Sinonasal Mucosal Melanoma: A Population-based Comparison of the EUROCARE and SEER Registries

Abstract Introduction Sinonasal melanomas are rare tumors with no comparative survival studies between Europe and the US. Objective To provide a population-based survival analysis between the two continents. Methods The European Cancer Registry (EUROCARE) and the United States Surveillance, Epidemiology, and End Results (SEER) databases were queried to identify patients diagnosed with sinonasal melanoma between 2000 and 2007. Relative survival (RS) data were grouped by age, gender, geographic region, extent of disease, and treatment modality. Results A total of 1,294 cases were identified between 2000 and 2007 (935 from EUROCARE-5 and 359 from SEER). Females were most commonly identified in Europe (56.4%) and in the US (54.9%). Patients over the age of 65 years comprised the greatest proportion of patients in Europe (70%) and in the US (71%). By region, Southern Europe had the highest 5-year RS (31.6%, 95% confidence interval [CI] = [21.3-42.5%]), and Eastern Europe the lowest (16.5%, [7.5-28.5%]). The aggregate European 5-year RS was 25.4% [21.8-29.1%] and the U.S. was (29.7%, [23.6-36%]). Conclusions Althoughincreasinginincidence, sinonasal melanomas remain rare. Women were more commonly affected. The most common age group was those older than 65 years, although age did not confer a prognostic value. The most common subsite was the nasalcavity followedbythe maxillarysinus.Five-yearRSwas similar betweencontinents withaninverse relationship between extentofdiseaseand survival. The treatment of choice throughout Europe and the US remains primarily surgical.

Sinonasal Mucosal Melanoma: A Population-based Comparison of the EUROCARE and SEER Registries.

Introduction Sinonasal melanomas are rare tumors with no comparative survival studies between Europe and the US. Objective To provide a population-based survival analysis between the two continents. Methods The European Cancer Registry (EUROCARE) and the United States Surveillance, Epidemiology, and End Results (SEER) databases were queried to identify patients diagnosed with sinonasal melanoma between 2000 and 2007. Relative survival (RS) data were grouped by age, gender, geographic region, extent of disease, and treatment modality. Results A total of 1,294 cases were identified between 2000 and 2007 (935 from EUROCARE-5 and 359 from SEER). Females were most commonly identified in Europe (56.4%) and in the US (54.9%). Patients over the age of 65 years comprised the greatest proportion of patients in Europe (70%) and in the US (71%). By region, Southern Europe had the highest 5-year RS (31.6%, 95% confidence interval [CI] = [21.3-42.5%]), and Eastern Europe the lowest (16.5%, [7.5-28.5%]). The aggregate European 5-year RS was 25.4% [21.8-29.1%] and the U.S. was (29.7%, [23.6-36%]). Conclusions Although increasing in incidence, sinonasal melanomas remain rare. Women were more commonly affected. The most common age group was those older than 65 years, although age did not confer a prognostic value. The most common subsite was the nasal cavity followed by the maxillary sinus. Five-year RS was similar between continents with an inverse relationship between extent of disease and survival. The treatment of choice throughout Europe and the US remains primarily surgical.

Systematic review of olfactory outcomes after nasoseptal flap harvest for endoscopic skull base surgery: Does using cold steel or olfactory strip preservation matter?

BACKGROUND: Several studies have described techniques aimed at mitigating olfactory dysfunction after nasoseptal flap (NSF) harvest for endoscopic skull base surgery (ESBS). No consensus exists as to whether popular methods including using cold steel (CS) versus electrocautery (EC) or septal olfactory strip (SOS) preservation offer an advantage. This systematic review was performed to examine the impact of these two technical variations of NSF harvest on postoperative olfactory outcomes. METHODS: Following PRISMA guidelines, PubMed, Scopus, and Web of Science were searched for articles reporting olfactory outcomes in ESBS cases employing an NSF. Original articles focusing on technique variations of the NSF and reporting at least one objective olfactory measure were included. RESULTS: Nine studies comprising 610 patients were included. Various, olfactory testing outcomes were reported, and postoperative follow-up ranged from 6 weeks to 12 months. Three studies, including a randomized controlled trial, compared the use of CS and EC for the superior incision of the NSF. No significant difference was found in objective olfactory function (p > 0.05) when comparing these techniques. Five studies comprising 504 patients reported results from SOS-sparing. SOS-sparing technique in NSF harvest demonstrated smell preservation in the postoperative setting when compared with preoperative measures (p > 0.05); however, no direct comparison to non-SOS-sparing techniques was made. CONCLUSION: The use of CS as opposed to EC for the superior NSF incision does not appear to confer an advantage in preserving postoperative olfactory function (grade B evidence). SOS preservation may be associated with better olfactory outcomes in NSF harvest (grade C evidence).

