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The biologic classification of vascular anomalies was first proposed by Mulliken and Glowacki in 1982. Since then, the International Society for the Study of Vascular Anomalies (ISSVA) has continued to update this original schema to reflect a growing number and improved understanding of vascular anomalies. A working knowledge of the classification of vascular anomalies is critical for making the correct diagnosis and guiding management. At the most basic level, vascular anomalies are divided into tumors or malformations and can usually be distinguished from one another based on clinical appearance and growth characteristics. If diagnosis is unclear, observation over time, imaging studies, and/or biopsy may be required. The past few years have brought about major advances in our understanding of the genetic basis for many vascular tumors and malformations, in particular for those associated with other anomalies. An overview of the most recent ISSVA classification is presented in Tables 359-1, 359-2, 359-3, adapted from the 2014 updated classification.
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