Vascular anomalies: Difference between revisions

	Vascular Anomalies
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Latest revision as of 15:32, 23 October 2018

For information on vascular tumors, click here.

For information on vascular malformations, click here.

For information on provisionally unclassified vascular anomalies, click here.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hannan Javed, M.D.[2], Anmol Pitliya, M.B.B.S. M.D.[3]

Overview

Vascular anomalies constitute a wide array of disorders ranging from benign lesions such as infantile hemangioma to aggressive malignant tumors such as angiosarcoma. Commonly used misnomers and confusing nomenclature has often presented difficulties for accurate diagnosis and appropriate management. International Society for the Study of Vascular Anomalies (ISSVA) has now classified vascular anomalies into vascular tumors and vascular malformations with an unclassified category for lesions that show clinical and histological characteristics unique from disorders classified in vascular tumors and vascular malformations.

Classification

Vascular Anomalies
Vascular Tumors	Vascular Malformations
Vascular Tumors	Simple vascular malformation	Combined vascular malformation*	Vascular malformation of major named vessels	Vascular malformation associated with other anomalies
Benign Locally aggressive or Borderline Malignant	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 For details, Click here	For details, Click here	For details, Click here
^* Defined as two or more vascular malformations found in one lesion ^** High flow lesions
Adapted from International Society for the Study of Vascular Anomalies^[1]

Classification of Vascular Tumors

Vascular tumors

Benign

Locally aggressive or borderline

Malignant

Infantile hemangioma / Hemangioma of infancy

Kaposiform hemangioendothelioma

Angiosarcoma

Congenital hemangioma

Retiform hemangioendothelioma

Epithelioid hemangioendothelioma

Tufted angioma

Papillary intralymphatic angioendothelioma (PILA), Dabska tumor

Others

Spindle-cell hemangioma

Composite hemangioendothelioma

Epithelioid hemangioma

Pseudomyogenic hemangioendothelioma

Pyogenic granuloma (also known as lobular capillary hemangioma)

Polymorphous hemangioendothelioma

Others
• Microvenular hemangioma
• Anastomosing hemangioma
• Glomeruloid hemangioma
• Papillary hemangioma
• Intravascular papillary endothelial hyperplasia
• Cutaneous epithelioid angiomatous nodule
• Acquired elastotic hemangioma
• Littoral cell hemangioma of the spleen

Hemangioendothelioma not otherwise specified

'

Related lesions
• Eccrine angiomatous hamartoma
• Reactive angioendotheliomatosis
• Bacillary angiomatosis

Kaposi sarcoma

Others

^*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
Adapted from International Society for the Study of Vascular Anomalies^[1]

Classification of Vascular Malformations

Vascular malformations

Simple vascular malformations

Combined vascular malformations

Vascular malformations of major named vessels

Vascular malformations asscoiated with other anomalies

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	CLVM
CM + VM + AVM	Capillary-venous-arteriovenous malformation	CVAVM
CM + LM + VM + AVM	Capillary-lymphatic-venous-arteriovenous malformation	CLVAVM

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

Affect
• Lymphatics
• Veins
• Arteries
Anomalies of
• Origin
• Course
• Number
• Diameter (aplasia, hypoplasia, stenosis, ectasia / aneurysm)
• Valves
• Communication (AVF)
• Persistence (of embryonal vessel)

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

Capillary malformations (CM)

Lymphatic malformations (LM)

Venous malformations (VM)

Arteriovenous malformation (AVM)

Arteriovenous fistula

Nevus simplex / salmon patch, “angel kiss”, “stork bite”

Common (cystic) LM
• Macrocystic LM
• Microcystic LM
• Mixed cystic LM

Common VM

Sporadic

Cutaneous and/or mucosal CM (also known as “port-wine” stain)
• Nonsyndromic CM
• CM with CNS and/or ocular anomalies (Sturge-Weber syndrome)
• CM with bone and/or soft tissues overgrowth
• Diffuse CM with overgrowth (DCMO)

Generalized lymphatic anomaly (GLA)
Kaposiform lymphangiomatosis (KLA)

Familial VM cutaneo-mucosal (VMCM)

In HHT

Reticulate CM
• CM of MIC-CAP (microcephaly-capillary malformation)
• CM of MCAP (megalencephaly-capillary malformation-polymicrogyria)

LM in Gorham-Stout disease

Blue rubber bleb nevus (Bean) syndrome VM

In CM-AVM

CM of CM-AVM

Channel type LM

Glomuvenous malformation (GVM)

Others

Cutis marmorata telangiectatica congenita (CMTC)

“Acquired” progressive lymphatic anomaly (so called acquired progressive "lymphangioma")

Cerebral cavernous malformation (CCM)

Others

Primary lymphedema

Familial intraosseous vascular malformation (VMOS)

Telangiectasia
• Hereditary hemorrhagic telangiectasia (HHT)
• Others

Others

Verrucous venous malformation (formerly verrucous hemangioma)

Others

Abbreviations: CM:capillary malformation; VM:venous malformation; CVM:capillary venous malformation; LM:lymphatic malformation; CLM:capillary lymphatic malformation; AVM:arteriovenous malformation; CAVM:capillary arteriovenous malformation; LVM:lymphatic venous malformation; CLVM:capillary lymphatic venous malformation; CVAVM:capillary venous arteriovenous malformation; CLVAVM:capillary lymphatic venous arteriovenous malformation; AVF:arteriovenous fistula; CLOVES:congenital lipomatous overgrowth, vascular malformations, epidermal nevi, skeletal/scoliosis and spinal abnormalities; M-CM:macrocephaly-capillary malformation; MCAP:megalencephaly-capillary malformation-polymicrogyria; MICCAP:microcephaly-capillary malformation; CNS:central nervous system; DCMO:diffuse capillary malformation with overgrowth; CM-AVM:capillary malformation-arteriovenous malformation; CMTC:cutis marmorata telangiectatica congenita; HHT:hereditary hemorrhagic telangiectasia; GLA:generalized lymphatic anomaly; KLA:kaposiform lymphangiomatosis; VMCM:venous malformation cutaneo mucosal; GVM:glomuvenous malformation; CCM:cerebral cavernous malformation.

Adapted from International Society for the Study of Vascular Anomalies^[1]

Provisionally unclassified vascular anomalies

Provisionally unclassified vascular anomalies
Intramuscular hemangioma *
Angiokeratoma
Sinusoidal hemangioma
Acral arteriovenous "tumour"
Multifocal lymphangioendotheliomatosis with thrombocytopenia / cutaneovisceral angiomatosis with thrombocytopenia (MLT/CAT)
PTEN (type) hamartoma of soft tissue / "angiomatosis" of soft tissue (PHOST)
Fibro adipose vascular anomaly (FAVA)
^* Distinct from infantile hemangioma, from intramuscular common VM, PHOST/AST, FAVA and AVM. Some lesions may be associated with thrombocytopenia and/or consumptive coagulopathy.
Adapted from International Society for the Study of Vascular Anomalies^[1]

Abbreviations: VM:venous malformation; AVM:arteriovenous malformation; CAVM:capillary arteriovenous malformation; MLT:Multifocal lymphangioendotheliomatosis with thrombocytopenia; CAT:cutaneovisceral angiomatosis with thrombocytopenia; PHOST:PTEN hamartoma of soft tissue; FAVA:Fibro adipose vascular anomaly; AST:angiomatosis of soft tissue.

