Objective: In this study we tried to evaluate a long-term treatment outcome of olfactory neuroblastoma
(ONB).
Materials and Methods: A retrospective review of patients was performed for those who had definitive treatment
for ONB from 1992 to 2021. Tumor stage, treatment modalities, and survivals were analyzed.
Results: One hundred one patients were analyzed. Mean age was 41.2 ± 14.3 years and mean duration
of follow-up was 84.1 ± 78.3 months. 35.7% (N = 31) were Kadish A/B, 58.4% (N = 59) were Kadish C, and the other 10.9% (N = 11) were Kadish D. In total, 76.2% (N = 77) underwent surgical resection with or without adjuvant therapy and the other
23.8% (N = 24) underwent definitive radiotherapy. The 5-year and 10-year cancer-specific survival
(CSS) was 81.9% and 79.4%. Overall relapse rate was 41.6% and the mean time to initial
relapse was 37.5 ± 44.1 months. First relapse was local in 13 (12.9%) patients, regional
in 13 (12.9%) patients, meningeal in 6 (5.9%) patients, and distant in 10 (9.9%) patients.
The 5-year CSS after initial relapse was significant different among recur site; 64.2%
for local, 100% for regional, 44.4% for meningeal, and 0% for distant (log-rank p < 0.001). Kadish D was associated with lower distant free survival (log-rank p < 0.001), while definitive radiotherapy without surgical resection was associated
with lower local relapse free survival and distant free survival (log-rank p < 0.001, respectively).
Conclusion: Survival of ONB patients was associated with initial relapse pattern. Especially
those with nodal relapse showed good survival. Definitive treatment modality and advanced
Kadish stage was associated with local and distant control and therefore affects the
overall survival. Factors associated with meningeal recurrence remains elusive.
