Esthesioneuroblastoma with distant metastases: Systematic review & meta-analysis.

Background: The purpose of this study was to determine the clinical outcomes and review the management strategies for metastatic esthesioneuroblastoma.

Methods: We conducted a systematic review and meta-analysis.

Results: Forty-eight studies totaling 118 patients met inclusion criteria. Chemotherapy in combination with surgery and/or radiation exhibited the best overall survival when compared to monotherapy and no treatment (P < .001). However, most patients (66%) received either monotherapy or no therapy. The number and location of metastases among the 3 treatment groups did not significantly differ (P = .85). Treatment modality remained significantly associated with overall survival on multivariable analysis (P < .001). Platinum-based chemotherapy was most commonly utilized but did not provide a survival benefit when compared with all other regimens (P = .88).

Conclusion: Distant metastases with esthesioneuroblastoma portend a poor prognosis. Chemotherapy in combination with surgery and/or radiation was associated with improved overall survival. Further research into the optimal systemic therapeutic regimen for patients with distant metastases is critical.