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Refractory Undifferentiated Pleomorphic Sarcoma

MedGen UID:
1658566
•Concept ID:
C4724966
•
Neoplastic Process

Definition

Undifferentiated pleomorphic sarcoma that does not respond to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRefractory Undifferentiated Pleomorphic Sarcoma

Professional guidelines

PubMed

Gemcitabine-Containing Chemotherapy for the Treatment of Metastatic Myxofibrosarcoma Refractory to Doxorubicin: A Case Series.
Elkrief A, Kazandjian S, Alcindor T
Curr Oncol 2021 Feb 5;28(1):813-817. doi: 10.3390/curroncol28010078. PMID: 33562486Free PMC Article
Refractory malignant fibrous histiocytoma: CT-guided treatment with a multidisciplinary, minimally invasive approach.
Shi F, Zhang F, Gao F, Wu K, Maharjan R, Li C
Technol Cancer Res Treat 2015 Feb;14(1):3-9. Epub 2014 Nov 11 doi: 10.7785/tcrt.2012.500390. PMID: 24325132
Combination of gemcitabine and docetaxel in the treatment of children and young adults with refractory bone sarcoma.
Navid F, Willert JR, McCarville MB, Furman W, Watkins A, Roberts W, Daw NC
Cancer 2008 Jul 15;113(2):419-25. doi: 10.1002/cncr.23586. PMID: 18484657

Recent clinical studies

Etiology

Establishment and characterization of NCC-UPS3-C1: a novel patient-derived cell line of undifferentiated pleomorphic sarcoma.
Tsuchiya R, Yoshimatsu Y, Noguchi R, Sin Y, Ono T, Akiyama T, Sugaya J, Nakatani F, Kojima N, Yoshida A, Ohtori S, Kawai A, Kondo T
Hum Cell 2022 Jan;35(1):384-391. Epub 2021 Oct 19 doi: 10.1007/s13577-021-00633-w. PMID: 34665443
Pemetrexed plus cisplatin in patients with previously treated advanced sarcoma: a multicenter, single-arm, phase II trial.
Kim JH, Kim SH, Jeon MK, Kim JE, Kim KH, Yun KH, Jeung HC, Rha SY, Ahn JH, Kim HS
ESMO Open 2021 Oct;6(5):100249. Epub 2021 Sep 2 doi: 10.1016/j.esmoop.2021.100249. PMID: 34482181Free PMC Article
Gemcitabine-Containing Chemotherapy for the Treatment of Metastatic Myxofibrosarcoma Refractory to Doxorubicin: A Case Series.
Elkrief A, Kazandjian S, Alcindor T
Curr Oncol 2021 Feb 5;28(1):813-817. doi: 10.3390/curroncol28010078. PMID: 33562486Free PMC Article
Refractory malignant fibrous histiocytoma: CT-guided treatment with a multidisciplinary, minimally invasive approach.
Shi F, Zhang F, Gao F, Wu K, Maharjan R, Li C
Technol Cancer Res Treat 2015 Feb;14(1):3-9. Epub 2014 Nov 11 doi: 10.7785/tcrt.2012.500390. PMID: 24325132
Soft tissue sarcomas, a look into the future: different treatments for different subtypes.
Reichardt P
Future Oncol 2014 Jun;10(8 Suppl):s19-27. doi: 10.2217/fon.14.116. PMID: 25048045

Diagnosis

Unveiling the Genomic Basis of Chemosensitivity in Sarcomas of the Extremities: An Integrated Approach for an Unmet Clinical Need.
Vanni S, Fausti V, Fonzi E, Liverani C, Miserocchi G, Spadazzi C, Cocchi C, Calabrese C, Gurrieri L, Riva N, Recine F, Casadei R, Pieri F, Guerrieri AN, Serra M, Ibrahim T, Mercatali L, De Vita A
Int J Mol Sci 2023 Apr 8;24(8) doi: 10.3390/ijms24086926. PMID: 37108089Free PMC Article
Gemcitabine-Containing Chemotherapy for the Treatment of Metastatic Myxofibrosarcoma Refractory to Doxorubicin: A Case Series.
Elkrief A, Kazandjian S, Alcindor T
Curr Oncol 2021 Feb 5;28(1):813-817. doi: 10.3390/curroncol28010078. PMID: 33562486Free PMC Article
Soft tissue sarcomas, a look into the future: different treatments for different subtypes.
Reichardt P
Future Oncol 2014 Jun;10(8 Suppl):s19-27. doi: 10.2217/fon.14.116. PMID: 25048045
Combination of gemcitabine and docetaxel in the treatment of children and young adults with refractory bone sarcoma.
Navid F, Willert JR, McCarville MB, Furman W, Watkins A, Roberts W, Daw NC
Cancer 2008 Jul 15;113(2):419-25. doi: 10.1002/cncr.23586. PMID: 18484657
Case for diagnosis. Angiomatoid malignant fibrous histiocytoma.
Grieve JH, Neal JA
Mil Med 1989 Nov;154(11):578, 584-5. PMID: 2555744

