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Recommendations for the Management of Rare Kidney Cancers
Rachel H Giles1, Toni K Choueiri2, Daniel Y Heng3
1International Kidney Cancer Coalition, Duivendrecht, The Netherlands; Department Of Nephrology and Hypertension, University Medical Center Utrecht, Regenerative Medicine Center Utrecht, Uppsalalaan, Utrecht, The Netherlands.
Context:
The European Association of Urology Renal Cell Carcinoma Guideline Panel recently conducted a systematic review of treatment options for patients with advanced non-clear-cell renal cell carcinomas (RCCs), which showed a substantial lack of evidence for management recommendations.
Objective:
To improve the outcomes of patients with rare kidney cancers (RKCs), we performed a subsequent unstructured review to determine current treatment strategies and druggable pathways, involving key stakeholders with a global perspective to generate recommendations.
Evidence Acquisition:
Based on the systematic review, literature was queried in Pubmed, Medline, and abstracts from proceedings of European Society for Medical Oncology and American Society of Clinical Oncology, in addition to consulting key opinion leaders and stakeholders. A conventional narrative review strategy was adopted to summarize the data.
Evidence Synthesis:
The systematic review showed an absence of evidence for treating RKCs, with data only supporting sunitinib or MET inhibitors for some specific subtypes. However, a growing body of evidence implicates druggable pathways in specific RKC subtypes. To test hypotheses, the small patient numbers in each subtype require coordinated multicenter efforts. Many RKC patients are currently excluded from studies or are not analyzed using subtype-specific parameters, despite their unmet medical need.
Conclusions:
We recognize the need for additional multicenter studies and subtype-specific analyses; however, we present management recommendations based on the data available. Web-based tools facilitating subtype-specific global registries and shared translational research resources will help generate sufficient data to formulate evidence-based recommendations for guidelines.
Patient Summary:
Patients confronted with rare kidney cancers are often treated the same way as clear-cell renal cell carcinoma patients, despite little evidence from randomized trials. Molecular characterization of tumors to stratify patients may improve outcomes. Availability of potential agents and trials remain a problem. Collaboration among medical centers is important to pool scarce data.
Insights
Evidence for treating rare kidney cancers (RKCs) is limited. Further multicenter studies and subtype-specific analyses are needed to develop evidence-based management recommendations for these uncommon renal cell carcinomas.
Area of Science:
- Oncology
- Urology
- Genetics
Background:
- Advanced non-clear-cell renal cell carcinomas (RCCs) lack sufficient evidence for treatment recommendations.
- Rare kidney cancers (RKCs) often receive standard clear-cell RCC treatment despite limited supporting data.
- There is a critical unmet medical need for RKC patients due to exclusion from studies and lack of subtype-specific analysis.
Purpose of the Study:
- To review current treatment strategies and identify druggable pathways for rare kidney cancers (RKCs).
- To generate management recommendations for RKCs involving global stakeholders.
- To address the lack of evidence in treating advanced non-clear-cell renal cell carcinomas.
Main Methods:
- Systematic review of treatment options for advanced non-clear-cell renal cell carcinomas (RCCs).
- Unstructured review of current treatment strategies and druggable pathways for rare kidney cancers (RKCs).
- Literature search of PubMed, Medline, and oncology conference abstracts; consultation with key opinion leaders and stakeholders.
Main Results:
- A significant lack of evidence for treating RKCs was identified.
- Sunitinib or MET inhibitors show promise for specific RKC subtypes.
- Druggable pathways are implicated in RKC subtypes, necessitating coordinated multicenter efforts due to small patient numbers.
Conclusions:
- Management recommendations are presented based on available data, acknowledging the need for more research.
- Multicenter studies and subtype-specific analyses are crucial for advancing RKC treatment.
- Web-based tools for global registries and translational research can facilitate evidence-based guideline development for rare kidney cancers.
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