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PH-RN08

Kidney tumours

How renal cell carcinomas arise from tubule cells, why VHL loss makes clear cell carcinoma act as if starved of oxygen, how it grows into the renal vein and spreads, and where benign tumours and paraneoplastic hormones fit.

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  • 4 steps · 10 questions
  • 3D anatomy, affected parts marked

By the end, you can

  • Relate the main kidney tumours to the tubule segments and cells they resemble.
  • Explain how loss of VHL on chromosome 3p drives clear cell renal cell carcinoma through hypoxia signalling.
  • Describe the gross and microscopic appearance of clear cell, papillary and chromophobe carcinoma, oncocytoma and angiomyolipoma, and what nucleolar grade measures.
  • Explain how renal cell carcinoma spreads, including growth into the renal vein, and what its stage records.
  • Relate the local and paraneoplastic effects of renal cell carcinoma (erythropoietin, PTHrP, renin) to their mechanisms.
  • Recognise the classic gross and microscopic features, WHO types, grade, stage and imaging of kidney tumours, including Wilms tumour, and explain how nephrectomy, ablation, VEGF-targeted kinase inhibitors, HIF-2α and mTOR inhibitors and checkpoint inhibitors act on the mechanism.

What it is

Most kidney tumours in adults are renal cell carcinomas (RCC), malignant tumours of the tubule epithelium. The main types, clear cell, papillary and chromophobe, differ in the cell they resemble, their genetics and their behaviour. Oncocytoma and angiomyolipoma are benign; tumours of the renal pelvis are urothelial carcinomas, and Wilms tumour (nephroblastoma) is the main kidney cancer of childhood.

5 types to tell apart
Clear cell renal cell carcinoma
The commonest type (about 70–75% of RCC), resembling proximal tubule cells. Both copies of VHL are inactivated; nests of clear cells lie among delicate capillaries. It tends to invade veins.
Papillary renal cell carcinoma
About 10–15%, also resembling proximal tubule cells: papillae with fibrovascular cores, often with gains of chromosomes 7 and 17 (MET mutations in the hereditary form). More often multiple or bilateral.
Chromophobe renal cell carcinoma
About 5%, resembling the intercalated cells of the collecting duct: pale cells with sharp borders and loss of several whole chromosomes. Usually less aggressive than clear cell carcinoma.
Benign tumours: oncocytoma and angiomyolipoma
Oncocytoma, also from intercalated cells, is a brown tumour of cells packed with mitochondria. Angiomyolipoma mixes thick-walled vessels, smooth muscle and fat, and is linked to tuberous sclerosis.
Wilms tumour (nephroblastoma)
An embryonal tumour of persisting immature kidney tissue, mainly in young children; it is covered with the other childhood tumours.

US spelling: tumours → tumors, behaviour → behavior, tumour → tumor.

Normal function

Tubule segments: the origin map

Each kidney holds about a million nephrons. Blood is filtered at the and the filtrate runs along a tubule whose segments are lined by different cells. The has tall cells with a brush border and many mitochondria, which reclaim most of the filtered salt, water and glucose. The and fine-tune the urine; the collecting duct has principal cells (sodium and water) and intercalated cells (acid and base).

These segments are an origin map. Clear cell and papillary carcinomas resemble proximal tubule cells; chromophobe carcinoma and the benign oncocytoma resemble intercalated cells. Angiomyolipoma comes from a mesenchymal cell (the perivascular epithelioid cell), and the urothelium lining the renal pelvis gives rise to urothelial carcinoma, as in the bladder.

The kidney is also an endocrine organ. Interstitial cells of the cortex make erythropoietin when oxygen delivery falls, and the release renin. Tumour cells can release these signals, and others, in excess.

Point to it on the model