Optic pathway and hypothalamic/chiasmatic gliomas in children younger than age 5 years with a 6-year follow-up

Cancer. 1995 Feb 15;75(4):1051-9. doi: 10.1002/1097-0142(19950215)75:4<1051::aid-cncr2820750423>3.0.co;2-s.


Background: Gliomas of the hypothalamus and optic pathways (H/OPG) comprise 5% of pediatric intracranial tumors, present most frequently in patients younger than age 5 years, and may have a more aggressive course in younger children. This study examined clinical characteristics and consequences of treatment of young children diagnosed with H/OPG:

Methods: The authors reviewed the course, treatment, and outcomes of 46 children diagnosed with H/OPG younger than age 5 years; the median follow-up was 72 months. The median age at diagnosis was 27 months.

Results: Fifteen (33%) of 46 patients had neurofibromatosis-1 (NF-1). Forty children (87%) had tumor progression in the follow-up period, and tumor 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 required 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. Endocrinopathy did not correlate with therapy. Ten of 17 children evaluated by questionnaire required special education. There was a trend for educational problems to occur in children who were irradiated before the age of 5 years.

Conclusions: Gliomas of the hypothalamus and optic pathways and their treatment cause long term morbidity in young children. Chemotherapy postpones radiation effectively, and this delay may reduce neurologic morbidity; however, 60% of children eventually relapse. By contrast, patients with NF-1 have indolent disease.

MeSH terms

  • Child Behavior Disorders / etiology
  • Child, Preschool
  • Combined Modality Therapy
  • Cranial Nerve Neoplasms / complications
  • Cranial Nerve Neoplasms / mortality
  • Cranial Nerve Neoplasms / therapy*
  • Disease-Free Survival
  • Endocrine System Diseases / etiology
  • Female
  • Follow-Up Studies
  • Glioma / complications
  • Glioma / mortality
  • Glioma / therapy*
  • Humans
  • Hypothalamic Neoplasms / complications
  • Hypothalamic Neoplasms / mortality
  • Hypothalamic Neoplasms / therapy*
  • Infant
  • Intelligence
  • Male
  • Optic Nerve Diseases / complications
  • Optic Nerve Diseases / mortality
  • Optic Nerve Diseases / therapy*
  • Radiotherapy / adverse effects*
  • Retrospective Studies
  • Survival Analysis
  • Treatment Outcome