Abstract
Intra-abdominal desmoplastic small round cell tumor (IDSRCT) is a rare and highly malignant soft tissue neoplasm, which is characterized by rapid progression and poor prognosis. The mechanism underlying the development of this neoplasm remains elusive, but all cases are characterized by the chromosomal translocation t (11;22) (p13; q12), which results in a formation of EWSR1-WT1 gene fusion. The diagnosis of IDSRCT is often made with core-needle tissue biopsy specimens or laparoscopy or laparotomy. Immunohistochemical analyses have shown the co-expression of epithelial, neuronal, myogenic, and mesenchymal differentiation markers. FISH or reverse transcription polymerase chain reaction detecting EWS-WT1 fusion can be performed to assist in molecular confirmation. There is no standard of care for patients with IDSRCT currently, and majority of newly diagnosed patients received the aggressive therapy, which includes >90% resection of surgical debulking, high-dose alkylator-based chemotherapy, and radiotherapy. More recently, targeted therapy has been increasingly administered to recurrent IDSRCT patients and has been associated with improved survival in clinical conditions. Immunotherapy as a possible therapeutic strategy is being explored in patients with IDSRCT. In this review, we summarize currently available knowledge regarding the epidemiology, potential mechanisms, clinical manifestations, diagnosis, treatment, and prognosis of IDSRCT to assist oncologists in comprehensively recognizing and accurately treating this malignancy.
Highlights
Desmoplastic small round cell tumor (DSRCT), according to the International Classification of Disease for Oncology (2020), is categorized as a malignant tumor of uncertain differentiation
intra-abdominal desmoplastic small round cell tumor (IDSRCT) patients who progress or relapse after first- and second-line treatment can first receive anti-vascular endothelial growth factor (VEGF) monoclonal antibodies, like bevacizumab or tyrosine kinase inhibitors (TKI), that act on multiple pathways simultaneously (e.g., c-Kit, PDGFR-a and -b, and VEGFR-1, 2, and 3, Flt3, RET), including pazopanib, sunitinib, anlotinib, and apatinib
IDSRCT refers to a rare and aggressive soft tissue malignancy that predominantly occurs in a young male population
Summary
Desmoplastic small round cell tumor (DSRCT), according to the International Classification of Disease for Oncology (2020), is categorized as a malignant tumor of uncertain differentiation. DSRCT typically occurs in the abdominal cavity, which is known as intra-abdominal desmoplastic small round cell tumor (IDSRCT) [1]. Other primary sites have been reported (Figure 1). It is a rare and aggressive malignant soft tissue sarcoma that predominantly occurs in young male adults [20]. There is a lack of international consensus regarding the treatment of IDSRCT, and therapeutic regimens were derived from Ewing sarcoma’s (ES) treatment because of the involvement of the EWS gene and activation of similar oncogenic pathways in both ES and IDSRCT. This review summarizes the current knowledge on the epidemiology, potential mechanisms, clinical manifestations, diagnosis, treatment, and prognosis of IDSRCT, highlighting the modalities of treatment
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