Renal Tumors Flashcards
What is the adult form of poycystic kidney disease?
autosomal dominant form 1/500
What are the symptoms of renal cell carcinoma?
painless hematuria, palpable abdominal mass and dull flank pain
most frqt presentation is hematuria
polycythemai in 5-10% of cases due to EPO production
What is the clear cell type of the kidney?
tumor looks clear on histoology, fatty
What is are the histological and morphologic signs of chromophobe carcinoma?
circumscribed tumor
halo around a wrinkled nucleus
binucleated cell
What are the sings of collecting duct carcinoma?
irregular aggregates of tumor cells
tumor is based in medulla/colecting system
What is medullary carcinoma signs?
restricted to individuals african or mediterranean descent
pts who have sickle cell trait
presents at very high stage and resists chemo, worst outcomes
What patients get acquired cystic disease-associated renal cancer?
chronic dialysis dependency have a 100x risk
What are the histology and morphologic signs of acquired cystic disease-associated renal cancer?
oxalate crystals
variety of patterns but lots of vacuoles
In what patients do clear cell tubulopapillary cancer occurs?
endstage kidneys whether non-cystic or cystic
What are the staging of renal cell carcinoma?
avg 5-yr survival of 50% but varies according to histologic subtype
T1 <7cm
T3a at peak
T3b is invading renal vein
What is the grading of renal cell carcinoam?
1: nuclei are like tiny dots
2: nucleoli inconspicuous
3: nucleoli appreciated at low power
4: bizarre cells
Where do oncocytoma look like on histology?
arises from intercalated cells of collecting duct
What is angiomyolipoma?
mesenchymal tumor vessels+smooth muscle + fat
most common benign tumor of kidney; common complication of hemorrhage
What is a wilms tumor?
amost always a pediatric tumor; contains a variety fo cell and tissue components all derived from mesoderm
What is undulated lesion: polypoid and papillary cystitis?
polypoid and papillary cysts arise from catheter or stone
setting of submucosal edema, usually mixed inflammation
polypoid cystitis=more blunt projections; has a tip that is wider than its basee; distinguishes it from papillary cystitis
What is nephrogenic adenoma most likely to present in?
61% of cases following GU surgery
often associated with chronic cystitis/longstanding infection
benign proliferation of tubules
What is staging of TNM?
key determinant is whether muscularis propia is invaded; non-invasion leads to conservative management
What are the urothelial papilloma histology?
minimally branching delicate apillae with fibrovasccular core
urothelium of normal thickness and polarity and no significant cytologic atypia
What are the morphology and histology signs of flat intra-urothelial neoplasia?
loss of polarity nuclear clustering-touch each other increased nuclear size nuclear pleomorphism increased chromatin granularity scattered nucleoli
What are the signs of upper urothelial tract urothelial carcinoma?
renal pelvis and ureter
most cases are high grade and half are locally advanced, stage pT2
more agressive
mismatch repair genes (lynch syndrome) instability of at least two microsatellite markers was detected in 21% of cases
What is the structure of glomerular endothelial cells?
fenestrated
negatively charged surface
form initial filtration barrier
synthesize and maintain GBM
What is the glomerular basement membrane?
composed type 4 collagen
size and charge are main determinants of filtration
-heparan sulfate
-water and cationic proteins of lmw are permeable
-albumin permability limited
What are the visceral epithelial cells?
podocytes
synthesize and maintain GBM
cytoplasmic foot processes form filtration slit
podocytes are negatively charged
What are mesangial cells?
cell cytoplasm contains myosin filaments
surrounded by GBM like matrix
provides structural support
modulate glomerular filtration
What is a horseshoe kidney associated with?
turner syndrome
increased risk of infection and kidney stones
What is ARPKD associated with?
located on chromosome 6p21 diagnosed in utero by U/S large hyperechoic kidney, oligohydramnios, decreased urine in fetal bladder enlarged kidney at birth high mortality
What are the main extrarenal manifestation ARPKD?
hepatic fibrosis
cholangitis
portal hypertension
What are the morphology associated with ARPKD?
smooth kidney with numerous small cysts
cut section-cylindrical cysts extending radially through cortex
microscopically-cysts lined by cuboidal epithelium
glomeruli normal
What is the cyst formation in autosomal dominant cyst formation?
abnormal differentiation of epithelial cells
high proliferation rate
secretion of fluid into cysts with loss of connection to functionting nephrons
abnormal extracellular matrix
interstitial fibrosis
What are the clinical manifestations of ADPKD?
hematuria and mild proteinuria HTN progressive renal failure infections stones pain
What are ADPKD have extrarenal manifestations?
hepatic cysts
intracranial aneurysms
berry aneurysm due to HTN
What are the ADPKD diagnosis?
pts present in several different ways:
symptomatic presentation: typically flank pain and hematuria
mutliple bilateral cysts note icidentally on imaging study
screening due to famhx with U/S
What are hte ADPKD for treatment goals?
slow progression to ESRD -control BP -Tx Infxns Identify and manage extrarenal manifestations control pain renal replacement therapy when necessary
What are the signs of medullary sponge kidney?
no inheritance pattern dilated medullary and papillary collecting ducts spongy appearance on pyelogram complicated by: recurrent urinary tract infections kidney stones hematuria
