05.22 - Renal Neoplasia (Handorf) - PP + Handout Flashcards
WAGR and Denys-Drash are both associated with abnormalities of
Wilms Tumor 1 gene
Malignant neoplasm composed of cells with clear or eosinophilic cytoplasm w/in delicated vascular network
Clear Cell RC
2 different appearances of Papillary Carcinoma depending on angle of section
Nipples vs Papillae
Small renal cortical papillary neoplasms
Very common, look just like renal papillary carcinomas under microscope
Does RCC spread better hematogenously or by lymph
About equal
Most common type of renal cell carcinoma
Clear cell
Which subtype is more prone to bilateral or multifocal tumors
Papillary
Beckwith-Wiedemann Syndrome is associated with
loss of genetic imprinting that normally silences allele controlling ILGF2
loss of genetic imprinting that normally silences allele controlling ILGF2
Beckwith-Wiedemann Syndrome is associated with
What is helpful to radiologists in dx oncocytoma
Central Stellate Scar
Risk factors and average age for renal cell carcinoma
Smoking, HTN, Obesity - 64 years
3 congenital malformations associated with Wilms Tumor
WAGR Syndrome, Denys-Drash, Beckwith-Wiedemann
Large pale cells with prominent cell membranes
Chromophobe Carcinoma
Origin of Renal Cell Carcinoma
Renal Tubular Epithelium
Papillary Renal Cell Carcinoma
Short nipple-like or long finger-like projections of tumor cells in fibrovascular stalk
Behavior of Oncocytoma
Benign
Chromophobe Renal Cell Carcinomas
Large pale cells with prominent membranes
Chromophobe RCC is positive for
Hale’s colloidal iron stain
Genetic signature of Oncocytoma
None
Abundant cytoplasmic little red bacteria-size dots
Mitochondria in Oncocytoma
What is predictably common in renal cell carcinomas
Hemorrhage - tumoral blood vessels are abnormal and prone to rupture
Genetics of Papillary RCC
MET mutation
Where do Renal Cell Carcinomas spread
Perinephric Fat, Lungs, Bone, Lymph Nodes
Uncommon benign epithelial neoplasms arising from intercalated cells of CD
Oncocytoma
Why do CCC’s appear cystic
Liquefactive necrosis
MET mutation
Genetics of Papillary RCC
Oncocytoma
Uncommon benign epithelial neoplasms arising from intercalated cells of CD
Mahogony Brown kidney mass
Oncocytoma
Wilm’s tumor is composed, microscopically, of
a mixture of cellular elements: blastemal, stromal, epithelial
Very common, look just like renal papillary carcinomas under microscope
Small renal cortical papillary neoplasms
What causes “empty” appearance of CCC on US
Liquefaction
vHL mutations
Clear Cell Renal Carcinoma
Small round blue cells, abortive tubules or glomeruli, fibroblastic stromal cells, and anaplastic cells
Wilms Tumor is composed of
Which subtype of Renal Cell Carcinoma has best prognosis
Chromophobe (aneuploidy)
Most common presentation of Wilms tumor
Abdominal mass
Clear Cell Carcinoma cells may form
Abortive Tubules
Grossly mahagony brown w/ central stellate scar
Oncocytoma
Wilms Tumor is composed of
Small round blue cells, abortive tubules or glomeruli, fibroblastic stromal cells, and anaplastic cells
Activating mutations in MET –> TKR for HGF
Papillary Renal Cell Carcinoma is associated with what mutations
Mutations in Clear Cell Carcinoma and consequence
vHL -> excess HIFs lead to excess VEGF
Clear Cell Carcinoma is composed of cells with ____ corrleated with ___ apparent grossly
Vacuolated (lipid-laden) cytoplasm, correlated with yellow color grossly
Short nipple-like or long finger-like projections of tumor cells in fibrovascular stalk
Papillary Renal Cell Carcinoma
Why does CCC have delicate vascular network
VEGF/vHL
Second and Third Most common types of Renal Cell Carcinoma
Papillary and then Chromophobe
Central Stellate Scar
Oncocytoma
Papillary Renal Cell Carcinoma is associated with what mutations
Activating mutations in MET –> TKR for HGF
Acquired dialysis related renal cystic disease
60% clear/ 40% papillary
Positive for Hale’s colloidal iron stain
Chromophobe Carcinoma
First and Second most frequent presenting symptom of RCC
Hematuria, Dull Flank Pain
