“Tertiary” adrenocortical tumor and non-rhythmic ACTH excess in untreated congenital adrenal hyperplasia(CAH)

Abstract

A 60-y-old single woman(46, XX)was admitted Oct. 1975 with evidence of a left adrenal tumor on IVP. The history and finding of urogenital sinus and clitoral hypertrophy suggested CAH. Preoperative steroid excretion: 17-kgs 218, 17-ks 91, pregnanetriol 60, DHEA 1.9, 11-OH-andro 21, etio 32mg/24th, resp. 94, 28, 14,0. 3,5 and 9mg/24h after a standard oral dexamethasone test. Basal CSR 30mg/24h, plasma testosterone 202ng%, plasma ACTH 62 (8h), 54(17h)pg/ml. A large adrenal tumor(410gm)without meta stases was removed;normal internal genitalia, including ovarian histology. Steroid excretion fell approx. 50%, but remained poorly suppressable. After removal of the right adrenal(58gm, nodular hyperplasia)steroid excretion reached very low values. Multiple plasma ACTH assays during oral cortisol substitution, (Dexter et al. JCS 30, 573, 1970)revealed little fluctuation (max. 280, min. 180pg/ml), suggesting pituitary microadenoma. These findings support the need for continued ACTH-suppression in CAH.

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