Comparison of Open and Endoscopic Approaches in the Resection of Esthesioneuroblastoma.

BACKGROUND: Olfactory neuroblastoma, or esthesioneuroblastoma (ENB), is an uncommon sinonasal malignancy arising from olfactory neuroepithelium that is optimally treated with surgical resection. The objective of this study is to determine preoperative predictors of undergoing open versus endoscopic approach for ENB and to investigate significant postoperative survival outcomes between the two surgical approaches. METHODS: The National Cancer Database (NCDB) was queried for cases of ENB histology that were treated surgically from 2010 to 2015. Groups were stratified into open or endoscopic approach cohorts. Patient demographics, tumor characteristics, treatment modality, and 5-year overall survival were compared between the two groups using Chi-Square analysis and Kaplan-Meier survival analysis. Cases were classified as Kadish stage A, B, C, or D based on the "Collaborative Stage-Extension" codes in NCDB. RESULTS: Of 533 patients meeting inclusion criteria, 276 (51.8%) patients underwent open, and 257 (48.2%) patients underwent endoscopic surgical approaches. Patients undergoing endoscopic surgery were more likely to be Kadish stages A and B and less likely to be stages C and D (P = .020). Those undergoing endoscopic approach overall had a shorter mean hospital stay postoperatively (3.8 vs. 7.0 days, P < .001). Endoscopic cases had a greater 5-year overall survival (81.9% vs. 75.6%, P = .030); after multivariate regression, there was a trend toward survival benefit to endoscopic surgery that did not reach clinical significance (HR 0.644, [0.392-1.058], P = .083). CONCLUSION: Although not statistically significant, there is a trend toward increased overall survival with an endoscopic approach in patients undergoing surgery for ENB as compared to an open approach, regardless of Kadish stage. An endoscopic approach is an adequate alternative to an open approach for the surgical treatment of ENB.

Clinicopathologic traits and prognostic factors associated with pediatric sinonasal rhabdomyosarcoma.

BACKGROUND: Pediatric sinonasal rhabdomyosarcoma (RMS) is an aggressive and rare malignancy. This is the first multi-institutional study on the prognostic factors associated with outcomes in this population. METHODS: The National Cancer Database was queried for the period from 2004 to 2013 for all cases of malignant sinonasal RMS in the pediatric population. The impact of patients' demographics, tumor characteristics, and Intergroup Rhabdomyosarcoma Study Group (IRSG) staging on survival was assessed using chi-square test, Fisher's exact test, Kaplan-Meier test, and Cox regression analyses. RESULTS: A total of 157 cases of pediatric sinonasal RMS were identified. Mean age at diagnosis was 9.38 years and male patients comprised 48.4% of the cohort. The nasal cavity (31.8%) and maxillary sinus (30.6%) were the most common primary sites. Alveolar was the most common histology (49.7%), followed by embryonal type (32.5%). The majority of patients received solely chemoradiation (52.9%), followed by surgery with adjuvant chemoradiation (30.6%). Five-year overall survival (OS) was 55.2% (±4.5%). Metastatic disease was associated with a poorer 5-year OS rate (24.4% vs 61.5%; p = 0.010). Maxillary sinus site was associated with an improved survival (71.8% vs 47.6%; p = 0.009). On multivariate analysis, chemoradiation with or without surgery was an additional prognostic factor. Although IRSG clinical stages did not correlate with survival, high-risk patients in the IRSG clinical risk groups were associated with poorer survival on multivariate analysis (hazard ratio [HR], 2.005; 95% confidence interval, 1.007-3.993; p = 0.048). CONCLUSION: To date, this is the largest study on pediatric sinonasal RMS. IRSG clinical risk groups may be useful in stratifying high-risk patients with poor prognosis.

