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Pediatrics. 2015 Jul;136(1):e203-14. doi: 10.1542/peds.2014-3673. Epub 2015 Jun 8.

Vascular Anomalies Classification: Recommendations From the International Society for the Study of Vascular Anomalies.

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Assistance Publique-Hopitaux de Paris, Lariboisière Hospital, Department of Pathology, Paris Diderot University, Paris, France;
Vascular Birthmark Program, Lenox Hill Hospital of North Shore Long Island Jewish Healthcare System, New York, New York;
Cincinnati Children's Hospital Medical Center, Cancer and Blood Disease Institute, University of Cincinnati, Cincinnati, Ohio;
Department of Radiology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts;
Pediatric Dermatology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain;
Mt Sinai Healthcare System, Ichan School of Medicine, New York, New York;
Department of Radiology, Medical College of Wisconsin, Milwaukee, Wisconsin;
Department of Dermatology, University of California, San Francisco, San Francisco, California;
Department of Dermatology, Columbia University, New York, New York;
La Paz Children´s Hospital, Madrid, Spain;
Interventional Radiology, Sydney Children's Hospitals Network, Sydney, Australia;
Departments of Radiology, Surgery, and Pediatrics, The Johns Hopkins Hospital, Baltimore, Maryland;
Division of Dermatology (Pediatrics), Centre Hospithalier Universitaire Sainte-Justine, University of Montreal, Montreal, Canada;
Pediatric Dermatology, University of British Columbia, Vancouver, Canada; and.
Laboratory of Human Molecular Genetics, Christian de Duve Institute of Cellular Pathology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.


Vascular anomalies represent a spectrum of disorders from a simple "birthmark" to life- threatening entities. Incorrect nomenclature and misdiagnoses are commonly experienced by patients with these anomalies. Accurate diagnosis is crucial for appropriate evaluation and management, often requiring multidisciplinary specialists. Classification schemes provide a consistent terminology and serve as a guide for pathologists, clinicians, and researchers. One of the goals of the International Society for the Study of Vascular Anomalies (ISSVA) is to achieve a uniform classification. The last classification (1997) stratified vascular lesions into vascular malformations and proliferative vascular lesions (tumors). However, additional disease entities have since been identified that are complex and less easily classified by generic headings, such as capillary malformation, venous malformation, lymphatic malformation, etc. We hereby present the updated official ISSVA classification of vascular anomalies. The general biological scheme of the classification is retained. The section on tumors has been expanded and lists the main recognized vascular tumors, classified as benign, locally aggressive or borderline, and malignant. A list of well-defined diseases is included under each generic heading in the "Simple Vascular Malformations" section. A short definition is added for eponyms. Two new sections were created: one dealing with the malformations of individually named vessels (previously referred to as "truncular" malformations); the second groups lesions of uncertain or debated nature (tumor versus malformation). The known genetic defects underlying vascular anomalies are included in an appendix. This classification is meant to be a framework, acknowledging that it will require modification as new scientific information becomes available.

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