A 3-year-old boy presented with uniocular proptosis and ophthalmoplegi
a. Investigation revealed a mass involving the right orbit with extens
ion into the left orbit and paranasal sinuses, and intracranial extens
ion involving both frontal lobes. Biopsy of an enlarged cervical node
and the intranasal mass revealed esthesioneuroblastoma. This tumor has
been reported rarely in a child this age, and only 12 case reports do
cument patients under 10 years of age. The presentation as an orbital
mass is previously unreported and must now be considered in the differ
ential diagnosis of proptosis in childhood. The usual clinical, radiol
ogical, and pathological features of olfactory esthesioneuroblastoma a
re reviewed. (C) 1994 Wiley-Liss, Inc.