Classification, Diagnosis, and Interventional Radiologic Management of Vascular Malformations

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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

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