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MALIGNANT TUMORS
Wilms tumor Renal Cell Carcinoma Transitional Cell Carcinoma
•Most common tumor in patients with ages 2-5 •Also known as Hypernephroma •2% of all malignant tumors
yrs old •80 to 90% of malignant tumor of the kidney •Predominantly found in patients between the ages to 50
•Good survival rate (90%) •1 to 3% of all cancers to 80
•Malformation syndromes occur with •Predominantly occurs on the 6 to 7 decade of life •Chromosome 9 and 17p deletion are implicated
chromosome 11p •Presents as painless hematuria (90%) •Most important evaluation tool for prognostication is
Types: Risk factors: depth of invasion
1. WAGR (Wilms tumor, aniridia, genital –Cigarette smoking
anomalies, mental retardation) –Obesity Otherwise known as urothelial tumors
–a. involves deletion of chromosome 11p band –Hypertension •95% of all bladder tumors
13 –Unopposed estrogen therapy
–Exposure to heavy metals Epidemiology:
2. Denys Drash syndrome –80% of patients are between 50-80 yrs ol
–Genetic abnormality is the dominant negative Appears as yellowish spherical masses (3-15 cm diameter) –cigarette smoking
mutation in the WT-1 gene •von Hippel-Lindau (VHL) syndrome 50-70% develop RCC –schistosoma haematobium infections
–patients present with gonadal dysgenesis, •inherited as autosomal dominant trait –cyclophosphamide
nephropathy and renal failure –industrial exposure to arylamines (2-napthylamine)
Major classification types:
3. Beckwith-Wiedemann syndrome •Clear cell (70-80%) Prognosis
–involves deletion of chromosome 11 band •Papillary carcinoma (10-15%) Grade Prognosis (10 yr survival)
p15.5 (WT-2 gene) •Chromophobe renal carcinoma (5%) Grade 1(papilloma) 98%
–characterized histologically by blastema, Prognosis Grade 3 65%
immature stroma and tubules –45% five year survival rate
–may be associated with anaplasia (5%) –70% in the absence of metastasis