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Advanced Clear Cell Sarcoma of Soft Tissue

MedGen UID:
1704361
•Concept ID:
C5205916
•
Neoplastic Process

Definition

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

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdvanced Clear Cell Sarcoma of Soft Tissue

Professional guidelines

PubMed

Clear cell chondrosarcoma: a review of clinicopathologic characteristics, differential diagnoses, and patient management.
Alexiev BA, Vormittag-Nocito ER, Peabody TD, Samet J, Laskin WB
Hum Pathol 2023 Sep;139:126-134. Epub 2023 Jun 16 doi: 10.1016/j.humpath.2023.06.004. PMID: 37805864
Canadian Consensus Recommendations on the Management of KRAS G12C-Mutated NSCLC.
Cheema PK, Banerji SO, Blais N, Chu QS, Juergens RA, Leighl NB, Sacher A, Sheffield BS, Snow S, Vincent M, Wheatley-Price PF, Yip S, Melosky BL
Curr Oncol 2023 Jul 6;30(7):6473-6496. doi: 10.3390/curroncol30070476. PMID: 37504336Free PMC Article
The current management of clear cell sarcoma.
Ikuta K, Nishida Y, Imagama S, Tanaka K, Ozaki T
Jpn J Clin Oncol 2023 Oct 4;53(10):899-904. doi: 10.1093/jjco/hyad083. PMID: 37451697

Recent clinical studies

Etiology

Pembrolizumab in patients with rare and ultra-rare sarcomas (AcSé Pembrolizumab): analysis of a subgroup from a non-randomised, open-label, phase 2, basket trial.
Blay JY, Chevret S, Le Cesne A, Brahmi M, Penel N, Cousin S, Bertucci F, Bompas E, Ryckewaert T, Soibinet P, Boudou-Rouquette P, Saada Bouzid E, Soulie P, Valentin T, Lotz JP, Tosi D, Neviere Z, Cancel M, Ray-Coquard I, Gambotti L, Legrand F, Lamrani-Ghaouti A, Simon C, Even C, Massard C
Lancet Oncol 2023 Aug;24(8):892-902. Epub 2023 Jul 7 doi: 10.1016/S1470-2045(23)00282-6. PMID: 37429302
Pulmonary Sarcomatoid Carcinomas: A Review.
Weissferdt A
Adv Anat Pathol 2018 Sep;25(5):304-313. doi: 10.1097/PAP.0000000000000202. PMID: 29912718
Cancer and mTOR Inhibitors in Transplant Recipients.
de Fijter JW
Transplantation 2017 Jan;101(1):45-55. doi: 10.1097/TP.0000000000001447. PMID: 27547865
Role of chemotherapy in the management of soft tissue sarcomas.
Krikelis D, Judson I
Expert Rev Anticancer Ther 2010 Feb;10(2):249-60. doi: 10.1586/era.09.176. PMID: 20132000
Malignant tumors and AIDS.
Nasti G, Vaccher E, Errante D, Tirelli U
Biomed Pharmacother 1997;51(6-7):243-51. doi: 10.1016/S0753-3322(97)83539-1. PMID: 9309244

