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==Classification==
==Classification==
=== Classification of Vascular Tumors ===
{{Family tree/start}}
{{Family tree | | | | | | | | | | | | | | | A01 | | | | | | | | | | | | | | | | A01= Vascular tumors}}
{{Family tree | | | | | | | |,|-|-|-|-|-|-|-|+|-|-|-|-|-|-|-|.| | | | | | | | | }}
{{Family tree | | | | | | | B01 | | | | | | B02 | | | | | | B03 | | | | | | | | B01=Benign|B02=Locally aggressive or borderline|B03=Malignant}}
{{Family tree | | | | | | |!| | | | | | | |!| | | | | | | |!| | | | | | | | | | }}
{{Family tree | | | | | | |)| C01 | | | | |)| D01 | | | | |)| E01 | | | | | | | C01=Infantile hemangioma / Hemangioma of infancy|D01=Kaposiform hemangioendothelioma<sup>*</sup>|E01=Angiosarcoma}}
{{Family tree | | | | | | |!| | | | | | | |!| | | | | | | |!| | | | | | | | | | }}
{{Family tree | | | | | | |)| C02 | | | | |)| D02 | | | | |)| E02 | | | | | | | C02=Congenital hemangioma<sup>*</sup>|D02=Retiform hemangioendothelioma|E02=Epithelioid hemangioendothelioma }}
{{Family tree | | | | | | |!| | | | | | | |!| | | | | | | |!| | | | | | | | | | }}
{{Family tree | | | | | | |)| C03 | | | | |)| D03 | | | | |`| E03 | | | | | | | C03=Tufted angioma<sup>*</sup>|D03=Papillary intralymphatic angioendothelioma (PILA), Dabska tumor|E03=Others}}
{{Family tree | | | | | | |!| | | | | | | |!| | | | | | | | | | | | | | | | | | }}
{{Family tree | | | | | | |)| C04 | | | | |)| D04 | | | | | | | | | | | | | | | C04=Spindle-cell hemangioma|D04=Composite hemangioendothelioma}}
{{Family tree | | | | | | |!| | | | | | | |!| | | | | | | | | | | | | | | | | | }}
{{Family tree | | | | | | |)| C05 | | | | |)| D05 | | | | | | | | | | | | | | | C05=Epithelioid hemangioma|D05=Pseudomyogenic hemangioendothelioma}}
{{Family tree | | | | | | |!| | | | | | | |!| | | | | | | | | | | | | | | | | | }}
{{Family tree | | | | | | |)| C06 | | | | |)| D06 | | | | | | | | | | | | | | | C06=Pyogenic granuloma (also known as lobular capillary hemangioma)|D06=Polymorphous hemangioendothelioma}}
{{Family tree | | | | | | |!| | | | | | | |!| | | | | | | | | | | | | | | | | | }}
{{Family tree | | | | | | |)| C07 | | | | |)| D07 | | | | | | | | | | | | | | | C07=Others <br>Hobnail hemangioma <br>Microvenular hemangioma <br>Anastomosing hemangioma <br>Glomeruloid hemangioma <br>Papillary hemangioma <br>Intravascular papillary endothelial hyperplasia <br>Cutaneous epithelioid angiomatous nodule <br>Acquired elastotic hemangioma <br>Littoral cell hemangioma of the spleen |D07=Hemangioendothelioma not otherwise specified}}
{{Family tree | | | | | | |!| | | | | | | |!| | | | | | | | | | | | | | | | | | }}
{{Family tree | | | | | | |`| C08 | | | | |)| D08 | | | | | | | | | | | | | | | C08=Related lesions<br>Eccrine angiomatous hamartoma<br>Reactive angioendotheliomatosis<br>Bacillary angiomatosis|D08=Kaposi sarcoma}}
{{Family tree | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | }}
{{Family tree | | | | | | | | | | | | | | |`| D09 | | | | | | | | | | | | | | | D09=Others}}
{{Family tree/end}}
<sup>*</sup>congenital hemangioma (rapidly involuting type) and tufted angioma may be associated with thrombocytopenia and/or consumptive coagulopathy in some cases.
°many experts consider tufted angioma and kaposiform hemangioendothelioma to be part of a spectrum rather than distinct entities


