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原发性肝内胆管囊性肿瘤（ＰＣＴＩＨＢＤ）比较罕见可分为囊腺瘤和囊腺癌。它们分泌大量的粘液形成单房和多房性囊肿，囊内壁发出乳头状赘生物突入囊腔，囊腺癌通常被认为由囊腺瘤恶变而来。文章报告ＰＣＴＩＨＢＤ５例，其中囊腺癌４例，囊腺瘤１例。５例经Ｂ超和ＣＴ检查后均诊断为肝癌，只是在剖腹探查和病理学检查后才获得正确的诊断，手术切除为唯一有效的治疗。４例囊腺癌中１例术后第６天死于大出血，１例随访７个月后死于肝内复发；另２例分别随访４年及５年均存活。１例囊腺瘤手术７年仍健在。
Primary intrahepatic bile duct cystic tumors (PCTIHBD) are rare and can be divided into cystadenoma and cystadenocarcinoma. They secrete a large amount of mucus to form single- and multi-room cysts, and the inner wall of the capsule causes papillary neoplasms to protrude into the cyst. Cystic adenocarcinoma is usually thought to be caused by the malignant transformation of cystadenoma. The article reported 5 cases of PCTIHBD, including 4 cases of cystadenocarcinoma and 1 case of cystadenoma. Five cases were diagnosed as liver cancer after B-ultrasonography and CT examination. Only after laparotomy and pathological examination did they obtain the correct diagnosis. Surgical resection was the only effective treatment. One of 4 cases of cystadenocarcinoma died of massive hemorrhage on the 6th day after operation. One patient died of intrahepatic recurrence 7 months after follow-up. The other 2 patients survived after 4 and 5 years of follow-up, respectively. One case of cystadenoma was still alive for 7 years.