Adenocarcinoma of the renal cortex; most common type of kidney tumour
Aetiology
Risk factors
- Renal failure and dialysis
- Low socio-economic status
- Asbestos, cadmium exposure, phenacetin
- Genetic
- VHL accounts for most sporadic renal cancers
- 50% of cases of VHL syndrome will develop RCC
Pathophysiology
- Believed to arise from the proximal convoluted tubule
- 10-25% contains cysts or are predominantly cystic
- Bosniak score used to predict cancer vs cystic kidney disease
Histological classification
- Conventional clear cell carcinoma (80%) - loss of VHL
- Clear cells - cytoplasm rich in lipids and glycogen 3p deletion
- Papillary (10-15%)
- Elongated papillae often with foamy cells
- Chromophone (5%)
- Large cells with defined cell borders
- Atypical nuclei resembling raisins - 'raisinoid'
- Histologically similar to oncocytomas
- Collecting duct - rare, young patients, poor prognosis
- Most aggressive cancer
- High grade carcinoma of mixed cell type
- Medullary cell - young sickle cell patients, very poor prognosis
Spread
- RCC has a propensity for renal vein involvement and can even extend into vena cava and grow up towards the heart
Clinical presentation
- Haematuria, loin pain, mass - all 3 together in less than 10% of cases
- Pyrexia of unknown origin (8-9%)
- Paraneoplastic syndrome
- Polycythemia - renal cell carcinoma may produce excessive erythropoietin (EPO)
- Hypertension - increased renin production by the tumour
- Hypercalcaemia - parathyroid hormone-related peptide (PTHrP) produced by the tumour
Investigations
- CT chest abdomen, pelvis for staging
Management
Small tumours < 3-4 cm
- Surveillance in elderly unfit patients
- Ablation techniques in fit, elderly patients and selected younger patients
> 3-4 cm
- Surveillance, ablative techniques
Large tumours
Follow-up
- FBC, renal and liver functions
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