Abstract
PurposeThe main objective of this study was to evaluate the cumulative incidence of cause-specific mortality and other causes of mortality for patients with olfactory neuroblastoma (ONB). The secondary aim was to model the probability of cause-specific death and build a competing risk nomogram to predict cause-specific mortality for this disease.MethodsPatients with ONB from 1975 to 2016 were identified from the Surveillance, Epidemiology, and End Results database. We estimated the cumulative incidence function (CIF) for cause-specific mortality and other causes of mortality, and constructed the Fine and Gray’s proportional subdistribution hazard model, as well as a competing-risk nomogram based on Fine and Gray’s model, to predict the probability of cause-specific mortality for patients with ONB.ResultsAfter data selection, 826 cases were included for analysis. Five-year cumulative incidence of cause-specific mortality was 19.5% and cumulative incidence of other causes of mortality was 11.3%. Predictors of cause-specific mortality for ONB included tumor stage, surgery and chemotherapy. Age was most strongly predictive of other causes of mortality: patients aged > 60 years exhibited subdistribution hazard ratios of 1.063 (95 % confidence interval [CI] 1.05–1.08; p = 0.001). The competing risk nomogram for cause-specific mortality was well-calibrated, and had good discriminative ability (concordance index = 0.79).ConclusionsWe calculated the CIF of cause-specific mortality and other causes of mortality in patients with the rare malignancy ONB. We also built the first competing risk nomogram to provide useful individualized predictive information for patients with ONB.
Highlights
Olfactory neuroblastoma (ONB) is an uncommon malignancy that arises from the olfactory epithelium and represents approximately 3% of all sinonasal malignancies [1, 2]
We reviewed all ONB cases registered in the Surveillance, Epidemiology, and End Results (SEER) database from 1975 to 2016 and conducted a competing risk analysis
The study cohort consisted of patients with ICD-O-3 histology codes 9522/2 (ONB) and ICD-O-3 site codes as follows: C30.0, C31.0, C31.1, C31.2, C31.3, C31.8, and C31.9
Summary
Olfactory neuroblastoma (ONB) is an uncommon malignancy that arises from the olfactory epithelium and represents approximately 3% of all sinonasal malignancies [1, 2]. With improvement in pathological recognition, the incidence of ONB is steadily increasing in recent years [3]. Based on several retrospective reviews [6,7,8,9,10] and meta-analyses[2, 11], surgical resection combined with radiotherapy is the current best treatment modality resulting in prolonged 5 year OS ranging from 65 to 75% [1, 2, 12,13,14]. Given the high incidence in the elderly as well as the relatively long-term survival for patients with ONB, a considerable number of patients may die of other non-cancer causes, especially for the elderly.
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