Esthesioneuroblastoma: An Update on the Ucla Experience 2002-2013

Introduction: Esthesioneuroblastomas are rare malignant tumors of the superior nasal vault. Controversy exists surrounding optimal treatments and staging systems that best predict survival. Here, we report an update on the UCLA experience and identify prognostic factors for survival and also provide an update on the surgical techniques being utilized.

Objectives: To profile the clinical presentation and treatment results of esthesioneuroblastomas at UCLA from 2002–2013 with emphasis on prognostic factors and treatment outcomes.

Study Design: A retrospective review was performed by examining the medical records, imaging, and pathology reports of 41 patients with esthesioneuroblastomas treated at UCLA Medical Center from 2002 to 2013.

Results: Thirty-six patients were included in the study. The mean age of the study population was 50.1 +/− 16.9 years. Males and females were evenly distributed. Mean duration of follow-up for the entire group was 31.5 months. Eight patients underwent traditional craniofacial resection with craniotomy (CFR), twenty patients underwent transnasal, transfacial resection without craniotomy (TFR), and eight patients underwent an expanded-endoscopic, endonasal approach (ECR). Negative margins were achieved in 83.3% of patients. 5-year recurrence-free and overall survival was 55% and 82%, respectively. Univariate analysis revealed that tumor grade, intracranial extension with dural invasion, presentation with recurrent disease, and Dulguerov-Calcaterra staging all affected RFS. Modified Kadish stage was the only factor identified to affect OS. Multivariate analysis demonstrated that tumor grade was the only factor that independently impacted RFS. There was no statistical difference in survival among the surgical approaches chosen.

Conclusion: The updated data on the UCLA experience reveals that traditional CFR, TFR, and ECR all provide comparable survival, although longer follow up will be needed to ascertain if these findings hold true. The endoscopic approach had a statistically significant decrease in length of hospital stay and trended toward reduced blood loss, ICU admission, and complications. The modified Kadish staging was the only factor identified to predict overall survival. Multivariate analysis revealed that tumor grade was an independent predictor of recurrence; therefore, its importance should be emphasized in current staging systems.