Esthesioneuroblastoma: The Johns Hopkins experience

BACKGROUND: Esthesioneuroblastoma (ENB) is an uncommon malignant neoplasm of the upper nasal cavity. Therapeutic management approaches for this neoplasm lack uniformity and there is no universally accepted staging system. METHODS: A retrospective review of 27 patients with histologically confirmed 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% of patients who had a craniofacial resection. Eighty percent of patients with negative surgical margins remain with no evidence of disease, with a median follow-up of 5.6 years. Adjuvant radiation therapy was beneficial to 62% of patients with positive surgical margins. Clinical responses were observed with cisplatin- and etoposide-containing chemotherapy regimens in patients with advanced disease. A revised staging system based on our experience is proposed. CONCLUSIONS: ENB is best managed by craniofacial resection with complete tumor resection. Adjuvant radiation therapy is warranted in patients that remain with positive histologic margins of resection. Chemotherapy with cisplatin- and etoposide-containing regimens may be useful for palliation of advanced disease.