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Retroperitoneal sarcoma

Elena Maryamchik, Kirill A Lyapichev, Bradford Halliday, Andrew E Rosenberg
We report a first case of paraneoplastic human chorionic gonadotropin (HCG) production in a dedifferentiated liposarcoma with rhabdosarcomatous differentiation in an 83-year-old man with a retroperitoneal mass, unilateral scrotal enlargement, and a serum HCG level of 843 IU/L. Core biopsy of the retroperitoneal mass revealed rhabdomyosarcoma. Orchiectomy revealed a paratesticular dedifferentiated liposarcoma with rhabdosarcomatous differentiation. Fluorescence in situ hybridization analysis performed on both the retroperitoneal and paratesticular masses revealed amplification of MDM2...
March 1, 2018: International Journal of Surgical Pathology
Naruhiko Ikoma, Christina L Roland, Keila E Torres, Yi-Ju Chiang, Wei-Lien Wang, Neeta Somaiah, Gary N Mann, Kelly K Hunt, Janice N Cormier, Barry W Feig
PURPOSE: Current evidence regarding salvage resection for recurrent retroperitoneal (RP) sarcomas generally lacks detailed histology-specific analyses, but the aggressiveness of these tumors varies widely by histology. We investigated associations between timing and extent of salvage surgery and survival outcomes in patients with recurrent RP well-differentiated liposarcoma (WDLPS). METHODS: The University of Texas MD Anderson Cancer Center Surgical Oncology sarcoma database was reviewed to identify patients with RP WDLPS who underwent surgical resection for first recurrent disease (salvage surgery) in 1995-2015...
March 8, 2018: Annals of Surgical Oncology
Sergio Sandrucci, Agostino Ponzetti, Claudio Gianotti, Baudolino Mussa, Patrizia Lista, Giovanni Grignani, Marinella Mistrangelo, Oscar Bertetto, Daniela Di Cuonzo, Giovannino Ciccone
Background: Retroperitoneal sarcomas (RPS) should be surgically managed in specialized sarcoma centers. However, it is not clearly demonstrated if clinical outcome is more influenced by Center Case Volume (CCV) or by Surgeon Case Volume (SCV). The aim of this study is to retrospectively explore the relationship between CCV and SCV and the quality of surgery in a wide region of Northern Italy. Methods: We retrospectively collected data about patients M0 surgically treated for RPSs in 22 different hospitals from 2006 to 2011, dividing them in two hospital groups according to sarcoma clinical activity volume (HCV, high case volume or LCV, low case volume hospitals)...
2018: Clinical Sarcoma Research
Briony K Varda, Patricia Cho, Andrew A Wagner, Richard S Lee
BACKGROUND: In adult urologic oncology the use of robotics has become commonplace; in pediatric urology it is rare. Herein, we describe a collaboration between an adult and a pediatric urologist performing robotic surgery for children and young adults with suspicious or cancerous genitourinary (GU) lesions. OBJECTIVES: To evaluate clinical and oncologic outcomes in children and young adults undergoing robotic surgery for suspicious or cancerous lesions of the GU tract; to describe our collaborative model between an adult and pediatric surgeon at a free-standing children's hospital...
March 2, 2018: Journal of Pediatric Urology
Angela C Hirbe, Jack Jennings, Nael Saad, Joseph D Giardina, Yu Tao, Jingqin Luo, Shellie Berry, Jacqui Toeniskoetter, Brian A Van Tine
LESSONS LEARNED: Ablation therapy appears to be a reasonably safe and effective approach to obtain a significant treatment-free interval for a subset of patients with limited sites of metastatic disease for which systemic control can be obtained with six cycles of chemotherapy. BACKGROUND: Metastatic sarcoma often becomes resistant to treatment by chemotherapy. There is sometimes prolonged stable disease from active chemotherapy that provides a window of opportunity for an intervention to prolong disease-free survival...
February 27, 2018: Oncologist
L Max Almond, Alessandro Gronchi, Dirk Strauss, Mariam Jafri, Samuel Ford, Anant Desai
Extended surgery remains the mainstay of treatment in retroperitoneal sarcoma, although conflicting data exist on the benefit of neoadjuvant and adjuvant therapies, particularly with regard to tumour grade and histological type. Experience of radiotherapy and chemotherapy in extremity soft tissue sarcoma can inform treatment strategies, however these data cannot be universally extrapolated to the retroperitoneum where disease biology and anatomical considerations are different. The present review sets a historical context before discussing recent evidence and on-going multi-centre trials in retroperitoneal sarcoma...
