Wilms tumor gene 1 (WT1), TP53, RAS/BRAF and KIT aberrations in testicular germ cell tumors

Cancer Lett. 2016 Jul 1;376(2):367-76. doi: 10.1016/j.canlet.2016.04.016. Epub 2016 Apr 13.

Abstract

Purpose: Wilms tumor gene 1 (WT1), a zinc-finger transcription factor essential for testis development and function, along with other genes, was investigated for their role in the pathogenesis of testicular germ cell tumors (TGCT).

Methods: In total, 284 TGCT and 100 control samples were investigated, including qPCR for WT1 expression and BRAF mutation, p53 immunohistochemistry detection, and massively parallel amplicon sequencing.

Results: WT1 was significantly (p < 0.0001) under-expressed in TGCT, with an increased ratio of exon 5-lacking isoforms, reaching low levels in chemo-naïve relapsed TGCT patients vs. high levels in chemotherapy-pretreated relapsed patients. BRAF V600E mutation was identified in 1% of patients only. p53 protein was lowly expressed in TGCT metastases compared to the matched primary tumors. Of 9 selected TGCT-linked genes, RAS/BRAF and WT1 mutations were frequent while significant TP53 and KIT variants were not detected (p = 0.0003).

Conclusions: WT1 has been identified as a novel factor involved in TGCT pathogenesis, with a potential prognostic impact. Distinct biologic nature of the two types of relapses occurring in TGCT has been demonstrated. Differential mutation rate of the key TGCT-related genes has been documented.

Keywords: KIT; Next generation sequencing; TP53; Testicular germ cell tumors; Wilms tumor gene 1 (WT1).

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Biomarkers, Tumor / genetics*
  • Cell Line, Tumor
  • DNA Mutational Analysis / methods
  • Down-Regulation
  • Feasibility Studies
  • Genes, ras*
  • Genetic Predisposition to Disease
  • High-Throughput Nucleotide Sequencing
  • Humans
  • Immunohistochemistry
  • Male
  • Mutation*
  • Neoplasms, Germ Cell and Embryonal / enzymology
  • Neoplasms, Germ Cell and Embryonal / genetics*
  • Neoplasms, Germ Cell and Embryonal / pathology
  • Phenotype
  • Prospective Studies
  • Proto-Oncogene Proteins B-raf / genetics*
  • Proto-Oncogene Proteins c-kit / genetics*
  • Real-Time Polymerase Chain Reaction
  • Retrospective Studies
  • Testicular Neoplasms / enzymology
  • Testicular Neoplasms / genetics*
  • Testicular Neoplasms / pathology
  • Tumor Suppressor Protein p53 / genetics*
  • WT1 Proteins / genetics*

Substances

  • Biomarkers, Tumor
  • TP53 protein, human
  • Tumor Suppressor Protein p53
  • WT1 Proteins
  • WT1 protein, human
  • Proto-Oncogene Proteins c-kit
  • BRAF protein, human
  • Proto-Oncogene Proteins B-raf

Supplementary concepts

  • Testicular Germ Cell Tumor