Genetics in Vascular Anomalies

Causal genes of vascular anomalies
ACVRL1	Telangiectasia, AVM and AVF of HHT2
AKT1	Proteus syndrome
BRAF	Pyogenic granuloma PG
CAMTA1	Epithelioid hemangioendothelioma EHE
CCBE1	Primary generalized lymphatic anomaly (Hennekam lymphangiectasia-lymphedema syndrome)
ELMO2	Familial intraosseous vascular malformation VMOS
ENG	Telangiectasia, AVM and AVF of HHT1
EPHB4	CM-AVM2
FLT4	Nonne-Milroy syndrome (gene also named VEGFR3)
FOS	Epithelioid hemangioma EH
FOSB	Pseudomyogenic hemangioendothelioma
FOXC2	Lymphedema-distichiasis
GATA2	Primary lymphedema with myelodysplasia
GJC2	Primary hereditary lymphedema
Glomulin	Glomuvenous malformation
GNA11	Congenital hemangioma CH CM with bone and/or soft tissue hyperplasia Diffuse CM with overgrowth DCMO
GNA14	Tufted angioma TA Pyogenic granuloma PG Kaposiform hemangioendothelioma KHE
GNAQ	Congenital hemangioma CH CM "Port-wine" stain, nonsyndromic CM CM of Sturge-Weber syndrome
IDH1	Maffucci syndrome Spindle-cell hemangioma
IDH2	Maffucci syndrome Spindle-cell hemangioma
KIF11	Microcephaly with or without chorioretinopathy, lymphedema, or mental retardation syndrome
KRIT1	Cerebral cavernous malformation CCM1
Malcavernin	Cerebral cavernous malformation CCM2
MAP2K1	Arteriovenous malformation AVM (sporadic)
MAP2K1	Ateriovenous fistula AVF (sporadic)
MAP3K3	Verrucous venous malformation (somatic)
MYC	Post radiation angiosarcoma
NPM11	Maffucci syndrome
PDCD10	Cerebral cavernous malformation CCM3
PIK3CA	Common (cystic) LM (somatic)* Common VM (somatic)* Klippel-Trenaunay syndrome* Megalencephaly-capillary malformation-polymicrogyria (MCAP)* CLOVES syndrome* CLAPO syndrome* Fibro adipose vascular anomaly FAVA
PTEN	Bannayan-Riley-Ruvalcaba syndrome PTEN (type) Hamartoma of soft tissue / "angiomatosis" of soft tissue
PTPN14	Lymphedema-choanal atresia
RASA1	CM-AVM1 Parkes Weber syndrome
SMAD4	Telangiectasia, AVM and AVF of Juvenile polyposis hemorrhagic telangiectasia JPHT
SOX18	Hypotrichosis-lymphedema-telangiectasia
STAMBP	Microcephaly-CM (MIC-CAP)
TEK (TIE2)	Common VM (somatic) Familial VM cutaneo-mucosal VMCM Blue rubber bleb nevus (Bean) syndrome (somatic)
TFE3	Epithelioid hemangioendothelioma EHE
VEGFC	Primary hereditary lymphedema
VEGFR3	Nonne-Milroy syndrome (gene also named FLT4)
^*Some of these lesions, associated with overgrowth, belong to the PIK3CA related overgrowth spectrum PROS
Adapted from International Society for the Study of Vascular Anomalies^[1]

Vascular anomalies possibly associated with platelet count / coagulation disorders


Anomalies	Hematological disorders
Tufted angioma Kaposiform hemangioendothelioma	Profound and sustained thrombocytopenia with profound hypofibrinogenemia, consumptive coagulopathy and elevated D-dimer (Kasabach-Merritt phenomenon)
Rapidly involuting congenital hemangioma	Transient mild/moderate thrombocytopenia, +/- consumptive coagulopathy and elevated D-dimer
Venous malformations / Lymphatic-venous malformations	Chronic localized intravascular coagulopathy with elevated D-dimer, +/- hypofibrinogenemia, and +/- moderate thrombocytopenia (may progress to DIC after trauma or operation)
Lymphatic malformations	Chronic localized intravascular coagulopathy with elevated D-dimer and +/- mild to moderate thrombocytopenia (consider Kaposiform lymphangiomatosis) (may progress to DIC after trauma or operation)
Multifocal lymphangioendotheliomatosis with thrombocytopenia / Cutaneovisceral angiomatosis with thrombocytopenia	Sustained, fluctuating, moderate to profound thrombocytopenia with gastrointestinal tract bleeding or pulmonary hemorrhage
Kaposiform lymphangiomatosis	Mild/moderate thrombocytopenia, +/- hypofibrinogenemia, and D-dimer elevation
Adapted from International Society for the Study of Vascular Anomalies^[1]

References

↑ ^1.0 ^1.1 ^1.2 ^1.3 ^1.4 ^1.5 "Classification | International Society for the Study of Vascular Anomalies".

[urlClassification_|_International_Society_for_the_Study_of_Vascular_Anomalies-1] 1.0 ^1.1 ^1.2 ^1.3 ^1.4 ^1.5 "Classification | International Society for the Study of Vascular Anomalies".

[1]

Vascular anomalies: Difference between revisions

Latest revision as of 15:32, 23 October 2018

Overview

Classification

Classification of Vascular Tumors

Classification of Vascular Malformations

Provisionally unclassified vascular anomalies

Genetics in Vascular Anomalies

Vascular anomalies possibly associated with platelet count / coagulation disorders

