Classification, Diagnosis, and Interventional Radiologic Management of Vascular Malformations
Section snippets
Classification of vascular anomalies
In 1982, Mulliken and Glowacki [4] published a landmark article proposing characterization of vascular defects based on biologic and pathologic differences. Their work differentiated between two major categories of vascular lesions: hemangiomas and vascular malformations. Hemangiomas were described as lesions exhibiting a history of rapid neonatal growth and slow involution characterized by hypercellularity during the proliferating phase and fibrosis and diminished cellularity during the
Histology and immunohistochemistry
As described in Mulliken's original dissertation, traditional light microscopic staining techniques using hematoxylin and eosin among others is usually sufficient to differentiate vascular tumors such as infantile hemangioma from vascular malformations [4]. More recent advances in immunohistochemical techniques have allowed greater tissue diagnostic accuracy in lesions of ambiguous or mixed histology on conventional microscopy.
Hemangiomas, in proliferative phase, show increased endothelial cell
Embryology, vascular morphogenesis, and molecular genetics
Because of early rapid tissue growth and need for tissue oxygenation, the embryonic circulation is the first functional system to develop, with intraluminal blood circulating at 3 weeks' gestation [26]. In the first phase of vascular morphogenesis, vasculogenesis occurs by differentiation and organization of mesoderm into the primitive capillary network. Mesodermal angioblasts congregate to form blood islands, which cavitate centrally forming short tubes. Outermost cells form into primordial
Clinical diagnosis
The diagnosis of presence and type of vascular malformation usually can be made purely on clinical history and physical examination. By definition, all vascular malformations are present at birth, and most become evident to some degree during infancy or childhood. Lesions labeled as “acquired” during adolescence are usually those of insufficient size or symptoms to have been detected during the period of pari passu growth in childhood, which became clinically evident as the lesion underwent
Imaging diagnosis and workup of vascular malformations
Although clinical history and examination are sufficient to establish the diagnosis of a vascular malformation, imaging is an indispensable part of the full patient workup. In addition to confirming the diagnosis, defining the extent of the lesion, and detecting often occult associated pathologic findings, imaging allows feasibility assessment and planning of any potential percutaneous image-guided or surgical therapy [47].
Therapeutic options for vascular malformations
Essential to the optimal care of patients with vascular malformations is the assembly of an experienced multidisciplinary team that is well versed in the latest diagnostic and therapeutic techniques and controversies within the field of study of vascular anomalies. This level of familiarity and expertise usually can be achieved only through frequent exposure to vascular anomalies facilitated through streamlined interdisciplinary communication and usually is found at larger referral institutions
Rationale
Whether percutaneous or surgical, the ultimate goal of any therapy for vascular malformations is to correct or remove the focal structural vascular derangement or mass responsible for the patient's symptoms. Because vascular malformations are the result of continued disordered growth, or more precisely enlargement of structures originating and controlled at the endothelial level, only therapy directed to this end is effective. Endothelial cells not only direct continued vascular growth and
Summary
Confusion still exists as to the classification and nomenclature of vascular anomalies, which has an impact on the clinical diagnosis and management. The term hemangioma should be reserved for lesions histologically exhibiting rapid hypercellular endothelial growth, most commonly seen in the form of infantile hemangioma in pediatric patients. Essentially all other vascular anomalies are termed vascular malformations and are described as being present at birth and growing commensurately or pari
References (160)
- et al.
Advanced management of congenital vascular malformations: a multidisciplinary approach
Cardiovasc Surg
(2002) - et al.
GLUT1: a newly discovered immunohistochemical marker for juvenile hemangiomas
Hum Pathol
(2000) - et al.
Monoclonal antibody D2–40, a new marker of lymphatic endothelium, reacts with Kaposi's sarcoma and a subset of angiosarcomas
Mod Pathol
(2002) - et al.
Cultured endothelial cells from human arteriovenous malformations have defective growth regulation
Blood
(1999) - et al.
Management of arteriovenous malformations: a multidisciplinary approach
J Vasc Surg
(2004) - et al.
Molecular genetics of vascular malformations
Matrix Biol
(2001) - et al.
Angiopoietin and Tie signaling pathways in vascular development
Matrix Biol
(2001) - et al.
The angiopoietins and Tie2/Tek: adding to the complexity of cardiovascular development
Semin Cell Dev Biol
(2002) - et al.
Requisite role of angiopoietin-1, a ligand for the TIE2 receptor, during embryonic angiogenesis
Cell
(1996) - et al.
