Prophylactic Gastrectomy in Familial Gastric Cancer: Case Report and Review of Literature

• Main Authors: Nassim Sodagari, Reza Malekzadeh, Zhamak Khorgami, Shirzad Nasiri, Anushiravan Hedayat
• Format: Article
• Language: English
• Published: Iran University of Medical Sciences 2010-11-01
• Series: Medical Journal of The Islamic Republic of Iran
• Subjects: CDH1 mutations; Hereditary Diffuse Gastric Cancer; Prophylactic Total Gastrectomy
• Online Access: http://mjiri.tums.ac.ir/browse.php?a_code=A-10-1-122&slc_lang=en&sid=1
• ISSN: 1016-1430; 2251-6840

Gastric cancer is the fourth most common cancer and the second leading cause of cancer death.Most cases are sporadic and only 10% of patients,show familial clustering.Among these patients, 1 to 3 % have hereditary diffuse gastric cancer(HDGC),which is autosomal-dominant and present in younger ages.Mutations in Ecadherin gene CDH1 has been identified in 30 to 50% of patients.Because endoscpoic surveillance is not effective in identifying early HDGC, gene-directed prophylactic gasterectomy is recommended for CDH1 muta-tion carriers.But in most carriers who underwent prophylactic ga-strectomy, histological examinations on the resected stomach revealed foci of diffuse gastric cancer. It shows that gasterectomy   could be curative instead of prophylactic in these cases. Individuals from families with HDGC should be recommended to genetic testing and in patients that have a CDH1 gene mutation, total gasterectomy is in-dicated.We are presenting a 27 year old patient with striking history of familial gastric cancer and positive E-cadherin gene mutation who underwent prophylactic gasterectomy.