Ovarian dysgerminoma associated with Pseudo-Meigs' syndrome and functioning ovarian stroma: a case report

BACKGROUND: We present the first case of an ovarian dysgerminoma complicated by pseudo-Meigs' syndrome. Furthermore, this is the fourth reported case of ovarian dysgerminoma with functioning ovarian stroma resulting elevated androgen levels preoperatively. CASE: A 25-year-old white female was referred to our department for abdominal swelling and a rapidly enlarging abdominal mass. Chest X-ray showed massive right pleural effusion. Abdominopelvic CT scan showed a left adnexal solid mass and ascites. Preoperative abnormally elevated hormone levels were as follows: free testosterone 7.7 pg/mL, androstenodione 13.6 ng/mL, and cortisol 29.4 microg/dL. Left salpingo-oophorectomy and wedge resection of the right ovary were performed. Final histopathological investigation of the left ovary was dysgerminoma associated with stromal luteinization. CONCLUSION: Dysgerminoma should be considered in the differential diagnosis in a young patient with a pelvic mass, ascites, and pleural effusion and preoperative counseling should be directed accordingly. In addition, dysgerminomas may be accompanied by ovarian stromal luteinization and steroid hormone production, which occasionally result in chemical or clinical hyperandrogenism.     

Primary carcinoid tumor of the ovary: a case report

Primary ovarian carcinoid tumors are rare. A 47-year-old woman presented to our emergency room with lower abdominal pain. Physical examination, pelvic ultrasonographic evaluation and abdominal computed tomography revealed a 10-cm mass in the right ovary containing cystic and solid components, as well as calcifications typical of a dermoid cyst. At laparotomy, a smooth-surfaced, firm and mobile right adnexal mass with solid and cystic portions was detected. Initially, right salpingo-oophorectomy was performed. Frozen-section examination identified the mass as a sex cord stromal tumor containing a mature cystic teratoma. Based on this finding, total abdominal hysterectomy, left salpingo-oophorectomy, omentectomy, appendectomy were performed, and the pelvic-paraaortic lymph nodes were also removed. All histological findings in the right ovary were similar to the features of cystic teratoma and trabecular carcinoid tumor. Examination of the resected lymphatic, omental, and appendiceal tissue indicated no tumoral invasion. The diagnosis was ovarian carcinoid Stage IA. Serum testing post-surgery revealed that the levels of cancer antigen (CA) 19-9 and CA125 were 18.5 u/ml and 10.5 u/ml, respectively. The patient was discharged on postoperative day 5. The report describes the clinicopathologic and immunohistochemical features of a primary ovarian carcinoid that contained a mature cystic teratoma.     

Virilizing adrenal adenoma: a report of a case

Androgen excess can be due to different entities such as polycystic ovarian syndrome, ovarian tumors or adrenal tumors. It is presented the case of a 26 year-old woman that suffered from amenorrhea, hirsutism, voice deepening, reduction of mammary volume and 10 kg weight loss, without response to different treatments. At physical exam she had hirsutism (24 points, Ferriman-Gallaway) the clitoris had 3 cm length. Laboratory: androstenedione 29.5 ng/mL, DHEAS >1000 microg/dL, T 6.23 ng/mL, 17 OHP 4.9 ng/mL. At pelvic ultrasound the uterus and left ovary were normal, the right ovary had subcortical follicles no greater than 3 mm. The CAT scan and nuclear magnetic imaging of adrenal glands showed an oval retroperitoneal image of 7.2 x 6.5 x 8.4 cm at the right adrenal gland. Surgery was performed and the right adrenal gland excised. The histopathologic report indicated a benign cortical adenoma. The patient's postsurgical evolution was satisfactory with regression of the virilizing signs, with spontaneous return of menstrual periods. Hirsutism can be the initial sign, and even in some occasions the only one of different pathologies. The study of the hyperandrogenic patient should be integral with clinical, hormonal and imaging evaluations to be able to specify the origin of androgen production.     

Ovarian cystadenoma with stromal cell hyperplasia and postmenopausal virilization: a case report

A 73-yr-old woman had noted progressive virilization. The plasma testosterone levels were elevated (340 ng/dl) and the pelvic ultrasound examination revealed an enlarged left ovary. At laparotomy, a large cystic tumor replaced the left ovary. The plasma testosterone became normal after the removal of the tumor. On anatomical analysis, the tumor was an ovarian cystadenoma with hyperplasia of stromal cells in the periphery of the tumor.     

