Pediatric Clear Cell Sarcoma of Kidney

  • Etiology:
  • Imaging:
    — Large solitary and heterogenous mass that is unencapsulated but well-defined with occasional calcification
    — Most arise in medulla
    — Cysts common – dilated tubules or accumulations of mucopolysaccharide matrix
    — Solid components enhance heterogeneously and less than normal kidney
    — Renal hilar involvement and lymph node spread common
  • Imaging MRI:
    — T1WI: Hypointense to intermediate intense
    — T2WI: Hyperintense with fluid signal in cysts
  • DDX:
  • Complications: Bone metastases are a unique feature but are not always present
  • Treatment:
  • Clinical:
    — 5% of renal tumors
    — Mean age 3 years old

Radiology Cases of Clear Cell Sarcoma of Kidney

CT of clear cell sarcoma of the kidney
Axial CT with contrast of the abdomen (above) shows a round, heterogenously enhancing, well-defined lesion arising from the medulla of the left kidney that demonstrates a claw sign. There is also a round focus of high density in the center of the mass anteriorly. Coronal CT (below) shows fluid in the left perirenal and pararenal spaces.