hereditary papillary renal cell carcinoma
Findings
No curated finding names hereditary papillary renal cell carcinoma yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
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.
Definition from the Mondo Disease Ontology (MONDO:0003789), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal dominant inheritance · Typified by somatic mosaicism · Typified by incomplete penetrance
HPO, annotations 2026-09-02
Features
1 feature
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Papillary renal cell carcinomaHPOHP:0006766
Genes
1 gene
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- METHGNC:7029
- Definitive · Ambry Genetics · Autosomal dominant · 2023
- Definitive · G2P · Autosomal dominant · 2022
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2023
Where it sits
Other names
8 names
Resolves to: hereditary papillary renal cell carcinoma
- Also called
- familial renal papillary carcinomahereditary kidney papillary carcinomahereditary papillary carcinoma of kidneyhereditary papillary carcinoma of the kidneyhereditary papillary renal carcinomahereditary papillary renal cell cancerrenal cell carcinoma, papillaryrenal cell carcinoma, papillary, 1, familial and somatic