Profile and outcome of pure androgen-secreting adrenal tumors in women: experience of 21 cases

Abstract

Background The aim of the study was to determine the outcome and possible prognostic factors of pure androgen-secreting adrenal tumors (PASATs).Methods In a review of 801 adrenal operations from 1970 through 2003, 21 women with PASATs were divided into 2 groups, benign and undetermined tumors (Weiss score < or = 3) (BT = 11) and malignant tumors (Weiss > or =4 or nonresectable) (MT = 10).Results In both groups, age at presentation was similar. There were no differences concerning type of secretion, but increase in testosterone level was 2.6-fold greater in malignant tumors (MT) than benign tumors (BT). Imaging studies allowed diagnosis of malignancy in 4 of 10 MT. Size and weight were greater for MT than for BT (average, 13.7 vs 9.2 cm and 1462 vs 206 g). At follow-up (median, 17 y; range, 1 to 33 y) 2 of 11 patients with BT died of unrelated causes, and 9 of 11 are alive without recurrence; 5 of 10 patients with MT died of disease, and 3 are alive with disease; 7 of those 8 patients had stage III or IV disease and/or had a Weiss score of 6 or greater. Two patients with MT are alive without disease; both were Weiss 7, stage II and received mitotane postoperatively.Conclusions PASATs of undetermined benign condition share the good prognosis of BT. Weiss score is diagnostic of malignancy. MacFarlane classification determines the prognosis, and long-term disease-free survivors at stages III/IV are never observed after operation only. Postoperative mitotane therapy might be beneficial at stage II.