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Background. The purpose of this study was to review patients with ovarian cancer in pregnancy, the effectiveness of the available methods of treatment and their prognosis. Methods. A retrospective review of all women diagnosed to have cancer of the ovary associated with pregnancy who delivered at the authors' hospitals between January 1976 and December 2000. The demography, clinical presentation, time and mode of diagnosis, treatment, pregnancy outcome and maternal survival were noted. Results. The incidence of ovarian carcinoma in pregnancy in the series was 0.08/1000 deliveries. Of the 9 patients, 7 had epithelial cancers; 4 serous cystadenocarcinoma, 2 mucinous cystadenocarcinomas and one undifferentiated cancer. One patient each had dysgerminoma and granulosa cell tumor. Six patients were in FIGO stage Ia, one Ic, one IIa. One patient was in stage III. Five patients were treated by unilateral salpingo-oophorectomy during pregnancy. Three patients had total abdominal hysterectomy, bilateral salpingo-oophorectomy and omentectomy followed by chemotherapy. Debulking of the tumor was done in a patient in stage III with subsequent chemotherapy. This patient died 13 months from the time of diagnosis of the tumor. The overall 5-year survival rate in the series was 78% and 100% for stage Ia. Conclusions. Association of ovarian cancer with pregnancy is a rare occurrence. Early diagnosis and appropriate treatment offers the best prognosis for the patient. The higher survival rates in the series was attributed to a larger number of patients in stage I of the disease and 2 patients with a germ cell tumor and dysgerminoma which have the best prognosis. Aggressive postoperative chemotherapy also contributed to the better outcome.

OBJECTIVES To determine the incidence of ovarian malignant tumors in childhood and adolescence, to ascertain the frequency distribution of the various tumor types and to assess time trends in Israel on a nationwide basis. METHODS The study group included all Israeli Jewish patients < or = 19 years old with histologically confirmed ovarian malignancies, diagnosed in Israel from 1970 to 1994. Data were obtained from the Israel Cancer Registry. The effects of age at diagnosis and period of diagnosis were analyzed using the Poisson regression. RESULTS Among the 82 patients identified, the most frequent tumors (72.0%) were of germ cell origin and among those about one third were dysgerminomas. Epithelial tumors were diagnosed in 26.6% of the patients and most of these were borderline malignancies. The incidence rate (IR) for the total group of ovarian malignancies in the 0-19 age group was 0.52 and for ages 5-19 it was 0.71 per 100,000. After adjustment for age, a significant linear trend for a decrease of germ cell tumors over time was found, stemming from a decrease of dysgerminomas. A significant trend for increase in the IRs with age was also found. In addition, a steep rise in the age specific IRs of epithelial borderline tumors was noted in the last 5 year period. CONCLUSIONS The IRs of ovarian malignancies in childhood and adolescence in Israel, as in other countries, is very low as compared to adults and the most common tumors are of germ cell origin while malignant epithelial tumors are very rare. A time period effect in the germ cell tumors that resulted from an inexplicable significant decrease in the age specific IRs of dysgerminomas, was observed. A significant increase in borderline tumors was also noted and may be attributed to greater awareness of pathologists to this entity.

To determine the most important prognostic factors in patients with malignant germ cell tumors of the ovary.Age, histologic type, tumor size, stage, treatment mode, type of surgery of 67 patients with malignant germ cell tumors managed consecutively were evaluated retrospectively using multivariate analysis.The overall survival rate was 60.6%. This figure was 78.5% for dysgerminoma, 53.3% for immature teratoma, 12.0% for endodermal sinus tumor, 33.3% for mixed germ cell tumor and 100.0% for others. The patients with endodermal sinus tumor had significantly poorer survival than other groups. Among the factors analyzed for survival histologic type was found to be the most significant predictor for survival.Finding of histologic type as the strongest independent risk factor may be evaluated in correlation with the literature that these tumors have historically been investigated as dysgerminomatous and nondysgerminomatous tumors. Furthermore, endodermal sinus tumors had long been known for poor survival. Based on this data, despite advent of staging and effective chemotherapy, prognosis still is basically related to histologic type and histology of tumor should be taken into account while comparing results.

A case is reported, of a 25-year-old phenotypic female, karyotypic male patient with metastatic gonadal dysgerminoma-seminoma and severe hypercalcemic paraneoplastic syndrome. Patient manifested normal female external genitalia, vagina, uterus, and fallopian tubes but with 46 XY karyotype. Adnexal gonads were calcified. External beam radiation therapy resolved the hypercalcemic state. Exploratory laparotomy after irradiation revealed gonado-blastoma originating from dysgenetic gonads.

Specific estradiol (ER) and progesterone receptors (PR) in the tumor cytosol of 54 patients with primary ovarian cancer were examined. Also 30 benign ovarian neoplasms and 19 normal ovaries were analysed. Of the malignant tumors, 46% contained ER and 41% PR. The ER content of most malignant tumors was 3-30 fmoles/mg protein; three tumors revealed an ER content over 30 fmoles/mg protein. The PR content of most malignant tumors was 8-80 fmoles/mg protein. Three endometrioid adenocarcinomas and three serous cystadenocarcinomas had a PR level over 100 fmoles/mg protein. ER and PR were simultaneously detected in 26% of the malignancies, whereas 39% of them were receptor negative. No significant differences were found between premenopausal and postmenopausal women, as regards the presence of steroid receptors. Carcinomas were significantly more often ER-positive than were benign tumors. The ER content of both the benign neoplasms and the normal ovary was low, less than or equal to 15 fmoles/mg protein, whereas higher PR levels were found in these two groups. The presence of steroid receptors in malignant ovarian tumors can possibly be used as an indicator of hormone dependency of these malignancies.

Gonadal tumors in patients with somatosexual ambiguity are rare but of considerable clinical and pathological interest. Two patients with dysgerminomas and abnormal sexual differentiation were recently treated at the Department of Gynecologic Oncology. One (Case 1) was an 18-year old girl and the other (Case 2) an unmarried woman of 58 years. In spite of their female phenotype both showed a male genotype, 46, XY. In Case 1 the dysgerminoma (or rather seminoma) originated in a gonadoblastoma in one of two dysgenetic testes but in Case 2 in one of two "feminizing testes". The clinico-pathological features of both cases will be reported and commented and the relation between the germ cell tumors, gonadal dysgenesis and the Y-chromosome will be discussed.

One of 7 patients with pure dysgerminoma had slightly elevated serum human chorionic gonadotropin (HCG) and alpha-fetoprotein (AFP) levels as determined by radio-immunoassay before operation. The values returned to the normal range after the removal of the tumor. The cells that produced the HCG or AFP were identified by immunohistochemical staining of the tumor tissues. HCG proved positive both in the multinucleated giant cells with eosinophilic cytoplasm and in the spindle-shaped cells with a hyperchromatic nucleus. AFP was positively stained in the round cells. These results suggest that HCG and AFP are produced by the cells which constitute pure dysgerminoma.