RESUMEN
AIM: To analyse incidence, treatment and outcomes of paediatric liver malignancies in Finland during 1987-2017. METHODS: Medical records and national cancer registry data of 47 children with liver malignancies were reviewed. Survival was calculated with the Kaplan-Meier method. RESULTS: During follow-up, liver malignancy incidence remained stable at 1.1:106 . Altogether, 42 patients with hepatoblastoma (n = 24), hepatocellular carcinoma (n = 11) and undifferentiated embryonal sarcoma (n = 7) underwent surgery at median age 4.6 (interquartile range, 2.0-9.6) years and were followed up for 13 (7.0-19) years. Cumulative 5-year survival was 86% for hepatoblastoma, 41% for hepatocellular carcinoma and 67% for undifferentiated embryonal sarcoma. Five-year survival was decreased among hepatoblastoma patients aged ≥ 2.4 years (73% versus 100%, P = .040), with PRETreatment EXTent of disease IV (PRETEXT, 60% vs 100%, P = .004), and with recurrent disease (67% vs 88%, P = .029). Recurrent/residual disease associated with decreased 5-year survival in hepatocellular carcinoma (0% vs 83%, P = .028). Survival was similar among 19 transplanted and 23 resected patients. In total, 14 deaths occurred either for the underlying malignancy (n = 8), adverse effects of chemotherapy (n = 5) or unrelated reasons (n = 1). CONCLUSION: Outcomes for PRETEXT I-III hepatoblastoma and un-metastasized hepatocellular carcinoma were encouraging. Adverse effects of chemotherapy significantly contributed to mortality.
Asunto(s)
Hepatoblastoma , Neoplasias Hepáticas , Trasplante de Hígado , Anciano , Niño , Preescolar , Finlandia/epidemiología , Hepatoblastoma/tratamiento farmacológico , Hepatoblastoma/epidemiología , Hepatoblastoma/cirugía , Humanos , Incidencia , Lactante , Neoplasias Hepáticas/epidemiología , Neoplasias Hepáticas/cirugía , Resultado del TratamientoRESUMEN
We report a case of a 13-year-old boy with a presumed neck cyst who developed sudden tracheal obstruction and out-of-hospital cardiac arrest. Cardiorespiratory collapse occurred due to an improperly diagnosed mediastinal tumor. This report serves to alert Emergency Physicians and emergency medical services personnel of the rare and rapidly progressive nature of respiratory compromise caused by a mediastinal tumor, which may have lethal consequences if not recognized and treated promptly.