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. 2021 Mar 26;11:641376. doi: 10.3389/fonc.2021.641376

Table 1.

Summary of studies evaluating anti-VEGFR treatments for PEComas/soft tissue sarcomas (1824).

Trail Anti-VEGFR Phase Total No. of patients No. of patients with PEComa No. of patients receiving anti-VEGFR Line of treatment Response
Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomized, double-blind, placebo-controlled phase 3 trial Pazopanib III 372 Not specified 246 Any 14 PR, 164 SD, 57 PD. Median PFS 4.6 mos, OS 12.5 mos
Treatment patterns and clinical outcomes with pazopanib in patients with advanced soft tissue sarcomas in a compassionate use setting: results of the SPIRE study Pazopanib II 211 2 211 Mostly second- and third-line 0 CR, 15 PR, 38 SD. Median OS 11.1 mos,
Role of chemotherapy, VEGFR inhibitors, and mTOR inhibitors in advanced perivascular epithelioid cell tumors (PEComas) Pazopanib, sorafenib, sunitinib Observational 53 53 12: 9 pazopanib, 2 sorafenib, 1 sunitinib 3 second-line, 4 third-line, and 5 fourth- or more lines. 1 PR (sorafenib), 9 SD, 2 PD. Prolonged disease stabilization (12 mos): patients (pazopanib, sorafenib)
Combination targeted therapy of VEGFR inhibitor, sorafenib, with an mTOR inhibitor, sirolimus induced a remakable response of rapid progressive Uterine PEComa Sorafenib Case-report 1 1 1 First CR 7 months (then lost of follow up)
1450P—systemic therapy in perivascular epithelioid cell tumors (PEComa) Pazopanib, sunitinib Observational 49 49 4: pazopanib or sunitinib 1–7 (no more info) 1 PR and 2 SD, with a median PFS 7.3 mos
Metastatic perivascular epithelioid cell tumor of the kidney: a case report with emphasis on response to the tyrosine-kinase inhibitor sunitinib Sunitinib Case-report 1 1 1 First SD 9 months
Anthracycline, gemcitabine, and pazopanib in epithelioid sarcoma Pazopanib Observational 115 Not specified 18 Any PFS 3 months, OS 14 months
Phase II trial of VEGFR2 inhibitor apatinib for metastatic sarcoma: focus on efficacy and safety Apatinib II 64 not specified 64 After chemotherapy failure 0 CR, 10 PR, 41 SD, 8 PD, median PFS 7.93 mos
Real-world experiences with pazopanib in patients with advanced soft tissue and bone sarcoma in Northern California Pazopanib Observational 123 1 123 Any (median number of prior lines: three) 1 CR, 12 PR, 34 SD, 76 PD. Median PFS 3 months
Pazopanib, a multikinase angiogenesis inhibitor, in patients with relapsed or refractory advanced soft tissue sarcoma: a phase II study from the European Organization for research and treatment of cancer–soft tissue and bone sarcoma group (EORTC Study 62043) Pazopanib PHASE II 142 Not specified 142 No more than 2 prior lines of cytotoxic treatment 0 CR 9 PR
The clinical outcome of pazopanib treatment in Japanese patients with relapsed soft tissue sarcoma: A Japanese Musculoskeletal Oncology Group (JMOG) study Pazopanib Retrospective 156 Not specified 156 Any 13 PR, 74 SD, median PFS 15.4 weeks