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Background Diagnosing hyperandrogenemia in postmenopausal women is very difficult. It occasionally manifests as excessive hair growth or with no clinical manifestations, and is therefore often misdiagnosed or missed altogether. Ovarian steroid cell tumors that cause hyperandrogenemia in women account for approximately 0.1% of all ovarian tumors. Due to the low incidence, corresponding imaging reports are rare, so ovarian steroid cell tumors lacks typical imaging findings to differentiate it from other ovarian tumors. Therefore, we summarized its clinical and imaging characteristics through this case series, and elaborated on the differential diagnosis of steroid cell tumors. Case summary We report three cases of postmenopausal women with hyperandrogenemia. Only 1 patient showed virilization symptoms, the other two patients were completely asymptomatic. All patients underwent total hysterectomy + bilateral adnexectomy. Histological results showed one case of Leydig cell tumor and two cases of benign, non-specific steroid cell tumor. After the operation, the androgen levels of all patients returned to normal, and there was no clinical recurrence since follow-up. Conclusion Although virilization caused by increased serum testosterone levels is an important clinical feature of ovarian steroid cell tumors, it is often asymptomatic. A solid, slightly hypoechoic, round or oval mass with uniform internal echo, richer blood flow in the solid part, and low resistance index are typical imaging features of ovarian steroid cell tumors. Diagnosis of ovarian steroid cell tumors after menopause is challenging, but surgery can be used for both diagnosis and clear treatment.

Background Ovarian steroid cell tumor, not otherwise specified (NOS), is a rare type of sex cord stromal tumor, which often presents with androgenic symptoms and has a high frequency of malignancy. Case This is a case of a 14-year-old Native American girl who presented with acne, amenorrhea, and virilization and was found to have a 2.9-cm solid ovarian mass. Initial pathology revealed steroid-appearing cells with round nuclei, clear/vacuolated cytoplasm, and a low mitotic index. Final diagnosis was ovarian steroid cell tumor, NOS Stage IA. A laparoscopic left salpingo-oophorectomy was subsequently performed. No tumor recurrence was noted 2 years after her initial diagnosis. Summary and Conclusion Long-term data on these tumors are limited; however, malignancy, recurrence, and death have been reported. This suggests that close follow-up is essential for appropriate management.

We report a rare case of an ovarian steroid cell tumor with a diagnosis prompted by heart failure symptoms. A 28-year-old Japanese nulligravida/nullipara with a chief complaint of respiratory discomfort during physical exertion and exhibiting heart failure symptoms was referred to our hospital. She also had signs of virilization, including secondary menorrhea since the age of 20, hirsutism and balding. Cushing's syndrome was suspected, and further examinations showed hypertestosteronemia and right ovarian tumor. Symptomatic treatment for heart failure with diuretics and antihypertensives was followed by abdominal right adnexectomy performed due to the androgen-producing ovarian tumor. The tumor was solid and larger than a fist, and confirmed as a steroid cell tumor through postoperative histopathology. Serum total testosterone levels normalized at day 3 postoperatively, and menstruation resumed 2 months later. Our case was diagnosed due to heart failure symptoms, and its treatment resulted in improvement in virilization signs.

Steroid cell tumors (SCTs) (not otherwise specified (NOS)) are rare sex cord-stromal tumors of the ovary. These are associated with hormonal disturbances resulting in menstrual bleeding patterns and androgenic effects. We report the case of a 36-year-old female presented with hirsutism, signs of virilization, and elevated androgen levels. Transvaginal ultrasound showed a solid-appearing right ovarian mass. She underwent fertility-sparing surgery with a laparoscopic left oophorectomy. Histological examination showed a benign steroid cell tumor, NOS. These tumors often small can then present a problem of positive diagnosis responsible for a delay in the diagnosis.

We report a case of a 4-year-old girl with an ovarian steroid cell tumor, not otherwise specified (SCT-NOS). She was admitted to the hospital with progressing virilization and Cushing’s syndrome, which included abnormality of the perineum, hirsutism, hypertrichosis, flushing of face, hoarseness, and weight gain. Blood testing showed a significantly increased testosterone level and slightly increased cortisol level. Computed tomography scan revealed an 8.0 × 5.0 × 5.0 cm tumor of the right ovary. The patient underwent right salpingo-oophorectomy, and pathological examination showed malignant potential. Three courses of bleomycin, etoposide, and cisplatin were administered as postoperative chemotherapy. After tumor resection, her testosterone decreased to undetectable levels. However, during the course of the treatment, the patient suffered from adrenal insufficiency resulting in the need for hydrocortisone replacement therapy. Although SCT-NOS in childhood are typically benign, pathological findings should be carefully observed for potential malignancy. In cases of cortisol-producing SCT-NOS, serum levels should be monitored, and hydrocortisone replacement therapy should be considered before resection.

