Abstract
A 33 year old woman presented with recurrent Cushing’s disease 4 years after complete remission induced by pituitary surgery. On relapse she exhibited the unusual pattern of elevated indices of Cortisol secretion with markedly suppressed serum DHEA-S; urinary 17-ketosteroid excretion was also below the normal range. Biochemical testing was otherwise consistent with ACTH-mediated hyper-cortisolism, and adrenal histopathology showed bilateral hyperplasia with no evidence of tumor. This case illustrates that serum DHEA-S is not an infallible guide to the differential diagnosis of Cushing’s syndrome, and it supports the existence of a pituitary-secreted adrenal androgen stimulating factor that is distinct from ACTH.
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Tyrrell J.D., Baxter J.D. The Adrenal Cortex. In: Felig P., Baxter J.D., Broadus A.E., Frohman L.A. (Eds)., Endocrinology and Metabolism. McGraw-Hill, New York 1987, p. 511.
Yamaji T., Ibayashi H. Plasma dehydroepiandrosterone sulfate in normal and pathological conditions. J. Clin. Endocrinol. Metab. 29: 273, 1969.
Ashcraft M.W., Van Herle A.J., Vener S.L., Geffner D.L. Serum Cortisol levels in Cushing’s syndrome after low and high dose dexamethasone suppression. Ann. Intern. Med. 97: 21, 1982.
Hauffa B.P., Kaplan S.L, Grumbach M.M. Dissociation between plasma adrenal androgens and Cortisol in Cushing’s disease and ectopic ACTH-producing tumour: relation to adrenarche. Lancet 1: 1373, 1984.
Yamaji T., Ishibashi M., Sekihara H., Itabashi A., Yanaihara T. Serum dehydroepiandrosterone sulfate in Cushing’s syndrome. J. Clin. Endocrinol. Metab. 59: 1164, 1984.
Gelfand R.A. Cushing’s disease associated with ovarian dysgenesis. Am. J. Med. 77: 1108, 1984.
Parker L.N., Odell W.D. Control of adrenal androgen secretion. Endocr. Rev. 1: 392, 1980.
De Peretti E., Forest M.G. Unconjugated dehydroepiandrosterone plasma levels in normal subjects from birth to adolescence in humans: the use of a sensitive radioimmunoassay. J. Clin. Endocrinol. Metab. 43: 982, 1976.
Cutler Jr G.B., Loriaux D.L. Andrenarche and its relationship to the onset of puberty. Fed. Proc. 39: 2384, 1980.
Abraham G.E., Buster J.E., Kyle F.W., Corrales P.C., Teller R.C. Radioimmunoassay of plasma pregnenolone, 17-hydroxypregnenolone and dehydroepiandrosterone under various physiological conditions. J. Clin. Endocrinol. Metab. 37: 140, 1973.
Cattaneo S., Forti G., Fiorelli G., Barbieri U., Serio M. A rapid radioimmunoassay for determination of dehydroepiandrosterone sulfate in human plasma. Clin. Endocrinol. (Oxf.) 4: 505, 1975.
Pont A., Gutierrez-Hartman A. Cushing’s disease: recurrence after a surgically induced remission. Arch. Intern. Med. 139: 938, 1979.
Bigos S.T., Somma M., Rasio E., Eastman R.C., Lanthier A., Johnston H.H., Hardy J. Cushing’s disease: management by transsphenoidal pituitary microsurgery. J. Clin. Endocrinol. Metab. 50: 348, 1980.
Aron D.C., Findling J.W., Fitzgerald P.A., Forsham P.H., Wilson C.B., Tyrrell J.B. Cushing’s syndrome: problems in management. Endocr. Rev. 3: 229, 1982.
Styne D.M., Grumbach M.M., Kaplan S.L., Wilson C.B., Conte F.A. Treatment of Cushing’s disease in childhood and adolescence by transsphenoidal microadenomectomy. N. Engl. J. Med. 310: 889, 1982.
Boggan J.E., Tyrrell J.B., Wilson C.B. Transsphenoidal microsurgical management of Cushing’s disease. J. Neurosurg. 59: 195, 1983.
