PEComa

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

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

1 regimens on this page 1 variants on this page

All lines of therapy

nab-Sirolimus monotherapy

Regimen

FDA-recommended dose

Study	Dates of enrollment	Evidence
Wagner et al. 2021 (AMPECT) ESMO-MCBS (3)	2016-2018	Phase 2 (RT)

Targeted therapy

• Sirolimus protein-bound particles (Fyarro) 100 mg/m² IV once per day on days 1 & 8

21-day cycles

References

1. AMPECT: Wagner AJ, Ravi V, Riedel RF, Ganjoo K, Van Tine BA, Chugh R, Cranmer L, Gordon EM, Hornick JL, Du H, Grigorian B, Schmid AN, Hou S, Harris K, Kwiatkowski DJ, Desai NP, Dickson MA. nab-Sirolimus for Patients With Malignant Perivascular Epithelioid Cell Tumors. J Clin Oncol. 2021 Nov 20;39(33):3660-3670. Epub 2021 Oct 12. link to original article contains dosing details in manuscript link to PMC article PubMed https://clinicaltrials.gov/ct2/show/NCT02494570