Pancreas angiosarcoma—Case report of a rare cause of abdominal pain

Abstract

Highlights Pancreatic angiosarcoma is a rare entity, with 7 cases reported in literature so far. Diagnosis is often difficult, since histology and immunohistochemistry is mandatory. Surgery is the only curative option, though metastatic disease is frequent. Prognosis is very poor for angiosarcomas overall, with a 5-year survival of only 35%.

A Resected Primary Angiosarcoma of the Pancreas Presenting Aggressive Metastatic Liver Recurrence with Uncontrollable Intra-abdominal Bleeding: a Case Report

“…Angiosarcoma can arise in any part of the body, but is found most commonly in cutaneous lesions and rarely arises from visceral organs [2]. Speci cally, a primary angiosarcoma of the pancreas is extremely rare, having so far only six cases reported (Table1) [4][5][6][7][8][9]. Five out of seven cases (including ours) expired within two months after the initial diagnosis or surgery.…”

Comments and illustrations of the European Federation of Societies for Ultrasound in Medicine guidelines: Rare pancreatic tumors, ultrasound and contrast-enhanced ultrasound features—Malignant mesenchymal tumors

“…One case with survival within 1 year without evidence of recurrence is described [ 158 ] and 1 patient with a follow-up of 4 months without tumor recurrence after surgical resection and therapy with paclitaxel-based chemotherapy. [ 68 ]…”