Pediatric sinonasal malignancies: A population-based analysis

Abstract

Introduction: Pediatric Sinonasal Malignancies (PedsSNM) are rare and usually associated with a poor prognosis. We aim to investigate the epidemiology, tumor characteristics, and survival of PedsSNM using a population-based database to augment the scant literature on this topic.

Methods: The Surveillance, Epidemiology, and End Results database was queried for patients ≤18 years diagnosed with PedsSNM between the years of 1973 and 2013. Data on incidence, tumor characteristics, and survival were analyzed.

Results: In total, 210 patients with PedsSNM were identified. Demographically, 54.3% were female, 72.6% were white, and the mean age was 10.7 years. Overall incidence was 0.036 per 100,000 individuals between the years of 2000 and 2013. The nasal cavity was the most frequent primary site (37.1%) and rhabdomyosarcoma was the most frequent malignancy (50.5%). Five-, 10-, and 20-year disease-specific survival (DSS) rates were 60.2%, 46.1%, and 20.6%, respectively. Grade IV tumors made up the largest group (37.3%), and such tumors exhibited the worst 5-, 10-, and 20-year survival (P < 0.05). Distant disease predicted the worst 5-, 10-, and 20-year survival, followed by regional, then localized disease (P < 0.01). Patients treated with surgery alone had a higher 20-year survival (P = 0.0425). No significant differences in survival were observed between race, gender, primary site, or histology.

Conclusions: PedsSNM frequently presented as Grade IV tumors. The nasal cavity was the most common primary site and rhabdomyosarcoma was the most frequent histology. Patients receiving surgery alone had the highest survival; however, this may be a reflection of smaller, less aggressive tumors preferentially being treated with surgery alone.

Keywords: Pediatric carcinoma; Pediatric malignancies; Rhabdomyosarcoma; Sinonasal carcinoma; Sinonasal malignancies; Survival.