Ewing Sarcoma of the Sinonasal Tract: A Scoping Review.

Abstract

Objective: Ewing sarcoma (ES) is a rare aggressive malignancy that can present in the sinonasal region. The objective of this study is to investigate the demographics, presentation, management, and outcomes of patients with sinonasal ES.

Data sources: PubMed, Web of Science, SCOPUS, CINAHL, and Cochrane Library.

Review methods: A scoping review of cases of sinonasal ES was performed. Inclusion criteria consisted of case reports, series, or retrospective reviews.

Results: 785 total articles were retrieved. 72 articles met inclusion criteria and were included in the final review for a total of 93 cases. 48 (53%) patients were male. Mean age at diagnosis was 26.4 years old (range 1-89). Nasal obstruction (N = 55, 59%), epistaxis (N = 35, 38%), and impaired vision (N = 29, 29%) were the most common symptoms. On examination, 38 (41%) patients had a nasal cavity mass. Most tumors (N = 33, 35%) were located in the maxillary sinus. 44 (47%) were left sided and 4 (4%) were bilateral. The most utilized treatment modalities were surgical resection with adjuvant chemoradiotherapy (N = 27, 29%), chemoradiotherapy alone (N = 24, 26%), and surgical resection with adjuvant chemotherapy (N = 14, 15%). 56 patients had no evidence of disease (60%), 14 patients died with disease (15%), and 9 patients were alive with disease (10%) at the time of follow-up.

Conclusions: To our knowledge, we report the first scoping review of sinonasal ES. Patients generally present with non-specific sinonasal symptoms. Surgery with adjuvant chemoradiation is the most common treatment modality for these patients.

Level of evidence: NA Laryngoscope, 2024.