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Rare Tumors of the Bile Ducts

Pathogenesis, Epidemiology, Diagnosis, and Management

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Hepato-Pancreato-Biliary Malignancies

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Abstract

While cholangiocarcinoma is the most common type of bile duct tumor, other neoplasia may involve the biliary tract, and it is important to include them in the differential diagnosis as management varies depending on tumor type. Benign biliary tumors are extremely rare with an incidence ranging from 0.02% to 0.10% based on pathological examination of resected ducts. These tumors along with malignant and premalignant tumors will be reviewed in this chapter.

Tumors discussed are biliary adenofibroma, bile duct adenoma, adenomyoma and adenomyomatous hyperplasia, ciliated hepatic foregut cyst, cystadenoma, cystadenocarcinoma, granular cell tumor, von Meyenburg complex, schwannoma, traumatic neuroma, intraductal papillary neoplasia of the bile duct, embryonal rhabdomyosarcoma, lymphoma, melanoma, carcinoid, paraganglioma, and squamous cell carcinoma. Given the rarity of these tumors, information on pathogenesis is limited, statistics are based on small patient populations, and management is not standardized. A comprehensive summary, however, for each of these tumor types has been compiled based on available literature.

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Authors and Affiliations

  1. Section of Digestive Diseases, Yale University School of Medicine, New Haven, CT, USA

    Earl V. Campbell III & Priya Jamidar

Authors
  1. Earl V. Campbell III
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  2. Priya Jamidar
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Corresponding author

Correspondence to Priya Jamidar .

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Editors and Affiliations

  1. Medical Director, Capital Health Medical Center Hopewell, Pennington, NJ, USA

    Cataldo Doria

  2. Capital Health Center for Digestive Health, Pennington, NJ, USA

    Jason N. Rogart

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Campbell, E.V., Jamidar, P. (2021). Rare Tumors of the Bile Ducts. In: Doria, C., Rogart, J.N. (eds) Hepato-Pancreato-Biliary Malignancies. Springer, Cham. https://doi.org/10.1007/978-3-030-37482-2_20-1

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  • DOI: https://doi.org/10.1007/978-3-030-37482-2_20-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-37482-2

  • Online ISBN: 978-3-030-37482-2

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