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Updated: Jun 25, 2026

The Use of Reverse Phase Protein Arrays (RPPA) to Explore Protein Expression Variation within Individual Renal Cell Cancers
12:22

The Use of Reverse Phase Protein Arrays (RPPA) to Explore Protein Expression Variation within Individual Renal Cell Cancers

Published on: January 22, 2013

Altered expression of key cell cycle regulators in renal cell carcinoma associated with Xp11.2 translocation

H Barroca1, S Castedo, J Vieira

  • 1Laboratório de Anatomia Patológica, Al. Hernani Monteiro 4200-451, Hospital de S. João, Porto, Portugal. hbarroca@gmail.com

Pathology, Research and Practice
|February 28, 2009
PubMed

Insights

Pediatric TFE3-positive renal cell carcinoma (RCC) shows cell cycle deregulation, unlike typical childhood RCC. This finding highlights distinct molecular pathways in rare pediatric kidney cancers.

Area of Science:

  • Oncology
  • Pediatric Pathology
  • Molecular Biology

Background:

  • Renal cell carcinoma (RCC) is uncommon in children.
  • A distinct pediatric subtype of RCC is associated with Xp11.2 translocations or TFE3 gene fusions.
  • This TFE3-positive RCC subtype is hypothesized to involve cyclin D1, cyclin D3, and p21 accumulation.

Observation:

  • This study investigated two pediatric RCC cases: one clear cell-type and one TFE3-positive.
  • Immunohistochemical analysis included markers for cell proliferation (Ki67), cell cycle regulators (p16, p21, p27), tumor suppressors (p53, mdm2), and specific markers (TFE3, CD10, vimentin, E-cadherin, RCC-antigen).

Findings:

  • TFE3-positive RCC displayed intense p21, cyclin D1, and cyclin D3 immunoreactivity.
  • The TFE3-positive tumor lacked expression of p53, p16, p27(kip1), and mdm2.
  • The clear cell RCC showed an immunoexpression profile similar to adult clear cell RCC.

Implications:

  • The cell cycle deregulation observed in TFE3-positive RCC appears independent of patient age.
  • These findings suggest unique molecular mechanisms driving TFE3-positive pediatric kidney cancer.
  • Distinguishing TFE3-positive RCC from other pediatric renal tumors is crucial for understanding pathogenesis and guiding treatment.

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