February 9, 2018: European Journal of Surgical Oncology
William W Tseng, Denice D Tsao-Wei, Dario Callegaro, Giovanni Grignani, Lorenzo D'Ambrosio, Sylvie Bonvalot, Cecilia G Ethun, Kenneth Cardona, John T Mullen, Robert J Canter, John E Mullinax, Ricardo J Gonzalez, Frits van Coevorden, Markus Albertsmeier, Kiran K Dhanireddy, Salvatore L Renne, Alessandro Gronchi
BACKGROUND: In retroperitoneal sarcoma (RPS), the optimal extent of resection must balance adequate disease control with potential for morbidity. We sought to study the frequency and outcomes after a Whipple procedure or pancreaticoduodenectomy (PD) in patients undergoing resection for primary RPS. METHODS: Participating referral centers within the Trans-Atlantic Retroperitoneal Sarcoma Working Group provided retrospective data from January 2007 to December 2016 for patients with primary RPS who underwent PD along with the total number of consecutive resections done during the same time period...
February 5, 2018: European Journal of Surgical Oncology
Zhen Wang, Jian-Hui Wu, Ang Lv, Cheng-Peng Li, Xiu-Yun Tian, Chun-Yi Hao
BACKGROUND There is no standard surgical approach for the management of retroperitoneal sarcoma. The aim of this clinical study was to describe the experience of an anterior approach to en bloc resection in left-sided retroperitoneal sarcoma with adjacent organ involvement. MATERIAL AND METHODS This retrospective clinical study included 25 patients who were diagnosed with left-sided retroperitoneal sarcoma and underwent tumor resection at a single center between May 2012 and July 2017. All patients had tumors that were adjacent to the left colon, pancreas, left kidney, left adrenal gland, and psoas major; some of the tumors were adjacent to the diaphragm, stomach, and small intestine...
February 16, 2018: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
A J MacNeill, W J Van Houdt, C J Swallow, A Gronchi
Introduction: Retroperitoneal sarcoma (RPS) is a rare disease accounting for 0.1-0.2% of all malignancies. Management of RPS is complex and requires multidisciplinary, tailored treatment strategies at all stages, but especially in the context of metastatic or multifocal recurrent disease. Due to the rarity and heterogeneity of this family of diseases, the literature to guide management is limited. Methods: The Trans-Atlantic Retroperitoneal Sarcoma Working Group (TARPSWG) is an international collaboration of sarcoma experts from all disciplines convened in an effort to overcome these limitations...
February 7, 2018: Annals of Oncology: Official Journal of the European Society for Medical Oncology
Rym Bouriga, Nihed Abdessaied, Makram Hochlef, Najoua Mallat, Mohamed Mahjoub, Badereddine Sriha, Slim Ben Ahmed
INTRODUCTION: Follicular dendritic cell sarcoma (FDCS) is an uncommon tumor that usually arises in lymph nodes, especially in the cervical, mediastinal, or axillary areas, but rarely in extranodal sites. Few cases have been reported in English literature so far. The scarcity may be partially due to under-recognition of this entity. Through this case report we analyzed the difficulties of clinical and pathological diagnosis of this rare tumor with its unusual location mistaken it with gynecological cancer's iliac lymph nodes metastases...
January 6, 2018: Current Problems in Cancer
Tatsuhiko Kohno, Katsunobu Oyama, Shiro Terai, Koichi Okamoto, Shinichi Nakanuma, Jun Kinoshita, Isamu Makino, Keishi Nakamura, Hironori Hayashi, Tomoharu Miyashita, Hidehiro Tajima, Hiroyuki Takamura, Itasu Ninomiya, Sachio Fushida, Tetsuo Ohta
A jejunal tumor was found with computed tomography in a 79-year-old man with a history of gastrectomy reconstructed with Billroth II method for gastric ulcers. The tumor with contrast effect extended into the afferent loop and invaded the retroperitoneum in the dorsal side. The tumor occupied the jejunal lumen in endoscopic examination. Malignant spindle cells were found in the biopsy specimen. An increase of MDM2 and CDK4 signals were observed in fluorescence in situ hybridization( FISH). Given the preoperative diagnosis of jejunal liposarcoma, we performed a resection of the tumor with partial jejunectomy and partial gastrectomy...
November 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
Nasir Ud Din, Zubair Ahmad, Riyam Zreik, Andrew Horvai, Andrew L Folpe, Karen Fritchie
Objectives: Low-grade fibromyxoid sarcoma (LGFMS) is a rare tumor often arising in the lower extremities. Only rare examples in the abdominal cavity, pelvis, and retroperitoneum have been reported. Methods: cases of abdominopelvic and retroperitoneal LGFMS were retrieved. MUC4, Actin, ALK, β-catenin, desmin, DOG1, KIT, S100 protein, and STAT6 testing was performed, and a subset was tested for FUS rearrangement. Results: Sites included intra-abdominal/abdominal wall (four cases), retroperitoneum (three cases), pelvis (three cases), small bowel (two cases), and kidney (one case) (median size, 15 cm; age range, 5-61 years)...
