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Advanced Sarcoma

MedGen UID:
1658595
•Concept ID:
C4727079
•
Neoplastic Process

Definition

A sarcoma that has spread extensively to other anatomic sites or is no longer responding to treatment. [from NCI]

Professional guidelines

PubMed

Making the Best of Available Options for Optimal Sarcoma Treatment.
Le Cesne A
Oncology 2018;95 Suppl 1:11-20. Epub 2018 Dec 14 doi: 10.1159/000494861. PMID: 30554197
Advances of systemic treatment for adult soft-tissue sarcoma.
Liu W, Jiang Q, Zhou Y
Chin Clin Oncol 2018 Aug;7(4):42. doi: 10.21037/cco.2018.08.02. PMID: 30173532
Systemic treatment in advanced soft tissue sarcoma: what is standard, what is new.
Frezza AM, Stacchiotti S, Gronchi A
BMC Med 2017 Jun 2;15(1):109. doi: 10.1186/s12916-017-0872-y. PMID: 28571564Free PMC Article

Recent clinical studies

Etiology

Autologous HER2-specific CAR T cells after lymphodepletion for advanced sarcoma: a phase 1 trial.
Hegde M, Navai S, DeRenzo C, Joseph SK, Sanber K, Wu M, Gad AZ, Janeway KA, Campbell M, Mullikin D, Nawas Z, Robertson C, Mathew PR, Zhang H, Mehta B, Bhat RR, Major A, Shree A, Gerken C, Kalra M, Chakraborty R, Thakkar SG, Dakhova O, Salsman VS, Grilley B, Lapteva N, Gee A, Dotti G, Bao R, Salem AH, Wang T, Brenner MK, Heslop HE, Wels WS, Hicks MJ, Gottschalk S, Ahmed N
Nat Cancer 2024 Jun;5(6):880-894. Epub 2024 Apr 24 doi: 10.1038/s43018-024-00749-6. PMID: 38658775
Safety and Efficacy of 90Y-FAPI-46 Radioligand Therapy in Patients with Advanced Sarcoma and Other Cancer Entities.
Fendler WP, Pabst KM, Kessler L, Fragoso Costa P, Ferdinandus J, Weber M, Lippert M, Lueckerath K, Umutlu L, Kostbade K, Mavroeidi IA, Schuler M, Ahrens M, Rischpler C, Bauer S, Herrmann K, Siveke JT, Hamacher R
Clin Cancer Res 2022 Oct 3;28(19):4346-4353. doi: 10.1158/1078-0432.CCR-22-1432. PMID: 35833949Free PMC Article
Targeted and immuno-based therapies in sarcoma: mechanisms and advances in clinical trials.
Tang F, Tie Y, Wei YQ, Tu CQ, Wei XW
Biochim Biophys Acta Rev Cancer 2021 Dec;1876(2):188606. Epub 2021 Aug 8 doi: 10.1016/j.bbcan.2021.188606. PMID: 34371128
Advances of systemic treatment for adult soft-tissue sarcoma.
Liu W, Jiang Q, Zhou Y
Chin Clin Oncol 2018 Aug;7(4):42. doi: 10.21037/cco.2018.08.02. PMID: 30173532
Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.
Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, D'Angelo S, Attia S, Riedel RF, Priebat DA, Movva S, Davis LE, Okuno SH, Reed DR, Crowley J, Butterfield LH, Salazar R, Rodriguez-Canales J, Lazar AJ, Wistuba II, Baker LH, Maki RG, Reinke D, Patel S
Lancet Oncol 2017 Nov;18(11):1493-1501. Epub 2017 Oct 4 doi: 10.1016/S1470-2045(17)30624-1. PMID: 28988646Free PMC Article

Diagnosis

A Phase I/II Investigation of Safety and Efficacy of Nivolumab and nab-Sirolimus in Patients With a Variety of Tumors With Genetic Mutations in the mTOR Pathway.
Gordon EM, Angel NL, Omelchenko N, Chua-Alcala VS, Moradkhani A, Quon D, Wong S
Anticancer Res 2023 May;43(5):1993-2002. doi: 10.21873/anticanres.16360. PMID: 37097693
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.
Grosso F, D'Ambrosio L, Zucchetti M, Ibrahim T, Tamberi S, Matteo C, Rulli E, Comandini D, Palmerini E, Baldi GG, DeCensi A, Bergaglio M, Marra D, Marchesi E, Siri G, D'Incalci M, Grignani G
Cancer 2020 Nov 1;126(21):4726-4734. Epub 2020 Aug 4 doi: 10.1002/cncr.33120. PMID: 32749681
Maintenance therapy and drug holiday in sarcoma patients: systematic review.
Lebellec L, Defachelles AS, Cren PY, Penel N
Acta Oncol 2020 Sep;59(9):1084-1090. Epub 2020 May 13 doi: 10.1080/0284186X.2020.1759825. PMID: 32400254
Advances of systemic treatment for adult soft-tissue sarcoma.
Liu W, Jiang Q, Zhou Y
Chin Clin Oncol 2018 Aug;7(4):42. doi: 10.21037/cco.2018.08.02. PMID: 30173532
The past, present, and future of cytotoxic chemotherapy and pathway-directed targeted agents for soft tissue sarcoma.
Ryan CW, Desai J
Am Soc Clin Oncol Educ Book 2013 doi: 10.14694/EdBook_AM.2013.33.e386. PMID: 23714556

