Esthesioneuroblastoma of the pituitary gland: a clinicopathological entity?

Abstract

Esthesioneuroblastoma (olfactory neuroblastoma) is a rare, malignant neoplasm that typically arises in the nasal vault, invades adjacent tissues, and causes locoregional (cervical lymph nodes) and distant metastases. Only two cases of tumors arising in the sellar region that had the histological characteristics of esthesioneuroblastoma have been reported in the literature to date. The authors present the case of a 35-year-old woman with secondary amenorrhea and a rapidly growing tumor located in the adenohypophysis. After total removal of the lesion through a transseptal-transsphenoidal approach, the histological examination revealed an esthesioneuroblastoma Grade II/III according to Hyams. Considering the particular location of the lesion and the absence of residual tumor on postoperative magnetic resonance imaging, no adjuvant therapy was performed. The patient remained free from tumor recurrence 2 years postoperatively. Because all published cases of this esthestoneuroblastoma have been large neuroblastic tumors of the pituitary gland arising in middle-aged women, pituitary neuroblastoma might represent a rare, specific clinicopathological entity.