Characterization and Targeting of Platelet-Derived Growth Factor Receptor alpha (PDGFRA) in Inflammatory Breast Cancer (IBC)

Neoplasia. 2017 Jul;19(7):564-573. doi: 10.1016/j.neo.2017.03.002. Epub 2017 Jun 10.

Abstract

Purpose: Inflammatory breast cancer (IBC) is arguably the deadliest form of breast cancer due to its rapid onset and highly invasive nature. IBC carries 5- and 10-year disease-free survival rates of ~45% and <20%, respectively. Multiple studies demonstrate that in comparison with conventional breast cancer, IBC has a unique molecular identity. Here, we have identified platelet-derived growth factor receptor alpha (PDGFRA) as being uniquely expressed and active in IBC patient tumor cells.

Experimental design: Here we focus on characterizing and targeting PDGFRA in IBC. Using gene expression, we analyzed IBC patient samples and compared them with non-IBC patient samples. Further, using IBC cells in culture, we determined the effect of small molecules inhibitors in both in vitro and in vivo assays.

Results: In IBC patients, we show more frequent PDGFRA activation signature than non-IBC samples. In addition, the PDGFRA activation signature is associated with shorter metastasis-free survival in both uni- and multivariate analyses. We also demonstrate that IBC cells express active PDGFRA. Finally, we show that PDGFRA targeting by crenolanib (CP-868-596), but not imatinib (STI571), two small molecule inhibitors, interferes with IBC cell growth and emboli formation in vitro and tumor growth in vivo.

Conclusions: Our data suggest that PDGFRA may be a promising target for therapy in IBC.

MeSH terms

  • Adult
  • Aged
  • Animals
  • Antineoplastic Agents / pharmacology*
  • Antineoplastic Agents / therapeutic use
  • Benzimidazoles / pharmacology*
  • Benzimidazoles / therapeutic use
  • Cell Line, Tumor
  • Cell Survival / drug effects
  • Cell Survival / genetics
  • Disease Models, Animal
  • Female
  • Follow-Up Studies
  • Gene Expression
  • Humans
  • Inflammatory Breast Neoplasms / drug therapy
  • Inflammatory Breast Neoplasms / genetics
  • Inflammatory Breast Neoplasms / metabolism*
  • Inflammatory Breast Neoplasms / pathology
  • Mice
  • Middle Aged
  • Mutation
  • Neoplasm Grading
  • Piperidines / pharmacology*
  • Piperidines / therapeutic use
  • Platelet-Derived Growth Factor / pharmacology
  • Receptor, Platelet-Derived Growth Factor alpha / antagonists & inhibitors*
  • Receptor, Platelet-Derived Growth Factor alpha / genetics
  • Receptor, Platelet-Derived Growth Factor alpha / metabolism*
  • Xenograft Model Antitumor Assays

Substances

  • Antineoplastic Agents
  • Benzimidazoles
  • Piperidines
  • Platelet-Derived Growth Factor
  • Receptor, Platelet-Derived Growth Factor alpha
  • crenolanib