Objective Ovarian neoplasms in children are rare. The objective of this report is to emphasize the importance of considering those neoplasms in the differential diagnosis of hyperandrogenism even with negative diagnostic imaging. Methods We report the case of a 12-year-old girl who presented with virilization and elevated 17 hydroxyprogesterone (17-OHP) and who was subsequently diagnosed with an ovarian neoplasm. Results The patient was initially seen for hirsutism and deepening of the voice. Elevated 17-OHP, androstenedione, and testosterone prompted the initial diagnosis of nonclassic congenital adrenal hyperplasia due to 21-hydroxylase deficiency, but those levels failed to suppress on corticosteroid therapy. Ultrasound, computed tomography scan, and magnetic resonance imaging of the abdomen and pelvis were normal. Genetic testing for congenital adrenal hyperplasia was negative. Bilateral selective adrenal and ovarian venous sampling confirmed the ovarian origin of her hyperandrogenism. A unilateral salpingo-oophorectomy revealed a steroid cell tumor. Postoperatively there was normalization of testosterone and 17-OHP. Conclusion This report highlights the utility of selective adrenal and ovarian sampling when suspecting a primary androgen-secreting neoplasm, even in the setting of elevated 17-OHP levels and negative imaging studies, as early diagnosis can prevent manifestation of irreversible symptoms of virilization

OBJECTIVE: Ovarian steroid cell tumor (SCT) is a rare tumor with steroid-producing ability. We report a 22-year-old woman with secondary amenorrhea and hirsutism caused by an ovarian SCT-not otherwise specified (NOS), who underwent successfully laparoscopic resection of the tumor. CASE REPORT: A 22-year-old null gravida woman presented to a hospital, having amenorrhea for 18 months and increasing facial hair. Physical examination revealed obesity (body mass index, 37.3 kg/m(2)) with evident facial and trunk hair. Total and free serum testosterone, and dehydroepiandrosterone sulfate levels were found to be elevated. Levels of serum adrenocorticotropic hormone, gonadotropins, cortisol, aldosterone, and ovarian steroids were observed to be within reference intervals. Although polycystic ovaries were not found, a hyperechogenic solid tumor (3 cm) was detected on transvaginal ultrasonography. Laparoscopic resection of the tumor was performed. One month post-surgery, total and free testosterone levels were observed to have decreased, and menstruation resumed two months thereafter. The patient was histologically diagnosed with ovarian SCT-NOS. Expression of ovarian steroidogenic enzymes, which are related to hyperandrogenism, was observed. No disease recurrence has been reported for more than 5 years post-surgery.

Ovarian Leydig cell tumor, a sub-type of ovarian steroid cell tumor, accounts for less than 0.1% of all ovarian tumors. It can affect women of any age group but is most common in postmenopausal women. We here report a case of virilizing ovarian Leydig cell tumor with multiple non-functional endocrine neoplasias (pituitary and adrenal adenomas) in a 48-year-old woman. She first presented with sub-abdominal pain and hirsutism since menopause three years ago. Subsequently, she had slight facial acne, voice deepening, breast atrophy, and a prominent Adam’s apple. Her hormone profile showed an elevated level of testosterone, high free androgen index, low levels of luteinizing hormone and follicle stimulating hormone, and normal levels of random cortisol, androstenedione, 17-hydroxyprogesterone and dehydroepiandrosterone sulfate. A pelvic enhanced magnetic resonance imaging (MRI) scan showed nodules in the right ovary, and a pituitary enhanced MRI revealed a microadenoma. An enhanced computerized tomography scan of the adrenal gland revealed left adrenal nodules, possibly adenomas. After a right cystectomy and right fallopian tube resection, her testosterone level declined to 0.38 nmol/L and the symptoms associated with hyperandrogenism improved. This is a rare case of virilizing ovarian Leydig cell tumor with multiple non-functional endocrine neoplasias. We believe our findings will be helpful in the clinical diagnosis and treatment of hyperandrogenism.

