Liu Quan-da, Zhou Ning-xin, Huang Zhi-qiang, Zhang Wen-zhi, Liu Gui-lin, Wang Yue-hua
Institute of Hepatobiliary Surgery, General Hospital of People's Libration Army, Beijing 100853, China.
Zhonghua Wai Ke Za Zhi. 2005 Jul 1;43(13):839-41.
To summarize the experience in diagnosis, prevention and treatment of carcinoma arising from congenital biliary duct cyst.
The clinical and pathological data of 185 patients with congenital biliary duct cyst admitted to Chinese PLA General Hospital were analyzed retrospectively.
Among 185 patients, twenty-seven cases had carcinomas arising from congenital biliary duct cyst, and the frequency of malignant transformation was 14.6%, which closely related to the age (P < 0.001). The incidences of malignancy for different age groups were: 0 for 0-9 age group, 5.1% for 0-19, 9.1% for 20-29, 16.2% for 30-39, 26.7% for 40-49, 33.3% for 50-59, and 50% for over 60, respectively. Six patients had the history of cyst-enterostomy. Abdominal pain, fever, jaundice and weight loss were the main clinical manifestations. Abdominal ultrasonography, CT, MRI or magnetic resonance cholangiopancreatography, MRCP and endoscopic retrograde choledochopancreatography (ERCP) were the main diagnostic methods. For twenty patients (74.1%), a definite diagnosis was made preoperatively, but it's hard to make an early diagnosis. Nine patients (33.3%) underwent curative resection.
Congenital biliary duct cyst is a premalignant lesion, and the incidence of carcinogenesis increases remarkably with age. The most effective method for prevention of carcinogenesis in choledochal cyst is complete excision of choledochal cyst during childhood, and the prognosis is poor for patients with biliary malignancy.
总结先天性胆管囊肿癌变的诊断、预防及治疗经验。
回顾性分析解放军总医院收治的185例先天性胆管囊肿患者的临床及病理资料。
185例患者中，27例发生先天性胆管囊肿癌变，恶变率为14.6%，与年龄密切相关（P<0.001）。不同年龄组的恶变发生率分别为：0～9岁组为0，0～19岁组为5.1%，20～29岁组为9.1%，30～39岁组为16.2%，40～49岁组为26.7%，50～59岁组为33.3%，60岁以上组为50%。6例有囊肿肠吻合术史。腹痛、发热、黄疸及体重减轻为主要临床表现。腹部超声、CT、MRI或磁共振胰胆管造影（MRCP）及内镜逆行胰胆管造影（ERCP）为主要诊断方法。20例（74.1%）术前获得明确诊断，但早期诊断困难。9例（33.3%）行根治性切除。
先天性胆管囊肿是一种癌前病变，癌变发生率随年龄显著增加。预防胆管囊肿癌变的最有效方法是在儿童期完整切除胆管囊肿，胆管恶性肿瘤患者预后较差。
