Esthesioneuroblastoma: reflections of a 21-year experience.

OBJECTIVES: To evaluate the results of standardized treatment of esthesioneuroblastoma at a single institution during a 21-year period and calculate pertinent parameters, i.e., metastatic disease (local, regional, distant), disease-free survival, and complications of treatment.

STUDY DESIGN: A retrospective review was conducted of all patients treated at a single institution from September 1976 through May 1998.

METHODS: Only those patients who received their complete evaluation and treatment at our institution were included in this analysis. Thirty-five patients met this criterion. In addition, results of epidemiological, pathological, and molecular analyses were evaluated to seek accurate indicators for clinical outcomes.

RESULTS: Six percent of patients presented with cervical metastatic disease, but ultimately 25.7% developed at least one episode of cervical metastases; 14.3% of patients developed a local recurrence an average of 6 years after diagnosis; and 37% of the patients ultimately developed at least one episode of metastatic disease. The disease-free survival for this cohort of 35 patients was 80.4% at 8 years. CNS complications occurred in 25.7% of the patients, 22.9% had orbital complications, 20% had systemic posttreatment problems, 18.2% had chemotoxic sequelae, 8.6% had infectious complications, and 14.3% had cosmetic sequelae. No epidemiological, pathological, or molecular factors appeared to be more accurate clinical indicators than the Kadish staging system.

CONCLUSIONS: This series of esthesioneuroblastoma patients (N=35) reflects an 8-year disease-free survival of 80.4%, representing a significant number of patients treated and followed at one institution for an extended period of time. No valuable pathological or molecular indicators to predict aggressive clinical behavior were found. The average time interval before recurrent disease developed was more than 6 years, far greater than that expected for other sinonasal malignancies. Therefore, extended follow-up is necessary for this patient group.