Eastern Hepatobiliary Surgery Hospital, Second Military Medical University, Shanghai, China.
Eur J Gastroenterol Hepatol. 2009 May;21(5):580-4. doi: 10.1097/MEG.0b013e3282fc73b1.
Multiple bile duct hamartomas (BDHs)/von Meyenburg complexes, are tumor-like lesions of the liver. Malignant transformation in BDHs has been previously reported in very rare instances, and the most common tumor arising in this clinical setting is cholangiocarcinoma. Herein, we report on clinicopathological findings in two cases of cholangiocarcinoma occurring in liver with multiple BDHs. Histopathologically, multiple BDHs showed morphologic transition from clearly benign to dysplasia or carcinoma in situ, then to invasive carcinoma sequence of the biliary epithelium. The neoplastic epithelium showed positivity for cytokeratin 19, CA 19-9, and epithelial membrane antigen. Staining for Hep Par 1, alpha-fetoprotein, cytokeratin 20, and alpha1-antitrypsin was negative. All sections from the non-neoplastic liver in each specimen showed multiple BDHs. Any other clinically detectable primary tumor was not found. These two neoplasms were interpreted as a cholangiocarcinoma arising in BDHs. This suggested BDHs might be a risk factor of development of cholangiocarcinoma.
多发性胆管错构瘤（BDH）/von Meyenburg 复合体是肝脏的肿瘤样病变。BDH 曾有过极罕见恶变的报道，而在此种临床情况下最常见的肿瘤是胆管癌。本文报道了 2 例发生于多发性 BDH 肝脏的胆管癌的临床病理特征。组织病理学上，多发性 BDH 从明显良性到异型增生或原位癌，再到胆管上皮的浸润性癌序列，呈现出形态学的转变。肿瘤上皮细胞角蛋白 19、CA 19-9 和上皮膜抗原阳性，Hep Par 1、甲胎蛋白、角蛋白 20 和α1-抗胰蛋白酶染色阴性。每个标本的非肿瘤性肝组织的所有切片均显示多发性 BDH。未发现任何其他临床上可检测到的原发性肿瘤。这两个肿瘤被解释为起源于 BDH 的胆管癌。这表明 BDH 可能是胆管癌发展的一个危险因素。
