Abstract
Background. A 33-year-old woman presented to an endocrinology clinic with a 5-year history of secondary amenorrhea. 2 years before presentation, she had noticed progressively worsening signs of virilization.
Investigations. Measurement of levels of serum free and total testosterone, androstenedione, dehydroepiandrosterone sulfate and gonadotropins; transvaginal ultrasonography, abdominal and pelvic MRI and 18F-fluorodeoxyglucose PET imaging.
Diagnosis. Virilization secondary to an ovarian Leydig cell tumor.
Management. The patient underwent a left salpingo-oophorectomy that confirmed the diagnosis of a unilateral Leydig cell tumor. Complete normalization of androgens and gonadotropin levels was achieved after surgery.
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15 February 2011
In the version of this article initially published online, there was an error in the legend of Figure 4. The magnification of Figure 4a should have read 200× instead of 20× and the magnification of Figure 4b should have read 400× instead of 40×. The error has been corrected in all electronic versions of the text
References
Young, R. H. & Scully, R. E. Fibromatosis and massive edema of the ovary, possibly related entities: a report of 14 cases of fibromatosis and 11 cases of massive edema. Int. J. Gynecol. Pathol. 3, 153–178 (1984).
Carmina, E., Rosato, F., Jannì, A., Rizzo, M. & Longo, R. A. Extensive clinical experience: relative prevalence of different androgen excess disorders in 950 women referred because of clinical hyperandrogenism. J. Clin. Endocrinol. Metab. 91, 2–6 (2006).
Koonings, P. P., Campbell, K., Mishell Jr. D. R. & Grimes, D. A. Relative frequency of primary ovarian neoplasms: a 10-year review. Obstet. Gynecol. 74, 921–926 (1989).
Martin, K. A. et al. Evaluation and treatment of hirsutism in premenopausal women: an endocrine society clinical practice guideline. Clin. Endocrinol. Metab. 93, 1105–1120 (2008).
Meldrum, D. R. & Abraham, G. E. Peripheral and ovarian venous concentrations of various steroid hormones in virilizing ovarian tumors. Obstet. Gynecol. 53, 36–43 (1979).
Surrey, E. S., de Ziegler, D., Gambone, J. C. & Judd, H. L. Preoperative localization of androgen-secreting tumors: clinical, endocrinologic, and radiologic evaluation of ten patients. Am. J. Obstet. Gynecol. 158, 1313–1322 (1988).
Waggoner W., Boots L. R. & Azziz R. Total testosterone and DHEAS levels as predictors of androgen-secreting neoplasms: a populational study. Gynecol. Endocrinol. 13, 394–400 (1999).
Pugeat, M., Mirakian, P., Dutrieux-Berger, N., Forest, M. G. & Tourniaire, J. in Androgen excess disorders in women (eds Azziz, R., Nestler, J. E. & Dewailly, D.) 555–568 (Lippincott-Raven, Philadelphia, 1997).
Kaltsas, G. A. et al. The value of the low-dose dexamethasone suppression test in the differential diagnosis of hyperandrogenism in women. J. Clin. Endocrinol. Metab. 88, 2634–2643 (2003).
Shenker, Y., Malozowski, S. N., Ayers, J., Grekin, R. J. & Barkan, A. L. Steroid secretion by a virilizing lipoid cell ovarian tumor: origins of dehydroepiandrosterone sulfate. Obstet. Gynecol. 74, 502–506 (1989).
Hoffman, D. I., Klove, K. & Lobo, R. A. The prevalence and significance of elevated dehydroepiandrosterone sulfate levels in anovulatory women. Fertil. Steril. 42, 76–81 (1984).
Young, R. H., Dudley, A. G. & Scully, R. E. Granulosa cell, Sertoli-Leydig cell, and unclassified sex cord-stromal tumors associated with pregnancy: a clinicopathological analysis of thirty-six cases. Gynecol. Oncol. 18, 181–205 (1984).
Paraskevas, M. & Scully, R. E. Hilus cell tumor of the ovary. A clinicopathological analysis of 12 Reinke crystal-positive and nine crystal-negative cases. Int. J. Gynecol. Pathol. 8, 299–310 (1989).
Wajchenberg B. L. et al. Adrenocortical carcinoma: clinical and laboratory observations. Cancer. 88, 711–736 (2000).
Young, R. H. & Scully, R. E. Ovarian steroid cell tumors associated with Cushing's syndrome: a report of three cases. Int. J. Gynecol. Pathol. 6, 40–48 (1987).
