Pediatric Malignant Liver Tumors: Results of a Single Center

Rejin Kebudi
2012 International Journal of Hematology and Oncology  
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
more » ... t 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. ÖZET Pediatrik Malign Karaci¤er Tümörleri: Tek Merkez Sonuçlar› Primer malign karaci¤er tümörleri çocukluk ça¤›nda seyrek görülmekte ve tüm çocukluk ça¤› malignitelerinin%1-2'sini oluflturmakta-d›r. Bu retrospektif çal›flmada tek bir merkezde 1996 -2010 y›llar› aras›nda tedavi gören çocukluk ça¤› malign karaci¤er tümörü ta-n›l› olgular›n klinik özellikleri, tedavi ve sonuçlar› de¤erlendirilmifltir. Tedavi öncesi hastal›¤›n derecelendirilmesi PRETEXT s›n›fland›rma-s›na göre yap›lm›fl ve tedavide SIOPEL-3 veya modifiye protokol kullan›lm›flt›r. Primer malign karaci¤er tümörü (11 hepatosellüler karsinom [HCC], 9 hepatoblastom [HBS]) tan›s› alan ve tedavi edilen 20 çocuk hasta (13 erkek, 7 k›z) de¤erlendirilmifltir. Serimizde HCC'li olgular HBS'lu hastalardan daha fazla idi. HCC'li hastalar›n 8'inde kronik hepatit B enfeksiyonu vard›. Serum alfa-fetoprotein düzeyleri HBS'li olgular›n tümünde ve HCC'li hastalar›n 6's›nda yüksekti. PRETEXT s›n›fland›rmas›na göre hastalar›n 12'si evre IV, 2'si evre III, 6's› evre II idi. 4 hastan›n akci¤er metastaz› vard›. HBS'li 4 hasta ve HCC'li bir hasta hastal›k bulgusu olmaks›z›n (19, 20, 21, 26, 102 ayd›r) yaflamaktad›r. Serimizde hepatosellüler karsinom insidans›n›n Türkiye'de kronik hepatit B enfeksiyonun yüksek insi-dans› nedeni ile yüksek oldu¤u düflünülmektedir. Kronik hepatit B enfeksiyonu olan çocuklar HCC riski göz önünde tutularak izlenmelidir. Hemihipertrofi ve di¤er predispozan durumlar› olan çocuklar karaci¤er tümörü geliflimi yönünden takip edildiklerinde erken dönemde teflhis edilebilirler.
doi:10.4999/uhod.11049 fatcat:re5uearbhfdm3ebs3olzcnycru