Non-orbital Sclerosing Rhabdomyosarcoma Presented With Optic Neuropathy

Sclerosing rhabdomyosarcoma presentations are widely variable and non-specific initial features. We report a rare case of non-orbital sclerosing rhabdomyosarcoma presented with optic neuropathy. A 15-year-old female patient initially presented with upper gum swelling and pain for 3 months. It was associated with loosening of teeth. Subsequently, the patient developed recurrent epistaxis follow by left facial swelling and blurring of vision. Examination showed marked left facial swelling with mild proptosis. Visual acuity in the left eye was no perception of light with the presence of relative afferent pupillary defect. Fundoscopy showed left optic atrophy. Neuroimaging showed large aggressive soft tissue mass on the left infratemporal, masticator, and parapharyngeal space with a local extension to the sphenoid sinus. There was also an intracranial extension to the left temporal lobe with the base of skull bone destruction. Transnasal endoscopic biopsy revealed sclerosing rhabdomyosarcoma. Management was with chemotherapy. Sclerosing rhabdomyosarcoma may present with optic nerve involvement that may carry a guarded prognosis to the eyes.