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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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Authors

SASeyed Amir AledavoodESEhsan SoltaniMOMahsa Akbari Oryani

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Overview

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

Key Points

  • 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.

Cite This Study

Aledavood et al. (2026) studied this question.

synapsesocial.com/papers/6a03cbfc1c527af8f1ecfdbbhttps://doi.org/10.1002/ccr3.72240
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