Rare cancers in childhood and adolescence in Brazil: First report of data from 19 population‐based cancer registries

Abstract

Background Rare childhood cancer is challenging to define. The Italian Pediatric Rare Tumor (TREP) Study considers rare tumors to include solid malignancies characterized by an annual incidence rate of <2 cases per 1 million and not enrolled in clinical trials. The objective of the current study was to analyze the population incidence rate of rare tumors among children and adolescents (those aged birth‐19 years) in Brazil. Methods Incidence data were obtained from 19 population‐based cancer registries covering the 5 geographic regions in Brazil. Newly diagnosed cases were selected according to the TREP definition, using the International Classification of Diseases for Oncology. To calculate the crude incidence rate, the numbers of incident children and adolescents with a specific rare cancer were divided by the corresponding person‐years lived for the population aged <20 years during the same period. Results Two tumors had an incidence rate that was >2 cases per 1 million (thyroid and skin cancers) in adolescents only. Several tumors demonstrated variations in incidence across the Brazilian regions. Adrenocortical carcinoma had a high incidence rate (4 cases per 1 million) in the south region among children aged <10 years. Thyroid and skin carcinoma had higher incidence rates in the midwest, southeast, and south regions. Conclusions Due to the extraordinary rarity of these events, networking is important for improving basic research, clinical studies, and trials. Centralization of diagnosis is the only way to improve the diagnosis and treatment of children affected by these rare diseases. The registration and surveillance of rare pediatric cancers are crucial from a public health point of view, and therefore the quality of registration has to be improved.

Thyroid carcinoma in children, adolescents, and young adults in Brazil: A report from 11 population-based cancer registries

“…As shown in a previous report, the incidence of thyroid carcinomas was higher for all ages in the Midwest, Southeast, and South regions of Brazil, with the highest percentages observed in white residents [1,40]. Unfortunately, we do not have reliable data among the PBCRs regarding race/skin color.…”

“…Thyroid cancer in children and adolescents is one of the most common rare tumors, as defined by TREP in Brazil [1]. According to a recent cancer incidence in five continents (CI5C) monograph, Brazil has the highest incidence rates within South and Central America [2].…”

“…A recent analysis of rare tumors in children and adolescents (0-19 years old) in Brazil showed different incidence rates of thyroid carcinomas in Brazilian geographic regions varying from 1.87 per million in the North region to 6.48 per million in the Southeast region. Incidence rates increase with age and were found to be the highest among adolescents (15)(16)(17)(18)(19) years old) [1], Incidence rates in Latin American in adolescents and young adults (AYAs) are the most common cancer in females was thyroid cancer, with rates ranging from 0.9 to 10.0/ 100,000 [7]. It is well described that the increase is attributable to an increase in incidence of the histologic subtype papillary thyroid cancer.…”

Development of paediatric non-stage prognosticator guidelines for population-based cancer registries and updates to the 2014 Toronto Paediatric Cancer Stage Guidelines

“…However some very rare cancers may be particularly prevalent in certain jurisdictions, such as adrenocortical carcinomas in Southern Brazil. 23 In these contexts, registries may choose to collect locally endorsed NSPs for these cancers.…”

Adrenocortical tumours in children: a review of surgical management at a tertiary care centre

“…The high incidence in Brazil is attributed to TP53 mutation. Most ACTs occur sporadically but they may be associated with hereditary cancer syndromes such as Li Fraumeni and Beckwith Wiedemann syndrome 1–6 …”