Bile duct adenoma - Altmeyers Encyclopedia - Department Internal medicine

Bile duct adenoma History Definition Bile duct adenoma (BDA) is a liver tumor arising from the intrahepatic bile duct epithelium. BDA is considered a benign tumor (Wei 2015). However, conversions to malignant cholangiocarcinoma have been reported according to Zheng Jie Teng (2022). Classification Intrahepatic BDA is one of the primary liver tumors. It accounts for approximately 1.3% of primary liver tumors (Wei 2015). Occurrence/Epidemiology BDA is rare (Herold 2022), and the incidence is unknown to date (Wei 2015). Only 5 BDAs were found in 50,000 autopsies. Because of the rarity of BDA, all cases should be reported to add to the current literature (Zheng Jie Teng 2022).To date, patients developed the disease between 20 - 70 years, and the average age is 55 years. There is no significant gender preference (Chen 2014). Etiopathogenesis The etiopathogenesis is controversial to date (Wei 2015). Pathophysiology BDAs are thought to arise from reactive focal injury to the bile duct according to current theories (Zheng Jie Teng 2022).Wei et al. (2015) suggested that BDAs represent disorganized peribiliary glands, with similar phenotype of secretory gland cells. They consist of small, uniform ducts of cuboidal cells containing nuclei. Clinical features A BDA is typically found incidentally (Malek 2022) during e.g. abdominal surgery or autopsy. It may occur in association with diseases such as chronic he patitis due to hepatitis C disease, hemangioma, biliary stasis, alcoholic cirrhosis, focal nodular hyperplasia. BDAs may also occur after trauma or inflammatory changes (Zheng Jie Teng 2022). Diagnostics There are no typical imaging features that allowed a preoperative diagnosis. Thus, the diagnosis is based solely on histopathological and immunochemical findings (Wei 2015).The BDA is relatively small, ranging in diameter from 1 - 20 mm. It is always located under the liver capsule, clearly demarcated, but itself without a capsule (Chen 2014). Imaging Abdominal sonographyHere, nodular enhancement in the periphery of a hepatic tumor can be visualized with a "quick wash-in and wash-out" sign (Wei 2015).BDAs can be anechoic with internal echogenic areas or mildly anechoic with incomplete anechoic halo (Chuy 2018). Computed tomographyShowing a tumor mass usually of < 2 cm, lying subcapsular but not encapsulated (Wei 2015).On CT, BDAs may present as relatively hypodense to the liver parenchyma in the phase without enhancement and with hyperenhancement in the arterial phase (Chuy 2018). MRIHere, T1 hypointense and T2 hyperintense lesions are found (Chuy 2018). Laboratory There are no tumor markers (Wei 2015). Histology Typical of BDA are:- large number of proliferating bile ducts showing variable amounts of collagen and infiltration of inflammatory cells- immunohistochemical stains for CK 19, CD 10, and CD 56 are positive.- staining for p53 and AFP, however, negative (Wei 2015). Differential diagnosis - intrahepatic cholangiocarcinoma (Zheng Jie Teng 2022)- metastatic adenocarcinoma- von Meyenberg syndrome- primary sclerosing cholangitis- biliary adenofibroma (Wei 2015) General therapy However, differentiation of malignant versus benign lesions can be difficult on both imaging and histopathology (Chuy 2018). Surgical resection is therefore the treatment of choice, as there is a possibility of carcinogenesis in BDA (Wei 2015). Progression/forecast A BDA is a benign tumor with a good prognosis. The possibility of carcinogenesis has been described (Wei 2015). Aftercare Tumor recurrences have not been described to date (Wei 2015). Literature Chen L, Xu A Y, Chen F (2014) Bile duct adenoma: a case report and literature review. World J Surg Oncol. 12, 125Chuy J A, Garg I, Graham R P, Van Buren W M, Venkatesh S K (2018) Imaging features of bile duct adenoma: case series and review of literature. Diagn Interv Radiol. 24 (5) 249 - 254Herold G et al (2022) Internal Medicine. Herold Publishers 560Kasper D L, Fauci A S, Hauser S L, Longo D L, Jameson J L, Loscalzo J et al (2015) Harrison's Principles of Internal Medicine. Mc Graw Hill Education 553, 2083Malek N P, Bitzer M, Galle P R, Voesch S (2022) S3- guideline: diagnosis and therapy of hepatocellular carcinoma and biliary carcinoma. AWMF- Register- No. 032 / 053OLWei J, Zhang D, Yang J, Xu C (2015) Intrahepatic bile duct adenoma (peribiliary gland hamartoma): a case report and review of literature. Int J Clin Exp Pathol. 8 (5) 5908 - 5913.Zheng Jie Teng T, Szu Lyn Ding C, Shelat V G (2022) Bile duct adenoma: rare benign liver tumour. Singapore Med J. 63 (12) 769 - 770

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