Diagnosis

Clear cell chondrosarcoma: a review of clinicopathologic characteristics, differential diagnoses, and patient management.
Alexiev BA, Vormittag-Nocito ER, Peabody TD, Samet J, Laskin WB
Hum Pathol 2023 Sep;139:126-134. Epub 2023 Jun 16 doi: 10.1016/j.humpath.2023.06.004. PMID: 37805864
The current management of clear cell sarcoma.
Ikuta K, Nishida Y, Imagama S, Tanaka K, Ozaki T
Jpn J Clin Oncol 2023 Oct 4;53(10):899-904. doi: 10.1093/jjco/hyad083. PMID: 37451697
Molecular Signature of Biological Aggressiveness in Clear Cell Sarcoma of the Kidney (CCSK).
Fiore M, Taddia A, Indio V, Bertuccio SN, Messelodi D, Serravalle S, Bandini J, Spreafico F, Perotti D, Collini P, Di Cataldo A, Pasquinelli G, Chiarini F, Fois M, Melchionda F, Pession A, Astolfi A
Int J Mol Sci 2023 Feb 13;24(4) doi: 10.3390/ijms24043743. PMID: 36835166Free PMC Article
Clear Cell Sarcoma of the Kidney.
Aw SJ, Chang KTE
Arch Pathol Lab Med 2019 Aug;143(8):1022-1026. Epub 2019 Jan 10 doi: 10.5858/arpa.2018-0045-RS. PMID: 30628851
Options for treating different soft tissue sarcoma subtypes.
Ray-Coquard I, Serre D, Reichardt P, Martín-Broto J, Bauer S
Future Oncol 2018 May;14(10s):25-49. doi: 10.2217/fon-2018-0076. PMID: 29768052

Therapy

Pembrolizumab in patients with rare and ultra-rare sarcomas (AcSé Pembrolizumab): analysis of a subgroup from a non-randomised, open-label, phase 2, basket trial.
Blay JY, Chevret S, Le Cesne A, Brahmi M, Penel N, Cousin S, Bertucci F, Bompas E, Ryckewaert T, Soibinet P, Boudou-Rouquette P, Saada Bouzid E, Soulie P, Valentin T, Lotz JP, Tosi D, Neviere Z, Cancel M, Ray-Coquard I, Gambotti L, Legrand F, Lamrani-Ghaouti A, Simon C, Even C, Massard C
Lancet Oncol 2023 Aug;24(8):892-902. Epub 2023 Jul 7 doi: 10.1016/S1470-2045(23)00282-6. PMID: 37429302
Targeting epigenetic features in clear cell sarcomas based on patient-derived cell lines.
Karner C, Anders I, Vejzovic D, Szkandera J, Scheipl S, Deutsch AJA, Weiss L, Vierlinger K, Kolb D, Kühberger S, Heitzer E, Habisch H, Zhang F, Madl T, Reininger-Gutmann B, Liegl-Atzwanger B, Rinner B
J Transl Med 2023 Jan 29;21(1):54. doi: 10.1186/s12967-022-03843-4. PMID: 36710341Free PMC Article
Options for treating different soft tissue sarcoma subtypes.
Ray-Coquard I, Serre D, Reichardt P, Martín-Broto J, Bauer S
Future Oncol 2018 May;14(10s):25-49. doi: 10.2217/fon-2018-0076. PMID: 29768052
Cancer and mTOR Inhibitors in Transplant Recipients.
de Fijter JW
Transplantation 2017 Jan;101(1):45-55. doi: 10.1097/TP.0000000000001447. PMID: 27547865
Role of chemotherapy in the management of soft tissue sarcomas.
Krikelis D, Judson I
Expert Rev Anticancer Ther 2010 Feb;10(2):249-60. doi: 10.1586/era.09.176. PMID: 20132000

Prognosis

The current management of clear cell sarcoma.
Ikuta K, Nishida Y, Imagama S, Tanaka K, Ozaki T
Jpn J Clin Oncol 2023 Oct 4;53(10):899-904. doi: 10.1093/jjco/hyad083. PMID: 37451697
Molecular Signature of Biological Aggressiveness in Clear Cell Sarcoma of the Kidney (CCSK).
Fiore M, Taddia A, Indio V, Bertuccio SN, Messelodi D, Serravalle S, Bandini J, Spreafico F, Perotti D, Collini P, Di Cataldo A, Pasquinelli G, Chiarini F, Fois M, Melchionda F, Pession A, Astolfi A
Int J Mol Sci 2023 Feb 13;24(4) doi: 10.3390/ijms24043743. PMID: 36835166Free PMC Article
Clear cell sarcoma: state-of-the art and perspectives.
Wetterwald L, Riggi N, Kyriazoglou A, Dei Tos G, Dei Tos A, Digklia A
Expert Rev Anticancer Ther 2023 Mar;23(3):235-242. Epub 2023 Feb 27 doi: 10.1080/14737140.2023.2183846. PMID: 36811446
Pulmonary Sarcomatoid Carcinomas: A Review.
Weissferdt A
Adv Anat Pathol 2018 Sep;25(5):304-313. doi: 10.1097/PAP.0000000000000202. PMID: 29912718
Options for treating different soft tissue sarcoma subtypes.
Ray-Coquard I, Serre D, Reichardt P, Martín-Broto J, Bauer S
Future Oncol 2018 May;14(10s):25-49. doi: 10.2217/fon-2018-0076. PMID: 29768052

