摘要
目的提高对胆管神经内分泌肿瘤的认识.方法报道国内首例肝总管神经内分泌癌合并肝脏转移,结合国内已经报道的8例未发生转移病例,总结该病的诊断和治疗.结果男性3例,女性6例,8例以梗阻性黄疸起病,2例合并类癌综合征,梗阻部位胆总管下段2例,肝总管6例,胆总管中段1例,手术前均未能确诊;采取探查性手术,5例行根治性切除胆管空肠吻合术,2例行Whipple手术,1例行姑息切除+肝动脉插管化疗,1例外引流,手术后病理确诊.结论胆管神经内分泌癌是一种少见的胆道恶性肿瘤,梗阻性黄疸是常见的症状,NSE、Cg-A、SYP等免疫组化染色是诊断的要点;手术切除率高,根治性切除是治疗的首选,如果肿瘤转移,姑息性切除+肝动脉化疗是比较好的选择。
Objective To improve the cognition of duct neuroendocrine carcinoma. Method To summarize this disease＇ s diagnosis and treatment by analyzing a local first patient who was reported to suffer from the bile duct neuroendocrine carcinoma with liver transfer and 8 reported domestic cases of the bile duct neuroendocrine earcinoma without metastasis. Result Male 3, Female 6,Among them 8 cases＇ onsets with obstructive jaundice and 2 cases with carcinoid syndrome. Obstructive parts,2 cases in lower common bile duct,6 cases in common hepatic duct, 1 case in middle common hile duct. None of these cases can be surely diagnosed befor operation. Exploratomy laparotomy,haveto be done. 5 patients with curative cholangiojejunostomy,2 with whipple operation, i with palliative excision ＋ hepatic artery chemotherapy and 1 with external drain. Pathologic confirmed diagnosis after operation. ConclusionThc bile duct neuroendcocrine carcinoma is an infrequent bile duet malignant tumor, obstrunction jaundcocrine is a familiar syptom,the cctimmunohist chemical methods shuch as NSA, Cg- A, SYP are key pointe in diagnosis. Radical operation is the preferred therape. If the tumor have transfer, pallialive excision ＋ hepatic artery catheterization therapy is a better choice.
关键词
胆管
神经内分泌癌
诊断
治疗
the bile duct
neurendocrine carcinoma
diagnosis
treatment
