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Rhabdoid Tumor NOS (Extrarenal)

Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: April 21, 2025 · Captured 2026-09-09.

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Context: Treatment of Tumors of Uncertain Differentiation

Malignant rhabdoid tumors were first described in children with renal tumors in 1981. These tumors were later found in a variety of extrarenal sites. These tumors are uncommon and highly malignant, especially in children younger than 2 years. For more information, see the Rhabdoid Tumors of the Kidney section in Wilms Tumor and Other Childhood Kidney Tumors Treatment.

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Rhabdoid Tumors of the Kidney

Extrarenal (extracranial) rhabdoid tumors account for 2% of soft tissue sarcomas in patients younger than 20 years (see Table 1).

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Table 1

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Genetic and genomic alterations →Genetic testing and surveillance →Prognosis and clinical presentation →Treatment of extrarenal (extracranial) rhabdoid tumor →Treatment options under clinical evaluation →

Preserved source evidence · Not medical advice