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Key Features

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Essentials of Diagnosis
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  • The classic triad of morning headache, vomiting, and papilledema is present in less than 30% of children at presentation

  • School failure and personality changes are common in older children

  • Irritability, failure to thrive, and delayed development are common in very young children

  • Recent-onset head tilt can result from a posterior fossa tumor

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General Considerations
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  • Account for 1500–2000 new malignancies in children each year in the United States and for 25–30% of all childhood cancers

  • Brain tumors in childhood are biologically and histologically heterogeneous, ranging from low-grade localized lesions to high-grade tumors with neuraxis dissemination

  • The risk of developing a brain tumor is increased in children who received cranial irradiation for treatment of meningeal leukemia

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Classification
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  • About 50% of the common pediatric brain tumors occur above the tentorium and 50% in the posterior fossa

  • In the very young child, posterior fossa tumors are more common

  • Most childhood brain tumors can be divided into two categories according to the cell of origin

    • Glial tumors, such as astrocytomas and ependymomas

    • Nonglial tumors, such as medulloblastoma and other primitive neuroectodermal tumors

  • Some tumors contain both glial and neural elements (eg, ganglioglioma)

  • A group of less common CNS tumors does not fit into either category (ie, craniopharyngiomas, AT/RTs, germ cell tumors, choroid plexus tumors, and meningiomas)

  • Astrocytoma

    • Most common brain tumor of childhood

    • Most are juvenile pilocytic astrocytoma found in the posterior fossa with a bland cellular morphology and few or no mitotic figures

    • Low-grade astrocytomas are in many cases curable by complete surgical excision alone

  • Medulloblastoma and related primitive neuroectodermal tumors

    • Most common high-grade brain tumors

    • Usually occur in the first decade of life, with a peak incidence between ages 5 and 10 years and a female-male ratio of 2.1:1.3

    • Tumors typically arise in the midline cerebellar vermis, with variable extension into the fourth ventricle

  • Brainstem tumors

    • Third in frequency of occurrence

    • Often of astrocytic origin and are high grade

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Clinical Findings

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Symptoms and Signs
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  • Clinical findings at presentation vary depending on the child's age and the tumor's location

  • Children younger than 2 years more commonly have infratentorial tumors

    • Presenting symptoms usually nonspecific and include vomiting, unsteadiness, lethargy, and irritability

    • Signs may be surprisingly few or may include macrocephaly, ataxia, hyperreflexia, and cranial nerve palsies

    • Papilledema is often absent

    • Measuring head circumference and observing gait are essential in evaluating a child for possible brain tumor

    • Eye findings and apparent visual disturbances such as difficulty tracking can occur in association with optic pathway tumors such as optic glioma

  • Older children more commonly have supratentorial tumors

    • Associated with headache, visual symptoms, seizures, and focal neurologic deficits

    • Initial presenting features are often nonspecific

    • School failure and personality changes are common

    • Vaguely described visual disturbance is often present, but the child must be directly asked

  • Older children ...

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