Therapy

Pilot study of bempegaldesleukin in combination with nivolumab in patients with metastatic sarcoma.
D'Angelo SP, Richards AL, Conley AP, Woo HJ, Dickson MA, Gounder M, Kelly C, Keohan ML, Movva S, Thornton K, Rosenbaum E, Chi P, Nacev B, Chan JE, Slotkin EK, Kiesler H, Adamson T, Ling L, Rao P, Patel S, Livingston JA, Singer S, Agaram NP, Antonescu CR, Koff A, Erinjeri JP, Hwang S, Qin LX, Donoghue MTA, Tap WD
Nat Commun 2022 Jun 16;13(1):3477. doi: 10.1038/s41467-022-30874-8. PMID: 35710741Free PMC Article
Larotrectinib followed by selitrectinib in a novel DCTN1-NTRK1 fusion undifferentiated pleomorphic sarcoma.
Goh XN, Seng MS, Loh AHP, Gupta A, Chang KTE, Iyer P
J Oncol Pharm Pract 2021 Mar;27(2):485-489. Epub 2020 Jul 21 doi: 10.1177/1078155220938849. PMID: 32693686
Therapeutic response of metastatic angiomatoid fibrous histiocytoma carrying EWSR1-CREB1 fusion to the interleukin-6 receptor antibody tocilizumab.
Potter SL, Quintanilla NM, Johnston DK, Naik-Mathuria B, Venkatramani R
Pediatr Blood Cancer 2018 Oct;65(10):e27291. Epub 2018 Jun 22 doi: 10.1002/pbc.27291. PMID: 29932283
Safety and Efficacy of Anlotinib, a Multikinase Angiogenesis Inhibitor, in Patients with Refractory Metastatic Soft-Tissue Sarcoma.
Chi Y, Fang Z, Hong X, Yao Y, Sun P, Wang G, Du F, Sun Y, Wu Q, Qu G, Wang S, Song J, Yu J, Lu Y, Zhu X, Niu X, He Z, Wang J, Yu H, Cai J
Clin Cancer Res 2018 Nov 1;24(21):5233-5238. Epub 2018 Jun 12 doi: 10.1158/1078-0432.CCR-17-3766. PMID: 29895706
Tandem high-dose chemotherapy supported by autologous peripheral blood stem cell transplantation for recurrent soft tissue sarcoma.
Kozuka T, Kiura K, Katayama H, Fujii N, Ishimaru F, Ikeda K, Ueoka H, Hamasaki S, Yoshino T, Kashihara Y, Date H, Tanimoto M, Harada M
Anticancer Res 2002 Sep-Oct;22(5):2939-44. PMID: 12530021

