Olfactory Neuroblastoma: Fate of the Neck—A Long-term Multicenter Retrospective Study

Abstract

Objectives:

Determine the incidence and factors influencing neck disease, at presentation and delayed, in patients with olfactory neuroblastoma.

Methods:

Patients with a histological diagnosis of olfactory neuroblastoma that were treated across 6 tertiary hospitals were included. Treatment modalities to primary site and neck included radiotherapy, surgery, and combinations. The status of cervical lymph node metastases at presentation and at last follow-up was defined. Disease-free survival (DFS) was calculated as time taken for patients to develop delayed neck disease following primary treatment of olfactory neuroblastoma. Pearson correlation, regression analysis, and Kaplan Meier plots were performed to identify risk factors for developing cervical neck metastases.

Results:

A total of 113 patients (46 females, 49.7 ± 13.2 years) with median follow-up of 41.5 months (interquartile range, 58.2 months) were identified. Of the patients, 7.1% presented with primary neck disease while 8.8% of patients presented with delayed neck disease. Neck disease, both primary and delayed, was present in patients with Hyams grade II (22.2%), III (55.6%), and IV (22.2%) lesions (χ² 2 5.66, P = .13). Histologic grade was higher in patients with primary neck disease (χ² 2 16.22, P = .001). Positive surgical margins were associated with a higher risk of delayed neck disease compared with clear surgical margin (17.9% vs 5%, P = .034). Five- and 10-year disease-free survival for regional neck disease was 92.4% (SE: 2.8%) and 79.6% (SE: 9.0%), respectively.

Conclusions:

Neck metastasis is an important clinical consideration for olfactory neuroblastoma both at presentation and in surveillance. Primary treatment of the neck could be considered in select patients.