OBJECTIVE: To report a case of sinonasal rhabdomyosarcoma evaluated by the department of otorhinolaryngology at a hospital in Porto Alegre, Brazil. CASE REPORT: M. L. M., 19 years of age, presented at the emergency room on December 8, 2012 with blurred vision in her right eye for 15 days, progressing to amaurosis. The patient did not present sinonasal complaints or fever. Computed tomography (CT) showed an inflammatory mass occupying the right ethmoid and sphenoid sinuses and an inflammatory component in the right papyraceous lamina with compression of the optic nerve and lateral rectus muscle. Magnetic resonance imaging (MRI) revealed obliteration of the right sphenoid sinus and posterior ethmoid cells by a mass, and a sinuous course of the optic nerve without invasion of the orbit. On physical examination, the patient displayed mild proptosis and decreased ocular movements. Nasal endoscopy did not reveal pus. She was treated for complicated rhinosinusitis with an antibiotic and corticosteroid; however, her symptoms worsened. An endoscopic biopsy of the mass in the sphenoid sinus was performed and embryonal rhabdomyosarcoma was diagnosed. Chest CT showed mediastinal and breast metastases. After 1 chemotherapy cycle, she developed neutropenic fever and died of sepsis. CONCLUSION: Rhabdomyosarcoma of the paranasal sinus is an aggressive tumor with a poor prognosis. The treatment for parameningeal rhabdomyosarcoma is chemotherapy and radiotherapy. Surgery treatment can be used for initial, resectable tumors and in those who do not respond to chemotherapy. We must be aware of the existence of neoplasms among paranasal sinus diseases, since early diagnosis improves the prognosis.
