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Article Synopsis
  • This study investigates the characteristics of 10 rare primary renal perivascular epithelioid cell tumors (PEComas) that have a specific genetic alteration (Xp11.2 translocation), focusing on their presentation and outcomes.
  • The patient group included both genders aged 14-65, with positive outcomes for most after surgical treatment, and showed consistent expression of certain markers (TFE3 being the most significant) while lacking others typically seen in similar tumors.
  • The findings reveal the genetic variety among these tumors, with unique TFE3 fusion partners identified, and emphasize the importance of TFE3 immunohistochemistry for diagnosis and differentiation from other kidney cancers.
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