Epithelioid Angiosarcoma Arising in the Vicinity of an Upper Extremity PTFE Vascular Graft: A Case Report and Brief Literature Review

Abstract

Angiosarcomas are rare malignant tumors of vascular origin that occur commonly in the skin followed by soft tissues and viscera. These tumors have rarely been described to arise at the site of synthetic vascular grafts even after a long period of latency. The exact etiology of such foreign body-associated sarcomas is not known although their occurrence has been described under both experimental (in rats) and clinical conditions (in humans). Of four such angiosarcoma cases described in clinical literature originating at the site of a vascular graft, three of them had an epithelioid morphology. Of these three cases, one originated from the abdominal aorta and the other two from the iliac artery and the femoral artery respectively. We herein describe the fourth case of epithelioid angiosarcoma developing from the site of the left brachial artery 16 years after the placement of a polytetrafluoroethylene (PTFE) vascular graft. The patient, a 47-year-old man on chronic hemodialysis presented in our hospital with a 3-week history of pain and nodularity at the site of the prosthesis which was initially thought to be an aneurysmal degeneration of the graft. Excision and subsequent histopathologic examination of the graft and surrounding tissue however revealed a locally invasive 1.4 cm epithelioid angiosarcoma consisting of packed highly pleomorphic tumor cells with an epithelioid morphology, clear nuclei, and prominent nucleoli. On immunohistochemistry, the tumor was positive for CD31 and pancytokeratin and negative for CD68, SMA, melan A, and CD34. A wide local excision of the graft area was subsequently done and the patient was treated with adjuvant radiotherapy. Although rare, this aggressive variant of angiosarcoma should be a concern among surgeons inserting artificial vascular grafts in patients with advanced vascular disease and end-stage renal disease and also among pathologists handling such explanted graft specimens.