Los tumores de células esteroides de ovario se clasifican en luteoma estromal, tumor de células de Leydig y tumor de células esteroideas sin otra especificación, según su origen embrionario. El tumor ovárico de células esteroideas sin otra especificación es un tumor benigno raro, pero con potencial maligno; representa menos del 0,1% de todos los tumores de ovario. Deben ser considerados como causa de virilización en mujeres adultas por la producción de testosterona. Solo un feto femenino corre riesgo de virilización. Al igual que otros tumores del estroma ovárico, los tumores deben ser tratados quirúrgicamente. La cirugía está indicada en casos de agrandamiento ovárico unilateral sólido, debido a un 50% de probabilidad de malignidad. En el embarazo, los tumores ováricos de células esteroideas sin otra especificación son excepcionalmente raros y deben ser diferenciados del luteoma del embarazo y otras neoplasias malignas del ovario. Con mayor frecuencia pueden complicarse con rotura y/o torsión. Se presenta un caso de tumor ovárico de células esteroideas sin otra especificación durante el embarazo.

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.

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.

To characterize the expression of steroidogenic enzymes implicated in the development of ovarian steroid cell tumors, not otherwise specified (SCT-NOS). We present 4 ovarian SCT-NOS evaluated by immunohistochemical staining of steroidogenic enzymes as an approach to define this entity pathologically. All 4 ovarian SCT-NOS showed increased expression for cholesterol side-chain cleavage enzyme (CYP11A1), 17α-hydroxylase (CYP17A1), 17β-hydroxysteroid dehydrogenase 1 (HSD17B1), aldo-ketoreductase type 1 C3 (AKR1C3), 3β-hydroxysteroid dehydrogenase 2 (HSD3B2), 5α-reductase type 2 (SRD5A2), steroid sulfatase (SULT2A1), estrogen sulfotransferase (EST), and aromatase (CYP19A1). Expression was negative for 21-hydroxylase (CYP21A2) and 17β-hydroxysteroid dehydrogenase 2 (HSD17B2). 17β-hydroxysteroid dehydrogenase 3 (HSD17B3) and 5α-reductase type 1 (SRD5A1) showed variable expression. Our analysis reveals a novel finding of increased expression of AKR1C3, HSD17B1, SRD5A2, SULT2A1, and EST in ovarian SCT-NOS, which is clinically associated with androgen excess and virilization. Further studies are needed to validate these enzymes as new markers in the evaluation of hyperandrogenic ovarian conditions.

Background: Ovarian tumors are divided into non functioning and functioning, within the last group, we can find those with endocrine activity that produce androgenization. Ovarian cell tumors, not otherwise specified (SCT-NOS) is a rare type of ovarian sex cordomal tumor and represents the 60% of this tumors, which compromise less than 0.1% of the ovarian tumors. (1) Clinical Case: We here present a 28 year old woman who was referred to the Endocrine Clinic due to secondary amenorrhea and virilization signs. At the age of twelve a diagnosis of polycystic ovarian syndrome (POS) was made and treated with combined oral anticonceptive (COA). Menses became regular only with medication. Six months after she stopped medication, amenorrhea and virilization signs worsened. Biochemically she had levels of serum total testosterone 6.8 ng/mL (0.02-0.45) and free testosterone 42 pg/mL (0.1-6.4) since only pelvic ultrasonography has been made by physician, a transvaginal ultrasound and abdomen – pelvic CT scan showed a anexial tumor. After analysis of biochemical and imaging results a multidisciplinary team performed a surgical extirpation of the primary lesion, which was diagnosed by histopathology as a tumor of lipoidic cells NOS. A month after the surgery, menses became regular. Conclusion: The purpose of this article is to present the available information about this kind of tumors and the treatment recommended. It is mandatory to follow a correct approach among a multidisciplinary team, in order to get the correct diagnosis at the proper time. (1) Zang L, Ye M, Yang G, Li J, Liu M, Du J et al. Accessory ovarian steroid cell tumor producing testosterone and cortisol. Medicine. 2017;96(37):e7998.

