Liver and intrahepatic bile ducts

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Liver and intrahepatic bile ducts - Tumor

Benign tumors

Paraganglioma

Reviewers: Deepali Jain, M.D. (see Reviewers page)
Revised: 9 January 2013, last major update February 2012
Copyright: (c) 2004-2013, PathologyOutlines.com, Inc.

General
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● Very rare benign tumor that may arise from adrenal rests

Case reports
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● 46 year old man (Am J Surg Pathol 2002;26:945)

Treatment
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● Excision

Gross description
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● Firm, pale gray nodule with variable fibrosis and thin fibrous capsule, large (~ 10 cm); no cirrhosis

Micro description
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● Polygonal eosinophilic tumor cells, round nuclei, indistinct nucleoli, arranged in small nests (“zellballen”) or trabeculae in vascular stroma
● No pleomorphism, no mitotic figures

Positive stains
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● Chromogranin A, synaptophysin, neuron-specific enolase, insulin like growth factor II (IGF-II) by IHC or ISH
● Sustentacular cells are S100+

Negative stains
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● Albumin mRNA, keratin, CD10, vimentin, smooth muscle actin

Differential diagnosis
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● Fibrolamellar hepatocellular carcinoma: broad fibrous bands, atypia, invasion, HepPar1+
● Neuroendocrine tumors: no absence of Zellballen or sustentacular cells

End of Liver and intrahepatic bile ducts - Tumor > Benign tumors > Paraganglioma

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