Symptomatic Ovarian Steroid Cell Tumor not Otherwise Specified in a Post-Menopausal Woman

Abstract

Steroid cell tumor not otherwise specified (NOS) is a rare subtype of sex cord stromal tumor of the ovary and contributes less than 0.1% of all ovarian neoplasms. The majority of tumors occur in pre-menopausal women (mean age: 43 years), in which 56-77% of patients present with virilization due to excess testosterone. An 80-year-old woman with worsening alopecia and excessive growth of coarse hair on abdomen and genital area was found to have elevated serum testosterone level (462 ng/mL). Radiologic studies were consistent with bilateral adrenal adenomas. Bilateral adrenal venous sampling ruled out the adrenal gland as origin of hormone secretion. A diagnostic and therapeutic bilateral salpingo-oophorectomy confirmed steroid cell tumor NOS of the left ovary. Post-operatively, the patient had complete resolution of her symptoms and normalization of testosterone level. Our case emphasizes the importance of a clinical suspicion for an occult testosterone secreting ovarian tumor in a symptomatic patient without obvious ovarian mass on imaging.

Androgen Secreting Ovarian Tumors

“…10 These tumors include 14 DICER1 mutations were present in 63% of SLCT. They also report that there is no association between DICER1 mutation and tumor differentiation as mutations in poorly differentiated and moderately differentiated SLCT were found to have similar frequencies.…”

“…SLCTs may consist of testicular elements and produce androgens or estrogens (1). Due to androgen secretion, certain SLCT patients may present with masculinization, with symptoms that include anovulation, amenorrhea, defeminization, hirsutism, cliteromegaly, laryngeal protuberance and voice raucity (10). The presence of an ovarian tumor with clinical signs of hormonal disturbances, particularly in younger women, should alert clinicians to the potential presence of an ovarian SLCT.…”

“…If the mass is large it may cause abdominal pain and distension. In addition, rupture of the mass with acute abdominal pain is possible (10). CT and magnetic resonance imaging have made a poor contribution to the diagnosis of SLCTs; ~20% of SLCTs are small in size, and thus unidentifiable, rendering their accurate diagnosis and localization challenging (10).…”

“…Hysterectomy and bilateral salpingo-oophorectomy comprise the radical treatment strategies (4). Well-differentiated SLCTs typically present with benign behavior (10). Malignancy is observed in 11% of moderately-differentiated SLCTs (10-year survival rate, 87%) and 59% of poorly-differentiated SLCTs (10-year survival rate, 41%).…”

“…Chemotherapy may be administered following surgical treatment, particularly for tumors demonstrating poor or moderate differentiation (10). According to Gui et al (10), adjuvant chemotherapy does not provide any significant advantages, and it should be considered primarily in cases of poorly-and moderately-differentiated tumors or disease recurrence. Additionally, a previous study (MITO) was able to demonstrate the benefit of the administration of post-operative chemotherapy for patients exhibiting advanced stage (II-IV) disease (17).…”