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Mit family translocation renal cell carcinoma


Definition

  • Subtype of renal cell carcinoma harboring gene fusions involving two members of MiT family transcription factors, TFE3 (with Xp11 translocation), and TFEB (with t(6;11) translocation)

Clinical features

Epidemiology

  • The majority of cases are reported in children and young adults.

    • 40% of pediatric RCCs are Xp11 translocation RCCs

  • The incidence is rare in adults, but it is rising because of increased recognition.

    • 2%–4% of adult RCCs are Xp11 translocation RCCs

  • t(6;11) translocation RCCs much less common than Xp11 translocation RCCs

  • Some patients have a history of chemotherapy for other malignancies.

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