===Classification of Vascular Malformations===
===Classification of Vascular Malformations===

Revision as of 14:01, 20 September 2018


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hannan Javed, M.D.[2]

Overview

Vascular Anomalies
Vascular Tumors Vascular Malformations
Benign

Locally aggressive or

Borderline

Malignant

Simple Combined° of major named vessels associated with other anomalies
Capillary malformations

Lymphatic malformations

Venous malformations

Arteriovenous malformations*

Arteriovenous fistula*

Capillary venous malformation , Capillary lymphatic malformation

Lymphatic venous malformation, Capillary lymphatic venous malformation

Capillary arteriovenous malformation

Capillary lymphatic arteriovenous malformation

others

See details See list

° defined as two or more vascular malformations found in one lesion

* high flow lesions

Classification

Classification of Vascular Malformations

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Vascular malformations
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ B01 }}}
 
 
 
 
 
{{{ B02 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ B03 }}}
 
 
 
 
 
{{{ B04 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ C01 }}}
 
 
{{{ C02 }}}
 
 
{{{ C03 }}}
 
 
{{{ C04 }}}
 
 
{{{ C05 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ D01 }}}
 
 
 
{{{ E01 }}}
 
 
 
{{{ F01 }}}
 
 
 
{{{ G01 }}}
 
 
 
{{{ H01 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ D02 }}}
 
 
 
{{{ E02 }}}
 
 
 
{{{ F02 }}}
 
 
 
{{{ G02 }}}
 
 
 
{{{ H02 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ D03 }}}
 
 
 
{{{ E03 }}}
 
 
 
{{{ F03 }}}
 
 
 
{{{ G03 }}}
 
 
 
{{{ H03 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ D04 }}}
 
 
 
{{{ E04 }}}
 
 
 
{{{ F04 }}}
 
 
 
{{{ G04 }}}
 
 
 
{{{ H04 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ D05 }}}
 
 
 
{{{ E05 }}}
 
 
 
{{{ F05 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ D06 }}}
 
 
 
{{{ E06 }}}
 
 
 
{{{ F06 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ D07 }}}
 
 
 
{{{ E07 }}}
 
 
 
{{{ F07 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
{{{ F08 }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 


Anomalies of major named vessels

(also known as "channel type" or "truncal" vascular malformations)

Affect
  lymphatics
  veins
  arteries

Anomalies of

  origin
  course
  number
  length
  diameter (aplasia, hypoplasia, stenosis, ectasia / aneurysm)
  valves
  communication (AVF)
  persistence (of embryonal vessel)


Combined vascular malformations*
CM + VM capillary-venous malformation CVM
CM + LM capillary-lymphatic malformation CLM
CM + AVM capillary-arteriovenous malformation CAVM
LM + VM lymphatic-venous malformation LVM
CM + LM + VM capillary-lymphatic-venous malformation CLVM
CM + LM + AVM capillary-lymphatic-arteriovenous malformation CLAVM
CM + VM + AVM capillary-venous-arteriovenous malformation CVAVM
CM + LM + VM + AVM capillary-lymphatic-venous-arteriovenous m. CLVAVM


Vascular malformations associated with other anomalies
Klippel-Trenaunay syndrome * CM + VM +/-LM + limb overgrowth
Parkes Weber syndrome CM + AVF + limb overgrowth
Servelle-Martorell syndrome limb VM + bone undergrowth
Sturge-Weber syndrome facial + leptomeningeal CM + eye anomalies

+/-bone and/or soft tissue overgrowth

Limb CM + congenital non-progressive limb overgrowth
Maffucci syndrome VM +/-spindle-cell hemangioma + enchondroma
Macrocephaly-CM (M-CM / MCAP) *
Microcephaly-CM (MICCAP)
CLOVES syndrome * LM + VM + CM +/-AVM+ lipomatous overgrowth
Proteus syndrome CM, VM and/or LM + asymmetrical somatic overgrowth
Bannayan-Riley-Ruvalcaba sd lower lip CM + face and neck LM + asymmetry and partial/generalized overgrowth

Pathogenesis of xxx disease is due to:

  • yyy
  • zzz
    1. aaa
    2. vvv

Physiology

Medical Therapy

Naural History

Historical Perspective