See also

References

Navigation menu

@@ Line 1: / Line 1: @@
 __NOTOC__
+'''For information on vascular tumors, [[Vascular tumor|click here]].'''
+'''For information on vascular malformations, [[Vascular malformation|click here]].'''
+'''For information on provisionally unclassified vascular anomalies, [[Provisionally unclassified vascular anomalies|click here]].'''
 {{Vascular anomalies}}
 {{CMG}}; {{AE}} {{HMHJ}}, {{Anmol}}
@@ Line 24: / Line 29: @@
 *Malignant
 | style="background:#F5F5F5;" + |
-*Capillary malformations
+*[[Capillary malformations]]
-*Lymphatic malformations
+*[[Lymphatic malformations]]
-*Venous malformations
+*[[Venous malformations]]
-*Arteriovenous malformations**
+*[[Arteriovenous malformations]]**
-*Arteriovenous fistula**
+*[[Arteriovenous fistula]]**
 | style="background:#F5F5F5;" + |
 *Capillary venous malformation
@@ Line 44: / Line 49: @@
 <sup>*</sup> Defined as two or more vascular malformations found in one lesion<br>
 <sup>**</sup> High flow lesions
+|-
+| colspan="5" style="background:#7d7d7d; color: #FFFFFF;" + |<small>'''Adapted from International Society for the Study of Vascular Anomalies'''<ref name="urlClassification | International Society for the Study of Vascular Anomalies">{{cite web |url=http://www.issva.org/classification |title=Classification &#124; International Society for the Study of Vascular Anomalies |format= |work= |accessdate=}}</ref></small>
 |}
-=== Classification of Vascular Tumors ===
+===[[Classification of Vascular Tumors]]===
 {{Family tree/start}}
-{{Family tree | | | | | | | | | | | | | | | A01 | | | | | | | | | | | | | | | | A01= '''Vascular tumors'''}}
+{{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 | | | | | | |)| C01 | | | | |)| D01 | | | | |)| E01 | | | | | | | C01=[[Infantile hemangioma]] / [[Hemangioma of infancy]]|D01=[[Kaposiform hemangioendothelioma]]|E01=[[Angiosarcoma]]}}
 {{Family tree | | | | | | |!| | | | | | | |!| | | | | | | |!| | | | | | | | | | }}
-{{Family tree | | | | | | |)| C02 | | | | |)| D02 | | | | |)| E02 | | | | | | | C02=Congenital hemangioma<sup>*</sup>|D02=Retiform hemangioendothelioma|E02=Epithelioid hemangioendothelioma }}
+{{Family tree | | | | | | |)| C02 | | | | |)| D02 | | | | |)| E02 | | | | | | | C02=[[Congenital hemangioma]]|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 | | | | | | |)| C03 | | | | |)| D03 | | | | |`| E03 | | | | | | | C03=[[Tufted angioma]]|D03=[[Papillary intralymphatic angioendothelioma]] ([[PILA]]), [[Dabska tumor]]|E03=Others}}
 {{Family tree | | | | | | |!| | | | | | | |!| | | | | | | | | | | | | | | | | | }}
-{{Family tree | | | | | | |)| C04 | | | | |)| D04 | | | | | | | | | | | | | | | C04=Spindle-cell hemangioma|D04=Composite hemangioendothelioma}}
+{{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 | | | | | | |)| 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 | | | | | | |)| C06 | | | | |)| D06 | | | | | | | | | | | | | | | C06=[[Pyogenic granuloma]] (also known as [[lobular capillary hemangioma]])|D06=[[Polymorphous hemangioendothelioma]]}}
 {{Family tree | | | | | | |!| | | | | | | |!| | | | | | | | | | | | | | | | | | }}
 {{Family tree | | | | | | |)| C07 | | | | |)| D07 | | | | | | | | | | | | | | | | |C07=<table>
 <tr><td>Others</td></tr>
-<tr><td>• Microvenular hemangioma<td></tr>
+<tr><td>• [[Microvenular hemangioma]]<td></tr>
-<tr><td>• Anastomosing hemangioma<td></tr>
+<tr><td>• [[Anastomosing hemangioma]]<td></tr>
-<tr><td>• Glomeruloid hemangioma<td></tr>
+<tr><td>• [[Glomeruloid hemangioma]]<td></tr>
-<tr><td>• Papillary hemangioma<td></tr>
+<tr><td>• [[Papillary hemangioma]]<td></tr>
-<tr><td>• Intravascular papillary endothelial hyperplasia<td></tr>
+<tr><td>• [[Intravascular papillary endothelial hyperplasia]]<td></tr>
-<tr><td>• Cutaneous epithelioid angiomatous nodule<td></tr>
+<tr><td>• [[Cutaneous epithelioid angiomatous nodule]]<td></tr>
-<tr><td>• Acquired elastotic hemangioma<td></tr>
+<tr><td>• [[Acquired elastotic hemangioma]]<td></tr>
-<tr><td>• Littoral cell hemangioma of the spleen<td></tr>
+<tr><td>• [[Littoral cell hemangioma of the spleen]]<td></tr>
 </table> |D07=Hemangioendothelioma not otherwise specified}}
 {{Family tree | | | | | | |!| | | | | | | |!| | | | | | | | | | | | | | | | | | }}
 {{Family tree | | | | | | |`| C08 | | | | |)| D08 | | | | | | | | | | | | | | | |C08=<table>
 <tr><td>Related lesions</td></tr>
-<tr><td>• Eccrine angiomatous hamartoma<td></tr>
+<tr><td>• [[Eccrine angiomatous hamartoma]]<td></tr>
-<tr><td>• Reactive angioendotheliomatosis<td></tr>
+<tr><td>• [[Reactive angioendotheliomatosis]]<td></tr>
-<tr><td>• Bacillary angiomatosis<td></tr>'
+<tr><td>• [[Bacillary angiomatosis]]<td></tr>'
-</table> |D08=Kaposi sarcoma}}
+</table> |D08=[[Kaposi sarcoma]]}}
 {{Family tree | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | }}
 {{Family tree | | | | | | | | | | | | | | |`| D09 | | | | | | | | | | | | | | | D09=Others}}
@@ Line 89: / Line 95: @@
-<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
+<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<br>
+'''Adapted from International Society for the Study of Vascular Anomalies'''<ref name="urlClassification | International Society for the Study of Vascular Anomalies">{{cite web |url=http://www.issva.org/classification |title=Classification &#124; International Society for the Study of Vascular Anomalies |format= |work= |accessdate=}}</ref></small>
 ===Classification of Vascular Malformations===
 {{Family tree/start}}
-{{Family tree | | | | | | | A01 | | | | | | | |A01= '''Vascular malformations'''}}
+{{Family tree | | | | | | | A01 | | | | | | | |A01= '''[[Vascular malformations]]'''}}
 {{Family tree | |,|-|-|-|v|-|^|-|v|-|-|-|.| | |}}
-{{Family tree | B01 | | B02 | | B03 | | B04 | |B01='''''Simple vascular malformations'''''|B02='''''Combined vascular malformations'''''|B03='''''Vascular malformations of major named vessels'''''|B04='''''Vascular malformations asscoiated with other anomalies'''''}}
+{{Family tree | B01 | | B02 | | B03 | | B04 | |B01='''''[[Simple vascular malformations]]'''''|B02='''''[[Combined vascular malformations]]'''''|B03='''''Vascular malformations of major named vessels'''''|B04='''''[[Vascular malformations asscoiated with other anomalies]]'''''}}
 {{Family tree | |!| | | |!| | | |!| | | |!| | |}}
-{{Family tree | |!| | | I02 | | I03 | | I04 | |I02=*<table class="wikitable">
+{{Family tree | |!| | | I02 | | I03 | | I04 | |I02=<table class="wikitable">
 <tr><td>'''CM + VM'''</td><td>Capillary-venous malformation</td><td>CVM</td></tr>
 <tr><td>'''CM + LM'''</td><td>Capillary-lymphatic malformation</td><td>CLM</td></tr>
@@ Line 108: / Line 115: @@
 <tr><td>'''CM + LM + VM + AVM'''</td><td>Capillary-lymphatic-venous-arteriovenous malformation</td><td>CLVAVM</td></tr>
 </table>|I03=<br>(also known as "channel type" or "truncal" vascular malformations)<br><table>
-<tr><td>Affect</td></tr>
+<tr><td>'''Affect'''</td></tr>
-<tr><td>• Lymphatics<td></tr>
+<tr><td>• [[Lymphatics]]<td></tr>
-<tr><td>• Veins</tr>
+<tr><td>• [[Veins]]</tr>
-<tr><td>• Arteries</tr>
+<tr><td>• [[Arteries]]</tr>
-<tr><td>Anomalies of</td></tr>
+<tr><td>'''Anomalies of'''</td></tr>
 <tr><td>• Origin<td></tr>
 <tr><td>• Course<td></tr>
@@ Line 121: / Line 128: @@
