[Nelson's syndrome concomitant with Cushing's syndrome]. 1991

A Vicente Delgado, and T Lucas Morante, and C Páramo Fernández, and J Estrada García, and M Marazuela Aspiroz, and C de la Cuerda Compés
Servicio de Endocrinología, Hospital Puerta de Hierro, Madrid.

A case is presented of Nelson's Syndrome concomitant with Cushing's Syndrome in a female patient who underwent five years earlier bilateral adrenalectomy due to Cushing's Disease. Together with hyperpigmentation, very elevated ACTH, and intrasellar mass in CT scan, plasma cortisol levels without rythm and not suppressible were observed as well as increased cortisol in urine. The macroadenoma was resected though the sphenoid and later hypophysis radiotherapy was given, with a clinical remission and biochemical improvement of the syndrome. At the present time, slightly elevated ACTH levels persist, with panhypopituitarism and empty sella turcica. The clinical picture is described and a literature search is carried out.

UI MeSH Term Description Entries
D009347 Nelson Syndrome A syndrome characterized by HYPERPIGMENTATION, enlarging pituitary mass, visual defects secondary to compression of the OPTIC CHIASM, and elevated serum ACTH. It is caused by the expansion of an underlying ACTH-SECRETING PITUITARY ADENOMA that grows in the absence of feedback inhibition by adrenal CORTICOSTEROIDS, usually after ADRENALECTOMY.
D010913 Pituitary-Adrenal System The interactions between the anterior pituitary and adrenal glands, in which corticotropin (ACTH) stimulates the adrenal cortex and adrenal cortical hormones suppress the production of corticotropin by the anterior pituitary. Pituitary Adrenal System,Pituitary-Adrenal Systems,System, Pituitary-Adrenal,Systems, Pituitary-Adrenal
D003480 Cushing Syndrome A condition caused by prolonged exposure to excess levels of cortisol (HYDROCORTISONE) or other GLUCOCORTICOIDS from endogenous or exogenous sources. It is characterized by upper body OBESITY; OSTEOPOROSIS; HYPERTENSION; DIABETES MELLITUS; HIRSUTISM; AMENORRHEA; and excess body fluid. Endogenous Cushing syndrome or spontaneous hypercortisolism is divided into two groups, those due to an excess of ADRENOCORTICOTROPIN and those that are ACTH-independent. Cushing's Syndrome,Hypercortisolism,Syndrome, Cushing,Syndrome, Cushing's
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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