Alveolar rhabdomyosarcoma of the sphenoid sinus mimicking optic neuritis presenting with intermittent visual loss in an adult

Abstract

A 23-year-old male with intermittent visual loss in his left eye for 4 months was originally treated as optic neuritis elsewhere. Upon presentation to our hospital (Beijing Tongren Hospital), his left eye visual acuity was reduced to hand motion at face. The left eye ocular motility was limited in upgaze, downgaze, adduction, and abduction. Computed tomography and magnetic resonance imaging revealed lesions in the sphenoid and ethmoid sinuses, orbital apex and carvernous sinus invading adjacent extraocular muscles. Endoscopic ethmo-sphenoidostomy and debulking surgery was performed and histopathology of the specimen demonstrated alveolar rhabdomyosarcoma. Despite having treatment with a combination of orbital radiation therapy and systemic chemotherapy, the patient developed generalized metastasis with cachexia and the left eye became no light perception due to optic atrophy.