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Dermatofibrosarcoma protuberans of the jejunum
A K Khanna1, L Chaudhury, S Khanna
1Department of General Surgery, K E M Hospital, Mumbai. akk_dr@satyam.net.in
Summary
A rare case of dermatofibrosarcoma protuberans, a skin tumor, was found in the small intestine of a 55-year-old man. This unusual presentation caused recurrent gastrointestinal bleeding, highlighting the need for thorough investigation of small bowel tumors.
Area of Science:
- Gastroenterology
- Surgical Oncology
- Dermatopathology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive skin tumor.
- DFSP typically occurs on the trunk and extremities, rarely involving internal organs.
- Small intestine tumors can present with occult or overt gastrointestinal bleeding.
Observation:
- A 55-year-old male presented with recurrent episodes of small intestine bleeding.
- Preoperative imaging studies were indicative of a neoplastic lesion within the small bowel.
- Surgical resection of the affected small bowel segment was performed.
Findings:
- Histopathological examination of the resected specimen confirmed the diagnosis of dermatofibrosarcoma protuberans.
- The tumor was located in the small bowel, an extremely rare site for DFSP.
- The histological features were consistent with a primary small bowel DFSP.
Implications:
- This case expands the known anatomical distribution of dermatofibrosarcoma protuberans.
- It underscores the importance of considering rare diagnoses in cases of unexplained gastrointestinal bleeding.
- Highlights the diagnostic challenges and management of intra-abdominal DFSP.