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hereditary clear cell renal cell carcinoma - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

hereditary clear cell renal cell carcinoma

MONDO:0018492

Also known as:Hereditary clear cell renal cell cancerhereditary clear cell renal carcinomahereditary clear cell renal cell adenocarcinoma

Overview

A manifestation of von Hippel-Lindau disease or other familial renal cell cancer syndromes that present as a malignant epithelial neoplasm of the kidney. It is characterized by the presence of lipid-containing clear cells within a vascular network. The tumor usually is bilateral and polycentric, and metastasizes to unusual sites. Late metastasis is common.

Diagnosis

Biomarker and diagnostic research for hereditary clear cell renal cell carcinoma has been reported in the published literature.

Prognosis

Estimated prevalence: Unknown (Unknown prevalence).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for hereditary clear cell renal cell carcinoma.

172 publications have been identified in PubMed for hereditary clear cell renal cell carcinoma. Kisho has analyzed 125 by research type. Research spans Review / Meta-Analysis (38%), Case Report / Case Series (24%), and Epidemiology / Natural History (10%).

Research Type	Count	% of Total
Research summaries	48	38%

Data assembled from 3 of 12 sources · Last updated Sep 19, 2026, 6:25 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:422526

GARD

Genetic and Rare Diseases Info Center

GARD:9571

Questions for Your Care Team

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Kisho

hereditary clear cell renal cell carcinoma

Overview

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Recent Publications

Latest News & Developments

Tumor-to-tumor metastasis: a rare case of clear cell renal cell carcinoma metastasizing into lung adenocarcinoma.

Tumor-informed ctDNA surveillance after radical nephrectomy and level IV IVC thrombectomy for clear cell renal cell carcinoma: Early detection of molecular residual disease.

hereditary clear cell renal cell carcinoma

Overview

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Recent Publications

Latest News & Developments

Tumor-to-tumor metastasis: a rare case of clear cell renal cell carcinoma metastasizing into lung adenocarcinoma.

Tumor-informed ctDNA surveillance after radical nephrectomy and level IV IVC thrombectomy for clear cell renal cell carcinoma: Early detection of molecular residual disease.