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Tumours of the biliary system

机译：胆道系统肿瘤

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Biliary hamartoma, bile-duct adenoma (BDA) and mucinous cystadeno-ma are the most common benign biliary tumours. Hamartomas occur sporadically or in association with polycystic liver disease. BDAs may represent a localized response to injury rather than true neoplasms. Cystadenomas are multilocular lesions lined by mucinous epithelium, often with cellular ovarian-like stroma. Cytologic atypia may suggest malignant transformation, but stromal invasion must be identified for diagnosis of cystadenocarcinoma. Cholangiocarcinoma (CC) is the most common malignant neoplasm. Risk factors include primary sclerosing cholangitis, liver flukes, hepatolithiasis and thorotrast exposure. The tumours show mass-forming, periduc-tal-infiltrating or intraductal growth patterns. Intrahepatic CC presents as a hepatic mass and can be confused with hepatocellular carcinoma or metastatic adenocarci-noma. Immunohistochemistry and albumin in situ hybridization are helpful in this distinction. The diagnosis of extrahepatic CC can often be elusive due to the marked stromal desmoplasia, and multiple endoscopic biopsies and brushings may be required. Loss of p53 is common in mass-forming intrahepatic CC, and K-ras mutations in periductal-infiltrative extrahepatic tumours. Prognosis is poor, with 5-year survival of <20%. Lymph-node metastasis and surgical margin status are the most significant prognostic factors.
机译：胆道错构瘤，胆管腺瘤（BDA）和粘液性囊腺瘤是最常见的良性胆道肿瘤。血肿偶发或与多囊性肝病有关。 BDA可能代表对损伤的局部反应，而不是真正的肿瘤。囊腺瘤是由黏液上皮衬砌的多眼病变，常伴有细胞性卵巢样基质。细胞学上的非典型性可能提示恶性转化，但必须鉴别基质浸润才能诊断出膀胱腺癌。胆管癌（CC）是最常见的恶性肿瘤。危险因素包括原发性硬化性胆管炎，肝吸虫，肝结石症和胸腔镜暴露。肿瘤显示肿块形成，周周浸润或导管内生长模式。肝内CC表现为肝块，可与肝细胞癌或转移性腺癌混为一谈。免疫组织化学和白蛋白原位杂交有助于这种区分。由于明显的间质增生，肝外CC的诊断通常难以捉摸，可能需要多次内镜活检和刷牙。 p53丢失常见于大量形成肝内CC，而在导管周围浸润性肝外肿瘤中K-ras突变。预后差，5年生存率<20％。淋巴结转移和手术切缘状态是最重要的预后因素。

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