Personalised pharmacotherapy options for soft tissue sarcomas

References

• The WHO Classification of Tumours Editorial Board. WHO Classification of Tumours Soft Tissue and Bone Tumours. 5th ed. Lyon: IARC Press; 2020.
   Google Scholar
• Casali PG, Abecassis N, Bauer S, et al. Soft tissue and visceral sarcomas: ESMO-EURACAN clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018;29(May):iv51–iv67.
   PubMedGoogle Scholar
• Gamboa AC, Gronchi A, Cardona K. Soft‐tissue sarcoma in adults: an update on the current state of histiotype‐specific management in an era of personalized medicine. CA Cancer J Clin. 2020;70(3):200–229.
   PubMed Web of Science ®Google Scholar
• Schaefer I-M, Cote GM, Hornick JL. Contemporary sarcoma diagnosis, genetics, and genomics. J Clin Oncol. 2017;36(2):101–110.
   PubMed Web of Science ®Google Scholar
• Dei Tos AP. A current perspective on the role for molecular studies in soft tissue tumor pathology. Semin Diagn Pathol. 2013;30(4):375–381.
   PubMed Web of Science ®Google Scholar
• Demetri GD, Antonescu CR, Bjerkehagen B, et al. Diagnosis and management of tropomyosin receptor kinase (TRK) fusion sarcomas: expert recommendations from the world sarcoma network. Ann Oncol. 2020;31(11):1506–1517.
   PubMed Web of Science ®Google Scholar
• Sbaraglia M, Bellan E, Dei Tos AP. Perspectives, The 2020 WHO classification of soft tissue tumours: news and. Pathologica. 2021;113(2):70–84.
   PubMed Web of Science ®Google Scholar
• Antonescu CR. Emerging soft tissue tumors with kinase fusions: an overview of the recent literature with an emphasis on diagnostic criteria. Genes Chromosom Cancer. 2020;59(8):437–444.
   PubMed Web of Science ®Google Scholar
• Miettinen M, Lasota J. Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis. Arch Pathol Lab Med. 2006;130(10):1466–1478.
   PubMed Web of Science ®Google Scholar
• Gasparotto D, Rossi S, Polano M, et al. Quadruple-negative GIST is a sentinel for unrecognized neurofibromatosis type 1 syndrome. Clin Cancer Res. 2017;23(1):273–282.
   PubMed Web of Science ®Google Scholar
• Cassier PA, Dufresne A, Arifi S, et al. Imatinib mesilate for the treatment of gastrointestinal stromal tumour. Expert Opin Pharmacother. 2008;9(7):1211–1222.
   PubMed Web of Science ®Google Scholar
• Skubitz KM. Biology and treatment of aggressive fibromatosis or desmoid tumor. Mayo Clin Proc. 2017;92(6):947–964.
   PubMed Web of Science ®Google Scholar
• Nacev BA, Jones KB, Intlekofer AM, et al. The epigenomics of sarcoma. Nat Rev Cancer. 2020;20(10):608–623.
   PubMed Web of Science ®Google Scholar
• Heilig CE, Horak P, Lipka DB, et al. Germline SDHB-inactivating mutation in gastric spindle cell sarcoma. Genes Chromosom Cancer. 2020;59(10):601–608.
   PubMed Web of Science ®Google Scholar
• Mariño-Enríquez A, Bovée JVMG. Molecular pathogenesis and diagnostic, prognostic and predictive molecular markers in sarcoma. Surg Pathol Clin. 2016;9(3):457–473.
   PubMedGoogle Scholar
• Samstein RM, Lee C-H, Shoushtari AN, et al. Tumor mutational load predicts survival after immunotherapy across multiple cancer types. Nat Genet. 2019;51(2):202–206.
   PubMed Web of Science ®Google Scholar
• Klaver Y, Rijnders M, Oostvogels A, et al. Differential quantities of immune checkpoint-expressing CD8 T cells in soft tissue sarcoma subtypes. J Immunother Cancer. 2020;8(2):1–11.
   Web of Science ®Google Scholar
• Thomas DM, Ballinger ML. Etiologic, environmental and inherited risk factors in sarcomas. J Surg Oncol. 2015;111(5):490–495.
   PubMed Web of Science ®Google Scholar
• Upton B, Chu Q, Li BDL. Li-Fraumeni syndrome: the genetics and treatment considerations for the sarcoma and associated neoplasms. Surg Oncol Clin N Am. 2009;18(1):145–156.