 <tr><td>• Persistence (of<br>embryonal vessel)<td></tr>
 </table>|I04=<table class="wikitable">
-<tr><td>'''Klippel-Trenaunay syndrome'''</td><td>CM + VM +/-LM + limb overgrowth</td></tr>
+<tr><td>'''[[Klippel-Trenaunay syndrome]]'''</td><td>CM + VM +/-LM + limb overgrowth</td></tr>
-<tr><td>'''Parke's Weber syndrome'''</td><td>CM + AVF + limb overgrowth</td></tr>
+<tr><td>'''[[Parke's Weber syndrome]]'''</td><td>CM + AVF + limb overgrowth</td></tr>
-<tr><td>'''Servelle-Martorell syndrome'''</td><td>Limb VM + bone undergrowth</td></tr>
+<tr><td>'''[[Servelle-Martorell syndrome]]'''</td><td>Limb VM + bone undergrowth</td></tr>
-<tr><td>'''Sturge-Weber syndrome'''</td><td>Facial + leptomeningeal CM + eye anomalies +/-bone and/or soft tissue overgrowth</td></tr>
+<tr><td>'''[[Sturge-Weber syndrome]]'''</td><td>Facial + leptomeningeal CM + eye anomalies +/-bone and/or soft tissue overgrowth</td></tr>
-<tr><td>'''Maffucci syndrome'''</td><td>VM +/-spindle-cell hemangioma + enchondroma</td></tr>
+<tr><td>'''[[Maffucci syndrome]]'''</td><td>VM +/-spindle-cell hemangioma + enchondroma</td></tr>
-<tr><td>'''CLOVES syndrome'''</td><td>LM + VM + CM +/-AVM+ lipomatous overgrowth</td></tr>
+<tr><td>'''[[CLOVES syndrome]]'''</td><td>LM + VM + CM +/-AVM+ lipomatous overgrowth</td></tr>
-<tr><td>'''Proteus syndrome'''</td><td>CM, VM and/or LM + asymmetrical somatic overgrowth</td></tr>
+<tr><td>'''[[Proteus syndrome]]'''</td><td>CM, VM and/or LM + asymmetrical somatic overgrowth</td></tr>
-<tr><td>'''Bannayan-Riley-Ruvalcaba sd'''</td><td>lower lip CM + face and neck LM + asymmetry and partial/generalized overgrowth</td></tr>
+<tr><td>'''[[Bannayan-Riley-Ruvalcaba syndrome]]'''</td><td>lower lip CM + face and neck LM + asymmetry and partial/generalized overgrowth</td></tr>
 <tr><td colspan="2">'''Limb CM + congenital non-progressive limb overgrowth'''</td></tr>
 <tr><td colspan="2">'''Macrocephaly-CM (M-CM / MCAP)'''</td></tr>
@@ Line 136: / Line 143: @@
 {{Family tree | |!| | |}}
 {{Family tree | |)|-|-|-|v|-|-|-|v|-|-|-|v|-|-|-|.| | |}}
-{{Family tree | | C01 | | C02 | | C03 | | C04 | | C05 | |C01=Capillary malformations (CM)|C02=Lymphatic malformations (LM)|C03=Venous malformations (VM)|C04=Arteriovenous<br>malformation(AVM)|C05=Arteriovenous fistula}}
+{{Family tree | | C01 | | C02 | | C03 | | C04 | | C05 | |C01=[[Capillary malformations]] (CM)|C02=[[Lymphatic malformations]] (LM)|C03=[[Venous malformations]] (VM)|C04=[[Arteriovenous malformation]] (AVM)|C05=[[Arteriovenous fistula]]}}
 {{Family tree | |!| | | |!| | | |!| | | |!| | | |!| | | |}}
-{{Family tree | |)| D01 |)| E01 |)| F01 |)| G01 |)| H01 |D01=Nevus simplex / salmon patch, “angel kiss”, “stork bite”|E01=<table>
+{{Family tree | |)| D01 |)| E01 |)| F01 |)| G01 |)| H01 |D01=[[Nevus simplex]] / [[salmon patch]], “[[angel kiss]]”, “[[stork bite]]”|E01=<table>
-<tr><td>Common (cystic) LM </td></tr>
+<tr><td>[[Common (cystic) LM]] </td></tr>
-<tr><td>• Macrocystic  LM<td></tr>
+<tr><td>• [[Macrocystic  LM]]<td></tr>
-<tr><td>• Microcystic LM<td></tr>
+<tr><td>• [[Microcystic LM]]<td></tr>
-<tr><td>• Mixed cystic LM<td></tr>
+<tr><td>• [[Mixed cystic LM]]<td></tr>
-</table>|F01=Common VM|G01=Sporadic|H01=Sporadic}}
+</table>|F01=[[Common VM]]|G01=Sporadic|H01=Sporadic}}
 {{Family tree | |!| | | |!| | | |!| | | |!| | | |!| | | |}}
 {{Family tree | |)| D02 |)| E02 |)| F02 |)| G02 |)| H02 |D02=<table>
-<tr><td>Cutaneous and/or mucosal CM (also known as “port-wine” stain) </td></tr>
+<tr><td>[[Cutaneous and/or mucosal CM]] (also known as [[“port-wine” stain]]) </td></tr>
-<tr><td>• Nonsyndromic CM<td></tr>
+<tr><td>• [[Nonsyndromic CM]]<td></tr>
-<tr><td>• CM with CNS and/or ocular anomalies (Sturge-Weber syndrome)<td></tr>
+<tr><td>• CM with CNS and/or ocular anomalies ([[Sturge-Weber syndrome]])<td></tr>
 <tr><td>• CM with bone and/or soft tissues overgrowth <td></tr>
-<tr><td>• Diffuse CM with overgrowth (DCMO) <td></tr>
+<tr><td>• [[Diffuse CM with overgrowth]] ([[DCMO]]) <td></tr>
-</table>|E02=Generalized lymphatic anomaly (GLA)<br>Kaposiform lymphangiomatosis (KLA)|F02=Familial VM cutaneo-mucosal (VMCM)|G02=In HHT|H02=In HHT|}}
+</table>|E02=[[Generalized lymphatic anomaly]] ([[GLA]])<br>[[Kaposiform lymphangiomatosis]] ([[KLA]])|F02=[[Familial VM cutaneo-mucosal]] ([[VMCM]])|G02=In [[HHT]]|H02=In [[HHT]]|}}
 {{Family tree | |!| | | |!| | | |!| | | |!| | | |!| | | |}}
 {{Family tree | |)| D03 |)| E03 |)| F03 |)| G03 |)| H03 |D03=<table>
-<tr><td>Reticulate CM </td></tr>
+<tr><td>[[Reticulate CM]] </td></tr>
 <tr><td>• CM of MIC-CAP (microcephaly-capillary malformation)<td></tr>
 <tr><td>• CM of MCAP (megalencephaly-capillary malformation-polymicrogyria)<td></tr>
 </table>
-|E03=LM in Gorham-Stout disease|F03=Blue rubber bleb nevus (Bean) syndrome VM|G03=In CM-AVM|H03=In CM-AVM|}}
+|E03=LM in [[Gorham-Stout disease]]|F03=[[Blue rubber bleb nevus (Bean) syndrome]] VM|G03=In [[CM-AVM]]|H03=In [[CM-AVM]]|}}
 {{Family tree | |!| | | |!| | | |!| | | |!| | | |!| | | | |}}
-{{Family tree | |)| D04 |)| E04 |)| F04 |`| G04 |`| H04 |D04=CM of CM-AVM|E04=Channel type LM|F04=Glomuvenous malformation (GVM)|G04=Others|H04=Others|}}
+{{Family tree | |)| D04 |)| E04 |)| F04 |`| G04 |`| H04 |D04=[[CM of CM-AVM]]|E04=Channel type LM|F04=[[Glomuvenous malformation]] ([[GVM]])|G04=Others|H04=Others|}}
 {{Family tree | |!| | | |!| | | |!| | | | | | | | | | | |}}
-{{Family tree | |)| D05 |)| E05 |)| F05 | | | | | | | | |D05=Cutis marmorata telangiectatica congenita (CMTC)|E05=“Acquired” progressive lymphatic anomaly (so called acquired progressive "lymphangioma")|F05=Cerebral cavernous malformation (CCM) |}}
+{{Family tree | |)| D05 |)| E05 |)| F05 | | | | | | | | |D05=[[Cutis marmorata telangiectatica congenita]] ([[CMTC]])|E05=[[“Acquired” progressive lymphatic anomaly]] (so called [[acquired progressive "lymphangioma"]])|F05=[[Cerebral cavernous malformation]] ([[CCM]]) |}}
 {{Family tree | |!| | | |!| | | |!| | | | | | | | | | | |}}
-{{Family tree | |)| D06 |)| E06 |)| F06 | | | | | | | | |D06=Others|E06=Primary lymphedema |F06=Familial intraosseous vascular malformation (VMOS)|}}
+{{Family tree | |)| D06 |)| E06 |)| F06 | | | | | | | | |D06=Others|E06=[[Primary lymphedema]] |F06=[[Familial intraosseous vascular malformation]] ([[VMOS]])|}}
 {{Family tree | |!| | | |!| | | |!| | | | | | | | | | | |}}
 {{Family tree | |`| D07 |`| E07 |)| F07 | | | | | | | | |D07=<table>
-<tr><td>Telangiectasia </td></tr>
+<tr><td>[[Telangiectasia]] </td></tr>
-<tr><td>• Hereditary hemorrhagic telangiectasia (HHT) <td></tr>
+<tr><td>• [[Hereditary hemorrhagic telangiectasia]] (HHT) <td></tr>
 <tr><td>• Others<td></tr>
-</table>|E07=Others|F07=Verrucous venous malformation (formerly verrucous hemangioma)|}}
+</table>|E07=Others|F07=[[Verrucous venous malformation]] (formerly [[verrucous hemangioma]])|}}
 {{Family tree | | | | | | | | | |!| | | | | | | | | | | |}}
 {{Family tree | | | | | | | | | |`| F08 | | | | | | | | |F08=Others|}}
 {{Family tree/end}}
