Abstract
We report the third case of a composite corticotroph pituitary adenoma with interspersed adrenocortical cells. The 16-year-old male patient presented with findings of delayed growth and pubertal arrest. In contrast to the previous two cases, this patient's tumor showed evidence of function as demonstrated by an elevated urinary cortisol level. Imaging studies revealed a sellar mass that was excised transsphenoidally. Histologic examination revealed a composite tumor composed of distinct populations of large and small cells. The small cell population was PAS-positive and immunohistochemically positive for adrenocorticotrophic hormone. The large cell population had abundant vacuolated cytoplasm, was negative for PAS and adrenocorticotrophic hormone, and stained positively for a panel of markers found in steroid-producing adrenocortical cells. Both populations showed evidence of proliferation as manifest by the presence of MIB-1 positive cells. Ultrastructural examination confirmed the presence of distinct populations of large adrenocortical cells and small corticotrophs, with intercellular junctions between the 2 cell types. The intimate relationship between the 2 cell population and the activated appearance of the adrenocortical cells suggests the possibility of a paracrine relationship between the two cell types. The identification of 3 patients with sellar tumors demonstrating strikingly similar morphological and ultrastructural features, and all occurring in the second decade of life, suggests that this represents a distinct pathologic entity.
Similar content being viewed by others
References
Thapar K, Kovacs K. Neoplasms of the sellar region, Russell & Rubinstein' Pathology of Tumors of the Nervous System. Oxford University Press, 1998:561–677.
Lloyd RC, Chandler WF, McKeever PE, Schteingart DE. The spectrum of ACTH-producing pituitary lesions. Am J Surg Pathol 1986;10:618–626.
Oka H, Kameya T, Sasano H, Aiba M, Kovacs K, Horvath E, Yokota Y, Kawano,Yada K, Pituitary choristoma composed of corticotrophs and adrenocortical cells in the sella turcica. Virchows Arch 1996;427:613–617.
Coire CI, Horvath E, Kovacs K, Smyth HS, Sasano H, Iino K, Feig DS. A composite silent corticotroph pituitary adenoma with interspersed adrenocortical cells: Case report. Neurosurgery 1998;42:650–654.
Shi S-R, Imam A, Young L, Cote R, Taylor CR. Antigen retrieval immunohistochemistry under the influence of pH using monoclonal antibodies. J Histochem Cytochem 1995; 43:193–201.
Sasano H, Shizawa S, Suzuki T, Takayama K, Fukaya T, Morohashi K, Nagura H. Transcription factor adrenal 4 binding protein as a marker of adrenocortical malignancy. Hum Pathol 1995;26:1154–1156.
Sasano H, Mason JI, Sasano N, Nagura H. Immunolocalization of 3 beta-hydroxysteroid dehydrogenase in human adrenal cortex and its disorders. Endocr Pathol 1990;20:94–101.
Sasano H, Sato J, Shizawa S, Nagura H, Coughtrie MWH. Immunolocalization of dehydroepiandrosterone sulfotransferase in normal and pathological human adrenal gland. Mod Pathol 1995;8:891–896.
Sasano H, White PC, New MI, Sasano N. Immunohistochemical localization of cytochrome P-450 c21 in human adrenal cortex and its relation to endocrine function. Hum Pathol 1988;19:181–185.
Sasano H, Sasano N, Okamoto M. Immunohistochemical demonstration of cholesterol sidechain cleavage cytochrome P-450 in bovine and human adrenal. Pathol Res Pract 1989; 184:337–342.
Wiener MF, Dallgaard SA. Intracranial adrenal gland: A case report. Arch Pathol 1959;67:.228–233.
Mindermann T, Wilson CB. Pediatric pituitary adenomas. Neurosurgery 1995;36:259–269.
Abe T, Ludecke DK, Saeger W. Clinically nonsecreting pituitary adenomas in childhood and adolescence. Neurosurgery 1998;42:744–751.
Magiakou MA, Mastorakos G, Oldfield EH, Gomez MT, Doppman JL, Cutler GB, Nueman LK, Chrousos GP. Cusing' syndrome in children and adolescents. N Engl J Med 1994;331:629–636.
Reibord H, Fisher ER. Electron microscopic study of adrenal cortical hyperplasia in Cushing' syndrome. Arch Pathol 1968;86:419–426.
Kovacs K, Horvath E, Singer W, Lillenfield H. Fine structure of adrenal cortex in ectopic ACTH syndrome. 1977;69: 94–102.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Albuquerque, F.C., Weiss, M.H., Kovacs, K. et al. A Functioning Composite ‘Corticotroph’ Pituitary Adenoma with Interspersed Adrenocortical Cells. Pituitary 1, 279–284 (1999). https://doi.org/10.1023/A:1009962627216
Issue Date:
DOI: https://doi.org/10.1023/A:1009962627216