Leonard: Renal Tumors
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| Wilms Tumor | Pediatric tumor compromised of embryonal nephrogenis elements
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| Wilms Tumor Triphasic Neoplasm | 3 possible tissue types
Blastema: small densely packed cells with little cytoplasm
Epithelium: abortive tubules and glomeruli
Stroma: spindle cells
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| Wilms Tumor Clinical | 98% before 10, peak 1-3
85% of pediatric renal neoplasms
Lung metastases are common
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| Wilms Tumor Rules of 5's | Accounts for ~5% of kid cancers
5% bilateral
5% multicentric
5% anaplastic- worse prognosis
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| Wilms Tumor Typical Clinical Presentation | Abdominal mass
Abdominal pain
Intestinal obstruction
Hematuria
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| Wilms Tumor Prognosis | Poor Px if anaplastic, age >2, large size
80-90% overall cure rate
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| Wilms Tumor Treatment | Nephectomy plus chemotherapy
Second neoplasms can occur form chemo
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| 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
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| 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)
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| Congential Mesoblastic Nephroma | 5-10% recur or metastasize by 1 y/o
Metastases are rare: lungs, brain, rarely bone
Tx: resection with wide margins
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| Adult Renal Neoplasms | Majority show histogenesis from epithelial components
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| Benign Adult Renal Neoplasms | Papillary adenoma
-Importance of size of neoplasm
Renal oncocytoma
Angiomyolipoma
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| Renal papillary adenoma | Tumor size distinguishes papillary adenoma form carcinoma
<5mm = adenoma
>5mm = carcinoma
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| Renal papillary adenoma Clinical | Most common renal tubular epithelial neoplasm
found in ~33% of cases of acquired cystic renal disease
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| Renal papillary adenoma Morphologic features | Well circumscribed, nonencapsulated
Generally subcapsular
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| 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
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| Renal Oncocytoma Pathology | Well circumscribed
tan/yellow/mahogany brown
Central stellate scar (~1/3)
Typically solitary
Avg size = 6cm
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| Angiomyolipoma | Mesenchymal tumor
~1% of renal neoplasms
Although benign, fatality may result from:
Massive hemorrhage
Renal failure from loss of parenchyma
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| Angiomyolipoma Associated with hereditary disorders | Tuberous sclerosis
von Recklinhausen disease
von-Hippel Lindau syndrome
Autosomal dominant polycystic kidney disease
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| Angiomyolipoma Pathology | Usually solitary
Multifocality suggests tuberous sclerosis
LM: mature adipose, spindled cells w/smooth muscle features, thick walled blood vessels
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| Angimyolipoma Pathology (cont) | May involve veins and regional lymph nodes
-probably indicative of multifocal growth, NOT metastases
May coexist with other renal neoplasms
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| Malignant Renal Neoplasms (Adults) Renal cell carcinoma | The most common malignant renal neoplasm in adults
Clear cell RCC is the most common type
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| Malignant Renal Neoplasms (Adults) Urothelial carcinoma (Transitional cell carcinoma) | Involves the collecting system
Caylces and Pelvis
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| 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
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| Renal Cell Carcinoma Clinical symptoms | Classic Triad
- flank pain
- palpable mass
- hematuria*
Only seen in 10% of cases
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| Renal Cell Carcinoma Metastases | ~25% of the time, metastases at time of Dx
Typically metastases to lung, bone, lymph nodes, adrenals, liver, brain
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| Renal Cell Carcinoma Prognosis | Occasionally regress w/o Tx
Overall 5 year survival=70%
Tumor stage and nuclear grade most important Px factors
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| Renal Cell Carcinoma Treatment | Surgical resection - extent depends upon location and extent of disease
Chemotherapy minimally effective
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| 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
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| 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)
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| 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
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| Clear Cell RCC Nuclear grade | Fuhrman grade 1-4
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| Papillary RCC | 10-15% of RCC (second most common RCC)
Hereditary tumors-mutation in c-met gene
Associated ESRD (dialysis kidneys)
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| Papillary RCC (cont) | More multiple than RCC
>5mm by definition
Typically present at early tumor stage
Sig. better Px than Clear Cell RCC
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| 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
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| Renal Medullary Carcinoma | Very rare, Very aggressive
African-American or Mediterranean
Die within 4-6 months of Dx
Associated with sickle cell trait/dz
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| Renal Urothelial Carcinomas | Neoplasms of transitional cell epithelium (TCC)
5% of primary renal neoplasms
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| Renal Urothelial Carcinomas (cont) | Typically ass. with urothelial neoplasms elsewhere
Multifocal
Carcinogenic "field effect" (tobacco)
Histologic grade and invasion
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