Renal TumoursRenal Tumours

Mr C Dawson MS FRCSMr C Dawson MS FRCS Consultant UrologistConsultant Urologist Fitzwilliam HospitalFitzwilliam Hospital PeterboroughPeterborough

Classification of Renal Classification of Renal TumoursTumours

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)

OncocytomaOncocytoma

OncocytomaOncocytoma

AngiomyolipomaAngiomyolipoma

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

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)

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

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

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

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

CT Scan of Renal CT Scan of Renal tumour tumour

MRI of lymph node MRI of lymph node from renal tumourfrom renal tumour

Staging of renal carcinomaStaging of renal carcinoma

Staging of renal carcinomaStaging of renal carcinoma

TreatmentTreatment

Radical nephrectomy remains the only Radical nephrectomy remains the only effective method of treating primary renal effective method of treating primary renal carcinomacarcinoma

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

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

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

SurvivalSurvival