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 |