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

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.