Evaluation and management of worsening virilization in a postmenopausal woman diagnosed with PCOS

The manifestations of common disorders can infrequently obscure the presence of very rare conditions that share similar clinical findings. Polycystic ovary syndrome (PCOS) is a common disorder characterized by hyperandrogenism, ovulatory dysfunction, and/or polycystic ovarian morphologic features. A key component of the Rotterdam PCOS diagnostic criteria is the exclusion of other conditions that can mimic the signs and symptoms of PCOS. Compared to PCOS, androgen-producing tumors are rare neoplasms that originate in either the ovaries or adrenals and appear in only about 0.2% of people with hyperandrogenism. Ovarian steroid cell tumors (OSCTs) are an exceptionally rare subset of this class that represents less than 0.1% of all ovarian neoplasms. OSCTs are often hormonally active and classically cause the sudden and severe onset of secondary virilizing characteristics. In the case presented here, we describe a postmenopausal patient who was referred for management of PCOS but ultimately found to have an OSCT. A detailed history elicited a worsening of symptoms after menopause that raised the suspicion of a virilizing tumor; however, the clinical challenge was to determine whether the patient had both PCOS and an OSCT or whether the OSCT had been "causing" PCOS all along. This required careful scrutiny of the patient's 24-year clinical course and a comprehensive literature review in an effort to distinguish the manifestations of an OSCT from those of PCOS.