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Adult Clear Cell Sarcoma of Soft Tissue

MedGen UID:
233963
•Concept ID:
C1332198
•
Neoplastic Process
Synonym: Adult Clear Cell Sarcoma of Soft Parts

Definition

A clear cell sarcoma of soft tissue occurring during adulthood. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Management of Recurrent Retroperitoneal Sarcoma (RPS) in the Adult: A Consensus Approach from the Trans-Atlantic RPS Working Group.
Trans-Atlantic RPS Working Group
Ann Surg Oncol 2016 Oct;23(11):3531-3540. Epub 2016 Aug 1 doi: 10.1245/s10434-016-5336-7. PMID: 27480354Free PMC Article
About thymic carcinomas: challenges in diagnosis and management.
Mlika M, Boudaya S, Braham E, Chermiti F, Sayi A, Marghli A, El Mezni F
Asian Cardiovasc Thorac Ann 2016 May;24(4):350-4. Epub 2016 Mar 21 doi: 10.1177/0218492316640129. PMID: 27002097
Diagnosis, classification, and management of soft tissue sarcomas.
Mankin HJ, Hornicek FJ
Cancer Control 2005 Jan-Feb;12(1):5-21. doi: 10.1177/107327480501200102. PMID: 15668648

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
Histopathological spectrum of adult renal tumours: A single center experience and review of literature.
Sheenu VS, Paul RS, Pushpa M, Ami EM, Rashmi R, Iona ML, Elizabeth M
Indian J Pathol Microbiol 2023 Apr-Jun;66(2):307-313. doi: 10.4103/ijpm.ijpm_53_21. PMID: 37077073
Primary Pulmonary Hyalinizing Clear Cell Carcinoma: Case Series With Review of Literature.
Thakur S, Nambirajan A, Larsen BT, Butt YM, Roden AC, Kumar S, Jain D
Int J Surg Pathol 2023 Oct;31(7):1187-1194. Epub 2022 Dec 13 doi: 10.1177/10668969221137516. PMID: 36514272
Soft-tissue sarcoma in adolescents and young adults.
Kunisada T, Nakata E, Fujiwara T, Hosono A, Takihira S, Kondo H, Ozaki T
Int J Clin Oncol 2023 Jan;28(1):1-11. Epub 2022 Jan 27 doi: 10.1007/s10147-022-02119-7. PMID: 35084598
Soft Tissue and Visceral Organ Sarcomas With BCOR Alterations.
Whittle SB, Fetzko S, Roy A, Venkatramani R
J Pediatr Hematol Oncol 2022 Jul 1;44(5):195-200. Epub 2022 May 4 doi: 10.1097/MPH.0000000000002480. PMID: 35537005Free PMC Article

Diagnosis

Intestinal Clear Cell Sarcoma-A Case Presentation of an Extremely Rare Tumor and Literature Review.
Rotaru V, Chitoran E, Mitroiu MN, Ionescu SO, Neicu A, Cirimbei C, Alecu M, Gelal A, Prie AD, Simion L
Medicina (Kaunas) 2024 May 22;60(6) doi: 10.3390/medicina60060847. PMID: 38929464Free PMC Article
PRAME immunohistochemistry in soft tissue tumors and mimics: a study of 350 cases highlighting its imperfect specificity but potentially useful diagnostic applications.
Cammareri C, Beltzung F, Michal M, Vanhersecke L, Coindre JM, Velasco V, Le Loarer F, Vergier B, Perret R
Virchows Arch 2023 Aug;483(2):145-156. Epub 2023 Jul 21 doi: 10.1007/s00428-023-03606-6. PMID: 37477762
The many faces of Atypical fibroxanthoma.
Agaimy A
Semin Diagn Pathol 2023 Jul;40(4):306-312. Epub 2023 Jun 23 doi: 10.1053/j.semdp.2023.06.001. PMID: 37438163
Primary Pulmonary Hyalinizing Clear Cell Carcinoma: Case Series With Review of Literature.
Thakur S, Nambirajan A, Larsen BT, Butt YM, Roden AC, Kumar S, Jain D
Int J Surg Pathol 2023 Oct;31(7):1187-1194. Epub 2022 Dec 13 doi: 10.1177/10668969221137516. PMID: 36514272
Machine learning for rhabdomyosarcoma histopathology.
Frankel AO, Lathara M, Shaw CY, Wogmon O, Jackson JM, Clark MM, Eshraghi N, Keenen SE, Woods AD, Purohit R, Ishi Y, Moran N, Eguchi M, Ahmed FUA, Khan S, Ioannou M, Perivoliotis K, Li P, Zhou H, Alkhaledi A, Davis EJ, Galipeau D, Randall RL, Wozniak A, Schoffski P, Lee CJ, Huang PH, Jones RL, Rubin BP, Darrow M, Srinivasa G, Rudzinski ER, Chen S, Berlow NE, Keller C
Mod Pathol 2022 Sep;35(9):1193-1203. Epub 2022 Apr 21 doi: 10.1038/s41379-022-01075-x. PMID: 35449398

