Papillary renal cell carcinoma
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.
Overview
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.
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.
Anatomy and lymph node drainage
Renal cell carcinoma comes from the kidney's filtering cortex, urothelial cancer from the lining of the collecting system and bladder, and the adrenal on top hosts cortical and medullary (neuroblastoma) tumours.
- Renal cortex (RCC)MET-altered papillary renal cell carcinoma (former type 1) · Hereditary papillary renal carcinoma (germline MET) · Papillary renal neoplasm with reverse polarity (indolent)
- Renal pelvis and ureter (upper tract urothelial)
- Bladder lining (non-muscle-invasive)MET-altered papillary renal cell carcinoma (former type 1) · Hereditary papillary renal carcinoma (germline MET) · Papillary renal neoplasm with reverse polarity (indolent)
- Bladder muscle wall (muscle-invasive)
- Adrenal cortex
- Adrenal medulla and sympathetic chain (neuroblastoma)
- Developing kidney (Wilms tumour)
- renal hilar
- para-aortic and paracaval
- obturator and iliac (bladder)
Same organ: Non-muscle-invasive bladder cancer, Muscle-invasive and advanced bladder cancer, Bladder & urothelial cancer, Clear cell renal cell carcinoma, Chromophobe renal cell carcinoma, Renal cell carcinoma, Wilms tumour (nephroblastoma), Neuroblastoma (paediatric), Adrenocortical carcinoma, Pheochromocytoma and paraganglioma (PPGL), Urethral cancer, Penile cancer
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.
- AI in radiologyEstablished
- cfDNA fragmentomicsEstablished
- Colorectal cancer screening (colonoscopy, FIT, stool DNA, blood)Standard of care
- DNA methylation profilingEstablished
- HCC surveillance in cirrhosis (ultrasound + AFP)Standard of care
- High-risk pancreatic surveillance (CAPS / PRECEDE)Established
- A 28-day national pathway for people with a positive multi-cancer blood test
- A breath test to rule out cancer in people with vague symptoms
- A cancer blood test for older people arriving at A&E with unexplained symptoms
- A legislated, publicly reported 28-day standard from urgent referral to diagnosis
- A live national dashboard of stage at diagnosis as the scorecard for early detection
- A single 'cancer check at 60' appointment bundling all screening tests
Background: Alpha-fetoprotein (AFP), Barrett's oesophagus, CA 19-9, Early detection, Faecal immunochemical test (FIT). Also on OnCo: Symptoms and red flags · Early detection roadmap.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
Partial or radical nephrectomy, ablation or surveillance as for other kidney cancers; adjuvant therapy evidence is thin.
Cabozantinib first line (PAPMET); savolitinib for MET-driven tumours; immunotherapy combinations on single-arm data; clinical trials preferred.
Early surgery for FH-deficient tumours because they spread early; surveillance in MET carriers; genetic counselling of relatives.
Subtypes & biomarkers
top- 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)
- 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
How often this target appears
- 1997Delahunt and Eble separate type 1 and type 2 papillary carcinoma
- 2002Fumarate hydratase mutations found in HLRCC
- 2021PAPMET: cabozantinib beats sunitinib
- 2022WHO drops type 1 and 2 in favour of molecular groups
What is in development for Papillary renal cell carcinoma, drawn from the whole corpus: 0 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Nothing recorded in development for this cancer yet.
Open problems and what is being done
Small trials; most evidence is extrapolated from clear cell disease.
and how the field plans to fix it →What is being done about thisRare cancers and small trialsAvailable now- AI trial matching & clinical decision supportEstablished
- Comprehensive genomic profilingStandard of care
- Dinutuximab (ch14.18) / dinutuximab betaApproved
- Eflornithine (DFMO)Approved
- LarotrectinibApproved
- Limb-salvage surgery and endoprosthetic reconstructionStandard of care
In trials- ACTIONRecruiting
- COG AALL1731Positive
- COG ANBL0032Positive
- DeFiPositive
- Euro Ewing 2012Positive
- Functional (ex vivo) drug testingEmerging
Ideas and roadmaps- A digital second-opinion network answering community oncologists within 72 hours
- A DRUP-style protocol for off-label generic targeted drugs in rare tumours
- A funded expert second opinion for every new high-stakes or rare cancer diagnosis
- A global first-in-human network for academic cancer trials with single ethics review
- A global open trials operating system any hospital can plug into
- A live 'seats available' feed for trial slots, like airline inventory
Background: Basket, umbrella, and platform trials, Centralisation and high-volume centres, FNCLCC grade (soft-tissue sarcoma), Histotype-tailored therapy, INRG staging and risk groups. Also on OnCo: Find a trial · Expert centres.
