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Clear cell sarcoma of kidney

MedGen UID:
90791
Concept ID:
C0334488
Neoplastic Process
Synonyms: CCSK; CCSK - clear cell sarcoma of kidney; Childhood Clear Cell Sarcoma of the Kidney; childhood clear cell sarcoma of the kidney; Childhood Kidney Clear Cell Sarcoma; childhood kidney clear cell sarcoma; Childhood Renal Clear Cell Sarcoma; childhood renal clear cell sarcoma; clear cell sarcoma - kidney; Clear Cell Sarcoma of Kidney; clear cell sarcoma of kidney; Clear Cell Sarcoma of the Kidney; clear cell sarcoma of the kidney; Kidney Clear Cell Sarcoma; kidney clear cell sarcoma; paediatric kidney clear cell sarcoma; paediatric renal clear cell sarcoma; Pediatric Kidney Clear Cell Sarcoma; pediatric kidney clear cell sarcoma; Pediatric Renal Clear Cell Sarcoma; pediatric renal clear cell sarcoma; Renal Clear Cell Sarcoma; renal clear cell sarcoma
SNOMED CT: Clear cell sarcoma of kidney (24007003); Clear cell sarcoma of kidney (1187464007); CCSK - clear cell sarcoma of kidney (1187464007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0005006
Orphanet: ORPHA457246

Definition

Clear cell sarcoma of kidney is a rare, primary, genetic renal tumor usually characterized by a unilateral, unicentric, morphologically diverse tumor that arises from the renal medulla and has a tendency for vascular invasion. Clinically it presents with a palpable abdominal mass, abdominal or flank pain, hematuria, anemia and/or fatigue. Metastatic spread to lymph nodes, bones, lungs, retroperitoneum, brain and liver is common at time of diagnosis and therefore bone pain, cough or neurological compromise may be associated. Metastasis to unusual sites, such as the scalp, neck, nasopharynx, axilla, orbits and epidural space, have been reported. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVClear cell sarcoma of kidney

Professional guidelines

PubMed

Qureshi SS, Bhagat M, Verma K, Yadav S, Prasad M, Vora T, Chinnaswamy G, Amin N, Smriti V, Baheti A, Laskar S, Khanna N, Ramadwar M, Shah S
J Pediatr Urol 2020 Aug;16(4):475.e1-475.e9. Epub 2020 Jun 5 doi: 10.1016/j.jpurol.2020.05.168. PMID: 32620510
Kenny C, Grehan D, Ulas M, Banga GB, Coulomb A, Vokuhl C, O'Sullivan MJ
Pediatr Dev Pathol 2020 Sep-Oct;23(5):345-351. Epub 2020 May 4 doi: 10.1177/1093526620910658. PMID: 32364435

Recent clinical studies

Etiology

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
Libes J, Hol J, Neto JCA, Vallance KL, Tinteren HV, Benedetti DJ, Villar GLR, Duncan C, Ehrlich PF
Pediatr Blood Cancer 2023 Jan;70(1):e30006. Epub 2022 Nov 3 doi: 10.1002/pbc.30006. PMID: 36326750
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
Singh V, Gupta K, Saraswati A, Peters NJ, Trehan A
J Pediatr Hematol Oncol 2021 Nov 1;43(8):294-300. doi: 10.1097/MPH.0000000000002262. PMID: 34673711
Trejo Bittar HE, Radder JE, Ranganathan S, Srinivasan A, Madan-Khetarpal S, Reyes-Múgica M
Pediatr Dev Pathol 2014 Jul-Aug;17(4):297-301. Epub 2014 Apr 15 doi: 10.2350/14-03-1450-CR.1. PMID: 24735155

Diagnosis

Aviral G, Sarvesh CM, Sushila J, Ansari MS
J Cancer Res Ther 2023 Jul-Sep;19(5):1468-1470. doi: 10.4103/jcrt.jcrt_432_21. PMID: 37787333
Treece AL
Surg Pathol Clin 2020 Dec;13(4):695-718. Epub 2020 Oct 9 doi: 10.1016/j.path.2020.08.003. PMID: 33183728
Kenny C, Grehan D, Ulas M, Banga GB, Coulomb A, Vokuhl C, O'Sullivan MJ
Pediatr Dev Pathol 2020 Sep-Oct;23(5):345-351. Epub 2020 May 4 doi: 10.1177/1093526620910658. PMID: 32364435
Khan MZ, Akhtar N, Hassan U, Mushtaq S
Int J Surg Pathol 2020 Aug;28(5):477-481. Epub 2019 Dec 26 doi: 10.1177/1066896919895119. PMID: 31875746
Dhamne S, Brown RE, Covinsky M, Dhamne C, Eldin K, Tatevian N
Pediatr Dev Pathol 2013 Jan-Feb;16(1):20-7. Epub 2012 Jan 18 doi: 10.2350/11-01-0968-OA.1. PMID: 22256788