Stromal Leydig cell tumor of the ovary. Case report and literature review.

The stromal Leydig cell tumor is a very rare benign tumor originating from the ovarian stroma. Only seven cases have been reported, all in postmenopausal women, except for one in a 15-year-old girl. In the present case, masculinization developed over a few months in a 24-year-old woman. The serum concentration of testosterone was 4.7 ng/ml before operation. Left salpingo-oophorectomy and wedge resection of the right ovary were performed. The encapsulated left ovarian tumor was an ovarian stromal Leydig cell tumor on microscopic examination.     

LH receptor in testosterone-producing ovarian tumor. In vivo and in vitro HCG-stimulated testosterone production

The aim of the present work is to report in vivo and in vitro hormonal studies performed on a 71 year old virilized woman due to a Leydig cell tumor of the ovary. Testosterone (T), Cortisol, DHEA(s) and ACTH concentrations were determined in blood samples taken every 4 h throughout 24 h previous to surgery. T average concentration from the 6 samples was 3.3 ng/ml (range: 2.5-4.2 ng/ml). DHEA(s) was normal; Cortisol and ACTH levels were normal and their circadian rhythms were present. T value obtained during Dexamethasone administration (2 mg daily/3 days) was 2.4 ng/ml. This value was significantly higher than those obtained from postcorticoid normal women (0.3 +/- 0.1 ng/ml), suggesting an extraadrenal source. T concentration was 5.4, 6.0 and 6.6 ng/ml at 24, 48 and 72 h after hCG injection (5000 IU). After tumor removal, T values decreased progressively up to 6.0 ng/ml values, 5 days later, and remained steady on the following days. The studies performed in vitro were: determination of T in the tumor cytosol, specific binding of LH to ovarian tumor cell membrane fraction and T production in tissue culture in both with and without added hCG conditions. Normal ovarian tissue from the same patient under similar experimental conditions was used as control. The T concentration expressed as ng/mg of protein in the tumor and normal ovarian cytosol was 9.1 and 1.1, respectively. Scatchard analysis of specific 125I-hCG binding to tumor and normal ovarian cells indicated 53 pg and 28 pg of labeled hCG bound/mg of membrane protein, respectively, suggesting that this Leydig cell tumor of the ovary contained LH (hCG) receptors. The amounts of T, expressed as ng/mg of tissue/6 days, generated by tumoral and normal tissue ere 4.1 and 0.3, respectively. The addition of hCG elicited a response of 6.3 and 0.6 ng/mg protein/6 days in both preparation, respectively. These results demonstrate in vivo and in vitro hCG-stimulated T production in this particularly masculinizing ovarian tumor and suggest tumoral LH dependence.     

Endocrine profile and electron microscopic study of ovarian androblastoma

The endocrine profile of a 23-year-old woman with an androblastoma of the right ovary and the results of electron microscopic observation of the tumor are presented. The calculated ratio of testosterone in testosterone intraoperative peripheral vein blood, right ovarian vein blood, and right ovarian tumor fluid was 1:1.4:4.0. The peripheral levels of hormones before right salpingo-oophorectomy were testosterone, 6.26 ng/ml; dehydroepiandrosterone, 17.80 ng/ml; androstenedione, 1.61 ng/ml; and cortisol, 16.5 micrograms/ml, and the corresponding levels at 14 days after surgery were 0.50 ng/ml; 13.80 ng/ml; 1.27 ng/ml; and 15.2 micrograms/ml, respectively. The tumor was responsive to hCG, resulting in a marked increase in the serum concentrations of testosterone, androstenedione, and dehydroepiandrosterone (3.7 times, 5.3 times, and 2.4 times, respectively). Preoperatively, an elevated basal level of luteinizing hormone (LH) and a normal basal level of follicle-stimulating hormone (FSH) (high LH:FSH ratio, 6.6) were found. The electron microscopic findings for the tumor revealed Leydig cells, Sertoli cells, transitional cells with features of both Sertoli cells and Leydig cells, and dark cells. The dark cells had features similar to dark cells of normal ovarian stroma.     

