ISSVA classification of vascular anomalies

The ISSVA classification of vascular anomalies encompasses all vascular malformations and tumors in a framework of internationally consistent nomenclature. 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 revised in May 2018 (ISSVA is the International Society for the Study of Vascular Anomalies).

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 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
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)
Vascular tumours and malformations
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Article information

rID: 30963
Synonyms or Alternate Spellings:
  • ISSVA vascular anomaly classification

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