Mammosomatotroph hyperplasia associated with acromegaly and hyperprolactinemia in a patient with the McCune-Albright syndrome. A histologic, immunocytologic and ultrastructural study of the surgically-removed adenohypophysis.
An 11-year-old girl, with the McCune-Albright syndrome, exhibited fibrous dysplasia of several bones, skin pigmentation, precocious puberty, growth hormone hypersecretion, acromegaly and hyper-prolactinemia. Histologic, immunocytologic and ultrastructural investigation of the surgically-removed pituitary showed massive mammosomatotroph hyperplasia. Since no adenoma was found, the abundance of these bihormonal cells, capable of producing both growth hormone and prolactin, was implicated in the causation of growth hormone and prolactin excess. Somatoliberin overproduction and/or somatostatin and dopamine deficiency could not account for the hypophysial abnormality, since changes in secretory rates of these hypothalamic hormones would lead to proliferation of mature somatotrophs and lactotrophs, rather than mammosomatotrophs. In our patient, a congenital hypothalamic malfunction might have been accompanied by hypersecretion of an unidentified releasing factor, resulting in pathologic differentiation of the pituitary and mammosomatotroph hyperplasia. Alternatively, mammosomatotroph hyperplasia may have been due to an inherent genetic or embryonic defect affecting primarily the pituitary. According to this interpretation, the pituitary lesion represented yet another developmental error in the setting of the McCune-Albright syndrome.
D000172:Acromegaly / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005357:Fibrous Dysplasia of Bone / Q000150:complications*; D005359:Fibrous Dysplasia, Polyostotic / Q000097:blood / Q000150:complications* / Q000473:pathology; D013006:Growth Hormone / Q000032:analysis / Q000097:blood; D006651:Histocytochemistry; D006801:Humans; D006965:Hyperplasia / Q000209:etiology; D010903:Pituitary Gland, Anterior / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D011388:Prolactin / Q000032:analysis / Q000097:blood*
D011388:Prolactin; D013006:Growth Hormone