Opinion statement
The therapeutic approach of pleomorphic liposarcoma (PLPS), a rare high-grade subgroup of soft tissue sarcoma, is commonly extrapolated from the management of other LPS subtypes. Only published retrospective data on PLPS currently serve as a guide for oncologists without clear recommendations or specific guidelines. In the advanced setting, specific systemic therapy such as eribulin and trabectedin showed promising activity in comparison to conventional therapy (doxorubicin- and gemcitabine-based protocols), which currently remains the current standard of care at initial stages of the disease. The better understanding of soft tissue sarcoma (STS) pathophysiology and disease course has led to the development of adapted clinical trial designs for rare STS histotypes with specific treatment approach.
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References and Recommended Reading
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Fletcher CDM, Bridge JA, Hogendoorn PCW. WHO classification of tumours of soft tissue and bone. Lyon: IARC Press; 2013.
Siegel RL, Miller KD, Jemal A. Cancer statistics, 2020. CA Cancer J Clin. 2020;70(1):7–30.
Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F. World Health Organization classification of tumours of soft tissue and bone. 4th ed. Lyon: IARC Press; 2013.
Fletcher CD. The evolving classification of soft tissue tumours–an update based on the new 2013 WHO classification. Histopathology. 2014;64(1):2–11.
Enzinger FM, Winslow DJ. Liposarcoma. Virchows Archiv. 1962;335(4):367–88.
Manji GA, Schwartz GK. Managing liposarcomas: cutting through the fat. J Oncol Pract. 2016;12(3):221–7.
Rudzinski E, Mawn L, Kuttesch J, Wushensky C, Wills M. Orbital pleomorphic liposarcoma in an eight-year-old boy. Pediatr Dev Pathol. 2011;14(4):339–44.
Ghadimi MP, Liu P, Peng T, Bolshakov S, Young ED, Torres KE, et al. Pleomorphic liposarcoma: clinical observations and molecular variables. Cancer. 2011;117(23):5359–69.
Gebhard S, Coindre JM, Michels JJ, Terrier P, Bertrand G, Trassard M, et al. Pleomorphic liposarcoma: clinicopathologic, immunohistochemical, and follow-up analysis of 63 cases: a study from the French Federation of Cancer Centers Sarcoma Group. Am J Surg Pathol. 2002;26(5):601–16.
Tandon B, Hagemann IS, Maluf HM, Pfeifer JD, Al-Kateb H. Association of Li-Fraumeni syndrome with small cell carcinoma of the ovary, hypercalcemic type and concurrent pleomorphic liposarcoma of the cervix. Int J Gynecol Pathol. 2017;36(6):593–9.
Italiano A, Garbay D, Cioffi A, Maki RG, Bui B. Advanced pleomorphic liposarcomas: clinical outcome and impact of chemotherapy. Ann Oncol. 2012;23(8):2205–6.
Toulmonde M, Italiano A, Penel N, Cioffi A, Le Cesne A, Isambert N, et al. Advanced well-differentiated/dedifferentiated liposarcomas: role of chemotherapy and survival. J Clin Oncol. 2011;29(15_suppl):10071–10071.
• Gronchi A, Miah AB, Dei Tos AP, Abecassis N, Bajpai J, Bauer S, et al. Soft tissue and visceral sarcomas: ESMO–EURACAN–GENTURIS clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2021;32(11):1348–65. Most recent recommendations for the management of soft tissue sarcomas.
Casali PG, Bruzzi P, Bogaerts J, Blay JY, Panel RCE (RCE) C. Rare Cancers Europe (RCE) methodological recommendations for clinical studies in rare cancers: a European consensus position paper. Ann Oncol. 2014;26(2):300–6.
Frezza AM, Jones RL, Vullo SL, Asano N, Lucibello F, Ben-Ami E, et al. Anthracycline, gemcitabine, and pazopanib in epithelioid sarcoma: a multi-institutional case series. JAMA Oncol. 2018;4(9):e180219–e180219.
Frezza AM, Assi T, Lo Vullo S, Ben-Ami E, Dufresne A, Yonemori K, et al. Systemic treatments in MDM2 positive intimal sarcoma: a multicentre experience with anthracycline, gemcitabine, and pazopanib within the World Sarcoma Network. Cancer. 2020;126(1):98–104.
