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-month-old infant. Biopsy findings were consistent with clear cell sarcoma of the kidney.

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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 Examination

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.

Differential Diagnoses

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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. [QxMD MEDLINE Link].
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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. [QxMD MEDLINE Link].
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.
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Ueno-Yokohata H, Okita H, Nakasato K, Akimoto S, Hata J, Koshinaga T, et al. Consistent in-frame internal tandem duplications of BCOR characterize clear cell sarcoma of the kidney. Nat Genet. 2015 Aug. 47 (8):861-3. [QxMD MEDLINE Link].
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Astolfi A, Melchionda F, Perotti D, Fois M, Indio V, Urbini M, et al. Whole transcriptome sequencing identifies BCOR internal tandem duplication as a common feature of clear cell sarcoma of the kidney. Oncotarget. 2015 Dec 1. 6 (38):40934-9. [QxMD MEDLINE Link].
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Kenny C, McDonagh N, Lazaro A, O'Meara E, Klinger R, O'Connor D, et al. Dysregulated mitogen-activated protein kinase signalling as an oncogenic basis for clear cell sarcoma of the kidney. J Pathol. 2018 Mar. 244 (3):334-345. [QxMD MEDLINE Link].
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Argani P, Pawel B, Szabo S, Reyes-Múgica M, Timmons C, Antonescu CR. Diffuse Strong BCOR Immunoreactivity is a Sensitive and Specific Marker for Clear Cell Sarcoma of the Kidney (CCSK) in Pediatric Renal Neoplasia. Am J Surg Pathol. 2018 May 30. [QxMD MEDLINE Link].
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Media Gallery

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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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: American Association for Cancer Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society for Experimental Hematology, Society for Pediatric Research, Western Society for Pediatric Research

Disclosure: Nothing to disclose.