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Unresectable Undifferentiated Pleomorphic Sarcoma

MedGen UID:
1670156
•Concept ID:
C4725869
•
Neoplastic Process

Definition

Undifferentiated pleomorphic sarcoma that is not amenable to surgical resection. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Biology and Management of High-Grade Chondrosarcoma: An Update on Targets and Treatment Options.
Tlemsani C, Larousserie F, De Percin S, Audard V, Hadjadj D, Chen J, Biau D, Anract P, Terris B, Goldwasser F, Pasmant E, Boudou-Rouquette P
Int J Mol Sci 2023 Jan 10;24(2) doi: 10.3390/ijms24021361. PMID: 36674874Free PMC Article
Receptor Tyrosine Kinase Inhibitors for the Treatment of Recurrent and Unresectable Bone Sarcomas.
Albarrán V, Villamayor ML, Chamorro J, Rosero DI, Pozas J, San Román M, Calvo JC, Pérez de Aguado P, Moreno J, Guerrero P, González C, García de Quevedo C, Álvarez-Ballesteros P, Vaz MÁ
Int J Mol Sci 2022 Nov 9;23(22) doi: 10.3390/ijms232213784. PMID: 36430263Free PMC Article
The role of radiation treatment in the contemporary management of bone tumors.
Hristov B, Shokek O, Frassica DA
J Natl Compr Canc Netw 2007 Apr;5(4):456-66. doi: 10.6004/jnccn.2007.0040. PMID: 17442236

Recent clinical studies

Etiology

Receptor Tyrosine Kinase Inhibitors for the Treatment of Recurrent and Unresectable Bone Sarcomas.
Albarrán V, Villamayor ML, Chamorro J, Rosero DI, Pozas J, San Román M, Calvo JC, Pérez de Aguado P, Moreno J, Guerrero P, González C, García de Quevedo C, Álvarez-Ballesteros P, Vaz MÁ
Int J Mol Sci 2022 Nov 9;23(22) doi: 10.3390/ijms232213784. PMID: 36430263Free 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
Efficacy and safety of trabectedin for patients with unresectable and relapsed soft-tissue sarcoma in Japan: A Japanese Musculoskeletal Oncology Group study.
Kobayashi H, Iwata S, Wakamatsu T, Hayakawa K, Yonemoto T, Wasa J, Oka H, Ueda T, Tanaka S
Cancer 2020 Mar 15;126(6):1253-1263. Epub 2019 Dec 11 doi: 10.1002/cncr.32661. PMID: 31825533
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
Rare pulmonary neoplasms.
Miller DL, Allen MS
Mayo Clin Proc 1993 May;68(5):492-8. doi: 10.1016/s0025-6196(12)60199-2. PMID: 8386792

Diagnosis

Biology and Management of High-Grade Chondrosarcoma: An Update on Targets and Treatment Options.
Tlemsani C, Larousserie F, De Percin S, Audard V, Hadjadj D, Chen J, Biau D, Anract P, Terris B, Goldwasser F, Pasmant E, Boudou-Rouquette P
Int J Mol Sci 2023 Jan 10;24(2) doi: 10.3390/ijms24021361. PMID: 36674874Free PMC Article
An Analysis of 20 Cases of Radiation-Associated Sarcoma, Including 4 Cases Treated by Carbon Ion Radiotherapy.
Negishi T, Matsunobu A, Endo M, Yokoyama R, Kusano S, Furubayashi N, Taguchi K, Shioyama Y, Iida K, Fujiwara T, Setsu N, Matsumoto Y, Nakashima Y, Kohashi K, Yamamoto H, Oda Y, Nakamura M
Oncology 2022;100(3):148-154. Epub 2021 Dec 16 doi: 10.1159/000521504. PMID: 34915507
Liposarcomas with mixed well-differentiated and pleomorphic features: a clinicopathologic study of 12 cases.
Boland JM, Weiss SW, Oliveira AM, Erickson-Johnson ML, Folpe AL
Am J Surg Pathol 2010 Jun;34(6):837-43. doi: 10.1097/PAS.0b013e3181dbf2f7. PMID: 20431480
Unusual primary tumors of the heart.
Vander Salm TJ
Semin Thorac Cardiovasc Surg 2000 Apr;12(2):89-100. doi: 10.1053/ct.2000.5080. PMID: 10807431
Rare pulmonary neoplasms.
Miller DL, Allen MS
Mayo Clin Proc 1993 May;68(5):492-8. doi: 10.1016/s0025-6196(12)60199-2. PMID: 8386792

