Infantile myofibromatosis with orbital involvement: a case presentation and review of the literature

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.

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Publication Details

Journal
Orbit
Published
2026-01-13
DOI
https://doi.org/10.1080/01676830.2025.2599281
Primary Topic
Soft tissue tumor case studies
Type
article
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article

Infantile myofibromatosis with orbital involvement: a case presentation and review of the literature

Kirsten Simmons, H. Demirci, Sarinee Juntipwong, VM Elner
Orbit
Soft tissue tumor case studies
article

Infantile myofibromatosis with orbital involvement: a case presentation and review of the literature

Kirsten Simmons, H. Demirci, Sarinee Juntipwong, VM Elner
article en

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.

OrbitVol. 45(2)
University of Michigan (US)
Good health and well-being
Openalex Percentile: Top 90%
Soft tissue tumor case studies
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Infantile myofibromatosis with orbital involvement: a case presentation and review of the literature — Kirsten Simmons, H. Demirci, et al. · Orbit (2026) | TGRS Research Map | TGRS