Esthesioneuroblastoma

A retrospective review of the clinical records of 31 patients with esthesioneuroblastoma is presented. A bimodal age distribution was noted. The tumor is extremely rare among blacks. Most patients presented with locally advanced disease. However, regional and distant metastases at the time of initial diagnosis are uncommon. Local recurrence at the primary site was very common, and this reflects either the conservative initial surgical treatment employed or the multicentric nature of the tumor. Cervical lymph node metastasis is present in less than 10 percent of patients at the time of diagnosis. The survival rates were better in patients with early stages of disease. From this study, we conclude that the current management of these tumors should consist of a combination of radiation therapy and surgery. Radical resection followed by postoperative radiation therapy appears to be the treatment combination of choice. The five-year survival rate in our series was 52 percent.