Case Of Hyperandrogenism Research Articles

11-Oxygenated androgens are not secreted by the human ovary: in-vivo data from four different cases of hyperandrogenism.

Differentiation of an adrenal from an ovarian source of hyperandrogenemia can be challenging. Recent studies have highlighted the importance of 11-oxygenated C19 steroids to the androgen pool in humans. The aim of this study was to confirm the origin of 11-oxygenated androgens in females and to explore their potential use in the diagnostics of hyperandrogenic disorders. We measured testosterone and its precursors (dehydroepiandrosterone-sulfate and androstenedione) and 11-oxygenated androgens (11β-hydroxyandrostenedione (11-OHA4) and 11-ketotestosterone (11-KT)) in the periphery, adrenal and ovarian veins in four different cases of hyperandrogenism in females (polycystic ovary syndrome (PCOS), primary bilateral macronodular adrenal hyperplasia, Sertoli-Leydig cell tumor and ovarian steroid cell tumor). Two patients demonstrate excessive testosterone secretion in neoplastic ovarian tumors which was not paralleled by a significant secretion of 11-oxygenated androgens as determined by adrenal and ovarian vein sampling. In androgen-secreting bilateral adrenal macronodular hyperplasia, steroid profiles were characterized by elevated 11-KT and 11-OHA4 concentrations in adrenal veins and the periphery. In the patient with PCOS, peripheral 11-KT concentrations were slightly elevated in comparison to the other patients, but the 11-KT and 11-OHA4 concentrations were comparable in ovarian veins and in the periphery. This study confirms that 11-OHA4 and 11-KT are not biosynthesized by the ovary. We propose that the testosterone/11-KT ratio as well as 11-OHA4 could help identify predominant adrenal androgen excess and distinguish neoplastic and non-neoplastic ovarian androgen source. This study confirms that 11β-hydroxyandrostenedione (11-OHA4) and 11-ketotestosterone (11-KT) are not biosynthesized by the human ovary. We propose that the testosterone/11-KT ratio as well as 11-OHA4 could help to identify predominant adrenal androgen excess and distinguish neoplastic and non-neoplastic ovarian androgen source.

ODP420 Ovarian Venous Sampling and the Diagnostic Challenges Associated with Identifying the Source of Severe Hyperandrogenism in an Adolescent Female

Abstract Background Polycystic ovarian syndrome (PCOS) accounts for most cases of hyperandrogenism in adolescent females. Rarely, evaluation reveals testosterone concentrations (> 200 ng/dL). In those instances, imaging is recommended to evaluate for an ovarian or adrenal neoplasm. Recommendations are lacking for when imaging is nondiagnostic, yet testosterone levels continue to rise on standard treatment. Ovarian venous sampling may be considered as a diagnostic tool to locate the source of hyperandrogenism. A 12-year-old female was referred for evaluation of secondary amenorrhea, weight gain, and possible PCOS. Thelarche was at age 10 years and menarche at age 11 years. Eighteen months post-menarche she developed amenorrhea, acne, and hirsutism. She had no clitoromegaly. Her height was 50th percentile, weight >99 th percentile, and BMI 130th of the 95th percentile. She had no family history of PCOS. Initial lab evaluation showed mild hyperandrogenism: total testosterone of 52ng/dL (17-75), free testosterone of 1.3ng/dL (<0. 04-0.84), and DHEA of 423ng/dL (<202). OGTT done with only glucoses was normal. HbA1c was 5.6%. DHEAS, androstenedione, 17-OH progesterone, prolactin and TSH were normal. LH was 3.5mIU/mL, FSH 4.8mIU/mL, and estradiol 45pg/mL. Pelvic ultrasound showed enlarged right (25mL) and left ovaries (15.6mL). The follicular distribution was normal and bilateral simple cysts were noted. PCOS was suspected and she started on metformin and oral contraceptive pills. Her testosterone levels increased during subsequent visits with a peak total and free testosterone of 344ng/dL and 7.83ng/dL. HbA1c rose to 5.9%. She had fasting and postprandial insulin levels of 74.3 and 260mcIU/mL, respectively. Further evaluation revealed a normal AMH level of 6.4ng/mL (0.49-6.9), SHBG of 22.9nmol/L (7.7-108), a negative screen for anabolic steroid use, and 46,XX karyotype. Imaging over 8 months (multiple transabdominal ultrasounds, MRI of abdomen/pelvis and brain) was negative for neoplasm. The potential next steps discussed with the patient were continued serial imaging, GnRH agonist, or ovarian venous sampling with possible oophorectomy. Due to concern for neoplasm, the patient opted for venous sampling which showed a 10-fold gradient in total testosterone between right and left gonadal veins (2300ng/dL vs. 203ng/dL). She underwent right oophorectomy and switched from OCP to transdermal hormone therapy. Three months later, her total testosterone was normal (26ng/dL), she had lost 5kg and menstrual cycles returned. Pathology resulted as PCOS, with no evidence of neoplasm. Conclusions Localization of the source of severe hyperandrogenism can be a challenge. In our case, ovarian venous sampling results prompted unilateral oophorectomy, yet pathology showed no ovarian neoplasm. While ovarian venous sampling is a potential diagnostic tool, there is no data guiding when it should be used and interpretation of results, specifically if/when oophorectomy should be pursued. This is particularly relevant in the management of young, premenopausal females. Presentation: No date and time listed

