Clinical Outcomes and Patterns of Failure in Esthesioneuroblatoma: A Single Institutional Experience

Esthesioneuroblastoma (ENB) is a rare sinonasal malignancy that is typically managed with a combination of surgical resection, radiotherapy and chemotherapy. Data used to guide therapeutic decision making are scant and typically obtained from small retrospective series due to the relative infrequency of the diagnosis. Here, we report our own institutional experience in the management of patients with ENB to help compliment these prior single institutional reports. Records from patients receiving treatment for ENB at the University of Minnesota Medical Center were obtained from 1994 to 2019. A total of 17 patients were identified from our retrospective review. Kadish stage at initial presentation was A in 2 (12%), B in 5 (29%), C in 9 (53%) and D in 1 (6%). All patients underwent surgical resection. Adjuvant radiotherapy was utilized in 12 (71%) patients with concurrent chemotherapy administered in 3 (18%) patients. One patient received neoadjuvant chemoradiotherapy followed by surgical resection. Four patients developed recurrent disease with locoregional failure presenting as the most common site of initial relapse within our study population. Isolated local recurrence occurred in 2 patients, one patient developed combined local and regional failure, while another had combined regional and distant failure with osseous metastases. Recurrent disease was managed by either combined salvage surgery and radiotherapy (RT) or RT alone. Three of the 4 patients who developed recurrence eventually succumbed to their disease. Estimates for 5-year DFS and OS were 65% and 90%, respectively, for the entire cohort.