Question 4

A 54 yo woman with family history of colon cancer in her father at 45 years and brother at 43 years undergoes surgery for stage I colon cancer. She had genetic testing showing Lynch syndrome. She wants to know about management options to reduce extracolonic cancer. What is the best next step?

Answer Choices:

Hysterectomy and bilateral salpingectomy

Hysterectomy and bilateral salpingo-oophorectomy

Annual endometrial biopsy

Annual ultrasound

Oral contraceptives

Correct Answer:

Hysterectomy and bilateral salpingo-oophorectomy

Explanation:

  • Lynch syndrome (HNPCC) is a genetic predisposition to malignancies from germline mutations in DNA mismatch repair genes

  • 40-60% lifetime risk of colorectal cancer, but also associated with endometrial (up to 60%) and ovarian cancer (12%)

  • Surveillance with yearly endometrial samples can be considered between ages 30-50

  • Can also do yearly ultrasound and CA-125 but it is unknown if this truly reduces morbidity and mortality

  • At younger ages, oral contraceptives can be considered as they have been shown to reduce risk of both endometrial and ovarian cancers

  • Most effective method for prevention is risk-reducing hysterectomy and BSO

  • Lynch syndrome-associated endometrial cancer is more likely to happen in the lower uterine segment so total hyst with removal of cervix should be performed

  • There still remains a small risk of primary peritoneal cancer