OPTIC PATHWAY AND HYPOTHALAMIC CHIASMATIC GLIOMAS IN CHILDREN YOUNGERTHAN AGE 5 YEARS WITH A 6-YEAR FOLLOW-UP

Background. Gliomas of the hypothalamus and optic pathways (H/OPG) com prise 5% of pediatric intracranial tumors, present most frequently in patients younger than age 5 years, and may have a more aggressive cour se in younger children. This study examined clinical characteristics a nd consequences of treatment of young children diagnosed with H/OPG. M ethods. The authors reviewed the course, treatment, and outcomes of 46 children diagnosed with H/OPG younger than age 5 years; the median fo llow-up was 72 months. The median age at diagnosis was 27 months. Resu lts. Fifteen (33%) of 46 patients had neurofibromatosis-l (NF-1). Fort y children (87%) had tumor progression in the follow-up period, and tu mor growth was less common in children with NF-1. Initial therapy was limited to surgical resection in three and radiation in five children. To postpone radiation until after the age of 5 years, initial therapy was limited to chemotherapy in 32 patients. Radiation was not require d in 9 of these patients and was postponed for 40 months (mean) in 17. Of the 46 children, 5 died of tumor progression, 4 became blind, and 20 of 34 evaluable patients had endocrine abnormalities. Endocrinopath y did not correlate with therapy. Ten of 17 children evaluated by ques tionnaire required special education. There was a trend for educationa l problems to occur in children who were irradiated before the age of 5 years. Conclusions. Gliomas of the hypothalamus and optic pathways a nd their treatment cause long term morbidity in young children. Chemot herapy postpones radiation effectively, and this delay may reduce neur ologic morbidity; however, 60% of children eventually relapse. By cont rast, patients with NF-1 have indolent disease.