+<small>'''Abbreviations:''' CM:capillary malformation; VM:venous malformation; CVM:capillary venous malformation; LM:lymphatic malformation; CLM:capillary lymphatic malformation; AVM:arteriovenous malformation; CAVM:capillary arteriovenous malformation; LVM:lymphatic venous malformation; CLVM:capillary lymphatic venous malformation; CVAVM:capillary venous arteriovenous malformation; CLVAVM:capillary lymphatic venous arteriovenous malformation; AVF:arteriovenous fistula; CLOVES:congenital lipomatous overgrowth, vascular malformations, epidermal nevi, skeletal/scoliosis and spinal abnormalities; M-CM:macrocephaly-capillary malformation; MCAP:megalencephaly-capillary malformation-polymicrogyria; MICCAP:microcephaly-capillary malformation; CNS:central nervous system; DCMO:diffuse capillary malformation with overgrowth; CM-AVM:capillary malformation-arteriovenous malformation; CMTC:cutis marmorata telangiectatica congenita; HHT:hereditary hemorrhagic telangiectasia; GLA:generalized lymphatic anomaly; KLA:kaposiform lymphangiomatosis; VMCM:venous malformation cutaneo mucosal; GVM:glomuvenous malformation; CCM:cerebral cavernous malformation.</small>
+'''Adapted from International Society for the Study of Vascular Anomalies'''<ref name="urlClassification | International Society for the Study of Vascular Anomalies">{{cite web |url=http://www.issva.org/classification |title=Classification &#124; International Society for the Study of Vascular Anomalies |format= |work= |accessdate=}}</ref></small>
 ===Provisionally unclassified vascular anomalies===
@@ Line 179: / Line 189: @@
 ! style="background:#4479BA; color: #FFFFFF;" align="center" + |Provisionally unclassified vascular anomalies
 |-
-|Intramuscular hemangioma *
+|[[Intramuscular hemangioma]] *
+|-
+|[[Angiokeratoma]]
 |-
-|Angiokeratoma
+|[[Sinusoidal hemangioma]]
 |-
-|Sinusoidal hemangioma
+|[[Acral arteriovenous "tumour"]]
 |-
-|Acral arteriovenous "tumour"
+|[[Multifocal lymphangioendotheliomatosis with thrombocytopenia / cutaneovisceral angiomatosis with thrombocytopenia (MLT/CAT)]]
 |-
-|Multifocal lymphangioendotheliomatosis with thrombocytopenia / cutaneovisceral<br>angiomatosis with thrombocytopenia (MLT/CAT)
+|[[PTEN (type) hamartoma of soft tissue]] / [["angiomatosis" of soft tissue]]
+([[PHOST]])
 |-
-|PTEN (type) hamartoma of soft tissue / "angiomatosis" of soft tissue
+|[[Fibro adipose vascular anomaly]] ([[FAVA]])
-(PHOST)
 |-
-|Fibro adipose vascular anomaly (FAVA)
+| style="background:#DCDCDC;" + |<sup>*</sup> Distinct from [[infantile hemangioma]], from intramuscular common VM, [[PHOST]]/[[AST]], [[FAVA]] and [[AVM]].<br>Some lesions may be associated with thrombocytopenia and/or consumptive coagulopathy.
 |-
-| style="background:#DCDCDC;" + |<sup>*</sup> Distinct from infantile hemangioma, from intramuscular common VM, PHOST/AST, FAVA and AVM.<br>Some lesions may be associated with thrombocytopenia and/or consumptive coagulopathy.
+| colspan="2" style="background:#7d7d7d; color: #FFFFFF;" + |<small>'''Adapted from International Society for the Study of Vascular Anomalies'''<ref name="urlClassification | International Society for the Study of Vascular Anomalies">{{cite web |url=http://www.issva.org/classification |title=Classification &#124; International Society for the Study of Vascular Anomalies |format= |work= |accessdate=}}</ref></small>
 |}
-== Genetics in Vascular Anomalies ==
+<small>'''Abbreviations:''' VM:venous malformation; AVM:arteriovenous malformation; CAVM:capillary arteriovenous malformation; MLT:Multifocal lymphangioendotheliomatosis with thrombocytopenia; CAT:cutaneovisceral angiomatosis with thrombocytopenia; PHOST:PTEN hamartoma of soft tissue; FAVA:Fibro adipose vascular anomaly; AST:angiomatosis of soft tissue.</small>
+==Genetics in Vascular Anomalies==
 {| class="wikitable"
-|+
 ! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" + |Causal genes of vascular anomalies
 |-
 | style="background:#DCDCDC;" align="center" + |ACVRL1
-|Telangiectasia, AVM and AVF of HHT2
+|[[Telangiectasia]], [[AVM]] and [[AVF]] of [[HHT]]2
 |-
 | style="background:#DCDCDC;" align="center" + |AKT1
-|Proteus syndrome
+|[[Proteus syndrome]]
 |-
 | style="background:#DCDCDC;" align="center" + |BRAF
-|Pyogenic granuloma PG
+|[[Pyogenic granuloma]] PG
 |-
 | style="background:#DCDCDC" align="center" + |CAMTA1
-|Epithelioid hemangioendothelioma EHE
+|[[Epithelioid hemangioendothelioma]] EHE
 |-
 | style="background:#DCDCDC" align="center" + |CCBE1
-|Primary generalized lymphatic anomaly (Hennekam lymphangiectasia-lymphedema syndrome)
+|[[Primary generalized lymphatic anomaly]] (Hennekam lymphangiectasia-lymphedema syndrome)
 |-
 | style="background:#DCDCDC;" align="center" + |ELMO2
-|Familial intraosseous vascular malformation VMOS
+|[[Familial intraosseous vascular malformation]] [[VMOS]]
 |-
 | style="background:#DCDCDC;" align="center" + |ENG
-|Telangiectasia, AVM and AVF of HHT1
+|[[Telangiectasia]], [[AVM]] and [[AVF]] of [[HHT]]1
 |-
 | style="background:#DCDCDC;" align="center" + |EPHB4
@@ Line 227: / Line 240: @@
 |-
 | style="background:#DCDCDC;" align="center" + |FLT4
-|Nonne-Milroy syndrome (gene also named VEGFR3)
+|[[Nonne-Milroy syndrome]] (gene also named VEGFR3)
 |-
 | style="background:#DCDCDC;" align="center" + |FOS
-|Epithelioid hemangioma EH
+|[[Epithelioid hemangioma]] EH
 |-
 | style="background:#DCDCDC;" align="center" + |FOSB
-|Pseudomyogenic hemangioendothelioma
+|[[Pseudomyogenic hemangioendothelioma]]
 |-
 | style="background:#DCDCDC;" align="center" + |FOXC2
-|Lymphedema-distichiasis
+|[[Lymphedema-distichiasis]]
 |-
 | style="background:#DCDCDC;" align="center" + |GATA2
-|Primary lymphedema with myelodysplasia
+|[[Primary lymphedema with myelodysplasia]]
 |-
 | style="background:#DCDCDC;" align="center" + |GJC2
-|Primary hereditary lymphedema
+|[[Primary hereditary lymphedema]]
 |-
 | style="background:#DCDCDC;" align="center" + |Glomulin
-|Glomuvenous malformation
+|[[Glomuvenous malformation]]
 |-
 | style="background:#DCDCDC;" align="center" + |GNA11
-|Congenital hemangioma CH
+|[[Congenital hemangioma]] CH
 CM with bone and/or soft tissue hyperplasia
-Diffuse CM with overgrowth DCMO
+[[Diffuse CM with overgrowth]] [[DCMO]]
 |-
 | style="background:#DCDCDC;" align="center" + |GNA14
-|Tufted angioma TA
+|[[Tufted angioma]] TA
-Pyogenic granuloma PG
+[[Pyogenic granuloma]] PG
-Kaposiform hemangioendothelioma KHE
+[[Kaposiform hemangioendothelioma]] KHE
 |-
 | style="background:#DCDCDC;" align="center" + |GNAQ
-|Congenital hemangioma CH
+|[[Congenital hemangioma]] CH
-CM "Port-wine" stain, nonsyndromic CM
+CM [["Port-wine" stain]], [[nonsyndromic CM]]
-CM of Sturge-Weber syndrome
+CM of [[Sturge-Weber syndrome]]
 |-
 | style="background:#DCDCDC;" align="center" + |IDH1
-|Maffucci syndrome
+|[[Maffucci syndrome]]
-Spindle-cell hemangioma
+[[Spindle-cell hemangioma]]
 |-
 | style="background:#DCDCDC;" align="center" + |IDH2
-|Maffucci syndrome
+|[[Maffucci syndrome]]
-Spindle-cell hemangioma
+[[Spindle-cell hemangioma]]
 |-
 | style="background:#DCDCDC;" align="center" + |KIF11
@@ Line 277: / Line 290: @@
 |-
 | style="background:#DCDCDC;" align="center" + |KRIT1
-|Cerebral cavernous malformation CCM1
+|[[Cerebral cavernous malformation]] [[CCM]]1
 |-
 | style="background:#DCDCDC;" align="center" + |Malcavernin
-|Cerebral cavernous malformation CCM2
+|[[Cerebral cavernous malformation]] [[CCM]]2
 |-
 | style="background:#DCDCDC;" align="center" + |MAP2K1
-|Arteriovenous malformation AVM (sporadic)
+|[[Arteriovenous malformation]] AVM (sporadic)
