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

Full Abstract

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.

Author information

Author/s: Janss, A J (AJ); Grundy, R (R); Cnaan, A (A); Savino, P J (PJ); Packer, R J (RJ); Zackai, E H (EH); Goldwein, J W (JW); Sutton, L N (LN); Radcliffe, J (J); Molloy, P T (PT);

Affiliation: Division of Neuro-Oncology, Children's Hospital of Philadelphia, PA 19104.

Journal and publication information

Publication Type: Journal Article

Journal: Cancer (Cancer), published in UNITED STATES. (Language: eng)

Reference: 1995-Feb; vol 75 (issue 4) : pp 1051-9

Dates: Created 1995/03/06; Completed 1995/03/06; Revised 2004/11/17;

PMID: 7842408, status: MEDLINE (last retrieval date: 2/18/2009, IMS Date: )

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