Educational paper: Pathogenesis of infantile haemangioma, an update 2014 (part I)

Eur J Pediatr. 2015 Jan;174(1):97-103. doi: 10.1007/s00431-014-2403-6. Epub 2014 Aug 26.

Abstract

Infantile haemangioma (IH) is the most frequent childhood tumour. Although it is benign and self-limiting, severe complications can arise due to localisation and fast tumour growth. Management and therapy of IH has changed greatly after 2008 with propranolol. However, the pathogenesis remains elusive. This update provides an overview of all possible mechanisms currently considered. We discuss the possibility that several mechanisms act together, although local hypoxia seems to be important. Clinically, in about half of the cases, an IH is preceded by an anaemic macula (local ischaemia) or a so-called precursor lesion. Laboratory findings indicate stabilisation and an increased transcription activity of hypoxia-inducible factor 1 alpha (HIF1α), leading to up-regulation of its downstream target genes (such as vascular endothelial growth factor (VEGF)), which normally occurs in cases of hypoxia.

Conclusion: Three main hypotheses have been proposed, namely (1) the theory of tissue hypoxia, (2) the theory of embolization of placental endothelial cells and (3) the theory of increased angiogenic and vasculogenic activity.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adrenergic beta-Antagonists / therapeutic use
  • Hemangioma, Capillary / drug therapy
  • Hemangioma, Capillary / etiology*
  • Hemangioma, Capillary / metabolism
  • Humans
  • Hypoxia-Inducible Factor 1, alpha Subunit / metabolism
  • Infant
  • Neoplastic Syndromes, Hereditary / drug therapy
  • Neoplastic Syndromes, Hereditary / etiology*
  • Neoplastic Syndromes, Hereditary / metabolism
  • Propranolol / therapeutic use
  • Vascular Endothelial Growth Factor A / metabolism

Substances

  • Adrenergic beta-Antagonists
  • HIF1A protein, human
  • Hypoxia-Inducible Factor 1, alpha Subunit
  • VEGFA protein, human
  • Vascular Endothelial Growth Factor A
  • Propranolol

Supplementary concepts

  • Hemangioma, capillary infantile