Vascular Anomalies Classification: Recommendations From the International Society for the Study of Vascular Anomalies Michel Wassef, MDa, Francine Blei, MDb, Denise Adams, MDc, Ahmad Alomari, MDd, Eulalia Baselga, MDe, Alejandro Berenstein, MDf, Patricia Burrows, MDg, Ilona J. Frieden, MDh, Maria C. Garzon, MDi, Juan-Carlos Lopez-Gutierrez, MD, PhDj, David J.E. Lord, MDk, Sally Mitchel, MDl, Julie Powell, MDm, Julie Prendiville, MDn, Miikka Vikkula, MD, PhDo, on behalf of the ISSVA Board and Scientific Committee Vascular anomalies represent a spectrum of disorders from a simple “birthmark” abstract 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 aAssistance Publique–Hopitaux de Paris, Lariboisière multidisciplinary specialists. Classification schemes provide a consistent Hospital, Department of Pathology, Paris Diderot University, Paris, France; bVascular Birthmark Program, Lenox Hill terminology and serve as a guide for pathologists, clinicians, and researchers. Hospital of North Shore Long Island Jewish Healthcare OneofthegoalsoftheInternationalSociety for the Study of Vascular Anomalies System, New York, New York; cCincinnati Children’s Hospital fi fi Medical Center, Cancer and Blood Disease Institute, University (ISSVA) is to achieve a uniform classi cation. The last classi cation (1997) of Cincinnati, Cincinnati, Ohio; dDepartment of Radiology, stratified vascular lesions into vascular malformations and proliferative vascular Boston Children’s Hospital and Harvard Medical School, e fi Boston, Massachusetts; Pediatric Dermatology, Hospital de lesions (tumors). However, additional disease entities have since been identi ed la Santa Creu i Sant Pau, Barcelona, Spain; fMt Sinai that are complex and less easily classified by generic headings, such as capillary Healthcare System, Ichan School of Medicine, New York, New York; gDepartment of Radiology, Medical College of Wisconsin, malformation, venous malformation, lymphatic malformation, etc. We hereby Milwaukee, Wisconsin; hDepartment of Dermatology, present the updated official ISSVA classification of vascular anomalies. The University of California, San Francisco, San Francisco, i general biological scheme of the classification is retained. The section on tumors California; Department of Dermatology, Columbia University, New York, New York; jLa Paz Children´s Hospital, Madrid, has been expanded and lists the main recognized vascular tumors, classified as Spain; kInterventional Radiology, Sydney Children’s Hospitals benign, locally aggressive or borderline, and malignant. A list of well-defined Network, Sydney, Australia; lDepartments of Radiology, “ Surgery, and Pediatrics, The Johns Hopkins Hospital, diseases is included under each generic heading in the Simple Vascular Baltimore, Maryland; mDivision of Dermatology (Pediatrics), Malformations” section. A short definition is added for eponyms. Two new Centre Hospithalier Universitaire Sainte-Justine, University of Montreal, Montreal, Canada; nPediatric Dermatology, sections were created: one dealing with the malformations of individually named University of British Columbia, Vancouver, Canada; and vessels (previously referred to as “truncular” malformations); the second groups oLaboratory of Human Molecular Genetics, Christian de Duve Institute of Cellular Pathology, Cliniques Universitaires Saint- lesions of uncertain or debated nature (tumor versus malformation). The known Luc, Université Catholique de Louvain, Brussels, Belgium genetic defects underlying vascular anomalies are included in an appendix. This classification is meant to be a framework, acknowledging that it will require Dr Wassef conceptualized and designed the classification and drafted the initial manuscript; Dr modification as new scientific information becomes available. Blei, as chair of the scientific committee of the International Society for the Study of Vascular Anomalies, led the initiative for the updated Vascular anomalies (vascular tumors common. Others such as port wine classification and spearheaded the project and critically reviewed and revised the manuscript; Drs and vascular malformations), often stain are uncommon, and still others Adams, Baselga, Burrows, Frieden, Garzon, Lord, and named “angioma” or hemangioma, in are quite rare. Despite long-standing Prendiville participated in the design of the fact represent a broad spectrum of efforts to promulgate standard classification and critically reviewed and revised the manuscript; Dr Alomari participated in the design of disorders from a simple “birthmark” to classification, nomenclature the classification; Drs Berenstein, Lopez-Gutierrez, life-threatening entities, which affect terminology of vascular anomalies and Vikkula