What are NCCN guidelines for evaluation and management hereditary diffuse gastric cancer (HDGC)?

Updated: May 21, 2019
  • Author: Elwyn C Cabebe, MD; Chief Editor: N Joseph Espat, MD, MS, FACS  more...
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Answer

HDGC is the most common genetic predisposing syndrome for gastric cancer, with germline truncating mutations of the E-cadherin gene (CDH1) detected in 30-50% of diffuse-type gastric cancers. Families that harbor these mutations have an autosomal dominant pattern of inheritance with a very high penetrance. NCCN guidelines recommendations for CDH1 mutation carriers include the following [4] :

  • The efficacy of endoscopic surveillance has not been established

  • Prophylactic gastrectomy (without a D2 lymph node dissection) between the ages of 18 and 40 for asymptomatic carriers with a family history of HDGC

  • Prophylactic gastrectomy is not recommended before age 18 except in carriers with family members diagnosed with gastric cancer before age 25

  • For individuals who decline prophylactic gastrectomy, upper endoscopy with multiple random biopsies should be offered every 6-12 months

  • Women with CDH1 mutations are at increased risk for breast cancer and should be followed similar to BRCA1/BRCA2 mutation carriers


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