Table 1. Relevant studies conducted on PEComa treatment.
NR: not reported, m: month, d: day, RECIST: response evaluation criteria in solid tumor, PR: partial response, N/A: not available, SD: stable disease, PD: progressive disease, CR: complete response, PFS: progression-free survival.
| Study | No. of patients | Treatment | Treatment duration | Response | Side effects | RECIST1.1 response | Survival rate (%) | PFS (months) | Median follow-up (months) |
| GenBenson et al. [13] | 10 | Sirolimus, temsilorimus | 7 d-45.5 m | Good (7), No response (3) | Hypertriglyceridemia, hyperglycemia, thrombocytopenia | PR (5), N/A (3), SD (1), PD (1) | 78.80% | 26 m | 21 m |
| Sanlippo et al. [14] | 53 | Chemotherapy (23), VEGFR, mTOR inhibitors (40), sirolimus (32), everolimus (5), temsirolimus (3) | 24 m | Good | NR | CR (1), PR (15), SD (14), PD (9) | 41% | 9 m | 32.5 m |
| Gennata et al. [15] | 1 | Surgery, imatinib, everolimus | 10 m | Good | Stomatitis, nasopharyngitis, acne-like skin lesions | CR | NR | >10 m | 37 m |
| Switaj et al. [16] | 15 | Sirolimus (11), chemo (4) | 25 m | Good (15) | Hypertriglyceridemia, hypercholesteremia | PR (8), SD-= (4), CR (3) | 83% (sirolimus), 65% (overall) | 42.6 m | 55.7 m |