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Kirsten N. Kangelaris, MD; Stephen B. Gruber, MD, PhD, MPH

JAMA. 2007;297:2410-2411. Published online June 3, 2007 (doi:10.1001/jama.297.21.2410).

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

Hereditary diffuse gastric cancer (HDGC) is an autosomal-dominant cancer susceptibility syndrome characterized by early onset diffuse gastric cancer and lobular breast cancer.¹ The clinical presentation of this particular form of familial gastric cancer was described more than 40 years ago² and the phenotype can be dramatic. One descendant of the original Maori kindred from New Zealand died of gastric cancer at the age of 14 years; more than 25 of his relatives have succumbed to cancer.³ In a Michigan family with HDGC, 1 family member died from gastric cancer at the age of 17 years, and numerous other family members died of metastatic disease before reaching age 50 years.⁴ Fortunately, genetic testing has already transformed the care of families with HDGC, and the tragic outcomes in these 2 representative families can now largely be avoided. Prophylactic gastrectomy has the potential . . . [Full Text of this Article]

Author Affiliations: Departments of Internal Medicine (Drs Kangelaris and Gruber), Epidemiology (Dr Gruber), and Human Genetics (Dr Gruber), University of Michigan, Ann Arbor.

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ABSTRACT | FULL TEXT  

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