title: Survival of patients with ovarian cancer due to a mismatch repair defect.
abstract: OBJECTIVE
  Hereditary non-polyposis colorectal cancer (HNPCC, Lynch syndrome) is characterized by the development of cancer of the colorectum, endometrium and other cancers. Cancer of the ovaries (OC) has frequently been reported in HNPCC. Colorectal cancer associated with HNPCC has a better survival chance compared to sporadic colorectal cancer. It is yet unknown whether patients with OC from HNPCC families (OC-HNPCC) also have a better survival. Therefore, the aim of the study was to compare the survival between patients with OC-HNPCC and a control group.
  
  
  METHODS
  A total of 26 patients with OC were identified from the Dutch HNPCC Registry. A control group (52 cases) matched for age, stage and year of diagnosis was derived from the population-based Eindhoven Cancer Registry. Data on treatment were collected for all patients. Kaplan-Meier analysis was used to calculate the crude survival.
  
  
  RESULTS
  The mean age at diagnosis of OC-HNPCC was significantly lower than the age of sporadic OC (49.5 vs 60.9 years). Compared to sporadic OC, OC-HNPCC was diagnosed at an earlier stage. The survival rate was not significantly different between patients with OC-HNPCC and the controls with sporadic OC. The cumulative 5-year-survival rates were 64.2 and 58.1% respectively.
  
  
  CONCLUSIONS
  On the basis of our findings, we recommend to treat OC-HNPCC similar to sporadic OC.
authors.affiliation: The Netherlands Foundation for the Detection of Hereditary Tumors, Leiden University Medical Center, Poortgebouw Zuid, 2333 AA, Leiden, The Netherlands.
authors.forename: Th E M
authors.initials: TE
authors.lastname: Crijnen
authors.forename: M L G
authors.initials: ML
authors.lastname: Janssen-Heijnen
authors.forename: H
authors.initials: H
authors.lastname: Gelderblom
authors.forename: J
authors.initials: J
authors.lastname: Morreau
authors.forename: M A
authors.initials: MA
authors.lastname: Nooij
authors.forename: G G
authors.initials: GG
authors.lastname: Kenter
authors.forename: H F A
authors.initials: HF
authors.lastname: Vasen
mesh_terms: D000328:Adult; D000368:Aged; D003123:Colorectal Neoplasms, Hereditary Nonpolyposis / Q000150:complications* / Q000235:genetics / Q000401:mortality*; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008875:Middle Aged; D051718:MutS Homolog 2 Protein / Q000235:genetics; D009154:Mutation; D010051:Ovarian Neoplasms / Q000235:genetics* / Q000401:mortality*; D016019:Survival Analysis
chemical_list: C497172:MSH2 protein, human; D051718:MutS Homolog 2 Protein