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BCL9L expression in pancreatic neoplasia with a focus on SPN: a possible explanation for the enigma of the benign neoplasia.


ABSTRACT: Solid pseudopapillary neoplasms of the pancreas (SPN) are rare tumors affecting mainly women. They show an activating mutation in CTNNB1, the gene for ?-catenin, and consequently an overactivation of the Wnt/?-catenin pathway. This signaling pathway is implied in the pathogenesis of various aggressive tumors, including pancreatic adenocarcinomas (PDAC). Despite this, SPN are characterized by an unusually benign clinical course. Attempts to explain this lack of malignancy have led to the discovery of an aberrant expression of the transcription factor FLI1 in SPN.In 42 primary pancreatic tumors the RNA-expression of the FLI1 targets DKK1, INPP5D, IGFBP3 and additionally two members of the Wnt/?-catenin pathway, namely BCL9 and BCL9L, was investigated using quantitative real time PCR. Expression of these genes was evaluated in SPN (n?=?18), PDAC (n?=?12) and the less aggressive intraductal papillary mucinous neoplasm IPMN (n?=?12) and compared to normal pancreatic tissue. Potential differences between the tumor entities were evaluated using students t-test.The results demonstrated a differential RNA-expression of BCL9L with a lack of expression in SPN (p?

SUBMITTER: Hallas C 

PROVIDER: S-EPMC4991076 | biostudies-literature | 2016 Aug

REPOSITORIES: biostudies-literature

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BCL9L expression in pancreatic neoplasia with a focus on SPN: a possible explanation for the enigma of the benign neoplasia.

Hallas Cora C   Phillipp Julia J   Domanowsky Lukas L   Kah Bettina B   Tiemann Katharina K  

BMC cancer 20160818


<h4>Background</h4>Solid pseudopapillary neoplasms of the pancreas (SPN) are rare tumors affecting mainly women. They show an activating mutation in CTNNB1, the gene for β-catenin, and consequently an overactivation of the Wnt/β-catenin pathway. This signaling pathway is implied in the pathogenesis of various aggressive tumors, including pancreatic adenocarcinomas (PDAC). Despite this, SPN are characterized by an unusually benign clinical course. Attempts to explain this lack of malignancy have  ...[more]

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