From: Childhood Vascular Tumors Treatment (PDQ®)
PDQ Cancer Information Summaries [Internet].
Bethesda (MD): National Cancer Institute (US); 2002-.
NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.
Table 2. 2018 International Society for the Study of Vascular Anomalies (ISSVA) Classification of Vascular Tumorsa
| Category | Vascular Tumor Type (Causal Genes) |
|---|---|
| Benign (type 1b) | Infantile hemangioma/hemangioma of infancy |
| Congenital hemangioma (GNAQ, GNA11) | |
| —Rapidly involuting (RICH) | |
| —Non-involuting (NICH) | |
| —Partially-involuting (PICH) | |
| Tufted angiomac | |
| Spindle cell hemangioma (IDH1, IDH2) | |
| Epithelioid hemangioma (FOS) | |
| Pyogenic granuloma (also known as lobular capillary hemangioma) (BRAF, RAS, GNA14) | |
| Others | |
| Locally aggressive or borderline | Kaposiform hemangioendothelioma (KHE) (GNA14) |
| Retiform hemangioendothelioma | |
| Papillary intralymphatic angioendothelioma (PILA), Dabska tumor | |
| Composite hemangioendothelioma | |
| Pseudomyogenic hemangioendothelioma (FOSB) | |
| Polymorphous hemangioendothelioma | |
| Hemangioendothelioma not otherwise specified | |
| Kaposi sarcoma | |
| Others | |
| Malignant | Angiosarcoma (MYC: postradiation therapy) |
| Epithelioid hemangioendothelioma (EHE) (CAMTA1, TFE3) | |
| Others |
aAdapted from ISSVA Classification of Vascular Anomalies. ©2018 International Society for the Study of Vascular Anomalies. Available at "issva
.org/classification." Accessed June 2018.[4] bSee the ISSVA classification 2018 for benign vascular tumors 2.[4]
cTufted angioma and kaposiform hemangioendothelioma are a spectrum of the same entity and will be discussed together.