Rare case of an ovarian monodermal teratoma with functional stroma and extensive ovarian decidualization in a 74-year-old woman

We present the clinicopathological findings of a rare case of a monodermal teratoma of the right ovary with functional ovarian stroma and extensive decidualization in a 74-year-old woman. The patient presented with vaginal bleeding. Ultrasound scan revealed a pelvic mass measuring 9.5 cm in the lower right abdomen. A right oophorectomy was performed. The tumor was cystic and multilocular filled with colloid material. Histological examination revealed follicles of thyroid type, and stromal clusters of fusiform or polygonal cells were found in the stroma. An extensive decidual reaction was observed. Morphological and immunohistochemical examination of the tumor revealed cystic struma ovarii with functional ovarian stroma and ectopic decidua. Total abdominal hysterectomy with oophorectomy was performed. A benign endometrial polyp, proliferative endometrium, two fibroids, and an ovarian cyst were observed.     

A large pelvic mass in an adolescent patient with granulomatous nephritis: case report and discussion of treatment challenges

BACKGROUND: An adolescent patient with granulomatous nephritis presents with a large, solid pelvic mass. Pertinent differential diagnosis for this solid ovarian mass as well as discussion regarding treatment challenges for this patient is delineated. CASE: A 15-year-old female presented to her primary care doctor with fatigue and syncope. Initial laboratory workup revealed a hemoglobin of 7.9 g/dL, an elevated creatinine of 3.5 mmol/L, and an elevated ionized calcium of 13.1 mg/dL. Renal biopsy revealed diffuse non-caseating granulomatous nephritis with rare acid-fast bacilli. Renal ultrasound first noted a pelvic mass. Pelvic ultrasound revealed a 15.0 x 8.4 x 12.2 cm mass, characterized as mostly solid with diffuse spaces, in the location of the right ovary. CA-125 and the lactate dehydrogenase (LDH) tumor markers were elevated. The patient underwent a left salpingo-oophorectomy and pelvic staging. Intra-operative frozen section revealed a dysgerminoma. Final pathology report revealed extensive non-caseating granulomatous inflammation within the ovarian tumor. Special stains showed no evidence of acid-fast organisms. CONCLUSION: Dysgerminoma is the most likely solid ovarian tumor in a patient of this age. In light of her initial renal biopsy with acid-fast bacilli, pelvic tuberculosis needs to be considered. Due to its extreme rarity, sarcoidosis of her genital tract should be lower on the differential, yet this patient presented with pathology consistent with non-caseating granulomas suggesting this diagnosis. Once ovarian dysgerminoma was diagnosed, the possibility that this patient's renal findings may represent paraneoplastic syndrome also becomes important for her treatment.     

Ovarian steroid cell tumor as an example of severe hyperandrogenism in 45-year-old woman

Abstract

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.     

Ovarian Hemangioma Associated with Concomitant Stromal Luteinization and Ascites

A 62-year-old female presented with a pelvic mass and ascites. The Papanicolaou vaginal smear showed an unusual maturation, maturation index being 0/80/20. The serum level of estradiol was 48.7 pg/ml. The preoperative checkup suggested a pelvic malignancy with a differential diagnosis of hormone-secreting ovarian tumor. On surgical exploration, she had a hemangioma of the ovary without malignant cytology in the ascitic fluid. Histologically, this tumor was associated with stromal luteinization. This is the first case, reported in the literature, possessing ovarian hemangioma with stromal luteinization accompanying massive ascites. It should be noted that an ovarian hemangioma can be associated with stromal luteinization and ascites, and that MR imaging is sometimes of value for making a preoperative diagnosis of ovarian hemangioma.     

Ovarian thecoma with androgenic manifestations in a postmenopausal woman.

A 50-year-old woman, who presented with progressive androgenization, central obesity and severe hypertension, was initially suspected to have an adrenal virilizing tumor. Her serum testosterone level was in the male range (9.3-11.6 ng/ml) and was not suppressed with dexamethasone. Although no pathological abdominal or pelvic mass was detected, total hysterectomy and bilateral salpingo-oophorectomy was performed. Histopathological examination revealed a theca-cell tumor of the right ovary. Postoperatively the testosterone level returned to normal and the patient had regression of virilism. Our case illustrates that a virilizing ovarian tumor can be small and elude imaging studies, but may be detected by means of well-considered clinical management.     

