What’s the latest with investigational drugs for soft tissue sarcoma?

References

• Kallen ME, Hornick JL. The 2020 WHO classification: what’s new in soft tissue tumor pathology? Am J Surg Pathol. 2021;45(1):e1–e23.
   PubMed Web of Science ®Google Scholar
• Gronchi A, Miah AB, Dei Tos AP, et al. Soft tissue and visceral sarcomas: ESMO–EURACAN–GENTURIS clinical practice guidelines for diagnosis, treatment and follow-up☆. Ann Oncol. 2021;32(11):1348–1365.
   PubMed Web of Science ®Google Scholar
• Seddon B, Strauss SJ, Whelan J, et al. Gemcitabine and docetaxel versus doxorubicin as first-line treatment in previously untreated advanced unresectable or metastatic soft-tissue sarcomas (GeDDiS): a randomised controlled phase 3 trial. Lancet Oncol. 2017 Oct;18(10):1397–1410.
   PubMed Web of Science ®Google Scholar
• Tap WD, Wagner AJ, Schöffski P, et al. Effect of doxorubicin plus olaratumab vs doxorubicin plus placebo on survival in patients with advanced soft tissue sarcomas: the ANNOUNCE randomized clinical trial. JAMA. 2020 Apr;323(13):1266–1276.
   PubMed Web of Science ®Google Scholar
• Tap WD, Papai Z, Van Tine BA, et al. Doxorubicin plus evofosfamide versus doxorubicin alone in locally advanced, unresectable or metastatic soft-tissue sarcoma (TH CR-406/SARC021): an international, multicentre, open-label, randomised phase 3 trial. Lancet Oncol. 2017 Aug;18(8):1089–1103.
   PubMed Web of Science ®Google Scholar
• Ryan CW, Merimsky O, Agulnik M, et al. PICASSO III: a phase III, placebo-controlled study of doxorubicin with or without palifosfamide in patients with metastatic soft tissue sarcoma. J Clin Oncol Off J Am Soc Clin Oncol. 2016 Nov;34(32):3898–3905.
   PubMed Web of Science ®Google Scholar
• Araujo DM, Druta M, Agulnik M, et al. SPEARHEAD-1: a phase II trial of ADP-A2M4 SPEAR T cells in patients with advanced synovial sarcoma or myxoid/round cell liposarcoma. J Clin Oncol. 2020May20;Internet; 38(15_suppl):TPS11569TPS11569.):TPS11569TPS11569.
   Google Scholar
• Dallos M, Tap WD, D’Angelo SP. Current status of engineered T-cell therapy for synovial sarcoma. Immunotherapy. 2016;8(9):1073–1080.
   PubMed Web of Science ®Google Scholar
• Zhao L, Cao YJ. Engineered T cell therapy for cancer in the clinic. Front Immunol. 2019;10(October).
   Google Scholar
• Lai JP, Rosenberg AZ, Miettinen MM, et al. NY-ESO-1 expression in sarcomas a diagnostic marker and immunotherapy target. Oncoimmunology. 2012;1(8):1409–1410.
   PubMed Web of Science ®Google Scholar
• Hemminger JA, Iwenofu OH. NY-ESO-1 is a sensitive and specific immunohistochemical marker for myxoid and round cell liposarcomas among related mesenchymal myxoid neoplasms. Mod Pathol an off J United States Can Acad Pathol Inc. 2013 Sep;26(9):1204–1210.
   PubMed Web of Science ®Google Scholar
• Robbins PF, Kassim SH, Tran TLN, et al. A pilot trial using lymphocytes genetically engineered with an NY-ESO-1-reactive T-cell receptor: long-term follow-up and correlates with response. Clin Cancer Res. 2015;21(5):1019–1027.
   PubMed Web of Science ®Google Scholar
• Pn P, Sp DA, Demetri G, et al. Phase I trial of NY-ESO-1-specific adoptive T-cell therapy with GSK3377794 in patients with advanced synovial sarcoma : report of Cohorts 2 and 4. 2019;3377794.
   Google Scholar
• A pilot study of genetically engineered NY-ESO-1 specific NY-ESO-1c259T in HLA-A2+ patients with synovial sarcoma (NY-ESO-1). ClinicalTrials.gov. [cited 2021 Dec 5]. Available from: https://clinicaltrials.gov/ct2/show/results/NCT01343043?view=results
   Google Scholar
• D’Angelo SP, Demetri GD, Van Tine B, et al. Phase I trial of NY-ESO-1-specific adoptive T-cell therapy with GSK3377794 in patients with advanced synovial sarcoma: report of Cohorts 2 and 4. Society for immunotherapy of cancer congress. Journal for ImmunoTherapy of Cancer. 2019;7(Suppl 1):282.
