Management of Vascular Sarcoma

Surg Oncol Clin N Am. 2022 Jul;31(3):485-510. doi: 10.1016/j.soc.2022.03.014.


Vascular sarcomas encompass 3 well-defined sarcoma types: hemangioendothelioma, Kaposi sarcoma, and angiosarcoma. These distinct types are exceedingly rare and very different in terms of clinical behavior, biological features, and treatment approach. Because of this rarity and heterogeneity, it is crucial that vascular sarcomas are treated in sarcoma reference centers or networks, in order to ensure optimal management. The diversity of vascular sarcomas also needs to be taken into account in the design of clinical trials, in order to produce meaningful results that can be consistently translated into everyday clinical practice.

Keywords: Angiosarcoma; Epithelioid hemangioendothelioma; Hemangioendothelioma; Kaposi sarcoma; Vascular sarcoma.

Publication types

  • Review

MeSH terms

  • Hemangioendothelioma*
  • Hemangioendothelioma, Epithelioid*
  • Hemangiosarcoma* / therapy
  • Humans
  • Sarcoma* / therapy
  • Soft Tissue Neoplasms*