An Unusual Oncocytic Sellar Neoplasm in a Patient with Birt-Hogg-Dubé Syndrome

Abstract

Spindle cell oncocytoma (SCO) is a rare and recently described neoplasm arising from the pituitary adenohypophysis thought to be derived from the folliculostellate cells. Even though SCO has been classified as a distinct World Health Organization (WHO) grade I tumor, sellar neoplasms with an oncocytic appearance are uncommon and have a wide differential diagnosis that includes primary and metastatic lesions. Herein we describe an oncocytic sellar neoplasm of uncertain histiogenesis arising in a 49-year-old man with BirtHogg-Dubé (BHD) syndrome. This case is also unusual in that individuals with BHD have a propensity to develop oncocytomas, yet to the best of our knowledge this is the first occurrence of a primary oncocytic neoplasm of the sella in BHD syndrome.