Genotype/Phenotype of Familial Pancreatic Cancer

The challenges in defining pancreatic cancer are great because of our recognition that even sporadic cancer cases are likely at the end of a spectrum, where the individual genetic etiologies are more difficult to identify [24]. A polygenic model that takes into account nongenetic risk factors, most notably smoking, seems to be most appropriate for pancreatic cancer. With few exceptions (eg, hereditary pancreatitis), Mendelian inherited pancreatic cancer-prone syndromes involve a variety of cancers of different anatomic sites, wherein their phenotypic and genotypic heterogeneity may be extant.