Hereditary papillary renal cell carcinoma

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Hereditary papillary renal cell carcinoma

ORPHA:47044Disease

Also called HPRC

What it is

A rare familial renal cancer syndrome characterized by a predisposition for developing bilateral and multifocal classic type papillary renal cell carcinomas (formerly known as type 1 papillary renal cell carcinoma until the 2022 WHO classification of renal tumors).

Key facts

Age of onset
Adult
Inheritance
Autosomal dominant
Classified as
Disease

Gene

METDisease-causing germline mutation(s)

ICD-10 codes

C64filed under a broader ICD-10 category — shared with 18 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MEDDRA 10067943MESH C538614MONDO 0003789OMIM 605074UMLS C0879257

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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