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Pediatric sinonasal malignancies: A population-based analysis
Sei Y Chung1, Aykut A Unsal2, Suat Kılıç1
1Department of Otolaryngology - Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, NJ, USA.
Insights
Pediatric sinonasal malignancies (PedsSNM) are rare, often aggressive tumors. Surgery alone was associated with better survival, though this may reflect less advanced disease.
Area of Science:
- Pediatric Oncology
- Epidemiology
- Cancer Research
Background:
- Pediatric sinonasal malignancies (PedsSNM) are rare cancers with poor prognoses.
- Limited epidemiological data exists for PedsSNM.
Purpose of the Study:
- To investigate the epidemiology, tumor characteristics, and survival rates of PedsSNM.
- To provide population-based data to supplement existing literature on PedsSNM.
Main Methods:
- Utilized the Surveillance, Epidemiology, and End Results (SEER) database.
- Analyzed data from 210 patients diagnosed with PedsSNM between 1973 and 2013.
- Examined incidence, tumor characteristics, and survival outcomes.
Main Results:
- Rhabdomyosarcoma was the most common histology (50.5%), and the nasal cavity was the most frequent primary site (37.1%).
- Five-, 10-, and 20-year disease-specific survival rates were 60.2%, 46.1%, and 20.6%, respectively.
- Grade IV tumors and distant disease stage were associated with significantly worse survival outcomes.
Conclusions:
- PedsSNM frequently present as high-grade tumors, commonly rhabdomyosarcoma in the nasal cavity.
- While surgery alone correlated with higher survival, this may be due to selection bias for less aggressive tumors.
- Further research is needed to optimize treatment strategies for PedsSNM.
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.
