Synchronous adult-type granulosa cell tumor of the ovary with ovarian fibroma: a case report

The coexistence of two different types of sex-cord stromal tumors, with various clinical symptoms, is extremely rare. We report a case of a 73-year-old woman showing coexistence of adult-type granulosa cell tumor in one ovary with ovarian fibroma in the other. Simultaneously, she was affected by Meigs' syndrome and simple endometrial hyperplasia without nuclear atypia. The different clinical symptoms of the disease according to the available literature are discussed.     

Meigs' syndrome with high blood levels of CA 125. Clinical case and review of the literatureø

A case of Meigs' syndrome is reported. The patient presented with: hydrothorax, ascites, benign ovarian tumor (thecoma) and increased serum levels of CA 125. Ovarian thecoma is a rare tumor, accounting for less than 1% of all ovarian tumors. When associated with Meigs' syndrome, it can produce a clinical picture similar to that of ovarian carcinoma, but the prognosis is much more favourable. The condition was managed by bilateral salpingo-oophorectomy and hysterectomy. The ascites and hydrothorax disappeared spontaneously after removal of the ovarian tumor and CA 125 level decreased.     

Ovarian sclerosing stromal tumor presenting as Meigs' syndrome with elevated CA-125

Meigs' syndrome caused by sclerosing stromal tumor is extremely rare and only two cases have been reported to date. An elevated serum level of CA-125 is also unusual and it has been thought that it is the consequence of physical irritation and inflammation. In this report, we present the case of a 50-year-old postmenopausal woman with a sclerosing stromal tumor presenting with Meigs' syndrome and an elevated CA-125 level (1476.8 IU/mL). This case highlights the difficulty in discerning the diagnosis of Meigs' syndrome from that of an ovarian malignancy and it should be considered in the differential diagnosis in postmenopausal patients with pelvic mass, ascites, pleural effusions and elevated serum CA-125.     

Ovarian fibroma associated with Demons-Meigs syndrome and elevated CA 125

We report a rare case of ovarian fibroma in a young woman. When ovarian masse is associated with Meigs' syndrome and elevated CA 125 serum level a malignant process may be suspected. But a negative cytological examination of ascitic effusion and absence of peritoneal implant recommend performing limited surgical procedures.     

Leiomyoma of the ovary presenting with Meigs' syndrome

Ovarian leiomyoma is a rare tumor. Ovarian leiomyoma accompanied with Meigs' syndrome is extremely rare. We report a case of ovarian leiomyoma presenting with Meigs' syndrome, which was first noted as dyspnea in a 79-year-old woman. A whole-body examination revealed a solid mass of the ovary, accumulation of ascites and pleural effusion, and elevated serum carbohydrate antigen 125. Abdominal hysterectomy and salpingo-oophorectomy were carried out. After the surgery, ascites and pleural effusion disappeared. The pathological diagnosis of the removed tumor was leiomyoma of the left ovary. The patient has been healthy for 24 months after the surgery, with no signs of recurrence. We also review the literature on this disease and the clinicopathologic characteristics of the current case are discussed.     

Elevated CA125 level associated with Meigs' syndrome: case report and review of the literature

Abstract.   Morán-Mendoza A, Luna GA, Ruiz GC, Olvera AS, López Graniel CM, Rincón DG. Elevated CA125 level associated with Meigs' syndrome: case report and review of the literature. Int J Gynecol Cancer 2006; 16(Suppl. 1): 315–318.
Meigs' syndrome is the association of ovarian fibroma, pleural effusion, and ascites. Meigs' syndrome with marked elevation of CA125 is an unusual clinical condition reported in 27 cases in the literature. The patient was a 46-year-old woman with right pleural effusion, ascites, ovarian tumor, and CA125 level of 1808 U/mL. Tomography revealed ascites and bilobate pelvic tumor of approximately 25 cm. The diagnosis of advanced epithelial ovarian cancer was considered, and the patient was treated with chemotherapy. Three chemotherapy schemes were applied due to the total lack of response in tumor volume; however, CA125 decreased to 90 U/mL. Thus, surgery was performed with resection of 25 cm of the left ovarian tumor, with intact capsule and without implants; the result of histopathologic analysis was fibroma. Postoperative CA125 was 11 U/mL. Patients with elevated CA125 and ascites cytology positive for malignancy must be cautiously treated due to the possibility of false positives, even if the probability is low. Therefore, minimally invasive surgery for biopsy collection must be considered. Although the association between ovarian tumor, pleural effusion, ascites, and marked elevation of CA125 is highly indicative of epithelial ovarian cancer, Meigs' syndrome must be considered in the differential diagnosis.     

