Clear Cell Sarcoma of the Kidney Clinical Presentation

  • Author: Nita Seibel, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Feb 29, 2012
 

History

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.

Large right-sided heterogeneous renal mass in a 9-Large right-sided heterogeneous renal mass in a 9-month-old infant. Biopsy findings were consistent with clear cell sarcoma of the kidney.

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.

Next

Physical

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.

Previous
Next

Causes

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.[9]

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.[10]

Previous
Proceed to Differential Diagnoses
 
 
Contributor Information and Disclosures
Author

Nita Seibel, MD  Senior Investigator, Pediatric Section, Clinical Investigations Branch, Cancer Therapy Evaluation Program, Division of Cancer Treatment and Diagnosis, National Cancer Institute; Adjunct Professor of Pediatrics, George Washington University School of Medicine and Public Health; Attending Physician, Center for Cancer and Blood Disorders, Children's National Medical Center

Nita Seibel, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Society of Clinical Oncology, and American Society of Hematology

Disclosure: Nothing to disclose.

Specialty Editor Board

Kathleen M Sakamoto, MD, PhD  Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Co-Associate Program Director of the Signal Transduction Program Area, Jonsson Comprehensive Cancer Center, California Nanosystems Institute and Molecular Biology Institute, University of California, Los Angeles, David Geffen School of Medicine

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society for Experimental Hematology, Society for Pediatric Research, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Timothy P Cripe, MD, PhD  Professor of Pediatrics, Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center; Clinical Director, Musculoskeletal Tumor Program, Co-Medical Director, Office for Clinical and Translational Research, Cincinnati Children's Hospital Medical Center; Director of Pilot and Collaborative Clinical and Translational Studies Core, Center for Clinical and Translational Science and Training, University of Cincinnati College of Medicine

Timothy P Cripe, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

References

  1. Beckwith JB, Palmer NF. Histopathology and prognosis of Wilms tumors: results from the First National Wilms' Tumor Study. Cancer. May 1978;41(5):1937-48. [Medline].

  2. Morgan E, Kidd JM. Undifferentiated sarcoma of the kidney: a tumor of childhood with histopathologic and clinical characteristics distinct from Wilms' tumor. Cancer. Oct 1978;42(4):1916-21. [Medline].

  3. Marsden HB, Lawler W, Kumar PM. Bone metastasizing renal tumor of childhood: morphological and clinical features, and differences from Wilms' tumor. Cancer. Oct 1978;42(4):1922-8. [Medline].

  4. 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. Jan 2000;24(1):4-18. [Medline].

  5. 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.

  6. Hadley GP, Sheik-Gafoor MH. Clear cell sarcoma of the kidney in children: experience in a developing country. Pediatr Surg Int. Apr 2010;26(4):345-8. [Medline].

  7. 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. Feb 1 2004;22(3):468-73. [Medline].

  8. 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. May 1 2011;52(4):448-57. [Medline].

  9. 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. Sep 2011;78(3):684-6. [Medline].

  10. 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].

  11. 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. Oct 1994;12(10):2132-7. [Medline].

  12. 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. Dec 1999;23(12):1455-63. [Medline].

  13. 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. Mar 2001;8(2):98-108. [Medline].

  14. 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. Mar 2007;131(3):446-51. [Medline].

  15. Charles AK, Vujanic GM, Berry PJ. Renal tumours of childhood. Histopathology. Apr 1998;32(4):293-309. [Medline].

  16. Jones C, Rodriguez-Pinilla M, Lambros M, et al. c-KIT overexpression, without gene amplification and mutation, in paediatric renal tumours. J Clin Pathol. Nov 2007;60(11):1226-31. [Medline].

  17. 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. Aug 1 2007;13(15 Pt 1):4360-4. [Medline].

  18. Punnett HH, Halligan GE, Zaeri N, Karmazin N. Translocation 10;17 in clear cell sarcoma of the kidney. A first report. Cancer Genet Cytogenet. Aug 1989;41(1):123-8. [Medline].

  19. Radulescu VC, Gerrard M, Moertel C, et al. Treatment of recurrent clear cell sarcoma of the kidney with brain metastasis. Pediatr Blood Cancer. Feb 2008;50(2):246-9. [Medline].

  20. 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].

  21. Sebire NJ, Vujanic GM. Paediatric renal tumours: recent developments, new entities and pathological features. Histopathology. Aug 11 2008;[Medline].

 
Previous
Next
 
Large right-sided heterogeneous renal mass in a 9-month-old infant. Biopsy findings were consistent with clear cell sarcoma of the kidney.
Recurrent clear cell sarcoma of the kidney occurring in a lymph node 18 months after therapy.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.