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There are several genes known to be involved in simple renal or combined renal-extrarenal aberrations. Of these, the transcription factor 2 gene is expressed longer, from very early embryogenesis and throughout organ development during pregnancy. Transcription factor 2 gene encodes the hepatocyte nuclear factor-1 beta transcript, which is a member of the homeodomain-containing superfamily of transcription factors.

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Background: Solid pseudopapillary tumors (SPTs) represent one of the most uncommon histotypes of all exocrine pancreatic neoplasms.

Aim: To delineate the clinical presentation and biological behavior of SPT and evaluate the efficacy of treatment.

Methods: Retrospective analysis of 31 patients (27 female, 4 male, mean age of 34 years, (range 7-56)) who underwent surgical resection with a definitive histological diagnosis of SPT.

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