Burch W.M. Cushing’s disease: a review. Arch. Intern. Med. 145: 1106, 1985
Carpenter P.C. Cushing’s syndrome: update of diagnosis and management. Mayo Clin. Proc. 61: 49, 1986.
Cappa M., Stoner E., DiMartino-Nardi J., Pang S., Temeck J., New M.I. Recurrence of Cushing’s disease in childhood after radiotherapy-induced remission. Am. J. Dis. Child. 141: 736, 1987.
Lamberts S.W.J., Klign J.G.M., de Jong F.H. The definition of true recurrence of pituitary-dependent Cushing’s syndrome after transsphenoidal operation. Clin. Endocrinol. (Oxf.) 26: 707, 1987.
Nakane T., Kuwayana A., Watanabe M., Takahashi T., Kato T., Ichihaara K., Kageyama N. Long term results of transsphenoidal adenomectomy in patients with Cushing’s disease. Neurosurgery 21: 218, 1987.
Kappas A., Pearson O.H., West C.D., Gallagher T.F. A study of idiopathic hirsutism: a transitional adrenal abnormality. J. Clin. Endocrinol. Metab. 16: 57, 1956.
Perloff W.H., Hadd H.E., Channick B.J., Nodine J.H. Hirsutism. Arch. Int. Med. 100: 981, 1957.
Nichols T., Nugent C.A., Tyler F.H. Glucocorticoid suppression of urinary testosterone excretion in patients with idiopathic hirsutism. J. Clin. Endocrinol. Metab. 26: 79, 1966.
Avgerinos P.C., Cutler Jr G.B., Tsokos G.C., Gold P.W., Feuillan P., Gallucci W.T., Pillemer S.R., Loriaux D.L., Chrousos G.P. Dissociation between Cortisol and adrenal androgen secretion in patients receiving alternate day prednisone therapy. J. Clin. Endocrinol. Metab. 65: 24, 1987.
Cutler Jr G.B., Davis S.E., Johnsonbaugh R.E., Loriaux D.L. Dissociation of Cortisol and adrenal androgen secretion in patients with secondary adrenal insufficiency. J. Clin. Endocrinol. Metab. 49: 604, 1979.
Albright F. Osteoporosis. Ann. Int. Med. 27: 861, 1947.
Mills I.H., Brooks R.V., Prunty F.T.G. The relationship between the production of Cortisol and of androgen by the human adrenal. In: Currie A., Symington T., Grant J. (Eds.), The Human Adrenal Cortex. Livingstone, Edinburgh, 1962, p. 204.
Grumbach M.M., Richards G.E., Conte F.A., Kaplan S.L. Clinical disorders of adrenal function and puberty: an assessment of the role of the adrenal cortex in normal and abnormal puberty in man and evidence for an ACTH-like pituitary adrenal androgen stimulating hormone. In: James V.H.T. (Ed.), The Endocrine Function of the Human Adrenal Cortex. Academic Press, New York, 1978, p. 583.
Parker L.N., Odell W.D. Evidence for existence of cortical androgen stimulating hormone. Am. J. Physiol. 23: E616, 1979.
Parker L.N., Lifrak E.T, Odell W.D. A 60,000 MW human pituitary glycopeptide stimulates adrenal androgen secretion. Endocrinology. 113: 2092, 1983.
Albertson B.D., Hobson W.C., Burnett B.S., Turner P.T., Clark R.V., Schiebinger R.J., Loriaux D.L., Cutler Jr G.B. Dissociation of Cortisol and adrenal androgen secretion in the hypophysectomized, adrenocorticotropin-replaced chimpanzee. J. Clin. Endocrinol. Metab. 59: 13, 1984.
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Dr Gelfand was supported by USPHS grant #DK 38578. and Dr. Louard by USPHS Training Grant #DK07476.
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Louard, R.J., Gelfand, R.A. Recurrent Cushing’s disease with low adrenal androgen production. J Endocrinol Invest 14, 965–969 (1991). https://doi.org/10.1007/BF03347123
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DOI: https://doi.org/10.1007/BF03347123