Ito Hiroharu, Wada Yoshiyuki, Takami Yuko, Ryu Tomoki, Ureshino Hiroki, Imamura Hajime, Sasaki Shin, Ohno Akihisa, Hijioka Masayuki, Kaku Toyoma, Kawabe Ken, Kawauchi Shigeto, Saitsu Hideki
Department of Hepato-Biliary-Pancreatic Surgery, Clinical Research Institute, National Hospital Organization Kyushu Medical Center, 1-8-1 Jigyohama, Chuo-ku, Fukuoka, 810-8563, Japan.
Department of Gastroenterology, National Hospital Organization Kyushu Medical Center, Fukuoka, Japan.
Surg Case Rep. 2020 Jun 26;6(1):150. doi: 10.1186/s40792-020-00915-9.
Gastroenteric neuroendocrine carcinomas (NECs) account for 6.2% of gastroenteric neuroendocrine tumors (NETs), and only 1% or less of gastroenteric NETs occur in the ampulla of Vater (AoV). Clinical features of NEC of the AoV remain obscure.
A 65-year-old man visited a general practitioner because of jaundice, and an abdominal contrast-enhanced computed tomography scan revealed a tumor of 11 mm in diameter, which was enhanced in the arterial phase at the duodenal papilla, with dilation of the upstream bile duct. Gastrointestinal scope revealed an unexposed tumor of the AoV. Based on a biopsy of the site, a moderately differentiated tubular adenocarcinoma was suspected, and pancreatoduodenectomy was performed. Histopathological examination revealed dysplasia and highly proliferative small tumor cells, with solid and nodular formation at the AoV. Histological analysis showed a high mitotic count, and immunohistochemical staining revealed a Ki-67 index of 40-50% and cells positive for synaptophysin, chromogranin A, and p53. Small cell-type NEC was finally diagnosed. Four months post pancreatoduodenectomy, multiple liver metastases developed, and systemic chemotherapy was administered. Salvage liver resection for liver metastases was performed 14 months after the pancreatoduodenectomy. Unfortunately, multiple liver metastases developed 2 months after liver resection, and the patient died 18 months after the pancreatoduodenectomy.
Neuroendocrine carcinoma originating from the bile duct is very rare; therefore, in this article, we provide a review of the literature and a case report.
胃肠神经内分泌癌（NECs）占胃肠神经内分泌肿瘤（NETs）的6.2%，而仅1%或更少的胃肠NETs发生于 Vater壶腹（AoV）。AoV的NEC的临床特征仍不明确。
一名65岁男性因黄疸就诊于全科医生，腹部增强计算机断层扫描显示直径11毫米的肿瘤，十二指肠乳头在动脉期强化，上游胆管扩张。胃肠镜检查发现AoV有一个未暴露的肿瘤。基于该部位的活检，怀疑为中度分化管状腺癌，遂行胰十二指肠切除术。组织病理学检查显示发育异常和高增殖性小肿瘤细胞，AoV处有实性和结节状形成。组织学分析显示有高有丝分裂计数，免疫组化染色显示Ki-67指数为40%-50%，突触素、嗜铬粒蛋白A和p53细胞呈阳性。最终诊断为小细胞型NEC。胰十二指肠切除术后4个月，出现多发肝转移，遂给予全身化疗。胰十二指肠切除术后14个月对肝转移灶进行挽救性肝切除。不幸的是，肝切除术后2个月出现多发肝转移，患者在胰十二指肠切除术后18个月死亡。
起源于胆管的神经内分泌癌非常罕见；因此，在本文中，我们提供了文献综述和病例报告。
