ISSVA Classification of Vascular Anomalies Overview

Vascular Anomaly
- Vascular Tumour
  - Benign
    - Infantile Haemangioma
      A common, benign vascular tumour in infants, characterised by rapid growth followed by slow involution.
    - Congenital Haemangioma
      A fully formed benign vascular tumour present at birth, known for rapid postnatal involution.
      - Rapidly Involuting (RICH)
        A subtype of congenital haemangioma that quickly regresses after birth.
      - Non-involuting (NICH)
        A congenital hemangioma subtype that does not regress.
      - Partially-involuting
    - Pyogenic Granuloma
    - Tufted Angioma
      A rare, benign tumour appearing as a red-brown, spongy skin lesion.
  - Locally Aggressive
    - Kaposiform Haemangioendothelioma
      A locally aggressive vascular tumour often associated with Kasabach-Merritt phenomenon (blood clotting disorder).
    - Kaposi’s Sarcoma
      A type of cancer that forms in the lining of blood and lymph vessels, characterised by purple or red patches on the skin or mucous membranes. It is associated with weakened immune systems, including in people with HIV/AIDS.
  - Malignant
    - Angiosarcoma
      A rare, aggressive cancer originating in the cells that line blood vessels or lymph vessels, known for rapid growth and a high potential for metastasis. Appears as a heterogeneously enhancing mass, often with areas of necrosis and haemorrhage; highly variable appearance based on location.
    - Epithelioid Haemangioendothelioma
      A rare vascular tumor of intermediate malignancy, occurring in the blood vessels, characterised by both endothelial and epithelioid features.
- Vascular Malformation
  - Simple
    - Capillary (CM)
      A flat, red skin mark caused by dilated capillaries.
    - Venous (VM)
      Soft, blue skin lesions due to abnormally formed veins.
    - Arteriovenous Malformation (AVM)
      A tangle of abnormal arteries and veins without a capillary bed, often with high blood flow.
    - Arteriovenous Fistula (AVF)
      An abnormal direct connection between an artery and a vein.
    - Lymphatic (LM)
      Sponge-like collections of abnormal lymphatic vessels, presenting as fluid-filled cysts.
  - Combined
    - CM + VM (CVM)
    - LM + VM (LVM)
    - CM + LM + VM (CLVM)
    - CM + AVM + VM (CAVM)
    - CM + LM + AVM + VM (CLAVM)
  - Associated with other Anomalies
    - Klippel-Trenaunay Syndrome
      Characterised by a triad of capillary malformation, venous malformation, and limb overgrowth, often affecting one limb.
    - Parkes-Weber Syndrome
      Similar to Klippel-Trenaunay Syndrome but includes arteriovenous fistulas, resulting in a high-flow vascular malformation along with limb overgrowth.
    - Sturge-Weber Syndrome
      A neurological and skin disorder marked by port-wine stains on the face, glaucoma, and neurological abnormalities including seizures and intellectual disability.
    - Servelle-Martorell Syndrome
      A rare vascular malformation syndrome causing progressive soft tissue and bone hypertrophy or hypotrophy, often leading to limb length discrepancy.
    - Marfucci Syndrome
      Characterised by multiple enchondromas (benign bone lesions) and soft tissue venous malformations, leading to skeletal deformities and a risk of malignant transformation.
    - CLOVES Syndrome
      Congenital Lipomatous Overgrowth, Vascular malformations, Epidermal nevi, and Skeletal/spinal anomalies. It is a rare complex overgrowth syndrome with vascular anomalies.
    - Proteus Syndrome
      A complex disorder involving overgrowth of multiple tissues including skin, bones, and other tissues, often resulting in asymmetrical growth and a variety of vascular anomalies.

Updated on 18 November 2023

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