Differential development of large-cell neuroendocrine or small-cell lung carcinoma upon inactivation of 4 tumor suppressor genes

Proc Natl Acad Sci U S A. 2019 Oct 29;116(44):22300-22306. doi: 10.1073/pnas.1821745116. Epub 2019 Oct 14.

Abstract

High-grade neuroendocrine lung malignancies (large-cell neuroendocrine cell carcinoma, LCNEC, and small-cell lung carcinoma, SCLC) are among the most deadly lung cancer conditions with no optimal clinical management. The biological relationships between SCLC and LCNEC are still largely unknown and a current matter of debate as growing molecular data reveal high heterogeneity with potential therapeutic consequences. Here we describe murine models of high-grade neuroendocrine lung carcinomas generated by the loss of 4 tumor suppressors. In an Rbl1-null background, deletion of Rb1, Pten, and Trp53 floxed alleles after Ad-CMVcre infection in a wide variety of lung epithelial cells produces LCNEC. Meanwhile, inactivation of these genes using Ad-K5cre in basal cells leads to the development of SCLC, thus differentially influencing the lung cancer type developed. So far, a defined model of LCNEC has not been reported. Molecular and transcriptomic analyses of both models revealed strong similarities to their human counterparts. In addition, a 68Ga-DOTATOC-based molecular-imaging method provides a tool for detection and monitoring the progression of the cancer. These data offer insight into the biology of SCLC and LCNEC, providing a useful framework for development of compounds and preclinical investigations in accurate immunocompetent models.

Keywords: LCNEC; SCLC; cell of origin; tumor suppressor.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Carcinoma, Small Cell / diagnostic imaging
  • Carcinoma, Small Cell / genetics*
  • Carcinoma, Small Cell / pathology
  • Genes, Tumor Suppressor*
  • Humans
  • Lung Neoplasms / diagnostic imaging
  • Lung Neoplasms / genetics*
  • Lung Neoplasms / pathology
  • Mice
  • Neuroendocrine Tumors / diagnostic imaging
  • Neuroendocrine Tumors / genetics*
  • Neuroendocrine Tumors / pathology
  • Octreotide / analogs & derivatives
  • Organometallic Compounds
  • PTEN Phosphohydrolase / genetics
  • PTEN Phosphohydrolase / metabolism
  • Positron-Emission Tomography
  • Radiopharmaceuticals
  • Retinoblastoma Binding Proteins / genetics
  • Retinoblastoma Binding Proteins / metabolism
  • Retinoblastoma-Like Protein p107 / genetics
  • Retinoblastoma-Like Protein p107 / metabolism
  • Transcriptome
  • Tumor Suppressor Protein p53 / genetics
  • Tumor Suppressor Protein p53 / metabolism

Substances

  • Ga(III)-DOTATOC
  • Organometallic Compounds
  • Radiopharmaceuticals
  • Rb1 protein, mouse
  • Rbl1 protein, mouse
  • Retinoblastoma Binding Proteins
  • Retinoblastoma-Like Protein p107
  • Tumor Suppressor Protein p53
  • PTEN Phosphohydrolase
  • Octreotide