Balsells J, Margarit C, Murio E, Lazaro J L, Charco R, Vidal M T, Bonnin J
Department of Surgery, Hospital General Vall d'Hebrón, Universidad Autónoma, Barcelona, Spain.
HPB Surg. 1993 Aug;7(1):81-6; discussion 86-7. doi: 10.1155/1993/61048.
Caroli's disease is characterized by congenital cystic dilatation of the intrahepatic bile ducts. In 7% of cases a malignant tumor develops complicating the course of the disease. We report the case of a 25 year-old woman in whom Caroli's disease was diagnosed at the age of 11. From that time on, she had several episodes of cholangitis. In 1989, the abdominal ultrasound and CT scan showed dilatation of the intrahepatic bile ducts, intracystic lithiasis and a solid mass. FNA cytology showed a papillary adenocarcinoma. At laparotomy a tumor was found occupying both hepatic lobes, and intraoperative US showed another two nodules in the left lobe. The tumor was considered unresectable. Examination of the hilar lymph nodes was tumor-negative. Two weeks later, the patient underwent an orthotopic liver transplantation (OLT). The pathological examination confirmed Caroli's disease with adenocarcinoma. Two years after OLT, the patient is alive with normal liver function and no evidence of disease. To our knowledge this is the first case report of adenocarcinoma in Caroli's disease treated by OLT.
卡罗里病的特征是肝内胆管先天性囊性扩张。7%的病例会并发恶性肿瘤，使病情复杂化。我们报告一例25岁女性病例，她11岁时被诊断为卡罗里病。从那时起，她多次发作胆管炎。1989年，腹部超声和CT扫描显示肝内胆管扩张、囊内结石及一个实性肿块。细针穿刺抽吸活检细胞学检查显示为乳头状腺癌。剖腹手术时发现一个肿瘤占据了两个肝叶，术中超声显示左叶还有另外两个结节。该肿瘤被认为无法切除。肝门淋巴结检查未发现肿瘤。两周后，患者接受了原位肝移植（OLT）。病理检查证实为卡罗里病合并腺癌。肝移植术后两年，患者存活，肝功能正常，无疾病迹象。据我们所知，这是首例通过原位肝移植治疗卡罗里病合并腺癌的病例报告。
