Primary Malignant Liver Tumors: eight-year experience in a Pediatric Hospital in Brazil. A cross-sectional study

• Autor(a) principal: FALQUETO,LORAINE ENTRINGER
• Data de Publicação: 2022
• Outros Autores: VILAR,PAULA RUBIO, CAMPOS,HELDER GROENWOLD, SCHULZ,CLAUDIO, MATTOS E SILVA,ELISANGELA DE
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Revista do Colégio Brasileiro de Cirurgiões
• Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0100-69912022000100217
• topic: Hepatectomy; Liver Neoplasms; Liver Transplantation; Pediatrics; Hepatoblastoma

ABSTRACT Introduction: liver tumors are rare neoplasms in childhood (1-2%), and about 2/3 are malignant. Hepatoblastoma (HB) is the most frequent, followed by hepatocellular carcinoma (HCC). In both, the main treatment is surgical resection. Currently, chemotherapy and liver transplantation have improved outcomes. Objective: study of the epidemiological profile and evolution of liver cancer cases in a referral pediatric hospital. Methodology: a retrospective survey of medical records of patients aged up to 18 years with a diagnosis of primary malignant hepatic neoplasm between 2012 and 2020, carried out in the largest exclusively pediatric hospital in Brazil. Results: a total of 13 patients with malignant liver tumors (HB 12, HCC 1) were treated. Of the HB cases, 66,7% were male, with a mean age of 2 years and the main alteration in the palpable abdominal mass. Tumors involved an average of 3 liver segments, more in the right lobe (54%). Only one patient was treated with surgery without neoadjuvant therapy, another one underwent transplantation like the first treatment, and another 2 required liver transplantation as a rescue. The middle follow-up time of patients with HB was 39 months and only 1 case died due to febrile neutropenia. The 5-year overall and disease-free survival was 91.7% and 81.5%, respectively. Conclusion: Advanced staging at the time of diagnosis has always been a poor prognostic factor in patients with primary malignant liver tumors. However, the results and survival have improved with the advancement of chemotherapy, surgical technique, and liver transplantation.