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Objective: Esthesioneuroblastoma is an uncommon malignancy of the sinonasal tract arising from the olfactory epithelium. Surgical management of the primary site, often via an endoscopic approach, with or without adjuvant radiation, is often curative. There is growing but ultimately limited data regarding management of the neck and the risk of nodal metastases. In this study, we examine the incidence and patterns of esthesioneuroblastoma-related cervical nodal metastases using the Surveillance, Epidemiology, and End Results (SEER) database.Methods: The SEER registry was queried for all patients with esthesioneuroblastomas diagnosed between 1973 and 2012. Patient data was then analyzed with respect to age, sex, race, modified Kadish stage, grade, survival functions, and nodal disease including specific nodal basins.Results: Three hundred and eighty-one cases of esthesioneuroblastoma with information on nodal metastases were identified. The overall cervical nodal metastasis rate was 8.7%. Level II metastases were most common (6.6%). A total of 4.5% of cases presented with multiple positive nodal basins. Male sex (P = 0.009) and higher tumor grade (P = 0.009) correlated with the presence of level II metastases. There was no association of primary tumor site to the presence of nodal metastases (P > 0.05). The presence of nodal disease significantly predicted poorer overall (P = 0.001) and disease-specific survival (P = 0.017).Conclusion: The incidence of nodal metastases in esthesioneuroblastoma at diagnosis is rare, and elective management of the neck remains controversial. Primary tumor site does not appear to predict metastases at specific nodal basins. Higher tumor grade may be a harbinger of eventual nodal metastases.Level Of Evidence: NA Laryngoscope, 129:1025-1029, 2019. [ABSTRACT FROM AUTHOR]

Background Advanced-stage olfactory neuroblastoma requires multimodal therapy for optimal outcomes. Debate exists over endoscopic endonasal surgery in this situation. Stage-matched open and endoscopic surgical therapy were compared. Methods Patients from 6 cancer institutions were assessed. Stratification included dural involvement, Kadish stage, nodal disease, Hyams' grade, approach, and margin status. At follow-up, local control, nodal status, and evidence of distant metastases were recorded with any subsequent therapy. Statistical analyses to identify risk factors for developing recurrence and survival differences were performed. Results One hundred nine patients were assessed (age 49.2 ± 13.0 years; 46% women) representing Kadish A stage (10%), Kadish B stage (25%), and Kadish C stage (65%). The majority of the patients (61.5%) underwent endoscopic resection, 53.5% within Kadish C stage. Within-stage survival analysis favored endoscopic subgroup for Kadish C stage (log-rank P = .017) nonsignificant for Kadish B stage (log-rank P = .39). Conclusion Stage-matched survival was better for the endoscopically treated group compared to the open surgery group, with high negative margin resections obtained. [ABSTRACT FROM AUTHOR]

Objective: Olfactory neuroblastoma and the management of neck disease has posed considerable challenges to the treating physician. The aims of the study were to determine the incidence and factors influencing neck disease and to identify at-risk patients with cervical node-negative disease at presentation.Study Design: Multicenter case series with retrospective chart review.Setting and Subjects: In sum, 113 patients with a histopathologic diagnosis of olfactory neuroblastoma across 6 tertiary hospitals in Australia and the United States.Methods: Treatment modalities for the primary site and neck included surgery, radiotherapy, and combined therapy. Treatment outcomes were measured in relation to date of primary treatment, and long-term follow-up was recorded. Disease-free survival was calculated as time for patients to develop delayed neck disease following primary treatment.Results: A total of 113 patients (46 females, 49.7 ± 13.2 years) were identified with a median follow-up of 41.5 months (interquartile range, 58.2); 7.1% of patients presented with primary neck disease, while 8.8% of patients presented with delayed neck disease. Neck disease was present in patients with Hyams grade II (22.2%), III (55.6%), and IV (22.2%) lesions (χ(2) = 5.66, P = .13). Histologic grade was higher in patients with primary neck disease (χ(2) = 16.22, P = .001). Positive surgical margins were associated with a higher risk of delayed neck disease as compared with clear surgical margin (17.9% vs 5%, P = .034).Conclusion: Neck metastasis is an important clinical consideration for olfactory neuroblastoma at presentation and in surveillance. Primary treatment of the neck could be considered in select patients. Long-term surveillance of the neck and primary site is essential. [ABSTRACT FROM AUTHOR]

