Assessing the Prognostic Value of Upfront Neck Disease in Olfactory Neuroblastoma: A Multicenter, Matched Cohort Study

Introduction: Olfactory neuroblastoma (ONB) is a rare sinonasal and skull base malignancy that is well known for its predilection for delayed cervical metastasis—often over 5 to 10 years from initial presentation. While only 5 to 8% of patients initially present with nodal disease, and is associated with an increased modified Kadish stage, the impact of nodal disease at presentation on treatment and outcomes is not clear or well-studied. Methods: Between February 2005 and April 2021, a total of 257 patients with histologically confirmed ONB were evaluated retrospectively from nine academic tertiary care centers within North America. Epidemiologic parameters and tumor characteristics were recorded for each patient, including factors such as age and gender, margin status, Hyams grade, Kadish staging, dural invasion, and survival follow up data. Due to the rarity of upfront neck disease on presentation (N+), we performed a 1:4 match of patients with N+ disease with clinically N0 disease across multiple covariates: age, sex, Kadish staging and Hyams grade (low vs. high). Survival analysis was performed using Kaplan–Meier and conditional logistic regressions with recurrence-free survival (RFS) and overall survival (OS) as the primary and secondary endpoints, respectively. Results: After matching, our final cohort included 121 patients, of which 27 presented with N+ disease. The mean follow-up time was 54 months [standard deviation [SD]: 37.7 months] for those with N+ disease versus 60.9 months [42.8 months] for N0 disease ( p = 0.42). Both N+ and N0 cohorts were more likely to be staged as modified Kadish C on presentation (92.6 vs. 79.8%, p = 0.12), respectively. Patients with N+ disease were more likely to undergo a unilateral (29.6 vs. 1.1%, p < 0.001) or bilateral neck dissection (11.1 vs. 0%, p < 0.001), compared with N0 patients. Similarly, patients with N+ disease were more likely to undergo neck radiation (75.0 vs. 22.1%, p < 0.001). There were no differences among the patterns of recurrence between the N+ and N0 groups: nasal cavity/paranasal sinuses (14.8 versus 18.1%), neck (14.8 vs. 20.2%), intracranial (7.4 vs. 2.1%), and distant metastasis (7.4 vs. 3.2%). There were no differences in 10-year OS (37.8 vs. 63.7%, p = 0.11) or 10-year RFS (30.4% vs. 32.6%, p = 0.44) between N+ and N0 patients, respectively. On multivariate conditional logistic regression, neck disease on presentation was not an independent, statistically significant factor for RFS (odds ratio [OR]: 1.1, 95% confidence interval [CI]: 0.47–2.60, p = 0.828) and OS (OR: 1.98, 95% CI: 0.77–5.10, p = 0.156). Conclusion: In this matched cohort study, no survival differences were demonstrated in patients who presented with N+ disease compared with N0 disease. Despite receiving definitive treatment of the neck more frequently, patients with N+ disease did not significantly differ in their pattern of recurrence when compared with patients who presented with N0 disease. In sum, our study highlights potential limitations in the modified Kadish D staging as no prognostic difference was noted when comparing N+ and N0 cohorts. Publication History Article published online: 07 February 2025 © 2025. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

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