ABSTRACT
In common with all tumors of the anterior skull base, esthesioneuroblastoma (ENB) is comparatively rare. This malignant neuroendocrine neoplasm arose from the olfactory mucosa and was first recognized by Berger et al. in 1924 (1), who coined the term “esthesioneuroepitheliome olfactif.” However, a wide range of other terms has been used including esthesioneurocytoma, esthesioneuroma, intranasal neuroblastoma, olfactory neuroepithelial tumor, and olfactory neuroblastoma. In 1966, Skolnik et al. (2) found only 97 cases reported in 42 papers in the English literature with most authors only treating 2 or 3 cases, and by 1989 O’Connor estimated that ≤300 cases had been published which represented 1% to 5% of all malignant tumors of the nasal cavity (3). However, this number had risen to 945 by 1997 (4), a report which did not include a large series from the Armed Forces Institute of Pathology (5) nor from the Institut Gustave-Roussy (6). In 2000, the National Cancer Data Base included 664 cases fromover 500U.S. hospitals over a 10-year period (1985-1995). In recent years, increasing numbers of this tumor are being described, almost certainly due to an increasing awareness and improved histological techniques for diagnosis. Despite this, it remains difficult to accrue large individual series, compromising statistical analysis of outcome. The authors, working in a tertiary referral center, have had the opportunity of managing 78 cases since 1970.
