ISSVA classification of vascular anomalies

The ISSVA classification of vascular anomalies encompasses all vascular malformations and tumors in a framework of internationally consistent nomenclature.  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 .

It is one of the two most widely used classification systems, the other being the Hamburg classification system of vascular malformations.


The main organisational principle behind this classification divides vascular lesions into:

  • vascular tumors (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 tumor or malformation)
Vascular tumors
Vascular malformations
  • simple
    • capillary malformation (CM)
    • venous malformation (VM)
    • lymphatic malformation (LM)
      • common (cystic) lymphatic malformation - macrocystic, microcystic, mixed cystic
      • generalized lymphatic anomaly
        • Kaposiform lymphangiomatosis
      • lymphatic malformation in Gorham-Stout disease
      • channel-type lymphatic malformation
      • "acquired" progressive lymphatic anomaly (acquired progressive "lymphangioma")
      • primary lymphedema
        • Nonne-Milroy syndrome
        • primary hereditary lymphedema (VEGFC, GJC2/Connexin 47)
        • lymphedema-distichiasis
        • hypotrichosis-lymphedema-telangiectasia
        • primary lymphedema with myelodysplasia
        • primary generalized lymphatic anomaly (Hennekam lymphangiectasia-lymphedema syndrome)
        • microcephaly with or without chorioretinopathy, lymphedema, or intellectual disability
        • lymphedema-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
Provisionally unclassified vascular anomalies
  • intramuscular hemangioma (distinct from infantile hemangioma, common venous malformation)
  • angiokeratoma
  • sinusoidal hemangioma
  • acral arteriovenous "tumor"
  • multifocal lymphangioendotheliomatosis with thrombocytopenia/cutaneovisceral angiomatosis with thrombocytopenia (MLT/CAT)
  • PTEN hamartoma of soft tissue/"angiomatosis" of soft tissue (PTEN)
  • fibroadipose vascular anomaly (FAVA)
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