to a surgical ward with lower abdominal pain and discomfort lasting for a month. Ultrasonography and computed tomography scanning revealed a large retroperitoneal cystic mass (9 cm multiply 4 cm) in contact with the left ovary and left ureter. There were no cysts in any other location. Serological tests were positive for Echinococcus. The patient was operated on and the entire cyst was excised intact. Histopathological results confirmed the diagnosis of echinococcosis. Antihelminthics were administered postoperatively and the patient was discharged after 6 d, and is now being closely followed up. Total cystectomy when possible represents the treatment of choice for large extrahepatic echinococcal cysts.

  15. 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

  16. 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

  17. [Chronic back pain resulting from a retroperitoneal lymphangioma].

    PubMed

    Justinger, C; Weinrich, M; Katoh, M; Schilling, M K

    2008-08-01

    A 39-year-old female patient presented with a 3-year history of lower back pain which had not been alleviated by pain treatment combined with physiotherapy. Radiological findings were normal with the exception of a cystic paravertebral tumor in the left retroperitoneum. The cyst was primarily regarded as a coincidental finding because no criteria for malignancy were present. Magnetic resonance tomography, however, the cyst showed a progressive increase in size and the pain remained unchanged so that surgical resection was performed. The histopathological examination showed a lymphangioma and the pain was nearly completely resolved after surgery.

  18. 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.

  19. Insulin-like growth factor-1 receptor acts as a growth regulator in synovial sarcoma.

    PubMed

    Friedrichs, N; Küchler, J; Endl, E; Koch, A; Czerwitzki, J; Wurst, P; Metzger, D; Schulte, J H; Holst, M I; Heukamp, L C; Larsson, O; Tanaka, S; Kawai, A; Wardelmann, E; Buettner, R; Pietsch, T; Hartmann, W

    2008-12-01

    Synovial sarcomas account for 5-10% of all soft tissue sarcomas and the majority of synovial sarcomas display characteristic t(X;18) translocations that result in enhanced transcription of the insulin-like growth factor-2 (IGF-2) gene. IGF-2 is an essential fetal mitogen involved in the pathogenesis of different tumours, leading to cellular proliferation and inhibition of apoptosis. Here we asked whether activation of IGF signalling is of functional importance in synovial sarcomas. We screened human synovial sarcomas for expression of IGF-2 and the phosphorylated IGF-1 receptor (IGF-1R), which mainly mediates the proliferative and anti-apoptotic effects of IGF-2. Since both the phosphatidylinositol 3'-kinase (PI3K)-AKT pathway and the MAPK signalling cascade are known to be involved in the transmission of IGF-1R signals, expression of phosphorylated (p)-AKT and p-p44/42 MAPK was additionally assessed. All tumours expressed IGF-2 and 78% showed an activated IGF-1R. All tumours were found to express p-AKT and 92% showed expression of activated p44/42 MAPK. To analyse the functional and potential therapeutic relevance of IGF-1R signalling, synovial sarcoma cell lines were treated with the IGF-1R inhibitor NVP-AEW541. Growth was impaired by the IGF-1R antagonist, which was consistently accompanied by a dose-dependent reduction of phosphorylation of AKT and p44/42 MAPK. Functionally, inhibition of the receptor led to increased apoptosis and diminished mitotic activity. Concurrent exposure of selected cells to NVP-AEW541 and conventional chemotherapeutic agents resulted in positive interactions. Finally, synovial sarcoma cell migration was found to be dependent on signals transmitted by the IGF-1R. In summary, our data show that the IGF-1R might represent a promising therapeutic target in synovial sarcomas.