   Google Scholar
• D’Angelo SP, Blay J-Y, Chow WA, et al. Safety and activity of autologous T cells with enhanced NY-ESO-1–specific T-cell receptor (GSK3377794) in HLA-a*02+ previously-treated and -untreated patients with advanced metastatic/unresectable synovial sarcoma: a master protocol study design (IGNYTE-E). J Clin Oncol. 2020 May 20;38(15_suppl):TPS11571TPS11571.
   Web of Science ®Google Scholar
• D’Angelo SP, Druta M, Liebner DA, et al. Pilot study of NY-ESO-1c259 T cells in advanced myxoid/round cell liposarcoma. J Clin Oncol. 2018May20;36(15_suppl):3005.
   Web of Science ®Google Scholar
• Iura K, Kohashi K, Ishii T, et al. MAGEA4 expression in bone and soft tissue tumors: its utility as a target for immunotherapy and diagnostic marker combined with NY-ESO-1. Virchows Arch. 2017 Sep;471(3):383–392.
   PubMed Web of Science ®Google Scholar
• Van Tine BA, Butler MO, Araujo D, et al. 1670O - ADP-A2M4 (MAGE-A4) in patients with synovial sarcoma. Ann Oncol. 2019;30:v684–5.
   Web of Science ®Google Scholar
• Sanderson JP, Crowley DJ, Wiedermann GE, et al. Preclinical evaluation of an affinity-enhanced MAGE-A4-specific T-cell receptor for adoptive T-cell therapy. Oncoimmunology. 2020;9(1):1682381.
   PubMed Web of Science ®Google Scholar
• Hong DS, Van Tine BA, Olszanski AJ, et al. Phase I dose escalation and expansion trial to assess the safety and efficacy of ADP-A2M4 SPEAR T cells in advanced solid tumors. J Clin Oncol. 2020 May 20;38(15_suppl):102.
   Google Scholar
• D’Angelo SP, Van Tine BA, Attia S, et al. SPEARHEAD-1: a phase 2 trial of afamitresgene autoleucel (Formerly ADP-A2M4) in patients with advanced synovial sarcoma or myxoid/round cell liposarcoma. J Clin Oncol. 2021 May 20;39(15_suppl):11504.
   Web of Science ®Google Scholar
• Tawbi HA, Burgess M, Bolejack V, et al. Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial. Lancet Oncol. 2017;18(11):1493–1501.
   PubMed Web of Science ®Google Scholar
• Clemente O, Ottaiano A, Di Lorenzo G, et al. Is immunotherapy in the future of therapeutic management of sarcomas? J Transl Med. 2021;19(1):1–24.
   PubMed Web of Science ®Google Scholar
• Minchom A, Jones RL, Fisher C, et al. Clinical benefit of second-line palliative chemotherapy in advanced soft-tissue sarcoma. Sarcoma. 2010. Vol. 2010: 264360.
   Google Scholar
• Martin-Broto J, Hindi N, Grignani G, et al. Nivolumab and sunitinib combination in advanced soft tissue sarcomas: a multicenter, single-arm, phase Ib/II trial. J Immunother Cancer. 2020 Nov;8(2):e001561.
   PubMed Web of Science ®Google Scholar
• Smrke A, Ostler A, Napolitano A, et al. 1526MO - GEMMK: a phase I study of gemcitabine (gem) and pembrolizumab (pem) in patients (pts) with leiomyosarcoma (LMS) and undifferentiated pleomorphic sarcoma UPS). Annals of Oncology. 2021;32(suppl_5):S1111–S1128.
   Google Scholar
• Toulmonde M, Penel N, Adam J, et al. Use of PD-1 targeting, macrophage infiltration, and IDO pathway activation in sarcomas: a phase 2 clinical trial. JAMA Oncol. 2018 Jan 1;4(1):93–97.
   PubMed Web of Science ®Google Scholar
• Petitprez F, de Reyniès A, Keung EZ, et al. B cells are associated with survival and immunotherapy response in sarcoma. Nature. 2020;577(7791):556–560.
