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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
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.
Abstract:
Current World Health Organization (WHO) Classification of Urinary and Male Genital Tumours (2022) has introduced minor updates in the classification of renal cell carcinomas (RCC). Among the most notable changes is the recognition of a new category: molecularly defined RCC. This group comprises several distinct tumour entities characterized by specific genetic alterations. The inclusion of this category reflects the growing importance of molecular testing in the diagnosis, prognosis and treatment planning of renal malignancies. This review summarizes the basic information, clinical data, and treatment options of these rare RCCs. Due to the low prevalence of molecularly defined RCCs, the clinical management remains challenging, and standardized, evidence-based treatment guidelines are still lacking, particularly for the systemic therapy of advanced RCCs. As part of the review, a clinical case of a female patient with metastatic TFE3-translocated RCC is also presented, illustrating the diagnostic and therapeutic challenges.
Insights
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.
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.
