Pulmonary vein stenosis: Expression of receptor tyrosine kinases by lesional cells

Wolfram F.J. Riedlinger, Amy L. Juraszek, Kathy J. Jenkins, Alan W. Nugent, Sowmya Balasubramanian, Monica L. Calicchio, Mark W. Kieran, Tucker Collins*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

77 Scopus citations


Background: Primary pulmonary vein stenosis (PVS) is a progressive disorder of infants. Although catheter based intervention and chemotherapy are used to manage the disorder, the benefit of these approaches is reduced considerably by restenosis. The nature of the intimal cells causing the occlusive lesions in PVS is poorly understood. Methods: Seven PVS cases were studied with antibodies for smooth muscle actin (SMA), muscle-specific actin (MSA), monoclonal desmin, S100 protein, CD31, CD34, CD45RO, CD68, CD99, Ki-67 (MIB-I), and with antibodies directed against several receptor tyrosine kinases (RTK), including platelet-derived growth factor alpha and beta receptor (PDGFR-α and -β), epidermal growth factor receptor (EGFR), fibroblast growth factor receptor (FGFR), vascular endothelial growth factor 1 and 2 receptor (VEGFR), and stem cell factor receptor (c-kit). Results: Lesional cells stained strongly and diffusely with SMA and MSA, but not for macrophage, lymphocyte, endothelial markers, or for Ki-67. RTK expression was strong and diffuse for PDGFR-α and -β, FGFR, and VEGFR-2. Lesional cells stained for VEGF and PDGF β receptor was phosphorylated. Conclusions: The histologic appearance, and the strong diffuse immunoreactivity for smooth muscle markers, indicates that the intimal lesional cells are myofibroblast-like. Expression of various receptor tyrosine kinases and some ligands suggests an autocrine or paracrine role of these proteins in the pathogenesis of the intimal occlusive lesion in PVS.

Original languageEnglish (US)
Pages (from-to)91-99
Number of pages9
JournalCardiovascular Pathology
Issue number2
StatePublished - Mar 2006


  • Myofibroblasts
  • Pulmonary vein stenosis
  • Receptor tyrosine kinases

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Cardiology and Cardiovascular Medicine


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