• Smrke A, Frezza AM, Giani C, Somaiah N, Brahmi M, Czarnecka AM, et al. Systemic treatment of advanced clear cell sarcoma: results from a retrospective international series from the World Sarcoma Network. ESMO open. 2022;7(3): 100522. International multicentric retrospective data on one of the most rare STS histotypes, clear cell sarcoma, assessing the role of current systemic therapies.
Hornick JL. Subclassification of pleomorphic sarcomas: how and why should we care? Ann Diagn Pathol. 2018;37:118–24.
Miettinen M, Enzinger FM. Epithelioid variant of pleomorphic liposarcoma: a study of 12 cases of a distinctive variant of high-grade liposarcoma. Mod Pathol. 1999;12(7):722–8.
Anderson WJ, Jo VY. Pleomorphic liposarcoma: updates and current differential diagnosis. Seminars in diagnostic pathology. WB Saunders; 2019;36(2).
Creytens D, Folpe AL, Koelsche C, Mentzel T, Ferdinande L, van Gorp JM, et al. Myxoid pleomorphic liposarcoma—a clinicopathologic, immunohistochemical, molecular genetic and epigenetic study of 12 cases, suggesting a possible relationship with conventional pleomorphic liposarcoma. Mod Pathol. 2021;34(11):2043–9.
Barretina J, Taylor BS, Banerji S, Ramos AH, Lagos-Quintana M, Decarolis PL, et al. Subtype-specific genomic alterations define new targets for soft-tissue sarcoma therapy. Nat Genet août. 2010;42(8):715–21.
Idbaih A, Coindre JM, Derré J, Mariani O, Terrier P, Ranchère D, et al. Myxoid malignant fibrous histiocytoma and pleomorphic liposarcoma share very similar genomic imbalances. Lab Invest. 2005;85(2):176–81.
Sugita S, Seki K, Yokozawa K, Tochigi N, Furuta K, Hisaoka M, et al. Analysis of CHOP rearrangement in pleomorphic liposarcomas using fluorescence in situ hybridization. Cancer Sci. 2009;100(1):82–7.
Lee JC, Jeng YM, Liau JY, Tsai JH, Hsu HH, Yang CY. Alternative lengthening of telomeres and loss of ATRX are frequent events in pleomorphic and dedifferentiated liposarcomas. Mod Pathol. 2015;28(8):1064–73.
Mariño-Enríquez A, Hornick JL, Dal Cin P, Cibas ES, Qian X. Dedifferentiated liposarcoma and pleomorphic liposarcoma: a comparative study of cytomorphology and MDM2/CDK4 expression on fine-needle aspiration. Cancer Cytopathol. 2014;122(2):128–37.
Åberg E, Saccoccia F, Grabherr M, Ore WYJ, Jemth P, Hultqvist G. Evolution of the p53-MDM2 pathway. BMC Evol Biol [Internet]. 2017 [cité 10 oct 2020];17(1). Disponible sur: http://bmcevolbiol.biomedcentral.com/articles/10.1186/s12862-017-1023-y.
Ciliberti V, Cretella P, Zeppa P, Caputo A. Pleomorphic liposarcoma with liver metastasis diagnosed by combined fine-needle aspiration cytology and core-needle biopsy. Diagn Cytopathol. 2022;50(1):E28-31.
Apostolou G, Biteli M, Chatzipantelis P. Cytopathological diagnosis of metastatic pleomorphic liposarcoma in the lung: a report of a case correlated with the histopathology of the primary tumour. Diagn Cytopathol. 2009;37(9):667–70.
Dodd LG, Jiang X, Rao K, Bui MM. Pleomorphic liposarcoma: a cytologic study of five cases. Diagn Cytopathol. 2015;43(2):138–43.
Wakely PE Jr, Wangsiricharoen S, Ali SZ. Pleomorphic liposarcoma: a clinicopathologic study of 20 FNA cases. Cancer Cytopathol. 2022;130:705–13.
May CD, Garnett J, Ma X, Landers SM, Ingram DR, Demicco EG, et al. AXL is a potential therapeutic target in dedifferentiated and pleomorphic liposarcomas. BMC Cancer. 2015;15(1):1–12.
Valenciaga A, Iwenofu OH, Tinoco G. Larotrectinib in a patient with advanced pleomorphic liposarcoma of the uterus. J Natl Compr Canc Netw. 2021;19(7):775–9.
Friedman JL, Bistritz JI, Robinson MJ. Pleomorphic liposarcoma of the pterygomandibular space involving the maxilla. Oral Surg Oral Med Oral Pathol Oral Radiol Endodontol. 1995;79(4):488–91.
