renal tumours n mr c dawson ms frcs n consultant urologist n fitzwilliam hospital n peterborough
TRANSCRIPT
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Renal TumoursRenal Tumours
Mr C Dawson MS FRCSMr C Dawson MS FRCS Consultant UrologistConsultant Urologist Fitzwilliam HospitalFitzwilliam Hospital PeterboroughPeterborough
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Classification of Renal Classification of Renal TumoursTumours
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Benign Renal tumoursBenign Renal tumours
Cysts account for 70% Cysts account for 70% asymptomatic renal massesasymptomatic renal masses
Cortical adenomaCortical adenoma OncocytomaOncocytoma Angiomyolipoma (80% associated Angiomyolipoma (80% associated
with tuberous sclerosis)with tuberous sclerosis)
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OncocytomaOncocytoma
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OncocytomaOncocytoma
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AngiomyolipomaAngiomyolipoma
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Renal cell carcinomaRenal cell carcinoma
3% adult cancers3% adult cancers M:F 2:1M:F 2:1 Majority of patients diagnosed in Majority of patients diagnosed in
6th to 7th decade6th to 7th decade Sporadic and hereditary formsSporadic and hereditary forms No specific causative agent No specific causative agent
detected - smoking suggested as a detected - smoking suggested as a significant risk factorsignificant risk factor
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Familial RCC (1)Familial RCC (1)
Only 2% of cases of RCC are familialOnly 2% of cases of RCC are familial Patients affected at an earlier age Patients affected at an earlier age
(mean 44 yrs)(mean 44 yrs) Relatives may be at risk and should be Relatives may be at risk and should be
screenedscreened Classified asClassified as
– RCC aloneRCC alone– RCC with additional features (e.g. as RCC with additional features (e.g. as
with Von Hippel Lindau disease)with Von Hippel Lindau disease)
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Familial RCC (2) - Von Familial RCC (2) - Von Hippel Lindau diseaseHippel Lindau disease
Von Hippel Lindau Disease - an Von Hippel Lindau Disease - an autosomally-inherited, autosomally-inherited, multisystemmultisystem disorder characterised by;disorder characterised by;– high risk of RCC (>70% by the age of 60)high risk of RCC (>70% by the age of 60)– retinal and cerebellar haemangioblastomataretinal and cerebellar haemangioblastomata– phaeochromocytomasphaeochromocytomas– renal, pancreatic, and epididymal cystrenal, pancreatic, and epididymal cyst
VHL is the most common cause of VHL is the most common cause of inherited RCC, and RCC is the most inherited RCC, and RCC is the most common cause of death in VHLcommon cause of death in VHL
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Familial RCC (3) - Von Familial RCC (3) - Von Hippel Lindau diseaseHippel Lindau disease
All patients (and relatives) should undergo screening All patients (and relatives) should undergo screening from age 15from age 15
Tumours >3cm should be removed to prevent Tumours >3cm should be removed to prevent metastasismetastasis
Tumours are often bilateral and/or multifocal - so Tumours are often bilateral and/or multifocal - so kidney preserving surgery often requiredkidney preserving surgery often required
Gene for VHL discovered in 1993 - acts normally as a Gene for VHL discovered in 1993 - acts normally as a tumour suppressor gene. Loss of both copies tumour suppressor gene. Loss of both copies therefore required.therefore required.
Inactivation of both VHL genes is Inactivation of both VHL genes is alsoalso the crucial step the crucial step in the development of most clear-cell type (non-in the development of most clear-cell type (non-familial) RCCsfamilial) RCCs
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Presentation of renal Presentation of renal cell carcinomacell carcinoma
Classic triad of pain, haematuria, Classic triad of pain, haematuria, and flank mass (rare)and flank mass (rare)
More commonly just pain and More commonly just pain and haematuriahaematuria
Symptoms of metastatic diseaseSymptoms of metastatic disease Paraneoplastic syndromesParaneoplastic syndromes INCIDENTAL - discovered while INCIDENTAL - discovered while
investigating another probleminvestigating another problem
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InvestigationInvestigation
Ultrasound - distinguish solid from Ultrasound - distinguish solid from cystic masscystic mass
CT - Staging, prior to surgeryCT - Staging, prior to surgery MRI - less sensitive than CT for MRI - less sensitive than CT for
lesions less than 3cmlesions less than 3cm Angiography - tumour in solitary Angiography - tumour in solitary
kidney if partial nephrectomy kidney if partial nephrectomy consideredconsidered
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CT Scan of Renal CT Scan of Renal tumour tumour
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MRI of lymph node MRI of lymph node from renal tumourfrom renal tumour
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Staging of renal carcinomaStaging of renal carcinoma
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Staging of renal carcinomaStaging of renal carcinoma
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TreatmentTreatment
Radical nephrectomy remains the only Radical nephrectomy remains the only effective method of treating primary renal effective method of treating primary renal carcinomacarcinoma
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Tumour in a solitary Tumour in a solitary kidney or bilateral kidney or bilateral tumourstumours Partial nephrectomy gives Partial nephrectomy gives
excellent short term results (72% excellent short term results (72% tumour free survival at 3 yrs)tumour free survival at 3 yrs)
Survival independent of whether Survival independent of whether tumour present in other kidneytumour present in other kidney
Survival dependent on stage of Survival dependent on stage of local tumourlocal tumour
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Treatment of Treatment of metastatic diseasemetastatic disease
Generally poor prognosisGenerally poor prognosis Renal cancer remains refractory to Renal cancer remains refractory to
treatment with Chemotherapy treatment with Chemotherapy Hormonal therapyHormonal therapy ImmunotherapyImmunotherapy Palliative nephrectomyPalliative nephrectomy
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PrognosisPrognosis
5 year survival5 year survival– 60-82% Stage I60-82% Stage I– 47-80% Stage II47-80% Stage II– 35-51% Stage III35-51% Stage III
Survival increased by pre-op radiotherapy in Survival increased by pre-op radiotherapy in some studiessome studies
Incidental tumours - 15-30% of all tumoursIncidental tumours - 15-30% of all tumours– Better prognosis (related to stage and grade)Better prognosis (related to stage and grade)– Significantly longer disease-free and overall Significantly longer disease-free and overall
survival than with symptomatic tumourssurvival than with symptomatic tumours
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SurvivalSurvival