 |-
 | style="background:#DCDCDC;" align="center" + |MAP2K1
-|Ateriovenous fistula AVF (sporadic)
+|[[Ateriovenous fistula]] [[AVF]] (sporadic)
 |-
 | style="background:#DCDCDC;" align="center" + |MAP3K3
-|Verrucous venous malformation (somatic)
+|[[Verrucous venous malformation]] (somatic)
 |-
 | style="background:#DCDCDC;" align="center" + |MYC
-|Post radiation angiosarcoma
+|Post radiation [[angiosarcoma]]
 |-
 | style="background:#DCDCDC;" align="center" + |NPM11
-|Maffucci syndrome
+|[[Maffucci syndrome]]
 |-
 | style="background:#DCDCDC;" align="center" + |PDCD10
-|Cerebral cavernous malformation CCM3
+|[[Cerebral cavernous malformation]] [[CCM]]3
 |-
 | style="background:#DCDCDC;" align="center" + |PIK3CA
-|Common (cystic) LM (somatic)*
+|[[Common (cystic) LM]] (somatic)*
-Common VM (somatic)*
+[[Common VM]] (somatic)*
-Klippel-Trenaunay syndrome*
+[[Klippel-Trenaunay syndrome]]*
 Megalencephaly-capillary malformation-polymicrogyria (MCAP)*
-CLOVES syndrome*
+[[CLOVES syndrome]]*
-CLAPO syndrome*
+[[CLAPO syndrome]]*
-Fibro adipose vascular anomaly FAVA
+[[Fibro adipose vascular anomaly]] [[FAVA]]
 |-
 | style="background:#DCDCDC;" align="center" + |PTEN
-|Bannayan-Riley-Ruvalcaba syndrome
+|[[Bannayan-Riley-Ruvalcaba syndrome]]
-PTEN (type) Hamartoma of soft tissue / "angiomatosis" of soft  tissue
+PTEN (type) Hamartoma of soft tissue / [["angiomatosis" of soft  tissue]]
 |-
 | style="background:#DCDCDC;" align="center" + |PTPN14
-|Lymphedema-choanal atresia
+|[[Lymphedema-choanal atresia]]
 |-
 | style="background:#DCDCDC;" align="center" + |RASA1
 |CM-AVM1
-Parkes Weber syndrome
+[[Parkes Weber syndrome]]
 |-
 | style="background:#DCDCDC;" align="center" + |SMAD4
-|Telangiectasia, AVM and AVF of Juvenile polyposis hemorrhagic telangiectasia JPHT
+|[[Telangiectasia]], [[AVM]] and [[AVF]] of [[Juvenile polyposis hemorrhagic telangiectasia]] JPHT
 |-
 | style="background:#DCDCDC;" align="center" + |SOX18
-|Hypotrichosis-lymphedema-telangiectasia
+|[[Hypotrichosis-lymphedema-telangiectasia]]
 |-
 | style="background:#DCDCDC;" align="center" + |STAMBP
@@ Line 335: / Line 348: @@
 |-
 | style="background:#DCDCDC;" align="center" + |TEK (TIE2)
-|Common VM (somatic)
+|[[Common VM]] (somatic)
-Familial VM cutaneo-mucosal VMCM
+[[Familial VM cutaneo-mucosal]] [[VMCM]]
-Blue rubber bleb nevus (Bean) syndrome (somatic)
+[[Blue rubber bleb nevus (Bean) syndrome]] (somatic)
 |-
 | style="background:#DCDCDC;" align="center" + |TFE3
-|Epithelioid hemangioendothelioma EHE
+|[[Epithelioid hemangioendothelioma]] EHE
 |-
 | style="background:#DCDCDC;" align="center" + |VEGFC
-|Primary hereditary lymphedema
+|[[Primary hereditary lymphedema]]
 |-
 | style="background:#DCDCDC;" align="center" + |VEGFR3
-|Nonne-Milroy syndrome (gene also named FLT4)
+|[[Nonne-Milroy syndrome]] (gene also named FLT4)
 |-
 | colspan="2" style="background:#DCDCDC;" + |<sup>*</sup>Some of these lesions, associated with overgrowth, belong to the PIK3CA related overgrowth spectrum PROS
+|-
+| colspan="2" style="background:#7d7d7d; color: #FFFFFF;" + |<small>'''Adapted from International Society for the Study of Vascular Anomalies'''<ref name="urlClassification | International Society for the Study of Vascular Anomalies">{{cite web |url=http://www.issva.org/classification |title=Classification &#124; International Society for the Study of Vascular Anomalies |format= |work= |accessdate=}}</ref></small>
 |}
-==Provisionally unclassified vascular anomalies==
+==Vascular anomalies possibly associated with platelet count / coagulation disorders==
-===Intramuscular hemangioma===
+{| class="wikitable"
-* Characterized by [[benign]] proliferation of [[vascular]] channels. Majority of [[lesions]] occur in [[subcutaneous]] [[adipose]] [[tissues]], followed by [[muscles]]. [[Thigh]] and [[calf]] are most common sites of occurrence. Majority of the [[lesions]] are [[asymptomatic]]. Typical clinical presentation includes chronic pain and swelling that both may increase with exercise of affected [[muscle]] due to increased [[blood]] flow. Other clinical manifestations may include pulsations, discoloration over the [[lesion]], [[lesion]] enlargement when in dependent position, increased temperature, [[muscle contracture]], tenderness, and [[muscle]] weakness and fatigue.<ref name="pmid25028288">{{cite journal |vauthors=Wang CS, Wu PK, Chiou HJ, Chen CF, Chen WM, Liu CL, Chen TH |title=Nonpalpable intramuscular hemangioma treated with hookwire localization and excision |journal=J Chin Med Assoc |volume=77 |issue=8 |pages=426–9 |date=August 2014 |pmid=25028288 |doi=10.1016/j.jcma.2014.02.017 |url=}}</ref><ref name="pmid25728120">{{cite journal |vauthors=Doddanna SJ, Dawar G, Rallan NS, Agarwal M |title=Intramuscular cavernous hemangioma: a rare entity in the buccinator muscle |journal=Indian J Dent Res |volume=25 |issue=6 |pages=813–5 |date=2014 |pmid=25728120 |doi=10.4103/0970-9290.152211 |url=}}</ref><ref name="pmid23845293">{{cite journal |vauthors=Righini CA, Berta E, Atallah I |title=Intramuscular cavernous hemangioma arising from the masseter muscle |journal=Eur Ann Otorhinolaryngol Head Neck Dis |volume=131 |issue=1 |pages=57–9 |date=February 2014 |pmid=23845293 |doi=10.1016/j.anorl.2013.03.003 |url=}}</ref><ref name="pmid25590509">{{cite journal |vauthors=Alami B, Lamrani Y, Addou O, Boubbou M, Kamaoui I, Maaroufi M, Sqalli N, Tizniti S |title=Presumptive intramuscular hemangioma of the masseter muscle |journal=Am J Case Rep |volume=16 |issue= |pages=16–9 |date=January 2015 |pmid=25590509 |pmc=4298281 |doi=10.12659/AJCR.890776 |url=}}</ref><ref name="pmid15155443">{{cite journal |vauthors=Brown RA, Crichton K, Malouf GM |title=Intramuscular haemangioma of the thigh in a basketball player |journal=Br J Sports Med |volume=38 |issue=3 |pages=346–8 |date=June 2004 |pmid=15155443 |pmc=1724833 |doi= |url=}}</ref><ref name="pmid28507959">{{cite journal |vauthors=Patnaik S, Kumar P, Nayak B, Mohapatra N |title=Intramuscular Arteriovenous Hemangioma of Thigh: A Case Report and Review of Literature |journal=J Orthop Case Rep |volume=6 |issue=5 |pages=20–23 |date=2016 |pmid=28507959 |pmc=5404154 |doi=10.13107/jocr.2250-0685.612 |url=}}</ref>
+|+
-* Intramuscular hemangiomas may be associated with [[Kasabach-Merritt syndrome]] characterized by [[thrombocytopenia]] and/or consumptive [[coagulopathy]]. This [[lesion]] may also lead to functional impairment, [[congestive cardiac failure]] due to arteriovenous shunting, pressure symptoms, [[skin]] [[necrosis]] and may also erode [[bone]].<ref name="pmid15155443" />
+!style="background:#4479BA; color: #FFFFFF;" align="center" + |Anomalies
-* [[Etiology]] and [[pathophysiology]] are not clearly defined but majority of the [[lesions]] are congenital while a one fifth may be associated with trauma.<ref name="pmid24427416">{{cite journal |vauthors=Wierzbicki JM, Henderson JH, Scarborough MT, Bush CH, Reith JD, Clugston JR |title=Intramuscular hemangiomas |journal=Sports Health |volume=5 |issue=5 |pages=448–54 |date=September 2013 |pmid=24427416 |pmc=3752185 |doi=10.1177/1941738112470910 |url=}}</ref>
+!style="background:#4479BA; color: #FFFFFF;" align="center" + |Hematological disorders