Agarwal J, Kadakia S, Agaimy A, Ogadzanov A, Khorsandi A, Chai RL. Pleomorphic liposarcoma of the head and neck: presentation of two cases and literature review. Am J Otolaryngol. 2017;38(4):505–7.
Wang JG, Wei ZM, Liu H, Li YJ. Primary pleomorphic liposarcoma of pericardium. Interact Cardiovasc Thorac Surg. 2010;11(3):325–7.
Tan NY, Najam M, Lyle MA, Maleszewski JJ, Collins JD, Klarich KW. Dramatic presentation of cardiac pleomorphic liposarcoma. Circulation: Cardiovasc Imaging. 2021;14(8):e012620.
Ganesan S, Moorthy N, Venkataraman SK, Mahipathy SRRV, Sundaramurthy N. Giant pleomorphic liposarcoma of anterior chest wall. JK Sci J Med Educ Res. 2022;24(2):140–2.
Kim MJ, Koo HL, Jun SY, Ro JY. Pleomorphic liposarcoma of the kidney: a case report. Kor J Pathol. 2003;37(3):210–3.
Dey T, Khosla D, Kumar D, Chatterjee D, Madan R, Singh H, et al. Rare case of primary pulmonary pleomorphic liposarcoma treated with multimodal therapy. Ochsner J. 2021;21(4):431–5.
Verdugo D, Voltaggio L, Hoot A, Rodriguez FJ. Cerebral pleomorphic liposarcoma: clinicopathologic findings. Clin Neuropathol. 2019;38(5):233.
Tiemeier GL, Brown JM, Pratap SE, McCarthy C, Kastrenopoulou A, Bradley K, et al. Pleomorphic liposarcoma of bone: a rare primary malignant bone tumour. Clin Sarcoma Res. 2018;8(1):1–9.
Torigoe T, Matsumoto T, Terakado A, Takase M, Yamasaki S, Kurosawa H. Primary pleomorphic liposarcoma of bone: MRI findings and review of the literature. Skelet Radiol. 2006;35(7):536–8.
Jo SJ, Jung HK, Nam KH. Recurrent primary pleomorphic liposarcoma of the breast: a case report with imaging findings. J Breast Cancer. 2020;23(5):567.
Gardner JM, Dandekar M, Thomas D, Goldblum JR, Weiss SW, Billings SD, et al. Cutaneous and subcutaneous pleomorphic liposarcoma: a clinicopathologic study of 29 cases with evaluation of: MDM2: gene amplification in 26. Am J Surg Pathol. 2012;36(7):1047–51.
Pawel BR, de Chadarevian JP, Inniss S, Kalwinski P. Mesenteric pleomorphic liposarcoma in an adolescent. Arch Pathol Lab Med. 1997;121(2):173.
Levine PH, Wei XJ, Gagner JP, Flax H, Mittal K, Blank SV. Pleomorphic liposarcoma of the uterus: case report and literature review. Int J Gynecol Pathol. 2003;22(4):407–11.
Yozu M, Symmans P, Dray M, Griffin J, Han C, Ng D, et al. Muir-Torre syndrome-associated pleomorphic liposarcoma arising in a previous radiation field. Virchows Arch. 2013;462(3):355–60.
Orosz Z, Rohonyi B, Luksander A, Szántó J. Pleomorphic liposarcoma of a young woman following radiotherapy for epithelioid sarcoma. Pathol Oncol Res. 2000;6(4):287–91.
Arbabi L, Warhol MJ. Pleomorphic liposarcoma following radiotherapy for breast carcinoma. Cancer. 1982;49(5):878–80.
Lee HG, Aurit S, Silberstein P, Gootee J. Primary anatomical site as a prognostic factor for pleomorphic liposarcoma. J Cancer Res Clin Oncol. 2020;146(6):1501–8.
Wan L, Tu C, Qi L, Li Z. Survivorship and prognostic factors for pleomorphic liposarcoma: a population-based study. J Orthop Surg Res. 2021;16(1):1–10.
Downes KA, Goldblum JR, Montgomery EA, Fisher C. Pleomorphic liposarcoma: a clinicopathologic analysis of 19 cases. Mod Pathol. 2001;14(3):179–84.
Wang L, Luo R, Xiong Z, Xu J, Fang D. Pleomorphic liposarcoma: an analysis of 6 case reports and literature review. Medicine. 2018;97(8):e9986.