Therapy

Autologous HER2-specific CAR T cells after lymphodepletion for advanced sarcoma: a phase 1 trial.
Hegde M, Navai S, DeRenzo C, Joseph SK, Sanber K, Wu M, Gad AZ, Janeway KA, Campbell M, Mullikin D, Nawas Z, Robertson C, Mathew PR, Zhang H, Mehta B, Bhat RR, Major A, Shree A, Gerken C, Kalra M, Chakraborty R, Thakkar SG, Dakhova O, Salsman VS, Grilley B, Lapteva N, Gee A, Dotti G, Bao R, Salem AH, Wang T, Brenner MK, Heslop HE, Wels WS, Hicks MJ, Gottschalk S, Ahmed N
Nat Cancer 2024 Jun;5(6):880-894. Epub 2024 Apr 24 doi: 10.1038/s43018-024-00749-6. PMID: 38658775
New targeted treatments for advanced sarcomas.
Li CC, Chen TW
Curr Opin Oncol 2023 Jul 1;35(4):309-314. Epub 2023 May 3 doi: 10.1097/CCO.0000000000000955. PMID: 37222206
A Phase II Study of Epacadostat and Pembrolizumab in Patients with Advanced Sarcoma.
Kelly CM, Qin LX, Whiting KA, Richards AL, Avutu V, Chan JE, Chi P, Dickson MA, Gounder MM, Keohan ML, Movva S, Nacev BA, Rosenbaum E, Adamson T, Singer S, Bartlett EK, Crago AM, Yoon SS, Hwang S, Erinjeri JP, Antonescu CR, Tap WD, D'Angelo SP
Clin Cancer Res 2023 Jun 1;29(11):2043-2051. doi: 10.1158/1078-0432.CCR-22-3911. PMID: 36971773Free PMC Article
Frequent amplification of HDAC genes and efficacy of HDAC inhibitor chidamide and PD-1 blockade combination in soft tissue sarcoma.
Que Y, Zhang XL, Liu ZX, Zhao JJ, Pan QZ, Wen XZ, Xiao W, Xu BS, Hong DC, Guo TH, Shen LJ, Fan WJ, Chen HY, Weng DS, Xu HR, Zhou PH, Zhang YZ, Niu XH, Zhang X
J Immunother Cancer 2021 Feb;9(2) doi: 10.1136/jitc-2020-001696. PMID: 33637599Free PMC Article
Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.
Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, D'Angelo S, Attia S, Riedel RF, Priebat DA, Movva S, Davis LE, Okuno SH, Reed DR, Crowley J, Butterfield LH, Salazar R, Rodriguez-Canales J, Lazar AJ, Wistuba II, Baker LH, Maki RG, Reinke D, Patel S
Lancet Oncol 2017 Nov;18(11):1493-1501. Epub 2017 Oct 4 doi: 10.1016/S1470-2045(17)30624-1. PMID: 28988646Free PMC Article

Prognosis

Phase II trial of CDK4/6 inhibitor palbociclib in advanced sarcoma based on mRNA expression of CDK4/ CDKN2A.
Martin-Broto J, Martinez-Garcia J, Moura DS, Redondo A, Gutierrez A, Lopez-Pousa A, Martinez-Trufero J, Sevilla I, Diaz-Beveridge R, Solis-Hernandez MP, Carnero A, Perez M, Marcilla D, Garcia-Foncillas J, Romero P, Fernandez-Jara J, Lopez-Lopez D, Arribas I, Hindi N
Signal Transduct Target Ther 2023 Oct 25;8(1):405. doi: 10.1038/s41392-023-01661-8. PMID: 37875500Free PMC Article
The Role of AKT in Soft Tissue Sarcoma: Review and Insights.
Wang Y, Ilyas FZ, Kheradmandi M, Tsilimigras DI, Grignol VP, Contreras C, Tsichlis PN, Pollock RE, Beane JD
Mol Cancer Res 2022 Oct 4;20(10):1471-1480. doi: 10.1158/1541-7786.MCR-21-0844. PMID: 35796636
Frequent amplification of HDAC genes and efficacy of HDAC inhibitor chidamide and PD-1 blockade combination in soft tissue sarcoma.
Que Y, Zhang XL, Liu ZX, Zhao JJ, Pan QZ, Wen XZ, Xiao W, Xu BS, Hong DC, Guo TH, Shen LJ, Fan WJ, Chen HY, Weng DS, Xu HR, Zhou PH, Zhang YZ, Niu XH, Zhang X
J Immunother Cancer 2021 Feb;9(2) doi: 10.1136/jitc-2020-001696. PMID: 33637599Free PMC Article
Characteristics and outcomes of patients with advanced sarcoma enrolled in early phase immunotherapy trials.
Groisberg R, Hong DS, Behrang A, Hess K, Janku F, Piha-Paul S, Naing A, Fu S, Benjamin R, Patel S, Somaiah N, Conley A, Meric-Bernstam F, Subbiah V
J Immunother Cancer 2017 Dec 19;5(1):100. doi: 10.1186/s40425-017-0301-y. PMID: 29254498Free PMC Article
Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.
Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, D'Angelo S, Attia S, Riedel RF, Priebat DA, Movva S, Davis LE, Okuno SH, Reed DR, Crowley J, Butterfield LH, Salazar R, Rodriguez-Canales J, Lazar AJ, Wistuba II, Baker LH, Maki RG, Reinke D, Patel S
Lancet Oncol 2017 Nov;18(11):1493-1501. Epub 2017 Oct 4 doi: 10.1016/S1470-2045(17)30624-1. PMID: 28988646Free PMC Article

