PEComa
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Section editor | |
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Elizabeth J. Davis, MD Vanderbilt University Nashville, TN, USA |
Note: these are regimens tested in subtype-specific populations, please see the main soft tissue sarcoma page for other regimens.
Last updated on 2024-07-23: 1 regimens on this page
1 variants on this page
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Guidelines
Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.
NCCN
- NCCN does not currently have guidelines at this granular level; please see NCCN Guidelines - Soft Tissue Sarcoma.
All lines of therapy
nab-Sirolimus monotherapy
Regimen
FDA-recommended dose |
Study | Dates of enrollment | Evidence |
---|---|---|
Wagner et al. 2021 (AMPECT) | 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
- 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 dosing details in manuscript have been reviewed by our editors link to PMC article PubMed NCT02494570
- Update: Wagner AJ, Ravi V, Riedel RF, Ganjoo K, Van Tine BA, Chugh R, Cranmer L, Gordon EM, Hornick JL, Du H, Ding L, Schmid AN, Navarro WH, Kwiatkowski DJ, Dickson MA. Phase II Trial of nab-Sirolimus in Patients With Advanced Malignant Perivascular Epithelioid Cell Tumors (AMPECT): Long-Term Efficacy and Safety Update. J Clin Oncol. 2024 May 1;42(13):1472-1476. Epub 2024 Mar 1. link to original article link to PMC article PubMed