Childhood optic chiasm gliomas: radiographic response following radiotherapy and long-term clinical outcome.
OBJECTIVE
In children with chiasmal gliomas, radiation therapy can arrest progressive visual and neurologic impairment. We examined the radiographic response and clinical outcomes after irradiation.


METHODS
Forty-two children (median age at diagnosis, 6.6 years) with chiasmal gliomas were managed as follows: 11 asymptomatic patients with neurofibromatosis-1 (NF-1) were observed only; 2 patients, less than 3 years old, underwent surgery and chemotherapy to delay irradiation; and 29 patients with progressive disease received radiation with or without prior surgery or chemotherapy. Time to radiographic response, long-term tumor control and late sequelae were reviewed for the 29 irradiated patients.


RESULTS
The probability of at least 50% radiographic response at 24 months after irradiation was 18.1% and increased to 38.2% by 48 months and 45.9% by 60 months. By actuarial analysis, the median time for such radiographic response was 62 months. For the 29 irradiated patients, the 10-year freedom from progression and overall survival rates were 100% and 89%, respectively (median follow-up for surviving patients, 108 months). Stabilization or improvement in vision occurred in 81% of 26 evaluable irradiated patients.


CONCLUSIONS
Notable radiographic response may be observed years after irradiation. Radiation therapy provides excellent long-term tumor control and vision preservation or improvement in the majority of patients with progressive chiasmal gliomas.
Tao
M L
ML
Joint Center for Radiation Therapy, The Children's Hospital, Harvard Medical School, Boston, MA 02115, USA.
Barnes
P D
PD
Billett
A L
AL
Leong
T
T
Shrieve
D C
DC
Scott
R M
RM
Tarbell
N J
NJ
D000293:Adolescent; D001927:Brain Diseases / Q000000981:diagnostic imaging / Q000209:etiology; D002114:Calcinosis / Q000000981:diagnostic imaging / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D003390:Cranial Nerve Neoplasms / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy; D018450:Disease Progression; D004700:Endocrine System Diseases / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D005910:Glioma / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy; D006801:Humans; D007223:Infant; D007859:Learning Disabilities / Q000209:etiology; D008279:Magnetic Resonance Imaging; D008297:Male; D009456:Neurofibromatosis 1 / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy; D009897:Optic Chiasm / Q000000981:diagnostic imaging* / Q000473:pathology; D011832:Radiation Injuries / Q000000981:diagnostic imaging / Q000209:etiology; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014786:Vision Disorders / Q000209:etiology