Endocrine Abstracts

Section 1: Case history: A 60 year-old lady presented with a 4-year history of progressively increasing virilisation on the background of right salpingo-oophorectomy for ectopic pregnancy in 1984 (no histology available).

Section 2: Investigations: Testosterone 23.6 nmol/l (NR < 1.7), LH 16 IU/l (7.7–58.5), FSH 33 IU/l (25.8–134.8), androstenedione 3.2 nmol/l (NR 1.4–14.3), DHEAs 1.2 umol/l (0.5–5.6), 17-OHP 2.7 nmol/l (1–4.5), cortisol 372 nmol/l (133–537 nmol/l), plasma metanephrines, CEA and CA-125 were likewise all normal. Urine steroid profile showed very high androgen metabolites, but no characteristic pattern of adrenal carcinoma. Overall, basal biochemistry indicated an ovarian source. The low dose dexamethasone suppression test failed to suppress the testosterone levels, indicating tumourous cause of raised testosterone levels. Treatment with GnRH-analog achieved complete suppression of LH, FSH and testosterone levels, indicating that pathological testosterone secretion was gonadotrophin dependent and, again pointing towards an ovarian source. However, laparoscopic left salpingo-oophorectomy showed a normal postmenopausal ovary, with no evidence of tumour or hyperthecosis

Section 3: Results and treatment: PET scan showed increase FDG uptake in a slightly bulky right adrenal gland, but adrenal protocol CT scan failed to definitively identify an adenoma. Pelvic MRI and transvaginal ultrasound indicated the possibility of an ovarian remnant within the right broad ligament, but laparoscopic removal found only fibrous adipose tissue. In view of ongoing high testosterone levels, imaging was done, which did not indicate towards any obvious cause. We therefore proceeded to sampling of ovarian and adrenal veins (see table below), which indicated right adrenal source of testosterone, and she then underwent right adrenalectomy that achieved biochemical cure (testosterone <1.0 nmol/l). Our hypothesis is that the right adrenal contains tumourous tissue that expressed the LHCG-receptor (Luteinizing hormone choriogonadotropin), but histology and staining remains in progress.

Section 4: Conclusions: This is an unusual and complex case of postmenopausal hyperandrogenism with unclear cause of persistently raised testosterone levels, which was only unravelled by the adrenal ovarian venous sampling, which we have never previously performed in Newcastle, that indicating the right adrenal as the source.

• Identification of the source of androgen excess based solely on testosterone, DHEAS and androstenedione may be misleading

• Pure testosterone-secreting adrenal adenomas are extremely rare

• In some selected cases of post-menopausal hyperandrogenism, biochemically proven to be gonadotrophin-dependent, imaging of the adrenals is appropriate and adrenal/ovarian venous sampling may be required.