Childhood Vascular Tumors Treatment (PDQ®)–Health Professional Version

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General Information About Childhood Vascular Tumors

The quality of evidence regarding childhood vascular tumors is limited by retrospective data collection, small sample size, cohort selection and participation bias, and heterogeneity of the disorders.

Vascular anomalies are a spectrum of rare diseases classified as vascular tumors or malformations. An updated classification system was adopted at the General Assembly of the International Society for the Study of Vascular Anomalies (ISSVA , April 2014) and recently published.[1] Generally, vascular tumors are proliferative, while malformations enlarge through expansion of a developmental anomaly without underlying proliferation. Growth and/or expansion of vascular anomalies can cause clinical problems such as disfigurement, chronic pain, recurrent infections, coagulopathies (thrombotic and hemorrhagic), organ dysfunction, and death. Individuals often experience progressive clinical symptoms with worsening quality of life. Limited treatment options are available; their efficacy has not been validated in prospective clinical trials. Historically, therapies have been mostly interventional and surgical to palliate symptoms.

Vascular tumors in children are rare. The classification of these tumors has been difficult, especially in the pediatric population, because of their rarity, unusual morphologic appearance, diverse clinical behavior, and the lack of independent stratification for pediatric tumors. In 2013, The World Health Organization (WHO) updated the classification of soft tissue vascular tumors. Pediatric tumors were not independently stratified and the terminology was mostly left unchanged, but the intermediate category of tumors was divided into locally aggressive and rarely metastasizing. The ISSVA classification of tumors is based on the WHO classification (refer to Tables 1 and 2) but the ISSVA classification uses more precise terminology and phenotypes that have been agreed upon by the members of ISSVA.

Table 1. 2013 World Health Organization Classification of Vascular Tumors

Category	Vascular Tumor Type
aAdapted from Fletcher et al.[2]
Benign	Hemangioma
	Epithelioid hemangioma
	Angiomatosis
	Lymphangioma
Intermediate (locally aggressive)	Kaposiform hemangioendothelioma
Intermediate (rarely metastasizing)	Retiform hemangioendothelioma
	Papillary intralymphatic angioendothelioma
	Composite hemangioendothelioma
	Kaposi sarcoma
Malignant	Epithelioid hemangioendothelioma
Malignant	Angiosarcoma of soft tissue

Table 2. 2014 International Society for the Study of Vascular Anomalies (ISSVA) Classification of Vascular Tumorsa

Category	Vascular Tumor Type
aAdapted from ISSVA Classification of Vascular Anomalies. ©2014 International Society for the Study of Vascular Anomalies. Available at "issva.org/classification Exit Disclaimer." Accessed January 2016.[3]
Benign	Infantile hemangioma/hemangioma of infancy
	Congenital hemangioma
	—Rapidly involuting (RICH)
	—Non-involuting (NICH)
	—Involuting (PICH)
	Tufted angioma
	Spindle cell hemangioma
	Pyogenic granuloma (also known as lobular capillary hemangioma)
	Others
Locally aggressive or borderline	Kaposiform hemangioendothelioma
	Retiform hemangioendothelioma
	Papillary intralymphatic angioendothelioma (PILA), Dabska tumor
	Composite hemangioendothelioma
	Kaposi sarcoma
	Others
Malignant	Epithelioid hemangioendothelioma
	Angiosarcoma
	Others

References

Wassef M, Blei F, Adams D, et al.: Vascular Anomalies Classification: Recommendations From the International Society for the Study of Vascular Anomalies. Pediatrics 136 (1): e203-14, 2015. [PUBMED Abstract]
Fletcher CDM, Bridge JA, Hogendoorn P, et al., eds.: WHO Classification of Tumours of Soft Tissue and Bone. 4th ed. Lyon, France: IARC Press, 2013.
International Society for the Study of Vascular Anomalies: ISSVA Classification for Vascular Anomalies. Melbourne, Australia: International Society for the Study of Vascular Anomalies, 2014. Available online Exit Disclaimer. Last accessed August 11, 2016.

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Benign Tumors