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Rhabdoid Tumor Predisposition Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
INI-1 is retained in both rhabdomyosarcoma and desmoplastic small round cell tumor, which aids in the differential diagnostic consideration [27]. INI-1 can be lost in synovial sarcoma, which is commonly immunoreactive to CD99, cytokeratin, and epithelial membrane antigen [27]. TLE1, positive in synovial sarcoma, has yet to be examined in malignant rhabdoid tumors [27]. Cytogenetic studies can be helpful in difficult cases. Epithelioid sarcoma proves to be the most difficult to separate from the rhabdoid tumors. It often has rhabdoid morphology, is positive for epithelial and mesenchymal markers, and also demonstrates loss of INI-1 [27]. There are differences in the clinical presentation, as malignant rhabdoid tumors tend to occur in very young children and epithelioid sarcoma tends to occur over a wide age range. The sites of presentation also differ [27].
Histopathological aspects of peritoneal malignancy
Published in Tom Cecil, John Bunni, Akash Mehta, A Practical Guide to Peritoneal Malignancy, 2019
Babatunde Rowaiye, Norman Carr
Desmoplastic small round cell tumour (DSRCT) usually occurs in children and young adults and is more common in males [63]. It generally arises in the abdominopelvic cavity and produces a large mass, sometimes with multifocal nodules. Almost all DSRCTs have a t(11;22)(p24;q12) translocation associated with an EWS-WT1 gene fusion transcript [63,64].
Oncology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Gill A. Levitt, Penelope Brock, Tanzina Chowdhury, Mark Gaze, Darren Hargrave, Judith Kingston, Antony Michalski, Olga Slater
Other STS subtypes more commonly seen in adolescents and young adults are synovial sarcoma, desmoplastic small round cell tumour and other entities, where, in general, local control of the primary tumour is the main problem and metastases are unusual. Two types of STS are seen in children.
Desmoplastic small round cell tumor: from state of the art to future clinical prospects
Published in Expert Review of Anticancer Therapy, 2023
Shushan Hovsepyan, Claudia Giani, Sandro Pasquali, Angela Di Giannatale, Stefano Chiaravalli, Chiara Colombo, Daniel Orbach, Luca Bergamaschi, Sabina Vennarini, Susanne Andrea Gatz, Patrizia Gasparini, Pablo Berlanga, Michela Casanova, Andrea Ferrari
Desmoplastic small round cell tumor (DSRCT) is an extremely rare and highly aggressive soft tissue sarcoma that generally affects male adolescents and young adults. Its incidence is approximately 0.2 cases per million people [1,2]. When first described by Gerald and Rosai in 1989 [3], DSRCT mainly presents with multiple nodules disseminated within the abdominopelvic cavity and arising from peritoneal surfaces. Its clinical presentation is typically related to an abdominal mass, and patients are usually diagnosed already in advanced stages of the disease: at diagnosis, patients with DSRCT have synchronous peritoneal metastases in more than 90% of cases, and synchronous extraperitoneal metastases in around 50% of cases, mostly to the liver, lung, and bones [4–6]. DSRCT is associated with a chromosomal translocation t(11;22) (p13; q12) that leads to the EWSR1:WT1 fusion gene [7,8].
Changing incidence and survival of desmoplastic small round cell tumor in the USA
Published in Baylor University Medical Center Proceedings, 2022
Syed Hamza Bin Waqar, Hassam Ali
Desmoplastic small round cell tumor (DSRCT) is a highly uncommon and aggressive soft tissue sarcoma with a poor prognosis. First described in 1989 by Gerald and Rosai based on distinct clinicopathological features of small round blue cells intermixed in the desmoplastic stroma, DSRCT remains a reasonably new tumor with sparse incidence.1 Limited cohort analyses, such as a Brazilian study by Campos et al, a UK study by Honore et al, and a Chinese study by Xiang et al, suggest a median age of diagnosis around 24 to 26 years with male predominance.2–5 Current literature is extremely scarce regarding the incidence, racial disparities, and survival of patients with DSRCT, perhaps because of its rarity. We examined incidence, risk ratios, and survival, focusing on patient demographics and factors affecting survival, by utilizing the Surveillance, Epidemiology, and End Results (SEER) database, which provides information on patient demographics and survival. We intended to extend knowledge about DSRCT by examining factors associated with improved survival in these patients.
Investigational therapies in phase II clinical trials for the treatment of soft tissue sarcoma
Published in Expert Opinion on Investigational Drugs, 2019
Juan Martin-Liberal, Ezequiel Pérez, Xavier García Del Muro
Chimeric antigen receptor (CAR) T-cell treatment is modality of adaptive cell therapy in which the patient’s own T-cells are isolated and then reinfused into the patient after being modified genetically to make them express a CAR able to recognize a specific tumor antigen [75]. The CAR is generally composed of an extracellular antigen-recognition domain linked to an intracellular signalling domain, usually the CD3ζ chain of the T-cell receptor. Its activation by the antigen recognition leads to T-cell proliferation and triggers the killing of the tumor cell [76]. CAR T-cell therapy is being highly successful in haematological malignancies and its efficacy in solid tumors is under assessment [77]. In sarcomas, a study published in 2015 reported the outcomes of 19 pretreated sarcoma patients with HER-2 expression treated with a CAR T-cell directed to HER-2. Nearly all patients had osteosarcoma or Ewing sarcoma but 1 of the patients enrolled had advanced desmoplastic small round cell tumor. This patient had SD for 14 months, which is encouraging for a STS subtype characterized by its aggressiveness and its rapid growth rate. Median OS of all 19 patients was 10.3 months and treatment was well tolerated. CAR T-cells were found in peripheral blood several weeks after the infusion without signs of toxicity [78].