Update of the ISSVA classification for vascular...
Transcript of Update of the ISSVA classification for vascular...
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.