UHOD-ULUSLARARASI HEMATOLOJI-ONKOLOJI DERGISI, cilt.22, ss.1-8, 2012 (SCI İndekslerine Giren Dergi)
Primary malignant tumors of the liver are rare in childhood and comprise 1-2% of all childhood malignancies. In this retrospective study the characteristics, treatment and outcome of childhood malignant hepatic tumors diagnosed and treated in a single center between 1996 and 2010 were evaluated. The pretreatment extent of disease (PRETEXT) system was used for staging and the SI-OPEL-3 or modified protocol for treatment. 20 children (13 male, 7 female), diagnosed and treated with primary malignant tumors of the liver (11 hepatocellular carcinoma [HCC], 9 hepatoblastoma [HBS]) were evaluated. In our series the cases with HCC were more than the cases with HBS. In cases with HCC, 8 had chronic hepatitis B infection. Serum alpha-fetoprotein levels were elevated in all cases with HBS and in six with HCC. According to the PRETEXT staging, 12 cases were stage (S) IV, 2 S III, 6 S II. Four had lung metastasis. Four cases with HBS, one patient with HCC are alive with no evidence of disease (19, 20, 21, 26, 102 months). The high incidence of hepatocellular carcinoma in our series is thought to be due to the high incidence of hepatitis B infection in Turkey. Children with chronic hepatitis B virus infection should be followed up keeping in mind the risk of HCC. Children with hemihypertrophia and other predisposing conditions should be followed up for the risk of liver tumors so that they can be diagnosed at early stages.