Therapy

Receptor Tyrosine Kinase Inhibitors for the Treatment of Recurrent and Unresectable Bone Sarcomas.
Albarrán V, Villamayor ML, Chamorro J, Rosero DI, Pozas J, San Román M, Calvo JC, Pérez de Aguado P, Moreno J, Guerrero P, González C, García de Quevedo C, Álvarez-Ballesteros P, Vaz MÁ
Int J Mol Sci 2022 Nov 9;23(22) doi: 10.3390/ijms232213784. PMID: 36430263Free PMC Article
An Analysis of 20 Cases of Radiation-Associated Sarcoma, Including 4 Cases Treated by Carbon Ion Radiotherapy.
Negishi T, Matsunobu A, Endo M, Yokoyama R, Kusano S, Furubayashi N, Taguchi K, Shioyama Y, Iida K, Fujiwara T, Setsu N, Matsumoto Y, Nakashima Y, Kohashi K, Yamamoto H, Oda Y, Nakamura M
Oncology 2022;100(3):148-154. Epub 2021 Dec 16 doi: 10.1159/000521504. PMID: 34915507
Efficacy and safety of trabectedin for patients with unresectable and relapsed soft-tissue sarcoma in Japan: A Japanese Musculoskeletal Oncology Group study.
Kobayashi H, Iwata S, Wakamatsu T, Hayakawa K, Yonemoto T, Wasa J, Oka H, Ueda T, Tanaka S
Cancer 2020 Mar 15;126(6):1253-1263. Epub 2019 Dec 11 doi: 10.1002/cncr.32661. PMID: 31825533
The histone deacetylase inhibitor LBH589 inhibits undifferentiated pleomorphic sarcoma growth via downregulation of FOS-like antigen 1.
Saitoh Y, Bureta C, Sasaki H, Nagano S, Maeda S, Furukawa T, Taniguchi N, Setoguchi T
Mol Carcinog 2019 Feb;58(2):234-246. Epub 2018 Nov 2 doi: 10.1002/mc.22922. PMID: 30303565
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

Biology and Management of High-Grade Chondrosarcoma: An Update on Targets and Treatment Options.
Tlemsani C, Larousserie F, De Percin S, Audard V, Hadjadj D, Chen J, Biau D, Anract P, Terris B, Goldwasser F, Pasmant E, Boudou-Rouquette P
Int J Mol Sci 2023 Jan 10;24(2) doi: 10.3390/ijms24021361. PMID: 36674874Free 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
Efficacy and safety of trabectedin for patients with unresectable and relapsed soft-tissue sarcoma in Japan: A Japanese Musculoskeletal Oncology Group study.
Kobayashi H, Iwata S, Wakamatsu T, Hayakawa K, Yonemoto T, Wasa J, Oka H, Ueda T, Tanaka S
Cancer 2020 Mar 15;126(6):1253-1263. Epub 2019 Dec 11 doi: 10.1002/cncr.32661. PMID: 31825533
The histone deacetylase inhibitor LBH589 inhibits undifferentiated pleomorphic sarcoma growth via downregulation of FOS-like antigen 1.
Saitoh Y, Bureta C, Sasaki H, Nagano S, Maeda S, Furukawa T, Taniguchi N, Setoguchi T
Mol Carcinog 2019 Feb;58(2):234-246. Epub 2018 Nov 2 doi: 10.1002/mc.22922. PMID: 30303565
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

The histone deacetylase inhibitor LBH589 inhibits undifferentiated pleomorphic sarcoma growth via downregulation of FOS-like antigen 1.
Saitoh Y, Bureta C, Sasaki H, Nagano S, Maeda S, Furukawa T, Taniguchi N, Setoguchi T
Mol Carcinog 2019 Feb;58(2):234-246. Epub 2018 Nov 2 doi: 10.1002/mc.22922. PMID: 30303565
Liposarcomas with mixed well-differentiated and pleomorphic features: a clinicopathologic study of 12 cases.
Boland JM, Weiss SW, Oliveira AM, Erickson-Johnson ML, Folpe AL
Am J Surg Pathol 2010 Jun;34(6):837-43. doi: 10.1097/PAS.0b013e3181dbf2f7. PMID: 20431480
Carbon ion radiotherapy for unresectable retroperitoneal sarcomas.
Serizawa I, Kagei K, Kamada T, Imai R, Sugahara S, Okada T, Tsuji H, Ito H, Tsujii H
Int J Radiat Oncol Biol Phys 2009 Nov 15;75(4):1105-10. Epub 2009 May 23 doi: 10.1016/j.ijrobp.2008.12.019. PMID: 19467578
Unusual primary tumors of the heart.
Vander Salm TJ
Semin Thorac Cardiovasc Surg 2000 Apr;12(2):89-100. doi: 10.1053/ct.2000.5080. PMID: 10807431
Malignant fibrous histiocytoma in children.
Corpron CA, Black CT, Raney RB, Pollock RE, Lally KP, Andrassy RJ
J Pediatr Surg 1996 Aug;31(8):1080-3. doi: 10.1016/s0022-3468(96)90091-3. PMID: 8863238

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