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Clear cell sarcoma

MedGen UID:
104909
Concept ID:
C0206651
Neoplastic Process
Synonyms: Clear Cell Sarcoma; Clear Cell Sarcoma of Soft Tissue; Clear Cell Sarcomas; Malignant Melanoma of Soft Parts; Melanoma of Soft Parts; Melanoma, Malignant, of Soft Parts; Sarcoma, Clear Cell; Sarcomas, Clear Cell
SNOMED CT: Malignant melanoma of soft tissues (402561003); Clear cell sarcoma (402561003); Malignant melanoma of soft tissue (271944004); Clear cell sarcoma (271944004)
 
Monarch Initiative: MONDO:0002926

Definition

A rare malignant neoplasm with melanocytic differentiation characterized by the presence of polygonal or spindle shaped clear cells. This sarcoma usually affects the tendons and aponeuroses and is associated with a poor prognosis due to recurrences and metastases. [from NCI]

Professional guidelines

PubMed

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
Datta NK, Das KP, Aish PK, Datta M, Banik SK, Sen SK, Chowdhury RM
Mymensingh Med J 2023 Jan;32(1):135-143. PMID: 36594313
Wu HY, Snyder HM 3rd, D'Angio GJ
Curr Opin Urol 2005 Jul;15(4):273-6. doi: 10.1097/01.mou.0000172403.69890.eb. PMID: 15928519

Recent clinical studies

Etiology

Geller JI, Hong AL, Vallance KL, Evageliou N, Aldrink JH, Cost NG, Treece AL, Renfro LA, Mullen EA; COG Renal Tumor Committee
Pediatr Blood Cancer 2023 Sep;70 Suppl 6(Suppl 6):e30586. Epub 2023 Jul 21 doi: 10.1002/pbc.30586. PMID: 37477907Free PMC Article
Libes J, Hol J, Neto JCA, Vallance KL, Tinteren HV, Benedetti DJ, Villar GLR, Duncan C, Ehrlich PF
Pediatr Blood Cancer 2023 May;70 Suppl 2:e30343. doi: 10.1002/pbc.30343. PMID: 37096796
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
Aldera AP, Pillay K
Arch Pathol Lab Med 2020 Jan;144(1):119-123. Epub 2019 Mar 27 doi: 10.5858/arpa.2018-0353-RS. PMID: 30917048
Astolfi A, Fiore M, Melchionda F, Indio V, Bertuccio SN, Pession A
Epigenomics 2019 May;11(7):835-855. Epub 2019 May 31 doi: 10.2217/epi-2018-0195. PMID: 31150281Free PMC Article

Diagnosis

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
Sze SK
Clin Perinatol 2021 Mar;48(1):71-81. Epub 2021 Jan 11 doi: 10.1016/j.clp.2020.11.004. PMID: 33583508
Aldera AP, Pillay K
Arch Pathol Lab Med 2020 Jan;144(1):119-123. Epub 2019 Mar 27 doi: 10.5858/arpa.2018-0353-RS. PMID: 30917048
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
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

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
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
Cornillie J, van Cann T, Wozniak A, Hompes D, Schöffski P
Expert Rev Anticancer Ther 2016 Aug;16(8):839-45. Epub 2016 Jun 20 doi: 10.1080/14737140.2016.1197122. PMID: 27253849
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
Wu HY, Snyder HM 3rd, D'Angio GJ
Curr Opin Urol 2005 Jul;15(4):273-6. doi: 10.1097/01.mou.0000172403.69890.eb. PMID: 15928519

Prognosis

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
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
Huang X, Liao X
N Engl J Med 2020 Jul 23;383(4):379. doi: 10.1056/NEJMicm1913435. PMID: 32706536
Aldera AP, Pillay K
Arch Pathol Lab Med 2020 Jan;144(1):119-123. Epub 2019 Mar 27 doi: 10.5858/arpa.2018-0353-RS. PMID: 30917048
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

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
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
Kang C, Shin HJ, Yoon H, Han JW, Lyu CJ, Lee MJ
Korean J Radiol 2021 Jul;22(7):1185-1193. Epub 2021 Apr 1 doi: 10.3348/kjr.2020.0882. PMID: 33856131Free PMC Article
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
Gooskens SL, Furtwängler R, Vujanic GM, Dome JS, Graf N, van den Heuvel-Eibrink MM
Eur J Cancer 2012 Sep;48(14):2219-26. Epub 2012 May 12 doi: 10.1016/j.ejca.2012.04.009. PMID: 22579455

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