Molecular and cellular biology of cerebral arteriovenous malformations: a review of current concepts and future trends in treatment

Neurosurg Focus. 2014 Sep;37(3):E1. doi: 10.3171/2014.7.FOCUS14214.


Object: Arteriovenous malformations (AVMs) are classically described as congenital static lesions. However, in addition to rupturing, AVMs can undergo growth, remodeling, and regression. These phenomena are directly related to cellular, molecular, and physiological processes. Understanding these relationships is essential to direct future diagnostic and therapeutic strategies. The authors performed a search of the contemporary literature to review current information regarding the molecular and cellular biology of AVMs and how this biology will impact their potential future management.

Methods: A PubMed search was performed using the key words "genetic," "molecular," "brain," "cerebral," "arteriovenous," "malformation," "rupture," "management," "embolization," and "radiosurgery." Only English-language papers were considered. The reference lists of all papers selected for full-text assessment were reviewed.

Results: Current concepts in genetic polymorphisms, growth factors, angiopoietins, apoptosis, endothelial cells, pathophysiology, clinical syndromes, medical treatment (including tetracycline and microRNA-18a), radiation therapy, endovascular embolization, and surgical treatment as they apply to AVMs are discussed.

Conclusions: Understanding the complex cellular biology, physiology, hemodynamics, and flow-related phenomena of AVMs is critical for defining and predicting their behavior, developing novel drug treatments, and improving endovascular and surgical therapies.

Keywords: ACVRL1 = activin receptor-like kinase 1; ANG = angiopoietin; ANGPTL = ANG-like; AVM = arteriovenous malformation; BDNF = brain-derived neurotrophic factor; BEC = brain endothelial cell; ENG = endoglin; HHT = hereditary hemorrhagic telangiectasia; ICH = intracranial hemorrhage; LPS/sTF = lipopolysaccharide and soluble tissue factor conjugate; MIF = macrophage migration inhibitory factor; SNP = single nucleotide polymorphism; SRS = stereotactic radiosurgery; TGF = transforming growth factor; TSP-1 = thrombospondin-1; VEGF = vascular endothelial growth factor; arteriovenous malformation; cellular biology; future trends; medical treatment; miR-18a = microRNA-18a; MMP = matrix metalloproteinase; molecular.

Publication types

  • Review

MeSH terms

  • Brain / pathology
  • Cell Biology* / trends
  • Embolization, Therapeutic
  • Humans
  • Intercellular Signaling Peptides and Proteins / genetics
  • Intercellular Signaling Peptides and Proteins / metabolism
  • Intracranial Arteriovenous Malformations* / genetics
  • Intracranial Arteriovenous Malformations* / pathology
  • Intracranial Arteriovenous Malformations* / therapy
  • Molecular Biology* / trends
  • PubMed / statistics & numerical data
  • Radiosurgery


  • Intercellular Signaling Peptides and Proteins