  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. Uterine sarcomas: clinical presentation and MRI features

    PubMed Central

    Santos, Pedro; Cunha, Teresa Margarida

    2015-01-01

    Uterine sarcomas are a rare heterogeneous group of tumors of mesenchymal origin, accounting for approximately 8% of uterine malignancies. They comprise leiomyosarcoma, endometrial stromal sarcoma, undifferentiated endometrial sarcoma, and adenosarcoma. Compared with the more common endometrial carcinomas, uterine sarcomas behave more aggressively and are associated with a poorer prognosis. Due to their distinct clinical and biological behavior, the International Federation of Gynecology and Obstetrics introduced a new staging system for uterine sarcomas in 2009, categorizing uterine carcinosarcoma as a variant of endometrial carcinoma, rather than a pure sarcoma. Magnetic resonance imaging (MRI) has a developing role in the assessment of these malignancies. Features such as tumor localization, irregular or nodular margins, necrosis, rapid growth, intense contrast enhancement, and restriction at diffusion-weighted imaging can suggest the diagnosis and help differentiate from more common leiomyomas and endometrial carcinoma. MRI is therefore extremely useful in preoperative detection and staging and, consequently, in determination of appropriate management. This pictorial review aims to discuss the clinical features of uterine sarcomas, as well as their most common appearances and distinct characteristics in MRI. PMID:25347940

  2. 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

  3. CD40 upregulation is independent of HHV-8 in the pathogenesis of Kaposi's sarcoma.

    PubMed Central

    Kennedy, M M; Biddolph, S; Lucas, S B; Howells, D D; Picton, S; McGee, J O; O'Leary, J J

    1999-01-01

    AIMS: Human herpesvirus 8 (HHV-8) is now acknowledged as the infective cofactor in the pathogenesis of Kaposi's sarcoma. The mode by which HHV-8 causes Kaposi's sarcoma is unresolved and it is probable that it acts in conjunction with other factors including cytokines, anti-apoptosis proteins, and cell surface receptors. CD40, a cell membrane receptor belonging to the tumour necrosis factor receptor super family, promotes B cell survival and is expressed constitutively on endothelial cells. It is upregulated on cytokine treatment and has been documented recently in Kaposi's sarcoma. Because the HHV-8 genome contains cytokine homologues, this study investigated whether CD40 expression in Kaposi's sarcoma correlated with HHV-8 status, using a unique set of HHV-8 positive and negative specimens. METHODS: Twenty one paraffin wax embedded samples of Kaposi's sarcoma were selected, of which 18 were screened for the presence of HHV-8 using both conventional solution phase and TaqMan polymerase chain reaction (PCR). CD40 immunohistochemistry was assessed using a biotinylated amplification system. Staining was scored semiquantitatively. RESULTS: The results indicated that the expression of CD40 is independent of viral status, being present in both HHV-8 positive and negative specimens. CONCLUSIONS: This suggests that HHV-8 promotes Kaposi's sarcoma cell survival following infection by mechanisms other than those involving CD40. PMID:10439837

  4. 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

  5. Multiplex RT-PCR assay for the differential diagnosis of alveolar rhabdomyosarcoma and Ewing's sarcoma.

    PubMed Central

    Downing, J. R.; Khandekar, A.; Shurtleff, S. A.; Head, D. R.; Parham, D. M.; Webber, B. L.; Pappo, A. S.; Hulshof, M. G.; Conn, W. P.; Shapiro, D. N.

    1995-01-01

    Cytogenetic analysis has defined specific translocations associated with two of the most common small round cell tumors of childhood, t(11;22) in Ewing's sarcoma and t(2;13) in alveolar rhabdomyosarcoma. We and others have previously demonstrated the diagnostic utility of a reverse transcriptase polymerase chain reaction (RT-PCR) assay for the detection of the t(11;22) encoded EWS/FLI-1 chimeric message in Ewing's sarcoma. More recently, we have cloned the t(2;13)(q35;q14) translocation and have shown that it results in the fusion of the PAX3 gene on chromosome 2 to FKHR, a novel member of the fork-head family of transcription factors on chromosome 13. To define the morphological spectrum of childhood sarcomas that express the t(2;13) encoded PAX3/FKHR chimeric message, we have performed RT-PCR analysis on samples from 44 primary pediatric sarcomas and 8 sarcoma cell lines. PAX3/FKHR chimeric messages were detected in 24 of 27 alveolar, 2 of 12 embryonal, and 0 of 1 pleomorphic rhabdomyosarcoma and in 1 of 2 ectomesenchymomas. In contrast, none of 8 Ewing's sarcomas or 2 undifferentiated sarcomas expressed this message. Chimeric transcripts were detected in all cases with cytogenetic evidence of the (2;13) translocation, and in each case the chimeric PAX3/FKHR message had the identical junction sequence, suggesting that genomic chromosome breaks were clustered in a single intron in both genes. By combining the PAX3/FKHR RT-PCR assay with primers for detection of the Ewing's sarcoma t(11;22) encoded EWS/FLI-1 chimeric transcript, we have developed a multiplex RT-PCR reaction that allows the rapid and accurate identification of either translocation in a biopsy sample. Images Figure 1 Figure 2 Figure 3 Figure 4 PMID:7887445