   PubMed Web of Science ®Google Scholar
• Italiano A, Bessede A, Bompas E, et al. PD1 inhibition in soft-tissue sarcomas with tertiary lymphoid structures: a multicenter phase II trial. J Clin Oncol. 2021May20;39(15_suppl):11507.
   Web of Science ®Google Scholar
• Pink D, Andreou D, Flörcken A, et al. Efficacy and safety of nivolumab and trabectedin in pretreated patients with advanced soft tissue sarcomas (STS): preliminary results of a phase II trial of the german interdisciplinary sarcoma group (GISG-15, NitraSarc) for the non-L sarcoma cohort. J Clin Oncol. 2021May20;39(15_suppl):11545.
   Web of Science ®Google Scholar
• Hoy SM. Tazemetostat : first Approval. Drugs. 2020;80(5):513–521.
   PubMed Web of Science ®Google Scholar
• Thway K, Jones RL, Noujaim J, et al. Epithelioid sarcoma: diagnostic features and genetics. Adv Anat Pathol. 2016 Jan;23(1):41–49.
   PubMed Web of Science ®Google Scholar
• Gulati N, Béguelin W, Giulino-Roth L. Enhancer of zeste homolog 2 (EZH2) inhibitors. Leuk Lymphoma. 2018 Jul;59(7):1574–1585.
   PubMed Web of Science ®Google Scholar
• Morschhauser F, Tilly H, Chaidos A, et al. Tazemetostat for patients with relapsed or refractory follicular lymphoma: an open-label, single-arm, multicentre, phase 2 trial. Lancet Oncol. 2020;21(11):1433–1442.
   PubMed Web of Science ®Google Scholar
• Gounder M, Schöffski P, Jones RL, et al. Tazemetostat in advanced epithelioid sarcoma with loss of INI1/SMARCB1: an international, open-label, phase 2 basket study. Lancet Oncol. 2020;21(11):1423–1432.
   PubMed Web of Science ®Google Scholar
• Jones RL, Blay J-Y, Agulnik M, et al. A phase II, multicenter study of the EZH2 inhibitor tazemetostat in adults (rhabdoid tumor cohort) (NCT02601950). Ann Oncol. 2018 Oct 1;29:viii580–1.
   Web of Science ®Google Scholar
• Schoffski P, Agulnik M, Stacchiotti S, et al. Phase 2 multicenter study of the EZH2 inhibitor tazemetostat in adults with synovial sarcoma (NCT02601950). J Clin Oncol. 2017 May 20;35(15_suppl):11057.
   Google Scholar
• FDA approves tazemetostat for advanced epithelioid sarcoma. [cited 2021 Sep 2]. Available from: https://www.fda.gov/drugs/resources-information-approved-drugs/fda-approves-tazemetostat-advanced-epithelioid-sarcoma
   Google Scholar
• Sen S, McKean MA, Sierra L, et al. A phase Ib/III randomized, double-blind, placebo-controlled study of tazemetostat plus doxorubicin as frontline therapy for patients with advanced epithelioid sarcoma. J Clin Oncol. 2020May20;38(15_suppl):TPS11573TPS11573.
   Web of Science ®Google Scholar
• Bahleda R, Wainberg ZA, Spreafico A, et al. 1525MO phase I/II study of MAK683 in patients (pts) with advanced malignancies including epithelioid sarcoma. Ann Oncol. 2021;32:S1113–4.
   Web of Science ®Google Scholar
• Xu Y, Yan W, Chen X. SNF5, a core component of the SWI/SNF complex, is necessary for p53 expression and cell survival, in part through eIF4E. Oncogene. 2010;29(28):4090–4100.
   PubMed Web of Science ®Google Scholar
• McBride MJ, Kadoch C. Disruption of mammalian SWI/SNF and polycomb complexes in human sarcomas: mechanisms and therapeutic opportunities. J Pathol. 2018 Apr;244(5):638–649.
   PubMed Web of Science ®Google Scholar
• Wang X, Bove AM, Simone G, et al. Molecular bases of VEGFR-2-mediated physiological function and pathological role. Front Cell Dev Biol. 2020;8: 1314.
   Web of Science ®Google Scholar
• Syed YY. Anlotinib: first global approval.Drugs.2018;78(10):1057–1062.
   PubMed Web of Science ®Google Scholar
• Xie C, Wan X, Quan H, et al. Preclinical characterization of anlotinib, a highly potent and selective vascular endothelial growth factor receptor-2 inhibitor. Cancer Sci. 2018 Apr;109(4):1207–1219.
