Clear Cell Sarcoma of the Kidney 

  • Author: Nita Seibel, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Feb 11, 2009
 

Background

Clear cell sarcoma of the kidney (CCSK), an uncommon renal neoplasm of childhood, represents one of the most common tumors with "unfavorable histology" listed in the National Wilms Tumor Study Group (NWTSG) clinical protocols.[1] In 1970, Kidd initially recognized clear cell sarcoma of the kidney as a distinct clinicopathologic entity, noting its propensity to metastasize to bone. The distinctive histopathologic features of clear cell sarcoma of the kidney were reported simultaneously in 1978 by Morgan and Kidd,[2] Marsden et al,[3] and Beckwith and Palmer.[1] These reports confirmed the propensity of the tumor to metastasize to bone, poor clinical outcome, and the sarcomatous nonepithelial nature of the tumor.

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Pathophysiology

Unlike Wilms tumor, clear cell sarcoma of the kidney has not been associated with intralobar nephrogenic rests. In a series of 351 cases from the NWTSG that was reviewed by Argani et al, only one case of clear cell sarcoma of the kidney was associated with a perilobar nephrogenic rest.[4] Gene expression profiles of clear cell sarcomas of the kidney suggest the cell of origin to be a renal mesenchymal cell with neural markers. Only one case has been associated with renal dysplasia, and no familial cases or syndromes have been identified in association with clear cell sarcoma of the kidney. Using the fifth National Wilms Tumor Study (NWTS-5) criteria for tumor staging, 25% of patients had localized stage I tumors, most patients presented with stage II (37%) or stage III (34%) disease, and only 4% of patients presented with distant metastases (see Wilms Tumor for staging information).[5]

No true bilateral primary tumors have been identified. One patient with widespread disseminated disease was noted to have a 1-cm tumor in the contralateral kidney, which was believed to be a metastasis. The most common site of metastasis at the time of presentation in patients with clear cell sarcoma of the kidney is the ipsilateral renal hilar lymph nodes. Skip metastases to periaortic lymph nodes have been reported in patients with clear cell sarcoma of the kidney in the presence of hilar lymph nodes that were histologically confirmed with negative results.

Only 4% of patients present with distant metastases. Bone is the most common site of metastases (15%), followed closely by lung, abdomen, retroperitoneum, brain, and liver. Unusual soft tissue sites (scalp, epidural, nasopharynx, neck, paraspinal, ovary, abdominal wall, axilla) and other sites (orbit) have been reported. Approximately 20% of documented clear cell sarcoma of the kidney metastases occurred at least 3 years after diagnosis; some occurred as long as 10 years later.

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Epidemiology

Frequency

United States

Clear cell sarcoma of the kidney represents less than 3% of pediatric renal tumors. Approximately 20 new cases are diagnosed each year in the United States. Clear cell sarcoma of the kidney is extremely rare in infants younger than 6 months and in young adults. Most patients are aged 1-4 years. A male predominance is observed. Fifty percent of cases are diagnosed in children aged 2-3 years. Around 5% of patients have metastatic disease at presentation.

Mortality/Morbidity

In the fourth National Wilms Tumor Study (NWTS-4), patients were randomized between 6 months of chemotherapy and 15 months of chemotherapy.[6] Patients randomized to 15 months of therapy had a better outcome compared with patients who received the shorter course of chemotherapy. The 8-year relapse-free survival and overall survival were 87.8% and 87.5%, respectively, for patients receiving 15 months of chemotherapy.

Race

Whites and blacks are affected in equal numbers.

Sex

A male predominance has been noted, with a male-to-female ratio of 2.04:1.

Age

Age of presentation ranges from 2 months to 14 years, with a mean age of 36 months. The highest incidence of clear cell sarcoma of the kidney is in children aged 2-3 years, in which 50% of the cases are diagnosed. A sharp decline in incidence occurs in children older than 3 years. Clear cell sarcoma of the kidney is extremely rare in infants younger than 6 months and in young adults, although it has been reported. The oldest reported patient was aged 57 years.

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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, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

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, David Geffen School of Medicine at UCLA and California Nanosystems Institute and Molecular Biology Institute, UCLA

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; Pharmacy Editor, eMedicine

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, Children's National Medical Center (Center for Cancer and Blood Disorders); Director, Center for Cancer and Immunology Research, Children's Research Institute, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University

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, Idaho Medical Association, 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. 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].

  7. 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].

  8. 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].

  9. 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].

  10. 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].

  11. 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].

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

  13. 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].

  14. 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].

  15. 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].

  16. 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].

  17. 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].

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

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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.
 
 
 
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