Long-Term Outcomes after Surgery for Esthesioneuroblastoma: A Single Comprehensive Cancer Center Experience

Introduction: Esthesioneuroblastoma (ENB) is a rare tumor originating from the olfactory epithelium. Most ENBs are located below the cribriform plate and often extend intracranially. The mainstay of the ENB treatment is surgery, which involves resection of the skull base via an open craniofacial, endoscopic, or combined approach. We sought to review long-term oncologic outcomes for those treated at a single center over four decades.

Methods: After institutional review board approval, patients were selected from our existing skull base tumor database if they were treated with surgery between 1973 and 2015. Forty-eight (11%) ENB were included out of 454 patients of the whole cohort. The follow-up interval was calculated in months from the date of initial curative surgery. To identify prognostic variables that were predictors of overall (OS) and disease-specific survival (DSS), exploratory analyses using Kaplan–Meier curves with the log-rank test for comparison were performed. Gender, age, type of tumor, Kadish stage, pT stage, pN stage, surgical margins, intracranial invasion, and orbital invasion were analyzed independently. A p-value of less than 0.05 was considered statistically significant.

Results: The median age was 53 (range: 14–76, SD: 15.8). There were 33 (69%) men. Most cases were primary tumors 42 (88%) and 10 (21%) and 28 (58%) were pT3 and pT4, respectively. Forty-four (92%) were cN0 and 47(98%) were M0. Kadish stage A and B diagnosed in 21 (44%), Kadish C and D in 26 (54%) patients. All cases were treated surgically, and the majority had a skull base resections 45 (94%). Craniofacial resection performed in 34 (71%) patients, in 11 (23%) cases surgery was carried out by an endoscopic or combined open-endoscopic approach. Negative margins, positive margins, and not recorded margins were noted in 30 (62%), 9 (19%), and 9 (19%) cases respectively. Thirty patients had adjuvant radiation therapy (63%) and 4 patients have adjuvant chemoradiation (8%), and 14 (29%) had no additional treatment. Overall and disease-specific survival at 10 years was 71.3% and 79.6%, respectively ([Fig. 1]). pT stage, pN stage, and Kadish stage showed a significant difference in OS and DSS ([Figs. 2] and 3). There was no significant difference in outcomes for the patients who received adjuvant treatment, compared with those who do not.

Conclusion: Primary surgical treatment shows excellent results in the treatment of ENB. Tumor extension and nodal metastasis are two parameters were patients showed a significant difference in OS and DSS.

Fig. 1

Fig. 2

Publication History

Article published online:
12 February 2021