   PubMed Web of Science ®Google Scholar
• Tang L, Yu W, Wang Y, et al. Anlotinib inhibits synovial sarcoma by targeting GINS1: a novel downstream target oncogene in progression of synovial sarcoma. Clin Transl Oncol Off Publ Fed Spanish Oncol Soc Natl Cancer Inst Mex. 2019 Dec;21(12):1624–1633.
   Google Scholar
• Tang L, Wang Y, Zhang J, et al. Efficacy and safety of anlotinib in advanced soft tissue sarcoma: results from one of multi-centers in a phase IIB trial (ALTER0203). J Clin Oncol. 2019 May 20;37(15_suppl):e22518–e22518.
   Google Scholar
• Van Tine BA, Chawla SP, Trent JC, et al. A phase III study (APROMISS) of AL3818 (Catequentinib, Anlotinib) hydrochloride monotherapy in subjects with metastatic or advanced synovial sarcoma. J Clin Oncol. 2021 May 20;39(15_suppl):11505.
   Web of Science ®Google Scholar
• Garon EB, Ciuleanu T-E, Arrieta O, et al. Ramucirumab plus docetaxel versus placebo plus docetaxel for second-line treatment of stage IV non-small-cell lung cancer after disease progression on platinum-based therapy (REVEL): a multicentre, double-blind, randomised phase 3 trial.London England: Lancet. 2014 Aug. Vol. 384(9944). p. 665–673.
   Google Scholar
• Khan U, Shah MA. Ramucirumab for the treatment of gastric or gastro-esophageal junction cancer. Expert Opin Biol Ther. 2019 Nov;19(11):1135–1141.
   PubMed Web of Science ®Google Scholar
• Demetri GD, Von Mehren M, Jones RL, et al. Efficacy and safety of trabectedin or dacarbazine for metastatic liposarcoma or leiomyosarcoma after failure of conventional chemotherapy: results of a phase III randomized multicenter clinical trial. J Clin Oncol. 2016;34(8):786–793.
   PubMed Web of Science ®Google Scholar
• Pautier P, Floquet A, Chevreau C, et al. Trabectedin in combination with doxorubicin for first-line treatment of advanced uterine or soft-tissue leiomyosarcoma (LMS-02): a non-randomised, multicentre, phase 2 trial. Lancet Oncol. 2015;16(4):457–464.
   PubMed Web of Science ®Google Scholar
• Pautier P, Floquet A, Chevreau C, et al. A single-arm multicentre phase II trial of doxorubicin in combination with trabectedin in the first-line treatment for leiomyosarcoma with long-term follow-up and impact of cytoreductive surgery. ESMO Open. 2021;6(4):100209.
   PubMed Web of Science ®Google Scholar
• Pautier P, Italiano A, Piperno-Neumann S, et al. Doxorubicin alone versus doxorubicin with trabectedin followed by trabectedin alone as first-line therapy for metastatic or unresectable leiomyosarcoma (LMS-04): a randomised, multicentre, open-label phase 3 trial. Lancet Oncol. 2022 Aug 1;23(8):1044–1054.
   PubMed Web of Science ®Google Scholar
• Pautier P, Italiano A, Piperno-Neumann S, et al. LBA59 - LMS-04 study: a randomised, multicenter, phase III study comparing doxorubicin alone versus doxorubicin with trabectedin followed by trabectedin in non-progressive patients as first-line therapy, in patients with metastatic or unresectable leiomyosarcoma - A French Sarcoma Group study. Annals of Oncology. 2021;32(suppl_5): S1283–S1346.
   Google Scholar
• Li X, Heyer WD. Homologous recombination in DNA repair and DNA damage tolerance. Cell Res. 2008;18(1):99–113.
   PubMed Web of Science ®Google Scholar
• Oza J, Doshi SD, Hao L, et al. Homologous recombination repair deficiency as a therapeutic target in sarcoma. Semin Oncol. 2020;47(6):380–389.
   PubMed Web of Science ®Google Scholar
• Rosenbaum E, Jonsson P, Seier K, et al. Clinical outcome of leiomyosarcomas with somatic alteration in homologous recombination pathway genes. JCO Precis Oncol. 2020 Nov;1;(4:1350–1360.
   Web of Science ®Google Scholar
• Ingham MA, McGuinness JE, Kalinsky K, et al. Exceptional response to dacarbazine in uterine leiomyosarcoma with homozygous BRCA2 deletion highlights the role of homologous recombination in response to DNA damage from alkylating agents. JCO Precis Oncol. 2018;2(2):1–6.