Meigs' syndrome: the history of the eponym

Meigs' syndrome is defined as presence of ascites with hydrothorax in association with benign ovarian tumor. The syndrome is named after Joe Vincent Meigs (1892-1963), a Harvard Medical School Professor of Gynecology. However, it was described by several authors in the 19th and the beginning of 20th centuries. Meigs and Cass deserve the credit for awakening the medical profession to the importance of the syndrome in 1937. At the end, the syndrome was coined Meigs' syndrome by Rhodes and Terrell in 1937.     

Markedly elevated levels of vascular endothelial growth factor, fibroblast growth factor, and interleukin 6 in Meigs syndrome

Analysis of serum and peritoneal and pleural fluid from a patient with Meigs' syndrome revealed high levels of vascular endothelial growth factor, fibroblast growth factor, and interleukin 6. Serum levels declined after removal of the ovarian tumor, along with resolution of ascites and hydrothorax. These findings suggest the involvement of these vasoactive factors in ascites and pleural fluid formation in Meigs' syndrome.     

Ovarian epithelial tumors in Thai women: A histological analysis of 291 cases

A semiquantitative histological analysis on 291 epithelial tumors of the ovary in Thai women was done to assess the association between stromal invasion and a number of other histological features. Cribriform pattern, back-to-back growth, and epithelial stratification deeper than three cells were significantly related with stromal invasion in all types of ovarian epithelial tumors combined as well as in mucinous tumors alone. This finding suggests that the three histological characteristics serve as additional criteria of malignancy when unequivocal stromal invasion cannot be demonstrated. Histological classification of ovarian epithelial tumors in Thai women, together with age distribution, laterality, extraovarian spread, and some associated pathology, are presented and discussed.     

Signet-ring stromal tumor of the ovary: clinicopathologic analysis and comparison with Krukenberg tumor.

Signet-ring stromal tumor is a rare ovarian neoplasm that can mimic Krukenberg tumor because of the presence of signet-ring cells in both tumors. The clinicopathologic features of three signet-ring stromal tumors, one of which has been previously reported, were analyzed and compared with 10 Krukenberg tumors. Patients with signet-ring stromal tumor ranged in age from 34 to 41 years (mean: 36.7 years). All signet-ring stromal tumors were unilateral and stage IA, whereas 60% and 40% of Krukenberg tumors were bilateral or associated with extraovarian tumor, respectively. The signet-ring stromal tumors were devoid of epithelial differentiation (glands, nests, cords), whereas all of the Krukenberg tumors contained these epithelial structures at least focally. In contrast to signet-ring stromal tumors, the signet-ring cells of Krukenberg tumors were positive for periodic acid-Schiff with diastase and cytokeratins but negative for vimentin. The patients with signet-ring stromal tumors were alive without disease at follow-up interval of 1 month to 17.4 years (mean: 7.4 years). In summary, signet-ring stromal tumor is a rare, benign, ovarian tumor that may be mistaken for Krukenberg tumor. Although the combination of operative and histopathologic findings allow their distinction, histochemical and immunohistochemical stains may also be useful.     

Ovarian cancer: histogenetic classification, histologic grading, diagnosis, staging, and epidemiology

This paper presents an overview of the histogenic classification, histologic grading, diagnosis, staging, and epidemiology of ovarian cancer. The pathology of ovarian tumors is the most complex area of gynecologic pathology. The current staging system divides the ovarian cancers into major categories based on the 4 distinct cell populations that make up the ovary. Ovarian tumors have been further subclassified by enzyme production, cellular ultrastructure, genetic composition, and characterization of gene products. About 75-80% of ovarian tumors are of epithelial origin, 10% are stromal, 5% are germ cell, and the remainder fall under other categories. The histologic grade of a primary tumor is at least as important a determinant of a patient's clinical course as the stage at diagnosis. Low-grade epithelial ovarian cancers limited to the ovary with an intact capsule and no ascites are associated with a favorable prognosis, whereas poorly differentiated tumors are associated with a high likelihood of occult metastases and decreased survival. There is no effective screening method for the early detection of ovarian cancer, although there is the prospect that a tumor specific antigen can be identified to aid in early diagnosis. In the US, 18,000 new cases of ovarian carcinoma are diagnosed each year, with a fatality rate of 65%. The peak incidence occurs in the 8th decade of life, and the disease appears to be silghtly more common in blacks. Parous women have a 30-60% lower incidence of ovarian carcinoma than nulliparous women, and multiparas have a lower incidence than primiparas. There is some evidence that ovarian cancer is associated with a diet low in fiber and vitamin A. Oral contraceptive (OC) use seems to exert a protective effect, and the decreased risk correlated with increasing duration of OC use is long lasting.     

