ISSVA classification of vascular anomalies

Last revised by Henry Knipe on 10 Jan 2024

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

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)

  • 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)

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.

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