   PubMed Web of Science ®Google Scholar
• Galiatsatos P, Foulkes WD. Familial adenomatous polyposis. Am J Gastroenterol. 2006;101(2):385–398.
   PubMed Web of Science ®Google Scholar
• Fallen T, Wilson M, Morlan B, et al. Desmoid tumors - A characterization of patients seen at mayo clinic 1976-1999. Fam Cancer. 2006;5(2):191–194.
   PubMed Web of Science ®Google Scholar
• Postow MA, Robson ME. Inherited gastrointestinal stromal tumor syndromes: mutations, clinical features, and therapeutic implications. Clin Sarcoma Res. 2012;2(1):1.
   PubMedGoogle Scholar
• Brems H, Beert E, de Ravel T, et al. Mechanisms in the pathogenesis of malignant tumours in neurofibromatosis type 1. Lancet Oncol. 2009;10(5):508–515.
   PubMed Web of Science ®Google Scholar
• Kleinerman RA, Tucker MA, Abramson DH, et al. Risk of soft tissue sarcomas by individual subtype in survivors of hereditary retinoblastoma. J Natl Cancer Inst. 2007;99(1):24–31.
   PubMedGoogle Scholar
• Scurr M. Histology-Driven chemotherapy in soft tissue sarcomas. Curr Treat Options Oncol. 2011;12(1):32–45.
   PubMed Web of Science ®Google Scholar
• Blay JY, Sleijfer S, Schöffski P, et al. International expert opinion on patient-tailored management of soft tissue sarcomas. Eur J Cancer. 2014;50(4):679–689.
   PubMed Web of Science ®Google Scholar
• Jones RL, Fisher C, Al-Muderis O, et al. Differential sensitivity of liposarcoma subtypes to chemotherapy. Eur J Cancer. 2005;41(18):2853–2860.
   PubMed Web of Science ®Google Scholar
• Arifi S, Belbaraka R, Rahhali R, et al. Treatment of adult soft tissue sarcomas: an overview. Rare Cancers Ther. 2015;3(1–2):69–87.
   PubMedGoogle Scholar
• Judson I, Verweij J, Gelderblom H, et al. Doxorubicin alone versus intensified doxorubicin plus ifosfamide for first-line treatment of advanced or metastatic soft-tissue sarcoma: a randomised controlled phase 3 trial. Lancet Oncol. 2014;15(4):415–423.
   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;10065(13):1266–1276.
   Google Scholar
• D’Ambrosio L, Touati N, Blay JY, et al. Doxorubicin plus dacarbazine, doxorubicin plus ifosfamide, or doxorubicin alone as a first-line treatment for advanced leiomyosarcoma: a propensity score matching analysis from the european organization for research and treatment of cancer soft tissue and. Cancer. 2020;126(11):2637–2647.
   PubMed Web of Science ®Google Scholar
• Penel N, Bui BN, Bay JO, et al. Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX study. J Clin Oncol. 2008;26(32):5269–5274.
   PubMed Web of Science ®Google Scholar
• Schöffski P, Chawla S, Maki RG, et al. Eribulin versus dacarbazine in previously treated patients with advanced liposarcoma or leiomyosarcoma: a randomised, open-label, multicentre, phase 3 trial. Lancet. 2016;387(10028):1629–1637.
   PubMed Web of Science ®Google Scholar
• Demetri GD, Schöffski P, Grignani G, et al. Activity of eribulin in patients with advanced liposarcoma demonstrated in a subgroup analysis from a randomized phase III study of eribulin versus dacarbazine. J Clin Oncol. 2017;35(30):3433–3439.
   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
• Grosso F, Jones RL, Demetri GD, et al. Efficacy of trabectedin (ecteinascidin-743) in advanced pretreated myxoid liposarcomas: a retrospective study. Lancet Oncol. 2007;8(7):595–602.
   PubMed Web of Science ®Google Scholar
• Takahashi M, Takahashi S, Araki N, et al. Efficacy of trabectedin in patients with advanced translocation‐related sarcomas: pooled analysis of two phase II studies. Oncologist. 2017;22(8):979–988.
   PubMed Web of Science ®Google Scholar
• Le Cesne A, Ray-Coquard I, Duffaud F, et al. Trabectedin in patients with advanced soft tissue sarcoma: a retrospective national analysis of the french sarcoma group. Eur J Cancer. 2015;51(6):742–750.
   PubMed Web of Science ®Google Scholar
• Recine F, Bongiovanni A, Riva N, et al. Update on the role of trabectedin in the treatment of intractable soft tissue sarcomas. Onco Targets Ther. 2017;10:1155–1164.