Background: Patients with sinonasal malignancy (SNM) present with significant sinonasal quality of life (QOL) impairment. Global sinonasal QOL as measured by the 22‐item Sinonasal Outcomes Test (SNOT‐22) has been shown to improve with treatment. This study aims to characterize SNOT‐22 subdomain outcomes in SNM. Methods: Patients diagnosed with SNM were prospectively enrolled in a multi‐center patient registry. SNOT‐22 scores were collected at the time of diagnosis and through the post‐treatment period for up to 5 years. Multivariable regression analysis was used to identify drivers of variation in SNOT‐22 subdomains. Results: Note that 234 patients were reviewed, with a mean follow‐up of 22 months (3 months–64 months). Rhinologic, psychological, and sleep subdomains significantly improved versus baseline (all p < 0.05). Subanalysis of 40 patients with follow‐up at all timepoints showed statistically significant improvement in rhinologic, extra‐nasal, psychological, and sleep subdomains, with minimal clinically important difference met between 2 and 5 years in sleep and psychological subdomains. Adjuvant chemoradiation was associated with worse outcomes in rhinologic (adjusted odds ratio (5.22 [1.69–8.66])), extra‐nasal (2.21 [0.22–4.17]) and ear/facial (5.53 [2.10–8.91]) subdomains. Pterygopalatine fossa involvement was associated with worse outcomes in rhinologic (3.22 [0.54–5.93]) and ear/facial (2.97 [0.32–5.65]) subdomains. Positive margins (5.74 [2.17–9.29]) and surgical approach—combined versus endoscopic (3.41 [0.78–6.05])—were associated with worse psychological outcomes. Adjuvant radiation (2.28 [0.18–4.40]) was associated with worse sleep outcomes. Conclusions: Sinonasal QOL improvements associated with treatment of SNM are driven by rhinologic, extra‐nasal, psychological, and sleep subdomains. [ABSTRACT FROM AUTHOR]

Esthesioneuroblastoma (ENB) is a rare sinonasal malignancy arising from olfactory neuroepithelium. Recurrence typically occurs locoregionally at the primary site or in the form of cervical metastasis. Delayed local recurrence away from the initial primary site is exceedingly rare. Retrospective review of 4 patients with histologically confirmed extraprimary local recurrence of ENB was performed with review of the literature. All cases initially presented with ENB isolated to the cribriform plate(s) treated with primary surgical resection and adjuvant radiotherapy. The first patient had ENB recurrence 8 years posttreatment involving the right orbit. She later developed metastases to the spine, neck, and mandible requiring composite resection and 4 courses of radiotherapy. The second patient had ENB recurrence of the dorsal septum 9 years posttreatment with cervical metastases requiring septectomy, bilateral neck dissection, and radiotherapy. The third patient had ENB recurrence 7 years posttreatment in the posterior nasopharynx requiring endonasal nasopharyngectomy. Finally, the fourth patient had ENB recurrence 12 years posttreatment in the sphenopalatine foramen, which was endoscopically resected. At the time of this review, all 4 patients were disease free at 32, 21, 4, and 24 months posttreatment follow-ups, respectively. This case series describes the rare phenomenon of delayed extraprimary local recurrence of histologically confirmed ENB. Treatment of extraprimary recurrences, analogous to other forms of ENB, should include primary surgical resection with adjuvant radiotherapy for generally favorable outcomes. Long-term close follow-up based on symptoms, endoscopy, and imaging is essential because of the risk of delayed recurrence. [ABSTRACT FROM AUTHOR]

