Chapter 12. Hereditary Ovarian and Endometrial Cancer

  1. Prof. Dr. Heike Allgayer PhD2,
  2. Prof. Dr. Helga Rehder3 and
  3. Prof. Dr. Simone Fulda4
  1. Marion Kiechle

Published Online: 21 AUG 2009

DOI: 10.1002/9783527627523.ch12

Hereditary Tumors: From Genes to Clinical Consequences

Hereditary Tumors: From Genes to Clinical Consequences

How to Cite

Kiechle, M. (2008) Hereditary Ovarian and Endometrial Cancer, in Hereditary Tumors: From Genes to Clinical Consequences (eds H. Allgayer, H. Rehder and S. Fulda), Wiley-VCH Verlag GmbH & Co. KGaA, Weinheim, Germany. doi: 10.1002/9783527627523.ch12

Editor Information

  1. 2

    University of Heidelberg and DKFZ (German Cancer Research Center) Heidelberg, Medical Faculty Mannheim, Chair of Experimental Surgery, Theodor-Kutzer-Ufer 1–3, 68167 Mannheim, Germany

  2. 3

    Medical University Vienna, Department of Medical Genetics, Währinger Strasse 10, 1090 Wien, Austria

  3. 4

    Ulm University Children's Hospital, Eythstrasse 24, 89075 Ulm, Germany

Author Information

  1. Technical University of Munich, Clinic for Gynecology and Obstetrics, Klinikum Rechts der Isar, Ismaningerstrasse 22, 81675 Munich, Germany

Publication History

  1. Published Online: 21 AUG 2009
  2. Published Print: 17 DEC 2008

ISBN Information

Print ISBN: 9783527320288

Online ISBN: 9783527627523



  • adenocarcinoma;
  • deleterious mutations;
  • genitourinary tract;
  • Lynch syndrome;
  • mismatch repair;
  • phenotype


Five percent of endometrial and ovarian carcinomas are based on a genetic predisposition. Most of the hereditary ovarian cancers (HOC) occur due to deleterious mutations in BRCA1 or BRCA2. Hereditary endometrial cancer (HEC) mainly occurs within the Lynch syndrome or hereditary non-polyposis colon cancer (HNPCC) and is based on mutations in the mismatch repair genes (MLH1, MSH2, MSH6). Besides the positive family history for further endometrial or colon carcinomas in the case of HEC, or further ovarian carcinomas and breast cancer cases in the case of HOC, the clinical feature is the relatively young age of onset, which is under the age of 50 for HEC, and under the age of 55 for HOC. The most effective prevention strategies are prophylactic surgical procedures.