   PubMed Web of Science ®Google Scholar
• Grosso F, D’Ambrosio L, Zucchetti M, et al. Pharmacokinetics, safety, and activity of trabectedin as first-line treatment in elderly patients who are affected by advanced sarcoma and are unfit to receive standard chemotherapy: a phase 2 study (TR1US study) from the Italian Sarcoma Group. Cancer. 2020;126(21):4726–4734.
   PubMed Web of Science ®Google Scholar
• Jones RL, Demetri GD, Schuetze SM, et al. Efficacy and tolerability of trabectedin in elderly patients with sarcoma: subgroup analysis from a phase III, randomized controlled study of trabectedin or dacarbazine in patients with advanced liposarcoma or leiomyosarcoma. Ann Oncol. 2018;29(9):1995–2002.
   PubMed Web of Science ®Google Scholar
• Le Cesne A, Ouali M, Leahy MG, et al. Doxorubicin-based adjuvant chemotherapy in soft tissue sarcoma: pooled analysis of two STBSG-EORTC phase III clinical trials. Ann Oncol. 2014;25(12):2425–2432.
   PubMed Web of Science ®Google Scholar
• Gronchi A, Ferrari S, Quagliuolo V, et al. Histotype-tailored neoadjuvant chemotherapy versus standard chemotherapy in patients with high-risk soft-tissue sarcomas (ISG-STS 1001): an international, open-label, randomised, controlled, phase 3, multicentre trial. Lancet Oncol. 2017;18(6):812–822.
   PubMed Web of Science ®Google Scholar
• Sleijfer S, Ray-Coquard I, Papai Z, et al. Pazopanib, a multikinase angiogenesis inhibitor, in patients with relapsed or refractory advanced soft tissue sarcoma: a phase II study from the European organisation for research and treatment of cancer-soft tissue and bone sarcoma group (EORTC Study 620. J Clin Oncol. 2009;27(19):3126–3132.
   PubMed Web of Science ®Google Scholar
• Van Der Graaf WTA, Blay JY, Chawla SP, et al. Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet. 2012;379(9829):1879–1886.
   PubMed Web of Science ®Google Scholar
• Mir O, Brodowicz T, Italiano A, et al. Safety and efficacy of regorafenib in patients with advanced soft tissue sarcoma (REGOSARC): a randomised, double-blind, placebo-controlled, phase 2 trial. Lancet Oncol. 2016;17(12):1732–1742.
   PubMed Web of Science ®Google Scholar
• Berry V, Basson L, Bogart E, et al. REGOSARC: regorafenib versus placebo in doxorubicin-refractory soft-tissue sarcoma—A quality-adjusted time without symptoms of progression or toxicity analysis. Cancer. 2017;123(12):2294–2302.
   PubMed Web of Science ®Google Scholar
• Martin-Broto J, Cruz J, Penel N, et al. Pazopanib for treatment of typical solitary fibrous tumours: a multicentre, single-arm, phase 2 trial. Lancet Oncol. 2020;21(3):456–466.
   PubMed Web of Science ®Google Scholar
• Conrad CA, Lazar AJ, Wang W. Activity of temozolomide and bevacizumab in the treatment of locally advanced, recurrent, and metastatic hemangiopericytoma and malignant solitary fibrous tumor. Cancer. 2011;117(21):4939–4947.
   PubMed Web of Science ®Google Scholar
• Maeda O, Ohka F, Maesawa S, et al. Solitary fibrous tumor/hemangiopericytoma treated with temozolomide plus bevacizumab: a report of four cases and literature review. Nagoya J Med Sci. 2020;82(4):631–644.
   PubMed Web of Science ®Google Scholar
• Brodowicz T, Liegl-Atzwanger B, Penel N, et al. Assessing prognostic and predictive biomarkers of regorafenib response in patients with advanced soft tissue sarcoma: regosarc study. Cancers (Basel). 2020;12(12):1–12.
   Web of Science ®Google Scholar
• Tap WD, Jones RL, Van Tine BA, et al. Olaratumab and doxorubicin versus doxorubicin alone in soft tissue sarcoma HHS Public Access. Lancet. 2016;388(10043):488–497.
   PubMed Web of Science ®Google Scholar
• Demetri GD, Chawla SP, Ray-Coquard I, et al. Results of an international randomized phase III trial of the mammalian target of rapamycin Inhibitor ridaforolimus versus placebo to control metastatic sarcomas in patients after benefit from prior chemotherapy. J Clin Oncol. 2013;31(19):2485–2492.
