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Ectopic Hepatocellular Carcinoma

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Tumors and Tumor-Like Lesions of the Hepatobiliary Tract
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Abstract

Ectopic hepatocellular carcinoma (ectopic HCC) is defined as an HCC that arises from intrahepatic or extrahepatic bile ducts, the gallbladder, from liver parenchyma situated in an extrahepatic organ or tissue, or from extrahepatic hepatogenic progenitor cells. In advanced ectopic HCC, the source of the tumor may be difficult to assess, as ectopic liver tissue may have been completely destroyed by the tumor. Ectopic HCC is a very rare neoplasm, as its most common source, ectopic or heterotopic liver, has an estimated incidence of only 0.24–0.47 %. There is a propensity of ectopic liver to give rise to HCC. Ectopic HCC may be suspected in patients with markedly elevated serum alpha-fetoprotein levels in the absence of an orthotopic liver tumor. Ectopic HCC that develops in large bile ducts can show several growth patterns, one of the patterns characterized by endoluminal polypoid growths that cause biliary obstruction. Ectopic HCCs situated outside the liver can be attached to the organ by a vascular stalk. Uncommon tumors occur in visceral abdominal organs, in the peritoneal surface, in the retroperitoneal space, and within the thoracic cavities.

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Zimmermann, A. (2017). Ectopic Hepatocellular Carcinoma. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Springer, Cham. https://doi.org/10.1007/978-3-319-26956-6_15

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