Pediatric Hepatocellular Carcinoma
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REVIEW ARTICLE
Pediatric Hepatocellular Carcinoma Fatma İlknur Varol 1
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose Pediatric hepatocellular carcinoma is rarely seen in childhood. It constitutes approximately 1% of childhood solid organ malignancies. Pediatric hepatocellular carcinoma is the second most common malignant liver tumor after hepatoblastoma in children. In this review, we aimed to review the diagnosis and treatment of pediatric hepatocellular carcinoma in the light of the latest literature. Methods We reviewed the literature in terms of the diagnosis and treatment of pediatric hepatocellular carcinoma. Results Hepatocellular carcinoma (HCC) and hepatoblastoma constitute 0.5–1.5% of all childhood malignant tumors. HCC is responsible for 27% of all liver tumors and 4% of all pediatric liver transplantations. While 99.6% of HCC is seen in adults, only 0.4% of it is seen in pediatric patients. Etiological predisposition and biological behavior are different from adults. In a child with cirrhosis or liver disease, HCC should be suspected in the presence of a high level of AFP and an abnormal nodule on ultrasonography. Hepatoblastoma should be considered first in the differential diagnosis. Conclusion Treatment of pediatric HCC is challenging. Complete surgical resection is essential for the cure. To this end, different neoadjuvant chemotherapy protocols have been designed to convert non-resectable tumors into resectable tumors. For tumors that cannot be resected, liver transplantation for each patient with childhood HCC should be decided individually. Keywords Pediatric . Hepatocellular carcinoma . Hepatoblastoma
Abbreviations HCC SEER AFP HCN-NOS NTBC GSD PFIC BSEP TJP2 AIH WD CT
Hepatocellular carcinoma Surveillance, Epidemiology, and End Result Alpha-fetoprotein Hepatocellular neoplasm not otherwise specified [2-(2-nitro-4-trifluoromethybenzoyl)1,3cyclohexanedion] Glycogen storage diseases Progressive familial intrahepatic cholestasis Bile salt export pump Tight junction protein 2 Autoimmune hepatitis Wilson disease Tomography
* Fatma İlknur Varol [email protected] 1
Department of Pediatric Gastroenterology, Hepatology, and Nutrition, Faculty of Medicine, Inonu University, 244280 Malatya, Turkey
MR APASL EFS OS SIOPEL GPOH NASPGHAN UNOS
Magnetic resonance Asian Pacific Association for the Study of the Liver Event-free survival Overall survival International Society of Pediatric Oncology Liver Tumor Study German Society for Pediatric Oncology and Hematology North America Society for Pediatric Gastroenterology, Hepatology and Nutrition United Network for Organ Sharing
Introduction Primary liver tumors are rarely seen in childhood [1, 2]. They constitute approximately 1% of childhood solid organ malignancies [1]. Pediatric hepatocellular carcinoma (HCC) is the second most common malignant liver tumor after hepatoblastoma in children. It affects especially adolescent children. HCC and hepatoblastoma constitute 0.5–1.5% of all childhood malignant
J Gastroin
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