Childhood hepatocellular adenoma in familial adenomatous polyposis: Mutations in adenomatous polyposis coli gene and p53
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Neonatal Liver Tumors
2021, Clinics in PerinatologyCitation Excerpt :HB has been associated with few/many/several underlying inherited conditions, including Beckwith-Wiedemann syndrome5,6 and hemihyperplasia overgrowth syndrome, which both require tumor screening with abdominal ultrasonography and AFP level measurement in affected syndromic patients every 3 months for the first 8 years of life.6 HB is also associated with familial adenomatous polyposis, and these patients should undergo routine periodic surveillance screening as well.7,8 The prognosis of HB, as it is for all primary liver tumors, is based on the presence of metastatic disease and the ability for the primary tumor to be completely resected.42
Chapter 95 - Hepatic tumors in childhood
2016, Blumgart's Surgery of the Liver, Biliary Tract and Pancreas: Sixth EditionFamilial adenomatous polyposis: A review of gastrointestinal manifestations
2015, Diagnostic HistopathologyCitation Excerpt :Reported cases showed APC (involving Wnt/beta-catenin pathway) and HNF1alpha mutations. Notably, in two cases, hepatocellular carcinoma was noted to arise in these hepatic adenomas.91–93 Hepatocellular carcinoma is seen rarely in FAP individuals (especially children), but the association has been suggested to alert the paediatrician to the possibility of FAP syndrome and initiate genetic testing for APC gene, especially in the absence of a clear aetiology, or if there is a family history of colon carcinoma or desmoid tumour.94,95
Hepatic tumors in childhood
2012, Blumgart's Surgery of the Liver, Biliary Tract and PancreasPediatric Polyposis Syndromes
2011, Pediatric Gastrointestinal and Liver DiseasePediatric Polyposis Syndromes
2010, Pediatric Gastrointestinal and Liver Disease