Most ovarian steroid cell tumors arise sporadically. However, they can also be observed as a rare manifestation of von Hippel Lindau disease. Here, we present a clinical, pathological and molecular characterization of a steroid cell tumor in a VHL patient. A 14 year old girl with molecularly confirmed diagnosis of VHL developed hirsutism and amenorrhea. Initial clinical hormonal evaluation was notable for elevated 17-OHP of 406ng/dl, androstenedione 275ng/dl, and testosterone 102ng/dl. In order to exclude congenital adrenal hyperplasia as a common cause of hirsutism in adolescents, ACTH stimulation was performed, but no increase in 17-OHP was observed. Anti Muellerian hormone, inhibin (INH) A and INH B were normal. Imaging revealed a bilobed 6cm left adnexal mass. The mass was resected en bloc via a left oophorectomy. Pathological evaluation showed multinodular steroid cell tumor with clear cytoplasm and delicate vascular meshwork. Immunoprofiling demonstrated positivity for inhibin and calretinin; while renal cell carcinoma markers were negative. All laboratory values normalized post-surgery. In addition to clinical measurements pre- and post-surgery, steroid profiles were evaluated by LC-MS/MS. Quantitative RT-PCR analysis showed robust tumor expression of enzymes facilitating the production of androgens, but not estrogens. Further preliminary analysis by exome sequencing confirmed the known germline pathogenic variant in VHL, but no additional obvious somatic driver mutations were identified. Interestingly, the NGS analysis of different specimens from the same tumor revealed multiple different single base pair variants in the VHL gene as a second hit. In summary, hirsutism in VHL patients should raise the suspicion for unusual ovarian tumors. In contrary to the usual theory of a monoclonal expansion after loss of the wt VHL allele, this tumor appeared to be oligoclonal as evidenced by different somatic VHL mutations. This could be either explained by initial parallel occurrence of several clones or that the VHL second hit is not an initial event, but the mutation instead supports tumor expansion following initial steps of tumorigenesis.

Background: Virilism is a female disorder in which secondary male sexual characteristics develop, caused by an excessive adrenal or ovarian androgen secretion. Case presentation: Here, we report an unusual case of an ovarian steroid cell tumor, not otherwise specified (NOS), in a 68-year-old female who presented with androgenic alopecia, clitoromegaly and an increased muscle mass. Laboratory investigations revealed both ovarian and adrenal hyperandrogenism with an elevation of androgen precursors mimicking congenital adrenal hyperplasia. A left adnexal mass was confirmed by imaging techniques. A laparoscopic bilateral salpingo-oophorectomy was performed and histopathology confirmed the diagnosis of an ovarian steroid cell tumor NOS. After surgical intervention, circulating androgen levels and their precursors returned to normal values in the postmenopausal woman. Conclusions: A detailed anamnesis and physical examination are key to the correct diagnosis in a woman with hyperandrogenism independent of her circulating androgen profile.

Objective: To raise awareness of the clinical presentation of a testosterone-secreting steroid cell tumor, not otherwise specified, causing pulmonary embolus (PE) and erythrocytosis. Methods: Report of the first known case of ovarian steroid cell tumor not otherwise specified leading to PE. Results: A 67-year-old Caucasian postmenopausal woman was referred for endocrine evaluation of a 1-year history of hirsutism, weight gain, and elevated total testosterone level of 672 ng/dL (normal

Context Cushing syndrome is rarely caused by the secretion of cortisol from ovarian tumors. In clinical decision-making, it is important to determine whether the ovarian tumor is capable of secreting cortisol. Selective ovarian and adrenal venous sampling is scarcely reported in the localization of ACTH-independent ectopic Cushing syndrome. Case Description We present a case of 40-year-old Chinese woman who had weight gain, hirsutism, hypertension, and menstrual disorder over 6 months. Her physical examination and biochemical assessment revealed adrenocorticotropic hormone-independent Cushing syndrome. Adrenal computed tomography scan indicated no abnormality. A mass of 5.7 cm × 4.2 cm × 3.4 cm was discovered by pelvic ultrasonography. Somatostatin receptor scintigraphy revealed no abnormal radioactivity intake. Combined ovarian and adrenal venous sampling together with a cortisol assay were conducted. Results revealed cortisol concentration of the right-side ovarian vein, left-side ovarian vein, and peripheral vein of 268.60, 29.00, and 35.18 μg/dL, respectively, suggesting a right-side ovarian origin. A right-side salpingo-oophorectomy was performed and the pathological diagnosis revealed ovarian steroid cell tumor, not otherwise specified. The cortisol level was substantially lower after the patient underwent surgery and symptoms of Cushing syndrome disappeared. At 3-year follow-up, the patient remained disease free, and no tumor was observed on pelvic ultrasonogram. Conclusion Combined ovarian and adrenal venous sampling is valuable in the localization of adrenocorticotropic hormone–independent ectopic Cushing syndrome.

