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Prophylactic gastrectomy in a 16-year-old.

Tehara Wickremeratne1, Cheng Hiang Lee, Judy Kirk

  • 1aSydney Medical School bJames Fairfax Institute cPaediatrics and Child Health dWestmead Millennium Institute, University of Sydney Departments of eGastroenterology fPathology gSurgery, The Children's Hospital at Westmead hFamilial Cancer Service, Department of Medicine, Westmead Hospital, Sydney, New South Wales, Australia iDepartment of Molecular Medicine and Pathology, University of Auckland, Auckland, New Zealand.

European Journal of Gastroenterology & Hepatology
|November 19, 2013
PubMed
Summary
This summary is machine-generated.

Prophylactic total gastrectomy in a 16-year-old asymptomatic CDH1 mutation carrier revealed early-stage gastric cancer. This case supports individualized prophylactic surgery consideration for adolescents with CDH1 mutations.

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Area of Science:

  • Oncology
  • Genetics
  • Surgical Oncology

Background:

  • Hereditary diffuse gastric cancer is linked to CDH1 gene mutations.
  • Family history of early-onset gastric cancer necessitates genetic screening.
  • Early detection and intervention are critical for managing hereditary gastric cancer.

Observation:

  • A 16-year-old asymptomatic patient with a known CDH1 mutation underwent prophylactic total gastrectomy.
  • Prior gastroscopies were normal, highlighting limitations of endoscopic surveillance.
  • The patient had a strong family history of gastric cancer, with mother and aunt dying at young ages.

Findings:

  • Multiple foci of early-stage carcinoma were discovered in the gastrectomy specimen.
  • The patient is the youngest reported CDH1 carrier to undergo prophylactic gastrectomy.
  • The findings underscore the aggressive nature and unpredictable onset of CDH1-associated gastric cancer.

Implications:

  • Prophylactic gastrectomy should be considered for adolescent CDH1 mutation carriers on an individual basis.
  • Current guidelines may not adequately address the risk in young, asymptomatic carriers.
  • This case highlights the need for personalized management strategies in hereditary cancer syndromes.