Background Esthesioneuroblastomas (ENB) are uncommon and data regarding outcomes are often limited to single-institution series. The National Cancer Database (NCDB), which contains outcomes information from treatment centers across the United States, represents an opportunity to evaluate outcomes for rare diseases such as ENB across multiple institutions. Methods The NCDB was queried for location codes corresponding to the nasal cavity and paranasal sinuses and the histology code for ENB. Multivariate analyses were performed to evaluate for contributing factors to overall survival. Results A total of 1225 patients with ENB met the inclusion criteria. The 5-year overall survival was 76.2% (95% confidence interval [CI], 73.4-79.0%). Overall survival was associated with Kadish stage, grade, treatment sequence, margin status, Charlson/Deyo score, age, and gender ( p < 0.05). Multivariate analysis demonstrated that, compared with surgery alone, surgery followed by radiation without chemotherapy had improved all-cause mortality (odds ratio [OR], 0.61; 95% CI, 0.40-0.95). Surgery with chemotherapy alone was associated with increased odds of all-cause mortality (OR, 4.86; 95% CI, 2.31-10.25). Multivariate subanalysis for Kadish stages A and B demonstrated no difference in survival between surgery and surgery followed by radiation, but surgery followed by chemoradiation had worse overall survival (OR, 3.03; 95% CI, 1.07-8.56). For Kadish stage C, surgery followed by radiation had improved overall survival compared with surgery alone (OR, 0.44; 95% CI, 0.24-0.81). Conclusion The most common treatment for ENB is surgery followed by radiation, which is associated with the highest overall survival. The role of adjunctive chemotherapy needs to be re-evaluated in further studies. [ABSTRACT FROM AUTHOR]

Background Current guidelines have identified 10 to 12 weeks posttreatment as the ideal time-point for improved diagnostic accuracy of positron emission tomography/computed tomography (PET/CT) for deep tissue sites of the head and neck. After treatment, the sinonasal skull base is predisposed to prolonged inflammation that may render this time-point inappropriate for initial posttreatment imaging surveillance for sinonasal malignancies. The purpose of this study is to evaluate temporal trends in 18F-fluorodeoxyglucose (18FDG) sinonasal uptake after treatment for sinonasal malignancies to better elucidate the optimal time-point for initial PET/CT posttreatment evaluation in this patient population. Methods A retrospective analysis of all successfully treated and non-locally recurrent sinonasal malignancies over a 15-year study period (2000 to 2015) was performed at our institution. Posttreatment 18FDG PET/CT standardized uptake value data were collected and compared between various time-points (2 to 4 months, 5 to 12 months, 5 to 24 months, and 13 to 24 months) using an independent-samples t test. Results A statistically significant difference was noted between the posttreatment time windows 2 to 4 and 5 to 12 months ( p = 0.048) as well as 2 to 4 and 5 to 24 months ( p = 0.02). A trend toward significance was seen when comparing 2 to 4 and 13 to 24 months ( p = 0.083). Conclusion Our analysis of PET/CT in patients previously treated for sinonasal malignancy suggests that the posttreatment sinonasal skull base is characterized by a prolonged period of hypermetabolism that endures beyond the period previously described for deep tissue sites of the head and neck. These findings prompt a reevaluation of the previously described 10- to 12-week cutoff point for initial posttreatment PET/CT for head and neck squamous cell carcinoma as applied to sinonasal malignancies. [ABSTRACT FROM AUTHOR]

Background The gold standard of treatment for esthesioneuroblastoma consists of en bloc craniofacial resection with postoperative therapy dictated by histology and tumor extent. Numerous studies have shown fully endoscopic approaches to provide comparable survival and recurrence rates with decreased patient morbidity. Here we report the first multi-institutional series assessing smell outcomes of patients who underwent unilateral endoscopic resection of esthesioneuroblastoma with preservation of the contralateral olfactory bulb. Methods A multi-institutional retrospective review was performed identifying patients who underwent endoscopic unilateral resection of esthesioneuroblastoma with preservation of 1 olfactory bulb between 2003 and 2015. After completion of postoperative radiation, patients were administered the University of Pennsylvania Smell Identification Test (UPSIT) to assess olfactory function. Results Fourteen patients (7 males, 7 females) were identified and tested for posttreatment olfactory function. All 14 patients received postoperative radiotherapy and 4 patients received additional chemotherapy. Mean follow-up time was 51.7 months. There was no disease recurrence. Six patients (43%) were found to have residual smell function with 2 patients (14%) having normal or mildly reduced smell function. Conclusion Here we report the first multi-institutional series demonstrating smell preservation after unilateral endoscopic resection of esthesioneuroblastoma. In carefully selected patients, this approach can yield comparable survival with decreased patient morbidity. [ABSTRACT FROM AUTHOR]