Congenital and Childhood CNS Tumors
Epidemiology
Primary CNS tumors: 20% of all childhood cancers, second only to leukemia. Incidence 2.8 per 100,000 under 15 years. Relative frequency: see Table 60.1.
Unlike adults, CNS metastasis from solid tumors rare in children.
Most tumors sporadic. Genetic neurocutaneous syndromes include neurofibromatosis (types 1, 2; see Chapter 100), tuberous sclerosis (see Chapter 103), epidermal nevus syndrome, von Hippel-Lindau disease (see Chapter 61).
Symptoms and Signs
Increased intracranial pressure: headache, vomiting, diplopia. Also fatigue, personality change, worsening school performance. Onset may be gradual.
Infants: nonspecific symptoms, including irritability, anorexia, persistent vomiting, developmental delay or regression, macrocephaly, downward deviation of eyes (“sunsetting”).
Table 60.1 Common Childhood CNS Tumors | ||||||||||||||
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Childhood CNS tumors equally frequently supratentorial and infratentorial (Table 60.2). Supratentorial tumors: headache, limb weakness, sensory loss, occasionally seizures, deteriorating school performance, personality change. Infratentorial tumors: headache, vomiting, diplopia, imbalance.
Congenital Tumors
Craniopharyngioma
Originates from remnants of embryonic tissue in Rathke pouch. Vary from small, well-circumscribed solid nodules to large multilocular cysts invading sella turcica. Histologically benign but extensive local invasion may make total surgical removal difficult.
Clinical features: Short stature, hypothyroidism, diabetes insipidus, visual loss, signs of increased intracranial pressure.
Treatment options: (a) cyst drainage, resection of nonadherent tumor, localized radiation therapy (main side effect: cognitive loss in younger children); (b) gross total resection without irradiation (main side effect: lifelong panhypopituitarism). (c) stereotactic radiosurgery (“gamma knife”) for small tumors.
Recurrence rates: 20% to 40%.
Table 60.2 Location of Central Nervous System Tumors in Infants, Children, Adolescents | ||||||||||||
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