Composite pituitary adenoma and craniopharyngioma?: an unusual sellar neoplasm with divergent differentiation.

Abstract

We report a young man who presented with atrial fibrillation and was subsequently found to have a markedly elevated serum thyroid stimulating hormone level and a solid, noncalcified intrasellar mass on imaging. The patient was treated with thyroid radioablation and hormone replacement and followed for 7 years, during which time the tumor grew to 4.6 cm. He had no visual disturbances or other hormonal symptoms. At transsphenoidal surgery, a tumor consisting of a pituitary adenoma and adamantinomatous craniopharyngiomalike components was resected. Both components were closely intermingled, but there was no evidence of an intermediate morphologic phenotype. Immunohistochemically, the adenoma was not only positive for beta-thyroid stimulating hormone, alpha subunit, and pituitary transcription factor 1, but also stained for beta-follicle stimulating hormone, steroidogenic factor-1, adrenocorticotropic hormone, and pituitary-restricted transcription factor (Tpit), exhibiting an unusual plurihormonal profile. The craniopharyngiomalike component showed immunoreactivity for steroidogenic factor-1, one of the pituitary transcription factors. This lesion may represent an unusual composite tumor attributable to divergent differentiation of a common precursor. Alternatively, it may be viewed as a pituitary adenoma showing metaplastic change analogous to the development of squamous cell nests of the pars tuberalis from adenohypophyseal endocrine cells.