Piver M S, Goldberg J M, Tsukada Y, Mettlin C J, Jishi M F, Natarajan N
Gilda Radner Familial Ovarian Cancer Registry, Roswell Park Cancer Institute, Buffalo, USA.
Eur J Gynaecol Oncol. 1996;17(3):169-76.
To determine the demographics of the first 1,000 families in the Gilda Radner Familial Ovarian Cancer Registry.
Any woman with a family history of two or more first or second degree relatives with ovarian cancer who was referred to the Registry was entered into a database. The demographics of the Registry population were analyzed, including total number of ovarian cancer cases, cases per family, age at diagnosis, prevalence of other malignancies, and histology. Comparisons were made to expected norms in the general population using the most recent surveillance, Epidemiology, and End Results data from the National Cancer Institute.
There were 2,425 reported cases of ovarian cancer among the 1,000 registered families. The number of cases per family ranged from 2-10 with 72.4% of the families having two cases of ovarian cancer and 27.6% with three or more cases of ovarian cancer. The mean age at diagnosis was 53.5 years, significantly younger than the 60.8 years reported in the general population (p < 0.1). The mean age at diagnosis was even younger, 52.1 years, in the subset of patients with a family history of three or more cases of ovarian cancer. Moreover, in the mother/daughter pairs of ovarian cancer where age of onset of ovarian cancer was known, the mean age of the mothers was 58.8 compared to a mean age of 50.2 among the daughters (p < 0.01). There was a significantly larger proportion of serous adenocarcinoma (40%) and a significantly smaller proportion of mucinous adenocarcinoma (3%) and stromal cell tumor (0%) when compared to the general population (p < 0.01). Breast and colon cancer were the two most common other cancers reported.
given the limits of the study due to the voluntary nature of the Registry, the data suggests that familial ovarian cancer occurs at a younger age than the general population, daughters of mothers with ovarian cancers develop ovarian cancer at an age significantly younger than their mothers, mucinous and stromal tumors occurred significantly less often than in the general population, and the most common associated other cancers are female breast cancer and female and male colon cancer.
确定吉尔达·拉德纳家族性卵巢癌登记处首批1000个家庭的人口统计学特征。
任何有两名或更多一级或二级亲属患卵巢癌家族史且被转诊至该登记处的女性均被录入数据库。分析登记处人群的人口统计学特征，包括卵巢癌病例总数、每个家庭的病例数、诊断时的年龄、其他恶性肿瘤的患病率以及组织学类型。使用美国国立癌症研究所最新的监测、流行病学和最终结果数据，与一般人群的预期标准进行比较。
在1000个登记家庭中，共报告了2425例卵巢癌病例。每个家庭的病例数在2至10例之间，72.4%的家庭有两例卵巢癌，27.6%的家庭有三例或更多例卵巢癌。诊断时的平均年龄为53.5岁，显著低于一般人群报告的60.8岁（p<0.1）。在有三例或更多例卵巢癌家族史的患者亚组中，诊断时的平均年龄更小，为52.1岁。此外，在已知卵巢癌发病年龄的母女卵巢癌对中，母亲的平均年龄为58.8岁，而女儿的平均年龄为50.2岁（p<0.01）。与一般人群相比，浆液性腺癌的比例显著更高（40%），黏液性腺癌（3%）和间质细胞瘤（0%）的比例显著更低（p<0.01）。乳腺癌和结肠癌是报告的两种最常见的其他癌症。
鉴于该登记处的自愿性质导致研究存在局限性，数据表明家族性卵巢癌的发病年龄低于一般人群，卵巢癌母亲的女儿患卵巢癌的年龄显著低于其母亲，黏液性和间质瘤的发生频率显著低于一般人群，最常见的相关其他癌症是女性乳腺癌以及女性和男性结肠癌。