  6. 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

  7. EWS/FLI1 Target Genes and Therapeutic Opportunities in Ewing Sarcoma.

    PubMed

    Cidre-Aranaz, Florencia; Alonso, Javier

    2015-01-01

    Ewing sarcoma is an aggressive bone malignancy that affect children and young adults. Ewing sarcoma is the second most common primary bone malignancy in pediatric patients. Although significant progress has been made in the treatment of Ewing sarcoma since it was first described in the 1920s, in the last decade survival rates have remained unacceptably invariable, thus pointing to the need for new approaches centered in the molecular basis of the disease. Ewing sarcoma driving mutation, EWS-FLI1, which results from a chromosomal translocation, encodes an aberrant transcription factor. Since its first characterization in 1990s, many molecular targets have been described to be regulated by this chimeric transcription factor. Their contribution to orchestrate Ewing sarcoma phenotype has been reported over the last decades. In this work, we will focus on the description of a selection of EWS/FLI1 targets, their functional role, and their potential clinical relevance. We will also discuss their role in other types of cancer as well as the need for further studies to be performed in order to achieve a broader understanding of their particular contribution to Ewing sarcoma development.

  8. EWS/FLI1 Target Genes and Therapeutic Opportunities in Ewing Sarcoma

    PubMed Central

    Cidre-Aranaz, Florencia; Alonso, Javier

    2015-01-01

    Ewing sarcoma is an aggressive bone malignancy that affect children and young adults. Ewing sarcoma is the second most common primary bone malignancy in pediatric patients. Although significant progress has been made in the treatment of Ewing sarcoma since it was first described in the 1920s, in the last decade survival rates have remained unacceptably invariable, thus pointing to the need for new approaches centered in the molecular basis of the disease. Ewing sarcoma driving mutation, EWS–FLI1, which results from a chromosomal translocation, encodes an aberrant transcription factor. Since its first characterization in 1990s, many molecular targets have been described to be regulated by this chimeric transcription factor. Their contribution to orchestrate Ewing sarcoma phenotype has been reported over the last decades. In this work, we will focus on the description of a selection of EWS/FLI1 targets, their functional role, and their potential clinical relevance. We will also discuss their role in other types of cancer as well as the need for further studies to be performed in order to achieve a broader understanding of their particular contribution to Ewing sarcoma development. PMID:26258070