Approximately, 5% of ovarian tumors have hormonal activity. Steroid cell tumors (SCTs) represent about 0.1% of all ovarian tumors. They cause hyperandrogenism associated with typical virilization. In this case report, we present 45-year-old women with unmalignant ovarian SCT-producing androgens which cause severe virilization and secondary amenorrhea lasting two years. Transvaginal ultrasound, computed tomography of adrenal glands, magnetic resonance imaging of small pelvis, laboratory tests (including serum concentration of FSH, LH, testosterone (T), androstenedione (A), dehydroepiandrosterone sulfate (DHEA-S), as well as ROMA index) were performed. During hormonal evaluation, elevated concentrations of serum T - on admission 1.72 ng/ml and one month later 3.75 ng/ml (normal range 0.08-0.82 ng/ml) and A - 24.90 ng/ml (normal range 0.40-3.40 ng/ml) were found. The ROMA index was within the normal range. Enlargement of the left ovary by solid mass 56 × 43 mm was found during ultrasound examination. Based on small pelvis MRI scan and hormonal finding, patient was qualified for laparotomy. During this procedure, the left salpingo-oophorectomy with removal of the tumor was performed. The histopathological examination identified SCT. During follow-up examination, one day after surgery, we found serum testosterone levels within normal ranges - 0.74 ng/ml (normal range 0.08-0.82 ng/ml). This case shows that hormone-producing ovarian tumors are rare but very important clinical causes of severe hyperandrogenism.

We describe a case of a 24-year-old overweight woman who presented with hirsutism, secondary amenorrhea, clitoromegaly, and symptoms of diabetes mellitus (DM). While a diagnosis of polycystic ovary syndrome (PCOS) with its associated metabolic disturbances was initially considered, serum total testosterone, androstenedione, and 17-hydroxyprogesterone (17-OHP) measured by liquid chromatography tandem mass spectrometry (LC-MS/MS) were significantly increased. As 17-OHP did not increase upon ACTH (Synacthen) stimulation and the urinary steroid profile (USP) was compatible with an ovarian source of 17-OHP excess rather than adrenal, non classical congenital adrenal hyperplasia (NCCAH) was unlikely and an androgen-secreting tumor was suspected. Transabdominal ultrasound revealed the presence of an enlarged right ovary with a polycystic ovary morphology and no discrete mass. Transvaginal ultrasound and [18F]− fluorodeoxyglucose positron emission tomography–computed tomography (FDG PET–CT) enabled the localization of a right ovarian tumor. Laparoscopic right salpingo-oophorectomy was performed and a histological diagnosis of steroid cell tumor, not otherwise specified (SCT–NOS) was made. Hyperandrogenism and menstrual disturbances resolved postoperatively. A literature review revealed that 17-OHP-secreting SCT–NOS may uncommonly show positive responses to ACTH stimulation similar to 21-hydroxylase deficiency. Alternatively, USP might be useful in localizing the source of 17-OHP to the ovaries. Its diagnostic performance should be evaluated in further studies.

Von Hippel-Lindau (VHL) syndrome is an autosomal dominant genetic disorder caused by germline mutation of the VHL gene. It is associated with multiple neoplasias including hemangioblastoma, clear cell renal cell carcinoma, pheochromocytoma, and neuroendocrine tumors. Ovarian tumors are extremely rare in this syndrome. We describe the case of a 16-yr-old girl with a previous diagnosis of bilateral pheochromocytoma and several pancreatic neuroendocrine tumors in VHL syndrome context. Follow-up abdominal-pelvic magnetic resonance imaging revealed a 33 mm, well-circumscribed nodule in the right ovary. The patient was submitted to laparoscopic right salpingo-oophorectomy. Microscopically, the tumor consisted of polygonal cells with abundant microvacuolized clear cytoplasm arranged in a solid pattern. The neoplastic cells were immunohistochemically positive for inhibin and calretinin. A diagnosis of ovarian steroid cell tumor was made. Only 4 cases with this association have been reported to date. Of the previously described cases, only one concerns a child; the others were all adult women. All of them had a previous diagnosis of VHL syndrome and presented with secondary amenorrhea and/or hirsutism due to testosterone-secreting ovarian steroid cell tumors. Although extremely rare, the association between VHL syndrome and ovarian steroid cell tumor has been reported, and our case suggests there is a link between the 2 entities.

Steroid cell tumor not otherwise specified is a subtype of steroid ovary tumors. These are a rare subgroup of ovarian sex cord-stromal tumors. We present a case of a young woman submitted to laparoscopic cystectomy with ovarian steroid cell tumor as histological finding. This represents the second case where laparoscopic cystectomy is performed in this type of tumor. Up to 36 months after surgery, the woman remains under surveillance, without recurrence of the tumor, and attempts to conceive. This case suggests that steroid cell tumors can be asymptomatic, and gynecologists must think about preserving fertility in women of reproductive age.

