Esthesioneuroblastoma: A Comprehensive Review of Diagnosis, Management, and Current Treatment Options

World Neurosurg. (2019) 126:194-211

Many controversies exist in the diagnosis and management of this aggressively malignant condition, mainly because of limited literature and lack of randomized control trials, resulting in nonstandardized treatment methods. We performed a comprehensive review of the literature to identify management approach and treatment options for esthesioneuroblastoma.

METHODS: An extensive review of the published literature was conducted in PubMed, OVID Medline, and EMBASE journals for studies of esthesioneuroblastoma. Terms for search included esthesioneuroblastoma (ENB) and olfactory neuroblastoma (ONB). No date restrictions were used.

RESULTS: The search yielded 3876 related articles. Cross-checking of articles led to exclusion of duplicate articles. The remaining 1170 articles were screened for their full text and English language availability. Of 609 full-text articles available, animal studies, irrelevant articles, and studies with mixed/ confusing data were excluded. We finalized 149 articles pertaining to the topic, including 119 original research articles, 3 book chapters, 11 reviews, 9 case reports, and 7 case series.

CONCLUSIONS: Surgical resection followed by radiotherapy is the standard for treatment for higher-grade lesions. The endoscopic endonasal approach is gaining further recognition with more favorable outcomes and better survival than for open surgery. Postoperative radiotherapy is associated with the highest overall survival and shows benefit for patients with higher-stage disease and those who receive chemotherapy. Recurrence rates after treatment vary drastically in the literature and, therefore, prolonged follow-up with repeated imaging is recommended. Lifelong surveillance is recommended because of late recurrences associated with this tumor.

Esthesioneuroblastoma: A Patterns-of-Care and Outcomes Analysis of the National Cancer Database

Neurosurgery 83:940–947, 2018

BACKGROUND: The available literature to guide treatment decision making in esthesioneuroblastoma (ENB) is limited.

OBJECTIVE: To define treatment patterns and outcomes in ENB according to treatment modality using a large national cancer registry.

METHODS: This study is a retrospective cohort analysis of 931 patients with a diagnosis of ENB who were treated with surgery, radiation therapy, and/or chemotherapy in the United States between the years of 2004 and 2012. Log-rank statisticswere used to compare overall survival by primary treatment modality. Logistic regression modeling was used to identify predictors of receipt of postoperative radiotherapy (PORT). Cox proportional hazardsmodeling was used to determine the survival benefit of PORT. Subgroup analyses identified subgroups that derived the greatest benefit of PORT.

RESULTS: Primary surgery was the most common treatment modality (90%) and resulted in superior survival compared to radiation (P < .01) or chemotherapy (P < .01). On multivariate analysis, PORT was associated with decreased risk of death (hazard ratio [HR] 0.53, P<.01). PORT showed a survival benefit in Kadish stage C (HR 0.42, P<.01) and D (HR 0.09, P = .01), but not Kadish A (HR 1.17, P = .74) and B (HR 1.37, P = .80). Patients who received chemotherapy derived greater benefit from PORT (HR 0.22, P < .01) compared with those who did not (HR 0.68, P=.13). Predictors of PORT included stage, grade, extent of resection, and chemotherapy use.

CONCLUSION: Best outcomes were obtained in patients undergoing primary surgery. The benefit of PORT was driven by patients with stages C and D disease, and by those also receiving chemotherapy.


Endonasal endoscopic resection of esthesioneuroblastoma: the Johns Hopkins Hospital experience and review of the literature

Neurosurg Rev. DOI 10.1007/s10143-011-0329-2

Esthesioneuroblastoma is an uncommon malignant tumor originating in the upper nasal cavity. The surgical treatment for this tumor has traditionally been via an open craniofacial resection. Over the past decade, there has been tremendous development in endoscopic techniques.

In this report, we performed a retrospective analysis of patients with esthesioneuroblastomas treated with a purely endonasal endoscopic approach and resection at the Johns Hopkins Hospital between January 2005 and April 2010.

A total of eight patients with esthesioneuroblastoma, five men and three women, were identified. Six patients were treated for primary disease, and two were treated for tumor recurrence. The modified Kadish staging was A in one patient (12.5%), B in two patients (25%), C in four patients (50%), and D in one patient (12.5%). All patients had a complete resection with negative intraoperative margins. One patient had intraoperative hypertension; there were no perioperative complications. With a mean follow-up of over 27 months, all patients are without evidence of disease. In addition, we reviewed the literature and identified several overlapping case series of patients with esthesioneuroblastoma treated via a purely endoscopic technique.

Our series adds to the growing experience of expanded endonasal endoscopic surgery in the treatment of skull base tumors including esthesioneuroblastoma. Longer follow-up on a larger number of patients is required to further demonstrate the utility of endoscopic approaches in the management of this malignancy.