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Renal Tumors

  1. Wilm’s Tumor
​WAGR Syndrome
It is characterized by aniridia, genital anomalies, and mental retardation and a 33% chance of developing Wilms’ tumor. Patients with WAGR syndrome carry constitutional (germline) deletions of two genes WT1 and PAX6 both located at chromosome 11p13.
Denys-Drash syndrome
It is characterized by gonadal dysgenesis (male pseudohermaphroditism) and early onset nephropathy leading to renal failure. The characteristic glomerular lesion in these patients is a diffuse mesangial sclerosis. These patients also have germline abnormalities in WT1. in addition to Wilms tumors these individuals are also at increased risk for developing germ cell tumors called gonadoblastomas.
Beck with – Wiedemann syndrome
It characterized by enlargement of body organs (organomegaly), macroglossia, hemihypertrophy, omphalocele and abnormal large cells in adrenal cortex (adrenal cytomegaly). The genetic locus involved in these patients is in band p15.5 of chromosome 11 called “WT2”. In addition to Wilms tumors patients with Beckwith Wiedemann syndrome are also at increased risk for developing hepatoblastoma, adrenocortical tumors, rhabdomyosarcomas and pancreatic tumors.
Grossly, willms tumors tends to present as a large, solitary, well circumscribed mass and on cut section, the tumor is soft, homogeneous, and tan to grey with occasional foci of hemorrhage, cyst formation and necrosis.
Microscopically, wilms tumors are characterized by the classic triphasic combination of blastemal, stromal, and epithelial cell types (immature glomeruli and tubules) seen in majority of lesions.

Renal Cell Carcinoma

Renal cell carcinoma (adenocarcinoma of the kidney) – Most common site is proximal convoluted tubular epithelium.
  1. Tobacco is the most significant risk factor.
  2. Additional risk factors, including obesity (particularly in women); hypertension; unopposed estrogen therapy; and exposure to asbestos, petroleum products, and heavy metals.
  3. Chronic renal failure and acquired cystic disease and in tuberous sclerosis.
    Most renal cancer is sporadic, but unusual forms of autosomal-dominant familial cancers occurs, usually in younger individuals.
Familial variants: Q
  1. Von Hippel-Lindau (VHL) syndrome.  
  2. Hereditary (familial) clear cell carcinoma  
  3. Hereditary papillary carcinoma.
  1. The major types of tumors are as follows.
    Major types of Renal cell carcinoma, their histology and cytogenetics.
    1. Clear cell carcinoma
      1. It is the most common' type of renal cancer accounting for about 70% to 80% of the renal cell cancer.
      2. They are so named because they are made up to cells with clear or granular cytoplasm and are non papillary.
      3. They occur as solitary unilateral lesions.
      4. It arises from proximal tubular epithelium.
  1. Papillary Carcinoma
  1. It accounts for 10% to 15% of renal cancers
  2. It is characterized by papillary growth pattern
  3. It arises from distal convoluted tubule
  4. It can be multifocal and bilateral.
  5. These are most common type of renal cancer in patients who develop dialysis associated cystic disease.
  6. Papillary carcinoma also occurs in two forms hereditary and sporadic.
  1. Chromophobe renal carcinoma-
  1. Accounts for about 5% of Renal cell cancer
  2. Cells have prominent cell membrane and pale eosinophilic cytoplasm.
  1. Collecting duct (Bellini duct) carcinoma
    1. These account for approximately 1 % or less of renal epithelial neoplasm.
  1. Triad of diagnosis ­
    1. Hematuria (M.C reliable and earliest)        
    2. Costovertebral pain                      
    3. Palpable mass (Upper lobe)
There is tendency of renal vein invasion (Hematogenous metastasis) site of metastasis in order of frequency are:
  1. Lung (may be pulsatile, cannon ball)          
  2. Liver                  
  3. Adrenal
  4. Brain                                                                  
  5. Bone                  
  6. Across the midline to opposite kidney
  1. Paraneoplastic syndrome:
    1. Erythrocytosis
    2. Anemia
    3. Usually there is increase in hemoglobin level which may be relative
    4. Relative polycythemia results from any cause of dehydration, or excessive use of diuretics or
    5. vomiting
    6. It is also associated with an obscure condition called stress polycythemia or Gaisböck’s syndrome
  • Hypercalcemia                              
  • Hypertension                                                    
  • Cushing's syndrome
  • Eosinophilia                                  
  • Leukemoid reaction                                        
  • Feminization or masculinization
  • Galactorrhoea                                              
  • Non metastasis hepatopathy (Stauffer's syndrome)                  
  • Amyloidosis
  1. Investigation of choice is CT scan for diagnosis and MRI for staging
  2. X-ray shows central calcification
  3. Nephrectomy is treatment of choice

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