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Clear Cell Sarcoma of the Kidney

  • Author: Nita Seibel, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
 
Updated: Oct 25, 2015
 

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.

International

Hadley et al concluded that in a developing country, such as South Africa, the disease is rare, clinically and radiologically indistinguishable from Wilms tumor, and commonly accompanied by hypertension. Treatment results are poor and difficult to sustain.[6]

Mortality/Morbidity

In the fourth National Wilms Tumor Study (NWTS-4), patients were randomized between 6 months of chemotherapy and 15 months of chemotherapy.[7] 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.[8]

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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 Society of Clinical Oncology, American Academy of Pediatrics, American Society of Hematology

Disclosure: Nothing to disclose.

Specialty Editor Board

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, FAAP Chief, Division of Hematology/Oncology/BMT, Gordon Teter Endowed Chair in Pediatric Cancer, Nationwide Children's Hospital; Professor of Pediatrics, Ohio State University College of Medicine

Timothy P Cripe, MD, PhD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Association for Cancer Research, American Pediatric Society, American Society of Gene and Cell Therapy, American Society of Pediatric Hematology/Oncology, Connective Tissue Oncology Society, Society for Pediatric Research, Children's Oncology Group

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath

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

Disclosure: Nothing to disclose.

Additional Contributors

Kathleen M Sakamoto, MD, PhD Shelagh Galligan Professor, Division of Hematology/Oncology, Department of Pediatrics, Stanford University School of Medicine

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

Disclosure: Nothing to disclose.

References
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  2. 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].

  3. 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].

  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. 2000 Jan. 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. 2010 Apr. 26(4):345-8. [Medline].

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  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. 2011 Sep. 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. 1994 Oct. 12(10):2132-7. [Medline].

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  16. 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].

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  19. 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].

  20. 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].

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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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