Ovarian steroid cell tumors are very rare but potentially life-threatening neoplasms. They represent less than 0.1% of all ovarian tumors, typically present in premenopausal women and frequently manifest with virilization. Signs of hyperandrogenism may appear in postmenopausal women due to tumorοus and non-tumorοus adrenal and ovarian causes as well due to the normal aging process. In any case, steroid cell tumor should be suspected in postmenopausal women who present with rapid progressive androgen excess symptoms. This report describes a case of a 67-year-old postmenopausal woman with signs of hyperandrogenism, where an ovarian steroid cell tumor was diagnosed and treated by laparoscopic bilateral salpingo-oophorectomy and synchronous hysterectomy.

Background: Steroid-cell tumors of the ovary are very rare, especially during pregnancy, and they must be distinguished from luteoma of pregnancy. Case: An 18-year-old female, gravida 3, para 1-0-1-1, at 38 weeks' of gestation, had an adnexal mass that was discovered incidentally during a Caesarean section. The tumor was excised and her male infant was normal. Results: Histologic workup revealed the tumor to be a steroid-cell tumor, which is exceedingly rare in pregnancy. Conclusions: Ovarian steroid-cell tumors, which are malignant one-third of the time, are difficult to distinguish from luteoma of pregnancy. (J GYNECOL SURG 32:351)

Steroid cell tumors (SCT), not otherwise specified (NOS) are particularly rare ovarian sex cord-stromal tumors, which comprise

Steroid cell tumors, not otherwise specified, are infrequently encountered ovarian neoplasms, which constitute

Aim Ovarian steroid cell tumors, not otherwise specified (SCT-NOS) are very rare neoplasms. No large study has been performed in Pakistan to establish the clinicopathological spectrum and immunohistochemical behavior in our region. The purpose of our study was to determine the various clinicopathological and immunohistochemical features of ovarian SCT-NOS along with follow-up in our institution. Methods This was a retrospective observational study. The study was conducted in the Section of Histopathology, Aga Khan University Hospital, Karachi, Pakistan. All reported cases of ovarian SCT-NOS occurring during January 1992 to August 2013 were retrieved. The slides were reviewed and patient demographics, and clinical and pathological features were noted with proforma software. SPSS version 19 was used for all analyses. Data is expressed as absolute values and percentages. Results A total of 12 SCT-NOS (2.3%) out of 528 ovarian sex cord stromal tumors were retrieved. The age range was 3–70 years, with mean of 40.75 years. The tumors ranged 2.5–13 cm in size, with a mean size of 6.1 cm. One patient had bilateral tumors. All of the tumors were positive for inhibin and calretinin. Four tumors were negative for Mic-2 (CD99). In two patients, the tumor recurred. Only one patient who had worse pathological features received adjuvant chemotherapy. Conclusion Steroid cell tumors are very rare ovarian tumors in the Pakistani population, mostly presenting in adulthood. Diverse histological differentials exist so special stains and immunohistochemical stains are needed to distinguish these from other tumors.

Steroid cell tumor, not otherwise specified, is a rare type of ovarian sex cord-stromal tumor with malignant potential. Some of these tumors produce testosterone. We describe a case of steroid cell tumor of the ovary associated with virilization. A 23-year-old nulliparous woman was found to have an ovarian tumor when she visited her primary doctor for virilization and oligomenorrhea. Magnetic resonance imaging revealed a solid left ovarian tumor 40 mm in size. Her laboratory data revealed elevated testosterone with normal levels of gonadotropins, estradiol, dehydroepiandrosterone sulfate and cortisol. She underwent left adnexectomy. On histopathologic and immunohistochemical analyses, the tumor was diagnosed as steroid cell tumor, not otherwise specified, without malignant behavior. After removal of the tumor, serum testosterone level decreased, and there have been no signs of recurrence.

