1. Renal Tumours n Mr C Dawson MS FRCS n Consultant Urologist n Fitzwilliam Hospital n Peterborough

2. Classification of Renal Tumours

3. Benign Renal tumours n Cysts account for 70% asymptomatic renal masses n Cortical adenoma n Oncocytoma n Angiomyolipoma (80% associated with tuberous sclerosis)

4. Oncocytoma

5. Oncocytoma

6. Angiomyolipoma

7. Renal cell carcinoma n 3% adult cancers n M:F 2:1 n Majority of patients diagnosed in 6th to 7th decade n Sporadic and hereditary forms n No specific causative agent detected - smoking suggested as a significant risk factor

8. Familial RCC (1) n Only 2% of cases of RCC are familial n Patients affected at an earlier age (mean 44 yrs) n Relatives may be at risk and should be screened n Classified as –RCC alone –RCC with additional features (e.g. as with Von Hippel Lindau disease)

9. Familial RCC (2) - Von Hippel Lindau disease n Von Hippel Lindau Disease - an autosomally- inherited, multisystem disorder characterised by; –high risk of RCC (>70% by the age of 60) –retinal and cerebellar haemangioblastomata –phaeochromocytomas –renal, pancreatic, and epididymal cyst n VHL is the most common cause of inherited RCC, and RCC is the most common cause of death in VHL

10. Familial RCC (3) - Von Hippel Lindau disease n All patients (and relatives) should undergo screening from age 15 n Tumours >3cm should be removed to prevent metastasis n Tumours are often bilateral and/or multifocal - so kidney preserving surgery often required n Gene for VHL discovered in 1993 - acts normally as a tumour suppressor gene. Loss of both copies therefore required. n Inactivation of both VHL genes is also the crucial step in the development of most clear-cell type (non-familial) RCCs

11. Presentation of renal cell carcinoma n Classic triad of pain, haematuria, and flank mass (rare) n More commonly just pain and haematuria n Symptoms of metastatic disease n Paraneoplastic syndromes n INCIDENTAL - discovered while investigating another problem

12. Investigation n Ultrasound - distinguish solid from cystic mass n CT - Staging, prior to surgery n MRI - less sensitive than CT for lesions less than 3cm n Angiography - tumour in solitary kidney if partial nephrectomy considered

13. CT Scan of Renal tumour

14. MRI of lymph node from renal tumour

15. Staging of renal carcinoma

17. Treatment n Radical nephrectomy remains the only effective method of treating primary renal carcinoma

18. Tumour in a solitary kidney or bilateral tumours n Partial nephrectomy gives excellent short term results (72% tumour free survival at 3 yrs) n Survival independent of whether tumour present in other kidney n Survival dependent on stage of local tumour

19. Treatment of metastatic disease n Generally poor prognosis n Renal cancer remains refractory to treatment with Chemotherapy n Hormonal therapy n Immunotherapy n Palliative nephrectomy

20. Prognosis n 5 year survival –60-82% Stage I –47-80% Stage II –35-51% Stage III n Survival increased by pre-op radiotherapy in some studies n Incidental tumours - 15-30% of all tumours –Better prognosis (related to stage and grade) –Significantly longer disease-free and overall survival than with symptomatic tumours

21. Survival