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

Rare Disease

hereditary papillary renal cell carcinoma

MONDO:0003789

Also known as:familial renal papillary carcinomahereditary kidney papillary carcinomahereditary papillary carcinoma of kidney

Overview

A familial carcinoma inherited in an autosomal dominant trait. It is characterized by the development of multiple, bilateral papillary renal cell carcinomas. The carcinomas range from microscopic lesions to clinically symptomatic tumors. It is associated with activating mutations of the MET oncogene.

GeneticsMultifactorial, Autosomal dominant · 2 genes (MET, PRCC)

Clinical Presentation

Features include: Papillary renal cell carcinoma.

Organ System	Phenotype Count	Example Features
Kidneys and urinary system	1	Papillary renal cell carcinoma

Explore clinical features

Genetic Landscape

Assembled from HGNC gene records, ClinGen gene-disease validity evidence, and ClinVar pathogenic variant data. Validity classifications reflect the strength of published evidence linking each gene to this disease.

MET

MET encodes MET proto-oncogene, receptor tyrosine kinase (1,390 aa). Receptor tyrosine kinase that transduces signals from the extracellular matrix into the cytoplasm by binding to hepatocyte growth factor/HGF ligand. Highest expression in Nerve Tibial (21.3 TPM) and Cells Cultured fibroblasts (18.5 TPM).

Hereditary papillary renal cell carcinoma is associated with mutations in the MET gene on chromosome 7.

The MET protein participates in GNAT1 (Met removed) and Signaling by MET pathways.

MET is classified as a druggable target (Cell Surface, Clinically Actionable, Drug Resistance, Druggable Genome, Kinase, and Tyrosine Kinase categories) with score 3.5.

PRCC

PRCC function has not been fully characterized.

Hereditary papillary renal cell carcinoma is associated with mutations in the PRCC gene on chromosome 1.

Diagnosis

Drawn from GeneReviews diagnostic guidelines, newborn screening panel status (RUSP), and ClinGen gene-disease validity classifications. Reflects current clinical evidence and consensus where available.

Genetic testing for MET, PRCC is available. Testing is considered confirmatory for diagnosis.

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

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 papillary renal cell carcinoma.

82 publications have been identified in PubMed for hereditary papillary renal cell carcinoma. Research spans Basic Science / Preclinical (35%), Review / Meta-Analysis (21%), and Case Report / Case Series (18%).

Research Type	Count	% of Total
Laboratory research	29	35%

Data assembled from 6 of 12 sources · Last updated Sep 19, 2026, 4:33 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

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Financial & Access Support (External Programs)

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Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:605074

Orphanet

European rare disease database

Orphanet:47044

GARD

Genetic and Rare Diseases Info Center

GARD:13157

Questions for Your Care Team

→Should I pursue genetic testing for the associated genes, and what would the results mean for my family?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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hereditary papillary renal cell carcinoma

Overview

Clinical Presentation

Explore clinical features

Genetic Landscape

MET

PRCC

Diagnosis

Research Landscape

Patient Support

Financial & Access Support (External Programs)

Are you a patient organization for this condition?

Medical References

External Resources

OMIM

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Recent Publications