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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 l.jpg
Renal Tumours

  • Mr C Dawson MS FRCS

  • Consultant Urologist

  • Fitzwilliam Hospital

  • Peterborough



Benign renal tumours l.jpg
Benign Renal tumours

  • Cysts account for 70% asymptomatic renal masses

  • Cortical adenoma

  • Oncocytoma

  • Angiomyolipoma (80% associated with tuberous sclerosis)





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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 l.jpg
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 l.jpg
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


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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


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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 l.jpg
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






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Treatment

  • Radical nephrectomy remains the only effective method of treating primary renal carcinoma


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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


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Treatment of metastatic disease

  • Generally poor prognosis

  • Renal cancer remains refractory to treatment with Chemotherapy

  • Hormonal therapy

  • Immunotherapy

  • Palliative nephrectomy


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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



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