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DSRCT — SCE Medical Oncology MCQ

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HardSarcomaDSRCTSCE Medical Oncology

A 50-year-old man with an intra-abdominal desmoplastic small round cell tumour (DSRCT) confirmed by EWSR1-WT1 fusion is discussed at the sarcoma MDT. What is the standard treatment approach?

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Correct answer: A — Multimodal treatment: alkylating agent-based chemotherapy (P6 protocol: cyclophosphamide, doxorubicin, vincristine alternating with ifosfamide, etoposide) + cytoreductive surgery ± HIPEC ± radiotherapy

DSRCT is a rare aggressive sarcoma with the pathognomonic EWSR1-WT1 fusion. It predominantly affects young males and presents with peritoneal dissemination. The P6 protocol (intensive alkylating agent chemotherapy) followed by cytoreductive surgery and HIPEC achieves the best outcomes (median survival 2-5 years). Whole abdominopelvic radiation may be added. Despite multimodal treatment, prognosis remains poor. Emerging approaches include anti-VEGF therapy and immunotherapy combinations.

Reference: ESMO 2024 Soft Tissue Sarcoma Guidelines; Lal et al JCO 2005