肝内胆管腺纤维瘤伴恶变临床病理观察

Clinico-pathological characteristics of intrahepatic biliary adenofibroma with malignant transformation

目的探讨肝内胆管腺纤维瘤(BAF)及其恶变的临床病理特点及鉴别诊断。方法对1例BAF伴恶变的病例进行回顾,分析其临床表现、组织病理学特点,并复习相关文献。结果患者男性,83岁。10年前腹部B超提示肝左叶占位性结节,自诉大小约2 cm×1 cm,未予系统治疗。入院后CT示肝左叶可见一类圆形低密度影,大小6.2 cm×3.6 cm×3.3 cm,边界清晰。组织病理学示大部分区为典型的胆管腺纤维瘤,即增生的腺管、腺泡和微囊性扩张的胆管包埋于丰富的纤维性间质中,腺管衬覆非黏液分泌型立方状或低柱状上皮;少部分区上皮细胞可见轻~重度不典型增生。局灶符合原位癌变伴小灶浸润。结论胆管腺纤维瘤是一种少见的肝内胆管来源的良性肿瘤,但有进展为恶性并发生远处转移的可能,且影像检查与其他肝肿瘤鉴别困难,故详细的组织病理学检查是诊断的关键。

Objective To observe the clinico-pathological characteristics of biliary adenofibroma（ BAF） and its differential diagnosis. Methods A rare ease of BAF was reported and the clinicopathological features were analyzed with review of related literature. Results A 83 year-old man complained of type-B ultrasonic discovering 2 cm × 1 cm hepatic mass 10 years ago without any symptom and therapy. He was in hospital this time,because MRI showed a quasicircular,low-density,well-circumscribed,6. 2 cm ＊ 3 cm. 6 ＊ 3. 3 cm mass in the left lobe of liver. Histologically,the tumor was composed of glandular tubulo-acinar and cystic structures lined by a monolayered cuboidal to low columnar nonmucous biliary-type epithelium,embedded in a moderately cellular fibrous stroma. There were multiple foci of epithelial atypia that represented low to high grade dysplasia. A small focus of in situ and tiny invasive carcinoma probably exited.Conclusions BAF is a rarely benign tumor derived from interhepatic duct and might be able to develop malignant transformation and even distant metastasis. Imaging studies are nonspecific and cannot distinguish BAF from other hepatic tumors. The key to diagnosis of BAF is mainly based on histological examination.