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Clear Cell Sarcoma of Kidney
Ethan A. Smith, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Clear cell sarcoma of kidney (CCSK)
          • Synonyms

            • Bone metastasizing tumor of childhood
          • Definitions

            • 2nd most common malignant renal neoplasm in young children
              • Still rare, only 5% of renal tumors in children

          IMAGING

          • General Features

            • Radiographic Findings

              • Ultrasonographic Findings

                • CT Findings

                  • MR Findings

                    • Nuclear Medicine Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Aldera AP et al: Clear cell sarcoma of the kidney. Arch Pathol Lab Med. 144(1):119-23, 2020
                                            2. Seibel NL et al: Impact of cyclophosphamide and etoposide on outcome of clear cell sarcoma of the kidney treated on the National Wilms Tumor Study-5 (NWTS-5). Pediatr Blood Cancer. 66(1):e27450, 2019
                                            3. Stanescu AL et al: Pediatric renal neoplasms: MR imaging-based practical diagnostic approach. Magn Reson Imaging Clin N Am. 27(2):279-90, 2019
                                            4. Chung EM et al: Renal tumors of childhood: radiologic-pathologic correlation part 1. The 1st decade: from the radiologic pathology archives. Radiographics. 36(2):499-522, 2016
                                            5. Gee MS et al: Magnetic resonance imaging of the pediatric kidney: benign and malignant masses. Magn Reson Imaging Clin N Am. 21(4):697-715, 2013
                                            6. Royer-Pokora B: Genetics of pediatric renal tumors. Pediatr Nephrol. 28(1):13-23, 2013
                                            7. Gooskens SL et al: Clear cell sarcoma of the kidney: a review. Eur J Cancer. 48(14):2219-26, 2012
                                            8. Geller E et al: Renal neoplasms of childhood. Radiol Clin North Am. 49(4):689-709, vi, 2011
                                            9. Argani P 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. 24(1):4-18, 2000
                                            10. Glass RB et al: Clear cell sarcoma of the kidney: CT, sonographic, and pathologic correlation. Radiology. 180(3):715-7, 1991
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Pathology

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Clear cell sarcoma of kidney (CCSK)
                                                    • Synonyms

                                                      • Bone metastasizing tumor of childhood
                                                    • Definitions

                                                      • 2nd most common malignant renal neoplasm in young children
                                                        • Still rare, only 5% of renal tumors in children

                                                    IMAGING

                                                    • General Features

                                                      • Radiographic Findings

                                                        • Ultrasonographic Findings

                                                          • CT Findings

                                                            • MR Findings

                                                              • Nuclear Medicine Findings

                                                                • Imaging Recommendations

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      • Staging, Grading, & Classification

                                                                        • Gross Pathologic & Surgical Features

                                                                          • Microscopic Features

                                                                            CLINICAL ISSUES

                                                                            • Presentation

                                                                              • Demographics

                                                                                • Natural History & Prognosis

                                                                                  • Treatment

                                                                                    DIAGNOSTIC CHECKLIST

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Aldera AP et al: Clear cell sarcoma of the kidney. Arch Pathol Lab Med. 144(1):119-23, 2020
                                                                                      2. Seibel NL et al: Impact of cyclophosphamide and etoposide on outcome of clear cell sarcoma of the kidney treated on the National Wilms Tumor Study-5 (NWTS-5). Pediatr Blood Cancer. 66(1):e27450, 2019
                                                                                      3. Stanescu AL et al: Pediatric renal neoplasms: MR imaging-based practical diagnostic approach. Magn Reson Imaging Clin N Am. 27(2):279-90, 2019
                                                                                      4. Chung EM et al: Renal tumors of childhood: radiologic-pathologic correlation part 1. The 1st decade: from the radiologic pathology archives. Radiographics. 36(2):499-522, 2016
                                                                                      5. Gee MS et al: Magnetic resonance imaging of the pediatric kidney: benign and malignant masses. Magn Reson Imaging Clin N Am. 21(4):697-715, 2013
                                                                                      6. Royer-Pokora B: Genetics of pediatric renal tumors. Pediatr Nephrol. 28(1):13-23, 2013
                                                                                      7. Gooskens SL et al: Clear cell sarcoma of the kidney: a review. Eur J Cancer. 48(14):2219-26, 2012
                                                                                      8. Geller E et al: Renal neoplasms of childhood. Radiol Clin North Am. 49(4):689-709, vi, 2011
                                                                                      9. Argani P 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. 24(1):4-18, 2000
                                                                                      10. Glass RB et al: Clear cell sarcoma of the kidney: CT, sonographic, and pathologic correlation. Radiology. 180(3):715-7, 1991