Patterns of Urinary Excretion of Steroids in Cushing's Syndrome1

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Abstract
Inthe differential diagnosis of 20 patients with Cushing''s syndrome a marked increase in urinary neutral 17-KS (17-keto steroid) excretion favored adreno-cortical carcinoma, low values an adenoma, whereas ACTH (adreno-corticotropic hormone) and Metyrapone (Su-4885) hyper-responsiveness and dexamethasone suppressibility suggested nontumorous hyperplasia. None of the above, however, could be relied upon to establish the correct diagnosis unequivocally. Chromatographic separation of neutral 17-KS in 11 patients confirmed abnormal patterns of excretion of A (andro-sterone), E (stiocholanolone), KE (ketoetiocholanolone), OHE (11-hydroxy-etiocholanolone) and OHA (11-hydroxyandrosterone), but failed to prove of diagnostic value. DHEA (11-dehydroepiandrosterone) was markedly elevated in 3 out of 3 patients with carcinoma as well as in one out of 3 with adenoma and 2 out of 5 patients with adrenocortical hyperplasia. One patient with an adenoma and one with hyperplasia had normal base line excretion but excessive ACTH responses. Two patients with hyperplasia associated with extra-adrenal neoplasia had normal DHEA excretion at all times. Urinary "pregnanediol" and "pregnanetriol," determined as sulfuric acid chromogens after chromatographic separation, were measured in 17 patients. Excretion was elevated in the control collections in 4 out of 4 patients with carcinoma tested. Base line levels were normal in 4 out of 6 patients with hyperplasia and 2 out of 2 with hyperplasia secondary to extra-adrenal carcinoma, but ACTH responses were elevated in 7 out of 8. In the adenoma group, in contrast, 4 out of 5 had normal control values and all had normal ACTH responses. The studies suggest that the urinary excretion and response to ACTH of pregnanediol and pregnanetriol may be a helpful test in determining the etiology of Cushing''s syndrome.