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胆管癌发生于伴有多个胆管错构瘤(冯·迈恩伯格复合体)的肝脏。

Cholangiocarcinoma occurring in a liver with multiple bile duct hamartomas (von Meyenburg complexes).

作者信息

Röcken C, Pross M, Brucks U, Ridwelski K, Roessner A

机构信息

Institute of Pathology, Otto-von-Guericke-University, Magdeburg, Germany.

出版信息

Arch Pathol Lab Med. 2000 Nov;124(11):1704-6. doi: 10.5858/2000-124-1704-COIALW.

DOI:10.5858/2000-124-1704-COIALW
PMID:11079031
Abstract

A 59-year-old woman presented with epigastric pain and weight loss. Ultrasound, computed tomography, and magnetic resonance imaging scans of the abdomen showed a tumor in segments 6 and 7 of the right liver lobe, measuring 8 cm in greatest diameter. The tumor was subsequently resected, and histopathology showed a poorly differentiated adenocarcinoma immunoreactive for CA 19-9 and cytokeratin 19. In the absence of any other clinically detectable primary tumor, the lesion was diagnosed as a peripheral intrahepatic cholangiocarcinoma. In addition, multiple bile duct hamartomas were found in the surrounding parenchyma. The tumor was unrelated to Caroli disease, primary sclerosing cholangitis, ulcerative colitis, or nonbiliary cirrhosis, as demonstrated by further clinical and histopathologic investigations, but probably was associated with the presence of multiple bile duct hamartomas. To our knowledge, this is the eighth reported case of a cholangiocarcinoma associated with multiple bile duct hamartomas.

摘要

一名59岁女性因上腹部疼痛和体重减轻就诊。腹部超声、计算机断层扫描和磁共振成像扫描显示右肝叶6段和7段有一个肿瘤,最大直径为8厘米。该肿瘤随后被切除,组织病理学显示为低分化腺癌,对CA 19-9和细胞角蛋白19呈免疫反应。在未发现任何其他临床可检测到的原发性肿瘤的情况下,该病变被诊断为周围型肝内胆管癌。此外,在周围实质中发现了多个胆管错构瘤。进一步的临床和组织病理学研究表明,该肿瘤与卡罗利病、原发性硬化性胆管炎、溃疡性结肠炎或非胆汁性肝硬化无关,但可能与多个胆管错构瘤的存在有关。据我们所知,这是第八例报告的与多个胆管错构瘤相关的胆管癌病例。

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