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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.
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.
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.
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.
Same organ: Collecting duct carcinoma of the kidney, Renal medullary carcinoma (SMARCB1-deficient), TFE3-rearranged (translocation) renal cell carcinoma, Fumarate hydratase-deficient renal cell carcinoma (HLRCC-associated), Succinate dehydrogenase-deficient renal cell carcinoma, Mucinous tubular and spindle cell carcinoma of the kidney, Eosinophilic solid and cystic renal cell carcinoma, Clear cell papillary renal cell tumour, Urothelial carcinoma of the urethra, Squamous cell carcinoma of the urethra, Adenocarcinoma of the urethra (including clear cell adenocarcinoma), Melanoma of the urethra, 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), Low-risk neuroblastoma (INRG very low and low risk, including stage MS), Intermediate-risk neuroblastoma, High-risk neuroblastoma, Adrenocortical carcinoma, Pheochromocytoma and paraganglioma (PPGL), Urethral cancer, Penile cancer, Localised penile cancer (organ-confined, node-negative), Node-positive and metastatic penile cancer, Localised adrenocortical carcinoma (ENSAT stage I to III, resectable), Advanced and metastatic adrenocortical carcinoma (ENSAT stage IV or unresectable), Hereditary pheochromocytoma and paraganglioma (SDHx, VHL, RET, NF1, MAX and TMEM127), Metastatic pheochromocytoma and paraganglioma
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.
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Kidney cancer subtype pages use these definitions; several rare entities are now diagnosed by molecular testing rather than appearance alone.
Cabozantinib is the preferred first-line targeted therapy for metastatic papillary renal cell carcinoma; immunotherapy combinations are being tested on top of it.
VEGF-directed therapy is the default for non-clear cell disease, with everolimus considered in chromophobe tumours; cabozantinib later became preferred for papillary disease.
Papillary renal cell carcinoma is several diseases; the finding of MET dependence in type 1 tumours underpins the use of cabozantinib and savolitinib, and the 2022 WHO classification dropped the type 1 and 2 split in favour of molecular entities.
Query for this cancer: (TITLE:"Papillary renal cell carcinoma" OR ABSTRACT:"Papillary renal cell carcinoma" OR TITLE:"KIRP" OR ABSTRACT:"KIRP" OR TITLE:"TCGA-KIRP" OR ABSTRACT:"TCGA-KIRP" OR TITLE:"kidney renal papillary cell carcinoma TCGA KIRP cohort" OR ABSTRACT:"kidney renal papillary cell carcinoma TCGA KIRP cohort" 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.
The targets of this cancer's medicines and the ones linked to it directly.
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.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
Tablets: take on an empty stomach (no food 2 hours before or 1 hour after). Avoid grapefruit.
No pharmacokinetic interactions expected (antibody). See the irAE guide for toxicity management.
Avoid grapefruit.
See all on the product pages:CabozantinibPembrolizumabSavolitinibSunitinib·Printable cards in the navigator
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