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Renal Cell Carcinoma

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Title: Renal Cell Carcinoma Author: Charles Chabert Last modified by: Charles Chabert Created Date: 5/2/2005 12:38:00 AM Document presentation format – PowerPoint PPT presentation

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Title: Renal Cell Carcinoma


1
Renal Cell Carcinoma Other Solid renal lesions
  • Dr Charles Chabert
  • POW
  • May 2005

2
Epidemiology
  • 12th most common site in men /17th female
  • Low amongst Asian African
  • Highest in Czech Republic 20/10 per 100,000

WHO 2004
3
Epidemiology
  • Increase in all groups till mid 80s
  • Increase in incidence after age 40 years
  • Decrease after age 75 years
  • Male more commonly affected

WHO 2004
4
Aetiology
  • Environmental
  • Tobacco smoking
  • Carcinogenic arsenic
  • Asbestos, cadmium, organic solvents, fungal
    toxins
  • Body Mass Index
  • Hypertension
  • Phenacetin

WHO 2004
5
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
6
von Hippel-Lindau
  • First described by opthalmologists
  • Autosomal dominant inherited
  • VHL gene located at 3p25
  • pVHL Tumour suppressor gene

Pavlovich et at Urol Clin N Am 30,2003 437-454
7
von Hippel-Lindau
  • Manifestations
  • Renal cysts, Clear cell RCC
  • Haemangiomas of retina CNS
  • Phaeochromocytoma
  • Pancreatic cysts
  • Endolymphatic sac
  • Epididymal papillary cystadenomas

Pavlovich et at Urol Clin N Am 30,2003 437-454
8
von Hippel-Lindau
  • Type 1
  • No phaeochromocytoma
  • Type2a
  • Phaeochromocytoma
  • CNS Retina haemangioblastomas
  • Type2b
  • pancreatic involvement

Pavlovich et at Urol Clin N Am 30,2003 437-454
9
Clinical Features
  • Retinal lesions occur first
  • Mean onset 25 years
  • CNS haemangiomas later- 30years
  • Renal lesions 37 years

10
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
11
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
12
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14
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15
Hereditary Papillary RC
  • Autosomal dominant - 50 penetrance
  • Papillary RCC
  • Late onset, bilateral multiple
  • Gene location 7q31 - mutations MET

16
Hereditary Papillary RC
  • MET proto-oncogene
  • Induces mitogenesis, morphogenesis and cellular
    migration
  • No documented extrarenal manifestations

17
Hereditary Leiomyomatosis RCC
  • Aggressive PRCC
  • Uterine leiomyomas/ leiomyosarcomas/ cutaneous
    nodules
  • Single lesions early metastasis

18
Clinical Features of RCC
  • Classic triad
  • Incidental finding
  • Systemic symptoms
  • Paraneoplastic syndromes 30

19
Paraneoplastic Syndromes
  • Haematological
  • Anaemia
  • Raised ESR
  • Stauffer syndrome
  • Fever

20
Paraneoplastic Syndromes
  • Amyloidosis 8
  • Hypercalcaemia 5-13
  • Erythrocytosis 3-10
  • Hypertension 10-40

21
Histologic Subtype
  • Clear cell RCC 70
  • Multilocular RCC Reduced mets LR
  • Papillary 15
  • less aggressive
  • 2 subtypes type 2 more aggressive

Leibovich et al Uro Clin Am 30 (2003) 481-497
22
Clear Cell RCC
23
Clear Cell RCC

24
Clear Cell RCC

25
Papillary RCC

26
Histology
  • Chromophobe RCC 5
  • Excellent prognosis
  • Mets rare- propensity for liver
  • Collecting Duct RCC lt1
  • Aggressive
  • Rapid mets

Leibovich et al Uro Clin Am 30 (2003) 481-497
27
Chromophobe RCC

28
Grading
  • Fuhrman System 4 grades
  • Features assessed
  • Nuclear size, contour, nucleoli
  • Grade 1 86 5 yr survival
  • Grade 4 24 5yr survival

29
Staging
  • AJCC TNM 2002
  • Changes from 1987-1997stage T1 cutoff
  • 2002 T1 substratified into T1a T1b
  • Validated by Salama et al J Urol 173(5) May
    2005.1492-1495

30
Management
  • Radical nephrectomy
  • Does Lap nephrectomy reproduce open technique?
  • Issues
  • Early vascular control
  • LN dissection
  • Adrenal gland

31
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 200188169-72
32
Adrenelectomy
  • Large upper pole tumour or involved on CT
  • Overall Incidence 5.7
  • T1-2 0.6
  • T4 40

Tsui et al J Urol 2000163437-41
33
Oncological Outcome
  • 5 year recurrence free CA specific survival 92
    98

Portis et al J Urol 20021671257-62
34
Conclusion
  • Laparoscopic approach mirrors open
  • New gold standard

35
Other Solid lesions
  • Oncocytoma
  • Aniomyolipoma
  • Adenoma

36
Oncocytoma
  • Benign epithelial neoplasms
  • Mitochondria-rich, eosinophilic cytoplasm
  • 5 renal neoplasms
  • Centrally placed scar, spoke-wheel pattern

37
Oncocytoma
38
Angiomyolipoma
  • Benign mesenchymal tumour
  • Adipose tissue, smooth muscle abnormally
    thickened blood vessels
  • Independently or in association with TS

39
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40
Metanephric Adenoma
  • Rare condition recently diagnosed
  • Benign course
  • Histopathological diagnosis

41
Conclusion
  • AML can be distinguished radiologically
  • Others managed accordingly
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