Sclerosing stromal tumor of the ovary in a 30-year-old woman. A case report and review of the literature

Sclerosing stromal tumor of the ovary (SST) is an extremely rare neoplasm occurring predominantly in the second and third decades of life. It is a distinct benign neoplasm that differs from fibromas, thecomas, luteinized tumors and lipoid cell tumors. It presents most often with non-specific symptoms. We describe the case of a 30-year-old woman who developed menstrual irregularities and pelvic pain. She was diagnosed and treated operatively at our Department. During surgery, a benign tumor was found in the right ovary. Light microscopic and ultrastructural study confirmed the diagnosis of sclerosing stromal tumor of the ovary.     

Sclerosing stromal tumor in an accessory ovary

BACKGROUND: An accessory ovary is an uncommon congenital anomaly of the female reproductive tract. Tumors arising in ectopic ovaries are extremely rare. CASE: This 18-year-old woman had a pelvic tumor attached to the right ovary which was solid cystic and lobulated and histologically and immunohistochemically showed the appearances of sclerosing stromal tumor, a benign entity. CONCLUSION: We present the first reported case of sclerosing stromal tumor that arose in an accessory ovary.     

Virilising Sertoli–Leydig cell tumour associated with thyroid papillary carcinoma: case report and general considerations

We present a case of a Sertoli–Leydig cell tumour manifested with progressive hirsutism, frontal alopecia and secondary amenorrhea in a 46-years-old female, evolving for 6 years until presentation. Serum testosterone level was 8.01 ng/ml and gonadotropic hormones were LH 8.57 mIU/ml and FSH 9.52 mIU/ml. Computed tomography revealed a dense, solid, heterogeneous mass of 3.5/2.8?cm in the right ovary. Bilateral ovariectomy and hysterectomy were performed. The histopathological report mentioned a Sertoli-Leydig cell tumor with intermediate grade of differentiation. Immunohistochemical stains showed positive reaction for α-inhibin, calretin and for progesterone receptor. The testosterone levels dramatically decreased after surgery (0.31 ng/ml) while levels of gonadotropes increased: LH 40.98 mIU/ml and FSH 50.41 mIU/ml. At 6 months follow-up the diagnosis of a left lobe thyroid nodule leaded to fine needle aspiration biopsy with suspicion of papillary carcinoma. Total thyroidectomy established the diagnosis of thyroid papillary carcinoma (2.17/2.18?cm) T2N0M0, stage II, followed by radioiodine administration. This is to our knowledge the first presented case of ovarian Sertoli–Leydig cell tumour associated with papillary thyroid carcinoma. This could suggest a common genetic background.     

Endocrinological study of Sertoli-Leydig cell tumor

A 32-year-old woman, 3, para 2 with a testosterone producing tumor of the left ovary was studied endocrinologically. She complained of amenorrhea, hirsutism and abdominal mass. The peripheral testosterone level was 8.4 ng/ml, remarkably elevated. After the combined dexamethasone suppression/hCG stimulation test, the plasma testosterone level rose from 9 ng/ml to 15.1 ng/ml. Prior to removal of the tumor, venous samples were drawn directly from the right and left ovarian veins during surgery and simple total hysterectomy with bilateral salpingo-oophorectomy was performed. Testosterone levels were 40.5 ng/ml on the tumor side and 7.1 ng/ml on the normal side. After tumor removal, the plasma testosterone level fell from 8.4 ng/ml to less than 1.0 ng/ml within 24 hours. Histological examination of the left ovarian tumor revealed a Sertoli-Leydig cell tumor. In incubation of small specimens of tumor tissues in oxygenated Krebs bicarbonate buffer, the release of testosterone into the medium containing hCG was twice as high as that into the medium without hCG. These results of in vitro and in vivo studies suggest that this tumor was an hCG-dependent testosterone producing Sertoli-Leydig cell tumor.     