   PubMed Web of Science ®Google Scholar
• Gounder MM, Zer A, Tap WD, et al. Phase IB study of selinexor, a first-in-class inhibitor of nuclear export, in patients with advanced refractory bone or soft tissue sarcoma. J Clin Oncol. 2016;34(26):3166–3174.
   PubMed Web of Science ®Google Scholar
• Gounder M, Abdul Razak AR, Gilligan AM, et al. Health-related quality of life and pain with selinexor in patients with advanced dedifferentiated liposarcoma. Futur Oncol. 2021;17(22):2923–2939.
   PubMed Web of Science ®Google Scholar
• Nakayama R, Zhang YX, Czaplinski JT, et al. Preclinical activity of selinexor, an inhibitor of XPO1, in sarcoma. Oncotarget. 2016;7(13):16581–16592.
   PubMedGoogle Scholar
• Zuco V, Pasquali S, Tortoreto M, et al. Selinexor versus doxorubicin in dedifferentiated liposarcoma PDXs: evidence of greater activity and apoptotic response dependent on p53 nuclear accumulation and survivin down‐regulation. J Exp Clin Cancer Res. 2021;40(1):1–12.
   PubMed Web of Science ®Google Scholar
• Jeitany M, Prabhu A, Dakle P, et al. Novel carfilzomib-based combinations as potential therapeutic strategies for liposarcomas. Cell Mol Life Sci. 2021;78(4):1837–1851.
   PubMed Web of Science ®Google Scholar
• Al-Ezzi E, Gounder M, Watson G, et al. Selinexor, a first in class, nuclear export inhibitor for the treatment of advanced malignant peripheral nerve sheath tumor. Oncologist. 2021;26(4):e710–e714.
   PubMed Web of Science ®Google Scholar
• Siozopoulou V, Domen A, Zwaenepoel K, et al. Immune checkpoint inhibitory therapy in sarcomas: is there light at the end of the tunnel? Cancers (Basel). 2021;13(2):1–20.
   Web of Science ®Google Scholar
• Tawbi HA, Burgess M, Bolejack V, et al. MD11 M. pembrolizumab in advanced soft tissue and bone sarcomas: results of SARC028, A multicentre, single arm, phase 2 trial. Lancet Oncol. 2021;18(11):1493–1501.
   Google Scholar
• Blay J-Y, Chevret S, Penel N, et al. 1619O high clinical benefit rates of single agent pembrolizumab in selected rare sarcoma histotypes: first results of the AcSé pembrolizumab study. Ann Oncol. 2020;31:S972.
   Web of Science ®Google Scholar
• D’Angelo SP, Mahoney MR, Van Tine BA, et al. Nivolumab with or without ipilimumab treatment for metastatic sarcoma (Alliance A091401): two open-label, non-comparative, randomised, phase 2 trials. Lancet Oncol. 2018;19(3):416–426.
   PubMed Web of Science ®Google Scholar
• Lien IC, Pollack SM. Limited activity of metronomic cyclophosphamide and pembrolizumab for soft tissue sarcomas. Transl Gastroenterol Hepatol. 2018;2018(JAN):3–5.
   Google Scholar
• Pollack SM, Redman MW, Baker KK, et al. Assessment of doxorubicin and pembrolizumab in patients with advanced anthracycline-Naive sarcoma A phase 1/2 nonrandomized clinical trial. JAMA Oncol. 2020;98109:1–5.
   Google Scholar
• Toulmonde M, Brahmi M, Giraud A, et al. LBA67 TRAMUNE, a phase Ib study combining trabectedin and durvalumab, results of the expansion cohort in patients with advanced pretreated soft tissue sarcomas. Ann Oncol. 2020;31:S1199.
   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
• Lai JP, Robbins PF, Raffeld M, et al. NY-ESO-1 expression in synovial sarcoma and other mesenchymal tumors: significance for NY-ESO-1-based targeted therapy and differential diagnosis. Mod Pathol. 2012;25(6):854–858.
   PubMed Web of Science ®Google Scholar
• Van Tine BA, Butler MO, Araujo D, et al. ADP-A2M4 (MAGE-A4) in patients with synovial sarcoma. Ann Oncol. 2019;30(October):v684–v685.
   Google Scholar
• Heinrich MC, Jones RL, von Mehren M, et al. Avapritinib in advanced PDGFRA D842V-mutant gastrointestinal stromal tumour (NAVIGATOR): a multicentre, open-label, phase 1 trial. Lancet Oncol. 2020;21(7):935–946.
