1. Renal Cell Carcinoma & Other Solid renal lesions Dr Charles Chabert POW May 2005

2. Epidemiology 12 th most common site in men /17 th female Low amongst Asian & African Highest in Czech Republic: 20/10 per 100,000 WHO 2004

3. Epidemiology Increase in all groups till mid ’80s Increase in incidence after age 40 years Decrease after age 75 years Male more commonly affected WHO 2004

4. Aetiology Environmental Tobacco smoking Carcinogenic arsenic Asbestos, cadmium, organic solvents, fungal toxins Body Mass Index Hypertension Phenacetin WHO 2004

5. Genetic Basis For RCC von Hippel-Lindau (VHL) Birt-Hog-Dube (BHD) Hereditary Papillary renal CA Hereditary Leiomyomatosis & RCC Syndrome Pavlovich et at Urol Clin N Am 30,2003 437-454

6. von Hippel-Lindau First described by opthalmologists Autosomal dominant inherited VHL gene located at 3p25 pVHL = Tumour suppressor gene Pavlovich et at Urol Clin N Am 30,2003 437-454

7. von Hippel-Lindau Manifestations Renal cysts, Clear cell RCC Haemangiomas of retina & CNS Phaeochromocytoma Pancreatic cysts Endolymphatic sac Epididymal papillary cystadenomas Pavlovich et at Urol Clin N Am 30,2003 437-454

8. von Hippel-Lindau Type 1 No phaeochromocytoma Type2a Phaeochromocytoma CNS & Retina haemangioblastomas Type2b + pancreatic involvement Pavlovich et at Urol Clin N Am 30,2003 437-454

9. Clinical Features Retinal lesions occur first Mean onset 25 years CNS haemangiomas later- 30years Renal lesions – 37 years

10. Clinical Diagnosis Haemangioblastoma of CNS or retina & extraneuronal lesion + Family History Any one lesion Pavlovich et at Urol Clin N Am 30,2003 437-454

11. Birt-Hog-Dube Autosomal dominant Fibrofolliculomas Pulmonary cysts (90%) Spontaneous pneumothorax (20%) Renal neoplasms (25%)– hybrid oncocytic RCC Pavlovich: J Urol, 173(5). May 2005 1482-1486

15. Hereditary Papillary RC Autosomal dominant - 50% penetrance Papillary RCC Late onset, bilateral & multiple Gene location 7q31 - mutations MET

16. Hereditary Papillary RC MET: proto-oncogene Induces mitogenesis, morphogenesis and cellular migration No documented extrarenal manifestations

17. Hereditary Leiomyomatosis & RCC Aggressive PRCC Uterine leiomyomas/ leiomyosarcomas/ cutaneous nodules Single lesions & early metastasis

18. Clinical Features of RCC Classic triad Incidental finding Systemic symptoms Paraneoplastic syndromes – 30%

19. Paraneoplastic Syndromes Haematological: Anaemia Raised ESR Stauffer syndrome Fever

20. Amyloidosis: 8% Hypercalcaemia: 5-13% Erythrocytosis: 3-10% Hypertension: 10-40% Paraneoplastic Syndromes

21. Histologic Subtype Clear cell RCC: 70% Multilocular RCC Reduced mets & LR Papillary: 15% less aggressive 2 subtypes; type 2 more aggressive Leibovich et al Uro Clin Am 30 (2003) 481-497

22. Clear Cell RCC

24. Clear Cell RCC

25. Papillary RCC

26. Histology Chromophobe RCC: 5% Excellent prognosis Mets rare- propensity for liver Collecting Duct RCC: <1% Aggressive Rapid mets Leibovich et al Uro Clin Am 30 (2003) 481-497

27. Chromophobe RCC

28. Grading Fuhrman System: 4 grades Features assessed: Nuclear size, contour, nucleoli Grade 1: 86% 5 yr survival Grade 4: 24% 5yr survival

29. Staging AJCC TNM 2002 Changes from 1987-1997:stage T1 cutoff 2002: T1 substratified into T1a & T1b Validated by Salama et al: J Urol 173(5) May 2005.1492-1495

30. Management Radical nephrectomy Does Lap nephrectomy reproduce open technique? Issues: 1.Early vascular control 2.LN dissection 3.Adrenal gland

31. Lymph Node Dissection LN dissection unnecessary, if node –ve on imaging No difference in 5yr survival. 2% node positive – correlates with EORTC 1% Minervini et al BJU Int 2001;88:169-72

32. Adrenelectomy Large upper pole tumour or involved on CT Overall Incidence 5.7% T1-2 0.6% T4 40% Tsui et al J Urol 2000:163;437-41

33. Oncological Outcome 5 year recurrence free & CA specific survival 92% & 98% Portis et al J Urol 2002;167:1257-62

34. Conclusion Laparoscopic approach mirrors open New gold standard

35. Other Solid lesions Oncocytoma Aniomyolipoma Adenoma

36. Oncocytoma Benign epithelial neoplasms Mitochondria-rich, eosinophilic cytoplasm 5% renal neoplasms Centrally placed scar, spoke-wheel pattern

37. Oncocytoma

38. Angiomyolipoma Benign mesenchymal tumour Adipose tissue, smooth muscle & abnormally thickened blood vessels Independently or in association with TS

40. Metanephric Adenoma Rare condition recently diagnosed Benign course Histopathological diagnosis

41. Conclusion AML can be distinguished radiologically Others managed accordingly