de Meeus J B, Bennouna C, Maréchaud M, Plocoste V, Magnin G
Service de Gynécologie-Obstétrique, Hôpital Jean-Bernard, Poitiers.
J Gynecol Obstet Biol Reprod (Paris). 1993;22(7):749-56.
Greggi and Kerlikowske have worked out that a woman has a 1.4% chance of developing ovarian cancer during her life. When cancer of the ovary is found, 5 to 10% of these cases have a familial form of this pathology. Thus there are some hereditary forms of cancer of the ovary and Lynch has demonstrated that there are three types of hereditary associations with ovarian neoplastic pathology: specific familial cancer of the ovary, cancer of the ovary associated with endometrial cancer and with non-polypoidal cancer of the caecum and rectum, cancer of the ovary associated with cancer of the breast. The clinical material we are presenting here is of the first type of association and we are reporting the study of a family in which 6 members in two generations had cancer of the ovary and of whom one had cancer of the breast as well. Familial cancer of the ovary shows different characteristics coming on as it does earlier (ten years earlier) and with a shorter length of survival (1.8 as against 5 years). The risk of the next generation having ovarian neoplastic pathology is clear because there is a 50% chance in a patient who has a history of cancer of the ovary in at least two first degree relatives. For most daughters when this type of familial cancer is found it is justifiable to carry out prophylactic oophorectomy from the age of 35 year onwards. Particular supervision should be carried out for patients who are members of a family where 2 index cases have been found within 20-35 years.(ABSTRACT TRUNCATED AT 250 WORDS)
格雷吉和凯尔利科夫斯基经研究发现,女性一生中患卵巢癌的几率为1.4%。在确诊的卵巢癌病例中,5%至10%为家族性病例。因此,卵巢癌存在一些遗传形式,林奇证实卵巢肿瘤病理存在三种遗传关联类型:特定的家族性卵巢癌、与子宫内膜癌以及盲肠和直肠癌的非息肉性癌相关的卵巢癌、与乳腺癌相关的卵巢癌。我们在此呈现的临床资料属于第一种关联类型,我们报告了一个家族的研究情况,该家族两代人中6名成员患有卵巢癌,其中1人还患有乳腺癌。家族性卵巢癌表现出不同特征,发病时间更早(早十年),生存期更短(1.8年相对5年)。下一代患卵巢肿瘤病理的风险很明显,因为在至少有两名一级亲属患卵巢癌病史的患者中,风险为50%。对于大多数发现这种家族性癌症的女儿来说,从35岁起进行预防性卵巢切除术是合理的。对于在20至35年内发现两例索引病例的家族中的患者,应进行特别监测。(摘要截短于250字)