Clinical genetics of pancreatic cancer
Journal Title: Postępy Nauk Medycznych - Year 2010, Vol 23, Issue 11
Abstract
Pancreatic cancer is an aggressive disease and the mortality rate is high. Pancreatic cancer is one of the 10 most frequent malignancies in Poland. The average time of a pancreatic cancer patients' survival is 6 months. Pancreatic cancer occurs in older age. Early diagnosis is difficult because of non-specific symptoms in the initial stage of the disease. Pancreatic cancer is a malignancy with poor prognosis. Despite many efforts, little is known about the genetic etiology of pancreatic cancer.There is no gene identified as specifically predisposing to pancreatic cancer yet. An inherited predisposition to pancreatic cancer is believed to occur in three distinct clinical settings. First, it occurs in hereditary tumor predisposition syndromes that are known to be associated with an increased risk of pancreatic cancer like Peutz-Jeghers syndrome, HBOC syndrome, FAMMM syndrome, Lynch syndrome, juvenile polyposis, familial adenomatous polyposis, ataxia-teleangiectasia and Werner syndrome. The second setting is hereditary pancreatitis and cystic fibrosis, in which genetically determined early age onset changes of the pancreas can predispose to the development of pancreatic cancer. The term familial pancreatic cancer refers to families with two or more first-degree relatives with pancreatic cancer without fulfilling the criteria for another inherited tumor syndrome.Diagnostic algorithms for pancreatic cancer refer to the situation when clinical symptoms occur. The usefulness of these algorithms in detecting early stages of pancreatic cancer in patients carrying DNA changes predisposing to this malignancy is unknown. It is possible that recognition of the genetic background of pancreatic cancer will enable effective chemoprevention and chemotherapy.
Authors and Affiliations
Katarzyna Nej-Wołosiak, Tomasz Huzarski, Jan Lubiński
Keywords
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