Dados do Trabalho

Título

Malignant Liver Tumors in Children and Adolescents: 12 Years Experience of a Referral Center for Pediatric Cancer in Brazil

Introdução

Liver tumors are rare in children and adolescents, representing 0.3 to 2% of all pediatric tumors, with an annual rate of 0.5 to 2.5 per million inhabitants in western countries, and their prognosis depends basically on the histological type, tumor size and resectability.

Objetivo

Report a series of cases of childhood and adolescence malignant liver tumors treated at a referral center for pediatric cancer in Brazil, describing the clinical findings, diagnosis, treatment and clinical evolution of cases.

Método

Retrospective, descriptive and cross-sectional study of 21 patients treated at a referral center for pediatric cancer from 2005 to 2017.

Resultados

Of the total number of patients (n = 21), hepatoblastoma (n=8) and hepatocellular carcinoma (n=3) were the most commom diagnosis, and the most common clinical signs were abdominal tumor (95.2%), abdominal pain (38%), and fever (19%). Abdominal ultrasound was the first complementary exam performed in 95.2% of patients and alpha-fetoprotein (AFP) was elevated in eleven cases, all cases of hepatoblastoma and hepatocellular carcinoma. Of the total of cases, nineteen patients (90.5%) were treated by chemotherapy combined to surgical resection. Five-year survival rates for all patients with malignant liver tumors were 32%.

Conclusão

Childhood and adolescence malignant liver tumors are rare, abdominal mass is the most common clinical manifestation, and abdominal ultrasound was the main complementary exam at initial investigation. Hepatoblastoma was the most common malignant histology followed by hepatocelullar carcinoma, and regarding its treatment, chemotherapy± radiotherapy combined to surgical resection was the most commom modality of treatment for primary tumors or liver metastases.

Palavras-chave

malignant, liver, pediatric, adolescent

Área

Tumores hepatobiliopancreáticos