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Intra-abdominal desmoplastic small round cell tumor (IDSRT).
1Department of Gastroenterology, Yaizu Municipal General Hospital, Japan.
Journal of Gastroenterology
|October 7, 2000
Summary
Intra-abdominal desmoplastic small round cell tumor (IDSRT) is a rare cancer in young adults. This case highlights the aggressive nature of IDSRT and challenges in its treatment, despite initial chemotherapy response.
Area of Science:
- Oncology
- Pathology
Background:
- Intra-abdominal desmoplastic small round cell tumor (IDSRT) is a rare, aggressive sarcoma primarily affecting young males.
- This neoplasm characteristically presents with widespread peritoneal dissemination.
Observation:
- A 27-year-old male presented with unexplained ascites and colonic compression.
- Colonoscopy revealed diffuse, aphthoid-like lesions throughout the colon.
- Biopsy confirmed the diagnosis of IDSRT.
Findings:
- The patient received standard chemotherapy (cyclophosphamide, etoposide, doxorubicin, cisplatin) resulting in a partial response.
- Tumor recurrence and progression were observed shortly after initial treatment.
- The patient succumbed to the disease during the second chemotherapy course.
Implications:
- This case underscores the aggressive behavior and poor prognosis of IDSRT, even with standard chemotherapy.
- The observed colonic lesions represent a potential diagnostic clue for IDSRT.
- Further research into novel therapeutic strategies for IDSRT is warranted to improve patient outcomes.