Effect of elderly status on postoperative complications in patients with sinonasal cancer.

BACKGROUND: Elderly patients with multiple comorbidities may be at higher risk of postoperative complications. With an increasingly aging population, more data assessing for predisposing factors are needed in this at-risk group. In this study, we analyzed the effect of elderly status on relative comorbidities and complications of sinonasal cancer (SNC) patients receiving surgery. METHODS: A retrospective database analysis was performed using cases from the Nationwide Inpatient Sample (NIS) from 2003 to 2012. Patients with a diagnosis of malignant neoplasm of the nasal cavity or paranasal sinuses, who received surgery for sinonasal malignancy, including neck dissections, were selected. Demographics of interest included age, sex, race, type of admission, mean length of stay, and median hospital charges. Elderly and nonelderly patients were compared for differences in rates of acute medical complications, acute surgical complications, and relevant procedures during hospitalization. RESULTS: Of the 920 cases identified in the NIS, 382 (41.5%) were elderly (≥65 years). Cases of SNC were more frequently seen in males than females (p < 0.001). Elderly patients had significantly higher comorbidity rates compared with nonelderly patients, which included congestive heart failure, hypertension, diabetes, chronic pulmonary disease, and chronic renal failure (p < 0.001 for all). Elderly patients more frequently had postoperative cardiac complications (6.0% vs 0.5%, p < 0.001), but this finding was not statistically significant on multivariate analysis when controlling for race, sex, and comorbidities. CONCLUSION: Elderly status is not an independent factor for postoperative complications in patients undergoing surgery for sinonasal malignancy.

Changing trends in management of vestibular schwannoma: A National Cancer Database study.

OBJECTIVE: To describe changes in management trends of vestibular schwannoma (VS) and the effects of median income, education level, insurance status, and tumor size on receipt of treatment. METHODS: Cross-sectional analysis using the National Cancer Database from 2004 to 2014. All patients with a diagnosis of VS were identified between 2004 and 2014. Trends in treatment modality over time were analyzed. Correlation between demographics, institution type, median income, education level, and insurance status were assessed using univariate and multivariate logistic regressions. RESULTS: Of the 22,290 VS patients identified, 16,011 (71.8%) received treatment, whereas 6,279 (28.2%) received observation. Management trend analysis over the 11-year period revealed patients with tumor size < 1 cm are more frequently observed (34.6% of cases in 2004 vs. 60.8% of cases in 2014) and less frequently received surgery (34.6% vs. 16.8%). Multivariate analysis showed observation was more frequently used in patients ≥ 65 years; black patients; and those on Medicaid, Medicare, and noninsured (P < 0.0001). All tumors > 2 cm were more likely to be treated with surgery, specifically subtotal resections (P < 0.0001). Patients treated at nonacademic centers were more likely to receive linear accelerator, or LINAC (Accuray Incorporated, Sunnyvale, CA) therapy than Gamma Knife (Elekta, Stockholm, Sweden) (P < 0.0001). CONCLUSION: Management of VS is shifting toward increased observation, most significantly in tumors size < 1 cm. Insurance provider plays a significant role on receipt of treatment and modality, whereas income and education do not. LEVEL OF EVIDENCE: NA Laryngoscope, 129:1197-1205, 2019.

Radiotherapy modality as a predictor of survival in hypopharyngeal cancer.

BACKGROUND: The impact of radiotherapy (RT) modality and dose on survival in hypopharyngeal cancer managed with definitive RT is unclear. METHODS: The National Cancer Database was queried for patients with hypopharyngeal squamous cell carcinoma (HPSCC) treated with definitive RT. Intensity-modulated radiotherapy (IMRT) was compared with other RT techniques. Clinicopathologic variables, RT modality, and dose impact on overall survival (OS) were assessed using log-rank test and Cox proportional hazard models. RESULTS: A total of 3928 patients with HPSCC were identified. Patients receiving IMRT (2098 patients) were more likely to be white, have higher income, have advanced classification, receive ≥66 Gy, and receive chemoradiotherapy compared with those receiving non-IMRT techniques (1830 patients). The 5-year OS was 41.9% (95% CI = 39.4%-44.4%) for the IMRT group and 36.8% for the non-IMRT group (95% CI = 34.3%-39.2%). After propensity score matching, IMRT had significantly better OS (P = .013). CONCLUSION: In HPSCC treated with definitive RT, IMRT may provide a significant survival benefit over non-IMRT modalities.