  9. Cannabinoid modulation of Kaposi's sarcoma-associated herpesvirus infection and transformation.

    PubMed

    Zhang, Xuefeng; Wang, Jian Feng; Kunos, George; Groopman, Jerome E

    2007-08-01

    Kaposi's sarcoma-associated herpesvirus (KSHV; also named human herpesvirus 8) is necessary but not sufficient for the development of Kaposi's sarcoma. A variety of factors may contribute to the pathogenesis of Kaposi's sarcoma in addition to KSHV. Marijuana is a widely used recreational agent, and Delta(9)-tetrahydrocannabinol (Delta(9)-THC), the major active component of marijuana, is prescribed for medicinal use. To evaluate how cannabinoids may affect the pathogenesis of Kaposi's sarcoma, we studied primary human dermal microvascular endothelial cells (HMVEC) exposed to KSHV. There was an increased efficiency of KSHV infection in the presence of low doses of Delta(9)-THC. We also found that Delta(9)-THC increased the viral load in KSHV-infected HMVEC through activation of the KSHV lytic switch gene, the open reading frame 50. Furthermore, we observed that Delta(9)-THC stimulated expression of the KSHV-encoded viral G protein-coupled receptor and Kaposi's sarcoma cell proliferation. Our results indicate that Delta(9)-THC can enhance KSHV infection and replication and foster KSHV-mediated endothelial transformation. Thus, use of cannabinoids may place individuals at greater risk for the development and progression of Kaposi's sarcoma. PMID:17671191

  10. EWS/FLI1 Target Genes and Therapeutic Opportunities in Ewing Sarcoma.

    PubMed

    Cidre-Aranaz, Florencia; Alonso, Javier

    2015-01-01

    Ewing sarcoma is an aggressive bone malignancy that affect children and young adults. Ewing sarcoma is the second most common primary bone malignancy in pediatric patients. Although significant progress has been made in the treatment of Ewing sarcoma since it was first described in the 1920s, in the last decade survival rates have remained unacceptably invariable, thus pointing to the need for new approaches centered in the molecular basis of the disease. Ewing sarcoma driving mutation, EWS-FLI1, which results from a chromosomal translocation, encodes an aberrant transcription factor. Since its first characterization in 1990s, many molecular targets have been described to be regulated by this chimeric transcription factor. Their contribution to orchestrate Ewing sarcoma phenotype has been reported over the last decades. In this work, we will focus on the description of a selection of EWS/FLI1 targets, their functional role, and their potential clinical relevance. We will also discuss their role in other types of cancer as well as the need for further studies to be performed in order to achieve a broader understanding of their particular contribution to Ewing sarcoma development. PMID:26258070

  11. 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.

  12. 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

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

    SciTech Connect

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

    2006-10-10

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

  14. A retroperitoneal neuroendocrine tumor in ectopic pancreatic tissue.

    PubMed

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

    2013-07-01

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

  15. 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

  16. Rare Case of Retroperitoneal Lipomatosis in an Indian Woman

    PubMed Central

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

    2016-01-01

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

  17. Refining the laparoscopic retroperitoneal lymph node dissection for testicular cancer.

    PubMed

    Romero, Frederico R; Wagner, Andrew; Brito, Fabio A; Muntener, Michael; Lima, Guilherme C; Kavoussi, Louis R

    2006-01-01

    Since its initial description, the laparoscopic retroperitoneal lymph node dissection has evolved considerably, from a purely diagnostic tool performed to stage germ cell testicular cancer to a therapeutic operation that fully duplicates the open technique. Herein, we describe the current technique employed at our institution, along with illustrations of all surgical steps, and delineate the refinements of the technique over time.

  18. Isolated retroperitoneal intradiaphragmatic bronchogenic cyst. A case report.

    PubMed

    Hoang, C; Aubriot, M H; Totobenazara, J L; Chigot, J P; Menegaux, F; Le Charpentier, Y

    1999-01-01

    Isolated retroperitoneal bronchogenic cysts are extremely rare. We report a case which was intradiaphragmatic intimately associated with the musculature of the left crus diaphragmatic and unconnected with any other structures. Ultrasound and computed tomography findings were consistent with a pancreatic or an adrenal mass. Pathology confirmed a bronchogenic cyst. The outcome is favourable and the overall prognosis is good.

  19. [Retroperitoneal cyst: a little known internal medicine problem].

    PubMed

    Ramadori, G; Rumpelt, H J; Keidl, E; Dormeyer, H H; Rothmund, M; Hütteroth, T; Meyer zum Büschenfelde, K H

    1984-11-01

    Retroperitoneal cysts are often a differential diagnostic problem. The diagnosis is not infrequently made only by laparotomy. Our experience with a 33 year old patient with pain localized in the left upper part of the abdomen and "cyst of the spleen" confirms this rule. The diagnosis of a lymphatic cyst of the retroperitoneum was made after the surgical enucleation.

