Asunto(s)
Enfermedades Autoinmunes/etiología , Colangitis Esclerosante/etiología , Síndrome Mucocutáneo Linfonodular/complicaciones , Enfermedades Autoinmunes/fisiopatología , Niño , Colangitis Esclerosante/fisiopatología , Humanos , Hígado/patología , Pruebas de Función Hepática , Masculino , Síndrome Mucocutáneo Linfonodular/fisiopatologíaRESUMEN
Primary hepatic malignancies are uncommon in pediatrics. Tumors such as hepatocellular carcinoma (HCC) develop typically in the setting of chronic liver disease. The incidence of HCC in Wilson's disease-related cirrhosis is disproportionately lower than in many other forms of end-stage liver disease. A preadolescent girl presented with Wilson's disease cirrhosis and a HCC requiring orthotopic liver transplantation. This case highlights the need to consider hepatic malignancies even in young Wilson's disease patients. Pediatric Wilson's disease and the hepatic tumor literature are reviewed.
Asunto(s)
Carcinoma Hepatocelular/diagnóstico , Ceruloplasmina/metabolismo , Degeneración Hepatolenticular/diagnóstico , Cirrosis Hepática/diagnóstico , Neoplasias Hepáticas/diagnóstico , Hígado/patología , Carcinoma Hepatocelular/etiología , Carcinoma Hepatocelular/cirugía , Niño , Femenino , Degeneración Hepatolenticular/complicaciones , Degeneración Hepatolenticular/metabolismo , Humanos , Hígado/diagnóstico por imagen , Cirrosis Hepática/etiología , Cirrosis Hepática/cirugía , Neoplasias Hepáticas/etiología , Neoplasias Hepáticas/cirugía , Trasplante de Hígado , Rasgo Drepanocítico/complicaciones , UltrasonografíaRESUMEN
Hepatocellular adenoma is an uncommon neoplasm, especially in the childhood age group. We describe a previously obese 13-year-old male with a giant hepatocellular adenoma requiring an extensive hepatic resection. The related pediatric tumor literature, diagnosis and clinical management is discussed.