ESMO issues clinical guidelines for hereditary gastrointestinal cancers

  • Stjepanovic N & al.
  • Ann Oncol
  • 5 Aug 2019

  • curated by Miriam Davis, PhD
  • Univadis Clinical Summaries
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  • 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.

Study design

  • Literature review with specific recommendations, using level of evidence by the ESMO Guidelines Committee.
  • Funding: None.

Key recommendations

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.

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