Clinical prediction guides

Autologous HER2-specific CAR T cells after lymphodepletion for advanced sarcoma: a phase 1 trial.
Hegde M, Navai S, DeRenzo C, Joseph SK, Sanber K, Wu M, Gad AZ, Janeway KA, Campbell M, Mullikin D, Nawas Z, Robertson C, Mathew PR, Zhang H, Mehta B, Bhat RR, Major A, Shree A, Gerken C, Kalra M, Chakraborty R, Thakkar SG, Dakhova O, Salsman VS, Grilley B, Lapteva N, Gee A, Dotti G, Bao R, Salem AH, Wang T, Brenner MK, Heslop HE, Wels WS, Hicks MJ, Gottschalk S, Ahmed N
Nat Cancer 2024 Jun;5(6):880-894. Epub 2024 Apr 24 doi: 10.1038/s43018-024-00749-6. PMID: 38658775
Phase II trial of CDK4/6 inhibitor palbociclib in advanced sarcoma based on mRNA expression of CDK4/ CDKN2A.
Martin-Broto J, Martinez-Garcia J, Moura DS, Redondo A, Gutierrez A, Lopez-Pousa A, Martinez-Trufero J, Sevilla I, Diaz-Beveridge R, Solis-Hernandez MP, Carnero A, Perez M, Marcilla D, Garcia-Foncillas J, Romero P, Fernandez-Jara J, Lopez-Lopez D, Arribas I, Hindi N
Signal Transduct Target Ther 2023 Oct 25;8(1):405. doi: 10.1038/s41392-023-01661-8. PMID: 37875500Free PMC Article
A Phase II Study of Epacadostat and Pembrolizumab in Patients with Advanced Sarcoma.
Kelly CM, Qin LX, Whiting KA, Richards AL, Avutu V, Chan JE, Chi P, Dickson MA, Gounder MM, Keohan ML, Movva S, Nacev BA, Rosenbaum E, Adamson T, Singer S, Bartlett EK, Crago AM, Yoon SS, Hwang S, Erinjeri JP, Antonescu CR, Tap WD, D'Angelo SP
Clin Cancer Res 2023 Jun 1;29(11):2043-2051. doi: 10.1158/1078-0432.CCR-22-3911. PMID: 36971773Free PMC Article
Safety and Efficacy of 90Y-FAPI-46 Radioligand Therapy in Patients with Advanced Sarcoma and Other Cancer Entities.
Fendler WP, Pabst KM, Kessler L, Fragoso Costa P, Ferdinandus J, Weber M, Lippert M, Lueckerath K, Umutlu L, Kostbade K, Mavroeidi IA, Schuler M, Ahrens M, Rischpler C, Bauer S, Herrmann K, Siveke JT, Hamacher R
Clin Cancer Res 2022 Oct 3;28(19):4346-4353. doi: 10.1158/1078-0432.CCR-22-1432. PMID: 35833949Free PMC Article
Characteristics and outcomes of patients with advanced sarcoma enrolled in early phase immunotherapy trials.
Groisberg R, Hong DS, Behrang A, Hess K, Janku F, Piha-Paul S, Naing A, Fu S, Benjamin R, Patel S, Somaiah N, Conley A, Meric-Bernstam F, Subbiah V
J Immunother Cancer 2017 Dec 19;5(1):100. doi: 10.1186/s40425-017-0301-y. PMID: 29254498Free PMC Article

Recent systematic reviews

Maintenance therapy and drug holiday in sarcoma patients: systematic review.
Lebellec L, Defachelles AS, Cren PY, Penel N
Acta Oncol 2020 Sep;59(9):1084-1090. Epub 2020 May 13 doi: 10.1080/0284186X.2020.1759825. PMID: 32400254

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