Patterns of failure and outcome in esthesioneuroblastoma

Abstract

Objectives: To evaluate the results of standardized treatment of esthesioneuroblastoma (ENB) during a 17-year period and to identify pertinent factors for clinical outcome.

Design: Review of clinical and radiographic data and retrospectively staging ENB according to 3 staging systems: Kadish, Biller, and Dulguerov and Calcaterra.

Setting: Hospital do Cancer I-Instituto Nacional de Cancer, Rio de Janeiro, Brazil.

Patients: Thirty-six patients with histologically confirmed ENB treated between January 1, 1983, and December 31, 2000; 35 fulfilled study inclusion criteria.

Interventions: Treatment included gross tumor resection through a transfacial approach with postoperative radiotherapy (RT) in 11 patients, craniofacial resection (CFR) and postoperative RT in 7, exclusive RT in 14, CFR alone in 1, and a combination of chemotherapy and RT in 2. Histopathological slides were reviewed and graded using the Hyams staging system. Analysis of prognostic factors was performed.

Main outcome measures: Evaluation of survival rates using the Kaplan-Meier method. Analysis of prognostic factors carried out with the Fisher exact test and the log-rank test.

Results: Analysis of survival showed that the Kadish classification best predicted disease-free survival (P =.046). The presence of regional and distant metastases adversely affected prognosis (P<.001 and P =.01, respectively). Craniofacial resection plus postoperative RT provided a better 5-year disease-free survival rate (86%) compared with the other therapeutic options used (P =.05). The 5-year disease-specific survival rate was 64% and 43% for the low- and high-grade tumors, respectively (P =.20). Disease-free survival for this cohort of 35 patient was 46% and 24% at 5 and 10 years, respectively. Overall survival was 55% and 46% at 5 and 10 years of follow-up, respectively.

Conclusions: The development of cervical nodal metastases and distant metastases had a significant adverse impact on prognosis. The value of the Kadish staging system was confirmed in our study, significantly correlating with prognosis. Tumor grade according to the Hyams staging system also seems to be an important factor in determining prognosis for tumor recurrence and survival. Aggressive multimodality therapeutic strategies, particularly CFR and adjuvant RT, yielded the best treatment outcome.