# 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: KIRP; TCGA-KIRP; kidney renal papillary cell carcinoma (TCGA KIRP cohort); 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 (shorter survival); Germline MET testing in young or multifocal disease

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/papillary-rcc/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/papillary-rcc/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/papillary-rcc/#what-it-is [4 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/papillary-rcc/#finding-it [4 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/papillary-rcc/#treating-it [3 settings, 2 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/papillary-rcc/#evidence [4 trials, 4 key papers, 4 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/papillary-rcc/#science [16 targets]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/papillary-rcc/where-you-are/
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/papillary-rcc/#living-with-it [13 questions, 6 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/papillary-rcc/coming/ [4 medicines, 4 trials, 3 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/papillary-rcc/data/ [41 connected records]

## 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

- cancers: [Chromophobe renal cell carcinoma](https://onco.cc/cancers/chromophobe-rcc/), [Clear cell papillary renal cell tumour](https://onco.cc/cancers/clear-cell-papillary-renal-cell-tumour/), [Clear cell renal cell carcinoma](https://onco.cc/cancers/clear-cell-rcc/), [Collecting duct carcinoma of the kidney](https://onco.cc/cancers/collecting-duct-carcinoma/), [Fumarate hydratase-deficient renal cell carcinoma (HLRCC-associated)](https://onco.cc/cancers/fh-deficient-renal-cell-carcinoma/), [Mucinous tubular and spindle cell carcinoma of the kidney](https://onco.cc/cancers/mucinous-tubular-spindle-cell-carcinoma/), [Renal cell carcinoma](https://onco.cc/cancers/rcc/), [Renal medullary carcinoma (SMARCB1-deficient)](https://onco.cc/cancers/renal-medullary-carcinoma/), [TFE3-rearranged (translocation) renal cell carcinoma](https://onco.cc/cancers/tfe3-rearranged-renal-cell-carcinoma/)
- trials: [PAPMET (SWOG S1500)](https://onco.cc/trials/papmet/), [Savolitinib Plus Durvalumab Versus Sunitinib and Durvalumab Monotherapy in MET-Driven, Unresectable and Locally Advanced or Metastatic PRCC](https://onco.cc/trials/nct05043090/), [Savolitinib vs. Sunitinib in MET-driven PRCC.](https://onco.cc/trials/nct03091192/), [Study of Orellanine in Metastatic Clear-Cell or Papillary Renal Cell Carcinoma](https://onco.cc/trials/nct05287945/)
- key papers: [ASPEN: everolimus versus sunitinib in metastatic non-clear cell renal cell carcinoma](https://onco.cc/key-papers/paper-aspen-armstrong-lancet-oncol-2016/), [Comprehensive molecular characterisation of papillary renal cell carcinoma (The Cancer Genome Atlas)](https://onco.cc/key-papers/paper-tcga-papillary-rcc-nejm-2016/), [PAPMET (SWOG 1500): cabozantinib versus sunitinib for metastatic papillary renal cell carcinoma](https://onco.cc/key-papers/paper-papmet-pal-lancet-2021/), [WHO classification of tumours of the urinary system and male genital organs, 2022: renal, penile and testicular tumours](https://onco.cc/key-papers/paper-who-2022-gu-moch-eur-urol-2022/)
- 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: [CHD2](https://onco.cc/targets/chd2/), [CUL3](https://onco.cc/targets/cul3/), [FH](https://onco.cc/targets/fh/), [FLCN](https://onco.cc/targets/flcn/), [HNF1A](https://onco.cc/targets/hnf1a/), [MET](https://onco.cc/targets/met/), [NUP214](https://onco.cc/targets/nup214/), [PCBP1](https://onco.cc/targets/pcbp1/), [PRDM2](https://onco.cc/targets/prdm2/), [SGK1](https://onco.cc/targets/sgk1/), [ZHX2](https://onco.cc/targets/zhx2/)
- terms: [CDKN2A/B homozygous deletion](https://onco.cc/terms/cdkn2a-homozygous-deletion/), [MET exon 14 skipping mutation](https://onco.cc/terms/met-exon-14-skipping/)

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JSON: https://onco.cc/api/v1/entities/papillary-rcc.json