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Renal Tumours Mr C Dawson MS FRCS Consultant Urologist Fitzwilliam Hospital Peterborough Classification of Renal Tumours Benign Renal tumours Cysts account for 70% asymptomatic renal masses Cortical adenoma Oncocytoma Angiomyolipoma (80% associated with tuberous sclerosis) Oncocytoma Oncocytoma Angiomyolipoma Renal cell carcinoma 3% adult cancers M:F 2:1 Majority of patients diagnosed in 6th to 7th decade Sporadic and hereditary forms No specific causative agent detected smoking suggested as a significant risk factor Familial RCC (1) Only 2% of cases of RCC are familial Patients affected at an earlier age (mean 44 yrs) Relatives may be at risk and should be screened Classified as – RCC alone – RCC with additional features (e.g. as with Von Hippel Lindau disease) Familial RCC (2) - Von Hippel Lindau disease Von Hippel Lindau Disease - an autosomallyinherited, multisystem disorder characterised by; – – – – high risk of RCC (>70% by the age of 60) retinal and cerebellar haemangioblastomata phaeochromocytomas renal, pancreatic, and epididymal cyst VHL is the most common cause of inherited RCC, and RCC is the most common cause of death in VHL Familial RCC (3) - Von Hippel Lindau disease All patients (and relatives) should undergo screening from age 15 Tumours >3cm should be removed to prevent metastasis Tumours are often bilateral and/or multifocal - so kidney preserving surgery often required Gene for VHL discovered in 1993 - acts normally as a tumour suppressor gene. Loss of both copies therefore required. Inactivation of both VHL genes is also the crucial step in the development of most clear-cell type (non-familial) RCCs Presentation of renal cell carcinoma Classic triad of pain, haematuria, and flank mass (rare) More commonly just pain and haematuria Symptoms of metastatic disease Paraneoplastic syndromes INCIDENTAL - discovered while investigating another problem Investigation Ultrasound - distinguish solid from cystic mass CT - Staging, prior to surgery MRI - less sensitive than CT for lesions less than 3cm Angiography - tumour in solitary kidney if partial nephrectomy considered CT Scan of Renal tumour MRI of lymph node from renal tumour Staging of renal carcinoma Staging of renal carcinoma Treatment Radical nephrectomy remains the only effective method of treating primary renal carcinoma Tumour in a solitary kidney or bilateral tumours Partial nephrectomy gives excellent short term results (72% tumour free survival at 3 yrs) Survival independent of whether tumour present in other kidney Survival dependent on stage of local tumour Treatment of metastatic disease Generally poor prognosis Renal cancer remains refractory to treatment with Chemotherapy Hormonal therapy Immunotherapy Palliative nephrectomy Prognosis 5 year survival – 60-82% Stage I – 47-80% Stage II – 35-51% Stage III Survival increased by pre-op radiotherapy in some studies 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 Survival