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Sarcoma retroperitoneal

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https://www.readbyqxmd.com/read/29761250/sarcoma-in-neurofibromatosis-2-case-report-and-review-of-the-literature
#1
C Linder, M J Smith, M Bulman, A Wallace, A J Freemont, D C Mangham, D G R Evans
Neurofibromatosis type 2 (NF2) is associated with the development of several types of benign nervous system tumours, while malignancies are rare. We report a 22-year-old man who presented with retroperitoneal and spinal high-grade sarcomas with epithelial features. Samples showed a mixed epithelioid and spindled cell content with little associated matrix and inconclusive immunochemistry. Genetic analysis of a schwannoma and matched blood samples demonstrated a constitutional de novo substitution at the splice donor site of intron 8 of the NF2 gene and aa acquired large deletion of the entire NF2 gene as a second hit, with some loss of SMARCB1...
May 14, 2018: Familial Cancer
https://www.readbyqxmd.com/read/29752328/soft-tissue-sarcoma-version-2-2018-nccn-clinical-practice-guidelines-in-oncology
#2
Margaret von Mehren, R Lor Randall, Robert S Benjamin, Sarah Boles, Marilyn M Bui, Kristen N Ganjoo, Suzanne George, Ricardo J Gonzalez, Martin J Heslin, John M Kane, Vicki Keedy, Edward Kim, Henry Koon, Joel Mayerson, Martin McCarter, Sean V McGarry, Christian Meyer, Zachary S Morris, Richard J O'Donnell, Alberto S Pappo, I Benjamin Paz, Ivy A Petersen, John D Pfeifer, Richard F Riedel, Bernice Ruo, Scott Schuetze, William D Tap, Jeffrey D Wayne, Mary Anne Bergman, Jillian L Scavone
Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for STS provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumors, desmoid tumors, and rhabdomyosarcoma...
May 2018: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/29723411/the-clinical-behavior-of-well-differentiated-liposarcoma-can-be-extremely-variable-a-retrospective-cohort-study-at-a-major-sarcoma-center
#3
Emily Z Keung, Naruhiko Ikoma, Robert Benjamin, Wei-Lien Wang, Alexander J Lazar, Barry W Feig
BACKGROUND: The pathogenesis of well differentiated liposarcoma (WDLPS) is poorly understood and pathologic characterization is often challenging. Descriptive terms (such as sclerosing, myxoid, inflammatory, spindle cell) are frequently encountered in the pathology reports and are of unknown clinical significance. METHODS: Sixty-two patients with primary retroperitoneal WDLPS resected at our institution were identified (1996-2011). Pathology reports of surgical resection specimens were retrospectively reviewed and descriptive qualifiers to the WDLPS diagnosis noted...
May 3, 2018: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29707226/a-rare-case-of-retroperitoneal-synovial-sarcoma
#4
C S Wong, A Harris, R Kennedy, O P Houghton, P D Carey
Management of retroperitoneal soft tissue sarcomas is complex. Treatment is usually multimodal; involving surgery, chemotherapy and radiotherapy.
April 2018: JRSM Open
https://www.readbyqxmd.com/read/29599376/effectiveness-and-safety-of-tissue-expander-for-adjuvant-helical-tomotherapy-in-curatively-resected-retroperitoneal-sarcoma
#5
Jeong Il Yu, DO Hoon Lim, Hee Chul Park, Sang Hoon Jung, Seong Jin Jeon, Heerim Nam, Bokyoung Kim, Sung-Joo Kim, Jae Berm Park
BACKGROUND/AIM: We investigated the effectiveness and safety of using a tissue expander (TE) for adjuvant helical tomotherapy (HT) for curatively resected retroperitoneal sarcoma (RPS). MATERIALS AND METHODS: This study was conducted with 60 RPS patients who underwent curative resection with or without TE insertion followed by HT from June 2009 to December 2016. Among the patients, TE was inserted in 37 (61.7%). The quality of TE insertion was evaluated. Median follow-up after surgery was 19...
April 2018: Anticancer Research
https://www.readbyqxmd.com/read/29532681/dedifferentiated-liposarcoma-with-rhabdomyosarcomatous-differentiation-producing-hcg-a-case-report-of-a-diagnostic-pitfall
#6
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
https://www.readbyqxmd.com/read/29520652/salvage-surgery-for-recurrent-retroperitoneal-well-differentiated-liposarcoma-early-reoperation-may-not-provide-benefit
#7
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
https://www.readbyqxmd.com/read/29507712/different-quality-of-treatment-in-retroperitoneal-sarcomas-rps-according-to-hospital-case-volume-and-surgeon-case-volume-a-retrospective-regional-analysis-in-italy
#8
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
https://www.readbyqxmd.com/read/29503221/collaborating-with-our-adult-colleagues-a-case-series-of-robotic-surgery-for-suspicious-and-cancerous-lesions-in-children-and-young-adults-performed-in-a-free-standing-children-s-hospital
#9
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
https://www.readbyqxmd.com/read/29487221/a-phase-ii-study-of-tumor-ablation-in-patients-with-metastatic-sarcoma-stable-on-chemotherapy
#10
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
https://www.readbyqxmd.com/read/29472043/neoadjuvant-and-adjuvant-strategies-in-retroperitoneal-sarcoma
#11
REVIEW
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...
May 2018: European Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29452860/pancreaticoduodenectomy-in-the-surgical-management-of-primary-retroperitoneal-sarcoma
#12
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
https://www.readbyqxmd.com/read/29449525/anterior-approach-to-en-bloc-resection-in-left-sided-retroperitoneal-sarcoma-with-adjacent-organ-involvement-a-study-of-25-patients-in-a-single-center
#13
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
https://www.readbyqxmd.com/read/29432564/management-of-metastatic-retroperitoneal-sarcoma-a-consensus-approach-from-the-trans-atlantic-retroperitoneal-sarcoma-working-group-tarpswg
#14
(no author information available yet)
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...
April 1, 2018: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/29395417/retroperitoneal-follicular-dendritic-cell-sarcoma-in-a-young-woman-diagnosis-and-treatment-challenges
#15
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
https://www.readbyqxmd.com/read/29394626/-a-case-of-retroperitoneal-liposarcoma-that-invaded-and-filled-the-jejunal-lumen
#16
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
https://www.readbyqxmd.com/read/29385413/abdominopelvic-and-retroperitoneal-low-grade-fibromyxoid-sarcoma-a-clinicopathologic-study-of-13-cases
#17
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
https://www.readbyqxmd.com/read/29365418/-the-preliminary-report-of-a-registration-clinical-trial-of-proton-and-heavy-ion-irradiation
#18
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]
https://www.readbyqxmd.com/read/29354752/primary-leiomyosarcoma-of-the-spine-treated-with-total-en-bloc-spondylectomy-the-first-case-in-thailand-a-case-report-and-literature-review
#19
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)
https://www.readbyqxmd.com/read/29353660/intraoperative-electron-radiation-therapy-in-retroperitoneal-sarcoma
#20
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
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