Taylor, N. J. & Moore, N. R. in Clinical Endocrine Oncology (eds Hay, I. D. & Wass, J. A. H) 396–403 (Wiley–Blackwell, Malden, 2008).
Taylor, L., Ayers, J. W., Gross, M. D., Peterson, E. P. & Menon, K. M. Diagnostic considerations in virilization: iodomethyl-norcholesterol scanning in the localization of androgen secreting tumors. Fertil. Steril. 46, 1005–1010 (1986).
Levens, E. D., Whitcomb, B. W., Csokmay, J. M. & Nieman, L. K. Selective venous sampling for androgen-producing ovarian pathology. Clin. Endocrinol. (Oxf) 70, 606–614 (2009).
Risum, S. et al. The diagnostic value of PET/CT for primary ovarian cancer—a prospective study. Gynecol. Oncol. 105, 145–149 (2007).
Wang, P. H. et al. Diagnosis and localization of testosterone-producing ovarian tumors: imaging or biochemical evaluation. Gynecol. Oncol. 83, 596–598 (2001).
Mattsson, C., Stanhope, C. R., Sam, S. & Young, W. F. Jr. Image in endocrinology: testosterone-secreting ovarian tumor localized with (fluorine-18)-2-deoxyglucose positron emission tomography. J. Clin. Endocrinol. Metab. 91, 738–739 (2006).
Matuszczyk, A. et al. Leydig cell tumor as a cause of hirsutism in a postmenopausal woman [German]. Med. Klin. 102, 259–262 (2007).
Mavroudis, K., Aloumanis, K., Papapetrou, P. D., Voros, D. & Spanos, I. Virilization caused by an ectopic adrenal tumor located behind the iliopsoas muscle. Fertil. Steril. 87, 1468.e13–1468.e16 (2007).
Hidlebaugh, D. A., Vulgaropulos, S. & Orr, R. K. Treating adnexal masses. Operative laparoscopy vs. laparotomy. J. Reprod. Med. 42, 551–558 (1997).
Medeiros, L.R, Fachel, J. M., Garry, R., Stein A. T. & Furness S. Laparoscopy versus laparotomy for benign ovarian tumours. Cochrane Database of Systematic Reviews, Issue 3, Art. No.: CD004751. doi: 10.1002/14651858.CD004751.pub2 (2005).
Hulka, J. F., Parker, W. H., Surrey, M. W. & Phillips, J. M. Management of ovarian masses. AAGL 1990 survey. J. Reprod. Med. 37, 599–602 (1992).
Fanfani, F. et al. A prospective randomized study of laparoscopy and minilaparotomy in the management of benign adnexal masses. Hum. Reprod. 19, 2367–2371 (2004).
Herrmann, UJ. Sonographic patterns of ovarian tumors. Clin. Obstet. Gynecol. 36, 375–383 (1993).
Barnor, Q., Kurzawinski T. R. & Conway, S. in Clinical Endocrine Oncology (eds Hay, I. D. & Wass, J. A.H) 390–395 (Wiley–Blackwell, Malden, 2008).
Pascale, M. M. et al. Androgen suppressive effect of GnRH agonist in ovarian hyperthecosis and virilizing tumours. Clin. Endocrinol. (Oxf) 41, 571–576 (1994).
Acknowledgements
Written consent for publication was obtained from the patient. We would like to acknowledge Manuel Faria and Malebranche B. C. da Cunha Neto for kindly reviewing this manuscript and Henrique Moura for gently editing and providing the pathology images.
C. P. Vega, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the MedscapeCME-accredited continuing medical education activity associated with this article.
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A. M. Faria, R. V. Perez, J. A. M. Marcondes, D. S. Freire and S. A. Y. Hayashida researched data for the article. A. M. Faria, J. A. M. Marcondes, R. Blasbalg, J. Soares Jr, K. Simões, S. A. Y. Hayashida and M. A. A. Pereira provided a substantial contribution to discussion of the content. A. M. Faria, R. V. Perez, J. A. M. Marcondes, D. S. Freire and K. Simões wrote the article. A. M. Faria, J. A. M. Marcondes and M. A. A. Pereira reviewed/edited the manuscript before submission.
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Faria, A., Perez, R., Marcondes, J. et al. A premenopausal woman with virilization secondary to an ovarian Leydig cell tumor. Nat Rev Endocrinol 7, 240–245 (2011). https://doi.org/10.1038/nrendo.2011.15
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DOI: https://doi.org/10.1038/nrendo.2011.15
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