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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).
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Renal Tumours • Mr C Dawson MS FRCS • Consultant Urologist • Fitzwilliam Hospital • Peterborough
Benign Renal tumours • Cysts account for 70% asymptomatic renal masses • Cortical adenoma • Oncocytoma • Angiomyolipoma (80% associated with tuberous sclerosis)
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 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 • 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
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