Vascular endothelial growth factor levels in pleural and peritoneal fluid in Meigs' syndrome

We report that we found differences in changes in vascular endothelial growth factor (VEGF) levels in pleural effusion and ascites after removal of ovarian tumor complicated by Meigs' syndrome. Postoperative VEGF levels decreased in the patient's pleural fluid but not in the peritoneal fluid. The mechanism of the development of the pleural effusion and ascites in Meigs' syndrome may differ.     

A rare case of low-grade endometrial stromal sarcoma with myxoid differentiation and atypical bizarre cells

Endometrial stromal sarcoma (ESS) is a rare mesenchymal tumor with characteristic histological appearances, consisting of diffuse infiltrate of small uniform endometrial stromal cells with a multinodular arrangement and distinct vascular pattern. Less common variants of ESS include "mixed endometrial stromal and smooth muscle tumors", "endometrial stromal tumors resembling ovarian sex cord tumors" and "endometrial stromal neoplasms with endometrial glands", and "aggressive endometriosis". Rarely do endometrial stromal tumors have a prominent fibrous or myxoid appearance which causes confusion and possible misdiagnosis as myxoid leiomyosarcoma. In this report we present a very unusual subtype of ESS in a 32-year-old woman. The tumor revealed atypical pleomorphic bizarre cells which were stained positive only with vimentin and CD10 in an abundant myxoid matrix. A low-proliferative rate was established with MIB-1 staining. To our knowledge such appearance has not been previously reported.     

Peutz-Jeghers syndrome with “adenoma malignum” of the cervix: A report of two cases

Two cases of the Peutz-Jeghers syndrome associated with cervical adenocarcinoma of the “adenoma malignum” type are reported with a review of four similar cases, three of which have been reported elsewhere. One of our two patients also had bilateral ovarian sex cord tumors with annular tubules and the other had bilateral ovarian mucinous cystadenomas of borderline malignancy. The relatively frequent occurrence of “adenoma malignum” of the cervix, an otherwise rare form of cervical cancer, in women with the Peutz-Jeghers syndrome, warrants close supervision of these patients by a gynecologist to assure early detection and treatment of this highly malignant tumor.     

Virilization in pregnancy due to a borderline mucinous ovarian tumor

Virilization in pregnancy due to borderline mucinous ovarian tumors is very rare. A case of a 28-year-old patient who was noted at 28 weeks' gestation to have marked virilization with raised serum androgens, ascites and a large complex right adnexal mass is presented. Delivery was carried out by cesarean section and at surgery a large tumor was noted in the right ovary. Histology revealed a borderline mucinous ovarian tumor with stromal luteinization, but there was no evidence of stromal invasion. Serum androgens returned to normal levels following surgery and the maternal virilization had resolved at the 6-week postnatal visit. Stromal changes in borderline mucinous ovarian tumors may result in virilization due to androgen production; surgical removal is associated with an excellent clinical outcome.     

The kinetics of leptin in Meigs' syndrome

OBJECTIVE: The aim of this study was to assess the kinetics and possible role of leptin in the pathophysiology of Meigs' syndrome. METHODS: We report on a 62-year-old patient admitted for a large ovarian tumor, hydrothorax, and ascites. The patient underwent abdominal hysterectomy and bilateral adnexectomy revealing a benign ovarian fibroma and no evidence of malignant cells in the pleural or peritoneal fluids. Analysis of serum, peritoneal, and pleural fluids from this patient was performed before, during, and after the operation. RESULTS: Preoperatively, the patient had low levels of leptin in the serum, peritoneal, and pleural fluids. Serum levels increased after removal of the ovarian tumor along with the resolution of ascites and hydrothorax. CONCLUSION: Leptin levels inversely correlate to tumor burden, third space fluid accumulation, and clinical status in Meigs' syndrome. These findings suggest the involvement of leptin in the pathophysiology of this syndrome.     

