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Metastatic presentation of dermatofibrosarcoma protuberans as massive lower gastrointestinal bleed

  • 0Department of General Surgery, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, Chandigarh, India drbasilms@gmail.com.
Bmj Case Reports +

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July 30, 2024

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July 30, 2024

View abstract on PubMed

Summary

This summary is machine-generated.

Dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous change is aggressive. This case highlights a rare instance of jejunal fibrosarcoma presenting as gastrointestinal bleeding, emphasizing the need for close patient monitoring.

Area Of Science

  • Oncology
  • Surgical Pathology

Background

  • Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive skin tumor.
  • Fibrosarcomatous transformation in DFSP increases recurrence and metastatic potential.
  • Gastrointestinal metastasis from DFSP is exceptionally rare.

Observation

  • A woman in her late 30s with a history of DFSP presented with massive lower gastrointestinal bleeding.
  • A jejunal mass was identified as the bleeding source and surgically excised.
  • Histopathology confirmed the jejunal mass as fibrosarcoma.

Findings

  • The patient developed progressive disease with lung nodules, ascites, and abdominal lymphadenopathy post-surgery.
  • The fibrosarcoma likely represented a metastatic or transformed DFSP.
  • Chemotherapy was initiated, with the patient showing good tolerance 3 months postoperatively.

Implications

  • This case underscores the aggressive nature of fibrosarcomatous DFSP.
  • Highlights the rare but severe presentation of gastrointestinal bleeding due to DFSP metastasis.
  • Emphasizes the critical need for vigilant long-term surveillance in DFSP patients with fibrosarcomatous changes due to heightened metastatic risk.

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