Examining the "July effect" on patients undergoing pituitary surgery.

BACKGROUND: Our aim in this study was to assess the impact of the turnover of residents in July on patients undergoing pituitary surgery. METHODS: This work was a retrospective cohort study of cases from the National Inpatient Sample (NIS). Patients who underwent pituitary surgery from 2005 to 2012 were selected in the NIS. Patients undergoing surgery in July and in non-July months were compared to determine differences in demographics, comorbidities, and complications. RESULTS: Of the 12,939 patients, 1098 (8.5%) underwent pituitary surgery in July. Patients receiving surgery in July had similar demographics and Agency for Healthcare Research and Quality comorbidity values compared with patients receiving surgery in other months. There were no significant differences in mortality, cerebral edema, cerebrospinal fluid leakage, iatrogenic pituitary complications, iatrogenic cerebrovascular accidents, urinary tract infections, pulmonary edema, pulmonary complications, or acute cardiac complications. There were no differences in the rate of postoperative fistulas, hematomas, perforations, or infections. The use of meningeal suturing, pedicled or free-flap reconstruction, and skin reconstruction was more frequent in July. Finally, hospitalization costs in July were similar to costs in other months. CONCLUSION: The turnover of new residents in July showed no change in complication rates for patients undergoing pituitary surgery. Patient care in July is similar to care during other months, demonstrating that hospitals are adequately supervising surgical residents during this transition.

Predictors of clinical-pathologic stage discrepancy in oral cavity squamous cell carcinoma: A National Cancer Database study.

BACKGROUND: Few studies have examined the frequency and survival implications of clinicopathologic stage discrepancy in oral cavity squamous cell carcinoma (SCC). METHODS: Oral cavity SCC cases with full pathologic staging information were identified in the National Cancer Database (NCDB). Clinical and pathologic stages were compared. Multivariate logistic regressions were performed to identify factors associated with stage discrepancy. RESULTS: There were 9110 cases identified, of which 67.3% of the cases were stage concordant, 19.9% were upstaged, and 12.8% were downstaged. The N classification discordance (28.5%) was more common than T classification discordance (27.6%). In cases of T classification discordance, downstaging is more common than upstaging (15.4% vs 12.1% of cases), but in cases of N classification discordance, the reverse is true; upstaging is much more common than downstaging (20.1 vs 8.4% of cases). CONCLUSION: Clinicopathologic stage discrepancy in oral cavity SCC is a common phenomenon that is associated with a number of clinical factors and has survival implications.

Incidence and survival of sinonasal adenocarcinoma by site and histologic subtype.

OBJECTIVE: To determine the incidence and survival of sinonasal adenocarcinoma (SNAC) by subsite and histologic subtype. STUDY DESIGN: Retrospective database review. METHODS: Using the SEER database, we performed a retrospective analysis, identified cases of SNAC diagnosed between 1973 and 2013 and analyzed demographic, histopathology, clinicopathology, and determinants of disease specific survival (DSS). RESULTS: A total of 746 patients with SNAC were identified. Median age at diagnosis was 64 years. Overall incidence was 0.44 per million, and was higher among blacks (O.R.:1.10-2.07:1) and males (O.R.:1.38-2.06:1). Nasal cavity (41.5%) was the most common site, followed by maxillary (26.5%), and ethmoid (17.4%) sinuses. Intestinal-type adenocarcinoma was less likely than Adenocarcinoma not otherwise specified (ANOS) to be found in the maxillary sinus (8.8% vs. 30.6%, p < .05). Surgery alone (48.56%) was the most common treatment modality, followed by surgery and radiotherapy (RT) (32.5%), and RT alone (11.6%). DSS at 5, 10, and 20 years were 63.8%, 57.6%, and 47.0%, respectively. DSS was higher for nasal cavity SNAC, lower grade, lower stage, and those receiving surgery only. CONCLUSIONS: SNAC is more common among men and blacks. Incidence has not changed significantly in the past 40 years. Survival varies with grade, stage, histology, subsite, and treatment.

