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Biliary Cystadenoma and Cystadenocarcinoma

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Fibrocystic Diseases of the Liver

Part of the book series: Clinical Gastroenterology ((CG))

Summary

Biliary cystadenoma (BCA) and its malignant counterpart biliary cystadenocarcinoma (BCAC) are rare cystic tumors originating from the biliary system. BCA appears as a multilocular intrahepatic cyst lined by benign cuboidal or columnar non-ciliated epithelium. If the epithelium shows neoplastic degeneration it is defined as BCAC. According to the presence or absence of ovarian-like mesenchymal stroma, BCA is classified into two subtypes with different prognosis. BCA is a tumor with a propensity for recurrence or malignant degeneration and should always be completely resected. Partial interventions like marsupialization or cyst drainage are not considered adequate. The diagnosis is based on radiologic imaging and the role of cytology and histology is limited to doubtful cases, the most frequent being the differentiation from hemorrhagic cysts. In these cases fine needle aspiration (FNA) may be used to confirm BCA by the identification of atypical biliary cells and elevated intracystic CA19-9 levels. FNA, however, lacks adequate sensitivity and specificity and should always be used in conjunction with imaging. Due to the slow growth of these tumors, the prognosis after adequate resection is good and prolonged survival has been unanimously reported in the literature. Even in the case of BCAC, long-term survival can be achieved, provided that the tumor is discovered and resected without distant foci or metastasis.

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Acknowledgment

We are indebted to Dr. L. Grazioli for kindly providing iconographic material.

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Authors and Affiliations

  1. Hepatology Unit, Treviglio Hospital, Treviglio, BG, Italy

    Paolo Del Poggio MD

  2. Pathology Department, Treviglio Hospital, Treviglio, BG, Italy

    Ezio Pezzica MD & Daniela Corti MD

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  1. Paolo Del Poggio MD
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  2. Ezio Pezzica MD
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Editors and Affiliations

  1. School of Medicine, Children's Hospital & Regional, University of Washington, Sand Point Way NE. 4800, Seattle, 98105, USA

    Karen F. Murray

  2. St. Paul University Hospital, Harry Hines Blvd. 5939, Dallas, 75390-9258, USA

    Anne M. Larson

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Del Poggio, P., Pezzica, E., Corti, D. (2010). Biliary Cystadenoma and Cystadenocarcinoma. In: Murray, K., Larson, A. (eds) Fibrocystic Diseases of the Liver. Clinical Gastroenterology. Humana Press. https://doi.org/10.1007/978-1-60327-524-8_20

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  • DOI: https://doi.org/10.1007/978-1-60327-524-8_20

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