Vascular sarcoma

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Section editor
Elizabeth J. Davis, MD
Vanderbilt University
Nashville, TN

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3 regimens on this page
3 variants on this page
  • Note: this page contains regimens for several subtypes of vascular sarcoma. The following links will take you to disease-specific pages:
  • Regimens for Kaposi sarcoma are here.




Angiosarcoma, all lines of therapy

Bevacizumab monotherapy


Study Evidence
Agulnik et al. 2013 (NU 04S1) Phase 2

Targeted therapy

21-day cycles


  1. NU 04S1: Agulnik M, Yarber JL, Okuno SH, von Mehren M, Jovanovic BD, Brockstein BE, Evens AM, Benjamin RS. An open-label, multicenter, phase II study of bevacizumab for the treatment of angiosarcoma and epithelioid hemangioendotheliomas. Ann Oncol. 2013 Jan;24(1):257-63. link to original article contains dosing details in manuscript PubMed NCT00288015

Paclitaxel monotherapy


Study Evidence
Penel et al. 2008 (ANGIOTAX) Phase 2


Supportive therapy

28-day cycle for 6 cycles


  1. ANGIOTAX: Penel N, Bui BN, Bay JO, Cupissol D, Ray-Coquard I, Piperno-Neumann S, Kerbrat P, Fournier C, Taieb S, Jimenez M, Isambert N, Peyrade F, Chevreau C, Bompas E, Brain EG, Blay JY. Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX Study. J Clin Oncol. 2008 Nov 10;26(32):5269-74. Epub 2008 Sep 22. link to original article contains dosing details in manuscript PubMed

Pazopanib monotherapy


Study Years of enrollment Evidence Comparator Comparative Efficacy
Jones et al. 2022 (TAPPAS) 2017-2019 Phase 3 (C) Pazopanib & Carotuximab Did not meet primary endpoint of PFS

Note: to our knowledge, this regimen was not tested as an experimental arm in an RCT in this context, prior to becoming a standard comparator arm.

Targeted therapy

Continued indefinitely


  1. TAPPAS: Jones RL, Ravi V, Brohl AS, Chawla S, Ganjoo KN, Italiano A, Attia S, Burgess MA, Thornton K, Cranmer LD, Cheang MCU, Liu L, Robertson L, Adams B, Theuer C, Maki RG. Efficacy and Safety of TRC105 Plus Pazopanib vs Pazopanib Alone for Treatment of Patients With Advanced Angiosarcoma: A Randomized Clinical Trial. JAMA Oncol. 2022 May 1;8(5):740-747. link to original article link to PMC article PubMed NCT02979899

Hemangioendothelioma, all lines of therapy

Hemangiopericytoma (solitary fibrous tumor), all lines of therapy