原发性胆管囊肿癌变的诊断与治疗

The diagnosis and treatment of malignant change in primary choledochal cysts.

目的 总结原发性胆管囊肿癌变的诊断及治疗经验。方法 对 1980～ 2 0 0 0年间收治的 7例原发性胆管囊肿癌变病人的临床资料进行回顾性分析。结果 7例原发性胆管囊肿癌变病人临床表现为非特异性 ,仅为胆管炎表现。癌变 6例位于囊壁 ,1例侵及胰腺。手术方式包括囊肿切除、肝管空肠Roux -en -Y吻合术 3例 ,囊肿部分切除、肝左外叶切除、肝管空肠Roux -en -Y吻合术 1例 ,囊肿切除加胰十二指肠切除术 1例 ,T管引流加活检 2例 ,术后病理结果大部分为腺癌。结论 原发性胆管囊肿癌变临床表现不典型 ,术前诊断困难 ,预后极差 ,目前强调预防为主 ,对先天性胆管囊肿病人推荐行新的预防癌变的手术方式 ,术中冰冻切片检查有助于确诊 。

Objective To summarize the experience of diagnosis and treatment of malignant change in primary choledochal cysts in the past 20 years(1980～2000).Methods The clinical data of 7 patients admitted from 1980 to 2000 were analyzed retrospectively.Results Of all the 108 cases,7 patients had primary carcinoma lesions arising in the biliary duct cyst.The clinical presentation was non-specific,in which 6 patients had biliary tract infection.The sites of carcinoma were cyst wall in 6 cases,pancreas in 1 patient.Operative procedures for bile duct malignancy included cysts excision with Roux-en-Y hepaticojejunostomy in 3 patients;partial cyst excision with left lobectomy and Roux-en-Y hepaticojejunostomy in 1 patient;cyst excision and pancreatoduodenectomy in 1 case;choledochotomy with T-tube drainage and metastatic lymph node biopsy in 2 patients.The pathologic feature of this disease was malignancy,most of them was adenocarcinoma.Conclusion The clinical symptoms of primary choledochal cysts are non-specific.The preoperative diagnosis is difficult and the prognosis is poor.At present,prevention should be emphasized.A new procedure which can prevent malignant change of choledochal cysts is recommended.Intraoperative frozen section biopsy is helpful to comfirm diagnosis.Cyst excision with pancreatoduodenectomy is the treatment of choice for the disease invading pancreatic head.