Postgraduate Program, Research Center, Hospital Cancer Foundation, Tumor registry, Rio de Janeiro, Brazil.
Hospital Cancer Foundation, Tumor registry, Rio de Janeiro, Brazil.
Cancer. 2019 Aug 1;125(15):2638-2646. doi: 10.1002/cncr.32121. Epub 2019 Apr 23.
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.
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.
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.
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.
罕见儿童癌症的定义颇具挑战。意大利儿科罕见肿瘤（TREP）研究将罕见肿瘤定义为每年发病率<2 例/100 万且未纳入临床试验的实体恶性肿瘤。本研究旨在分析巴西儿童和青少年（出生至 19 岁）罕见肿瘤的人群发病率。
发病率数据来自覆盖巴西 5 个地理区域的 19 个基于人群的癌症登记处。根据 TREP 定义，使用国际肿瘤学疾病分类，选择新诊断病例。为计算粗发病率，将特定罕见癌症的发病儿童和青少年人数除以同期 20 岁以下人群的相应人年数。
只有青少年期的两种肿瘤（甲状腺癌和皮肤癌）的发病率>2 例/100 万。巴西各地区的几种肿瘤发病率存在差异。在南部地区，<10 岁儿童的肾上腺皮质癌发病率较高（4 例/100 万）。甲状腺癌和皮肤癌在中西部、东南部和南部地区的发病率较高。
由于这些事件极为罕见，因此建立网络对于改善基础研究、临床研究和临床试验非常重要。诊断的集中化是改善受这些罕见疾病影响的儿童的诊断和治疗的唯一途径。从公共卫生的角度来看，罕见儿科癌症的登记和监测至关重要，因此必须提高登记的质量。
