Copy number aberrations of genes regulating normal thymus development in thymic epithelial tumors

Clin Cancer Res. 2013 Apr 15;19(8):1960-71. doi: 10.1158/1078-0432.CCR-12-3260. Epub 2013 Feb 26.

Abstract

Purposes: To determine whether the deregulation of genes relevant for normal thymus development can contribute to the biology of thymic epithelial tumors (TET).

Experimental design: Using array comparative genomic hybridization, we evaluated the copy number aberrations of genes regulating thymus development. The expression of genes most commonly involved in copy number aberrations was evaluated by immunohistochemistry and correlated with patients' outcome. Correlation between FOXC1 copy number loss and gene expression was determined in a confirmation cohort. Cell lines were used to test the role of FOXC1 in tumors.

Results: Among 31 thymus development-related genes, PBX1 copy number gain and FOXC1 copy number loss were presented in 43.0% and 39.5% of the tumors, respectively. Immunohistochemistry on a series of 132 TETs, including those evaluated by comparative genomic hybridization, revealed a correlation between protein expression and copy number status only for FOXC1 but not for PBX1. Patients with FOXC1-negative tumors had a shorter time to progression and a trend for a shorter disease-related survival. The correlation between FOXC1 copy number loss and mRNA expression was confirmed in a separate cohort of 27 TETs. Ectopic FOXC1 expression attenuated anchorage-independent cell growth and cell migration in vitro.

Conclusion: Our data support a tumor suppressor role of FOXC1 in TETs.

Trial registration: ClinicalTrials.gov NCT00965627.

Publication types

  • Clinical Trial
  • Research Support, N.I.H., Intramural

MeSH terms

  • Adult
  • Aged
  • Animals
  • Cell Adhesion
  • Cell Line, Tumor
  • Cell Movement / genetics
  • Cell Proliferation
  • Cohort Studies
  • Comparative Genomic Hybridization
  • DNA Copy Number Variations*
  • DNA-Binding Proteins / genetics*
  • DNA-Binding Proteins / metabolism
  • Female
  • Forkhead Transcription Factors / genetics*
  • Forkhead Transcription Factors / metabolism
  • Gene Expression Regulation, Neoplastic
  • HEK293 Cells
  • Humans
  • Immunohistochemistry
  • Male
  • Mice
  • Middle Aged
  • NIH 3T3 Cells
  • Neoplasms, Glandular and Epithelial / genetics*
  • Neoplasms, Glandular and Epithelial / pathology
  • Pre-B-Cell Leukemia Transcription Factor 1
  • Proto-Oncogene Proteins / genetics*
  • Proto-Oncogene Proteins / metabolism
  • Reverse Transcriptase Polymerase Chain Reaction
  • Sequence Analysis, DNA
  • Thymus Gland / growth & development
  • Thymus Gland / metabolism*
  • Thymus Neoplasms / genetics*
  • Thymus Neoplasms / pathology

Substances

  • DNA-Binding Proteins
  • FOXC1 protein, human
  • Forkhead Transcription Factors
  • Pre-B-Cell Leukemia Transcription Factor 1
  • Proto-Oncogene Proteins
  • PBX1 protein, human

Supplementary concepts

  • Thymic epithelial tumor

Associated data

  • ClinicalTrials.gov/NCT00965627