Diagnosis and management of esthesioneuroblastoma.

AIMS: Our purpose was to evaluate the diagnosis and combined modality treatment of esthesioneuroblastoma treated at 1 institution.

METHODS: A retrospective analysis of clinical information regarding presentation, immunohistochemical results, treatment and outcome was performed on 21 patients with esthesioneuroblastoma.

RESULTS: Two patients had Kadish A, 12 had Kadish B and 7 had Kadish C disease. The mean follow-up was 41.9 months. Immunohistochemical stains were performed in 17 cases, and the tumors were focal positive or showed positive immunoreactivity to neuron-specific enolase (17/17, 100%), synaptophysin (13/17, 76.5%), S-100 protein (8/17, 47.1%), epithelial membrane antigen (5/17, 29.4%), chromogranin A (4/17, 23.5%), vimentin (3/17, 17.6%) and cytokeratin (2/17, 11.8%). Generally, the patients were treated with preoperative radiotherapy (Kadish A and Kadish B) and preoperative chemoradiotherapy (Kadish C) after the initial biopsy, followed by surgery. Surgical approaches (n = 21) consisted of 12 lateral rhinotomies, 5 craniofacial resections, 3 endoscopic sinus surgeries and 1 midfacial degloving approach. The 5-year crude overall survival rate was 76.2%.

CONCLUSIONS: Esthesioneuroblastoma is an uncommon malignant tumor. Thorough histological evaluation is the key to correct diagnosis and differentiation. Preoperative radiotherapy or chemoradiotherapy can give surgeons the chance to choose different surgical approaches, especially the endoscopic surgical techniques, in the future.