Rhabdoid tumor

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[edit] Discussion of Rhabdoid tumor

  • Rhabdoid tumor is a rare, highly aggressive malignancy of early childhood.
  • Its name is derived from its histologic appearance, which resembles that of a tumor of skeletal muscle origin.
  • Rhabdoid tumor occurs exclusively in children, comprising 2% of pediatric renal malignancies.
  • Approximately 80% occur in patients less than 2 years of age and 60% in patients less than 1 year of age.

  • Rhabdoid tumor may manifest as hematuria, but due to its aggressive nature, symptoms may be referable to metastatic disease.
  • The association of rhabdoid tumor with synchronous or metachronous primary intracranial masses or brain metastases has been established as a distinctive feature.
  • Clinically, patients may develop hypercalcemia secondary to elevated parathormone levels. After surgical resection, the serum calcium level tends to normalize.

  • Rhabdoid tumor has the worst prognosis of all renal tumors. It is highly aggressive and metastasizes early, with most patients presenting with advanced disease.
  • Eighty percent develop metastases, most commonly to the lungs and less often to the liver, abdomen, brain, lymph nodes, or skeleton.
  • Survival is poor, with an 18-month survival rate of only 20%.

[edit] Imaging Findings for Rhabdoid tumor

  • Imaging demonstrates a large, centrally located, heterogeneous soft-tissue mass involving the renal hilum with indistinct margins.
  • Although the appearance may closely resemble that of Wilm's tumor, several features can suggest the diagnosis:
    • Subcapsular fluid collections
    • Tumor lobules separated by dark areas of necrosis or hemorrhage
    • Linear calcifications outlining tumor lobules.
  • Vascular and local invasion is common.

[edit] Images

Patient #1

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[edit] External Links

[edit] References for Rhabdoid tumor