# Hereditary papillary renal cell carcinoma

Source: https://onco.cc/terms/hereditary-papillary-renal-cell-carcinoma/  
OnCo record `hereditary-papillary-renal-cell-carcinoma` (Term). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Hereditary papillary renal cell carcinoma is a rare inherited condition, caused by an activating change in the MET gene, that carries a very high lifetime risk of papillary kidney cancer. It is managed by kidney specialists with experience of inherited kidney cancer.

## Summary

Autosomal dominant, caused by activating germline variants in the MET proto-oncogene (the hepatocyte growth factor receptor). The NCI PDQ summary of kidney cancer genetics lists papillary renal cell carcinoma (formerly type 1) as the tumour, a cumulative cancer risk approaching 100%, and no associated non-renal tumours. Clinical genetic tests for MET are available in CLIA-certified laboratories, and genetic counselling is a prerequisite.

No management guideline specific to this syndrome was read in this round: the PDQ summary gives no surveillance schedule, so none is recorded.

## Fields

- Kind: Term
- Last checked: 2026-11-04
- Also known as: HPRC; HPRCC; hereditary papillary renal carcinoma
- Tags: gap-fill; hereditary

## Notes

- Orphanet ORPHA:47044. In the NHS directory it is tested under R224, inherited renal cancer.
- Open gap: a primary source for surveillance and nephron-sparing management in HPRC.

## Sources

- NCI PDQ: genetics of kidney cancer (health professional version): https://www.cancer.gov/types/kidney/hp/kidney-genetics-pdq
- Orphanet ORPHA:47044: https://www.orpha.net/en/disease/detail/47044

## Connected records

- terms: [Birt-Hogg-Dubé syndrome](https://onco.cc/terms/birt-hogg-dube-syndrome/), [Hereditary cancer syndromes](https://onco.cc/terms/hereditary-cancer-syndromes/), [Von Hippel-Lindau disease](https://onco.cc/terms/vhl-disease/)
- cancers: [Papillary renal cell carcinoma](https://onco.cc/cancers/papillary-rcc/)
- technologies: [Germline (hereditary) testing](https://onco.cc/technologies/germline-testing/)
- targets: [MET](https://onco.cc/targets/met/)

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