Steroid cell tumors not otherwise specified (NOS) are a rare subgroup of sex cord-stromal tumors. The tumors can occur at any age, although the mean age of occurrence is 43 years old. The majority are benign, but have the capability of producing one or more steroids associated with virilization. The present study reports the case of a 29-year-old female who presented to the Second Xiangya Hospital suffering from lower back and leg pain that had persisted for five months. The patient had regular menstrual cycles and no virilization symptoms were present. Laboratory investigations revealed normal hormone levels. Multiple areas of bone destruction and a right ovarian mass were confirmed via positron emission tomography/computed tomography. The patient underwent an exploratory laparotomy, and a mass measuring ~6 cm in diameter was subsequently identified in the right ovary. A right salpingo-oophorectomy and pelvic washings for cytology were performed. Histopathological studies confirmed the diagnosis of a malignant steroid cell tumor NOS of the right ovary. The patient underwent eight cycles of chemotherapy (docetaxel, 120 mg and nedaplatin, 80 mg). The patient continued to have relatively good health, with no deterioration of the condition for one year and a half, however, the disease progressed and the patient succumbed to brain metastases six months later.

Steroid cell tumors (SCTs) of the ovary are a rare subgroup of sex cord tumors that account for less than 0.1% of all ovarian tumors. These tumors can produce steroids, especially testosterone, which produces symptoms such as hirsutism, amenorrhea/oligomenorrhea, and male patterned voice. For evaluation of the androgen excess, testosterone and dehydroepiandrosterone sulfate (DHEA-S) are the first laboratory tests to be measured. Abdominal ultrasound and magnetic resonance imaging (MRI) are useful radiologic imaging techniques. Although SCTs are generally benign, the risk of malignant transformation is always present. Surgical excision of tumor is the most important and hallmark treatment. The present case signifies the early preoperative diagnosis of a virilizing SCT, based on cytological features and its careful correlation with clinicopathological and radiological findings.

Introduction: Steroid cell tumors of the ovary present less than 0.1% of all ovarian tumors and belong in the group of sex cord-stromal tumors.Case description: We present a case of 69-year-old woman investigated because of postmenopausal bleeding, a 5-year history of excessive hirsutism, baldness and acne. The evaluation revealed elevated serum testosterone, but ultrasound detected a 2 cm-mass of the left ovary. The patient underwent hysterectomy and bilateral adnexectomy. The histopathology diagnosis was steroid cell tumor, not otherwise specified. Postoperative chemotherapy was administered at the discretion of the radio-oncologist. At the last follow-up 48 months after surgery, the hirsutism was completely resolved, the serum testosterone was within the normal range and there was no evidence of recurrence.Conclusion: In adult patients with hirsutism and elevated serum testosterone a possibility of a presence of an ovarian steroid cell tumor should be considered. Surgery is the main treatment of such patients.

Virilizing or masculinizing features in a female can be an alarm for the doctor and a source of embarrassment for the patient. These features appear as a result of hyperandrogenism, the source being ovary or adrenal gland. Here is a case of Virilizing ovarian tumour in a 51 year old diabetic female. This patient presented with a 14 year history of appearance of hair all over the body with loss of scalp hair. She also had complaints of pain in lower abdomen with excessive micturition for 3 months. Examination showed hirsutism and features of virilisation. Suprapubic mass was present on abdominal examination. Per vaginal exam revealed a soft mass? Ovarian. Radiological tests showed features suggestive of ovarian malignancy with possible uterine involvement and mild ascites. Most routine hematological investigations were normal. Total testosterone was grossly elevated (>1500 ng/dl); CA-125 was also raised (103.7 U/ml). FSH & LH values were low, TSH was normal while estradiol& progesterone were raised. Urine microscopy had features of urinary tract infection; hence treatment was started for the same. Patient underwent panhysterectomy. Histopathological studies confirmed steroid cell left ovarian tumour (T1aN0Mx), which was the source of virility in the patient. Patient improved dramatically

INTRODUCTION: Steroid cell tumor of the ovary a very rare androgen secreting tumor accounts for less than 0.1% of ovarian tumors. These tumors may present at any age ranging from 2 to 80 yrs & associated with hormonal activity & virilisation. These tumors are most commonly unilateral, may be both benign or malignant, most of these tumors are usually diagnosed in early stages because of virilisation caused by these tumors. In an extensive literature search (medline & pubmed) from 1979, only around 80 cases of ovarian steroid cell tumors have been reported. As ovarian stromal tumors are infrequent, little attention was given to their response to radiotherapy and chemotherapy. A recent report stated gonadotropin releasing hormone agonist was effective in treating steroid cell tumors. Here we present the case of a rare ovarian steroid cell tumor in a 32 yrs old woman who presented with virilisation. CASE REPORT: A 32 yrs old woman with P2L1D1A1 presented with history of amenorrhea of 7 months duration, hoarseness of voice since 6 yrs, decrease in breast size since 6 yrs, lower abdominal pain which is dull aching type, coarse hair growth over the body since 2 yrs. She attained menarche at the age of 12 yrs. Her previous menstrual cycles were normal. Physical examination revealed male pattern of coarse hair distribution in the beard region, face, chest wall, abdomen, arms and thighs. Both breasts were small in size, no mass palpable per abdominally.

