Epithelioid Angiosarcoma: A Rare Culprit of Small Bowel Overt Bleeding

Abstract

Introduction: Intestinal epithelioid angiosarcomas are extremely rare and aggressive vascular tumors, with a very limited number of cases reported in the literature. We report a case of this malignancy presenting with small bowel overt bleeding. Case Presentation: A 77-year-old male with recurrent maroon-colored stools and anemia was transferred to our hospital for tertiary care. Past medical history was significant for coronary artery disease status post bypass x 6. He was on daily aspirin 81 mg and denied NSAID use. He initially presented with melena and an EGD discovered a non-bleeding duodenum ulcer. Two weeks later, he was evaluated again for hematochezia and persistent anemia. An EGD was repeated with same findings. A colonoscopy showed a 1cm polyp with an arteriovenous malformation (AVM) in the rectum. This was treated with hot snare polypectomy. On video capsule endoscopy (VCE), there were multiple bleeding ulcers throughout the visualized small bowel. At our hospital, an upper double-balloon enteroscopy (DBE) revealed bleeding erosion on a polyp in distal duodenum which was treated with argon plasma coagulation. There were several blue-purple polypoid lesions on top of focal ulcers on jejunal mucosa (Image1). Biopsies of these polypoid lesions in both distal duodenum and jejunum had histological features consistent with epithelioid angiosarcoma. Immunostains were positive for CD31 and keratin (Image2-3), but negative for S-100 and melan A. Further review of pathology from the previous polypectomy of the rectum also confirmed the diagnosis. Staging imaging study did not show evidence of extraintestinal disease. Chemotherapy with paclitaxel 135 mg/m2 every 3 weeks were recommended. Unfortunately, the patient died 6 weeks later due to complications of infection and respiratory failure. Discussion: Suspected small intestinal bleeding should be investigated promptly with VCE to look for rarer causes. This may be followed by DBE for a tissue biopsy and intervention. Epithelioid angiosarcoma is a highly aggressive endothelial cell malignancy, most commonly arising in the deep soft tissues but rare in the small intestine. The prognosis is very poor and most patients die within 1 year of diagnosis, therefore, it is important for endoscopists to be aware of this disorder to obtain an early diagnosis. Treatment usually involves surgical resection of the bleeding lesions, chemotherapy, radiotherapy, and transfusions for symptom alleviation.Figure 1