Virilizing adrenocortical tumors in adult women. Report of 10 patients, 2 of whom each had a tumor secreting only testosterone

Abstract

Background. Virilizing adrenocortical tumors are uncommon in adult women. These lesions generally secrete dehydroepiandrosterone (DHEA) and dehydroepiandrosterone sulfate (DHEAS), but not testosterone, which usually is produced by ovarian tumors. Exceptionally, adrenal growths may give off testosterone and no other assessable androgen. The detection of the site of excess testosterone yield is paramount for proper surgery. The true nature of the growth often is unpredictable, even at the time the pathologist examines the surgical specimen. Methods. The workup in a virilized adult woman relies on biochemical tests such as 24‐hour urinary 17‐KS and 17‐OHCS levels and plasma corticosteroid levels (testosterone, DHEA, DHEAS, and androstenedione), and on modern imaging studies such as ultrasonography, computed tomography, and digital angiography. Results. Among a series of 190 adrenal tumors collected in the last 30‐year period, only 10 virilizing growths (5.3%) were detected. Two cases of virilization mixed with cushingoid features were observed. In two other cases, reported in detail, the tumor secreted testosterone only, without other assessable androgens. Seven of the 10 tumors were malignant. Conclusions. In cases of tumors secreting testosterone only, high‐resolution imaging has contributed significantly in pinpointing the site of the growth, whereas dynamic hormone testing, using selective stimulation or suppression studies, has been misleading. The malignant nature of the growth may be revealed only by the presence of metastases, because pleomorphism and capsular and vascular invasion have been detected histologically in clinically benign tumors. The prognosis for large tumors usually is dismal.

Virilization caused by an ectopic adrenal tumor located behind the iliopsoas muscle

“…Virilizing adrenal tumors may not respond to dexamethasone suppression or ACTH stimulation because of lack of ACTH receptors or postreceptor anomalies (21,22). Some investigators suggest that dynamic tests of androgen production are not very helpful and can occasionally be misleading (20), as in our case.…”

“…There have been reports of adenomas of adrenocortical origin that were producing only T (19). In a series of 190 adrenal tumors, 10 (5.3%) were virilizing, two of them producing only T (20). A serum T level 2.5-3 times above the upper limit of normal is strongly suggestive of the presence of an androgen-producing tumor (2,9).…”

Hyperandrogenism in menopause: a case report and literature review

“…Biochemical testing aims to differentiate between adrenal and ovarian sources. Isolated elevated testosterone, as in this case, is typically presumed to be of ovarian origin, although it has also been reported in the case of adrenal tumors [18, 19, 21, 24, 25]. Imaging is thus appropriately directed towards the ovaries and adrenals.…”

Florid hyperandrogenism due to a benign adrenocortical adenoma

“…Therefore, this case illustrates that even adrenal adenomas can cause very high androgen levels and hence the androgen levels per se cannot fully discriminate between adrenal adenomas and carcinomas. Notwithstanding, given the inherent difficulties with differentiating adenomas and carcinomas histopathologically6 and following advice from a reference centre on adrenal disease (Professor Wiebke Arlt, Birmingham, UK, personal communication), we have implemented and propose close monitoring in all such cases with clinical, biochemical (serum androgens coupled with urinary steroid profiling if available) and radiological surveillance 7 8. In particular, we would advocate clinical, biochemical and cross-sectional imaging surveillance every 6 months.…”