Vascular anomalies

Vascular Anomalies
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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
	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	See details	See list
* Defined as two or more vascular malformations found in one lesion ** High flow lesions

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

Classification of Vascular Malformations

Vascular malformations

Simple vascular malformations

Combined vascular malformations

Vascular malformations of major named vessels

Vascular malformations asscoiated with other anomalies

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

Anomalies of major named vessels (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)

Vascular malformations associated with other anomalies 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 sd 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

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

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

In CM-AVM

CM of CM-AVM

Channel type LM

Glomuvenous malformation (GVM)

Others

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