Fiore M, Grosso F, Lo Vullo S, Pennacchioli E, Stacchiotti S, Ferrari A, et al. Myxoid/round cell and pleomorphic liposarcomas. Cancer. 2007;109(12):2522–31.
Muratori F, Frenos F, Bettini L, Matera D, Mondanelli N, Scorianz M, et al. Liposarcoma: clinico-pathological analysis, prognostic factors and survival in a series of 307 patients treated at a single institution. J Orthop Sci. 2018;23(6):1038–44.
Hornick JL, Bosenberg MW, Mentzel T, McMenamin ME, Oliveira AM, Fletcher CD. Pleomorphic liposarcoma: clinicopathologic analysis of 57 cases. Am J Surg Pathol. 2004;28(10):1257–67.
Wang L, Ren W, Zhou X, Sheng W, Wang J. Pleomorphic liposarcoma: a clinicopathological, immunohistochemical and molecular cytogenetic study of 32 additional cases. Pathol Int. 2013;63(11):523–31.
Jones RL, Fisher C, Al-Muderis O, Judson IR. Differential sensitivity of liposarcoma subtypes to chemotherapy. Eur J Cancer. 2005;41(18):2853–60.
• Assi TS, Honoré C, Bahleda R, Henon C, Verret B, Faron M, et al. 48MO Characteristics and outcomes of 76 patients with pleomorphic liposarcoma: the Gustave Roussy experience. ESMO Open. 2023;8(1): 101085. Retrospective data collection of pleomorphic liposarcoma to assess the role of current systemic approaches in the advanced setting.
OncLive [Internet]. 2016 [cité 4 mai 2023]. FDA approves eribulin for advanced liposarcoma. Disponible sur: https://www.onclive.com/view/fda-approves-eribulin-for-advanced-liposarcoma.
Phillips E, Jones RL, Huang P, Digklia A. Efficacy of eribulin in soft tissue sarcomas. Front Pharmacol [Internet]. 2022 [cité 4 mai 2023];13. Disponible sur: https://www.frontiersin.org/articles/10.3389/fphar.2022.869754.
Cortes J, Schöffski P, Littlefield BA. Multiple modes of action of eribulin mesylate: emerging data and clinical implications. Cancer Treat Rev. 2018;70:190–8.
Demetri GD, Schöffski P, Grignani G, Blay JY, Maki RG, Van Tine BA, 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):3433–9.
Schöffski P, Chawla S, Maki RG, Italiano A, Gelderblom H, Choy E, 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):1629–37.
Chen TWW, Hsu CL, Hong RL, Lee JC, Chang K, Yu CW, et al. A single-arm phase Ib/II study of lenvatinib plus eribulin in advanced liposarcoma and leiomyosarcoma. Clin Cancer Res. 2022;28(23):5058–65.
Kim CG, Sim NS, Kim JE, Yun KH, Lee YH, Kim SH, et al. Phase II clinical trial of eribulin–gemcitabine combination therapy in previously treated patients with advanced liposarcoma or leiomyosarcoma. Clin Cancer Res. 2022;28(15):3225–34.
Kobayashi E, Naito Y, Asano N, Maejima A, Endo M, Takahashi S, et al. Interim results of a real-world observational study of eribulin in soft tissue sarcoma including rare subtypes. Jpn J Clin Oncol. 2019;49(10):938–46.
Escudero J, Heredia-Soto V, Wang Y, Ruiz P, Hu Y, Gallego A, et al. Eribulin activity in soft tissue sarcoma monolayer and three-dimensional cell line models: could the combination with other drugs improve its antitumoral effect? Cancer Cell Int. 2021;21:646.
Assi T, Kattan J, El Rassy E, Honore C, Dumont S, Mir O, et al. A comprehensive review of the current evidence for trabectedin in advanced myxoid liposarcoma. Cancer Treat Rev. 2019;72:37–44.
Demetri GD, Chawla SP, Von Mehren M, Ritch P, Baker LH, Blay JY, et al. Efficacy and safety of trabectedin in patients with advanced or metastatic liposarcoma or leiomyosarcoma after failure of prior anthracyclines and ifosfamide: results of a randomized phase II study of two different schedules. J Clin Oncol. 2009;27(25):4188–96.
