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The Retinoblastoma Gene01:20

The Retinoblastoma Gene

Tumor suppressor genes are normal genes that can slow down cell division, repair DNA mistakes, or program the cells for apoptosis in case of irreparable damage. Hence, they play an essential role in preventing the proliferation of damaged cells.
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...

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Updated: Jul 15, 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

Molecularly defined renal cell carcinomas.

Petr Stránský1, Jiří Kolář1, Dominika Šiková1

  • 1Department of Urology, Faculty of Medicine in Pilsen, Charles University, University Hospital Pilsen, Pilsen, Czech Republic.

Reports of Practical Oncology and Radiotherapy : Journal of Greatpoland Cancer Center in Poznan and Polish Society of Radiation Oncology
|July 14, 2026
PubMed
Summary

The 2022 WHO classification introduces molecularly defined renal cell carcinomas (RCC). This review covers their diagnosis, treatment, and challenges, highlighting the need for better guidelines for these rare tumors.

Keywords:
ALK-rearranged renal carcinomaELOC-altered renal carcinomaSMARCB1-deficient renal medullary carcinomaTFE3-rearranged renal carcinomaTFEB-altered renal carcinomafumarate hydratase-deficient renal carcinomamolecularly defined renal carcinomassuccinate dehydrogenase-deficient renal carcinoma

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Published on: February 8, 2020

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Last Updated: Jul 15, 2026

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Published on: February 8, 2020

Area of Science:

  • Oncology
  • Genetics
  • Pathology

Background:

  • The World Health Organization (WHO) Classification of Urinary and Male Genital Tumours (2022) updates renal cell carcinoma (RCC) categorization.
  • A new category, molecularly defined RCC, recognizes distinct tumor entities based on specific genetic alterations.

Purpose of the Study:

  • To review the basic information, clinical data, and treatment options for molecularly defined RCCs.
  • To highlight the diagnostic and therapeutic challenges associated with these rare renal malignancies.

Main Methods:

  • Literature review summarizing current knowledge on molecularly defined RCCs.
  • Inclusion of a clinical case study of metastatic *TFE3*-translocated RCC.

Main Results:

  • Molecularly defined RCCs represent a new classification reflecting the importance of molecular testing.
  • Low prevalence of these tumors complicates clinical management and treatment guideline development.
  • Systemic therapy for advanced molecularly defined RCCs remains a significant challenge.

Conclusions:

  • The recognition of molecularly defined RCCs signifies a shift towards precision oncology in renal cancer.
  • Standardized, evidence-based treatment guidelines are urgently needed, especially for advanced stages.
  • Further research is essential to overcome clinical management challenges for these rare entities.