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Renal tumors of adults
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Urinary Tract Tumors
• 2%-3% of all cancers in adults.
• The most common malignant tumor of the kidney is renal cell carcinoma.
• Tumors of the lower urinary tract are twice as common as renal cell carcinomas.
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Renal Cell Carcinoma (RCC)
• Origin: renal tubular epithelium.
• in cortex.
• 2%-3% of all cancers in adults.
• 6th-7th decades.
• M:F 2:1
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Predisposing factors
• smoking
• hypertension
• obesity
• occupational exposure to cadmium (nickel-cadmium batteries, etc).
• chronic dialysis & acquired polycystic disease
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New classification based on the
molecular origins of these tumors
• 1-Clear Cell Carcinomas
• 2-Papillary Renal Cell
Carcinomas
• 3-Chromophobe Renal
Carcinomas
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1-Clear Cell Carcinomas• most common type (70%- 80% of RCC).
• cells with clear or granular cytoplasm.
• may be:
1-Sporadic
2-Familial (including von Hippel-Lindau
(VHL) disease)
• The VHL gene is involved in familial and
also sporadic clear cell carcinomas (60%).
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VHL disease • VHL gene on chromosome 3p25 is a tumor
suppressor gene involved in limiting the angiogensis in response to hypoxia
• autosomal dominant
• predisposition to a variety of neoplasms:
1- hemangioblastomas of cerebellum
2- bilateral renal cysts
3- bilateral & multiple renal clear cell carcinomas (40%-60%).
4- Pheochromocytoma
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2-Papillary Renal Cell Carcinomas
• 10% to 15%.
• papillary growth pattern.
• multifocal and bilateral
• familial and sporadic forms.
• MET proto-oncogene on ch7q31.
• increased gene dosage due to duplications of chromosome 7 →↑ growth in proximal tubular epithelial cells
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3-Chromophobe Renal Carcinomas
• least common (5%)
• from intercalated cells of collecting ducts.
• tumor cells stain more darkly (i.e., they are less clear) than cells in clear cell ca.
• multiple losses of entire chromosomes, including 1, 2, 6, 10, 13, 17, and 21.
• extreme hypodiploidy.
• good prognosis.
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Renal cell carcinoma:
yellowish, spherical
neoplasm in one pole of
kidney.
Note the tumor in the
dilated, thrombosed renal
vein.
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Renal cell carcinoma (clear cell pattern)
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Clinical Course of all RCCThe characteristic triad of :
1- painless hematuria (50%)
2- palpable abdominal mass
3- dull flank pain
4-Fever
5-Polycythemia ( 5% - 10%): elaboration of erythropoietin by tumor.
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6- other Paraneoplastic syndromes:
• 1-hypercalcemia
• 2-Hypertension
• 3-Cushing syndrome
• 4-feminization or masculinization
• metastases most commonly to lungs and
bones.
• may invade the renal vein, sometimes
extending to inferior vena cava and even
into the right side of the heart.
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Urothelial tumors (transitional
cell carcinoma)• classified into :
1 -benign papilloma.
2-papillary urothelial neoplasms of low
grade
3-papillary urothelial carcinoma of high
grade
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Transitional cell carcinoma of
bladder
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Urothelial (transitional) cell
carcinomas
• Low-grade carcinomas are rarely invasive.
• may recur after removal.
• staging at the time of initial diagnosis is the most important prognostic factor
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Papillary Urothelial (transitional)
carcinoma-low grade
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squamous cell carcinomas
• only 5% of bladder cancers
• Associated with:
• Schistosomiasis infection
• chronic inflammation
• stone formation
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Clinical Course of bladder cancers
• Painless hematuria.
• M:F 3:1
• 50 to 70 years.
• Prognosis
• low-grade shallow →good prognosis.
• High grade lesions + deep →bad
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• Predisposing factors of bladder
cancers :
• not familial.
1- β-naphthylamine (paints; cigarettes)
2- Cigarette smoking.
3- Chronic cystitis.
4- Schistosomiasis.
5- drugs as cyclophosphamide.
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• Treatment:
• transurethral resection
• (BCG) injections➔ granulomatous
reaction (immune response against
cancer)
• Follow-up for recurrence with cystoscopy
and urine cytologic studies for the rest of
life.
• Radical cystectomy and chemotherapy
for advanced cases
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Papillary urothelial carcinoma of
ureter & renal pelvis
• rarest tumors of the collecting system (5%).
• Painless hematuria
• pain in costovertebral angle; hydronephrosis
• 5-year survival rate is less than 10%.
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Renal tumors of childhood
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Wilms Tumor• 3rd most common solid cancer < 10
years.
• derived from the mesoderm.
• sporadic or familial (autosomal dominant).
• Mutations: WT-1and 2 genes.
• primitive glomerular and tubular structures
• Treatment: surgery & chemotherapy
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Wilm's tumor of the kidney
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Wilm's tumor nests and sheets of dark blue cells
at the left with compressed normal renal parenchyma at the
right.
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The tumor shows attempts to form primitive glomerular and
tubular structures