Clinical prediction guides

Functional genomics of human clear cell sarcoma: genomic, transcriptomic and chemical biology landscape for clear cell sarcoma.
Rasmussen SV, Wozniak A, Lathara M, Goldenberg JM, Samudio BM, Bickford LR, Nagamori K, Wright H, Woods AD, Chauhan S, Lee CJ, Rudzinski ER, Swift MK, Kondo T, Fisher DE, Imyanitov E, Machado I, Llombart-Bosch A, Andrulis IL, Gokgoz N, Wunder J, Mirotaki H, Nakamura T, Srinivasa G, Thway K, Jones RL, Huang PH, Berlow NE, Schöffski P, Keller C
Br J Cancer 2023 May;128(10):1941-1954. Epub 2023 Mar 23 doi: 10.1038/s41416-023-02222-0. PMID: 36959380Free PMC Article
EWSR1-ATF1 dependent 3D connectivity regulates oncogenic and differentiation programs in Clear Cell Sarcoma.
Möller E, Praz V, Rajendran S, Dong R, Cauderay A, Xing YH, Lee L, Fusco C, Broye LC, Cironi L, Iyer S, Rengarajan S, Awad ME, Naigles B, Letovanec I, Ormas N, Finzi G, La Rosa S, Sessa F, Chebib I, Petur Nielsen G, Digklia A, Spentzos D, Cote GM, Choy E, Aryee M, Stamenkovic I, Boulay G, Rivera MN, Riggi N
Nat Commun 2022 Apr 27;13(1):2267. doi: 10.1038/s41467-022-29910-4. PMID: 35477713Free PMC Article
The clear cell sarcoma functional genomic landscape.
Panza E, Ozenberger BB, Straessler KM, Barrott JJ, Li L, Wang Y, Xie M, Boulet A, Titen SW, Mason CC, Lazar AJ, Ding L, Capecchi MR, Jones KB
J Clin Invest 2021 Aug 2;131(15) doi: 10.1172/JCI146301. PMID: 34156976Free PMC Article
Pulmonary Sarcomatoid Carcinomas: A Review.
Weissferdt A
Adv Anat Pathol 2018 Sep;25(5):304-313. doi: 10.1097/PAP.0000000000000202. PMID: 29912718
Cancer and mTOR Inhibitors in Transplant Recipients.
de Fijter JW
Transplantation 2017 Jan;101(1):45-55. doi: 10.1097/TP.0000000000001447. PMID: 27547865

Recent systematic reviews

Clinicopathological Features Associated with Microsatellite Instability/Mismatch Repair Deficiency in Uterine Carcinosarcoma: A Quantitative Systematic Review.
Travaglino A, Raffone A, Santoro A, Raimondo D, Orsini B, Casadio P, Zullo F, Seracchioli R, Zannoni GF, Insabato L, Mollo A
Pathobiology 2022;89(4):198-204. Epub 2022 Mar 1 doi: 10.1159/000521876. PMID: 35231921
Autologous haematopoietic stem cell transplantation following high-dose chemotherapy for non-rhabdomyosarcoma soft tissue sarcomas: a Cochrane systematic review*.
Peinemann F, Labeit AM
BMJ Open 2014 Jul 29;4(7):e005033. doi: 10.1136/bmjopen-2014-005033. PMID: 25079925Free PMC Article

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