Nguyen Cong Long, Vu Truong Khanh, Nguyen Ham Hoi, Nguyen Thanh Khiem, Luong Tuan Hiep, Tran Thi Tan, Le Van Khang, Nguyen Canh Hiep
Department of Gastroenterology & Hepatology, Bach Mai Hospital, Ha Noi, Viet Nam.
School of Medicine and Pharmacy, Vietnam National University Hanoi, Hanoi, Viet Nam.
Ann Med Surg (Lond). 2022 Mar 29;76:103547. doi: 10.1016/j.amsu.2022.103547. eCollection 2022 Apr.
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.
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.
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).
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).
胰腺血管肉瘤是一种极为罕见的疾病，预后较差。胰腺血管肉瘤的临床症状和体征不具有特异性，偶尔在疾病晚期才被诊断出来。在英文文献中，从未有过一名患者同时患有胰腺血管肉瘤和胰腺导管腺癌的报道。
一名56岁女性因出现胃肠道（GI）出血的临床和实验室症状入院。上消化道内镜检查显示十二指肠乳头出血。此外，腹部计算机断层扫描（CT）显示胰尾区域有一个实性肿块，被认为是出血的源头。由于持续性胃肠道出血，患者接受了远端胰腺切除术和脾切除术，胰尾肿瘤的最终组织学诊断为胰腺导管腺癌。30天后，她的十二指肠乳头再次出现出血，腹部CT扫描显示大网膜有巨大血肿。我们进行了经导管动脉栓塞术，但贫血仍持续恶化。因此，建议进行胰十二指肠切除术以切除该肿块，根据术后组织学检查结果，诊断为胰腺血管肉瘤。几天后，由于持续性腹腔内出血，再次进行了剖腹手术。尽管采取了所有重症监护和手术治疗措施，患者在术后两周内死亡。
胰腺血管肉瘤的原发起源极为罕见，本病例是英文文献中报道的第十例。血管肉瘤会形成一团杂乱无章的细胞，并伴有血液外渗，导致大量出血的特征。胰腺血管肉瘤的临床表现各不相同，免疫组织化学染色是必要的，血管标记物染色呈阳性，这些标记物包括CD31、CD34、血管性血友病因子（vWF）、凝血因子VIII、欧洲荆豆凝集素1（UEA-1）、Friend白血病整合1（Fli-1）和血管内皮生长因子受体（VEGFER）。
我们在此报告了一例极为罕见的病例，该患者同时患有胰腺血管肉瘤和同步性胰腺导管腺癌，临床表现为因胰源性出血（HP）继发的胃肠道出血。
