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Clear Cell Sarcoma

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  • Comprise ~3 of childhood renal tumors.
  • Age: 1-3 years old.
  • Presents as abdominal mass.
  • At least 10X as likely to metastasize to bone as any other pediatric renal tumor – "bone metastasizing tumor of childhood".
  • M>F (2:1).
  • Gross: well-circumscribed, may be homogeneous gray and lobular, or may be firm and light pink with whorled areas (images A); bilaterality has not been reported.

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  • Histology:
    • Cells are arranged in cords or packets separated by fibrovascular septa with "chicken-wire" appearance (image B).
    • Cells have lightly staining cytoplasm (not optically clear as in clear cell RCC).
    • Uncommon variant patterns include myxoid (image C) & (image D), sclerosing and spindle cell morphology.
  • Has frequent metastasis to ipsilateral renal hilar lymph node.
  • Aggressive clinical course but depends on stage (stage I has >95% survival).
  • May respond to doxorubicin.