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Paraganglioma of the Hepatobiliary Tract

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Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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Abstract

Extra-adrenal paragangliomas are a group of neoplasms that derive from extra-adrenal sympathetic and parasympathetic paraganglia and related neuroendocrine cell systems in various organs. The biological behavior of these neoplasms ranges from a relatively benign course to frankly malignant behavior. Primary hepatic paraganglioma is a very rare neoplasm that is usually situated within the liver substance and is nonfunctional. The tumors can grow to a size exceeding 5 cm in diameter, may show a fibrous capsule, and can undergo cystic change. Hepatic paraganglioma can metastasize to locoregional lymph nodes. Histologically, the leading cell type is the large eosinophilic cell that forms paraganglia. These cells are strongly reactive for chromogranin A and synaptophysin. The complex stroma of paragangliomas contains S100 protein-positive sustentacular cells. Paraganglioma can also develop in extrahepatic bile ducts and cause biliary obstruction. Exceptionally, paraganglioma synchronously involves liver and bile ducts. The tumor was also observed in the gallbladder and hepatic ligaments.

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  1. University of Bern, Bern, Switzerland

    Arthur Zimmermann

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  1. Arthur Zimmermann
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Zimmermann, A. (2017). Paraganglioma of the Hepatobiliary Tract. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Springer, Cham. https://doi.org/10.1007/978-3-319-26956-6_74

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  • DOI: https://doi.org/10.1007/978-3-319-26956-6_74

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