Sinonasal embryonal rhabdomyosarcoma presenting as bilateral visual loss: Case report
A 12 year old female, presented with rapidly progressive visual loss following left hemi facial pain for 15 days duration. Anterior segment and fundus examination was normal. She was diagnosed as retro bulbar neuritis and received steroid therapy. CT scan of PNS revealed a mass limited to the nasopharyngeal area and an endoscopic biopsy of mass revealed to be rhabdomyosarcoma, embryonal type. She was enrolled with pediatric oncology group rhabdomyosarcoma, as intermediate risk group to receive chemo-radiotherapy. A repeat MRI of brain done two weeks later, showed an intracranial extension of the tumor involving the optic nerve and chiasma. The fundus examination showed bilateral optic disc pallor. The patient responded well to chemotherapy but there was no visual improvement at four weeks follow-up. This case highlights the aggressive nature of rhabdomyosarcoma and should be kept as a differential diagnosis in cases of sudden loss of vision with normal fundus in children.
Keywords: Embryonal rhabdomyosarcoma2, Optic atrophy4, Nasopharyngeal mass3, Chemotherapy1