Cardiac myxoma and neural crests: a tense relationship

Cardiovasc Pathol. 2020 Jan-Feb:44:107163. doi: 10.1016/j.carpath.2019.107163. Epub 2019 Oct 25.

Abstract

In cardiac myxomas, the malignant transformation process, selecting incidental gene mutations and leading to loss of proliferation control, has not a so drastic effects in terms of growth rate of tumor mass, but frequently the particular location of lesion engrosses the high risk for health. For accurate cancer cell profiling, it is important to establish the embryologic origin of malignant cells and their initial commitments, above all, in the sight of therapeutic strategies and solutions. Here, we advance, for cardiac myxoma, the hypothesis of an origin from cardiac neural crest cells and we attempt to support it by an integrated discussion of current knowledge about embryological characteristics of neural crest cells and most recent studies focusing cardiac myxomas. We discuss the relationship between the basic plasticity of cardiac neural crest cells and some typical mutations arising in neoplastic lesions as well as the expression of typical cell markers of neural crests derivatives. Dysfunctions in proliferative and migratory programs, focused in other studies, are evaluated in the context of the topological and histopathological characteristics of cardiac myxomas.

Keywords: Cardiac myxoma; Cardiac neural crests; Carney complex; Epithelial mesenchymal transition; Mast cells.

Publication types

  • Review

MeSH terms

  • Animals
  • Biomarkers, Tumor / genetics
  • Biomarkers, Tumor / metabolism
  • Carney Complex / genetics
  • Carney Complex / metabolism
  • Carney Complex / pathology*
  • Cell Movement
  • Cell Plasticity
  • Cell Proliferation*
  • Cell Transformation, Neoplastic / genetics
  • Cell Transformation, Neoplastic / pathology*
  • Epithelial-Mesenchymal Transition*
  • Gene Expression Regulation, Developmental
  • Gene Expression Regulation, Neoplastic
  • Heart Neoplasms / genetics
  • Heart Neoplasms / metabolism
  • Heart Neoplasms / pathology*
  • Humans
  • Male
  • Mutation
  • Myocardium / metabolism
  • Myocardium / pathology*
  • Neural Crest / metabolism
  • Neural Crest / pathology*
  • Signal Transduction

Substances

  • Biomarkers, Tumor