  20. [Spontaneous massive retroperitoneal hemorrhage from an adrenal gland cyst].

    PubMed

    Colović, R; Havelka, M; Ostojić, S; Kovacević, N; Lotina, S; Barisić, G; Colić, M

    Adrenal cyst are rare disease. Bleeding, particularly massive, from these cysts is even rarer. The cyst causing spontaneous massive retroperitoneal bleeding in a 17 year old girl is presented. Adrenalectomy was successfully carried out. The patient stayed symptom free so far.

  1. Retroperitoneal foregut duplication cyst presenting as an adrenal mass.

    PubMed

    Terry, N Elizabeth; Senkowski, Christopher K; Check, William; Brower, Steven T

    2007-01-01

    A 75 year-old woman presented to the authors' institution with abdominal pain and early satiety. An adrenal mass was found on CT scanning. Laparoscopic adrenalectomy was performed, and the patient was found to have a retroperitoneal bronchogenic cyst adherent to the adrenal gland. The workup of an adrenal mass is discussed as well as the pathophysiology of bronchogenic cysts.

  2. Retroperitoneal cyst and congenital absence of ipsilateral kidney.

    PubMed

    Hodgins, T E; Hancock, R A

    1976-12-01

    The case report of a patient in whom a cystic retroperitoneal mass and the congenital absence of the ipsilateral kidney, ureter, ureteral orifice, and trigone is presented. The embryology and literature are noted. We can find only 2 similar cases in the literature.

  3. [Isolated retroperitoneal hydatid cyst. Apropos of a case].

    PubMed

    Ouadfel, J; Assem, A; Errougani, A; Jalil, A; Belkacem, R; Balafrej, S

    1990-01-01

    This is a report of a rarely observed localisation of hydatid cyst: the retroperitoneal space. The hypotheses of pathogenesis advanced are reviewed and the interest of intravenous urography and ultrasonography in the diagnosis is emphasized. Surgery the only treatment, consists of partial resection of the cyst.

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

    PubMed

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

    2012-01-01

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

  5. Lymphangiovenous malformation--a rare cause of giant retroperitoneal cyst.

    PubMed

    Ibrarullah, M D; Saxena, R; Sikora, S S; Haque, I; Choudhury, G; Gupta, R

    1993-12-01

    A giant retroperitoneal cyst manifesting as congenital inguino-scrotal swelling to begin with, is reported. The abdominal swelling became clinically obvious at the age of five years because of rapid enlargement over a period of one month. A multiloculated cyst was revealed on preoperative ultrasonography. Intracystic hemorrhage necessitated emergency surgical exploration and excision. Histology of the cyst revealed lymph-angio-venous malformation.

  6. Retroperitoneal dermoid presenting as an infected pancreatic cyst.

    PubMed

    Dewar, G; Arnold, M; Li, A K

    1990-06-01

    Bacteroides fragilis infection of a juxtapancreatic benign cystic retroperitoneal teratoma in a 28 year old Chinese male is reported. Preliminary drainage was followed by excision. The radiological and pathological findings of this rare tumour are reported with emphasis on the differential diagnosis which includes inflammatory and neoplastic pancreatic cysts.

  7. [Ureterohydronephrosis secondary to a cyst of retroperitoneal mesentery].

    PubMed

    Vicente Prados, F J; Martínez Morcillo, A; Tallada Buñuel, M; Cózar Olmo, J M; Espejo Maldonado, E; Pedrajas de Torres, G

    1998-02-01

    Retroperitoneal primary cysts are rare clinical entities. A contribution is made of one case presenting this condition with repercussion on the excretory route. Ultrasound and computerized axial tomography studies suggest the diagnosis but this is confirmed through laparotomy. Choice treatment is enucleation, typically easy because of the minimal adherence to surrounding structures. Recurrence is rare and malignancy practically non-existent.

