Author/Authors :
Nasiri, Shirzad Department of General Surgery - Tehran University of Medical Sciences, Tehran, Iran , Hedayat, Anushiravan Department of General Surgery - Tehran University of Medical Sciences, Tehran, Iran , malekzadeh, Reza Department of General Surgery - Tehran University of Medical Sciences, Tehran, Iran , Khorgami, Zhamak Department of General Surgery - Tehran University of Medical Sciences, Tehran, Iran , sodagari, Nassim Department of General Surgery - Tehran University of Medical Sciences, Tehran, Iran
Abstract :
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.