Prognosis

Unveiling the Genomic Basis of Chemosensitivity in Sarcomas of the Extremities: An Integrated Approach for an Unmet Clinical Need.
Vanni S, Fausti V, Fonzi E, Liverani C, Miserocchi G, Spadazzi C, Cocchi C, Calabrese C, Gurrieri L, Riva N, Recine F, Casadei R, Pieri F, Guerrieri AN, Serra M, Ibrahim T, Mercatali L, De Vita A
Int J Mol Sci 2023 Apr 8;24(8) doi: 10.3390/ijms24086926. PMID: 37108089Free PMC Article
Pilot study of bempegaldesleukin in combination with nivolumab in patients with metastatic sarcoma.
D'Angelo SP, Richards AL, Conley AP, Woo HJ, Dickson MA, Gounder M, Kelly C, Keohan ML, Movva S, Thornton K, Rosenbaum E, Chi P, Nacev B, Chan JE, Slotkin EK, Kiesler H, Adamson T, Ling L, Rao P, Patel S, Livingston JA, Singer S, Agaram NP, Antonescu CR, Koff A, Erinjeri JP, Hwang S, Qin LX, Donoghue MTA, Tap WD
Nat Commun 2022 Jun 16;13(1):3477. doi: 10.1038/s41467-022-30874-8. PMID: 35710741Free PMC Article
Establishment and characterization of NCC-UPS3-C1: a novel patient-derived cell line of undifferentiated pleomorphic sarcoma.
Tsuchiya R, Yoshimatsu Y, Noguchi R, Sin Y, Ono T, Akiyama T, Sugaya J, Nakatani F, Kojima N, Yoshida A, Ohtori S, Kawai A, Kondo T
Hum Cell 2022 Jan;35(1):384-391. Epub 2021 Oct 19 doi: 10.1007/s13577-021-00633-w. PMID: 34665443
Pemetrexed plus cisplatin in patients with previously treated advanced sarcoma: a multicenter, single-arm, phase II trial.
Kim JH, Kim SH, Jeon MK, Kim JE, Kim KH, Yun KH, Jeung HC, Rha SY, Ahn JH, Kim HS
ESMO Open 2021 Oct;6(5):100249. Epub 2021 Sep 2 doi: 10.1016/j.esmoop.2021.100249. PMID: 34482181Free PMC Article
Safety and Efficacy of Anlotinib, a Multikinase Angiogenesis Inhibitor, in Patients with Refractory Metastatic Soft-Tissue Sarcoma.
Chi Y, Fang Z, Hong X, Yao Y, Sun P, Wang G, Du F, Sun Y, Wu Q, Qu G, Wang S, Song J, Yu J, Lu Y, Zhu X, Niu X, He Z, Wang J, Yu H, Cai J
Clin Cancer Res 2018 Nov 1;24(21):5233-5238. Epub 2018 Jun 12 doi: 10.1158/1078-0432.CCR-17-3766. PMID: 29895706

Clinical prediction guides

Unveiling the Genomic Basis of Chemosensitivity in Sarcomas of the Extremities: An Integrated Approach for an Unmet Clinical Need.
Vanni S, Fausti V, Fonzi E, Liverani C, Miserocchi G, Spadazzi C, Cocchi C, Calabrese C, Gurrieri L, Riva N, Recine F, Casadei R, Pieri F, Guerrieri AN, Serra M, Ibrahim T, Mercatali L, De Vita A
Int J Mol Sci 2023 Apr 8;24(8) doi: 10.3390/ijms24086926. PMID: 37108089Free PMC Article
Pilot study of bempegaldesleukin in combination with nivolumab in patients with metastatic sarcoma.
D'Angelo SP, Richards AL, Conley AP, Woo HJ, Dickson MA, Gounder M, Kelly C, Keohan ML, Movva S, Thornton K, Rosenbaum E, Chi P, Nacev B, Chan JE, Slotkin EK, Kiesler H, Adamson T, Ling L, Rao P, Patel S, Livingston JA, Singer S, Agaram NP, Antonescu CR, Koff A, Erinjeri JP, Hwang S, Qin LX, Donoghue MTA, Tap WD
Nat Commun 2022 Jun 16;13(1):3477. doi: 10.1038/s41467-022-30874-8. PMID: 35710741Free PMC Article
Gemcitabine-Containing Chemotherapy for the Treatment of Metastatic Myxofibrosarcoma Refractory to Doxorubicin: A Case Series.
Elkrief A, Kazandjian S, Alcindor T
Curr Oncol 2021 Feb 5;28(1):813-817. doi: 10.3390/curroncol28010078. PMID: 33562486Free PMC Article
Phase 1/2 study of immunotherapy with dendritic cells pulsed with autologous tumor lysate in patients with refractory bone and soft tissue sarcoma.
Miwa S, Nishida H, Tanzawa Y, Takeuchi A, Hayashi K, Yamamoto N, Mizukoshi E, Nakamoto Y, Kaneko S, Tsuchiya H
Cancer 2017 May 1;123(9):1576-1584. Epub 2017 Feb 27 doi: 10.1002/cncr.30606. PMID: 28241093
Intra-arterial intervention chemotherapy for sarcoma and cancerous ulcer via an implanted pump.
Liu C, Cui Q, Guo J, Li D, Zeng Y
Pathol Oncol Res 2014 Apr;20(2):229-34. Epub 2014 Jan 21 doi: 10.1007/s12253-013-9673-6. PMID: 24446082

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