Ovarian Leydig cell tumor in a peri-menopausal woman with severe hyperandrogenism and virilization

Abstract

The authors report a case of Leydig cell tumor in a 46-year-old woman who first presented with severe clinical hyperandrogenism and associated complex medical history. Investigations revealed markedly raised serum concentrations of testosterone (28.3 nmol/l) and free androgen index (54.4), whereas sex hormone binding globulin, random cortisol, androstenedione, 17-hydroxyprogesterone and dehydroepiandrosterone sulphate concentrations were all within the normal range. Transabdominal ultrasound and computed tomography scan of the pelvis and abdomen showed a slightly bulky right ovary, but no other abnormalities. An ovarian source of androgens was suspected and surgery was arranged. Following a three-year history of defaulting appointments due to agoraphobia, she underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy and intraoperative selective ovarian venous sampling. Histopathological examination revealed a 2 cm Leydig cell tumor within the right ovary. Successful intraoperative ovarian venous sampling demonstrated significantly elevated testosterone levels (>260 nmol/l) from the right ovarian vein. Hyperandrogenaemia normalized post-operatively. The patient showed significant regression of clinical signs and symptoms, including the anxiety disorder. Clinical presentation, biochemistry and imaging modalities should allow to detect androgen-secreting ovarian tumors, while selective venous sampling should be reserved for patients whom uncertainty remains. The present case confirms that androgen-secreting ovarian tumors represent a diagnostic and therapeutic challenge. They have to be considered in the differential diagnosis of severe hyperandrogenism even in peri-menopausal women. Although selective venous sampling is of diagnostic value, however, its impact on future management should be considered on individual basis.

Androgen Secreting Steroid Cell Tumor of the Ovary Represented with Postmenopasal Bleeding and Extensive Hirsutism

“…In all androgen-producing ovarian tumors, serum testosterone level is elevated in conjunction with normal or mildly elevated serum DHEAS levels, but the increased dehydroepiandrosterone sulphate levels are often associated with adrenal tumors [17]. A dexamethasone suppression test may be useful to help distinguish between ovarian and adrenal sources of androgens.…”

Fast Intraoperative Testosterone Assay Confirms the Location of an Ovarian Virilizing Tumor in a Young Girl

“…Regarding the intraoperative assay of testosterone, there is a limited number of reports on women in whom the assay guided the localization of the tumor [3,5,6]. We report the first case of a teenage girl with a virilizing tumor of the ovary which could be precisely localized by an intraoperative testosterone assay.…”

“…Based on the extreme difference in the measured hormone levels, a right salpingo-oophorectomy could be carried out to remove the tumor. This procedure has been described before in adults [3,5,6]. In the limited number of cases reported, the success rate of identification of the tumor-affected ovary has been 100%.…”

An ovarian steroid cell tumor with virilization and hypothyroidism: a case report

“…Although the tumor with virilization and rarely associated massive ascites might look malignant clinically, the tumor might prove to be benign [12]. In young adult women with secondary amenorrhea, or in perimenopausal women with severe hyperandrogenism and virilization, Leydig cell tumor of the ovary should be considered [13,14]. Of the different markers that have been reported to stain ovarian stromal tumors, inhibin has proven to be the most helpful; immunostaining with calretinin has also being suggested as a sensitive marker [15,16].…”