The ISSVA classification of vascular anomalies encompasses all vascular malformations and tumours in a framework of internationally consistent nomenclature.
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Usage
It is one of the two most widely used classification systems, the other being the Hamburg classification system of vascular malformations.
Classification
The main organisational principle behind this classification divides vascular lesions into:
-
vascular tumours (neoplastic)
benign
locally aggressive or borderline
malignant
-
vascular malformations (non-neoplastic)
simple
combined
of major named vessels
associated with other anomalies
unclassified anomalies (unclear if tumour or malformation)
Vascular tumours
-
benign
congenital haemangiomas (rapidly involuting, non-involuting, partially involuting)
-
tufted angioma (+/- consumptive coagulopathy i.e. Kasabach Merritt syndrome)
possibly part of a spectrum along with Kaposiform haemangioendothelioma
epithelioid haemangioma
-
other:
hobnob haemangioma, microvenular haemangioma, microvenular haemangioma, anastomosing haemangioma, glomeruloid haemangioma, papillary haemangioma, intravascular papillary endothelial hyperplasia, cutaneous epithelioid angiomatous nodule, acquired elastotic haemangioma, littoral cell angioma of the spleen
-
locally aggressive or borderline
-
Kaposiform haemangioendothelioma (+/- consumptive coagulopathy i.e. Kasabach-Merritt syndrome)
possibly part of a spectrum along with tufted angioma
haemangioendothelioma not otherwise specified
-
-
malignant
others
Vascular malformations
-
simple
-
capillary malformation (CM)
naevus simplex/salmon patch ("angel kiss", "stork bite")
cutaneous and/or mucosal CM ("port-wine stain")
reticulate capillary malformation
capillary malformation of CM-AVM
cutis marmorata telangiectatica congenita
-
venous malformation (VM)
common venous malformation
blue rubber bleb naevus (Bean) syndrome venous malformation
glomuvenous malformation (glomangioma)
familial intraosseous vascular malformation
verrucous venous malformation
others
-
lymphatic malformation (LM)
common (cystic) lymphatic malformation - macrocystic, microcystic, mixed cystic
lymphatic malformation in Gorham-Stout disease
channel-type lymphatic malformation
"acquired" progressive lymphatic anomaly (acquired progressive "lymphangioma")
-
primary lymphoedema
primary hereditary lymphoedema (VEGFC, GJC2/Connexin 47)
lymphoedema-distichiasis
hypotrichosis-lymphoedema-telangiectasia
primary lymphoedema with myelodysplasia
primary generalised lymphatic anomaly (Hennekam lymphangiectasia-lymphoedema syndrome)
microcephaly with or without chorioretinopathy, lymphoedema, or intellectual disability
lymphoedema-choanal atresia
arteriovenous malformations (AVM) (with a nidus of multiple shunts)
arteriovenous fistulae (AVF) (with one or more shunts)
-
-
combined
CM + VM (CVM)
LM + VM (LVM)
CM + LM + VM (CLVM)
CM + AVM + VM (CAVM)
CM + LM + AVM + VM (CLAVM)
-
of major named vessels ("channel type" or "truncal" vascular malformations)
various abnormalities affecting origin, course, number, length, diameter, valves, communication, and persistence of primitive vessels
-
those associated with other anomalies
limb capillary malformation + congenital non-progressive limb overgrowth
microcephaly - capillary malformation
CLAPO syndrome (lower lip CM + face & neck LM + asymmetry and partial/generalised overgrowth)
Provisionally unclassified vascular anomalies
intramuscular haemangioma (distinct from infantile haemangioma, common venous malformation)
angiokeratoma
sinusoidal haemangioma
acral arteriovenous "tumour"
multifocal lymphangioendotheliomatosis with thrombocytopenia/cutaneovisceral angiomatosis with thrombocytopenia (MLT/CAT)
PTEN hamartoma of soft tissue/"angiomatosis" of soft tissue (PTEN)
History and etymology
ISSVA is the International Society for the Study of Vascular Anomalies. It is based on the initial classification published by Mulliken and Glowacki in 1982 and has since been updated with recognition of causal genetic mutations. The classification was most recently revised in May 2018 4.