Therapy

Paediatric Strategy Forum for medicinal product development of DNA damage response pathway inhibitors in children and adolescents with cancer: ACCELERATE in collaboration with the European Medicines Agency with participation of the Food and Drug Administration.
Pearson ADJ, Federico S, Gatz SA, Ortiz M, Lesa G, Scobie N, Gounaris I, Weiner SL, Weigel B, Unger TJ, Stewart E, Smith M, Slotkin EK, Reaman G, Pappo A, Nysom K, Norga K, McDonough J, Marshall LV, Ludwinski D, Ligas F, Karres D, Kool M, Horner TJ, Henssen A, Heenen D, Hawkins DS, Gore L, Bender JG, Galluzzo S, Fox E, de Rojas T, Davies BR, Chakrabarti J, Carmichael J, Bradford D, Blanc P, Bernardi R, Benchetrit S, Akindele K, Vassal G
Eur J Cancer 2023 Sep;190:112950. Epub 2023 Jun 21 doi: 10.1016/j.ejca.2023.112950. PMID: 37441939
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
Clear cell sarcoma of soft tissue with plasmacytoid morphology: A rare case report.
Han X, Li Q, Wang EH, Liu N
Medicine (Baltimore) 2022 Nov 4;101(44):e31631. doi: 10.1097/MD.0000000000031631. PMID: 36343072Free PMC Article
Vemurafenib in Multiple Nonmelanoma Cancers with BRAF V600 Mutations.
Hyman DM, Puzanov I, Subbiah V, Faris JE, Chau I, Blay JY, Wolf J, Raje NS, Diamond EL, Hollebecque A, Gervais R, Elez-Fernandez ME, Italiano A, Hofheinz RD, Hidalgo M, Chan E, Schuler M, Lasserre SF, Makrutzki M, Sirzen F, Veronese ML, Tabernero J, Baselga J
N Engl J Med 2015 Aug 20;373(8):726-36. doi: 10.1056/NEJMoa1502309. PMID: 26287849Free PMC Article

Prognosis

Soft-tissue sarcoma in adolescents and young adults.
Kunisada T, Nakata E, Fujiwara T, Hosono A, Takihira S, Kondo H, Ozaki T
Int J Clin Oncol 2023 Jan;28(1):1-11. Epub 2022 Jan 27 doi: 10.1007/s10147-022-02119-7. PMID: 35084598
Soft Tissue and Visceral Organ Sarcomas With BCOR Alterations.
Whittle SB, Fetzko S, Roy A, Venkatramani R
J Pediatr Hematol Oncol 2022 Jul 1;44(5):195-200. Epub 2022 May 4 doi: 10.1097/MPH.0000000000002480. PMID: 35537005Free PMC Article
Metastatic Clear-Cell Sarcoma.
Huang X, Liao X
N Engl J Med 2020 Jul 23;383(4):379. doi: 10.1056/NEJMicm1913435. PMID: 32706536
Sarcomas of the skin in the elderly.
Mentzel T
Clin Dermatol 2011 Jan-Feb;29(1):80-90. doi: 10.1016/j.clindermatol.2010.07.011. PMID: 21146736
Clear cell sarcoma.
Pinkerton CR, Philip T
Pediatr Hematol Oncol 1999 Nov-Dec;16(6):495-7. doi: 10.1080/088800199276769. PMID: 10599087

Clinical prediction guides

Intestinal Clear Cell Sarcoma-A Case Presentation of an Extremely Rare Tumor and Literature Review.
Rotaru V, Chitoran E, Mitroiu MN, Ionescu SO, Neicu A, Cirimbei C, Alecu M, Gelal A, Prie AD, Simion L
Medicina (Kaunas) 2024 May 22;60(6) doi: 10.3390/medicina60060847. PMID: 38929464Free PMC Article
The immune landscape of solid pediatric tumors.
Sherif S, Roelands J, Mifsud W, Ahmed EI, Raynaud CM, Rinchai D, Sathappan A, Maaz A, Saleh A, Ozer E, Fakhro KA, Mifsud B, Thorsson V, Bedognetti D, Hendrickx WRL
J Exp Clin Cancer Res 2022 Jun 11;41(1):199. doi: 10.1186/s13046-022-02397-z. PMID: 35690832Free 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
Vemurafenib in Multiple Nonmelanoma Cancers with BRAF V600 Mutations.
Hyman DM, Puzanov I, Subbiah V, Faris JE, Chau I, Blay JY, Wolf J, Raje NS, Diamond EL, Hollebecque A, Gervais R, Elez-Fernandez ME, Italiano A, Hofheinz RD, Hidalgo M, Chan E, Schuler M, Lasserre SF, Makrutzki M, Sirzen F, Veronese ML, Tabernero J, Baselga J
N Engl J Med 2015 Aug 20;373(8):726-36. doi: 10.1056/NEJMoa1502309. PMID: 26287849Free PMC Article
Genetics of pediatric renal tumors.
Royer-Pokora B
Pediatr Nephrol 2013 Jan;28(1):13-23. Epub 2012 Mar 30 doi: 10.1007/s00467-012-2146-4. PMID: 22461142

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