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Abstract A37: AACR
Background and Aim: There is scant
information on pancreatic cancer occurring at young age. About 5 to 10
percent of patients
with pancreatic cancer have a family history of
pancreatic cancer, which is partially due to defined hereditary
syndromes
like Lynch syndrome. Recently, we have identified
deletions of the 3’ end of the EPCAM gene as a novel cause of this hereditary syndrome. In the cohort of patients with EPCAM deletions a relatively high number of pancreatic cancers and duodenal cancers were observed. We therefore aimed to assess
whether development of pancreatic or duodenal cancer at young age might be an indication of EPCAM-associated Lynch syndrome.
Conclusion: This nationwide study cohort suggests that early onset pancreatic cancer and ampulla of Vater cancer are at most
rarely associated with (EPCAM-associated) Lynch syndrome, in contrast to early onset duodenal cancer.
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