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Hepatocellular carcinoma, childhood-onset

Orpha number ORPHA33402
Prevalence of rare diseases Unknown
Inheritance
  • Sporadic
Age of onset Childhood
ICD 10 code
  • C22.0
MIM number
Synonym(s) -

Summary

Hepatocellular carcinoma (HCC) is a very rare pediatric tumour. Primary hepatic tumours as a whole represent about 1-1,5% of all pediatric neoplasm with an incidence of 1 case in 650 000 children aged less than 15 years. Hepatoblastoma (see this term) is the most frequent primary hepatic tumour in this age group followed by HCC. HCC tends to occur in adolescents. In adults, HCC constitutes the most common liver cancer. Main symptoms are palpable hepatic mass, abdominal pain and also cachexia and jaundice in advanced cases. Serum alpha-fetoprotein levels are frequently elevated. In the pediatric population, HCC can complicate liver cirrhosis due to viral hepatitis, - especially in endemic areas (sub-Saharan Africa, East Asia, Native Eskimos of Alaska) - or metabolic diseases. HCC arising in otherwise healthy liver are more common in the pediatric population than in adults. Current treatment of HCC is largely inefficient due to its extreme chemoresistance and to usually advanced disease at diagnosis. Overall survival at 3 years remains below 25%. *Author: Drs P. Czauderna and G. Perilongo (July 2004)*.

Detailed information

Review article
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