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Secondary amenorrhea and infertility caused by an inhibin-B-producing ovarian fibrothecoma.

OBJECTIVE: To report a case of secondary amenorrhea and infertility caused by an inhibin-B-producing ovarian fibrothecoma. DESIGN: Case report. SETTING: Academic medical center. PATIENT: A 37-year-old woman with a 2-year history of secondary amenorrhea and infertility. INTERVENTION(S): Operative removal of a 5-cm ovarian fibrothecoma. MAIN OUTCOME MEASURE(S): Luteinizing hormone, FSH, E2, inhibin-B, TSH, and prolactin measured preoperatively and postoperatively. Immunostaining of tumor cells for inhibin and LH. RESULT(S): Preoperative hormone levels were as follows: FSH, 1.7 mIU/mL; LH, 23.4 mIU/mL; E2, 31 pg/mL; and inhibin B, 1,154 pg/mL. Three weeks postoperatively, the FSH was 1.5 mIU/mL, LH decreased to 7.1 mIU/mL, E2 increased to 276 pg/mL, and inhibin-B decreased to 17 pg/mL. The fibrothecoma did not stain for LH but was strongly positive for inhibin. Regular menstrual cycles resumed 28 days postoperatively. CONCLUSION(S): Inhibin-B produced by an ovarian tumor profoundly suppressed FSH levels and resulted in secondary amenorrhea and infertility. Use of sensitive and specific immunoassays for inhibin-A and -B may aid in the differential diagnosis of hormonally active ovarian tumors.

Adult↗

Malignant sex cord-stromal tumor of testis.

We report a case of sex cord-stromal tumor in the testis, which metastasized to the retroperitoneal space, resulting in death 13 months after orchiectomy. The primary lesion consisted of granulosa cells, theca cells, Sertoli cells and undifferentiated gonadal stroma. Biopsy of the retroperitoneal metastasis showed predominance of the Sertoli cell component.

Adult↗

Luteoma of pregnancy: ultrastructural features.

The ultrastructural configuration of the cells in one case of pregnancy luteoma was studied by conventional electron microscopy. The fine structure of these cells conforms closely to that of steroid hormone producing cells in other human organs, such as the adrenal cortex, testicular interstitium, and corpus luteum, particularly in terms of the presence of abundant smooth endoplasmic reticulum, dispersed Golgi apparatus, and tubular cristae in the mitochondria. Similarities were noted between the pregnancy luteoma cells and, as described by other authors, the luteinized granulosa and theca cells of the human corpus luteum and the cells of spontaneous mouse luteoma. There was a more pronounced resemblance to the granulosa cells of the corpus lutemum than to the luteinized theca cells or the mouse luteoma cells but with considerable overlap among all of them. Distinctive features, not previously described, were noted in the pregnancy luteoma cells in the form of deep cytoplasmic invaginations of the cell membrane closed by tight junctions, and peculiar pleated sinuous membranous arrays within the cytoplasm. The significance of these structures is not known. In addition mesenchymal cells of ambiguous differentiation were noted in the stroma of the tumor, suggesting that the stimulus to luteinized cell proliferation may transcend the specific ovarian mesenchymal cells and may also be exerted on the supporting stroma. On the basis of previous reports concerning the biochemical and biosynthetic patterns of sex steroid hormone production by these tumors, it is concluded that although the latter recapitulate the endocrine functions of the normal ovarian stroma rather than those of the corpus luteum, the cytoarchitecture of these tumors covers a much broader spectrum of differentiation, which may come to resemble closely although not exclusively that of luteinized granulosa cells.

Adult↗

Gonadotropic hormone binding to human ovarian tumors.

A variety of normal human ovarian tissues as well as benign and malignant human ovarian neoplasms have been examined in vitro for the presence of gonadotropin binding sites and gonadotropin-stimulable cAMP production, in order to determine whether gonadotropic hormones have an effect on ovarian tumors. Binding of either FSH or hCG, or both, was demonstrated in several tumors from each of the histologic subgroups of primary ovarian tumors, including epithelial, sex cord-stromal, and germ cell types. Cyclic AMP stimulation was found in one tumor of sex cord-stromal origin, in two of epithelial origin, in two of germ cell origin, and in one tumor-like condition, pregnancy luteoma. Additional biochemical studies are needed to characterize receptor sites and to relate them to histologic features, growth behavior in vivo, and serum gonadotropin levels.

Adolescent↗

Classification and pathology of testicular germ cell and sex cord-stromal tumors.

Germ cell tumors of the testis are the most frequent testicular neoplasms, with seminoma predominating. The pathologist must be able to discriminate between seminoma and nonseminomatous germ cell tumors as well as sex cord-stromal tumors and metastatic lesions. Appropriate therapy and accurate prognostic information are dependent on the proper classification of testicular neoplasia. Characteristic histologic features, serum markers, and immunohistochemistry are helpful in this regard. Sex cord-stromal tumors comprise a small minority of testicular neoplasms. It remains critically important not to confuse these neoplasms with testicular germ cell or metastatic tumors, and, again, recognition of the characteristic histologic features, immunohistochemical findings, and clinical information is diagnostic. The urologist can provide the pathologist with key clinical information in the attempt to make a correct diagnosis.