participated in the design of the mainly infants, children, and young continues to be confusing.1–4 The term classification and critically reviewed the adults. Certain vascular anomalies, “hemangioma” is, for example, manuscript; Drs Mitchel and Powell critically reviewed and revised the manuscript; and all such as salmon patch (nevus simplex) commonly used to name different authors approved the final manuscript as or infantile hemangioma, are very types of vascular tumors as well as submitted. PEDIATRICS Volume 136, number 1,Downloaded July 2015 from http://pediatrics.aappublications.org/ by guest on March 29, 2018 SPECIAL ARTICLE vascular malformations, despite the TABLE 2 2014 ISSVA Classification of Vascular Anomalies different constitution, natural Vascular Anomalies evolution, and treatment of these 2 Vascular Tumors Vascular Malformations groups of lesions. Incorrect nomenclature and misdiagnoses are Simple Combined Of Major Named Associated With Other commonly experienced by patients Vessels Anomalies with vascular anomalies.2 Accurate Benign CM See Table 5 See text See Table 6 diagnosis and common terminology Locally aggressive or LM borderline VM are crucial for appropriate evaluation Malignant AVM and management, often requiring Arteriovenous multidisciplinary specialists. fistula One of the goals of the International Society for the Study of Vascular Melbourne, Australia (April 2014). classifications misleading and Anomalies (ISSVA) is to achieve The goal of this article is to briefly confusing.8–10 It is then difficult for fi a uniform classi cation. The 1996 discuss some of the present clinicians across specialties to fi fi ISSVA classi cation strati ed vascular classifications and to introduce the communicate using a common anomalies into vascular 2014 updated ISSVA classification. The language specific for each entity, and malformations and proliferative interactive document is available at confusion in the naming of lesions vascular lesions (tumors) (Table 1).5,6 www.issva.org. This classification is creates inaccuracies in scientific This classification was then meant to represent the state-of-the-art advances and the dissemination of “unofficially” updated on the basis of in vascular anomalies classification, knowledge that may help patients. It evolving knowledge a decade later.7 acknowledging that it will require also makes coding and statistical data However, since then, knowledge modification as new scientific about the prevalence and incidence of about these disorders has increased information becomes available. these lesions inaccurate. considerably. The genetic basis of Several classifications of vascular The 1996 ISSVA classification many types of vascular anomalies are available; some are scheme5 is based on the fundamental malformations has been elucidated general classifications, and others deal separation of vascular anomalies into and additional disease entities have with specific organs or tissues or only been identified that need more with vascular tumors or vascular precise classification rather than TABLE 3 Classification of Vascular Tumors malformations. The earliest generic headings such as capillary Benign vascular tumors classification was that of Virchow, malformation (CM), venous Infantile hemangioma/hemangioma of infancy which was a pathologic classification malformation (VM), lymphatic Congenital hemangioma that classified vascular anomalies as Rapidly involuting CH (RICH)a malformation (LM), etc, which have “angioma simplex, angioma Noninvoluting CH (NICH) been used previously. The ISSVA Partially involuting CH (PICH) cavernosum, angioma racemosum and Classification of Vascular Anomalies Tufted angiomaa,b lymphangioma.”8–10 This was was recently updated by the Society’s Spindle cell hemangioma a primitive classification system but Scientific Committee and Board to Epithelioid hemangioma appropriate for the time. The World Pyogenic granuloma (or lobular capillary incorporate these changes and was Health Organization (WHO) hemangioma) adopted at the last workshop in Others classifications are generally Locally aggressive or borderline vascular tumors TABLE 1 1996 ISSVA Classification Scheme considered as the reference Kaposiform hemangioendotheliomaa,b fi Vascular Anomalies classi cation for tumors and tumorlike Retiform hemangioendothelioma diseases. The WHO classification of Papillary intralymphatic angioendothelioma, Vascular tumors Vascular Malformations skin vascular “tumors”11 is Dabska tumor Composite hemangioendothelioma Simple Combined a nonhierarchical list of a series of Kaposi sarcoma Hemangioma Capillary AVF, AVM different diseases, irrespective of their Others Others Lymphatic CVM, CLM tumor, malformation,