Staging and survival for sinus cancer in the pediatric population.

OBJECTIVE To determine histology, staging, and survival for pediatric sinus cancer. METHODS Cases of pediatric sinus cancer (maxillary, ethmoid, and frontal/sphenoid) were extracted from the Surveillance, Epidemiology, and End Results database (1988-2005). Demographic data were tabulated and non-lymphoid malignancies were staged according to the TNM system. Kaplan-Meier survival analyses were conducted for the cohort and individual histologies. RESULTS 63 pediatric sinus cancers were identified (38 maxillary sinus, 19 ethmoid sinus, and 6 frontal/sphenoid sinus) with a mean age at diagnosis of 10.5 years and a 1:1 male to female ratio. After exclusion of 11 lymphomas (17.5%), rhabdomyosarcoma was the most commonly encountered malignancy (52.0%) followed by sarcoma (17.3%) and olfactory neuroblastoma (13.5%). 41.4% and 42.1% of maxillary sinus and ethmoid sinus tumors presented as T4 tumors, respectively. At 47 months of follow-up, cumulative survival was 54.1% with a maximum follow-up of 196 months. Median survival for pediatric sinus rhabdomyosarcoma cases was estimated at 33.0 months. CONCLUSIONS Pediatric sinus malignancy commonly presents with advanced stage, and rhabdomyosarcoma is the most commonly encountered histology. Due to the rarity of pediatric sinus cancer, survival estimates are difficult to obtain. The SEER database provides large population-based data, although limitations of such a data set impact the analysis.