The Cushing Syndromes: Changing Views of Diagnosis and Treatment

Abstract

Cushing's syndrome is the common clinical presentation of three unique disorders that give rise to hypercortisolism. In most cases neoplasms underly each of these disorders. Clinical features are highly variable and not accounted for by cortisol alone; indeed, the multihormonal basis for much of the clinical syndrome remains uncertain. Demonstration of sustained, excessive cortisol production is essential and depends on a pattern of repeated measurements and several different procedures. Plasma adrenocorticotropin, although not helpful in establishing the diagnosis, has proved valuable in differentiating the three major entities that cause hypercortisolism. The renewed significance of pituitary microadenomas and their improved detection by sella tomography has accompanied recent, impressive advances in transsphenoidal microsurgery. This may become the preferred treatment for pituitary Cushing's syndrome in the adult, particularly where the appropriate equipment and skills are available; for children, external pituitary irradiation seems to offer safe and effective therapy.

Ectopic ACTH Syndrome in APUD Tumors

“…Treatment for these tumors has been primary resection or, if inoperable as in small cell carcinoma of the lung, chemotherapy for the primary tumor [6]. Isolated case reports [1,7] of primary and metastatic apudomas producing hypoglycemia, calcitonin, and 5-HIAA that responded favorably to chemotherapy encouraged similar treatment of the patients reported here.…”

The Androgen Excess and PCOS Society criteria for the polycystic ovary syndrome: the complete task force report

“…Cushing's syndrome, secondary to adrenal neoplasms, is discussed further in the next section (see below). Overall, in women with Cushing's syndrome menstrual irregularities are seen in 80% to 100%, hirsutism in 60% to 100%, and acne is present in 40% to 50% (271)(272)(273)(274). In patients with Cushing's syndrome hirsutism arises either from the exaggerated secretion of adrenal androgens in response to excess ACTH stimulation, which usually results in mild hair growth, or because of direct excessive secretion of adrenal androgens by an adrenocortical carcinoma, if which case hair growth may be more severe (275).…”

Cushing’s Disease: 50 Years Later

“…The identification of a microadenoma measuring a few mm became feasible. Selective microadenomectomy without permanent pituitary failure has become the ideal treatment of Cushing's disease (Gold, 1979). Within the last decade, pituitary microsurgical exploration has been advocated and has emerged as the primary treatment despite a radiographically normal sella turcica (Hardy, 1973(Hardy, , 1975Bigos et al, 1977Bigos et al, , 1980Salassa et al, 1978;Tyrell et al, 1978).…”