Clear Cell Sarcoma of the Kidney Clinical Presentation
- Author: Nita Seibel, MD; Chief Editor: Max J Coppes, MD, PhD, MBA more...
Manifestations in patients with clear cell sarcoma of the kidney (CCSK) are similar to those in patients with Wilms tumor. Patients present with an abdominal mass (see the image below), which is usually identified by a caregiver or family relative who has not seen the child in some time.
Often, abdominal swelling or the presence of an abdominal mass is noticed by a parent while bathing or dressing the child. Abdominal pain, gross hematuria, fever, and hypertension are other frequent findings.
Physical findings include a large palpable unilateral abdominal mass. Patients may have accompanying findings, such as hypertension and/or hematuria (gross or microscopic), depending on the size of the tumor. Extrarenal tumors with histologic features identical to those of clear cell sarcoma of the kidney have been reported.
The histogenesis of clear cell sarcoma of the kidney is unknown and appears to be unrelated to Wilms tumor. No specific chromosomal translocation has been associated with clear cell sarcoma of the kidney; a finding that generally indicates a normal karyotype.
The origin of clear cell sarcoma of the kidney has not been established. Dysregulation of the EGFR pathway has been observed at multiple levels in clear cell sarcoma of the kidney. The proto-oncogene c-kit is overexpressed in clear cell sarcoma of the kidney but is not accompanied by gene amplification or activating mutations. The t(10;17)(q22;p13) and deletion 14q have been described. Cells that have been suggested as the origin for clear cell sarcoma of the kidney include renomedullary interstitial cells, nonorgan specific mesenchymal cells, blastemal cap cells, primitive mesenchymal cells, and the cells that form the lower limbs of S-bodies. Cutcliffe et al have suggested that the cell of origin is within a renal mesenchymal cell that possesses neural markers.
Beckwith JB, Palmer NF. Histopathology and prognosis of Wilms tumors: results from the First National Wilms' Tumor Study. Cancer. 1978 May. 41(5):1937-48. [Medline].
Morgan E, Kidd JM. Undifferentiated sarcoma of the kidney: a tumor of childhood with histopathologic and clinical characteristics distinct from Wilms' tumor. Cancer. 1978 Oct. 42(4):1916-21. [Medline].
Marsden HB, Lawler W, Kumar PM. Bone metastasizing renal tumor of childhood: morphological and clinical features, and differences from Wilms' tumor. Cancer. 1978 Oct. 42(4):1922-8. [Medline].
Argani P, Perlman EJ, Breslow NE, et al. Clear cell sarcoma of the kidney: a review of 351 cases from the National Wilms Tumor Study Group Pathology Center. Am J Surg Pathol. 2000 Jan. 24(1):4-18. [Medline].
Seibel NL, Sun J, Anderson JR, et al. Outcome of clear cell sarcoma of the kidney (CCSK) treated on the National Wilms Tumor Study-5 (NWTS). [Abstract. J Clin Oncol(Supplement 18). 2006. 24:A9000.
Hadley GP, Sheik-Gafoor MH. Clear cell sarcoma of the kidney in children: experience in a developing country. Pediatr Surg Int. 2010 Apr. 26(4):345-8. [Medline].
Seibel NL, Li S, Breslow NE, et al. Effect of duration of treatment on treatment outcome for patients with clear-cell sarcoma of the kidney: a report from the National Wilms' Tumor Study Group. J Clin Oncol. 2004 Feb 1. 22(3):468-73. [Medline].
Lalwani N, Prasad SR, Vikram R, Katabathina V, Shanbhogue A, Restrepo C. Pediatric and adult primary sarcomas of the kidney: a cross-sectional imaging review. Acta Radiol. 2011 May 1. 52(4):448-57. [Medline].
Taguchi K, Kojima Y, Mizuno K, Kamisawa H, Kohri K, Hayashi Y. Molecular analysis of clear cell sarcoma with translocation (1;6)(p32.3;q21). Urology. 2011 Sep. 78(3):684-6. [Medline].
Cutcliffe C, Kersey D, Huang CC, et al. Clear cell sarcoma of kidney: up-regulation of neural markers with activation of the sonic hedgehog and Akt pathways. Clin Can Res. 2005. 11:7986-7994. [Medline]. [Full Text].
Green DM, Breslow NE, Beckwith JB, et al. Treatment of children with clear-cell sarcoma of the kidney: a report from the National Wilms' Tumor Study Group. J Clin Oncol. 1994 Oct. 12(10):2132-7. [Medline].
Gooskens SL, Furtwängler R, Spreafico F, van Tinteren H, de Kraker J, Vujanic GM, et al. Treatment and outcome of patients with relapsed clear cell sarcoma of the kidney: a combined SIOP and AIEOP study. Br J Cancer. 2014 Jul 15. 111 (2):227-33. [Medline]. [Full Text].
Kalapurakal JA, Perlman EJ, Seibel NL, Ritchey M, Dome JS, Grundy PE. Outcomes of patients with revised stage I clear cell sarcoma of kidney treated in National Wilms Tumor Studies 1-5. Int J Radiat Oncol Biol Phys. 2013 Feb 1. 85 (2):428-31. [Medline].
Amin MB, de Peralta-Venturina MN, Ro JY, et al. Clear cell sarcoma of kidney in an adolescent and in young adults: a report of four cases with ultrastructural, immunohistochemical, and DNA flow cytometric analysis. Am J Surg Pathol. 1999 Dec. 23(12):1455-63. [Medline].
Balarezo FS, Joshi VV. Clear cell sarcoma of the pediatric kidney: detailed description and analysis of variant histologic patterns of a tumor with many faces. Adv Anat Pathol. 2001 Mar. 8(2):98-108. [Medline].
Brownlee NA, Perkins LA, Stewart W, et al. Recurring translocation (10;17) and deletion (14q) in clear cell sarcoma of the kidney. Arch Pathol Lab Med. 2007 Mar. 131(3):446-51. [Medline].
Charles AK, Vujanic GM, Berry PJ. Renal tumours of childhood. Histopathology. 1998 Apr. 32(4):293-309. [Medline].
Jones C, Rodriguez-Pinilla M, Lambros M, et al. c-KIT overexpression, without gene amplification and mutation, in paediatric renal tumours. J Clin Pathol. 2007 Nov. 60(11):1226-31. [Medline].
Little SE, Bax DA, Rodriguez-Pinilla M, et al. Multifaceted dysregulation of the epidermal growth factor receptor pathway in clear cell sarcoma of the kidney. Clin Cancer Res. 2007 Aug 1. 13(15 Pt 1):4360-4. [Medline].
Punnett HH, Halligan GE, Zaeri N, Karmazin N. Translocation 10;17 in clear cell sarcoma of the kidney. A first report. Cancer Genet Cytogenet. 1989 Aug. 41(1):123-8. [Medline].
Radulescu VC, Gerrard M, Moertel C, et al. Treatment of recurrent clear cell sarcoma of the kidney with brain metastasis. Pediatr Blood Cancer. 2008 Feb. 50(2):246-9. [Medline].
Rakheja D, Weinberg AG, Tomlinson GE, et al. Translocation (10;17)(q22;p13): a recurring translocation in clear cell sarcoma of kidney. CancerGenet Cytogenet. 2004. 154:175-9. [Medline].
Sebire NJ, Vujanic GM. Paediatric renal tumours: recent developments, new entities and pathological features. Histopathology. 2008 Aug 11. [Medline].