January 29, 2018: American Journal of Clinical Pathology
J D Lu, M Ye, Q Guo, J F Fu, X M Moyers, S T Zhang, N Y Mao, Y S Kong, X Hsi, X Y Shahnazi, J H Zhao, B Zhang, J Y Ma, Y X Z Lai, W Zhang, J Ma, L N Gao, X M Cai, S Guan, G L Zhang, J F Wu, L Cheng, Z Sheng, X M Ren, Jun Zhao, Lining Sun, Guoliang Jiang
Objective: To verify the safety and efficacy of IONTRIS particle therapy system (IONTRIS) in clinical implementation. Methods: Between 6.2014 and 8.2014, a total of 35 patients were enrolled into this trial: 31 males and 4 females with a median age of 69 yrs (range 39-80). Ten patients had locally recurrent head and neck tumors after surgery, 4 cases with thoracic malignancies, 1 case with hepatocellular carcinoma, 1 case with retroperitoneal sarcoma, and 19 cases with non-metastatic prostate carcinomas. Phantom dose verification was mandatory for each field before the start of radiation...
January 23, 2018: Zhonghua Zhong Liu za Zhi [Chinese Journal of Oncology]
Tinnakorn Pluemvitayaporn, Chaiwat Piyaskulkaew, Sombat Kunakornsawat, Pritsanai Pruttikul
Leiomyosarcoma is a rare type of malignant soft tissue tumor and also one of the most aggressive soft tissue sarcomas. It commonly occurs in uterus, abdominal viscera, retroperitoneal space and soft tissue of the extremities. Primary osseous leiomyosarcoma is a rare condition. Furthermore, primary leiomyosarcoma of the spine is extremely rare. Only few cases have been reported. However, the treatment and outcomes remains controversial. Therefore, the objective of this case report is to illustrate the management of this extremely rare disease by using total en bloc spondylectomy (TES) procedure, which is one of a suitable option for surgical resection of the spinal tumors...
December 2017: Journal of Spine Surgery (Hong Kong)
Falk Roeder, Ingo Alldinger, Matthias Uhl, Ladan Saleh-Ebrahimi, Simon Schimmack, Gunhild Mechtersheimer, Markus W Büchler, Jürgen Debus, Robert Krempien, Alexis Ulrich
PURPOSE: To report our experience with surgery, intraoperative radiation therapy (IORT), and external beam radiation therapy (EBRT) in retroperitoneal soft-tissue sarcoma. METHODS AND MATERIALS: We conducted a retrospective evaluation of 156 patients (69 primary, 87 recurrent) treated with IORT since 1991. The dominant histology was dedifferentiated liposarcoma (49%); 89% of lesions were high grade. Median tumor size was 11 cm. Surgery resulted in gross complete resection in 92%, and 65% had microscopically positive margins...
February 1, 2018: International Journal of Radiation Oncology, Biology, Physics
Roberto Herrera-Goepfert
Synovial sarcoma is a ubiquitous neoplasm predominantly affecting soft tissues of young adults of any gender; few cases have been described in the digestive system, mostly in the stomach. The (X;18)(p11.2; q11.2) translocation yields unique SS18-SSX fusion genes. Synovial sarcoma has been related to radiotherapy, but no synovial sarcoma has been associated with the digestive system. This article describes the case of a synovial sarcoma arising along the extrahepatic biliary tree, 10 years after the application of an abdominal radiotherapy schedule due to a retroperitoneal metastatic seminoma in a male who developed progressive obstructive jaundice...
January 1, 2018: International Journal of Surgical Pathology
Sara A Mansfield, Raphael E Pollock, Valerie P Grignol
Retroperitoneal sarcomas are rare tumors of which liposarcoma is the most common histology. Surgical resection remains the mainstay of therapy, particularly for the well-differentiated subtype. They can grow to massive size before causing symptoms or detection. Well-differentiated liposarcoma, while having a negligible metastatic rate, is fraught with a high local recurrence rate, despite a complete surgical resection. Reasons for this are not completely known but may be related to a field defect of the retroperitoneal fat creating a niche for recurrence...
January 16, 2018: Current Treatment Options in Oncology
Mark Fairweather, Ricardo J Gonzalez, Dirk Strauss, Chandrajit P Raut
Surgery for primary retroperitoneal sarcomas (RPS) often requires a technically challenging, en bloc multivisceral resection to optimize outcomes. Surgery may also be appropriate for patients with localized recurrent RPS. Anatomic considerations and tumor biology driven by histologic subtype may guide the extent of resection in patients with RPS. This review provides an overview of the current surgical principles for primary and recurrent RPS.
January 2018: Journal of Surgical Oncology
Andrea J MacNeill, Marco Fiore
Resection of retroperitoneal sarcoma (RPS) typically involves multivisceral resection. The morbidity of RPS resection has decreased over time despite widespread adoption of radical resection. Certain patterns of resection are associated with higher complication rates and elderly patients are at increased risk of morbidity. Administration of preoperative radiotherapy does not increase morbidity, but intraoperative and brachytherapy techniques are associated with heightened toxicities. Long-term functional outcomes and quality of life scores after RPS resection are acceptable...
January 3, 2018: Journal of Surgical Oncology
Fabiano Reis, Eduardo Macedo, Marcondes Cavalcanti França Junior, Eliane Ingrid Amstalden, Simone Appenzeller
No abstract text is available yet for this article.
November 2017: Radiologia Brasileira
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