Pancreatic angiosarcoma with synchronous pancreatic ductal adenocarcinoma leading to hemosuccus pancreaticus: A surgical case report and review of literature.

Introduction: Angiosarcoma of pancreas is an extremely rare disease with a poor prognosis. The clinical signs and symptoms of pancreatic angiosarcoma are nonspecific, and it is occasionally diagnosed at an advanced stage. Pancreatic angiosarcoma and Pancreatic ductal adenocarcinoma in one patient was never ever known in English literature.

Case Presentation: A 56-year-old female was admitted with clinical and laboratory signs of gastrointestinal (GI) bleeding. Upper gastrointestinal endoscopy revealed bleeding from the ampulla of Vater. Besides, abdominal computed tomographic (CT) revealed a solid mass in the region of the pancreatic tail, which was considered the origin of bleeding. Distal pancreatectomy and splenectomy were performed because of persistent GI bleeding, and the final histological diagnosis of tumor in pancreatic tail was pancreatic ductal adenocarcinoma. After 30 days, she developed recurrent bleeding in ampulla and the abdominal CT-scan revealed a huge hematoma in omentum harem. We conducted transcatheter arterial embolization, but anemia continued to worsen. Therefore, pancreaticoduodenectomy was recommended to remove this mass, and based on postoperative histological findings, pancreatic angiosarcoma was diagnosed. After few days, laparotomy was indicated again because of persistent intra-abdominal bleeding. Despite all critical care and surgical therapeutic attempts, the patient died within two weeks after operation.

Discussion: A pancreatic angiosarcomas primary origin is especially rare, with the present case being the tenth accounted in the English literature. Angiosarcomas is creating a disorganized mass of cells with extravasated blood that led to characteristics, extensive amounts of hemorrhage. The clinical manifestations of pancreatic angiosarcoma are variable, and immunohistochemistry staining is mandatory, with positive staining for vascular markers, which include CD31, CD34, von Willebrand factor (vWF), factor-VIII, Ulex europaeus agglutinin 1 (UEA-1), Friend leukemia integration 1 (Fli-1) and vascular endothelial growth factor receptor (VEGFER).

Conclusion: We here present a report of an extremely rare case with had pancreatic angiosarcoma and synchronous pancreatic ductal adenocarcinoma with clinical picture of GI bleeding secondary to hemosuccus pancreaticus (HP).