Clear Cell Sarcoma of the Kidney Treatment & Management

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

Medical Care

The approach for treating clear cell sarcoma of the kidney (CCSK) is different from the approach for Wilms tumor because the overall survival of children with clear cell sarcoma of the kidney remains considerably lower than that of patients with favorable-histology Wilms tumor. In the third National Wilms Tumor Study (NWTS-3), the addition of doxorubicin to the combination of vincristine, dactinomycin, and radiation therapy resulted in an improvement in disease-free survival in patients with clear cell sarcoma of the kidney.[8]

NWTS-4 showed that patients treated with vincristine, doxorubicin, and dactinomycin for 15 months had an improved relapse-free survival rate compared with patients treated for 6 months (87.5% vs 60.6% at 8 y).[6] The overall survival has improved for patients with clear cell sarcoma of the kidney from NWTS-3 to NWTS-4 (83% vs 66.9% at 8 y). The 8-year relapse-free survival rate for localized clear cell sarcoma of the kidney stages I-III is 88%, but late relapses have been known to occur. In the NWTS-5 protocol, patients with all stages of CCSK are treated with the same regimen used in patients who have Wilms tumor with diffuse anaplasia (excluding stage I);[8] this treatment consists of a radical nephrectomy followed by radiotherapy and chemotherapy with cyclophosphamide, etoposide, vincristine, and doxorubicin for 24 weeks.

In the NWTSG series that was reviewed by Argani et al, a better prognosis was indicated in the subset of patients with clear cell sarcoma of the kidney that was characterized by stage I tumors in patients aged 2-4 years in whom no tumor necrosis was identified.[4]

In the current Children's Oncology Group protocol (AREN0321), all patients with clear cell sarcoma of the kidney, except patients with stage IV, continue treatment as in NWTS-5. However, patients with stage I who undergo lymph node sampling do not undergo radiation therapy to the tumor bed. Any patient with stage I who has not undergone lymph node sampling is upstaged to stage II. Patients with stage IV undergo treatment with irinotecan and vincristine in an upfront window approach before treatment with cyclophosphamide, etoposide, vincristine, doxorubicin, and cyclophosphamide.

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Surgical Care

At presentation, radical nephrectomy is the initial treatment of choice if the lesion is resectable. If the size or extension of the lesion is in question, a biopsy is performed, and chemotherapy is administered, followed by surgical resection after a response has been obtained.

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Consultations

Radiotherapist

Once the tumor has been resected, the tumor bed and any other sites of disease are irradiated.

Pediatric oncologist

Primary care physicians should consult with a pediatric oncologist to determine standard and investigational treatment protocols.

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Diet

No special diet is required.

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Activity

Patients with clear cell sarcoma of the kidney are advised not to participate in contact sports, especially football. Other activity recommendations are made on an individual basis.

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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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