Carcinoma, Embryonal↗

Luteinizing hormone in peritoneal and ovarian cyst fluids: a predictor of ovarian carcinoma.

OBJECTIVE: The aim of this study was to define the role of luteinizing hormone (LH) as a tumor marker, specific for ovarian cancer. METHODS: The study included 34 women with functional and benign ovarian cysts, 11 women with borderline ovarian tumors, 22 patients with advanced ovarian cancer and 15 patients with non-ovarian intraperitoneal malignancies. Serum, peritoneal fluid and ovarian cyst aspirates were obtained intraoperatively (laparoscopy or laparotomy) and were subjected to the LH analysis. RESULTS: Peritoneal fluid LH levels were significantly increased in patients with ovarian cancer and those with borderline ovarian tumors as compared to patients with functional and benign ovarian cysts (P=0.005 and P=0.007, respectively). The patients with non-ovarian malignancies demonstrated the same peritoneal fluid LH levels as patients with benign ovarian tumors. There was no significant difference in the level of peritoneal fluid LH between ovarian cancer patients with and without ascites. The patients with functional and benign ovarian cysts demonstrated also significantly lower cyst fluid LH levels as compared to patients with malignant and borderline ovarian cysts (P=0.01 and P=0.03, respectively). Peritoneal and ovarian cyst fluid levels of LH were significantly increased in patients with fibrothecomas as compared to patients with other benign ovarian cysts. There were no significant differences in the serum LH levels comparing patients from all study groups. CONCLUSION: LH, detectable in peritoneal and ovarian cyst fluids, can be used as a tumor marker for identification of patients with borderline and malignant ovarian tumors.

Ascitic Fluid↗

Cisplatin (P), vinblastine (V) and bleomycin (B) combination chemotherapy in recurrent or advanced granulosa(-theca) cell tumours of the ovary. An EORTC Gynaecological Cancer Cooperative Group study.

The aim of this study was to investigate the clinical activity and toxicity of a modified PVB regimen (cisplatin, vinblastine and bleomycin) in patients with advanced or recurrent, pure granulosa cell tumours (GCTs) or mixed granulosa-theca cell tumours (GTCTs). The PVB regimen consisted of cisplatin (P) 20 mg/m2 intravenous (i.v.) days 1-5, vinblastine (V) 0.15 mg/kg i.v. days 1-2 and bleomycin (B) 30 mg i.v. on day 2, and 15 mg on day 15, for 28 days. 38 eligible patients were entered in this trial. Prior to PVB all patients underwent surgery and 13 received postoperative radio- or other prior chemotherapy. The median number of PVB cycles was 4 in both groups. In the group of 25 patients who had received prior surgery only, 7 and 6 patients had complete and partial responses, respectively (response rate: 52%, 95% confidence limits: 31.3-72.2%). At a median follow-up of 39 months, 6 patients were alive with no evidence of disease, 6 were alive with disease, 12 died due to malignant disease and 1 died due to intercurrent disease. The median time to progression was 13.9 months. The median survival was 25.4 months. 3-year survival was 49% (95% confidence limits: 29-69%). In the group of 13 patients who had previously received postoperative radio- or chemotherapy, 5 complete and 5 partial responses were observed on PVB (response rate: 77%, 95% confidence limit: 46.2-95.0%). At a median follow-up of 50 months, 6 patients were still alive, only 1 without evidence of disease, 6 died due to malignant disease and 1 died due to intercurrent disease. The median time to progression was 19.3 months. The median duration of survival was 41.1 months. Accompanying toxicity was distributed in a similar pattern for both groups. Severe toxicity was mainly documented as haematological toxicity, nausea/vomiting and alopecia. Furthermore cisplatin-related peripheral neurotoxicity and mild/moderate signs of bleomycin-related pulmonary toxicity were observed. The present data confirm the therapeutic activity of the PVB regimen in advanced/recurrent GCTs. The response rate was moderately high compared with previous studies, with a median duration of response of 20 months for both groups.

Adult↗

[Demons-Meigs syndrome: apropos of 1 case].

A 33-year-old patient presented with ascites and a right abdominal mass. Ultrasound and scannography showed it was probably a solid ovarian tumor measuring 175 mm x 115 mm. Serum Ca 125 was 752 UI/mL and ACE was normal. Surgical exploration revealed 3.2 liters ascites without any suspect peritoneal implant and a bulky ovarian fibrothecoma. The patient recovered quickly and serum Ca 125 was 43 UI/mL one month post-operatively. In this case report of Meigs' syndrome serum CA 125 level was particularly high. Preoperative diagnostic difficulties are discussed.

Adult↗

[Ovarian fibrothecoma: MR imaging findings and differential diagnosis].

We report the preoperative MR imaging of a 70 year old woman with a large tumor of the lower abdomen. MRI shows a extensively large mass with low signal in T2-weighted images. The origin of this tumor was suspected to be either the left ovary or the uterus. In the differential diagnosis a large subserous pedunculated leiomyoma or a low cellular, primarily benign ovarian tumor were considered.

Aged↗