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Epidemiology
• 12th most common site in men /17th female
• Low amongst Asian & African
• Highest in Czech Republic: 20/10 per 100,000
WHO 2004
Epidemiology
• Increase in all groups till mid ’80s
• Increase in incidence after age 40 years
• Decrease after age 75 years
• Male more commonly affectedWHO 2004
Aetiology
Environmental • Tobacco smoking• Carcinogenic arsenic• Asbestos, cadmium, organic solvents, fungal
toxins• Body Mass Index• Hypertension• Phenacetin
WHO 2004
Genetic Basis For RCC
• von Hippel-Lindau (VHL)
• Birt-Hog-Dube (BHD)
• Hereditary Papillary renal CA
• Hereditary Leiomyomatosis & RCC Syndrome
Pavlovich et at Urol Clin N Am 30,2003 437-454
von Hippel-Lindau
• First described by opthalmologists
• Autosomal dominant inherited
• VHL gene located at 3p25
• pVHL = Tumour suppressor genePavlovich et at Urol Clin N Am 30,2003 437-454
von Hippel-Lindau
Manifestations
• Renal cysts, Clear cell RCC
• Haemangiomas of retina & CNS
• Phaeochromocytoma
• Pancreatic cysts
• Endolymphatic sac
• Epididymal papillary cystadenomasPavlovich et at Urol Clin N Am 30,2003 437-454
von Hippel-Lindau
Type 1
No phaeochromocytoma
Type2a
Phaeochromocytoma
CNS & Retina haemangioblastomas
Type2b
+ pancreatic involvementPavlovich et at Urol Clin N Am 30,2003 437-454
Clinical Features
• Retinal lesions occur first
• Mean onset 25 years
• CNS haemangiomas later- 30years
• Renal lesions – 37 years
Clinical Diagnosis
• Haemangioblastoma of CNS or retina & extraneuronal lesion
• + Family History
• Any one lesion
Pavlovich et at Urol Clin N Am 30,2003 437-454
Birt-Hog-Dube
• Autosomal dominant
• Fibrofolliculomas
• Pulmonary cysts (90%)
• Spontaneous pneumothorax (20%)
• Renal neoplasms (25%)– hybrid oncocytic RCC
Pavlovich: J Urol, 173(5). May 2005 1482-1486
Hereditary Papillary RC
• Autosomal dominant - 50% penetrance
• Papillary RCC
• Late onset, bilateral & multiple
• Gene location 7q31 - mutations MET
Hereditary Papillary RC
• MET: proto-oncogene
• Induces mitogenesis, morphogenesis and cellular migration
• No documented extrarenal manifestations
Hereditary Leiomyomatosis & RCC
• Aggressive PRCC
• Uterine leiomyomas/ leiomyosarcomas/ cutaneous nodules
• Single lesions & early metastasis
Clinical Features of RCC
• Classic triad
• Incidental finding
• Systemic symptoms
• Paraneoplastic syndromes – 30%
• Amyloidosis: 8%
• Hypercalcaemia: 5-13%
• Erythrocytosis: 3-10%
• Hypertension: 10-40%
Paraneoplastic Syndromes
Histologic Subtype
• Clear cell RCC: 70%
• Multilocular RCC Reduced mets & LR
• Papillary: 15%
less aggressive
2 subtypes; type 2 more aggressiveLeibovich et al Uro Clin Am 30 (2003) 481-497
Histology
• Chromophobe RCC: 5%Excellent prognosis
Mets rare- propensity for liver
• Collecting Duct RCC: <1%
Aggressive
Rapid mets
Leibovich et al Uro Clin Am 30 (2003) 481-497
Grading
• Fuhrman System: 4 grades
• Features assessed:
• Nuclear size, contour, nucleoli
• Grade 1: 86% 5 yr survival
• Grade 4: 24% 5yr survival
Staging
• AJCC TNM 2002
• Changes from 1987-1997:stage T1 cutoff
• 2002: T1 substratified into T1a & T1b
• Validated by Salama et al: J Urol 173(5) May 2005.1492-1495
Management
• Radical nephrectomy
• Does Lap nephrectomy reproduce open technique?
• Issues:1. Early vascular control2. LN dissection3. Adrenal gland
Lymph Node Dissection
• LN dissection unnecessary, if node –ve on imaging
• No difference in 5yr survival.
• 2% node positive – correlates with EORTC 1%
Minervini et al BJU Int 2001;88:169-72
Adrenelectomy
• Large upper pole tumour or involved on CT
• Overall Incidence 5.7%
• T1-2 0.6%
• T4 40%
Tsui et al J Urol 2000:163;437-41
Oncological Outcome
• 5 year recurrence free & CA specific survival 92% & 98%
Portis et al J Urol 2002;167:1257-62
Oncocytoma
• Benign epithelial neoplasms
• Mitochondria-rich, eosinophilic cytoplasm
• 5% renal neoplasms
• Centrally placed scar, spoke-wheel pattern
Angiomyolipoma
• Benign mesenchymal tumour
• Adipose tissue, smooth muscle & abnormally thickened blood vessels
• Independently or in association with TS
Metanephric Adenoma
• Rare condition recently diagnosed
• Benign course
• Histopathological diagnosis