Infantile myofibromatosis with orbital involvement: a case presentation and review of the literature
Kirsten L. Simmons, Sarinee Juntipwong, Victor M. Elner, Hakan Demirci
2026.1.13Orbit
tlooto Summary
A 4-year-old boy with a history of multicentric infantile myofibromatosis who presented with new-onset right-sided proptosis with potential for spontaneous regression of orbital myofibroma following limited surgical management is described.
Abstract
Infantile myofibromatosis is a rare fibrous tumor that typically presents during infancy or early childhood. We describe a 4-year-old boy with a history of multicentric infantile myofibromatosis who presented with new-onset right-sided proptosis. Magnetic resonance imaging (MRI) demonstrated an expansile intracranial lesion arising from the greater wing of the sphenoid bone, producing mass effect at the right orbital apex and compressing the optic nerve. Histopathologic examination of a biopsy specimen confirmed a myofibroma with associated reactive bone changes. Over a 36-month follow-up period, serial MRI studies showed gradual spontaneous regression of the orbital lesion. Although orbital involvement in infantile myofibromatosis is uncommon, prompt recognition and timely consideration of surgical or pharmacologic intervention are warranted. This case underscores the potential for spontaneous regression of orbital myofibroma following limited surgical management.
Citation format
SIMMONS, Kirsten L., et al. Infantile myofibromatosis with orbital involvement: A case presentation and review of the literature. Orbit, 2026, 45(2): 306–310.