   PubMed Web of Science ®Google Scholar
• Blay JY, Serrano C, Heinrich MC, et al. Ripretinib in patients with advanced gastrointestinal stromal tumours (INVICTUS): a double-blind, randomised, placebo-controlled, phase 3 trial. Lancet Oncol. 2020;21(7):923–934.
   PubMed Web of Science ®Google Scholar
• Demetri GD, van Oosterom AT, Garrett CR, et al. Efficacy and safety of sunitinib in patients with advanced gastrointestinal stromal tumour after failure of imatinib: a randomised controlled trial. Lancet. 2006;368(9544):1329–1338.
   PubMed Web of Science ®Google Scholar
• Demetri GD, Reichardt P, Kang Y, et al. Efficacy and safety of regorafenib for advanced gastrointestinal stromal tumours after failure of imatinib and sunitinib: an international, multicentre, prospective, randomised, placebo- controlled phase 3 trial (GRID). Lancet. 2013;381(9863):1–17.
   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
• Thway K, Fisher C. PEComa: morphology and genetics of a complex tumor family. Ann Diagn Pathol. 2015;19(5):359–368.
   PubMed Web of Science ®Google Scholar
• Ugure S, Mentzel T, Utikal J, et al. Neoadjuvant imatinib in advanced primary or locally recurrent dermatofibrosarcoma protuberans: a multicenter phase II DeCOG trial with long-term follow-up. Clin Cancer Res. 2014;20(2):499–510.
   PubMed Web of Science ®Google Scholar
• Rutkowski P, Klimczak A, Ługowska I, et al. Long-term results of treatment of advanced dermatofibrosarcoma protuberans (DFSP) with imatinib mesylate – the impact of fibrosarcomatous transformation. Eur J Surg Oncol. 2017;43(6):1134–1141.
   PubMed Web of Science ®Google Scholar
• McArthur GA, Demetri GD, Van Oosterom A, et al. Molecular and clinical analysis of locally advanced dermatofibrosarcoma protuberans treated with imatinib: imatinib target exploration consortium study B2225. J Clin Oncol. 2005;23(4):866–873.
   PubMed Web of Science ®Google Scholar
• Kérob D, Porcher R, Vérola O, et al. Imatinib mesylate as a preoperative therapy in dermatofibrosarcoma: results of a multicenter phase II study on 25 patients. Clin Cancer Res. 2010;16(12):3288–3295.
   PubMed Web of Science ®Google Scholar
• Salgia R, Ph D, Riely GJ, et al. Crizotinib in. N Engl J Med. 2010;363(18):1727–1733.
   PubMedGoogle Scholar
• Nishio M, Murakami H, Horiike A, et al. Phase I study of ceritinib (LDK378) in Japanese patients with advanced, anaplastic lymphoma kinase-rearranged non-small-cell lung cancer or other tumors. J Thorac Oncol. 2015;10(7):1058–1066.
   PubMed Web of Science ®Google Scholar
• Honda K, Kadowaki S, Kato K, et al. Durable response to the ALK inhibitor alectinib in inflammatory myofibroblastic tumor of the head and neck with a novel SQSTM1–ALK fusion: a case report. Invest New Drugs. 2019;37(4):791–795.
   PubMed Web of Science ®Google Scholar
• Xavier CB, Canedo FSNA, Lima FAS, et al. Complete response to alectinib following crizotinib in an ALK-positive inflammatory myofibroblastic tumor with CNS involvement. Curr Probl Cancer Case Reports. 2021;4(September):100117.
   Google Scholar
• Comandini D, Catalano F, Grassi M, et al. Outstanding response in a patient with ros1-rearranged inflammatory myofibroblastic tumor of soft tissues treated with crizotinib: case report. Front Oncol. 2021;11(June):1–9.
   Google Scholar
• Doebele RC, Drilon A, Paz-Ares L, et al. Entrectinib in patients with advanced or metastatic NTRK fusion-positive solid tumours: integrated analysis of three phase 1–2 trials. Lancet Oncol. 2020;21(2):271–282.
   PubMed Web of Science ®Google Scholar
• Bielack SS, Cox MC, Nathrath M, et al. Rapid, complete and sustained tumour response to the TRK inhibitor larotrectinib in an infant with recurrent, chemotherapy-refractory infantile fibrosarcoma carrying the characteristic ETV6-NTRK3 gene fusion. Ann Oncol. 2019;30(Supplement 8):VIII31–VIII35.
