The aim of the study was to delineate the clinical characteristics of visua
l pathway tumor in children without neurofibromatosis-I. The authors review
ed the charts of all patients meeting these criteria (n = 12) who were foll
owed in their center over a 13 year period. In 8 patients the disease was r
elentlessly progressive and imaging showed a chiasmatic/hypothalamic, exoph
ytic globular lesion. The remainder had a benign course with long periods o
f tumor stability; one showed some spontaneous visual improvement. The lesi
ons of the latter subgroup were multilobular with elongated posterior exten
sion into the optic tract. This differentiation, according to the imaging f
indings, may have significant therapeutic implications. In the first type,
every effort should be made to arrest the disease and decrease the size of
the lesion, whereas in the second despite decreased visual ability careful
observation in the appropriate approach. There are as yet no Known biologic
al markers to better delineate these two types of tumor behavior: