Silent Corticotroph Adenoma with Adrenal Cortical Choristoma: a Rare but Distinct Morphological Entity

Abstract

This report describes a case of pituitary adenoma with interspersed adrenal cortical cells. The pituitary cells were confirmed to be corticotrophs with Tpit and adrenocorticotropic hormone immunohistochemistry, whereas the adrenal cortical cells were verified to be such with steroidogenic factor-1 (SF-1), inhibin, calretinin, and Melan A staining. The presence of normal adrenal cortical cells in the heterotopic location of the sella fulfills the definition of choristoma. The origin of adrenal cortical cells within a pituitary adenoma remains unexplained. The important role of SF-1 in both pituitary and adrenal cortex may explain a relationship that supports the possibility of an abnormal proliferation and differentiation of uncommitted mesenchymal stem cells within the sella. However, it remains possible that misplaced adrenal cortical cells derived during embryogenesis give rise to this rare but distinct morphological entity that can pose a difficult diagnostic dilemma. The approach to differential diagnosis is discussed.

Silent Corticotroph Tumor with Adrenocortical Choristoma in an Eleven-year-old Boy

“…It has been demonstrated that SCAs can be more aggressive than any other clinically nonfunctioning adenomas with a higher prevalence of cavernous sinus invasion and a higher rate of recurrence ( 5 , 13 ). In the other three case reports ( 7 , 10 , 11 ), data with regard to postoperative follow-up period were lacking, but the tumor in our patient recurred after six years.…”

“…Similar to our case, all four previously reported tumors were macroadenomas (tumors exceeding 1.0 cm on MRI studies). Three of the four cases were diagnosed in the teenage period (16-18 years of age), while only one was adult ( 11 ). Our 11-year-old patient was the youngest case reported to date.…”

“…The adult patient was a 35-year-old male patient, and he had secondary hypothyroidism, hypogonadotropic hypogonadism and “low insulin-like growth factor-1 with growth hormone” ( 11 ). The other three teen-aged patients had either growth retardation (basal GH and insulin-like growth factor-1 levels were reported to be low) or delayed pubertal development.…”

“…Adrenocortical choristomas have been identified in a wide variety of other non-steroidogenic tissues, including kidney, lung, spinal canal and the leptomeningeal surface in the cranium ( 7 , 8 ). However only four previous reports have described the occurrence of interspersed adrenal cortical cells in three SCAs and in one clinically functioning CA ( 7 , 9 , 10 , 11 ).…”

Pituitary corticotroph tumour with adrenocortical cells: A distinct clinicopathologic entity with unique morphology and methylation profile

“…[1][2][3][4][5][6] In the sella turcica, several reports have described adrenocortical cells in corticotroph macrotumours, which exclusively affect young adults and are almost always hormonally inactive. [7][8][9][10][11][12] This phenomenon has been regarded as evidence of an adrenocortical choristoma within the sella. We describe a functioning corticotroph tumour with admixed adrenocortical cells and provide immunohistochemical and novel methylation profiling data.…”