No approved therapy specific to FH-deficient cancer.
and how the field plans to fix it →What is being done about thisCancers without a drug targetAvailable now- AdagrasibApproved
- CRISPR functional genomicsEstablished
- DaraxonrasibApproved
- KRAS & RAS inhibitorsApproved
- PROTACs & molecular glues (targeted protein degradation)Approved
- RevumenibApproved
In trials- AI-driven drug & target discoveryPhase 2
- CodeBreaK 200Positive
- ELI-002 7PPhase 2
- ElironrasibPhase 2
- KRYSTAL-12Positive
- MRTX1133Phase 1
Ideas and roadmaps- A drug screen that only rewards killing sleeping cancer cells
- A guaranteed purchase prize for the first drug against a named hard target
- A precompetitive consortium for the twenty hardest cancer targets
- A synthetic lethality map for every cancer driver in every tissue context
- A target de-risking index that counts failures as well as successes
- AI-designed proteins that grip the floppy parts of cancer drivers
Background: p53 / RB / cell-cycle checkpoint, RAS / RAF / MEK / ERK (MAPK). Also on OnCo: Targets · KRAS roadmap.
Which patients benefit from immunotherapy.
Trials
topTrials recruiting now
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
Expert centres
topExpert centres
- Johns Hopkins Hospital / Sidney Kimmel Comprehensive Cancer CenterBaltimore, USNewsweek oncology #10NCI comprehensivevia Pembrolizumab
- Alliance for Clinical Trials in OncologyChicago, IL, USvia Pembrolizumab
- GOG FoundationPhiladelphia, PA, USvia Pembrolizumab
- Institut Jules BordetBrussels, BEvia Pembrolizumab
- Istituto di Candiolo IRCCS (FPO)Candiolo, ITvia MET
- via Pembrolizumab
- Shanghai Chest HospitalShanghai, CNvia MET
- Zhongshan Hospital, Fudan UniversityShanghai, CNvia Thermal ablation (RFA, microwave, cryo)
Questions to ask
topQuestions to ask your oncologist about Papillary renal cell carcinoma
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example MET mutation, amplification or chromosome 7 gain, Fumarate hydratase losswith germline FH testing, CDKN2A loss, Germline MET testing in young or multifocal disease), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include MET-altered papillary renal cell carcinoma, Hereditary papillary renal carcinoma, Fumarate hydratase-deficient renal cell carcinoma.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Localised
- For my situation (localised), which of the standard options do you recommend and why?Why: Guideline options include: Partial or radical nephrectomy, ablation or surveillance as for other kidney cancers; adjuvant therapy evidence is thin.
Metastatic
- For my situation (metastatic), which of the standard options do you recommend and why?Why: Guideline options include: Cabozantinib first line (PAPMET); savolitinib for MET-driven tumours; immunotherapy combinations on single-arm data; clinical trials preferred.
- Am I a candidate for Cabozantinib, Savolitinib, Sunitinib or related drugs, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Hereditary syndromes
- For my situation (hereditary syndromes), which of the standard options do you recommend and why?Why: Guideline options include: Early surgery for FH-deficient tumours because they spread early; surveillance in MET carriers; genetic counselling of relatives.
Any stage
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “Small trials; most evidence is extrapolated from clear cell disease”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “No approved therapy specific to FH-deficient cancer”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
5targets
6drugs
4companies
6Latest papers
topQuery for this cancer: (TITLE:"Papillary renal cell carcinoma" OR ABSTRACT:"Papillary renal cell carcinoma" OR TITLE:"pRCC" OR ABSTRACT:"pRCC" OR TITLE:"Papillary RCC" OR ABSTRACT:"Papillary RCC") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Papillary renal cell carcinoma, not a curated reading list.
Similar pages
not linked directly; found by shared links- CancerChromophobe renal cell carcinoma
Shares Sunitinib, Thermal ablation (RFA, microwave, cryo), Active surveillance, Cabozantinib and the tag subtype-page.
- CancerClear cell renal cell carcinoma
Shares Sunitinib, Thermal ablation (RFA, microwave, cryo), Active surveillance, Cabozantinib and the tag subtype-page.
- CancerPeritoneal mesothelioma
Shares Active surveillance, Pembrolizumab and the tag subtype-page.
- CancerNon-seminomatous germ cell tumour
Shares Active surveillance and the tag subtype-page.
- CancerSeminoma
Shares Active surveillance and the tag subtype-page.
- CancerMedullary thyroid cancer
Shares Cabozantinib and the tag subtype-page.
- CancerClear cell ovarian cancer
Shares Pembrolizumab and the tag subtype-page.
- CancerPapillary thyroid cancer
Shares Active surveillance and the tag subtype-page.