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Renal 17 Renal Tumor
Leonard: Renal Tumors
| Question | Answer |
|---|---|
| Wilms Tumor | Pediatric tumor compromised of embryonal nephrogenis elements |
| Wilms Tumor Triphasic Neoplasm | 3 possible tissue types Blastema: small densely packed cells with little cytoplasm Epithelium: abortive tubules and glomeruli Stroma: spindle cells |
| Wilms Tumor Clinical | 98% before 10, peak 1-3 85% of pediatric renal neoplasms Lung metastases are common |
| Wilms Tumor Rules of 5's | Accounts for ~5% of kid cancers 5% bilateral 5% multicentric 5% anaplastic- worse prognosis |
| Wilms Tumor Typical Clinical Presentation | Abdominal mass Abdominal pain Intestinal obstruction Hematuria |
| Wilms Tumor Prognosis | Poor Px if anaplastic, age >2, large size 80-90% overall cure rate |
| Wilms Tumor Treatment | Nephectomy plus chemotherapy Second neoplasms can occur form chemo |
| Wilms Tumor Pathogenesis | Loss/mutation of tumor suppressor genes on chromosome 11 WT1: regulates transcription of growth-promoting genes WT2: also interacts with growth promoting factors |
| Congenital Mesoblastic Nephroma | Tumor compromised of spindled cells with varying resemblance to immature tissues from mesoderm Most common renal tumor of infancy (only 2% or pediatric neoplasms) |
| Congential Mesoblastic Nephroma | 5-10% recur or metastasize by 1 y/o Metastases are rare: lungs, brain, rarely bone Tx: resection with wide margins |
| Adult Renal Neoplasms | Majority show histogenesis from epithelial components |
| Benign Adult Renal Neoplasms | Papillary adenoma -Importance of size of neoplasm Renal oncocytoma Angiomyolipoma |
| Renal papillary adenoma | Tumor size distinguishes papillary adenoma form carcinoma <5mm = adenoma >5mm = carcinoma |
| Renal papillary adenoma Clinical | Most common renal tubular epithelial neoplasm found in ~33% of cases of acquired cystic renal disease |
| Renal papillary adenoma Morphologic features | Well circumscribed, nonencapsulated Generally subcapsular |
| Renal Oncocytoma Clinical | Accounts for ~5% of restricted renal neoplasms M:F = 2:1 median age 60-65 No recorded deaths from metastases Central scar within lesion |
| Renal Oncocytoma Pathology | Well circumscribed tan/yellow/mahogany brown Central stellate scar (~1/3) Typically solitary Avg size = 6cm |
| Angiomyolipoma | Mesenchymal tumor ~1% of renal neoplasms Although benign, fatality may result from: Massive hemorrhage Renal failure from loss of parenchyma |
| Angiomyolipoma Associated with hereditary disorders | Tuberous sclerosis von Recklinhausen disease von-Hippel Lindau syndrome Autosomal dominant polycystic kidney disease |
| Angiomyolipoma Pathology | Usually solitary Multifocality suggests tuberous sclerosis LM: mature adipose, spindled cells w/smooth muscle features, thick walled blood vessels |
| Angimyolipoma Pathology (cont) | May involve veins and regional lymph nodes -probably indicative of multifocal growth, NOT metastases May coexist with other renal neoplasms |
| Malignant Renal Neoplasms (Adults) Renal cell carcinoma | The most common malignant renal neoplasm in adults Clear cell RCC is the most common type |
| Malignant Renal Neoplasms (Adults) Urothelial carcinoma (Transitional cell carcinoma) | Involves the collecting system Caylces and Pelvis |
| Renal Cell Carcinoma General | ~85% of diagnosed renal cancers in adults 2/3 are men, 1% are bilateral Bilaterally strongly suggestive of underlying syndrome Risk factors: tobacco |
| Renal Cell Carcinoma Clinical symptoms | Classic Triad - flank pain - palpable mass - hematuria* Only seen in 10% of cases |
| Renal Cell Carcinoma Metastases | ~25% of the time, metastases at time of Dx Typically metastases to lung, bone, lymph nodes, adrenals, liver, brain |
| Renal Cell Carcinoma Prognosis | Occasionally regress w/o Tx Overall 5 year survival=70% Tumor stage and nuclear grade most important Px factors |
| Renal Cell Carcinoma Treatment | Surgical resection - extent depends upon location and extent of disease Chemotherapy minimally effective |
| Renal Cell Carcinoma Clear cell RCC | Conventional/classic type Accounts for ~70% of adult renal cancers VHL gene-mutation at this locus seen in >90% of cases |
| Clear Cell RCC VHL gene | VHL gene acts as a tumor suppressor gene Mutation causes not enough degradation resulting in high levels of proangiogenic factors (VEGF) |
| Clear Cell RCC Prognosis | 50% die of dz Tumor stage: T-size of primary tumor N-presence of metastases to regional lymph node M-distant metastasis |
| Clear Cell RCC Nuclear grade | Fuhrman grade 1-4 |
| Papillary RCC | 10-15% of RCC (second most common RCC) Hereditary tumors-mutation in c-met gene Associated ESRD (dialysis kidneys) |
| Papillary RCC (cont) | More multiple than RCC >5mm by definition Typically present at early tumor stage Sig. better Px than Clear Cell RCC |
| Collecting Duct Carcinoma | Rare 50-66% die in first 2 years Worst Px of typical RCC subtypes Painless gross hematuria 50% coexisting urinary bladder urothelial carcinoma |
| Renal Medullary Carcinoma | Very rare, Very aggressive African-American or Mediterranean Die within 4-6 months of Dx Associated with sickle cell trait/dz |
| Renal Urothelial Carcinomas | Neoplasms of transitional cell epithelium (TCC) 5% of primary renal neoplasms |
| Renal Urothelial Carcinomas (cont) | Typically ass. with urothelial neoplasms elsewhere Multifocal Carcinogenic "field effect" (tobacco) Histologic grade and invasion |
Created by:
bcriss
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