What is the management approach for Hereditary Nonpolyposis Colorectal Cancer (HNPCC) colorectal cancer?

From the Guidelines

Patients with Hereditary Nonpolyposis Colorectal Cancer (HNPCC) should undergo colonoscopy every 1-2 years beginning at age 20-25 or 2-5 years before the earliest colorectal cancer diagnosis in the family, whichever comes first, as recommended by the most recent guidelines 1. The management of HNPCC requires intensive surveillance and prophylactic interventions to reduce the risk of colorectal cancer and other associated cancers.

• Key aspects of HNPCC management include:
  • Colonoscopy every 1-2 years starting at age 20-25 or 2-5 years before the earliest colorectal cancer diagnosis in the family
  • Annual endometrial sampling and transvaginal ultrasound for women starting at age 30-35 due to increased risk of endometrial and ovarian cancers
  • Consideration of prophylactic colectomy for patients with multiple adenomas, severe dysplasia, or those who cannot comply with surveillance protocols
  • Extended colectomy (subtotal colectomy with ileorectal anastomosis) rather than segmental resection is preferred when surgery is needed for cancer treatment, as the risk of metachronous colorectal cancer is high (16-19% at 10 years after segmental resection) 1
• Family members should undergo genetic testing, and those who test positive require the same surveillance protocol, as the risk of colorectal cancer is higher in HNPCC patients (up to 80%) 1
• The use of aspirin at doses of 600 mg daily may reduce colorectal cancer risk in HNPCC patients, though this is not yet a standard recommendation 1
• Prophylactic hysterectomy and bilateral salpingo-oophorectomy should be discussed with female patients who have completed childbearing, as an alternative option to reduce the risk of endometrial and ovarian cancers 1

From the Research

Management Approach for HNPCC Colorectal Cancer

The management of Hereditary Nonpolyposis Colorectal Cancer (HNPCC) involves a combination of endoscopic surveillance, surgical intervention, and genetic testing. Key aspects of the management approach include:

• Endoscopic surveillance: Colonoscopic surveillance is recommended every 1 to 2 years beginning at age 20 to 25, or 10 years younger than the earliest CRC in the family (whichever is earlier) 2, 3, 4.
• Surgical management: Total abdominal colectomy is considered for HNPCC patients with colon cancer due to the increased risk of metachronous neoplasia associated with the condition 3, 4.
• Genetic testing: Genetic testing can aid in the identification of high-risk individuals and optimize the cost-effectiveness of the management approach 2, 5.

Surveillance and Screening Guidelines

Surveillance and screening guidelines for HNPCC include:

• Colonoscopy every 1 to 2 years starting at age 20 to 25 2, 3, 4.
• Annual endoscopic examination of the retained rectum after surgical resection of an HNPCC-associated cancer or adenoma 3.
• Screening of individuals at risk every 1-2 years by colonoscopy starting around the age of 25 years 3.

Chemoprevention

Chemoprevention may also play a role in the management of HNPCC, with:

• Aspirin shown to be effective in preventing colorectal neoplasia in prospective trials and considered for patients without contraindications 4.
• Ongoing trials for other chemopreventative agents in HNPCC 4.

Family Communication and Genetic Counseling

Family communication and genetic counseling are important aspects of HNPCC management, with:

• Genetic counseling and testing influencing the use of colonic endoscopy and adherence to recommendations for colon cancer screening 5.
• Family members who are persuaded to seek genetic counseling and testing by the proband more likely to have counseling and testing and seek those services sooner 6.