目录
概览
Classification of Vascular Tumors
Classification of Fast-flow Vascular Malformations
Classification of Slow-flow Vascular Malformations and Developmental Anomalies of Named Vessels
(BRBNS, Blue Rubber Bleb Nevus Syndrome; CCLA, Central Conducting Lymphatic Anomaly; CLA, Complex Lymphatic Anomaly; CLAPO, Capillary Malformation of the Lower Lip, Lymphatic malformations of the Head and Neck, Asymmetry and Partial or Generalized Overgrowth; CLOVES, Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevi, and Skeletal Anomalies; CLVM, Capillary-Lymphatic Venous Malformation; CM-AVM, Capillary Malformation-Arteriovenous Malformation; CM, Capillary Malformation; CVM, Capillary-venous Malformation; DCMO, Diffuse Capillary Malformation with Overgrowth; FAVA, Fibroadipose Vascular Anomaly; GLA, Generalized Lymphatic Anomaly; GLD, Generalized Lymphatic Dysplasia GSD, Gorham-Stout Disease; GVM, Glomuvenous Malformation; HCCVM/CCM, Hyperkeratotic Cerebrous Cavernous Venous malformations/Cerebral Cavernous Malformation; HHT, Hereditary Hemorrhagic Telangiectasia; JPHT, Juvenile Polyposis Hemorrhagic Telangiectasia; KLA, Kaposiform lymphangiomatosis; KTS, Klippel-Trenaunay Syndrome; LM, Lymphatic Malformation; LVM, Lymphatic-venous Malformation; M-CM, Macrocephaly Capillary Malformation Syndrome; MIC-CAP, Microcephaly Capillary Malformation; MSVM, Multifocal Sporadic Venous Malformation; PHTS, PTEN Hamartoma Tumor Syndrome; PROS, PIK3CA Related Overgrowth Spectrum; VM, Venous Malformation; VMCM, Familial VM cutaneo-mucosal; VMOS, Familial Intraosseous Vascular Malformation; VVM, Verrucous-venous Malformation).
Summary of main changes in the Updated Classification
| Vascular Anomaly | Main changes | New terms and diagnosis |
|---|---|---|
| Main Division | VA are divided into Tumors, Malformations and Potentially Unique Vascular Anomalies (PUVA). The term “Provisionally Unclassified Vascular Anomalies” no longer exist. Some terms were moved to the Glossary, making classification simpler. Genetic correlation is also available in Glossary. | PUVA |
| Tumors | Inclusion of all histopathologic variants of benign tumors in the same list (removal of term “other” and “related lesions”) | Inclusion in Borderline tumors – Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT) |
| Vascular Malformations | Main division is between fast-flow, slow-flow and Developmental Anomalies of Named Vessels Removal of term “Simple Vascular Malformation” The category “Vascular Malformations associated with other anomalies” is suppressed and the diagnosis incorporated into the VM subtypes | Developmental Anomalies of Named Vessels Fast-flow Vascular Malformation Slow-flow Vascular Malformation |
| Fast-flow Vascular Malformations | Subtypes are Isolated, Multifocal and Syndromic. Removal of terms “sporadic”, “in HHT” and “CM-AVM” | CAMS CM-AVM 1 and 2 Intramuscular fast-flow vascular anomaly PHTS/PHOST SAMS |
| Slow-flow Vascular Malformations | VM are divided into vascular type (Capillary, Lymphatic, Venous or Combined) | |
| Venous Malformations | Are divided into Isolated, Multifocal and Syndromic | CLOVES FAVA HCCVM/CCM Mafucci Syndrome MSVM Phlebecratic VM PHTS Sinus pericranii Spongiform VM |
| Lymphatic malformations | Are divided into Isolated, Complex Lymphatic Anomalies (CLA) and Lymphedemas Terms were relocated into these categories | Angiokeratoma CCLA Mixed macro-microcystic Primary and secondary Lymphedemas |
| Capillary Malformations | Inclusion of terms that facilitate grouping of lesions according to cutaneous pattern, the presence of genetic and syndromic components. CM were divided into Nevus simplex/ Salmon patch type, Port wine CM, Reticulate/telangiectatic CM, Geographic pattern CM, Low-resistance CM/CM with faster flow, Cutis Marmorata Telangiectatic Congenita, and Telangiectasias and Spider Angiomas | CM in CLOVES CM in Disorders of the PROS Spectrum CM-AVM 1 and 2 Geographic pattern CM Port wine CM |
| Combined VM | Divided into Isolated and Syndromic | CLAPO CLM CLOVES |
| Developmental Anomalies of Named Vessels | Simplified by naming vessels | Aorta Vena Cava Vein. of Galen |