Primary Jejunal Angiosarcoma

Abstract

Primary jejunal angiosarcoma is an aggressive and rare soft-tissue neoplasm with a poor prognosis. Multiple modalities such as endoscopic intervention, radiographic imaging, and immunohistochemistry are often used to confirm the diagnosis. Only 62 cases have been reported in the literature of primary jejunal angiosarcoma, and the majority of these patients had distant metastases at initial staging. This is a case of a 51-year-old man who presented with melena found to have a jejunal mass and diagnosed with angiosarcoma with no metastases detected on initial staging. The diagnosis of angiosarcoma was confirmed with histomorphology and immunohistochemistry. Despite early tumor staging and successful small bowel resection, 6 months postsurgical resection patient later presented with metastatic. This case report highlights the importance of a comprehensive diagnostic strategy and raises consideration for earlier systemic therapy in localized neoadjuvant therapy in early-stage small bowel angiosarcoma.

Keywords: jejunal angiosarcoma; small bowel angiosarcoma.

Figure 1.

Spiral enteroscopy reveals ulcerated, bleeding jejunal mass of the angiosarcoma.

Figure 2.

Capsule endoscopy localizes bleeding to small bowel that was presumed to be the jejunum.

Figure 3.

(A) The tumor was mainly located in the lamina propria extending into the muscularis propria (20×). (B) High-power view revealed a vascular tumor with plump, polygonal tumor cells, and pleomorphic nuclei (200×). (C) Endothelial marker, ERG, was diffusely positive (200×). (D) Ki-67 proliferative index was high (∼40%) (200×).

Figure 4.

(A and B) Positron electron tomography imaging showing uptake in the vertebrae and small bowel, indicating metastasis post-small bowel resection.