  8. Recurrent retroperitoneal mesenteric cyst. A case report and review.

    PubMed

    Chirathivat, S; Shermeta, D

    1979-04-15

    A mesenteric cyst is an uncommon cause of a palpable abdominal mass. A correct preoperative diagnosis can be made by the combined use of radiographic and sonographic examinations in conjunction with the clinical features. A patient with a retroperitoneal cyst extending into the leaves of the mesentery is presented. The cyst recurred 4 years after surgery.

  9. Retroperitoneal oncocytoma: case report and review of the imaging features.

    PubMed

    Roy, A A; Jameson, C; Christmas, T J; Aslam Sohaib, S

    2011-08-01

    Oncocytomas are uncommon tumours that occur in a number of specific anatomical locations within the head, neck, chest, abdomen and pelvis. When occurring in the retroperitoneum, oncocytomas almost always arise from either the kidney or adrenal gland. With this case we present the imaging findings of an exceptionally rare retroperitoneal oncocytoma whose site of origin is neither the kidney nor adrenal gland.

  10. A Retroperitoneal Neuroendocrine Tumor in Ectopic Pancreatic Tissue

    PubMed Central

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

    2013-01-01

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

  11. 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

  12. [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.

  13. [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

  14. 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. PMID:27630937

  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. Solitary pulmonary nodule: pleuropulmonary synovial sarcoma.

    PubMed

    Ward, Robert C; Birnbaum, Ariel E; Aswad, Bassam I; Healey, Terrance T

    2014-05-01

    Pleuropulmonary synovial sarcoma (PPSS) is an extremely rare primary malignancy of the lung. We present a case of a middle-aged female with PPSS that was initially discovered as an incidental indeterminate nodule on chest radiograph. Following evaluation with computed tomography (CT), the patient went on to positron-emission tomography (PET)/CT for work-up of the solitary pulmonary nodule, which demonstrated mild FDG-avidity and no other evidence of FDG-avid disease. The patient then underwent thoracotomy and right upper lobectomy for definitive treatment. Only after evaluation of the gross pathology, histology, immunohistochemistry and cytogenetics was the diagnosis of synovial sarcoma made. Importantly, the preceding PET/CT, in addition to physical exam of the upper and lower extremities, helped exclude the more common extra-thoracic soft-tissue variety of synovial sarcoma, which frequently metastasizes to lung, carrying a worse prognosis. Discussion of synovial sarcoma and PPSS follows. PMID:24791267

  18. 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

  19. 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 ...

  20. Penile Sarcoma: Report of a Rare Malignancy.

    PubMed

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

    2016-07-01

    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. PMID:27630937

  1. [Possibilities of computerized tomography in the diagnosis of abscesses in the abdominal cavity and retroperitoneal space].

    PubMed

    Kishkovskiĭ, A N; Bazhanov, E A

    1987-07-01

    The article presents results of the computer tomography examination of 205 patients at different terms after operations who were clinically suspected of the development of a limited pyo-inflammatory process in the abdominal cavity or retroperitoneal space. Abscesses were revealed in 112 patients, inflammatory infiltrates in 16 patients, the development of purulent complications was excluded in 77 patients which allowed relaparotomy to be rejected in 93 patients. The computer tomography symptoms of abdominal abscesses were established. The dependence of the abscess semiotics on the stage of the suppurative process, its specific localization, intra- or extraorganic disposition, contents and size of the abscess was shown.

  2. 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.

