Piver M S, Baker T R, Jishi M F, Sandecki A M, Tsukada Y, Natarajan N, Mettlin C J, Blake C A
Gilda Radner Familial Ovarian Cancer Registry, Roswell Park Cancer Institute, Buffalo, New York 14263.
Cancer. 1993 Jan 15;71(2 Suppl):582-8. doi: 10.1002/cncr.2820710214.
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.
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.
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.
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.
由于1929年至1969年期间报告的病例数较少（5例），且在20世纪70年代这十年间报告的病例数显著增加，因此于1981年设立了家族性卵巢癌登记处，以研究美国家族性卵巢癌的发病情况。
任何联系登记处并证明有卵巢癌家族史的女性（无论其本人是否患有卵巢癌）均作为索引病例被纳入登记处。
从1981年至1991年5月31日，共有658个家庭的1568例家族性卵巢癌病例被纳入登记处。在患有家族性卵巢癌的219名母亲和251名女儿中，母亲确诊时的平均年龄（58.5岁）和中位年龄（57.0岁）显著高于患卵巢癌女儿的平均年龄（49.8岁）和中位年龄（49.0岁）。与患有卵巢癌的索引病例相比，显著更多没有卵巢癌的索引病例使用过口服避孕药（P < 0.001）。与没有卵巢癌的索引病例相比，显著更多患有卵巢癌的索引病例使用过其他雌激素（P < 0.001）。与1978年监测、流行病学和最终结果（SEER）数据相比，登记处的病例中浆液性腺癌、低分化腺癌、交界性癌和性腺母细胞瘤的比例更高。母亲和至少一个女儿是最常见的关系，在患有家族性卵巢癌的家庭中占49.5%。姐妹关系是第二常见的，在658个家庭中占38.5%。
家族性卵巢癌最常发生于母女关系，其次是姐妹关系，似乎是一种具有可变外显率的常染色体显性遗传。