   PubMedGoogle Scholar
• Grignani G, D’Ambrosio L, Pignochino Y, et al. Trabectedin and olaparib in patients with advanced and non-resectable bone and soft-tissue sarcomas (TOMAS): an open-label, phase 1b study from the Italian sarcoma group. Lancet Oncol. 2018;19(10):1360–1371.
   PubMed Web of Science ®Google Scholar
• Ingham M, Allred JB, Gano K, et al. NCI protocol 10250: a phase II study of temozolomide and olaparib for the treatment of advanced uterine leiomyosarcoma. J Clin Oncol. 2020 May 20; 38(15_suppl):TPS11570TPS11570.
   Web of Science ®Google Scholar
• Edris B, Weiskopf K, Volkmer AK, et al. Antibody therapy targeting the CD47 protein is effective in a model of aggressive metastatic leiomyosarcoma. Proc Natl Acad Sci U S A. 2012;109(17):6656–6661.
   PubMed Web of Science ®Google Scholar
• Lee CH, Espinosa I, Vrijaldenhoven S, et al. Prognostic significance of macrophage infiltration in leiomyosarcomas. Clin Cancer Res. 2008;14(5):1423–1430.
   PubMed Web of Science ®Google Scholar
• Petrova PS, Viller NN, Wong M, et al. TTI-621 (SIRPαFc): a CD47-blocking innate immune checkpoint inhibitor with broad antitumor activity and minimal erythrocyte binding. Clin Cancer Res. 2017 Feb 15;23(4):1068–1079.
   PubMed Web of Science ®Google Scholar
• Ansell SM, Maris MB, Lesokhin AM, et al. Phase I study of the CD47 blocker TTI-621 in patients with relapsed or refractory hematologic malignancies. Clin Cancer Res an off J Am Assoc Cancer Res. 2021 Apr;27(8):2190–2199.
   PubMed Web of Science ®Google Scholar
• McCracken MN, Cha AC, Weissman IL. Molecular pathways: activating T cells after cancer cell phagocytosis from blockade of CD47 “don’t eat me” signals. Clin Cancer Res. 2015;21(16):3597–3601.
   PubMed Web of Science ®Google Scholar
• Zhang W, Huang Q, Xiao W, et al. Advances in anti-tumor treatments targeting the CD47/SIRPα axis. Front Immunol. 2020;11:18.
   PubMed Web of Science ®Google Scholar
• Weiskopf K, Jahchan NS, Schnorr PJ, et al. CD47-blocking immunotherapies stimulate macrophage-mediated destruction of small-cell lung cancer. J Clin Invest. 2016 Jul;126(7):2610–2620.
   PubMed Web of Science ®Google Scholar
• Oronsky B, Carter C, Reid T, et al. Just eat it: a review of CD47 and SIRP-α antagonism. Semin Oncol. 2020;47(2–3):117–124.
   PubMed Web of Science ®Google Scholar
• Petrova PS, Viller NN, Wong M, et al. TTI-621 (SIRPαFc): a CD47-blocking innate immune checkpoint inhibitor with broad antitumor activity and minimal erythrocyte binding. Clin Cancer Res an off J Am Assoc Cancer Res. 2017 Feb;23(4):1068–1079.
   PubMed Web of Science ®Google Scholar
• Rosenberg A, Agulnik M. Epithelioid hemangioendothelioma: update on diagnosis and treatment. Curr Treat Options Oncol. 2018 Mar;19(4):19.
   PubMed Web of Science ®Google Scholar
• Stacchiotti S, Miah AB, Frezza AM, et al. Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts. ESMO open. 2021;6(3):100170.
   PubMed Web of Science ®Google Scholar
• Frezza AM, Ravi V, Lo Vullo S, et al. Systemic therapies in advanced epithelioid haemangioendothelioma: a retrospective international case series from the world sarcoma network and a review of literature. Cancer Med. 2021;10(8):2645–2659.
   PubMed Web of Science ®Google Scholar
• Smrke AM, Huang PH, Jones RL. Sirolimus for patients with progressive epithelioid hemangioendothelioma. Cancer. 2021;127(4):504–506.
   PubMed Web of Science ®Google Scholar
• Robert C, Karaszewska B, Schachter J, et al. Improved overall survival in melanoma with combined dabrafenib and trametinib. N Engl J Med. 2015 Jan;372(1):30–39.
