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

Abstract

Silent corticotroph tumors are composed of corticotroph cells, but do not manifest any biochemical or clinical evidence of hypercortisolism. A choristoma is a benign, congenital proliferation of histologically mature tissue elements normally not present at the site of occurrence. The existence of adrenocortical cells within the pituitary gland, which can be explained as a choristoma, is a very rare entity, and the co-occurrence of these two entities have only been reported in few cases. We report an 11-year-old boy with central hypothyroidism. On cranial magnetic resonance imaging a pituitary tumor was detected, and histopathological studies led to a diagnosis of an adrenal choristoma and a silent corticotroph tumor in the pituitary gland. The presence of adrenocortical cells were confirmed by positive calretinin, inhibin and Melan A staining, and the corticotroph cells by immunohistochemistry demonstrating adrenocorticotropic hormone positivity. Herein, we report the fourth and the youngest case of silent corticotroph tumor with adrenocortical choristoma in the literature. Even though the underlying mechanism is not fully understood, suggested mechanisms are discussed.

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

“…Adrenal cortical choristomas are composed of adrenal cortical cells with bland cytomorphology, and they occur either isolated or in aggregates within normal or neoplastic cell populations (e.g., corticotroph PitNET) of the involved organ [ 50 , 51 ]. The mechanism underlying their development is incompletely understood.…”

“…The mechanism underlying their development is incompletely understood. It may be the result of misplacement of adrenal tissue far from its anatomic location during embryogenesis [ 50 , 51 ]. Alternatively, at least in pituitary locations, a role of SF1 (a transcription factor implicated in both pituitary and adrenal cortex functions), might be envisaged, possibly acting on uncommitted stem cells within the sella [ 51 ].…”

“…Rare locations of adrenal rests include the placenta [36], broad ligament [22,37,38], retroperitoneum [39,40], urinary bladder and prostate [41], groin [42,43], choroid plexus [44], spine [45][46][47], and lung [48,49]. Among various locations, adrenal cortical choristomas have also been described within TPIT-lineage pituitary neuroendocrine tumors (corticotroph PitNETs) [50,51].…”

“…com. Reproduced from Juhlin CC, Bertherat J, Giordano cell populations (e.g., corticotroph PitNET) of the involved organ [50,51]. The mechanism underlying their development is incompletely understood.…”

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.…”