Clinical epidemiology for childhood primary central nervous system tumors

Abstract

This work was conducted by the French Brain Tumor Data Bank (FBTDB) and aims to prospectively record all primary central nervous system tumors (PCNST), in France, for which histological diagnosis is available. Results concerning children are presented. This study analyzes the childhood cases (0-19 years) of newly diagnosed and histologically confirmed PCNST (during the years 2004-2006) which have been recorded by the FBTDB. All French neuropathology and neurosurgery departments participated in this program. Neurosurgeons and neuropathologists completed a data file containing socio-demographic, clinical, radiologic and anatomopathologic information. The Tumor Registry from Herault was authorized to compile the data files with personal identifiers. About 1,017 cases (533 boys and 484 girls) of newly diagnosed childhood PCNST have been recorded (gliomas: 52%, all other neuroepithelial tumors: 31%, craniopharyngioma: 5%, germ cell tumors, meningioma and neurinoma: approximately 3% each, all histological subtypes have been detailed). Tumor resections were performed in 83.3%, and biopsies in 16.7%. The distributions by histology, cryopreservation of the samples, age, sex, tumor site and surgery have been detailed. To our knowledge, this work is the first databank in Europe dedicated to PCNST that includes the collection of clinical, radiological and histological data (including cryopreservation of the specimen). The long term goals of the FBTDB are to create a national registry and a network to perform epidemiological studies, to implement clinical and basic research protocols, and to evaluate and harmonize the healthcare of children and adult patients affected by PCNST.

Oncology Referral Concerning Paediatric Neurosurgery? Analysis of the Situation between 2008-2013 in the North of Portugal

“…1,4 The supratentorial tumours are more common in older children and in girls; the infratentorial in the youngest and in boys, with statistically significant difference, as we reported.…”

“…1 In north of our country, the incidence rate ranged from 3.33 (between 2005-2007) to 3.31 per 100 000 children (between 2008-2009). This data was kindly provided by RORENO (Regional Oncology Reports of North) and is in agreement with that observed in all Europe (2.99 per 100 000 children under 15 years old), and slightly lower than that reported in United States of America (4.61 per 100 000 children under 15 years old, and 4.53 for children under 20 years old).…”

“…Studies based on PCNST epidemiology report that the incidence is increasing probably because of the improved diagnostic accuracy, better reporting and improved health care accessibility 1,2 and it varies between countries, child age, type and location of the mass. 1 This group of heterogeneous neoplasia has a large prognosis variation, concerning the different histology subtypes.…”

“…1 This group of heterogeneous neoplasia has a large prognosis variation, concerning the different histology subtypes. Thus, it is of great importance improving diagnosis strategies and driving treatments.…”

Incidence of atypical teratoid/rhabdoid tumors in children

“…[33][34][35] A similar prevalence of 1.1% was found in a multicenter, hospitalbased study of pediatric brain tumors in France. 38 However, definite conclusions on the population-based incidence can be provided only by cancer registries, in particular specialized brain tumor registries such as the Central Brain Tumor Registry of the United States 39 or projects such as Automated Childhood Cancer Information System, a European project on the incidence and survival of pediatric tumors. 1,40,41 However, data published by these organizations are mostly presented as summary rates for groups of tumors (eg, embryonal tumors or gliomas); exact incidence rates of rare tumor entities (including atypical teratoid/rhabdoid tumors) are not provided.…”