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10 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
| Setting | Approach | Guideline |
|---|---|---|
| Localised | Partial or radical nephrectomy, ablation or surveillance as for other kidney cancers; adjuvant therapy evidence is thin. | not mapped |
| Metastatic | Cabozantinib first line (PAPMET); savolitinib for MET-driven tumours; immunotherapy combinations on single-arm data; clinical trials preferred. | not mapped |
| Hereditary syndromes | Early surgery for FH-deficient tumours because they spread early; surveillance in MET carriers; genetic counselling of relatives. | not mapped |