Paediatric and Perinatal Pathology Update on vascular anomalies of childhood

Update of the ISSVA classification for vascular anomalies

Michel Wassef M.D. Dept of pathology and vascular anomalies clinics Lariboisière Hospital Paris, France Cavernous / Angioma Venous Malformation Cirsoid Aneurysm Tuberous Angioma Immature hemangioma

Venous angioma Venous hemangioma

Capillary hemangioma Artreriovenous Malformation World Health Organization Classification of Tumours Pathology and Genetics of Tumours of

Soft Tissue and Bone 2013 WHO 2002 - Synovial hemangioma "it is often difficult to determine whether - Intramuscular hemangioma benign vascular are - Venous hemangioma malformations, - Arteriovenous malformation/hemangioma true Epithelioid hemangioma or, in some cases, reactive processes." Lymphangioma Kaposiform haemangioendothelioma Retiform haemangioendothelioma Papillary intralymphatic angioendothelioma Composite haemangioendothelioma Kaposi Pseudomyogenic haemangioendothelioma Other intermediate vascular neoplasms polymorphous haemangioendothelioma giant cell angioblastoma Epithelioid haemangioendothelioma of soft tissue World Health Organization Classification of Tumours Pathology and Genetics of Tumours 2006

Also mentioned in the text: Bacillary angiomatosis Reactive angioendotheliomatosis Verrucous hemangioma Cavernous hemangioma Venous malformation

"Cavernous hemangioma" This results in:

• Discrepancies in the nomenclature used by the different physicians involved in the diagnosis and treatment of the same patient • Misinterpretation of imaging or pathological reports, leading to diagnostic errors • Non-optimal or inadequate treatments The major steps in the nowadays understanding of "vascular anomalies"

1976 : 1st biennal workshop on " vascular anomalies " (VA) John Mulliken, Anthony Young Boston, London, Paris, Milan, … 1982 : 1st histogenetical classification of VA Mulliken et Glowaki Distinction tumors / malformations 1992 : International Society for the Study of Vascular Anomalies (ISSVA) 1996 : adoption of the histogenetical scheme of classification ISSVA Rome 1996

2014 : adoption of a more detailed and updated classification ISSVA Melbourne 2014

ISSVA Classification (Rome 1996)

Classification and terminology of vascular malformations (round table) Rome 25/06/1996 Schobinger R. Enjolras O. Loose D. Mulliken J.B. Vanwijk R. Wassef M.

Vascular tumors and vascular malformations (new issues). Adv Dermatol 1997

due to active proliferation caused by inborn localized errors Combined: made of 2 or more vessel types of endothelial cells in vascular morphogenesis ISSVA Classification (Rome 1996)

Vascular tumors and vascular malformations (new issues). Adv Dermatol 1997

• This scheme of classification is simple and proofed useful • Oversimplification of the "Tumors" group hemangioma is intended here as "infantile hemangioma" • The classification of malformations lists vessel types instead of diseases Discussed at the Hamburg Classification 7th international workshop on vascular anomalies of vascular malformations Hamburg 1988

Forms Types Truncular Extratruncular Predominantly arterial Aplasia or obstructive Infiltrating Dilatation Limited Predominantly venous Aplasia or obstructive Infiltrating Dilatation Limited Predominantly lymphatic Aplasia or obstructive Infiltrating Dilatation Limited Predominantly A-V shunting Deep Infiltrating Superficial Limited Combined / mixed Arterial and venous, without A-V shunts Hemolymphatic, with Infiltrating hemolymphatic or without A-V shunt Limited hemolymphatic

"Troncular" Malformations

Mulliken JB et al. Vascular anomalies, Oxford Univ Press 2013 Courtesy of Dr. JC Lopez Gutierrez

Courtesy of Dr. JC Lopez Gutierrez Hofer Eur J Dermatol 2005 Courtesy of Dr. JC Lopez Gutierrez What should be a working classification of vascular anomalies

• a hierarchical list of the different existing diseases grouped in a logical manner • using a different name for each different disease. A classification is also a nomenclature • should be based on – Mulliken and Glowaki distinction between T. and M. – The 1996 ISSVA scheme of classification

• should include the "truncal / non truncal" concept • should be understandable and usable by all specialties

2018

Common VM VMCM Bean Sd VM GVM CCM

Boon et al. J Craniofac Surg 2009 Cooper et al. Neurology 2008

Update on vascular anomalies of childhood

• Sylvie Fraitag: Benign vascular tumours

• Rita Alaggio: Borderline and malignant vascular tumours

• Sophie El Zein: Simple vascular malformations

• Isabel Colmenero: Syndromic vascular malformations

• Michel Wassef: Provisionally unclassified vascular anomalies • Juan Carlos López Gutierrez: Current perspectives on management of vascular anomalies

The 2014/2018 ISSVA classification is

- published in "Pediatrics" Vascular anomalies classification: recommendations from the ISSVA Wassef et al. Pediatrics 2015; 136(1):e203-14

- freely available on www.ISSVA.org

- licensed under a Creative Commons Attribution 4.0 International License you can share — copy and redistribute it adapt — remix, transform, and build upon it you must only — give appropriate credit, provide a link to the license, and indicate if changes were made.