Since the publication of the seminal work on the histology-based classification of vascular anomalies by Mulliken and Glowacki in 1982 and the subsequent adoption of an expanded and modified version in 1996 by the International Society for the Study of Vascular Anomalies, an increasing number of vascular lesions have been recognized as histologically distinct entities. Furthermore, there have been significant advances in detailing the behavior and underlying genetics of previously identified lesions. These developments have required restructuring and expansion of the classification scheme so that appropriate therapies may be studied and implemented in affected patients. The new classification retains the broad categories of neoplasms and malformations but now divides the tumor group into benign, locally aggressive or borderline, and malignant, with the malformation group being divided into simple, combined, those of major named vessels, and those associated with other anomalies. Additionally, a category has been created for lesions in which the histology and behavior do not yet allow clear separation into neoplasm or malformation (thus named “provisionally unclassified vascular anomalies”). The known clinical courses and imaging, histologic, and genetic findings of the most common and/or clinically relevant lesions in the newly adopted revised system are reviewed in this article.
ASJC Scopus subject areas
- Radiology Nuclear Medicine and imaging