Background. According to previous reports, primary peritoneal carcinoma indistinguishable from primary ovarian adenocarcinoma had developed in five women with a history of familial ovarian cancer who had undergone prophylactic oophorectomy. Methods. The records from the Gilda Radner Familial Ovarian Cancer Registry were reviewed for instances of prophylactic oophorectomy and cases of primary peritoneal carcinoma occurring after prophylactic oophorectomy. Results. From 1981 through July 1992, the Gilda Radner Familial Ovarian Cancer Registry accessioned 931 families (a total of 2221 cases of familial ovarian cancer). Currently, 324 women in these families have undergone prophylactic oophorectomy as a preventive measure against the subsequent development of ovarian cancer. Primary peritoneal carcinoma indistinguishable histologically from primary ovarian adenocarcinoma has developed in six of these women 1‐27 years after prophylactic oophorectomy. Conclusions. Based on this finding and other reports of such primary peritoneal carcinoma, a prospective international study is planned. This study will compare the incidence of peritoneal carcinoma in first‐ or second‐degree relatives who underwent prophylactic oophorectomy because of a family history of ovarian cancer with that of those who did not undergo prophylactic oophorectomy.
Background. Because of the small number of cases (five) reported between 1929 and 1969 and a significant increase reported in the decade of the 1970s, the Familial Ovarian Cancer Registry was established in 1981 to study the occurrence of familial ovarian cancer in the United States. Methods. Any woman (with or without ovarian cancer herself) who contacted the Registry and demonstrated a familial history of ovarian cancer was added to the Registry as an index case. Results. From 1981 through May 31, 1991,658 families for a total of 1568 cases of familial ovarian cancer were accessioned into the Registry. Of the 219 mothers and 251 daughters with familial ovarian cancer, the mean (58.5 years) and median (57.0 years) age at diagnosis of the mothers was significantly older than the mean (49.8 years) and median (49.0 years) ages of their daughters with ovarian cancer. Significantly more index cases without ovarian cancer had used oral contraceptives as compared to index cases with ovarian cancer (P < 0.001). Significantly more index cases with ovarian cancer used other estrogens as compared to index cases without ovarian cancer (P < 0.001). The Registry cases exhibited a higher proportion of serous adenocarcinoma, poorly differentiated adenocarcinoma, borderline carcinoma, and gonadoblastoma as compared to the 1978 SEER data. Mother and a minimum of one daughter was the most common relationship and was represented in 49.5% of the families with familial ovarian cancer. Sister–sister relationships were the second most frequent and accounted for 38.5% of the 658 families. Conclusions. Familial ovarian cancer occurs most frequently in mother–daughter relationships followed by sister–sister and appears to be an autosomal dominant inheritance with variable penetrance.
Background. Increasing scientific and public interest in hereditary cancer syndromes has created a need for estimates of lifetime cancer risks among members of families with such syndromes. Methods. Data from the Gilda Radner Familial Ovarian Cancer Registry were used to evaluate risk for cancers of the breast, cervix, uterus, colorectum, and prostate in members of 143 families containing three or more reported cases of ovarian cancer among first‐ or second‐degree relatives. These risks were compared with those that were expected based on general population rates obtained from the Connecticut Tumor Registry. Results. Overall, family members' risk of cancer at any nonovarian site was 1.5 times that of the general population (P < 0.001). Among female members, risk for cancer of the breast was 2.5 times that of the general population. Risk for cancer of the uterus was 5 times that of the general population and increased with increasing number of first‐degree relatives with ovarian cancer. Among male family members having three or more first‐degree relatives with ovarian cancer, prostate cancer risk was 4.5 times that of the general population. No excess risks were observed for cancer of the colorectum. Conclusions. These data support previous reports of coaggregation of cancer of the breast, uterus and ovary, and suggest coaggregation between cancer of the ovary and prostate. Differences in cancer risk profiles observed in these families with multiple ovarian cancer and in carriers of the gene BRCA1 suggest that hereditary ovarian cancer is genetically heterogeneous.
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