# Papillary renal cell carcinoma

Source: https://onco.cc/cancers/papillary-rcc/  
OnCo record `papillary-rcc` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Papillary kidney cancer is the second commonest type and does not share the VHL biology of clear cell cancer, so the drugs work differently: the MET-targeting drug cabozantinib beat sunitinib in the first trial run just for this disease, and two hereditary syndromes account for some cases.

## Summary

Papillary renal cell carcinoma is a heterogeneous group defined by papillary architecture; the older split into type 1 and type 2 has given way to molecular groups in the WHO 2022 classification. MET alterations (mutation, amplification, chromosome 7 gain) are frequent, especially in the former type 1, and are inherited in hereditary papillary renal carcinoma. Fumarate hydratase-deficient renal cancer, from the hereditary leiomyomatosis and renal cell cancer syndrome, is an aggressive form once labelled type 2. Localised tumours are treated like other kidney cancers with surgery or ablation. For metastatic disease the PAPMET trial showed cabozantinib gave longer progression-free survival and more responses than sunitinib, making it the preferred first-line option; savolitinib is active in MET-driven tumours (SAVOIR), and immunotherapy combinations have shown activity in single-arm studies.

## Fields

- Kind: Cancer
- Last checked: 2026-09-17
- Also known as: pRCC; Papillary RCC
- Tags: subtype-page
- Group: genitourinary
- Burden: Ten to fifteen percent of kidney cancers, commoner in men, in Black patients and in end-stage kidney disease; localised tumours do well after surgery, while metastatic disease has fared worse than clear cell cancer on the same drugs.
- Subtypes: MET-altered papillary renal cell carcinoma (former type 1); Hereditary papillary renal carcinoma (germline MET); Fumarate hydratase-deficient renal cell carcinoma (HLRCC syndrome); Papillary renal neoplasm with reverse polarity (indolent)
- Biomarkers: MET mutation, amplification or chromosome 7 gain; Fumarate hydratase loss (2SC immunohistochemistry) with germline FH testing; CDKN2A loss (poor outlook); Germline MET testing in young or multifocal disease

## Standard of care

- Localised: Partial or radical nephrectomy, ablation or surveillance as for other kidney cancers; adjuvant therapy evidence is thin. ([Thermal ablation (RFA, microwave, cryo)](https://onco.cc/technologies/thermal-ablation/), [Active surveillance](https://onco.cc/technologies/active-surveillance/))
- Metastatic: Cabozantinib first line (PAPMET); savolitinib for MET-driven tumours; immunotherapy combinations on single-arm data; clinical trials preferred. ([Cabozantinib](https://onco.cc/drugs/cabozantinib/), [Savolitinib](https://onco.cc/drugs/savolitinib/), [MET](https://onco.cc/targets/met/), [Sunitinib](https://onco.cc/drugs/sunitinib/), [Pembrolizumab](https://onco.cc/drugs/pembrolizumab/))
- Hereditary syndromes: Early surgery for FH-deficient tumours because they spread early; surveillance in MET carriers; genetic counselling of relatives. ([MET](https://onco.cc/targets/met/))

## State of the art

- PAPMET was the first randomised trial in papillary kidney cancer and ended the practice of borrowing clear cell regimens unchanged.
- The molecular reclassification is replacing the type 1 and type 2 labels with actionable groups.
- FH-deficient cancer is now recognised as a distinct aggressive entity needing early treatment.

## Open problems

- Small trials; most evidence is extrapolated from clear cell disease.
- No approved therapy specific to FH-deficient cancer.
- Which patients benefit from immunotherapy.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Papillary_renal_cell_carcinomas
- Wikipedia: https://en.wikipedia.org/wiki/Papillary_renal_cell_carcinomas

## Connected records

- drugs: [Cabozantinib](https://onco.cc/drugs/cabozantinib/), [Pembrolizumab](https://onco.cc/drugs/pembrolizumab/), [Savolitinib](https://onco.cc/drugs/savolitinib/), [Sunitinib](https://onco.cc/drugs/sunitinib/)
- technologies: [Active surveillance](https://onco.cc/technologies/active-surveillance/), [Thermal ablation (RFA, microwave, cryo)](https://onco.cc/technologies/thermal-ablation/)
- targets: [MET](https://onco.cc/targets/met/)
- cancers: [Renal cell carcinoma](https://onco.cc/cancers/rcc/)

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