- The European Society of Medical Oncology (ESMO) has issued a new clinical practice guideline for prevention, diagnosis, treatment, and follow-up of at least 4 hereditary gastrointestinal (GI) cancers.
Why this matters
- Genetic testing and prophylactic surgery can prevent several hereditary GI cancers.
- Literature review with specific recommendations, using level of evidence by the ESMO Guidelines Committee.
- Funding: None.
Hereditary nonpolyposis colorectal cancer syndrome (Lynch syndrome):
- Individuals with colorectal cancer (CRC) should have tumor testing for mismatch repair proteins and/or microsatellite instability.
- Colonoscopy in mutation carriers should be undertaken every 1-2 years.
- Prophylactic hysterectomy and/or bilateral salpingo-oophorectomy should be considered in female carriers who have completed childbearing.
Hereditary polyposis colorectal cancer syndromes:
- The presence of multiple colorectal adenomas (>10) should trigger germline genetic testing.
- Families with classic familial adenomatous polyposis (FAP) should have sigmoidoscopy every 1-2 years, beginning at 12-15 years.
Hereditary diffuse gastric cancer:
- Families meeting clinical criteria should be tested for CDH1.
- Prophylactic gastrectomy in carriers is recommended, as is annual breast MRI in females starting at age 30 years.
Hereditary pancreatic cancer:
- Patients with pancreatic cancer should have a multigene cancer panel.
- Surveillance is recommended for those with a strong family history or with a PC susceptibility mutation and a close family history.