Primary peritoneal cancer after bilateral salpingo-oophorectomy in two patients with Lynch syndrome

Obstet Gynecol. 2010 Feb;115(2 Pt 2):432-4. doi: 10.1097/AOG.0b013e3181b6f4f9.

Abstract

Background: Women with Lynch syndrome or hereditary nonpolyposis colorectal carcinoma (HNPCC) have a 40-60% lifetime risk of endometrial cancer and a 7-12% lifetime risk of ovarian cancer. Risk-reducing surgery, including hysterectomy and bilateral salpingo-oophorectomy (BSO), is currently recommended once child bearing is complete. We describe two cases of primary peritoneal cancer after BSO in women with Lynch syndrome or HNPCC.

Cases: The first patient was a 44-year-old woman who underwent hysterectomy with BSO for benign disease. She presented 12 years later with a pelvic mass and was diagnosed with a high-grade serous primary peritoneal cancer. Genetic testing showed a mutation in the MSH2 DNA mismatch repair gene. The second case was a 58-year-old woman who had a hysterectomy and BSO for endometrial cancer. She developed a high-grade serous primary peritoneal cancer 8 years later and was found to have a mutation in the PMS2 DNA mismatch repair gene.

Conclusion: Women with Lynch syndrome or HNPCC should be counseled that they may be at risk for developing primary peritoneal cancer despite undergoing gynecologic cancer risk-reducing surgery. The magnitude of this risk remains to be determined.

Publication types

  • Case Reports

MeSH terms

  • Adenosine Triphosphatases / genetics
  • Adult
  • Colorectal Neoplasms, Hereditary Nonpolyposis / genetics*
  • DNA Repair Enzymes / genetics
  • DNA-Binding Proteins / genetics
  • Female
  • Humans
  • Hysterectomy*
  • Middle Aged
  • Mismatch Repair Endonuclease PMS2
  • MutS Homolog 2 Protein / genetics
  • Mutation
  • Neoplasms, Second Primary / genetics
  • Neoplasms, Second Primary / prevention & control*
  • Ovariectomy*
  • Peritoneal Neoplasms / genetics
  • Peritoneal Neoplasms / prevention & control*

Substances

  • DNA-Binding Proteins
  • Adenosine Triphosphatases
  • PMS2 protein, human
  • MSH2 protein, human
  • Mismatch Repair Endonuclease PMS2
  • MutS Homolog 2 Protein
  • DNA Repair Enzymes