Cesne AL, Blay JY, Cupissol D, Italiano A, Delcambre C, Penel N, et al. A randomized phase III trial comparing trabectedin to best supportive care in patients with pre-treated soft tissue sarcoma: T-SAR, a French Sarcoma Group trial. Ann Oncol. 2021;32(8):1034–44.
https://www.facebook.com/Drugscom. Drugs.com. [cité 5 mai 2023]. U.S. FDA grants priority review for Yondelis (trabectedin) for the treatment of patients with advanced soft tissue sarcoma. Disponible sur: https://www.drugs.com/nda/yondelis_150203.html.
Demetri GD, Von Mehren M, Jones RL, Hensley ML, Schuetze SM, Staddon A, 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.
Patel S, von Mehren M, Reed DR, Kaiser P, Charlson J, Ryan CW, et al. Overall survival and histology-specific subgroup analyses from a phase 3, randomized controlled study of trabectedin or dacarbazine in patients with advanced liposarcoma or leiomyosarcoma. Cancer. 2019;125(15):2610–20.
Kiyuna T, Tome Y, Murakami T, Kawaguchi K, Igarashi K, Miyake K, et al. Trabectedin arrests a doxorubicin-resistant PDGFRA-activated liposarcoma patient-derived orthotopic xenograft (PDOX) nude mouse model. BMC Cancer. 2018;18(1):1–8.
Chaigneau L, Jary M, Nerich V, Hervieu A, Aubry S, Barra CC, et al. Real-world experience of efficacy and safety of trabectedin in patients with soft tissue sarcoma: a bicentric retrospective analysis. Oncology. 2022;100(12):633–44.
Schuler MK, Richter S, Platzek I, Beuthien-Baumann B, Wieczorek K, Hamann C, et al. Trabectedin in the neoadjuvant treatment of high-grade pleomorphic sarcoma: report of a rare case and literature review. Case Rep Oncol Med. 2013;2013:1–5.
Wagner MJ, Zhang Y, Cranmer LD, Loggers ET, Black G, McDonnell S, et al. A phase 1/2 trial combining avelumab and trabectedin for advanced liposarcoma and leiomyosarcoma. Clin Cancer Res. 2022;28(11):2306–12.
Judson I, Verweij J, Gelderblom H, Hartmann JT, Schöffski P, Blay JY, 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):415–23.
Ryan CW, Merimsky O, Agulnik M, Blay JY, Schuetze SM, Van Tine BA, 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. 2016;34(32):3898–905.
Tap WD, Papai Z, Van Tine BA, Attia S, Ganjoo KN, Jones RL, 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;18(8):1089–103.
Wang BC, Kuang BH, Xiao BY, Lin GH. Doxorubicin/adriamycin monotherapy or plus ifosfamide in first-line treatment for advanced soft tissue sarcoma: a pooled analysis of randomized trials. Front Oncol [Internet]. 2021 [cité 7 mai 2023];11. Disponible sur: https://www.frontiersin.org/articles/10.3389/fonc.2021.762288.
Seddon B, Strauss SJ, Whelan J, Leahy M, Woll PJ, Cowie F, 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;18(10):1397–410.
D’Angelo SP, Mahoney MR, Van Tine BA, Atkins J, Milhem MM, Jahagirdar BN, 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):416–26.
Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, 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):1493–501.
Toulmonde M, Penel N, Adam J, Chevreau C, Blay JY, Le Cesne A, et al. Use of PD-1 targeting, macrophage infiltration, and IDO pathway activation in sarcomas: a phase 2 clinical trial. JAMA Oncol. 2018;4(1):93–7.
Burgess MA, Bolejack V, Schuetze S, Van Tine BA, Attia S, Riedel RF, et al. Clinical activity of pembrolizumab (P) in undifferentiated pleomorphic sarcoma (UPS) and dedifferentiated/pleomorphic liposarcoma (LPS): final results of SARC028 expansion cohorts. J Clin Oncol. 2019;11015.
Sharma A, Bode B, Studer G, Moch H, Okoniewski M, Knuth A, et al. Radiotherapy of human sarcoma promotes an intratumoral immune effector signature. Clin Cancer Res. 2013;19(17):4843–53.
Digklia A, Coukos G, Homicsko K. Trabectedin and durvalumab combination is feasible-and active in relapsing ovarian cancer. Clin Cancer Res. 2022; 28(9):1745–7.
Wilky BA, Trucco MM, Kolonias D, Wieder E, Subhawong T, Rosenberg A, et al. A phase II trial of axitinib plus pembrolizumab for patients with advanced alveolar soft part sarcoma (ASPS) and other soft tissue sarcomas (STS). American Society of Clinical Oncology. 2018;11547.
