Update of the ISSVA classification for

vascular anomalies

Michel Wassef M.D.

Dept of pathology and vascular anomalies clinics

Lariboisière Hospital

Paris, France

Paediatric and Perinatal Pathology

Update on vascular anomalies of childhood

Lymphangioma

AngiomaVenous Malformation

Venous angioma

Capillary hemangioma

Cirsoid Aneurysm

Tuberous Angioma

Cavernous Angioma/Hemangioma

Artreriovenous Malformation

Venous hemangioma

Cystic Hygroma

Immature hemangioma

Pathology and Genetics of Tumours of

Soft Tissue and Bone

World Health Organization Classification of Tumours

WHO 2002

"it is often difficult to determine whether

benign vascular lesions are

malformations,

true neoplasms

or, in some cases, reactive processes."

Hemangiomas

- Synovial hemangioma

- Intramuscular hemangioma

- Venous hemangioma

- Arteriovenous malformation/hemangioma

Epithelioid hemangioma

Angiomatosis

Lymphangioma

Kaposiform haemangioendothelioma

Retiform haemangioendothelioma

Papillary intralymphatic angioendothelioma

Composite haemangioendothelioma

Kaposi sarcoma

Pseudomyogenic haemangioendothelioma

Other intermediate vascular neoplasms

polymorphous haemangioendothelioma

giant cell angioblastoma

Epithelioid haemangioendothelioma

Angiosarcoma of soft tissue

2013

2006

Pathology and Genetics of

Skin Tumours

World Health Organization Classification of Tumours

Also mentioned in the text:

Bacillary angiomatosis

Reactive angioendotheliomatosis

Verrucous hemangioma

Pyogenic granuloma

Cavernous hemangioma

Angiokeratomas

"Cavernous hemangioma"Infantile Hemangioma

Venous malformation

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 YoungBoston, London, Paris, Milan, …

1982 : 1st histogenetical classification of VAMulliken 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

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

ISSVA Classification (Rome 1996)

Schobinger R. Enjolras O. Loose D. Mulliken J.B. Vanwijk R. Wassef M.

Classification and terminology of vascular malformations (round table) Rome 25/06/1996

due to active proliferation

of endothelial cells

caused by inborn localized errors

in vascular morphogenesisCombined: made of 2 or more vessel types

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

Hamburg Classificationof vascular malformations

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 or without A-V shunt

Infiltrating hemolymphatic Limited hemolymphatic

Discussed at the

7th international workshop

on vascular anomalies

Hamburg 1988

"Troncular" Malformations

Mulliken JB et al. Vascular anomalies, Oxford Univ Press 2013

Courtesy of Dr. JC Lopez Gutierrez

Courtesy of Dr. JC Lopez Gutierrez

Courtesy of Dr. JC Lopez Gutierrez Hofer Eur J Dermatol 2005

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

CCMGVMVMCMCommon VM

Cooper et al. Neurology 2008

Boon et al. J Craniofac Surg 2009

Bean Sd VM

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.