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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
Summary
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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