CUSHINGS-DISEASE IN A CHILD CAUSED BY A CORTICOTROPIN-RELEASING HORMONE-SECRETING INTRASELLAR GANGLIOCYTOMA ASSOCIATED WITH AN ADRENOCORTICOTROPIC HORMONE-SECRETING PITUITARY-ADENOMA

CUSHING'S DISEASE RESULTING from intrasellar corticotropin-releasing h ormone (CRH)-secreting gangliocytomas is very rare, and only two such cases have been reported in the literature to date. The authors presen t a third case in which an adrenocorticotropic hormone-secreting pitui tary adenoma was found in addition to a gangliocytoma in a 10-year-old girl with clinical and endocrinological symptoms of Cushing's disease . Computed tomographic and magnetic resonance imaging scans showed a s uprasellar and parasellar tumor. A green-colored, heterogeneous tumor and a small adenoma were removed transsphenoidally. Histological exami nation revealed a large gangliocytoma immunoreactive for CRH and a sma ll, mucoid cell pituitary adenoma immunoreactive for ACTH. This is the first case of such a tumor causing Cushing's disease in a child. It m ight exemplify induction of an ACTH-secreting pituitary adenoma by mea ns of chronic overstimulation of CRH.