Sixteen children underwent 18 operations for radical resection of chiasmatic-
hypothalamic tumors. The clinical presentation correlated with age: infants under 1 year of age presented with
macrocephaly,
failure to thrive, and severe visual failure; children aged 1 to 5 years predominantly had
precocious puberty with mild visual deficits; and older children (greater than 5 years old) had slowly progressive loss of vision. All three infants had biologically aggressive
tumors in spite of low-grade histology, and died from progressive
tumor growth. Eleven of the 13 children aged 1 year or over are alive and well, without clinical or radiographic evidence of
disease progression, 4 months to 4 1/2 years following surgery. Six of these patients, with a follow-up period of 10 months to 4 1/2 years (mean 27 months), have had no adjuvant
therapy following radical surgical resection. The authors conclude that: 1) radical surgical resection of chiasmatic-
hypothalamic tumors can be performed with minimal morbidity; 2) radical resection may delay the time to
disease progression in older children and postpone the need for irradiation; 3) resection of postirradiation recurrent
tumors may provide neurological improvement and long-lasting clinical remission; and 4) chiasmatic-
hypothalamic tumors of infancy are aggressive
neoplasms that require multimodality
therapy.