An unusual case of rhabdomyosarcoma presenting as orbital apex syndrome.
CONCLUSIONS
A 12-year-old female presented with symptoms and signs of orbital apex syndrome (OAS), secondary to stage IV alveolar rhabdomyosarcoma (RMS) originating in the sphenoid and ethmoid sinuses.


OBJECTIVE
To present a case of alveolar rhabdomyosarcoma, unusual in its presentation as orbital apex syndrome and also its origin from the sphenoid and ethmoid sinuses.


METHODS
: Observational case report.


METHODS
Ophthalmologic findings, neuroimaging, medical and surgical intervention, histopathologic analysis, and clinical course are described.


RESULTS
A 12-year-old female presented with progressive visual loss in her left eye, difficulty with eye movements, and mild headache. Her examination was consistent with orbital apex syndrome. Imaging with contrast revealed a mass originating in the left sphenoid and ethmoid sinuses invading the left optic canal. Emergent biopsy was interpreted as alveolar rhabdomyosarcoma; subsequent metastatic work-up revealed bone marrow metastases. The patient was diagnosed with stage IV alveolar rhabdomyosarcoma and immediately started on combination orbital radiation therapy (RT) and systemic chemotherapy. She experienced gradual improvement of ocular motility, though her optic neuropathy persisted.


CONCLUSIONS
Alveolar rhabdomyosarcoma of paranasal origin, specifically from the sphenoid and ethmoid sinuses, should be included in the differential diagnosis for orbital apex syndrome in children.
Machleder
Daniel J
DJ
Department of Otolaryngology-Head and Neck Surgery, Children's Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, NY, USA.
Banik
Rudrani
R
Rosenberg
Rhonda B
RB
Parikh
Sanjay R
SR
D001763:Blepharoptosis / Q000209:etiology; D002648:Child; D005260:Female; D006261:Headache / Q000209:etiology; D006801:Humans; D009918:Orbital Neoplasms / Q000175:diagnosis* / Q000556:secondary*; D010255:Paranasal Sinus Neoplasms / Q000175:diagnosis*; D018232:Rhabdomyosarcoma, Alveolar / Q000175:diagnosis*; D013577:Syndrome; D014786:Vision Disorders / Q000209:etiology