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APC-associated hepatic nodules
Tuesday 23 February 2010
Hepatoblastoma (HB), the most common hepatic neoplasm in children is associated with germline mutations in adenomatous polyposis coli tumor-suppressor gene that cause familial adenomatous polyposis syndrome. Individuals with familial adenomatous polyposis have a 750 to 7500× the risk of developing HB.
In children with APC gene mutation undergoing resection or liver transplant for HB, multiple independent adenoma-like nodules lacking qualities of intrahepatic metastases have been described.
These nodules have been interpreted as neoplastic with most being adenomas (GPC3 negative) that show features of independent origin and represent early stages of carcinogenesis, implying potential to progress to HB or hepatocellular carcinoma.
See also
APC-associated hepatoblastoma
References
Germline APC mutations are not commonly seen in children with sporadic hepatoblastoma. Harvey J, Clark S, Hyer W, Hadzic N, Tomlinson I, Hinds R. J Pediatr Gastroenterol Nutr. 2008 Nov;47(5):675-7. PMID: 18955873
The spectrum of APC mutations in children with hepatoblastoma from familial adenomatous polyposis kindreds. Hirschman BA, Pollock BH, Tomlinson GE. J Pediatr. 2005 Aug;147(2):263-6. PMID: 16126064
