Abstract
Pediatric sinonasal rhabdomyosarcoma (PSR) is a rare condition, with a limited number of previously reported cases. It is an aggressive malignancy associated with poor outcomes, and no consensus has been reached on the optimal therapeutic strategy. The present study reported the case of embryonal PSR in a 2-year old girl, presenting with unilateral nasal obstruction and a polypoid mass protruding from the left nasal cavity. The pediatric patient was initially treated with surgical resection, followed by adjuvant chemotherapy containing vincristine (1.5 mg/m(2), weekly) and actinomycin-D (1.5 mg/m(2), three times weekly). On the 10th month of follow-up, tumor recurrence was detected and a salvage surgery was performed, while the same chemotherapy regimen was resumed. Following the first cycle of chemotherapy, the patient developed a fungal bronchopneumonia and succumbed due to disease progression, acute respiratory distress syndrome and septic shock 12 months after diagnosis.