Abstract
Aim: to compare features of hepatocellular carcinoma (HCC) in children and adult patients and to assess the possibility of translating the experience of adult patients treating in pediatric oncology. Мethods: the literature dedicated to pediatric and adult HCC investigation and treatment has been analyzed. Pathomorphology, molecular-genetic features of disease, the drug and surgical treatment approaches, in particular immunotherapy and liver transplantation were carefully compared in both groups. Results: for pediatric HCC the analysis revealed that: fibrolamellar subtype as well as large size of primary tumor are more common; typical molecular-genetic markers are presented. It is advisable to use the adopted adults treatment methods such as targeted and immunotherapy in children. In particular, the HCC surgery, and the indications for liver transplantation in children should be different from adults. Conclusion: further translation of th e adult patients treating experience will help to improve outcome in children with HCC. In order to optimize the treatment of pediatric HCC it is necessary to continue the investigations in international research groups.
Highlights
ФГБУ “Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им
Aim: to compare the features of hepatocellular carcinoma in children and adult patients and to assess the possibility of translating the experience of adult patients treating in pediatric oncology
Pathomorphology, molecular genetic features of disease, the drug and surgical treatment approaches, in particular immunotherapy and liver transplantation were carefully compared in both groups
Summary
Первичные злокачественные опухоли печени составляют 1–2% всех злокачественных опухолей у детей [10]. Частота первичных ЗНО печени составляет 1,6 на 1 млн детского населения в возрасте 0–14 лет [6, 12], а на долю ГЦР приходится 0,5–1% всех опухолей у детей. ГБ составляет порядка 80% первичных ЗНО печени у детей, с наибольшей частотой в возрасте от 6 мес до 3 лет. 81,5% пациентов детского возраста имели хроническую HBV-инфекцию, при этом у 61,5% больных выявлены многоочаговые опухоли. В отличие от взрослых, для которых характерно развитие ГЦР при длительно текущем циррозе печени на фоне HBV, у детей опухоли встречаются на фоне перинатальной HBV-инфекции [9, 14]. Для пациентов детского возраста характерна большая частота фиброламеллярного ГЦР (фГЦР) по сравнению со взрослыми. Частота фГЦР у детей составляет 24,1% по сравнению с 0,3% у взрослых (p = 0,71) [11]. При анализе результатов исследования Pediatric Intergroup Hepatoma Protocol INT-0098 (Pediatric Oncology Group Study 8945 / Children's Cancer Group Study 8881) получены схожие данные: из 46 больных, включенных в исследование, 10 (22%) имели фГЦР, 36 (78%) – не-фГЦР [16]
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