ISSVA classification of vascular anomalies

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

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 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

• benign

  • infantile hemangiomas

  • congenital hemangiomas (rapidly involuting, non-involuting, partially involuting)

  • tufted angioma (+/- consumptive coagulopathy i.e. Kasabach Merritt syndrome)

    • possibly part of a spectrum along with Kaposiform hemangioendothelioma

  • spindle-cell hemangioma

  • epithelioid hemangioma

  • pyogenic granuloma (lobular capillary hemangioma)

  • other:

    • hobnob hemangioma, microvenular hemangioma, microvenular hemangioma, anastomosing hemangioma, glomeruloid hemangioma, papillary hemangioma, intravascular papillary endothelial hyperplasia, cutaneous epithelioid angiomatous nodule, acquired elastotic hemangioma, littoral cell angioma of the spleen

• locally aggressive or borderline

  • Kaposiform hemangioendothelioma (+/- consumptive coagulopathy i.e. Kasabach-Merritt syndrome)

    • possibly part of a spectrum along with tufted angioma

  • retiform hemangioendothelioma

  • composite hemangioendothelioma

  • papillary intralymphatic angioendothelioma (Dabska tumor)

  • polymorphous hemangioendothelioma

  • pseudomyogenic hemangioendothelioma

  • hemangioendothelioma not otherwise specified

  • Kaposi sarcoma

• malignant

  • angiosarcoma

  • epithelioid hemangioendothelioma

• others

Vascular malformations

• simple

  • capillary malformation (CM)

    • nevus 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

    • telangiectasia

      • hereditary hemorrhagic telangiectasia (HHT)

      • others

  • venous malformation (VM)

    • common venous malformation

    • blue rubber bleb nevus (Bean) syndrome venous malformation

    • familial VM cutaneo-mucosal

    • glomuvenous malformation (glomangioma)

    • cerebral cavernous malformation

    • familial intraosseous vascular malformation

    • verrucous venous malformation

    • others

  • 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

  • Klippel-Trenaunay syndrome

  • Parkes Weber syndrome

  • Servelle-Martorell syndrome

  • Sturge-Weber syndrome

  • limb capillary malformation + congenital non-progressive limb overgrowth

  • Maffucci syndrome

  • macrocephaly - capillary malformation

  • microcephaly - capillary malformation

  • CLOVES syndrome

  • Proteus syndrome

  • Bannayan-Riley-Ruvalcaba syndrome

  • CLAPO syndrome (lower lip CM + face & neck LM + asymmetry and partial/generalized overgrowth)

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)

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.