-* [[MRI]] is the [[diagnostic]] study of choice although [[X-RAY]] and [[ultrasound]] may be used as initial studies. Treatment is generally not indicated for [[asymptomatic]] [[lesions]]. Management options for [[symptomatic]], complicated [[lesions]] and for cosmetic reasons may include [[laser ablation]], systemic [[corticosteroids]], [[cryotherapy]], [[embolization]], [[radiation]], compression [[sclerotherapy]], and [[surgical excision]] although surgical excision is usually treatment of choice in majority of the cases.<ref name="pmid24427416" /><ref name="pmid15155443" /><ref name="pmid28507959" /><ref name="pmid25028288" /><ref name="pmid25728120" /><ref name="pmid23845293" /><ref name="pmid25590509" />
+|-
+|[[Tufted angioma]]
+[[Kaposiform hemangioendothelioma]]
+|Profound and sustained thrombocytopenia with profound
+hypofibrinogenemia, consumptive coagulopathy and
-===Angiokeratoma===
+elevated D-dimer (Kasabach-Merritt phenomenon)
-* A [[muco-cutaneous]] [[vascular]] [[lesion]] with wart-like papular appearance characterized by dilated [[capillaries]] in the [[dermis]] and [[hyperkeratotis]] of the overlying [[epidermis]]. Clinically it may manifest as solitary or multiple hyperkeratotic papules that may be localized or generalized, most typically on [[scrotum]], [[thighs]], lower extremity, [[abdomen]], [[trunk]], [[tongue]], [[penis]] and [[labia majora]]. Majority of the [[lesions]] are [[asymptomatic]] but some may ulcerate and/or bleed.<ref name="pmid25100920">{{cite journal |vauthors=Hussein RS, Kfoury H, Al-Faky YH |title=Eyelid angiokeratoma |journal=Middle East Afr J Ophthalmol |volume=21 |issue=3 |pages=287–8 |date=2014 |pmid=25100920 |pmc=4123288 |doi=10.4103/0974-9233.134702 |url=}}</ref><ref name="pmid16988295">{{cite journal |vauthors=Trickett R, Dowd H |title=Angiokeratoma of the scrotum: a case of scrotal bleeding |journal=Emerg Med J |volume=23 |issue=10 |pages=e57 |date=October 2006 |pmid=16988295 |pmc=2579622 |doi=10.1136/emj.2006.038745 |url=}}</ref><ref name="pmid26155544">{{cite journal |vauthors=Chowdappa V, Narasimha A, Bhat A, Masamatti SS |title=Solitary Angiokeratoma: Report of Two Uncommon Cases |journal=J Clin Diagn Res |volume=9 |issue=5 |pages=WD01–2 |date=May 2015 |pmid=26155544 |pmc=4484136 |doi=10.7860/JCDR/2015/12163.5946 |url=}}</ref>
+|-
-* It may be classified into following entities:<ref name="pmid26155544" />
+|[[Rapidly involuting congenital hemangioma]]
-** Fordyce’s angiokeratoma (arising on the genitals)
+|Transient mild/moderate thrombocytopenia, +/-
-** Mibelli’s angiokeratoma (dorsum of toes and fingers)
+consumptive coagulopathy and elevated D-dimer
-** Angiokeratoma circumscriptum naeviforme (unilateral large keratotic plaques)
+|-
-** Angiokeratoma corporis diffusum (ACD) (generalized [[lesions]] between umbilicus and the knee)
+|[[Venous malformations]] /
-* Angiokeratomas are more prevalent among [[males]] as compared to [[females]]. Increased [[venous]] pressure and [[radiation]] therapy have been cited as possible causes. Angiokeratomas have been associated with [[enzyme]] deficiencies such as  alpha-galactosidase A ([[Fabry disease]]), α-fucosidase (fucosidosis), neuraminidase (sialodosis), aspartyl glycosaminase (aspartyl glucosaminuria), β-mannosidase (β- mannosidosis), α-N-acetyl galactosaminidase (Kansaki disease), and β-galactosidase (adult onset GM1 gangliosidosis).<ref name="pmid26155544" /><ref name="pmid25100920" /><ref name="pmid16988295" /><ref name="pmid26312700">{{cite journal |vauthors=Ghosh SK, Ghosh S, Agarwal M |title=Multiple giant angiokeratoma of Fordyce on the shaft of the penis masquerading as keratoacanthoma |journal=An Bras Dermatol |volume=90 |issue=3 Suppl 1 |pages=150–2 |date=2015 |pmid=26312700 |pmc=4540534 |doi=10.1590/abd1806-4841.20153876 |url=}}</ref><ref name="pmid19468654">{{cite journal |vauthors=Rees R, Freeman A, Malone P, Garaffa G, Muneer A, Minhas S |title=Case study: the surgical management of angiokeratoma resulting from radiotherapy for penile cancer |journal=ScientificWorldJournal |volume=9 |issue= |pages=339–42 |date=May 2009 |pmid=19468654 |pmc=5823195 |doi=10.1100/tsw.2009.23 |url=}}</ref>
+Lymphatic-venous malformations
-* The [[diagnosis]] is mainly clinical but [[biopsy]] may be required. Associated [[enzyme]] deficiencies and systemic disorders must be ruled out. Treatment is generally not indicated but if so required then [[excision]], [[electrocautery]], [[cryotherapy]], or [[laser ablations]] are the options.<ref name="pmid25100920" /><ref name="pmid19468654" /><ref name="pmid26155544" /><ref name="pmid25118768">{{cite journal |vauthors=Vijay MK, Arava S |title=Solitary angiokeratoma of tongue: a rare entity clinically mistaken as a malignant tumor |journal=Indian J Pathol Microbiol |volume=57 |issue=3 |pages=510–1 |date=2014 |pmid=25118768 |doi=10.4103/0377-4929.138810 |url=}}</ref><ref name="pmid26312700" />
+|Chronic localized intravascular coagulopathy with
+elevated D-dimer, +/- hypofibrinogenemia, and +/-
+moderate thrombocytopenia (may progress to DIC
+after trauma or operation)
+|-
+|[[Lymphatic malformations]]
+|Chronic localized intravascular coagulopathy with
+elevated D-dimer and +/- mild to moderate
-===Sinusoidal hemangioma===
+thrombocytopenia
-* A variant of [[cavernous hemangioma]] characterized histopathologically by presence of dilated thin-walled [[vascular]] channels, that vary in size, exhibiting nodular proliferation with sinusoidal arrangement.  [[Pseudopapillary]] structures may also be present. Clinically majority of the [[lesions]] manifest in [[female]] [[adults]] as single, well-defined, painless, [[subcutaneous]] nodule with bluish color. Most frequent locations are [[trunk]], [[extremities]] and [[breasts]]. Painless swelling is the most common [[patient]] complaint.<ref name="pmid24250102">{{cite journal |vauthors=Halawar SS, Venugopal R, Varsha B, Kavya B |title=Intramuscular sinusoidal hemangioma with Masson's lesion |journal=J Oral Maxillofac Pathol |volume=17 |issue=2 |pages=315–7 |date=May 2013 |pmid=24250102 |pmc=3830250 |doi=10.4103/0973-029X.119762 |url=}}</ref><ref name="pmid21892538">{{cite journal |vauthors=Ciurea M, Ciurea R, Popa D, Pârvănescu H, Marinescu D, Vrabete M |title=Sinusoidal hemangioma of the arm: case report and review of literature |journal=Rom J Morphol Embryol |volume=52 |issue=3 |pages=915–8 |date=2011 |pmid=21892538 |doi= |url=}}</ref>
-* Abnormalities of [[vasculogenesis]] and [[angiogenesis]] have been proposed as possible [[pathogenesis]] but it is not well-established.<ref name="pmid21892538" />
-* Combination of clinical manifestations and histopathological features is used for [[diagnosis]]. [[Surgery]] (wide excision of tumor) is the treatment of choice if treatment is required.<ref name="pmid21892538" /><ref name="pmid26729822">{{cite journal |vauthors=Konda P, Bavle RM, Makarla S, Muniswamappa S |title=Intramuscular sinusoidal haemangioma with secondary Masson's phenomenon |journal=BMJ Case Rep |volume=2016 |issue= |pages= |date=January 2016 |pmid=26729822 |pmc=4716435 |doi=10.1136/bcr-2013-201457 |url=}}</ref>
-===Acral arteriovenous "tumour"===
+(consider [[Kaposiform lymphangiomatosis]])