Angiopoietin-2 is required for postnatal angiogenesis and lymphatic patterning, and only the latter role is rescued by Angiopoietin-1
Dev Cell
(2002)
25 years' experience with lymphangiomas in children
J Pediatr Surg
Lymphangioma: an otolaryngologic perspective
Int J Pediatr Otorhinolaryngol
Uterine arteriovenous malformations: the role of intravenous ‘dual-phase’ CT angiography
Clin Imaging
Magnetic resonance of vascular anomalies
Magn Reson Imaging Clin N Am
New approaches to the treatment of congenital vascular malformations (CMVs): a single centre experience
Eur J Vasc Endovasc Surg
Primum non nocere: is it always true? The use of absolute ethanol in the management of congenital vascular malformations
J Vasc Surg
Vascular malformations of the upper limb: a review of 270 patients
J Hand Surg Am
Vascular low-flow malformations in children: current concepts for classification, diagnosis and therapy
Eur J Radiol
Intramuscular low flow vascular malformations: treatment by means of direct percutaneous embolization
Eur J Radiol
Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics
Plast Reconstr Surg
Childhood hemangiomas and vascular malformations: angiographic differentiation
AJR Am J Roentgenol
Hemangiomas, vascular malformations, and lymphovenous malformations: classification and methods of treatment
Plast Reconstr Surg
Malformations (Angiodysplasias)
Classification of the vascular diseases of the limbs
J Cardiovasc Surg (Torino)
Classification of congenital vascular defects
Int Angiol
Anatomopathological classification of congenital vascular defects
Semin Vasc Surg
Classification and management of the various superficial vascular anomalies: hemangioma and vascular malformation
J Dermatol
Hemangioma versus vascular malformation: presence of nerve bundle is a diagnostic clue for vascular malformation
Arch Pathol Lab Med
Venous malformations of skeletal muscle
Plast Reconstr Surg
Diagnostic musculoskeletal surgical pathology: clinicoradiologic and cytologic correlations
Vascular anomalies
Curr Probl Surg
Vascular birthmarks: hemangiomas and malformations
Molecular basis of vascular anomalies
Trends Cardiovasc Med
Soft-tissue venous malformations in adult patients: imaging and therapeutic issues
Radiographics
Characterization and distribution of an oncofetal antigen (M2A antigen) expressed on testicular germ cell tumours
Br J Cancer
Port-wine stains: a disease of altered neural modulation of blood vessels?
Arch Dermatol
The developing human
Vascular malformations: localized defects in vascular morphogenesis
Clin Genet
Lymphatic vasculature development
Nat Rev Immunol
Hereditary vascular anomalies: new insights into their pathogenesis
Arterioscler Thromb Vasc Biol
Assignment of a locus for dominantly inherited venous malformations to chromosome 9p
Hum Mol Genet
Isolation of angiopoietin-1, a ligand for the TIE2 receptor, by secretion-trap expression closing
Cell
Distinct roles of the receptor tyrosine kinases Tie-1 and Tie-2 in blood vessel formation
Nature
Angiopoietin-2, a natural antagonist for Tie2 that disrupts in vivo angiogenesis
Science
Altered expression patterns of EphrinB2 and EphB2 in human umbilical vessels and congenital venous malformations
Pediatr Res
Role of PDGF-B and PDGFR-beta in recruitment of vascular smooth muscle cells and pericytes during embryonic blood vessel formation in the mouse
Development
Cystic hygroma of the head and neck: a review of 37 cases
J Laryngol Otol
Cited by (137)
Arteriovenous malformation on the sole of the foot treated successfully by embolization
2020, Radiology Case ReportsBleomycin Polidocanol Foam (BPF) Stability – In Vitro Evidence for the Effectiveness of a Novel Sclerosant for Venous Malformations
2020, European Journal of Vascular and Endovascular SurgeryEffect of foam and liquid bleomycin in the management of venous malformations in head and neck region: A comparative study
2020, Journal of Plastic, Reconstructive and Aesthetic SurgeryInterventional Management of Arteriovenous Malformations
2019, Techniques in Vascular and Interventional RadiologySclerotherapy with Adjunctive Stasis of Efflux (STASE) in Venous Malformations: Techniques and Strategies
2019, Techniques in Vascular and Interventional RadiologyBrief Description of ISSVA Classification for Radiologists
2019, Techniques in Vascular and Interventional Radiology