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目的探讨先天性胆管囊肿癌变的诊断与治疗方法。方法回顾性分析1993年—2004年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。结果185例中合并癌变27例,癌变率为14.6%。先天性胆管囊肿癌变与年龄密切相关(P<0.001),各年龄段癌变率分别为:0～9岁为0%,10～19岁5.1%,20～29岁9.1%,30～39岁16.2%,40～49岁26.7%,50～59岁33.3%,≥60岁50%。6例有胆肠内引流手术史。临床主要表现为上腹疼痛、发热、黄疸和体重下降。诊断方法包括腹部超声、CT、MRI和经内镜逆行胰胆管造影,术前确诊20例,但早期诊断困难。获得根治性切除9例(33.3%)。结论先天性胆管囊肿是一种癌前病变,随年龄增长癌变率急剧增加。儿童期实施完全性囊肿切除是预防胆管囊肿癌变的最有效方法。胆管囊肿癌变病例预后不佳。
Objective To investigate the diagnosis and treatment of congenital cholangiocarcinoma. Methods The clinical and pathological data of 185 patients with congenital cholangiocarcinoma admitted to PLA General Hospital from 1993 to 2004 were retrospectively analyzed. Results Of the 185 patients with malignant transformation in 27 cases, the rate of canceration was 14.6%. The incidence of congenital cholangiocarcinoma was closely related to age (P <0.001). The rates of carcinogenesis in all age groups were 0% in 0-9 years, 5.1% in 10-19 years, 9.1% in 20-29 years, 16.2% in 30-39 years %, 40-49 years old 26.7%, 50-59 years 33.3%, 60 years old 50%. 6 cases had a history of choledocho-intestinal drainage. The main clinical manifestations of abdominal pain, fever, jaundice and weight loss. Diagnostic methods include abdominal ultrasound, CT, MRI and endoscopic retrograde cholangiopancreatography, diagnosed 20 cases before surgery, but the early diagnosis is difficult. Radical resection in 9 cases (33.3%). Conclusions Congenital cholangiocarcinoma is a kind of precancerous lesion. The rate of canceration increases sharply with age. Complete cyst excision in childhood is the most effective way to prevent cancerous biliary cysts. Bile duct cyst cancers have a poor prognosis.