Androgenic Granulosa Cell Tumor in an Adolescent Girl

Virilizing tumors may arise from adrenals or ovary. Androgen secreting ovarian tumors constitute less than 0.2% of cases of hyperandrogenism and less than 1% of all ovarian tumors. Hyperandrogenism is seen with Leydig cell tumors, Sertoli cell tumors, steroid cell tumors not otherwise specified and ovarian thecomas. Adult granulosa cell tumors (GCTs) are the most common type of ovarian sex cord tumors. These tumors usually produce estrogens and hence cause symptoms and signs of estrogen excess. GCTs mostly present in perimenopausal and postmenopausal women with complaints of abnormal uterine bleeding. Androgen secreting GCT is a rare clinical entity. Here we are reporting one such case in an adolescent girl with features of hyperandrogenism and ovarian mass.

A Case of Hyperandrogenism, Insulin Resistance, and Acanthosis Nigricans Syndrome; Increase in Proliferating Cell Nuclear Antigen and Decrease in Loricrin in Acanthosis Nigricans.

A Case of Hyperandrogenism, Insulin Resistance, and Acanthosis Nigricans Syndrome; Increase in Proliferating Cell Nuclear Antigen and Decrease in Loricrin in Acanthosis Nigricans.

Report of a case of hyperandrogenism in a postmenopausal woman

Report of a case of hyperandrogenism in a postmenopausal woman

Isolated Transient Neonatal Clitoromegaly with Hyperandrogenism of Unknown Etiology

Background Neonatal clitoromegaly is usually attributed to androgen stimulation secondary to congenital adrenal hyperplasia or in utero androgen exposure. We present a unique case of transient, isolated neonatal clitoromegaly, associated with increased androgen levels of unidentifiable cause, which spontaneously resolved when the androgen levels normalized. This is the first report where, despite an exhaustive workup, the cause of hyperandrogenism remains unknown. Case A female infant was delivered at 25 + 5 weeks gestational age. Clitoromegaly was first documented at 3 months of age (18 mm long, 13 mm wide). There was no other evidence of virilization. There was no neonatal history of transfusions or androgen-medication exposure. Initial bloodwork documented extremely high levels of free testosterone (117 pmol/L) and dehydroepiandrosterone sulfate (DHEAS). Further tests ruled out the following diagnoses: 21-hydroxylase deficiency, 11-betahydroxylase deficiency, testicular feminization, presence of ovotesticular tissue, and androgen-secreting tumors. Maternal history ruled out in utero androgen exposure, maternal drug use, hyperandrogenism, or virilization during pregnancy, and antenatal imaging had not detected a maternal ovarian mass or adrenal tumor. The infant's androgen levels spontaneously declined, and by 4 months of age had normalized without therapy (free testosterone 8 pmol/L). At 5 months of age, the clitoral measurements normalized (10 mm long, 5 mm wide). Conclusion In this case of a premature female infant, hyperandrogenism and clitoromegaly resolved spontaneously. Only 1 other case of hyperandrogenism and clitoromegaly that resolved spontaneously in a preterm infant has been reported. In that case, the clitoromegaly and high testosterone levels were attributed to repeated blood transfusions from an adult male. The etiology of the hyperandrogenism and resultant clitoromegaly in our case is not known.