Esthesioneuroblastoma of the pituitary gland: a clinicopathological entity?

Case report and review of the literature

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  • 1 Division of Neuroradiology, Neurosurgical Department, University Hospital, Inselspital, and Division of Neuropathology, Institute of Pathology, University of Bern, Switzerland; and Institute of Neuropathology, Reinisch—Westfälische Hochschule Aachen, Germany
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✓ 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.

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Contributor Notes

Address reprint requests to: Luigi Mariani, M.D, Neurochirurgische Klinik, Inselspital, CH-3010 Bern, Switzerland. email: luigi.mariani@insel.ch.
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