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Desmoplastic small round cell tumors in a young man

Genryu Hirano1, Makoto Irie, Yuta Nakashima

  • 1Department of Gastroenterology and Medicine, Fukuoka University Faculty of Medicine, Japan. g.hirano@minf.med.fukuoka-u.ac.jp

Internal Medicine (Tokyo, Japan)
|September 3, 2013
PubMed

Insights

A rare desmoplastic small round cell tumor (DSRCT) diagnosis in an 18-year-old male liver patient was confirmed via biopsy and genetic testing. Despite palliative chemotherapy, the aggressive cancer led to partial remission and death within 20 months.

Area of Science:

  • Oncology
  • Genetics

Background:

  • Desmoplastic small round cell tumors (DSRCT) are rare, aggressive neoplasms typically affecting young males.
  • Intrahepatic manifestation of DSRCT is exceptionally uncommon, presenting diagnostic and therapeutic challenges.

Observation:

  • An 18-year-old male presented with abdominal pain and was diagnosed with intrahepatic DSRCT.
  • Percutaneous biopsy revealed a polyphenotypic immunoprofile and the characteristic EWS-WT1 gene fusion.

Findings:

  • The DSRCT had invaded the mesentery and disseminated to the liver.
  • Palliative chemotherapy including carboplatin, paclitaxel, vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide, and irinotecan resulted in partial remission.

Implications:

  • This case highlights the aggressive nature and poor prognosis of intrahepatic DSRCT, even with multi-agent chemotherapy.
  • Further research into novel therapeutic strategies for advanced DSRCT is warranted.

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