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Table 1 ISSVA classification of vascular tumors

From: Vascular tumors in infants and adolescents

Benign vascular tumors

Infantile hemangioma

Congenital hemangioma

 • Rapidly involuting (RICH)a

 • Non-involuting (NICH)

 • Partially involuting (PICH)

Tufted angiomaa

Spindle-cell hemangioma

Epitheloid hemangioma

Pyogenic granuloma

Others

Locally aggressive or borderline vascular tumors

Kaposiform hemangioendotheliomaa

Retiform hemangioendothelioma

Papillary intralymphatic angioendothelioma (PILA), Dabska tumor

Composite hemangioendothelioma

Pseudomyogenic hemangioendothelioma

Polymorpous hemangioendothelioma

Hemangioendothelioma not otherwise specified

Kaposi sarcoma

Others

Malignant vascular tumors

Angiosarcoma

Epitheloid hemangioendothelioma

Others

  1. Of note: (1) reactive proliferative vascular lesions are listed within benign tumors, and (2) tufted angioma and Kaposiform hemangioendothelioma are frequently regarded as part of a spectrum rather distinct entities
  2. aLesions may be associated with thrombocytopenia and/or consumptive coagulopathy (Kasabach-Merrit phenomenon)