妊娠合并卵巢幼年型颗粒细胞瘤一例报告及文献复习

目的：分析妊娠合并卵巢幼年型颗粒细胞瘤（juvenile granulosa cell tumor,JGCT）的临床特点、诊断要点、治疗及预后,提高鉴别诊断水平。方法：回顾性分析 1 例妊娠合并JGCT病例资料,复习相关文献。结果：患者27岁,因停经39+3周,间断右下腹疼痛3 d入院。彩色超声提示右下腹部一8.5 cm×6.1 cm囊实混合回声。查糖类癌抗原125（CA125） 32.3 U/mL, CA19-9为19.1 U/mL,诊断为先兆临产,盆腔包块（性质待排）,行剖宫产术+剖腹探查术,探查见右侧卵巢一囊性肿物,已破溃,行右侧卵巢肿物切除术,术后病理诊断为卵巢JGCT。行二次手术,给予右侧卵巢输卵管切除术+左侧卵巢活检术+盆腔淋巴结取样术+部分大网膜切除术+阑尾切除术+盆腔粘连松解术。术后予顺铂+长春新碱+博莱霉素（PVB方案）化疗3个疗程,随访8个月,肿瘤无复发。结论：JGCT是一种罕见的恶性肿瘤,发生于妊娠期者更为罕见,确诊需依赖病理及免疫组化检查。     

Virilizing adrenal adenoma and primary amenorrhea in a girl with adrenal hyperplasia

We report a 14-year-old girl with primary amenorrhea and virilization. The chromosomal analysis showed a normal 46,XX female karyotype and the hormonal assays disclosed high serum levels of testosterone (T): 450 ng/dL (normal 5-90), dehidroepiandrosterone-sulfate (DHEA-S): 690 microg/dL (normal 30-450) and 17-hydroxiprogesterone (17-OHP) >20 ng/mL (normal <2). A pelvic ultrasound disclosed a small uterus and bilateral enlargement of the ovaries, a computed axial tomographic scan of the abdomen identified a large right mass in the adrenal gland and a norcholesterol-I 131 adrenal gammagraphy revealed a functional adrenal tumor. The histological analysis of the surgical removed tumor revealed and adrenal adenoma. After surgery, a steep decline to normal serum levels of T and DHEA-S was observed, remaining an elevated level of 17-OHP: 5.4 ng/mL. During the first three months of follow up, the hirsutism declined sharply and spontaneous mammary development occurred, remaining elevated the 17-OHP serum level: 4.8 ng/mL. Prednisone 5 mg/day, was initiated decreasing the 17-OHP to normal level: 1.4 ng/mL, appearing the menarche followed by cyclical menses. One year after surgery, prednisone was withdrawn during one week, and an ACTH test and HLA typing were done, disclosing a 17-OHP response of an heterozygote for adrenal hyperplasia, and identifying B65 a subtype of B14, and DR1, that are frequently associated to adrenal hyperplasia. Previous reports have informed silent adrenal tumors associated to adrenal hyperplasia, but this is the first report of a functional adrenal tumor associated to adrenal hyperplasia.     

Enzyme immunoassay for placental protein 4 (PP4) and its possible diagnostic significance in patients with genital tract cancer

Summary We have established an enzyme immunoassay for placental protein 4 (PP4), by using avidin-biotin binding reaction, and set its normal range below 10.9 ng/ml (mean + 2σ). Throughout the menstrual cycle, the serum PP4 profile was similar to that of serum progesterone. In the follicular and ovulatory phase, PP4 remained relatively low, with the mean levels of 1.5 ng/ml and 1.8 ng/ml, respectively. In the luteal phase, the mean level was 3.2 ng/ml. In normal pregnancy, serum PP4 levels were low irrespective of gestational age, with a mean level of 3.0 ng/ml. There was only one case in which the serum PP4 level over 10.9 ng/ml. Mean serum PP4 levels and the frequencies of elevated serum PP4 levels were respectively 6.3 ng/ml and 11% in patients with benign ovarian neoplasms, 4.7 ng/ml and 6% in patients with endometriosis, and 5.5 ng/ml and 18% in patients with uterine myomata. The frequency of raised PP4 levels was 48% and the mean value was 13.3 ng/ml in patients with endometrial carcinoma, and the values were 44% and 13.4 ng/ml respectively in patients with cervical carcinoma. In patients with ovarian malignancy, the respective values were 15% and 7.0 ng/ml. The results did not relate to clinical stages of disease (FIGO), while the frequencies of elevated serum PP4 in patients with uterine carcinoma was over 40% in stage I diseases. Compared with other tumor markers such as carcinoembryonic antigen (CEA), tissue polypeptide antigen (TPA) and cancer antigen 125 (CA125), PP4 seems to be more promising as a marker of endometrial carcinoma. In patients with recurrent gynecological malignancy, 79% of serum PP4 levels were elevated. In endometrial carcinoma and recurrent gynecological malignancy, stromal destruction might be the cause of elevated serum PP4 levels.