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

    ClinicalTrials.gov

    2016-09-12

    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

  4. Myeloid Sarcoma: Current Approach and Therapeutic Options

    PubMed Central

    Avni, Batia; Koren-Michowitz, Maya

    2011-01-01

    Myeloid sarcoma is a rare disease that can present as an isolated extramedullary leukemic tumor, concurrently with or at relapse of acute myeloid leukemia. Owing to the rarity of this disorder, most of the literature comprises small retrospective studies and case reports. The aim of this review is to summarize the current published data regarding the clinical presentation, morphological, cytogenetic and molecular features, prognosis and treatment of myeloid sarcoma. PMID:23556098

  5. Imaging spectrum in soft tissue sarcomas.

    PubMed

    Aga, Pallavi; Singh, Ragini; Parihar, Anit; Parashari, Umesh

    2011-12-01

    Imaging plays an important role in detection, diagnosis as well as pre and post operative management of patients with soft tissue sarcomas. Soft tissue sarcomas are generally a diagnostic dilemma needing the complimentary use of both radiology and pathology for their accurate diagnosis. In this review article, we have tried to highlight the important facts about the various imaging modalities available as well as the recent advances in the field of radiology. PMID:23204782

  6. 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.

  7. 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

  8. 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

  9. Potential Therapeutic Targets in Uterine Sarcomas

    PubMed Central

    Cuppens, Tine; Tuyaerts, Sandra; Amant, Frédéric

    2015-01-01

    Uterine sarcomas are rare tumors accounting for 3,4% of all uterine cancers. Even after radical hysterectomy, most patients relapse or present with distant metastases. The very limited clinical benefit of adjuvant cytotoxic treatments is reflected by high mortality rates, emphasizing the need for new treatment strategies. This review summarizes rising potential targets in four distinct subtypes of uterine sarcomas: leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, and undifferentiated uterine sarcoma. Based on clinical reports, promising approaches for uterine leiomyosarcoma patients include inhibition of VEGF and mTOR signaling, preferably in combination with other targeted or cytotoxic compounds. Currently, the only targeted therapy approved in leiomyosarcoma patients is pazopanib, a multitargeted inhibitor blocking VEGFR, PDGFR, FGFR, and c-KIT. Additionally, preclinical evidence suggests effect of the inhibition of histone deacetylases, tyrosine kinase receptors, and the mitotic checkpoint protein aurora kinase A. In low-grade endometrial stromal sarcomas, antihormonal therapies including aromatase inhibitors and progestins have proven activity. Other potential targets are PDGFR, VEGFR, and histone deacetylases. In high-grade ESS that carry the YWHAE/FAM22A/B fusion gene, the generated 14-3-3 oncoprotein is a putative target, next to c-KIT and the Wnt pathway. The observation of heterogeneity within uterine sarcoma subtypes warrants a personalized treatment approach. PMID:26576131

  10. 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

  11. 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

  12. 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

  13. 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.

  14. [Systemic therapy and hyperthermia for locally advanced soft tissue sarcoma].

    PubMed

    Lindner, L H; Angele, M; Dürr, H R; Rauch, J; Bruns, C

    2014-05-01

    Patients with high-risk soft tissue sarcomas (FNCLCC grades 2-3, > 5 cm and deep lying) are at a high risk of local recurrence or distant metastases despite optimal surgical tumor resection. Therefore, multimodal treatment should be considered for this difficult to treat patient group. Besides surgery, radiation therapy and chemotherapy, hyperthermia has become a valid, complementary treatment option within multimodal treatment concepts. Hyperthermia in this context means the selective heating of the tumor region to temperatures of 40-43 °C for 60 min by microwave radiation in addition to simultaneous chemotherapy or radiation therapy. A randomized phase III study demonstrated that the addition of hyperthermia to neoadjuvant chemotherapy improved tumor response and was associated with a minimal risk of early disease progression as compared to chemotherapy alone. The addition of hyperthermia to a multimodal treatment regimen for high-risk soft tissue sarcoma consisting of surgery, radiation therapy and chemotherapy, either in the neoadjuvant or adjuvant setting after incomplete or marginal tumor resection, significantly improved local progression-free and disease-free survival. Based on these results and due to the generally good tolerability of hyperthermia, this treatment method in combination with chemotherapy should be considered as a standard treatment option within multimodal treatment approaches for locally advanced high-risk soft tissue sarcoma.

  15. 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).

  16. 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

  17. 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

  18. 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

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

    PubMed

    Mirilas, Petros; Skandalakis, John E

    2010-04-01

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

  20. 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