Lynch Syndrome by Unknown

Author:Unknown
Language: eng
Format: epub
ISBN: 9789811568916
Publisher: Springer Singapore

6.3 Ovarian Cancer

6.3.1 Characteristics

The overall lifetime risk of ovarian cancer is 1.3% in the general population, but women with Lynch syndrome have a much higher lifetime risk of 4–12%. The mean age at diagnosis is 42.5 years, and about 30% of patients with Lynch syndrome-related ovarian cancer are diagnosed at age <30 years, which is younger than for patients with sporadic ovarian cancer. The risks for ovarian cancer are 20% and 24% in women with MLH1 and MSH2 mutations, respectively [21]. Regarding the histological type, almost all cases (94%) are epithelial ovarian cancer, including various tissues similar to nonhereditary ovarian cancer and at a relatively early stage. The rate of ovarian borderline tumor is only 4%, which indicates that this tumor is rarely found in Lynch syndrome [22]. Ovarian cancer is sometimes found as a double cancer with endometrial cancer; however, it is rare that patients with double cancer have Lynch syndrome and many cases of double cancer are sporadic [10]. Many patients with ovarian cancer have gene profiles of germline and somatic mutations of HRD-related genes, including BRCA1 and BRCA2, and only 3% have MMR gene mutations; therefore, few patients with ovarian cancer have Lynch syndrome [23, 24].

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