Comparison of endoscopic and open resection of sinonasal squamous cell carcinoma: a propensity score-matched analysis of 652 patients.

BACKGROUND: The use of endoscopic resection as an alternative to open surgery for sinonasal malignancies has increased in the past 20 years. METHODS: The National Cancer Database was queried for cases of sinonasal squamous cell carcinoma (SNSCC) without cervical or distant metastases that were treated surgically between 2010 and 2014. They were split into 2 groups based on surgical approach: open or endoscopic. Demographics, facility and insurance type, stage, tumor characteristics, postoperative treatment, 30-day readmission rate, 30- and 90-day mortality, and overall survival (OS) were compared between the 2 groups. Cox proportional hazard analysis was performed. Propensity score matching (PSM) was used to mimic a randomized, controlled trial. RESULTS: A total of 1,483 patients were identified: 353 (23.8%) received endoscopic and 1130 (76.2%) received open surgery. Age, gender, race, geographic region, tumor size, surgical margins, postoperative chemoradiation, and 30-day readmissions did not vary significantly between the 2 groups. Open surgery was more common in academic centers (62.8% vs 54.2%; p = 0.004), less common for tumors of the ethmoid and sphenoid sinus (p < 0.0001), less common for stage IVB tumors, and associated with longer hospital stay (mean, 4.67 days vs 2.50 days; p < 0.0001). Five-year OS (5Y-OS) was not significantly different between the 2 approaches (p = 0.953; open: 5Y-OS, 56.5%; 95% confidence interval, 51.3% to 61.6%; endoscopic: 5Y-OS, 46.0%; 95% confidence interval, 33.2% to 58.8%). In the PSM cohort of 652 patients, there was also no significant difference in OS (p = 0.850). CONCLUSION: Endoscopic surgery is an effective alternative to open surgery, even after accounting for confounding factors that may favor its use over the open approach. It is also associated with a shorter hospital stay.

A population-based comparison of European and North American sinonasal cancer survival.

OBJECTIVE: Sinonasal cancers (SNC) are rare, thus limiting previous prognostic studies on a multinational level. The aim of this study is to utilize two population-based datasets to compare prognoses for SNC between the United States (US) and Europe. METHODS: The European Cancer Registry (EUROCARE) database and the United States National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) database were searched to identify survival of patients diagnosed with SNC between 1990 and 2007. Relative survival (RS) data were stratified by age group, gender, geographic location, and diagnostic time period. RESULTS: 12,541 SNC cases were identified in EUROCARE, while SEER identified 4,312. Males comprised the majority in Europe (62.3%) and the US (58.3%). Most patients were over 55 years in Europe (77.0%) and the US (69.5%). Age over 75 was a statistically significant poor prognostic indicator for 5-year RS in the US (48.2%; 95% CI=[43.9, 52.4]) and Europe (38.5%; [34.7, 42.7]). Female gender imparted a favorable 5-year RS in all regions except in Central Europe. By region, the US had the highest 5-year RS (58.8%; [56.4, 61.1]) and Eastern Europe had the lowest 5-year RS (37.1%; [34.0, 40.6]). The aggregate European 5-year RS was 48.1% [46.4, 49.8]. CONCLUSION: SNC in Europe and the US most commonly affects males and individuals over the age of 55 years. Male gender and age over 75 are poor prognostic factors at 5 years. Five-year RS in the US is higher than the 5-year European aggregate RS.

Updates in the management of sinonasal mucosal melanoma.

