Clinical Overview > Condition/ Syndrome

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  • Ovarian tumour x
  • Hyperandrogenism x
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E Bahaeldein Endocrinology, South Tipperary General Hospital, Clonmel, Ireland

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M J Brassill Endocrinology, South Tipperary General Hospital, Clonmel, Ireland

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Summary

Postmenopausal hyperandrogenism is a relatively rare diagnosis resulting from excess androgen production from the adrenals or ovaries. The exclusion of malignant causes is a priority. Laboratory tests and imaging are utilised to help differentiate the source of excess androgens. We report two cases of postmenopausal hyperandrogenism in women aged 75 and 67 years. Both cases presented with clinical features suggestive of hyperandrogenism which had developed gradually over the previous 2 years. Laboratory investigations confirmed a significant elevation in their serum testosterone levels. In both cases, imaging did not reveal any abnormality of the adrenals or ovaries. To help differentiate an adrenal vs ovarian source a single-dose GnRH analogue was given with measurement of testosterone and gonadotrophin levels pre and post. The reduction in gonadotrophins achieved by the GnRH analogue resulted in suppression of testosterone levels which suggested an ovarian source. Both patients proceeded to bilateral oophorectomy. Histology revealed a benign hilus cell tumour in one case and a benign Leydig cell tumour in the other.

Learning points:

  • A key part of the work-up of postmenopausal hyperandrogenism is to differentiate between an adrenal or an ovarian source of excess androgens;

  • Imaging may not identify small ovarian tumours or hyperthecosis and may also identify incidental adrenal masses which are non-functioning;

  • Current guidelines suggest ovarian and adrenal venous sampling when imaging is inconclusive but this requires technical expertise and has a high failure rate;

  • GnRH analogue use can successfully confirm ovarian source and should be considered as a diagnostic tool in this setting.

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Khaled Aljenaee Department of Endocrinology, Royal College of Surgeons in Ireland, Connolly Hospital Blanchardstown, Dublin, Ireland

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Sulaiman Ali Departments of Endocrinology

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Seong Keat Cheah Department of Endocrinology, Royal College of Surgeons in Ireland, Connolly Hospital Blanchardstown, Dublin, Ireland

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Owen MacEneaney Histopathology, Mater Misericordiae University Hospital, Dublin, Ireland

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Niall Mulligan Histopathology, Mater Misericordiae University Hospital, Dublin, Ireland

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Neil Hickey Department of Radiology, Connolly Hospital Blanchardstown, Dublin, Ireland

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Tommy Kyaw Tun Department of Endocrinology, Royal College of Surgeons in Ireland, Connolly Hospital Blanchardstown, Dublin, Ireland

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Seamus Sreenan Department of Endocrinology, Royal College of Surgeons in Ireland, Connolly Hospital Blanchardstown, Dublin, Ireland

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John H McDermott Department of Endocrinology, Royal College of Surgeons in Ireland, Connolly Hospital Blanchardstown, Dublin, Ireland

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Markedly elevated androgen levels can lead to clinical virilization in females. Clinical features of virilization in a female patient, in association with biochemical hyperandrogenism, should prompt a search for an androgen-producing tumor, especially of ovarian or adrenal origin. We herein report the case of a 60-year-old woman of Pakistani origin who presented with the incidental finding of male pattern baldness and hirsutism. Her serum testosterone level was markedly elevated at 21 nmol/L (normal range: 0.4–1.7 nmol/L), while her DHEAS level was normal, indicating a likely ovarian source of her elevated testosterone. Subsequently, a CT abdomen-pelvis was performed, which revealed a bulky right ovary, confirmed on MRI of the pelvis as an enlarged right ovary, measuring 2.9 × 2.2 cm transaxially. A laparoscopic bilateral salpingo-oophorectomy was performed, and histopathological examination and immunohistochemistry confirmed the diagnosis of a Leydig cell tumor, a rare tumor accounting for 0.1% of ovarian tumors. Surgical resection led to normalization of testosterone levels.

Learning points:

  • Hirsutism in postmenopausal women should trigger suspicion of androgen-secreting tumor

  • Extremely elevated testosterone level plus normal DHEAS level point toward ovarian source

  • Leydig cell tumor is extremely rare cause of hyperandrogenicity

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Jayshree Swain Department of Endocrinology, Institute of Medical Sciences, Benaras Hindu University, Varanasi 221005, India

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Shruti Sharma Department of Endocrinology, Institute of Medical Sciences, Benaras Hindu University, Varanasi 221005, India

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Ved Prakash Department of Endocrinology, Institute of Medical Sciences, Benaras Hindu University, Varanasi 221005, India

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N K Agrawal Department of Endocrinology, Institute of Medical Sciences, Benaras Hindu University, Varanasi 221005, India

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S K Singh Department of Endocrinology, Institute of Medical Sciences, Benaras Hindu University, Varanasi 221005, India

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Summary

Ovarian steroid cell tumors are very rare functioning sex-cord stromal tumors. They comprise <0.1% of all ovarian tumors. Previously designated as lipoid cell tumors, one-third of these tumors are considered malignant with the mean age of presentation at around 40 years. We present a case of a 28-year-old female with 2-year history of hirsutism, virilization, and amenorrhea. She was diagnosed with left ovarian tumor, for which she underwent left salpingo-oophorectomy. Histopathology revealed not otherwise specified subtype of steroid cell tumors. The patient resumed menses 2 months after the features of masculinization regressed. Within 1 year of surgery, the patient successfully conceived a full-term baby without any complications. In a young female, the neoplastic etiology of a rapid virilization or menses changing should always be kept in mind. Though commonly observed in adult females, steroid cell tumors have very good surgical outcomes if age at presentation is less and tumor is unilateral, and there are no evidences of bilateral malignancy. Bilateral salpingo-oophorectomy is not required.

Learning points

  • In a case of severe rapid hirsutism and virilization with serum testosterone level more than 200 ng/dl or more than threefold of the normal range, neoplastic conditions should always be suspected.

  • Steroid cell tumor in young women without evidence of malignancy on histopathology has excellent surgical outcomes.

  • Unilateral salpingo-oophorectomy is the surgery of choice.

  • As the frequency of bilateralism is only 6%, prophylactic unaffected side oophorectomy need not be done.

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