Pediatric Rhabdoid Tumor

  • Etiology: Monomorphous population of large polygonal rhabdoid cells
  • Imaging:
    — Arises centrally with hilar invasion common
    — Heterogenous with calcification in 66%
    — Unencapsulated with indistinct margin
    — Subcapsular hemorrhage
    — Peripheral tumor necrosis resulting in crescent in 71% which is characteristic but not diagnostic
  • DDX:
  • Complications: 25% with concurrent CNS tumor of primary atypical teratoid rhabdoid tumor in posterior fossa or brain metastasis from renal tumor
  • Treatment: Surgical
  • Clinical:
    — 2% of renal tumors
    — Presents at mean age of 11 months
    — Hypercalcemia of infancy
    — Highly aggressive with poor prognosis

Radiology Cases of Rhabdoid Tumor

CT of rhabdoid tumor of kidney
Axial (above), coronal (below left) and sagittal (below right) CT with contrast of the abdomen shows a large mass arising from within the right kidney. A claw sign is present. Inferolaterally, the mass extends outside the renal capsule. There is also lymphadenopathy encircling the aorta.

Gross Pathology Cases of Rhabdoid Tumor

Gross pathological image of rhabdoid tumor of the kidney
Gross pathological image of the kidney (above) and sectioned gross pathological image of the kidney (below) shows a bulging, well circumscribed, white-tan tumor that is approximately 20% necrotic and that did not penetrate the renal capsule.