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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

PRAME is a useful marker for the differential diagnosis of melanocytic tumours and histological mimics.
Chen YP, Zhang WW, Qiu YT, Ke LF, Chen H, Chen G
Histopathology 2023 Jan;82(2):285-295. Epub 2022 Oct 13 doi: 10.1111/his.14814. PMID: 36200756
Biology and management of clear cell sarcoma: state of the art and future perspectives.
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
Wilms' tumor management.
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

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
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
Clear Cell Sarcoma of the Kidney.
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
BCOR involvement in cancer.
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
Clear cell sarcoma of the gastrointestinal tract and malignant gastrointestinal neuroectodermal tumour: distinct or related entities? A review.
Green C, Spagnolo DV, Robbins PD, Fermoyle S, Wong DD
Pathology 2018 Aug;50(5):490-498. Epub 2018 Jun 30 doi: 10.1016/j.pathol.2018.05.001. PMID: 29970252

Diagnosis

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
Neonatal Renal Tumors.
Sze SK
Clin Perinatol 2021 Mar;48(1):71-81. Epub 2021 Jan 11 doi: 10.1016/j.clp.2020.11.004. PMID: 33583508
Clear Cell Sarcoma of the Kidney.
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
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

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
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
Biology and management of clear cell sarcoma: state of the art and future perspectives.
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
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
Wilms' tumor management.
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

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
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
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
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
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

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
Differentiation between Clear Cell Sarcoma of the Kidney and Wilms' Tumor with CT.
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
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
Clear cell sarcoma of the kidney: a review.
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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