Meigs Syndrome Superimposed on Gorlin Syndrome in a 14-Year-Old Girl

BackgroundMeigs syndrome is a rare complication associated with ovarian fibromas. Although ovarian fibromas are rare in children, they are common in women with Gorlin syndrome after puberty.CaseA 14-year-old girl with Gorlin syndrome was admitted to our hospital for ablation of basal cell carcinoma. A chest x-ray revealed pleural effusion. Ultrasonography revealed bilateral multinodular ovarian masses. Meigs syndrome associated with ovarian fibromas was considered. A laparotomy revealed bilateral ovarian masses, which were resected. Microscopically, the masses were composed of mitotically active fibroma and areas resembling hemangiopericytoma and luteinized thecoma. The pleural effusion disappeared soon after the surgery.Summary and ConclusionPhysicians should consider the possibility that pleural effusion might precede the diagnosis of ovarian fibroma in patients with Gorlin syndrome.     

Benign mucinous cystadenoma with stromal luteinization responsible for maternal virilization and fetal intrauterine growth restriction

Virilization caused by ovarian tumors with functioning stroma during pregnancy is extremely rare and has been reported to be due to different types of ovarian tumors. In mucinous cystadenomas with maternal virilization during pregnancy, the stromal cells responsible for hormone secretion resemble lutein or Leydig cells and have been referred to as luteinized stromal cells. Here we present a rare case of mucinous cystadenoma accompanied by virilization, which was also the cause of fetal intrauterine growth restriction during pregnancy and discuss the mechanisms of hormone production in these tumors in the light of the literature.     

Inhibin alpha and beta expression in ovarian stromal tumors and their histological equivalences

Inhibin is a heterodimeric protein hormone that appears to be a sensitive immunohistochemical marker of sex cord-stromal tumors. Although sex cord-stromal tumors can usually be distinguished from ovarian epithelial tumors or their metastases by morphology or by using antibodies against intermediate filaments, the diagnosis remains difficult in rare situations in such cases as sarcomatoid granulosa-theca cell tumors, ovarian small cell carcinomas, or soft-tissue sarcomas. The purposes of this study were to examine inhibin alpha and beta immunoreactivity in a wide range of gonadal stromal neoplasms and to assess its value in the differential diagnosis of problematic tumors. A total of 108 paraffin-embedded ovarian and extraovarian tumors were examined immunohistochemically by using anti-alpha inhibin and anti-beta inhibin. Inhibin alpha immunostaining was identified in 46 (81%) of 57 gonadal stromal tumors, one (14%) of seven endometrial stromal tumors, and one (50%) of two primary ovarian carcinoid tumors. Inhibin beta immunostaining was detected in 55 (96%) of 57 gonadal stromal tumors, two (29%) of seven endometrial stromal tumors, one (50%) of two dysgerminomas, and in all of two (100%) primary ovarian carcinoid tumors. Inhibin alpha expression was not detected in any ovarian surface epithelial tumor cells. Some surface epithelial tumors showed stromal inhibin alpha (15% of cases) and inhibin beta (48% of cases) positivity. Weak immunoreactivity for inhibin beta was found in most (83% of cases) ovarian surface epithelial tumors. Two ovarian Burkitt lymphomas were negative for inhibin alpha and beta. Inhibin alpha is a sensitive immunohistochemical marker of gonadal stromal tumors and is of value in the differential diagnosis of ovarian neoplasia. Inhibin beta is a nonspecific marker for ovarian neoplasms, showing expression on tumor and stromal cells of different epithelial or stromal tumors.     

Primary ovarian carcinoid tumor with luteinized stromal cells

Background  Primary ovarian carcinoid tumors are rare. Distinct histologic patterns have been described in the literature as insular, trabecular, mucinous and mixed types. Case report  We describe a case of 71-year-old woman diagnosed with a left ovarian tumor. Frozen section examination identified the mass as a sex-cord stromal tumor. The surgically resected tumor was diagnosed as an insular carcinoid tumor with unusual luteinized stromal cells. Histologic diagnosis complied with results acquired by immunohistochemical with positivity of tumor cells for neuroendocrine markers and positivity of luteinized stromal cells for alpha-inhibin, calretinin and Melan A. Conclusion  Ovarian carcinoid tumor can be extremely heterogeneous. The purpose of our report was to show that the ovarian carcinoid tumor can be associated with stromal luteinization, mimicking a sex-cord stromal tumor.