   PubMed Web of Science ®Google Scholar
• Schuetze S, Ballman KV, Ganjoo KN, et al. P10015/SARC033: a phase 2 trial of trametinib in patients with advanced epithelioid hemangioendothelioma (EHE). J Clin Oncol. 2021 May 20;39(15_suppl):11503.
   Web of Science ®Google Scholar
• Painter CA, Jain E, Tomson BN, et al. The angiosarcoma project: enabling genomic and clinical discoveries in a rare cancer through patient-partnered research. Nat Med. 2020;26(2):181–187.
   PubMed Web of Science ®Google Scholar
• Kollár A, Jones RL, Stacchiotti S, et al. Pazopanib in advanced vascular sarcomas: an EORTC Soft Tissue and Bone Sarcoma Group (STBSG) retrospective analysis. Acta Oncol. 2017 Jan;56(1):88–92.
   PubMed Web of Science ®Google Scholar
• Penel N, Bui BN, Bay J-O, et al. Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX study. J Clin Oncol Off J Am Soc Clin Oncol. 2008 Nov;26(32):5269–5274.
   PubMed Web of Science ®Google Scholar
• Skubitz KM, Haddad PA. Paclitaxel and pegylated-liposomal doxorubicin are both active in angiosarcoma. Cancer. 2005 Jul;104(2):361–366.
   PubMed Web of Science ®Google Scholar
• Jones RL, Ravi V, Brohl AS, et al. Results of the TAPPAS trial: an adaptive enrichment phase III trial of TRC105 and pazopanib (P) versus pazopanib alone in patients with advanced angiosarcoma (AS). Ann Oncol. 2019Oct1;30:v683. v683
   Google Scholar
• Mehta CR, Liu L, Theuer C. An adaptive population enrichment phase III trial of TRC105 and pazopanib versus pazopanib alone in patients with advanced angiosarcoma (TAPPAS trial). Ann Oncol. 2019;30(1):103–108.
   PubMed Web of Science ®Google Scholar
• Florou V, Rosenberg AE, Wieder E, et al. Angiosarcoma patients treated with immune checkpoint inhibitors: a case series of seven patients from a single institution. J Immunother Cancer. 2019 Aug;7(1):213.
   PubMed Web of Science ®Google Scholar
• Espejo-Freire AP, Elliott A, Rosenberg A, et al. Genomic landscape of angiosarcoma: a targeted and immunotherapy biomarker analysis. Cancers (Basel). 2021 Sep;13(19):4816.
   PubMed Web of Science ®Google Scholar
• Ravi V, Wagner M, Chen T-W-W, et al. A phase II study of oraxol in the treatment of unresectable cutaneous angiosarcoma. J Clin Oncol. 2020 May 20;38(15_suppl):11517.
   Web of Science ®Google Scholar
• Ladanyi M, Lui MY, Antonescu CR, et al. The der(17)t(X;17)(p11;q25) of human alveolar soft part sarcoma fuses the TFE3 transcription factor gene to ASPL, a novel gene at 17q25. Oncogene. 2001 Jan;20(1):48–57.
   PubMed Web of Science ®Google Scholar
• Aulmann S, Longerich T, Schirmacher P, et al. Detection of the ASPSCR1-TFE3 gene fusion in paraffin-embedded alveolar soft part sarcomas. Histopathology. 2007 Jun;50(7):881–886.
   PubMed Web of Science ®Google Scholar
• Lazar AJF, Das P, Tuvin D, et al. Angiogenesis-promoting gene patterns in alveolar soft part sarcoma. Clin Cancer Res an off J Am Assoc Cancer Res. 2007 Dec;13(24):7314–7321.
   PubMed Web of Science ®Google Scholar
• Ghose A, Tariq Z, Veltri S. Treatment of multidrug resistant advanced alveolar soft part sarcoma with sunitinib. Am J Ther. 2012 Jan;19(1):e56–8.
   PubMed Web of Science ®Google Scholar
• Stacchiotti S, Mir O, Le Cesne A, et al. Activity of pazopanib and trabectedin in advanced alveolar soft part sarcoma. Oncologist. 2018 Jan;23(1):62–70.
   PubMed Web of Science ®Google Scholar
• Judson I, Morden JP, Kilburn L, et al. Cediranib in patients with alveolar soft-part sarcoma (CASPS): a double-blind, placebo-controlled, randomised, phase 2 trial. Lancet Oncol. 2019 Jul;20(7):1023–1034.
