# Primary epithelioid angiosarcoma of the jejunum presenting with severe anemia and distant metastasis: a case report and literature review

## TL;DR

A 72-year-old man was diagnosed with a rare angiosarcoma in his jejunum, which caused severe anemia and spread to his lungs and bones.

## Contribution

This case report adds to the limited literature on primary jejunal angiosarcoma and its clinical presentation and diagnosis.

## Key findings

- Jejunal angiosarcoma can present with melena and severe anemia.
- Initial CT scans may miss the primary lesion, requiring enteroscopy for diagnosis.
- Immunohistochemistry confirmed the angiosarcoma with CD31, CD34, and ERG positivity.

## Abstract

Angiosarcoma (AS), constituting approximately 2% of all soft tissue sarcomas, is an aggressive endothelial malignancy. We report on the case of a 72-year-old man admitted with persistent melena and severe anemia, ultimately diagnosed with jejunal angiosarcoma (JAS) and associated lung and bone metastases. Temporary stabilization of his hemoglobin level was achieved via fasting, hemostasis, and blood transfusion. The initial plain CT identified multifocal solid lung nodules, but failed to reveal a primary digestive system lesion. Subsequent small enteroscopy localized a submucosal bulge in the jejunum, 70 cm from the pylorus. Following partial small bowel resection, the pathological examination confirmed a 0.9-cm × 0.8-cm × 0.7-cm angiosarcoma invading the intestinal wall and lamina propria. Immunohistochemical (IHC) staining showed strong positivity for CD31(++), alongside CD34(vascular+) and ERG(+). This report reviews the recent literature on JAS, summarizing the diagnostic and therapeutic strategies.