Vascular sarcoma
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Elizabeth J. Davis, MD Vanderbilt University Nashville, TN  ejdavis25
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- 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.
Guidelines
ESMO
- Soft tissue and visceral sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. (2014) PubMed
NCCN
Angiosarcoma, all lines of therapy
Bevacizumab monotherapy
Regimen
| Study | Evidence |
|---|---|
| Agulnik et al. 2013 (NU 04S1) | Phase 2 |
Targeted therapy
- Bevacizumab (Avastin) 15 mg/kg IV once on day 1
21-day cycles
References
- 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 verified protocol PubMed NCT00288015
Paclitaxel monotherapy
Regimen
| Study | Evidence |
|---|---|
| Penel et al. 2008 (ANGIOTAX) | Phase 2 |
Chemotherapy
- Paclitaxel (Taxol) 80 mg/m2 IV over 60 minutes once per day on days 1, 8, 15
Supportive medications
- Dexamethasone (Decadron) 8 mg IV once per day on days 1, 8, 15, prior to Paclitaxel (Taxol)
- Cimetidine (Tagamet) 200 mg IV once per day on days 1, 8, 15, prior to Paclitaxel (Taxol)
- Dexchlorpheniramine (Polaramine) 5 mg IV once per day on days 1, 8, 15, prior to Paclitaxel (Taxol)
- "Standard antiemetics (mainly Metoclopramide (Reglan)) were prescribed as clinically indicated by the treating physician"
28-day cycle for 6 cycles
References
- 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 verified protocol PubMed
Pazopanib monotherapy
Regimen
| 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 prior to becoming a standard comparator arm.
Targeted therapy
- Pazopanib (Votrient) 800 mg PO once per day
Continued indefinitely
References
- 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