Therapy

Deshpande RB
Indian J Pathol Microbiol 2017 Jan-Mar;60(1):115-116. doi: 10.4103/IJPM.IJPM_663_16. PMID: 28195107
Kalapurakal JA, Perlman EJ, Seibel NL, Ritchey M, Dome JS, Grundy PE
Int J Radiat Oncol Biol Phys 2013 Feb 1;85(2):428-31. Epub 2012 Jun 1 doi: 10.1016/j.ijrobp.2012.04.023. PMID: 22658515
Popov SD, Sebire NJ, Pritchard-Jones K, Vujanić GM
Pediatr Dev Pathol 2011 May-Jun;14(3):189-93. Epub 2010 Nov 5 doi: 10.2350/10-06-0855-OA.1. PMID: 21054158
Hazar V, Kutluk T, Akyüz C, Varan A, Yariş N, Büyükpamukçu M
Pediatr Hematol Oncol 1998 Jan-Feb;15(1):85-9. doi: 10.3109/08880019809009513. PMID: 9509511

Prognosis

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
Santiago T, Clay MR, Azzato E, Newman S, Fernandez-Pineda I, Nichols KE, Zhang J, Downing JR, Davidoff A, Brennan RC, Ellison DW
Pediatr Blood Cancer 2017 Nov;64(11) Epub 2017 Apr 25 doi: 10.1002/pbc.26602. PMID: 28440018
Kenny C, Bausenwein S, Lazaro A, Furtwängler R, Gooskens SL, van den Heuvel Eibrink M, Vokuhl C, Leuschner I, Graf N, Gessler M, O'Sullivan MJ
J Pathol 2016 Apr;238(5):617-20. doi: 10.1002/path.4693. PMID: 27000436
Jet Aw S, Hong Kuick C, Hwee Yong M, Wen Quan Lian D, Wang S, Liang Loh AH, Ling S, Lian Peh G, Yen Soh S, Pheng Loh AH, Hoon Tan P, Tou En Chang K
Pediatr Dev Pathol 2015 Jul-Aug;18(4):297-304. Epub 2015 Mar 9 doi: 10.2350/14-12-1581-OA.1. PMID: 25751590
Trejo Bittar HE, Radder JE, Ranganathan S, Srinivasan A, Madan-Khetarpal S, Reyes-Múgica M
Pediatr Dev Pathol 2014 Jul-Aug;17(4):297-301. Epub 2014 Apr 15 doi: 10.2350/14-03-1450-CR.1. PMID: 24735155

Clinical prediction guides

Zhu Y, Fu W, Huang Y, Sun N, Peng Y
BMC Med Imaging 2021 Dec 1;21(1):181. doi: 10.1186/s12880-021-00715-z. PMID: 34847857Free PMC Article
Qureshi SS, Bhagat M, Verma K, Yadav S, Prasad M, Vora T, Chinnaswamy G, Amin N, Smriti V, Baheti A, Laskar S, Khanna N, Ramadwar M, Shah S
J Pediatr Urol 2020 Aug;16(4):475.e1-475.e9. Epub 2020 Jun 5 doi: 10.1016/j.jpurol.2020.05.168. PMID: 32620510
Kenny C, Bausenwein S, Lazaro A, Furtwängler R, Gooskens SL, van den Heuvel Eibrink M, Vokuhl C, Leuschner I, Graf N, Gessler M, O'Sullivan MJ
J Pathol 2016 Apr;238(5):617-20. doi: 10.1002/path.4693. PMID: 27000436
Jet Aw S, Hong Kuick C, Hwee Yong M, Wen Quan Lian D, Wang S, Liang Loh AH, Ling S, Lian Peh G, Yen Soh S, Pheng Loh AH, Hoon Tan P, Tou En Chang K
Pediatr Dev Pathol 2015 Jul-Aug;18(4):297-304. Epub 2015 Mar 9 doi: 10.2350/14-12-1581-OA.1. PMID: 25751590
Trejo Bittar HE, Radder JE, Ranganathan S, Srinivasan A, Madan-Khetarpal S, Reyes-Múgica M
Pediatr Dev Pathol 2014 Jul-Aug;17(4):297-301. Epub 2014 Apr 15 doi: 10.2350/14-03-1450-CR.1. PMID: 24735155

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