Post Menopausal Hyperandrogenism: A Case of a Steroid Cell Tumor of the Ovary

Introduction: Postmenopausal hyperandrogenism is a rare condition that causes hirsutism, virilization, and clitoromegaly that should be carefully evaluated in order to avoid overlooking an androgen secreting tumor (1). Case: A 48 year old African American female with a prior history of polycystic ovarian syndrome (PCOS) presented for evaluation of hirsutism. Of note, she also underwent menopause at age 41 after receiving chemotherapy for a history of multiple myeloma, and she has been on steroids since the time of her diagnosis. On exam, she had thick, dark hair growth on her chin, upper lip, and chest, as well as male-patterned baldness, acne, easy bruising, proximal muscle weakness, deep voice, and elevated blood pressure. Prior to endocrinology evaluation, she was started on spironolactone 25 mg BID. Lab work up included dehydroepiandrosterone sulfate (DHEAS) 73 mcg/dL (27-240 mcg/dL), 17-hydroxyprogesterone 74 ng/dL (31-455 ng/dL), androstenedione 271 ng/dL (30-200 ng/dL), total testosterone 763 ng/dL (8-60 ng/dL), bioavailable testosterone 244 ng/dL (0.8-10 ng/dL), hemoglobin A1c 4.3%, follicle stimulating hormone 30 IU/L, luteinizing hormone 23.9 IU/L, insulin 11 mcIU/mL (2.6-24.9 mcIU/mL), glucose 71, insulin-like growth factor 1 236 ng/mL (44-227 ng/mL) with subsequent normal glucose suppression test. While transvaginal ultrasound did not note any abnormal findings, a computed tomography of the abdomen/pelvis showed a new hyperdense focus in the left ovary as well as a tiny right adrenal nodule, most likely an adenoma. Follow up magnetic resonance imaging confirmed a 1.6 cm enhancing solid left ovarian mass; it also confirmed a right adrenal adenoma and left adrenal thickening versus a tiny adenoma. Urine metanephrines and catecholamines were normal. Patient had total hysterectomy and bilateral oophorectomy; pathology showed a steroid cell tumor. Conclusion: Postmenopausal hyperandrogenism has several causes: insulin resistance, PCOS, non-classic congenital adrenal hyperplasia, medications, and tumors of the ovaries or adrenals. Severe hyperandrogenemia should raise the suspicion of an ovarian or adrenal neoplasm, necessitating prompt imaging (1). Certain imaging may not reveal smaller masses, and additional imaging or ovarian/adrenal vein sampling may be needed. Typically, an elevated DHEAS with a high testosterone suggests an adrenal source, while androstenedione can be elevated in both glands. Once identified, the involved gland is surgically resected. This patient was found to have a steroid cell tumor, which has malignant potential. They make up less than 0.1% of all ovarian tumors (2). Initial treatment is surgical resection and may necessitate chemotherapy if malignant. 1) Markopoulos MC, Kassi E, Alexandraki KI, Mastorakos G, Kaltsas G. Hyperandrogenism after menopause. Eur J Endocrinol. 2015 Feb;172(2):R79-91. doi: 10.1530/EJE-14-0468. Epub 2014 Sep 15. PMID: 25225480.2) Hayes, Mary C. M.D.; Scully, Robert E. M.D. Ovarian Steroid Cell Tumors (Not Otherwise Specified), The American Journal of Surgical Pathology: November 1987 - Volume 11 - Issue 11 - p 835-845

A large ovarian steroid cell tumor‐not otherwise specified with a unique combination of benign and malignant features as a challenging cause of oligomenorrhea and hirsutism in a 21‐year‐old Syrian female: a case report

Background Ovarian steroid cell tumors represent a rare category of sex cord-stromal tumors that constitute less than 0.1% of all ovarian tumors. These neoplasms are classified into three main subtypes according to the cell of origin: Leidyg cell tumors, stromal luteomas, and steroid cell tumors not otherwise specified (SCTs-NOS). The latter subtype is defined as a neoplasm of an uncertain lineage that mostly affects middle-aged women, whereas it’s rare in younger ages. Case presentation We report a case of a 21-year-old virgin female who presented to our hospital with complaints of mild abdominal pain, hirsutism, and oligomenorrhea for more than a year. Before her current admission, the patient had attended an external gynecologic clinic where she had been prescribed oral contraceptives to regulate her periods. Nevertheless, on presentation to our institution, physical examination revealed abdominal tenderness with a palpable pelvic mass and mild hirsutism in the thigh. Ultrasonography demonstrated a large left ovarian mass measuring 154 × 104 mm, and compressing the uterus. Therefore, a unilateral salpingo-oophorectomy was performed, and interestingly, pathologic examination of the large aforementioned mass alongside with immunohistochemical correlation revealed the diagnosis of a large ovarian steroid cell tumor-not otherwise specified with a unique combination of benign and malignant features. Conclusions Although ovarian steroid cell tumors represent a rare category, they must be considered in the differential diagnosis for mild virilization symptoms in young females due to the importance of early diagnosis and management. In this manuscript, we aimed to present the first case report from Syria that highlights the crucial role of detailed morphological examination for challenging cases despite the difficulties in differential diagnosis, and the absence of ancillary techniques. Furthermore, we managed to discuss a brief review of diagnostic methods, histological characteristics, and treatment recommendations.

A Rare Case Report of Postmenopausal Virilization: Ovarian Steroid Cell Tumor

Ovarian steroid cell tumors are rare, life-threatening neoplasms that make about 0.1% of all primary ovarian tumors. They frequently present in premenopausal women with the manifestation of virilization. We report a 58-year-old postmenopausal woman that referred to our clinic with clinical manifestation of virilization. Laboratory findings showed markedly elevated serum testosterone level and ultrasound showed 2 follicles with 7 mm diameters in the left ovary. She treated by bilateral salpingo-oophorectomy and synchronous hysterectomy. Histological pathology confirmed a benign steroid cell tumor. The presentation of new-onset and rapid progressive hyperandrogenism is rare in postmenopausal women. In diagnosis, we must consider adrenal and ovarian malignancies. Stromal luteoma is a rare benign ovarian tumor, Which is treated with surgical treatment.