Background. Adrenal tumors that secrete androgens exclusively are extraordinarily rare. The aim of this study was to characterize patients with pure androgen-secreting adrenal tumors. Methods. A retrospective chart review from January 1946 through November 2002 identified 11 female patients with pure androgen-secreting adrenal tumors. Results. The mean age was 23.4 years (range, 1-52). The most common presenting symptoms were hirsutismz acne, and clitoral enlargement. Elevated 17-ketosteroids were found in seven of nine tested patients. Computed tomogram, ultrasound, or both localized tumors in six of seven patients. All tumors were surgically resected, one laparoscopically, all without complications. Five of the 11 tumors were malignant. Mean weight and mean maximal diameter for benign and malignant tumors were 44 g and 4.2 cm and 232 g and 9.8 cm, respectively. Mean hospital stay was 8.5 days, with excess androgen production resolved in all patients. Recurrence and disease-related death occurred in only one patient who had pulmonary metastases at diagnosis. The remaining patients had no recurrence of tumor at mean follow-up of 11.7 years (range, 0.5-32 years). Conclusions. Pure androgen-producing tumors are extremely rare. Approximately 50% are benign, and surgical resection provides excellent treatment if the tumors are not metastatic at the time of diagnosis.
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