PURPOSE OF REVIEW: Sinonasal mucosal melanoma (SNMM) is an aggressive cancer with a poor prognosis. Although there is significant study surrounding the treatment of sinonasal malignancies and cutaneous melanomas, the rarity of this tumor has largely precluded robust outcomes analyses. The authors of this review seek to provide an overview of the recent literature related to the treatment of SNMM with added context from our institutional experience with this disease. RECENT FINDINGS: In the surgical management of sinonasal malignancies and SNMM specifically, resection via endoscopic endonasal technique appears to offer comparable oncologic outcomes versus an open approach. The role of adjuvant therapy continues to be debated, but there is strong evidence for improved rates of local control with radiotherapy after complete resection. In the last few years, significant developments have been made in the study of systemic therapies for cutaneous melanoma. The identification of genetic mutations common to mucosal melanoma has allowed for early trials of targeted therapies, but study is ongoing. SUMMARY: Although the study of SNMM is largely limited to small retrospective case series, treatment continues to evolve. Until effective systemic therapies can be identified, endoscopic resection with adjuvant radiotherapy may offer the best disease-free survival with acceptably low morbidity.

Predictors of Clinicopathologic Stage Discrepancy in Oropharyngeal Squamous Cell Carcinoma: A National Cancer Database Study.

Objective To determine the frequency, associated factors, and prognosis of clinicopathologic stage discrepancy in oropharyngeal squamous cell carcinoma (OPSCC). Study Design Retrospective study using a national database. Setting National Cancer Database. Subjects and Methods Cases of OPSCC diagnosed between January 1, 2004, and December 31, 2013, with full clinical and pathologic staging information available were identified. Demographic, clinicopathologic, and treatment variables associated with overall stage discrepancy were identified by multivariate logistic regression analysis. Results In total, 7731 cases of OPSCC were identified. Overall stage discrepancy was present in 30.2% of cases (21.9% upstaging, 8.2% downstaging). A total of 13.1% of cases were T-upstaged, and 10.5% of cases were T-downstaged; 22.9% of cases were N-upstaged, and 8.6% of cases were N-downstaged. Upstaging by overall stage was associated with a high Charlson-Deyo score, high tumor grade, number of lymph nodes examined, and increasing tumor size. No factors were positively associated with downstaging. High tumor grade was negatively associated with downstaging. For stage II, III, and IVA tumors, upstaging was associated with poorer OS. Conclusion Clinicopathologic stage discrepancy is common in OPSCC and is likely attributable to insensitive clinical staging techniques as well as to intrinsic tumor biologic properties. Upstaging is associated with poorer prognosis, which is likely due to advancement of disease.

Significance of human papillomavirus positivity in sinonasal squamous cell carcinoma.

BACKGROUND: The role of human papillomavirus (HPV) in sinonasal squamous cell carcinoma (SNSCC) is not well understood. METHODS: The National Cancer Database was queried for cases of SNSCC with known HPV status. Demographics, socioeconomic variables, TNM stage, histology, grade, treatment modalities, and overall survival (OS) through 5 years were compared between HPV-positive and HPV-negative tumors. Cox proportional hazard regression analyses were performed. RESULTS: Seven hundred seventy (770) cases were identified; 526 were HPV-negative (68.3%) and 244 (31.7%) were HPV-positive. Patients with HPV-positive tumors were younger (58.0 vs 63.7 years, p < 0.0001). Nasal cavity (49.4%) tumors were more likely to be HPV-positive (p < 0.05) than maxillary (18.8%), ethmoid (18.8%), and frontal (18.2%) sinus tumors. Large cell nonkeratinizing (42.4%), papillary (42.1%), and basaloid (56.5%) tumors were more likely than keratinizing (25.2%) tumors to be HPV-positive (p < 0.05). Well-differentiated (grade I) tumors (9.0%) were less likely than higher grade tumors to be HPV-positive (p < 0.05). Gender, race, facility type, insurance type, median income, education level, Charlson-Deyo comorbidity score, overall stage, T stage, N stage, M stage, tumor size, treatment modality, surgical approach, and surgical margins did not vary by HPV status (p ≥ 0.05). HPV-positive tumors had higher OS than HPV-negative tumors (p < 0.0001). At 5 years, OS was 68.1% and 51.5% for HPV-positive and HPV-negative tumors, respectively. On multivariate analyses, HPV positivity remained a favorable prognostic factor (hazard ratio, 0.49; 95% confidence interval, 0.34-0.70). CONCLUSION: HPV positivity is more common in nasal cavity SCC and nonkeratinizing SNSCC. It is also a favorable prognostic factor in SNSCC. Future studies on SNSCC should take HPV positivity into consideration.

Pediatric sinonasal malignancies: A population-based analysis.

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.