Hereditary Diffuse Gastric Cancer: Prophylactic Surgical Oncology Implications

Hereditary Diffuse Gastric Cancer: Prophylactic Surgical Oncology Implications

Henry T. Lynch,Edibaldo Silva,Debrah Wirtzfeld,Pamela Hebbard,Jane Lynch,David G. Huntsman;Henry T. Lynch;Edibaldo Silva;Debrah Wirtzfeld;Pamela Hebbard;Jane Lynch;David G. Huntsman;
surgical clinics of north america 2008 Vol. 88 pp. 759-778
148
huntsman2008surgicalhereditary

Abstract

Hereditary diffuse gastric cancer (HDGC) is an autosomal dominantly inherited syndrome attributed to mutations of the E-cadherin gene, CDH1. There is no proven effective screening for early HDGC, and symptomatic disease is almost universally fatal. The only available effective option for CDH1 carriers is prophylactic total gastrectomy, but the variable age of onset of HDGC and the reduced penetrance (about 70%) of the CDHI gene further complicate patients' decision making.

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10.1016/j.suc.2008.04.006
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