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Malignant Renal tumors. DR.Gehan Mohamed. Malignant renal tumors. It may be: primary tumors : i.e arise from kidney tissue itself Secondary tumors: represent a metastasis from any malignant tumor present anywhere in the body and reach kidney.
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Malignant Renal tumors DR.Gehan Mohamed
Malignant renal tumors • It may be: • primary tumors : i.e arise from kidney tissue itself • Secondary tumors: represent a metastasis from any malignant tumor present anywhere in the body and reach kidney.
Primary malignant renal tumors include: A- malignant epithelial tumors : 1- Renal cell carcinoma (hypernephroma = renal cell adenocarcinoma) represents 80-85% ,it is subdivided into : a- Clear cell renal cell carcinoma b- Papillary renal cell carcinoma c- chromophobe renal cell carcinoma d - Collecting duct carcinoma 2- Transitional cell carcinoma 8% arise from transitional epithelium which line renal pelvis. B- malignant mesodermal tumors (i.e arise from smooth muscles or blood vessels or fat present in the kidney ) : which called Renal sarcomas e.gleiomyosarcoma(malignant tumor arise from smooth muscle) ,generally poorer outcome . C- mixed malignant epithelial and mesenchymal tumors : e.gNephroblastoma(Wilms’ tumor in children)
We will study in details : • RENAL CELL CARCINOMA(RCC) :more common in adult age. • Wilms tumor (nephroblastoma): more common in children (2-5 years)
RENAL CELL CARCINOMA(RCC): usually form golden yellow mass (as it rich in lipid ),this mass commonly present at upper or lower poles of the kidney
EPIDEMIOLOGY • male to female distribution 2:1 • Occurs in 5th to 7th decade of life • 55% of the cases are sporadic but 45%,occurs with familial predisposition .
Risk factors for RCC • Tobacco greatest risk factor • Occupational exposure to asbestos, petroleum products. • Obesity, Hypertension • Chronic phenacetin or aspirin use • Acquired polycystic kidney disease secondary to dialysis results in 30% increase risk specially the papillary type of RCC.
Diagnosis • Clinical presentation • investigations
Clinical presentation • Many kidney tumors go undetected due to the lack of symptoms and are incidentally detected during the medical evaluation of an unrelated problem. • Kidney tumors can cause symptoms by compressing, stretching or invading structures near or within the kidney.
Renal Cell Carcinoma Symptoms • Hematuria Blood in the urine (making the urine slightly rusty to deep red) • Pain in the side that does not go away • A lump or mass in the side or the abdomen • Weight loss, fever, anemia • Feeling very tired or having a general feeling of poor health. • high blood pressure
If cancer spreads beyond the kidney, symptoms depend upon which organ is involved such as : a - Shortness of breath or coughing up blood may occur when cancer is in the lungs b - bone pain or fracture may occur when cancer in the bone c- neurologic symptoms may occur when cancer is in the brain
Paraneoplastic syndromes: are symptoms and signs produced due to hormone like substances secreted from the tumor cells and not secondary to distant metastasis • Anemia- anemia of chronic disease • Hepatic dysfunction • Hypercalcemia • Cachexia and Fever • Erythrocytosis as 1-5% of these tumors produce erythropoietin • Secondary AA amyloidosis 3-5%
Investigations: 1-Radiographic evaluation - Ultrasound: to detect solid versus cystic lesions. - Contrast CT: test of choice to evaluate tumor size, location, lymph node involvement -MRI: to evaluate collecting system and blood vessel involvement. -Angiography • MRI with angiography • Used for embolization of large lesions preoperatively • Take Biopsy to confirm diagnosis and detect histopathologic types of RCC. 2-Immunohistochemical staining using CD10,inhibin
Histopathologic types of RCC a- Clear cell renal cell carcinoma b- Papillary renal cell carcinoma c- chromophobe renal cell carcinoma d - Collecting duct carcinoma
Pathogenesis of Clear cell type of RCC • 1- arise from epithelium of proximal convoluted tubule. • 2- there is deletion in the short arm of chromosome 3 or translocation between chromosome 3 and 6. • 3- so there is loss of VHL gene(tumor suppressor gene) present on chromosome 3 so there will not be inactivation of hypoxia inducible factor(HIF) , and insulin like growth hormone. • 4- So active HIF stimulate new blood vessel formation(angiogenesis) by production of vascular endothelial growth factor . • 5- so there is enough blood supply to the newly growing tumor cells.
Clear cell renal cell carcinoma formed of : a- sheets of cuboidal cells with clear cytoplasm rich in glycogen and fat(red arrow). b- these sheets are separated by thin branching capillaries (blue arrow).
Pathogenesis of papillary type of RCC • 1- arise from epithelium of distal convoluted tubule. • 2- there is trisomy of chromosome 7,16,17 . • 3- so there is mutation in MET gene present on chromosome 7. MET gene produce protein act as receptor for different growth factors which stimulate growth and proliferation of tumor cells, so mutated MET gene is called oncogene (i.e this gene promote tumor growth and formation )
Wilms tumor • It is a Malignant renal tumor • There is Wilms tumor gene mutation. • common in children 2-5 years. • It arises from nephrogenic rests(NR) • Microscopic picture characterized by triphasic pattern: a- dark blue immature blastema cells b- stromal cells c- epithelial elements in form of abortive glomeruli and abortive tubules.