The distinction of ovarian granulosa cell tumors (GCTs) from other sex-cord stromal tumors may be difficult histologically. Many immunohistochemical markers have been studied for this differential diagnosis, but the available markers are not entirely specific for ovarian GCT. 14-3-3 sigma has been shown to play an anti-apoptotic role in maintaining the viability of immortalized granulosa cells. However, the potential use of this molecule as an immunohistochemical marker for the diagnosis of ovarian GCT has not been investigated. A total of 103 ovarian sex-cord stromal neoplasms were immunostained with 14-3-3 sigma. These tumors included 44 adult granulosa cell, 7 juvenile granulosa cell tumors, 12 steroid cell tumors, 3 well-differentiated Sertoli-Leydig cell tumors, 5 Sertoli cell tumors, 10 thecomas, 18 fibromas, 2 primary ovarian endometrial stromal sarcomas, and 2 unclassified sex-cord stromal tumors. Ten ovaries with cystic follicles were also included as controls. Perinuclear or cytoplasmic stain was considered to be positive. Granulosa cells within the cystic follicles were positive for 14-3-3 sigma protein. All ovarian GCT (51/51) and all steroid cell tumors (12/12) were positive for 14-3-3 sigma, and all Sertoli cell tumors, fibromas, thecomas, ovarian endometrial stromal sarcomas, and sex-cord stromal tumors, unclassified, were negative for 14-3-3 sigma. The percentage of positive cell staining is statistically significant (P

SUMMARY Steroid cell tumors are tumors that account for less than 0.1% of all ovarian malignant tumors and cause increase in secretion of androgen or estrogen. Steroid cell tumors usually occur in the post-menopausal period. This article reports four steroid cell tumor cases who underwent surgery for overian mass during post-menopausal period, who were diagnosed with steroid cell tumor after histopathological examination and displayed different clinical manifestations. No signs of increased hormonal activity in two of these cases were observed. On the other hand, the other two cases applied with complaints of menopausal vaginal bleeding, which led to the consideration of increased estrogenic activity in these two cases. Case 1 The case, who was 77 years of age in her postmenopausal period, applied to our hospital with the complaint of urinary incontinence. The routine laboratory findings of the patient, who had no feature in her history, were within normal limits. No findings of virilization were observed during the physical examination of the patient. During the gynaecological examination, a mass of approximately 6 cm was detected in the right adnexal area. The transvaginal ultrasonography (TVUSG) revealed an endometrial thickness of 9 mm, and an approximately 72mm-sized solid-cystic mass, surrounded with free liquid in the right ovary area. The preoperative Case 2 In the TVUSG performed during the routine follow- up examinations at the menopause clinic of the 52 year-old case, who did not have active complaints, a 66 X 35 mm mass with the possibility of dermoid cyst was detected in the right ovary. There was no feature in the patient's physical examination, her history and family history. The gynaecological examination, during which an approximately 6 cm mass was palpated in the right adnexial area, revealed a CA 125 value of 9.07 IU/ml. With the preliminary diagnosis of pelvic mass, laparoscopic right salpingo-ooforectomy was performed. The intraoperative pathological examination reported "malignancy was not observed". The paraffin block indicated a right ovarian steroid cell tumor; the largest diameter of the capsule was 8 cm and the capsule had a ruptured appearance; there was 1 mitosis /10 HPF, focal light atypia and focal hemorrhage but there was no presence of necrosis. Based on these findings, it was decided that the potential for malignancy was low. Adjuvant treatment during the postoperative period was not considered. The patient did not come to the follow-up examinations after the operation. Figure 2: The postoperative paraffin block section of case 1. Case 3 The 64-year-old case who was in her postmenopausal period applied to our hospital with vaginal bleeding complaints. The routine laboratory findings of the patient, who had no feature in her history and family history, were within normal limits. In the physical examination of the case, virilization and increased hormonal activity findings were not observed. It was thought that postmenopausal vaginal bleeding could be associated with hyperestrogenic environment. A 10 cm-diameter mobile mass, the borders of which could not be clearly differentiated from the uterus and right ovary and filled up the Douglas space, was detected during the gynaecological examination. The TVUSG