? Male predominance (M:F 1.5:1).
? Most common in sixth to eighth decades; peak
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incidence in sixth decade? Metastatic disease in 30% at diagnosis, and eventually
in 50% (lung, liver, bone, distant LN, adrenal, brain,
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opposite kidney, soft tissue)? Most sporadic RCCs are unilateral and unifocal
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? Stage at diagnosis is the most important prognostic factor? Predominant histologic type: adenocarcinoma arising from
tubular epithelium
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? Adenocarcinoma subtypes:? clear cel (75?85%)
? chromophilic/ papil ary (10?15%)
? chromophobe (5?10%)
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? oncocytic (rare)? Sarcomatoid (1?6%; poor prognosis)
Papil ary (chromophilic) renal cel carcinoma extending into the col ecting
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systemRisk factors
? Tobacco , urban environmental toxins (cadmium/ asbestos/ petrols),
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obesity, high dietary fat intake, acquired cystic renal disease from renalfailure
? Association with von Hippel-Lindau disease:
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? autosomal dominant? loss of 3p
? >70% chance developing RCC (almost al clear cel histology) risk of developing
multiple other benign and malignant tumors (retinal angiomas, CNS
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hemangioblastomas, pheochromocytoma , pancreatic cancer)Pathology
? Round to ovoid
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? Circumscribed by a pseudo capsule of compressed
parenchyma and fibrous tissue
? Nuclear features can be highly variable
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Diagnosis
? Common signs and symptoms:
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? hematuria (80%)
? flank pain (45%)
? flank mass (15%)
? classic triad of prior three only present in 10%
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? normocytic/normochromic anemia, fever, weight loss? Less common signs and symptoms:
? hepatic dysfunction without mets
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? Polycythemia? hypercalcemia (occurs in 25% of patients with RCC mets)
Paraneoplastic syndromes in 20% of patients with RCC
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Diagnosis
? Labs: CBC, LFT, BUN/Cr, LDH, urinalysis
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? Imaging:? CT abdomen
? MRI abdomen if CT suggests IVC involvement
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? Metastatic evaluation:? Chest X ray
? Bone scan or MRI brain only if clinically indicated
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CT scan shows right renal tumor with perinephric stranding suggesting invasion ofthe perinephric fat
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Contrast inferior venacavogram in patient with a right renal tumor showsinvolvement of the subdiaphragmatic vena cava
? PET: equivocal findings on conventional imaging
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? Percutaneous renal biopsy or aspiration: limited role
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Staging AJCC 7th Edition
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Prognostic Factors For RCC
Management
Stage I-I I
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Nephrectomy? Open radical nephrectomy, but laparoscopic gaining
popularity
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? Nephron sparing surgery via partial nephrectomy, ifpossible (open or laparoscopic)
? Possible to spare adrenal gland in ~75% cases
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No role for adjuvant chemo/immunotherapy
No widely accepted role for neoadjuvant or adjuvant
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radiotherapy.Retrospective data suggest possible utility in select
cases:
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? Positive surgical margins
? Local y advanced disease with perinephric fat invasion
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and adrenal invasion (IVC/renal vein extension alonedoes not increase local recurrence significantly)
? LN+
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? Unresectable (pre-op RT)
Stage IV
Cytoreductive nephrectomy: improved survival with
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nephrectomy followed by interferon alpha vs.interferon alpha alone
Systemic therapy
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? Immunotherapy (IL-2, interferon alpha, or combination)? High dose IL-2 only FDA approved treatment for
? Biologic agents show promise in recent trials
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? Bevacizumab
? Sorafenib or sunitinib
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? TemsirolimusConsider chemo (gemcitabine ? 5-FU or capecitabine)
Focal pal iation of metastases
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? RT alone
? Metastasectomy
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? Combination of both