Post TA. FDA approves pazopanib for advanced soft-tissue sarcoma - the ASCO post [Internet]. [cité 6 mai 2023]. Disponible sur: https://ascopost.com/issues/may-15-2012/fda-approves-pazopanib-for-advanced-soft-tissue-sarcoma/.
van der Graaf WTA, Blay JY, Chawla SP, Kim DW, Bui-Nguyen B, Casali PG, et al. Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet. 2012;379(9829):1879–86.
Sleijfer S, Ray-Coquard I, Papai Z, Le Cesne A, Scurr M, Schöffski P, 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 62043). J Clin Oncol. 2009;27(19):3126–32.
Samuels BL, Chawla SP, Somaiah N, Staddon AP, Skubitz KM, Milhem MM, et al. Results of a prospective phase 2 study of pazopanib in patients with advanced intermediate-grade or high-grade liposarcoma. Cancer. 2017;123(23):4640–7.
Valverde CM, Martin Broto J, Lopez-Martin JA, Romagosa C, Sancho Marquez MP, Carrasco JA, et al. Phase II clinical trial evaluating the activity and tolerability of pazopanib in patients (pts) with advanced and/or metastatic liposarcoma (LPS): a joint Spanish Sarcoma Group (GEIS) and German Interdisciplinary Sarcoma Group (GISG) study—NCT01692496. Am Soc Clin Oncol. 2016:11039.
Chamberlain FE, Wilding C, Jones RL, Huang P. Pazopanib in patients with advanced intermediate-grade or high-grade liposarcoma. Expert Opin Investig Drugs. 2019;28(6):505–11.
Nakamura T, Matsumine A, Kawai A, Araki N, Goto T, Yonemoto T, et al. The clinical outcome of pazopanib treatment in Japanese patients with relapsed soft tissue sarcoma: a Japanese Musculoskeletal Oncology Group (JMOG) study. Cancer. 2016;122(9):1408–16.
Seto T, Song MN, Trieu M, Yu J, Sidhu M, Liu CM, et al. Real-world experiences with pazopanib in patients with advanced soft tissue and bone sarcoma in Northern California. Med Sci. 2019;7(3):48.
Schöffski P. Established and experimental systemic treatment options for advanced liposarcoma. Oncol Res Treatment. 2022;45(9):525–43.
Assi T, Kattan J, Rassy E, Nassereddine H, Farhat F, Honore C, et al. Targeting CDK4 (cyclin-dependent kinase) amplification in liposarcoma: a comprehensive review. Crit Rev Oncol/Hematol. 2020;153:103029.
Burgess A, Chia KM, Haupt S, Thomas D, Haupt Y, Lim E. Clinical overview of MDM2/X-targeted therapies. Front Oncol [Internet]. 2016 [cité 7 oct 2020];6. Disponible sur: http://journal.frontiersin.org/Article/10.3389/fonc.2016.00007/abstract.
Yan P, Sun ML, Sun YP, Liu CY. Effective apatinib treatment of pleomorphic liposarcoma: a case report. Medicine. 2017;96(33):e7771.
Kiyuna T, Murakami T, Tome Y, Igarashi K, Kawaguchi K, Miyake K, et al. Doxorubicin-resistant pleomorphic liposarcoma with PDGFRA gene amplification is targeted and regressed by pazopanib in a patient-derived orthotopic xenograft mouse model. Tissue Cell. 2018;53:30–6.
Fu X, Niu W, Li J, Kiliti AJ, Al-Ahmadie HA, Iyer G, et al. Activating mutation of PDGFRB gene in a rare cardiac undifferentiated intimal sarcoma of the left atrium: a case report. Oncotarget. 2017;8(46):81709.
Kiyuna T, Tome Y, Murakami T, Zhao M, Miyake K, Igarashi K, et al. Tumor-targeting Salmonella typhimurium A1-R arrests a doxorubicin-resistant PDGFRA-amplified patient-derived orthotopic xenograft mouse model of pleomorphic liposarcoma. J Cell Biochem. 2018;119(9):7827–33.
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Assi, T., Ngo, C., Faron, M. et al. Systemic Therapy in Advanced Pleomorphic Liposarcoma: a Comprehensive Review. Curr. Treat. Options in Oncol. 24, 1598–1613 (2023). https://doi.org/10.1007/s11864-023-01139-3
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DOI: https://doi.org/10.1007/s11864-023-01139-3