   PubMed Web of Science ®Google Scholar
• Yang J, Yan J, Liu B. Targeting VEGF/VEGFR to modulate antitumor immunity. Front Immunol. 2018;9:978.
   PubMed Web of Science ®Google Scholar
• Wilky BA, Trucco MM, Subhawong TK, et al. Axitinib plus pembrolizumab in patients with advanced sarcomas including alveolar soft-part sarcoma: a single-centre, single-arm, phase 2 trial. Lancet Oncol. 2019;20(6):837–848.
   PubMed Web of Science ®Google Scholar
• Naqash AR, O’Sullivan Coyne GH, Moore N, et al. Phase II study of atezolizumab in advanced alveolar soft part sarcoma (ASPS). J Clin Oncol. 2021May20;39(15_suppl):11519.
   Web of Science ®Google Scholar
• Blay J-Y, Penel N, Ray-Coquard IL, et al. High clinical benefit rates of pembrolizumab in very rare sarcoma histotypes: first results of the AcSé pembrolizumab study. Ann Oncol. 2019 Oct 1;30:v517.
   Web of Science ®Google Scholar
• Liu J, Fan Z, Guo W, et al. Efficacy and safety of anlotinib plus TQB2450 in patients with advanced soft tissue sarcoma: a multicenter, single armed, phase 1b trial. J Clin Oncol. 2021 May 20;39(15_suppl):11547.
   Google Scholar
• Thway K, Fisher C. PEComa: morphology and genetics of a complex tumor family. Ann Diagn Pathol. 2015 Oct;19(5):359–368.
   PubMed Web of Science ®Google Scholar
• Sanfilippo R, Jones RL, Blay J-Y, et al. Role of chemotherapy, VEGFR inhibitors, and mTOR inhibitors in advanced perivascular epithelioid cell tumors (PEComas). Clin Cancer Res an off J Am Assoc Cancer Res. 2019 Sep;25(17):5295–5300.
   PubMed Web of Science ®Google Scholar
• Wagner AJ, Ravi V, Riedel RF, et al. Nab -sirolimus for patients with malignant perivascular epithelioid cell tumors . J Clin Oncol. 2021;39(33):3660–3670.
   PubMed Web of Science ®Google Scholar
• Kopparthy P, Murphy M. Rapid and durable response with nab-sirolimus after everolimus failure in a patient with perivascular epithelioid cell tumors (PEComas) of the uterus. Cureus. 2021;13(5):11–14.
   Web of Science ®Google Scholar
• FDA approves sirolimus protein-bound particles for malignant perivascular epithelioid cell tumore. fda.gov. [cited 2021 Dec 2]. Available from: https://www.fda.gov/drugs/resources-information-approved-drugs/fda-approves-sirolimus-protein-bound-particles-malignant-perivascular-epithelioid-cell-tumor
   Google Scholar
• Martínez Trufero J, Pajares Bernad I, Torres Ramón I, et al. Desmoid-type fibromatosis: who, when, and how to treat. Curr Treat Options Oncol. 2017 May;18(5):29.
   PubMed Web of Science ®Google Scholar
• Kasper B, Baumgarten C, Garcia J, et al. An update on the management of sporadic desmoid-type fibromatosis: a European consensus initiative between sarcoma patients EuroNet (SPAEN) and european organization for research and treatment of cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG). Ann Oncol.2017;28(10):2399–2408.
   PubMed Web of Science ®Google Scholar
• Penel N, Chibon F, Salas S. Adult desmoid tumors: biology, management and ongoing trials. Curr Opin Oncol. 2017 Jul;29(4):268–274.
   PubMed Web of Science ®Google Scholar
• Kim H-A, Koo B-K, Cho J-H, et al. Notch1 counteracts WNT/β-catenin signaling through chromatin modification in colorectal cancer. J Clin Invest. 2012 Sep;122(9):3248–3259.
   PubMed Web of Science ®Google Scholar
• Shang H, Braggio D, Lee Y-J, et al. Targeting the Notch pathway: a potential therapeutic approach for desmoid tumors. Cancer. 2015/Sept/08. 2015 Nov 15;121(22):4088–4096.
   PubMed Web of Science ®Google Scholar
• Villalobos VM, Hall F, Jimeno A, et al. Long-term follow-up of desmoid fibromatosis treated with PF-03084014, an oral gamma secretase inhibitor. Ann Surg Oncol. 2018 Mar;25(3):768–775.
