Pediatric Sinonasal Non-Ewing's Sarcoma: Case Report and Literature Review

Objective: To present a rare case of sinonasal non-Ewing’s sarcoma in a pediatric patient. Literature review of sinonasal sarcoma is provided.

Background: Tumors of the head and neck region are extremely rare in the pediatric population; only ~10% of all pediatric malignancies occur in this location. Of those tumors, even fewer (14%) are malignancies of non-epithelial or connective tissue, and can be characterized as sarcomas. Most sarcomas involving the nasopharynx or paranasal sinuses tend to be larger as compared with other types of tumors of the head and neck, and are also associated with considerable destruction of bone. Patients present with symptoms that are often nondescript and similar to a common cold. Symptoms can vary from nasal obstruction and nosebleeds to facial deformities, ocular and neurological indications in the case of large tumors with extra-sinonasal involvement. Here, we present a report of a sinonasal non-Ewing’s sarcoma in a pediatric patient who presented with epistaxis, facial pain, and nasal obstruction. The tumor was initially thought to be an antrochoanal polyp. Complete tumor excision via an endonasal endoscopic approach was performed and the patient’s outcome was monitored by frequent follow-ups.

Clinical Presentation: A 10-year-old boy with a history of progressively worsening nasal obstruction and congestion presented with unilateral epistaxis, pain in the nose and maxillary sinuses, and pressure in the left eye. Nasal endoscopy showed a large mass in the left nasal cavity extending toward the maxillary sinus and nasopharynx. Magnetic resonance imaging showed an extensive lesion in the ethmoidal sinus area of the left nasal cavity, and further endoscopic sinus surgery showed the large polypoid mass involving the maxillary sinus, middle turbinate, sphenoid sinus, and partial left frontal sinus. The left lamina papyracea was also eroded. Complete resection of the tumor was achieved with preservation of the anterior cranial base bone since the mucosa of the ethmoidal roof was not involved. Post-operatively, the patient noted remarkable improvement of nasal breathing and normal sleep, despite some minor epistaxis. Debridement was performed one month after the tumor excision to improve nasal breathing abilities. Approximately one year later, the patient developed forehead pain due to left frontal sinusitis, which did not improve with maximal medical therapy. The patient then underwent endoscopic sinus surgery for frontal sinusotomy one year after the first operation. During that procedure, samples from the anterior skull base, sphenoid sinus and mucosa covering the orbit were taken and all specimens showed no evidence of tumor. The patient’s forehead pain improved completely after this operation.

Conclusions: This is a rare care of non-Ewing’s pediatric sinonasal sarcoma fully resected through endoscopic endonasal approach. Though the patient developed left frontal sinusitis and required a sinusotomy one year after tumor excision, nasal function was eventually restored. The patient is currently free of disease, two years after the initial surgery.