Management of a pediatric patient with aggressive low-grade sarcoma of the maxillary sinus: Case report and literature review

Pediatric sinonasal tumors are characterized by histological diversity, nonspecific clinical presentations, and variable recurrence and metastatic potential. We describe a rare low-grade, undifferentiated sarcoma with bland/benign histopathology but an aggressive course in a 10-year-old boy with nasal obstruction, rhinorrhea, and left eye proptosis. Imaging revealed a large sinonasal soft tissue mass completely obstructing the left nasal cavity, without intraorbital involvement or distant metastasis. The mass stained positive for S100, vimentin, glial fibrillary acidic protein, and Ki-67 and negative for desmin and EGFR (epidermal growth factor receptor). The patient underwent neo-adjuvant chemoradiation therapy, and definitive surgical resection via a left lateral rhinotomy/maxillectomy approach. Post-treatment results were excellent, and the patient has remained disease free at one-year follow-up based on clinical, radiographic, and direct endoscopic visualization examinations with minimal morbidity or functional disability.