   PubMed Web of Science ®Google Scholar
• Kummar S, O’Sullivan Coyne G, Do KT, et al. Clinical activity of the γ-secretase inhibitor PF-03084014 in adults with desmoid tumors (aggressive fibromatosis). J Clin Oncol. 2017Mar28;Internet]; 35(14):1561–1569.
   PubMed Web of Science ®Google Scholar
• Reck M, Kaiser R, Mellemgaard A, et al. Docetaxel plus nintedanib versus docetaxel plus placebo in patients with previously treated non-small-cell lung cancer (LUME-Lung 1): a phase 3, double-blind, randomised controlled trial. Lancet Oncol. 2014 Feb;15(2):143–155.
   PubMed Web of Science ®Google Scholar
• Schöffski P, Toulmonde M, Estival A, et al. Randomised phase 2 study comparing the efficacy and safety of the oral tyrosine kinase inhibitor nintedanib with single agent ifosfamide in patients with advanced, inoperable, metastatic soft tissue sarcoma after failure of first-line chemotherapy: EORTC-. Eur J Cancer. 2021;152:26–40.
   PubMed Web of Science ®Google Scholar
• Borsi L, Balza E, Carnemolla B, et al. Selective targeted delivery of TNFα to tumor blood vessels. Blood. 2003;102(13):4384–4392.
   PubMed Web of Science ®Google Scholar
• Schliemann C, Hemmerle T, Berdel AF, et al. Dose escalation and expansion phase I studies with the tumour-targeting antibody-tumour necrosis factor fusion protein L19TNF plus doxorubicin in patients with advanced tumours, including sarcomas. Eur J Cancer. 2021;150:143–154.
   PubMed Web of Science ®Google Scholar
• Digklia A, Kollár A, Kronig M-N, et al. Paper 48 - Sakk 57/16 Nnab-Paclitaxel And Gemcitabine In Soft Tissue Sarcoma (NAPAGE): reslts from phase IB/II trial. In: CTOS. 2021.
   Google Scholar
• Rodrigues C, Macedo LC, Bruder AV, et al. Allele and haplotype frequencies of HLA-A, B, C, DRB1 and DQB1 genes in polytransfused patients in ethnically diverse populations from Brazil. Int J Immunogenet. 2015 Oct;42(5):322–328.
   PubMed Web of Science ®Google Scholar
• Ellis JM, Henson V, Slack R, et al. Frequencies of HLA-A2 alleles in five U.S. population groups: predominance of A*02011 and identification of HLA-A*0231. Hum Immunol. 2000;61(3):334–340.
   PubMed Web of Science ®Google Scholar
• Tshabalala M, Mellet J, Pepper MS. Human leukocyte antigen diversity: a Southern African perspective. J Immunol Res. 2015/Aug/12. 2015:746151.
   PubMed Web of Science ®Google Scholar
• Janse van Rensburg WJ, de Kock A, Bester C, et al. HLA major allele group frequencies in a diverse population of the Free State Province, South Africa. Heliyon. 2021;7(4):e06850
   PubMed Web of Science ®Google Scholar
• Wilding CP, Elms ML, Judson I, et al. The landscape of tyrosine kinase inhibitors in sarcomas: looking beyond pazopanib. Expert Rev Anticancer Ther. 2019/Nov/13. 2019 Nov;19(11):971–991
   PubMed Web of Science ®Google Scholar
• Cheng Y, Cui H, Wu C, et al. 532MO A phase Ib study of TQB2450 in combination with anlotinib in patients with advanced solid tumour. Ann Oncol. 2020;31:S467.
   Google Scholar
• Xu Q, Wang J, Sun Y, et al. Efficacy and safety of sintilimab plus anlotinib for PD-L1–positive recurrent or metastatic cervical cancer: a multicenter, single-arm, prospective phase II trial. J Clin Oncol. 2022 Feb 22;JCO.21.02091.
   Google Scholar
• Yang S, Zhang W, Chen Q, et al. Clinical investigation of the efficacy and safety of anlotinib with immunotherapy in advanced non-small cell lung cancer as third-line therapy: a retrospective study. Cancer Manag Res. 2020 Oct 19;12:10333–10340
   PubMed Web of Science ®Google Scholar
• van der Graaf WTA, Blay J-Y, Chawla SP, et al. Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet. 2012 May 19;379(9829):1879–1886
   PubMed Web of Science ®Google Scholar