Hereditary Diffuse Gastric Cancer (HDGC) - Endoscopy (4 of 4)

Some ulcerated nodules are appreciated at the antrum. Those cases presented here of that member of this family has the Hereditary diffuse gastric (stomach) cancer (HDGC) is a genetic cancer susceptibility syndrome characterized by a high risk for stomach and lobular breast cancer. HDGC is inherited in an autosomal dominant pattern, therefore several generations of relatives with stomach or lobular breast cancer are often seen clustering on one side of the family. Gastric cancers that occur in this syndrome are of the “diffuse” type (as opposed to “intestinal”) and often have “signet ring” cells through the stomach wall causing thickening (“linitis plastica”) without forming a discrete mass. The average age of onset of gastric cancer in HDGC is 38 years old, with individuals as young as 14 having been diagnosed. The estimated lifetime risk of developing gastric cancer by age 80 is 67% for men and 83% for women. Women with HDGC also have an elevated risk of breast cancer, predominantly of the lobular type, with a 20 – 40% lifetime risk. Most of these women are over 50 at diagnosis.