Nesi Gabriella, Lombardi Antonella, Batignani Giacomo, Ficari Ferdinando, Rubio Carlos A, Tonelli Francesco
Department of Human Pathology and Oncology, University of Florence, Viale GB Morgagni 85, Florence 50134, Italy.
Virchows Arch. 2006 Jul;449(1):104-11. doi: 10.1007/s00428-006-0207-4. Epub 2006 May 3.
Primary carcinoid tumors of the extrahepatic biliary tree are exceedingly rare, accounting for 0.2-2% of all digestive carcinoids. The authors in this study describe a case of biliary duct primary well-differentiated endocrine tumor in a 30-year-old man with symptoms of biliary obstruction and watery diarrhoea. Abdominal ultrasound showed a 2-cm solid lesion in the head of the pancreas, compressing the distal common bile duct. A computed tomography scan confirmed these findings, revealing the hypervascular pattern of the tumor. Gastrointestinal hormonal screening demonstrated an increase in plasma serotonin. The patient underwent standard pylorus-preserving pancreatoduodenectomy. Pathological examination showed a neuroendocrine tumor of the distal common bile duct measuring 1.8 cm in greatest dimension. The tumor cells were immunopositive for neuron-specific enolase (NSE), chromogranin A, synaptophysin, serotonin, and cytokeratin. Stains for gastrin and somatostatin were negative. Seven years later, the patient is well, with no evidence of disease. Given the site of these tumors and the difficulty in differentiating them from periampullary lesions, decisions as to the appropriate surgical approach may be problematic. After an exhaustive review of the literature, the authors conclude that pancreatoduodenectomy is the treatment of choice.
肝外胆管原发性类癌极为罕见，占所有消化系统类癌的0.2 - 2%。本研究的作者描述了一例30岁男性胆管原发性高分化内分泌肿瘤，该患者有胆管梗阻和水样腹泻症状。腹部超声显示胰腺头部有一个2厘米的实性病变，压迫远端胆总管。计算机断层扫描证实了这些发现，显示肿瘤的高血运模式。胃肠激素筛查显示血浆5-羟色胺升高。患者接受了标准的保留幽门胰十二指肠切除术。病理检查显示远端胆总管神经内分泌肿瘤，最大径为1.8厘米。肿瘤细胞对神经元特异性烯醇化酶（NSE）、嗜铬粒蛋白A、突触素、5-羟色胺和细胞角蛋白免疫阳性。胃泌素和生长抑素染色阴性。7年后，患者情况良好，无疾病迹象。鉴于这些肿瘤的部位以及将它们与壶腹周围病变区分开来的困难，关于合适手术方法的决策可能存在问题。在对文献进行详尽回顾后，作者得出结论，胰十二指肠切除术是首选治疗方法。
