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8 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.
Chromophobe kidney cancer comes from a different cell of the kidney's tubules, usually behaves gently and is cured by surgery. Its rare metastatic form responds poorly to immunotherapy, so kinase and mTOR inhibitors are used, and it runs in families with Birt-Hogg-Dube syndrome.
Chromophobe renal cell carcinoma arises from the intercalated cells of the collecting duct and is marked by loss of whole chromosomes (1, 2, 6, 10, 13, 17, 21) with TP53 and PTEN mutations in a minority; it must be told apart from the benign oncocytoma, which it resembles. It is a feature of Birt-Hogg-Dube syndrome, caused by germline FLCN mutations, along with skin fibrofolliculomas and lung cysts. Most tumours are found early and cured by partial nephrectomy, and surveillance is reasonable for small lesions. Metastatic disease is uncommon, responds poorly to PD-1 blockade and is treated with sunitinib or cabozantinib, everolimus, or lenvatinib plus everolimus, drawing on the mTOR pathway activity seen in the disease; sarcomatoid transformation carries the worst prognosis of any kidney cancer.
| Setting | Approach | Guideline |
|---|---|---|
| Localised | Partial nephrectomy or ablation; active surveillance for small tumours; no adjuvant therapy. | not mapped |
| Metastatic | Sunitinib, cabozantinib, everolimus or lenvatinib plus everolimus; immunotherapy has low response rates outside sarcomatoid disease; trials preferred. | not mapped |
| Birt-Hogg-Dube syndrome | Kidney surveillance with MRI, nephron-sparing surgery at 3 cm, and genetic counselling. | not mapped |