   Google Scholar
• Cassier PA, Italiano A, Gomez-Roca C, et al. Long-term clinical activity, safety and patient-reported quality of life for emactuzumab-treated patients with diffuse-type tenosynovial giant-cell tumour. Eur J Cancer. 2020;141:162–170.
   PubMed Web of Science ®Google Scholar
• Tap WD, Gelderblom H, Palmerini E, et al., WAE investigators. Pexidartinib for advanced tenosynovial giant cell tumor: results of the randomized phase 3 ENLIVEN study. Lancet. 2019;394(10197):478–487.
   PubMed Web of Science ®Google Scholar
• Spriano F, Chung EYL, Gaudio E, et al. The ETS inhibitors YK-4-279 and TK-216 are novel antilymphoma agents. Clin Cancer Res. 2019;25(16):5167–5176.
   PubMed Web of Science ®Google Scholar
• Ludwig JA, Federman N, Anderson P, et al. 1620O phase I study of TK216, a novel anti-ETS agent for Ewing sarcoma. Ann Oncol. 2020;31:S972.
   Google Scholar
• Tap WD, Demetri G, Barnette P, et al. Phase II study of ganitumab, a fully human anti-type-1 insulin-like growth factor receptor antibody, in patients with metastatic ewing family tumors or desmoplastic small round cell tumors. J Clin Oncol. 2012;30(15):1849– 1856.
   PubMed Web of Science ®Google Scholar
• Olmos D, Postel-Vinay S, Molife LR, et al. Safety, pharmacokinetics, and preliminary activity of the anti-IGF-1R antibody figitumumab (CP-751,871) in patients with sarcoma and ewing’s sarcoma: a phase 1 expansion cohort study. Lancet Oncol. 2010;11(2):129– 135.
   PubMed Web of Science ®Google Scholar
• Dickson MA, Schwartz GK, Keohan ML, et al. Phase 2 trial of the CDK4 inhibitor palbociclib (PD0332991) at 125 mg dose in well-differentiated or dedifferentiated liposarcoma. JAMA Oncol. 2016;2(7):937–940.
   PubMed Web of Science ®Google Scholar
• Dickson MA, Tap WD, Keohan ML, et al. Phase II trial of the CDK4 inhibitor PD0332991 in patients with advanced CDK4-amplified well-differentiated or dedifferentiated liposarcoma. J Clin Oncol. 2013;31(16):2024–2028.
   PubMed Web of Science ®Google Scholar
• Gahvari Z, Parkes A. Dedifferentiated liposarcoma: systemic therapy options. Curr Treat Options Oncol. 2020;21(2). DOI:10.1007/s11864-020-0705-7
   PubMed Web of Science ®Google Scholar
• Laroche-Clary A, Chaire V, Algeo MP, et al. Combined targeting of MDM2 and CDK4 is synergistic in dedifferentiated liposarcomas. J Hematol Oncol. 2017;10(1):1–10.
   PubMedGoogle Scholar
• Ray-Coquard I, Blay JY, Italiano A, et al. Effect of the MDM2 antagonist RG7112 on the P53 pathway in patients with MDM2-amplified, well-differentiated or dedifferentiated liposarcoma: an exploratory proof-of-mechanism study. Lancet Oncol. 2012;13(11):1133–1140.
   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
• Drilon A, Laetsch TW, Kummar S, et al. Efficacy of larotrectinib in TRK fusion–positive cancers in adults and children. N Engl J Med. 2018;378(8):731–739.
   PubMed Web of Science ®Google Scholar
• Prendergast SC, Strobl AC, Cross W, et al. Sarcoma and the 100,000 genomes project: our experience and changes to practice. J Pathol Clin Res. 2020;6(4):297–307.
   PubMedGoogle Scholar
• Flaherty KT, Gray RJ, Chen AP, et al. Molecular landscape and actionable alterations in a genomically guided cancer clinical trial: national cancer institute molecular analysis for therapy choice (NCI-MATCH). J Clin Oncol. 2020;38(33):3883–3894.
   PubMed Web of Science ®Google Scholar
• Le Tourneau C, Delord JP, Gonçalves A, et al. Molecularly targeted therapy based on tumour molecular profiling versus conventional therapy for advanced cancer (SHIVA): a multicentre, open-label, proof-of-concept, randomised, controlled phase 2 trial. Lancet Oncol. 2015;16(13):1324–1334.
   PubMed Web of Science ®Google Scholar