Vascular anomalies
For information on vascular tumors, click here.
For information on vascular malformations, click here.
For information on provisionally unclassified vascular anomalies, click here.
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Vascular Anomalies |
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 | |
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For details, Click here |
For details, Click here | For details, Click here | |
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* Defined as two or more vascular malformations found in one lesion | ||||
| 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 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Hemangioendothelioma not otherwise specified | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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| 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 | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| (also known as "channel type" or "truncal" vascular malformations)
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| Capillary malformations (CM) | Lymphatic malformations (LM) | Venous malformations (VM) | Arteriovenous malformation (AVM) | Arteriovenous fistula | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Nevus simplex / salmon patch, “angel kiss”, “stork bite” |
| Common VM | Sporadic | Sporadic | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Generalized lymphatic anomaly (GLA) Kaposiform lymphangiomatosis (KLA) | Familial VM cutaneo-mucosal (VMCM) | In HHT | In HHT | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| 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) | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| 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
Vascular anomalies possibly associated with platelet count / coagulation disorders
| Anomalies | Hematological disorders |
|---|---|
| Tufted angioma | 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 / | 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] | |