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%.
 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.
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 External Links
 References for Rhabdoid tumor
- Lisa H. Lowe, Bernardo H. Isuani, Richard M. Heller, Sharon M. Stein, Joyce E. Johnson, Oscar M. Navarro, and Marta Hernanz-Schulman. Pediatric Renal Masses: Wilms Tumor and Beyond. RadioGraphics 2000 20: 1585-1603.