Optic pathway and hypothalamic/chiasmatic gliomas in children younger than age 5 years with a 6-year follow-up.
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.
Janss
A J
AJ
Division of Neuro-Oncology, Children's Hospital of Philadelphia, PA 19104.
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
D002653:Child Behavior Disorders / Q000209:etiology; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003390:Cranial Nerve Neoplasms / Q000150:complications / Q000401:mortality / Q000628:therapy*; D018572:Disease-Free Survival; D004700:Endocrine System Diseases / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D005910:Glioma / Q000150:complications / Q000401:mortality / Q000628:therapy*; D006801:Humans; D007029:Hypothalamic Neoplasms / Q000150:complications / Q000401:mortality / Q000628:therapy*; D007223:Infant; D007360:Intelligence; D008297:Male; D009901:Optic Nerve Diseases / Q000150:complications / Q000401:mortality / Q000628:therapy*; D011878:Radiotherapy / Q000009:adverse effects*; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome