Desmoid tumor

Section editor
	Elizabeth J. Davis, MD Vanderbilt University Nashville, TN ejdavis25

Note: these are regimens tested in subtype-specific populations, please see the main soft tissue sarcoma page for other regimens.

5 regimens on this page 7 variants on this page

All lines of therapy

Pazopanib monotherapy

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Regimen

Study	Years of enrollment	Evidence
Toulmonde et al. 2019 (DESMOPAZ)	2012-2017	Randomized Phase II

Note: this study was randomized but non-comparative.

Targeted therapy

• Pazopanib (Votrient) 800 mg PO once per day

Up to 12 months of therapy

References

1. DESMOPAZ: Toulmonde M, Pulido M, Ray-Coquard I, Andre T, Isambert N, Chevreau C, Penel N, Bompas E, Saada E, Bertucci F, Lebbe C, Le Cesne A, Soulie P, Piperno-Neumann S, Sweet S, Cecchi F, Hembrough T, Bellera C, Kind M, Crombe A, Lucchesi C, Le Loarer F, Blay JY, Italiano A. Pazopanib or methotrexate-vinblastine combination chemotherapy in adult patients with progressive desmoid tumours (DESMOPAZ): a non-comparative, randomised, open-label, multicentre, phase 2 study. Lancet Oncol. 2019 Sep;20(9):1263-1272. Epub 2019 Jul 19. link to original article contains protocol PubMed NCT01876082

Sorafenib monotherapy

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Regimen

Study	Years of enrollment	Evidence	Comparator	Comparative Efficacy
Gounder et al. 2018 (Alliance A091105)	2014-2016	Phase III (E-esc)	Placebo	Superior PFS

Targeted therapy

• Sorafenib (Nexavar) 400 mg PO once per day

Continued indefinitely

References

1. Alliance A091105: Gounder MM, Mahoney MR, Van Tine BA, Ravi V, Attia S, Deshpande HA, Gupta AA, Milhem MM, Conry RM, Movva S, Pishvaian MJ, Riedel RF, Sabagh T, Tap WD, Horvat N, Basch E, Schwartz LH, Maki RG, Agaram NP, Lefkowitz RA, Mazaheri Y, Yamashita R, Wright JJ, Dueck AC, Schwartz GK. Sorafenib for Advanced and Refractory Desmoid Tumors. N Engl J Med. 2018 Dec 20;379(25):2417-2428. link to original article contains protocol link to PMC article PubMed NCT02066181