A Prospective Study of Total Gastrectomy for CDH1-Positive Hereditary Diffuse Gastric Cancer

Background Hereditary diffuse gastric cancer (HDGC) is an autosomal dominant cancer syndrome. Up to 30% of families with HDGC have mutations in the E-cadherin gene, CDH1 . The role of prophylactic versus therapeutic gastrectomy for HDGC was studied prospectively. Methods Eighteen consecutive patient...

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Bibliographic Details
Published in:Annals of surgical oncology 2011-09, Vol.18 (9), p.2594-2598
Main Authors: Chen, Yijun, Kingham, Kerry, Ford, James M., Rosing, James, Van Dam, Jacques, Jeffrey, R. Brooke, Longacre, Teri A., Chun, Nicki, Kurian, Allison, Norton, Jeffrey A.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Cadherins - genetics
Carcinoma, Signet Ring Cell - genetics
Carcinoma, Signet Ring Cell - pathology
Carcinoma, Signet Ring Cell - surgery
Female
Follow-Up Studies
Gastrectomy
Gastrointestinal Oncology
Genetic Predisposition to Disease
Humans
Male
Medicine
Medicine & Public Health
Middle Aged
Mutation - genetics
Oncology
Pedigree
Prognosis
Prospective Studies
Stomach Neoplasms - genetics
Stomach Neoplasms - pathology
Stomach Neoplasms - surgery
Surgery
Surgical Oncology
Survival Rate
Young Adult
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Summary:Background Hereditary diffuse gastric cancer (HDGC) is an autosomal dominant cancer syndrome. Up to 30% of families with HDGC have mutations in the E-cadherin gene, CDH1 . The role of prophylactic versus therapeutic gastrectomy for HDGC was studied prospectively. Methods Eighteen consecutive patients with CDH1 mutations and positive family history were studied prospectively, including 13 without and 5 with symptoms. Proportions were compared by Fisher’s exact test, and survival by the Breslow modification of the Wilcoxon rank-sum test. Results Each patient underwent total gastrectomy (TG), and 17 (94%) were found to have signet ring cell adenocarcinoma. Twelve of 13 asymptomatic patients had T1, N0 cancer, and only 2/12 (16%) had it diagnosed preoperatively despite state-of-the-art screening methods. Each asymptomatic patient did well postoperatively, and no patient has recurred. For five symptomatic patients, each (100%) was found to have signet ring cell adenocarcinoma ( P  = 0.002 versus asymptomatic) by preoperative endoscopy; three (60%) had lymph node involvement and two (40%) had distant metastases at time of operation. Two-year survival was 100% for asymptomatic and 40% for symptomatic patients ( P  
ISSN:1068-9265
1534-4681
DOI:10.1245/s10434-011-1648-9