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This is a Case Report study (also classified as: Literature Review).

May 13, 2026Clinical Case ReportsOpen Access

Aggressive Desmoplastic Small Round Cell Tumor in an Adult: A Case Report and Literature Review

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Case report reveals recurrence and distant metastasis in desmoplastic small round cell tumor, highlighting the need for targeted therapies.

To report a rare case of aggressive desmoplastic small round cell tumor in an adult and discuss treatment challenges.
Case report of a 35-year-old male with DSRCT presenting with abdominal symptoms.
Molecular confirmation of the EWS–WT1 fusion was not performed.
The patient received cytoreductive surgery, EPIC, and systemic chemotherapy.
Initial disease control was achieved; however, recurrence and liver metastasis occurred later.
Multiple lines of salvage chemotherapy and liver metastasectomy were required due to chemoresistance.
The case demonstrates a prolonged survival compared to historical series despite aggressive treatment.

Aledavood et al. (2026) studied this question.

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Case report reveals recurrence and distant metastasis in desmoplastic small round cell tumor, highlighting the need for targeted therapies.