from Section 8 - Pediatrics
Published online by Cambridge University Press: 05 March 2013
Introduction
Childhood brain tumors are the second most frequent malignancy of childhood, exceeded only by leukemia, and the most common form of solid tumor. There are approximately 2500 new diagnoses per year in the USA and the incidence of brain tumors has increased slightly over the decades, possibly as a result of improved diagnostic imaging. Brain tumors comprise 20–25% of all malignancies occurring among children under 15 years of age and 10% of tumors occurring among those aged 15–19 years. Brain tumors are the leading cause of death from cancer in pediatric oncology. In addition, either because of the effects of the tumor or because of the treatment required to control it, survivors of childhood brain tumors often have severe neurological, neurocognitive, and psychosocial sequelae. Among those with brain tumors, approximately 35% are younger than 5 years and 75% are younger than 10 years. The type of tumor, the overall incidence of brain tumors, and the risks for poor outcome change with the age. Young children are at the highest risk since tumors tend to be more malignant in their behavior in this age group. Childhood brain tumors display a high pathological heterogeneity. Whereas most brain tumors in adults are gliomas (~70% malignant anaplastic astrocytoma and glioblastoma), a significant portion of pediatric brain tumors are primitive neuroectodermal tumors such as medulloblastoma, pilocytic astrocytomas, ependymomas, and others (Tables 50.1 and 50.2). Also, the behavior of pediatric brain tumors ranges from relatively indolent growth to rapid growth and a tendency to disseminate. Genetic risk factors for brain tumors include neurofibromatosis types 1 and 2 (pilocytic astrocytoma, low-grade gliomas, ependymoma), Turcot syndrome (medulloblastoma and high-grade glioma), Li–Fraumeni syndrome, Gorlin syndrome, and von Hippel–Lindau syndrome (hemangioblastoma). The only confirmed environmental risk factor is previous exposure to ionizing radiation.
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