-* [[Congenital]] or acquired lesion manifesting clinically as [[asymptomatic]] mass or may present with pulsatile swelling, headache, localized throbbing pain, [[tinnitus]] and bleeding. Histopathologically they are characterized by [[arterio-venous]] connection without connecting [[capillary]] with or without intracranial component. The [[lesion]] derived its name from its acral distribution.<ref name="pmid25624933">{{cite journal |vauthors=Gupta R, Kayal A |title=Scalp arteriovenous malformations in young |journal=J Pediatr Neurosci |volume=9 |issue=3 |pages=263–6 |date=2014 |pmid=25624933 |pmc=4302550 |doi=10.4103/1817-1745.147587 |url=}}</ref><ref name="pmid29492122">{{cite journal |vauthors=Özkara E, Özbek Z, Özdemir AÖ, Arslantaş A |title=Misdiagnosed Case of Scalp Arteriovenous Malformation |journal=Asian J Neurosurg |volume=13 |issue=1 |pages=59–61 |date=2018 |pmid=29492122 |pmc=5820896 |doi=10.4103/1793-5482.181137 |url=}}</ref>
-* [[Etiology]] can be classified as following: [[Congenital]], traumatic, infectious and inflammatory and [[familial]].<ref name="pmid25624933" />
-* Although [[diagnosis]] can be made clinically, [[angiography]] is the gold standard [[diagnostic]] modality to [[diagnose]] and define the extent of the [[lesion]]. Management regimen may include [[surgical excision]], [[ligation]] of the supplying [[arteries]], [[embolization]], and intralesional [[sclerosing]] injection.<ref name="pmid29492122" />
-===Multifocal lymphangioendotheliomatosis with thrombocytopenia / cutaneovisceral angiomatosis with thrombocytopenia (MLT/CAT)===
+(may progress to DIC after trauma or operation)
-* Rare [[congenital]] disorder characterized by proliferation of [[vascular]] channels in multiple [[organs]] associated with [[thrombocytopenia]] of variable degree. [[Lesions]] may manifest themselves on [[skin]], [[gastrointestinal tract]], [[lungs]], [[brain]], [[bone]], [[liver]], [[spleen]] and [[muscles]]. Majority of [[cutaneous]] [[lesions]] present as multiple red to blue papules, plaques, nodules on [[trunk]] and [[extremities]]. [[Gastrointestinal]] bleeding due to multiple [[hemorrhagic]] [[lesions]] is the cause of mortality in majority of the [[patients]]. Similar [[lesions]] in [[brain]] and [[lungs]] may cause severe [[cerebral edema]] and [[pulmonary hemorrhage]].<ref name="pmid26148948">{{cite journal |vauthors=Droitcourt C, Boccara O, Fraitag S, Favrais G, Dupuy A, Maruani A |title=Multifocal Lymphangioendotheliomatosis With Thrombocytopenia: Clinical Features and Response to Sirolimus |journal=Pediatrics |volume=136 |issue=2 |pages=e517–22 |date=August 2015 |pmid=26148948 |doi=10.1542/peds.2014-2410 |url=}}</ref><ref name="pmid22565464">{{cite journal |vauthors=Zegpi MS, Zavala A, del Puerto C, Cárdenas C, González S |title=Newborn with multifocal lymphangioendotheliomatosis with thrombocytopenia |journal=Indian J Dermatol Venereol Leprol |volume=78 |issue=3 |pages=409 |date=2012 |pmid=22565464 |doi=10.4103/0378-6323.95494 |url=}}</ref>
+|-
-* Disease may manifest without [[cutaneous]] involvement or [[thrombocytopenia]]. [[Biopsy]] typically reveals proliferation of well differentiated [[vascular]] channels with intravascular [[papillary]] structure and thrombi, sometimes with hobnail appearance of lining [[endothelial cells]].<ref name="pmid26148948" /><ref name="pmid22565464" />
+|[[Multifocal lymphangioendotheliomatosis with thrombocytopenia]] /
-* [[Biopsy]] followed by histopathological and [[immunohistochemical]] are required for [[diagnosis]]. Management is not well-established and disorder has a poor [[prognosis]] with high mortality. Recently [[sirolimus]] and [[bevacizumab]] have been used to treat this diorder with some success.<ref name="pmid26148948" /><ref name="pmid22565464" /><ref name="pmid19101995">{{cite journal |vauthors=Kline RM, Buck LM |title=Bevacizumab treatment in multifocal lymphangioendotheliomatosis with thrombocytopenia |journal=Pediatr Blood Cancer |volume=52 |issue=4 |pages=534–6 |date=April 2009 |pmid=19101995 |doi=10.1002/pbc.21860 |url=}}</ref><ref name="pmid27282436">{{cite journal |vauthors=Lanöel A, Torres Huamani AN, Feliú A, Sala MJ, Alvarez M, Cervini AB |title=Multifocal Lymphangioendotheliomatosis with Thrombocytopenia: Presentation of Two Cases Treated with Sirolimus |journal=Pediatr Dermatol |volume=33 |issue=4 |pages=e235–9 |date=July 2016 |pmid=27282436 |doi=10.1111/pde.12879 |url=}}</ref>
+[[Cutaneovisceral angiomatosis with thrombocytopenia]]
+|Sustained, fluctuating, moderate to profound
+thrombocytopenia with gastrointestinal tract bleeding or
+pulmonary hemorrhage
+|-
+|[[Kaposiform lymphangiomatosis]]
+|Mild/moderate thrombocytopenia, +/-
+hypofibrinogenemia, and D-dimer elevation
+|-
+| colspan="2" style="background:#7d7d7d; color: #FFFFFF;" + |<small>'''Adapted from International Society for the Study of Vascular Anomalies'''<ref name="urlClassification | International Society for the Study of Vascular Anomalies">{{cite web |url=http://www.issva.org/classification |title=Classification &#124; International Society for the Study of Vascular Anomalies |format= |work= |accessdate=}}</ref></small>
+|}
-===Fibro adipose vascular anomaly (FAVA)===
-* [[Vascular]] [[disorder]] typically manifesting as infiltration of [[muscles]] by fibrofatty tissues, atypical [[venodilation]] associated with localized pain, and contracture of the affected [[muscles]]. Majority of the [[lesions]] involve [[calf]] [[muscles]] and may present as painful mass, [[contracture]] of the [[extremity]], and decreased dorsiflexion at ankle joint. [[Skin]] is not typically involved. Histological studies demonstrates fibrous and [[adipose tissue]] and congregations of [[venous]] channels with abnormal [[lymphatic]] component.<ref name="pmid25298836">{{cite journal |vauthors=Fernandez-Pineda I, Marcilla D, Downey-Carmona FJ, Roldan S, Ortega-Laureano L, Bernabeu-Wittel J |title=Lower Extremity Fibro-Adipose Vascular Anomaly (FAVA): A New Case of a Newly Delineated Disorder |journal=Ann Vasc Dis |volume=7 |issue=3 |pages=316–9 |date=2014 |pmid=25298836 |pmc=4180696 |doi=10.3400/avd.cr.14-00049 |url=}}</ref><ref name="pmid24322574">{{cite journal |vauthors=Alomari AI, Spencer SA, Arnold RW, Chaudry G, Kasser JR, Burrows PE, Govender P, Padua HM, Dillon B, Upton J, Taghinia AH, Fishman SJ, Mulliken JB, Fevurly RD, Greene AK, Landrigan-Ossar M, Paltiel HJ, Trenor CC, Kozakewich HP |title=Fibro-adipose vascular anomaly: clinical-radiologic-pathologic features of a newly delineated disorder of the extremity |journal=J Pediatr Orthop |volume=34 |issue=1 |pages=109–17 |date=January 2014 |pmid=24322574 |doi=10.1097/BPO.0b013e3182a1f0b8 |url=}}</ref>
-* [[Somatic]] activating [[mutations]] in PIK3CA that encodes phosphatidylinositol 3-kinase (PI3K), an [[enzyme]] functioning in cell growth, proliferation, differentiation, and survival.<ref name="urlwww.issva.org">{{cite web |url=http://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf |title=www.issva.org |format= |work= |accessdate=}}</ref>
-* Clinical and [[radiological]] findings are often sufficient to form the [[diagnosis]]. Inconclusive cases my require [[biopsy]]. [[Surgical resection]] is the often the preferred treatment and is more effective than [[sclerotherapy]], the alternative therapy.<ref name="pmid25298836" /><ref name="pmid24322574" />
 ==See also==
 * [[Vascular disease]]