Neuroblastoma: Experiência recente de um centro de oncologia pediátrica

Abstract

Neuroblastoma (NB) is the most frequent extracranial solid tumor in pediatric age. It is included in the major group of neuroblastic tumors (NT), as well as ganglioneuromas (GN) and ganglioneuroblastomas (GNB). NT are distributed along a malignance spectrum, expressing a wide variety of characteristics. This study aimed to characterize a recent series of patients followed in a Pediatric Oncology Centre. Patients diagnosed with NT between the 1st of January 2010 and 31st of December 2020 were selected and evaluated regarding variables such as: demographic data, tumor characteristics, staging, and mortality. Each variable was analyzed considering the outcome and associations with predictive variables. Of 42 patients, 4 were excluded, as their date of diagnosis was outside of this study period. The median age at diagnosis was 23 [3,25 ; 47,5] months, gender ratio (F:M) was 1,24:1 and the most common clinic presentation was the abdominal mass (28,9%). Abdominal location, stage M, very low risk, and NB were the main characteristics of this population. Age was significantly different among histology types, with a lower age being found in NB (p=0,001). Abdominal location was more associated with NB and stage M of NT (p=0,068 and p=0,041, respectively). Only NB showed disseminated disease at the age of diagnosis (p=0,003). Mean global survival time was 100, 1 ± 8,9 months, as tumors with MYCN amplification presented with lower age at diagnosis. This genetic abnormality as well as others abnormalities studied in this population influenced significantly the outcome (p=0,043 and 0,035, respectively). The wide heterogeneity of clinical presentation, histologic type and location was confirmed. NB and abdominal presentation were predominant. The outcome appears to be mainly influenced by MYCN amplification and presence of other genetic abnormalities.