Esthesioneuroblastoma: The Johns Hopkins experience

Background. Esthesioneuroblasloma (ENB) is an uncommon malignant neoplasm o f the upper nasal cavity. Therapeutic management approaches for this neopla sm lack uniformity and there is no universally accepted staging system. Methods. A retrospective review of 27 patients with histologically confirme d ENB managed at The Johns Hopkins Hospital. Results. Eighty-five percent of patients had surgical resection as part of their disease management. Complete surgical resection was achieved in 62% o f patients who had a craniofacial resection. Eighty percent of patients wit h negative surgical margins remain with no evidence of disease, with a medi an follow-up of 5.6 years. Adjuvant radiation therapy was beneficial to 62% of patients with positive surgical margins. Clinical responses were observ ed with cisplatin- and etoposide-containing chemotherapy regimens in patien ts with advanced disease. A revised staging system based on our experience is proposed. Conclusions. ENB is best managed by craniofacial resection with complete tu mor resection. Adjuvant radiation therapy is warranted in patients that rem ain with positive histologic margins of resection. Chemotherapy with cispla tin- and etoposide-containing regimens may be useful for palliation of adva nced disease. (C) 2000 John Wiley & Sons, Inc.