Sethi Harsheet, Madanur Mansoor, Srinivasan Parthi, Portmann Bernard, Heaton Nigel, Rela Mohamed
Department of Hepatobiliary and Transplant Surgery, Kings College Hospital, Denmark Hill, London SE5 9RS, UK.
Hepatobiliary Pancreat Dis Int. 2007 Oct;6(5):549-52.
Neuroendocrine tumors (NETs) arising in the biliary tree are extremely rare, and 37 cases were identified in the English literature.
A well-differentiated NET was found arising from the junction of the cystic and common hepatic ducts, in a 51-year-old male presenting with pedal edema and weight loss with abnormal liver enzymes and a normal serum bilirubin level. No mass was seen on radiological imaging and biopsy of the liver was suggestive of an early cholangiopathy. A bile leak complicating the liver biopsy led to an ERCP that demonstrated a filling defect suggestive of a mass in the common bile duct (CBD).
He underwent a successful excision of the tumor with a Roux-en-Y hepaticojejunostomy. The diagnosis of NET was made on histological and immunohistochemical analysis of the resected specimen. He remains well and disease free 22 months after surgery.
Recognition of biliary NET continues to be a challenge and an increased awareness of these tumors in rare sites will result in optimal management of these tumors.
起源于胆管树的神经内分泌肿瘤（NETs）极为罕见，英文文献中仅报道过37例。
在一名51岁男性患者中发现了一个高分化的NET，该患者表现为足部水肿、体重减轻、肝功能异常且血清胆红素水平正常。影像学检查未发现肿块，肝脏活检提示早期胆管病。肝脏活检引发的胆漏导致进行了内镜逆行胰胆管造影（ERCP），结果显示胆总管（CBD）内有一个提示肿块的充盈缺损。
他成功接受了肿瘤切除及Roux-en-Y肝空肠吻合术。通过对切除标本进行组织学和免疫组化分析确诊为NET。术后22个月，他情况良好，无疾病复发。
胆管NET的诊断仍然是一项挑战，提高对这些罕见部位肿瘤的认识将有助于对这些肿瘤进行最佳管理。
