Renal Tumors

Question 1

What is the adult form of poycystic kidney disease?

Answer 1

autosomal dominant form 1/500

Question 2

What are the symptoms of renal cell carcinoma?

Answer 2

painless hematuria, palpable abdominal mass and dull flank pain

most frqt presentation is hematuria

polycythemai in 5-10% of cases due to EPO production

Question 3

What is the clear cell type of the kidney?

Answer 3

tumor looks clear on histoology, fatty

Question 4

What is are the histological and morphologic signs of chromophobe carcinoma?

Answer 4

circumscribed tumor
halo around a wrinkled nucleus
binucleated cell

Question 5

What are the sings of collecting duct carcinoma?

Answer 5

irregular aggregates of tumor cells

tumor is based in medulla/colecting system

Question 6

What is medullary carcinoma signs?

Answer 6

restricted to individuals african or mediterranean descent
pts who have sickle cell trait
presents at very high stage and resists chemo, worst outcomes

Question 7

What patients get acquired cystic disease-associated renal cancer?

Answer 7

chronic dialysis dependency have a 100x risk

Question 8

What are the histology and morphologic signs of acquired cystic disease-associated renal cancer?

Answer 8

oxalate crystals

variety of patterns but lots of vacuoles

Question 9

In what patients do clear cell tubulopapillary cancer occurs?

Answer 9

endstage kidneys whether non-cystic or cystic

Question 10

What are the staging of renal cell carcinoma?

Answer 10

avg 5-yr survival of 50% but varies according to histologic subtype
T1 <7cm
T3a at peak
T3b is invading renal vein

Question 11

What is the grading of renal cell carcinoam?

Answer 11

1: nuclei are like tiny dots
2: nucleoli inconspicuous
3: nucleoli appreciated at low power
4: bizarre cells

Question 12

Where do oncocytoma look like on histology?

Answer 12

arises from intercalated cells of collecting duct

Question 13

What is angiomyolipoma?

Answer 13

mesenchymal tumor vessels+smooth muscle + fat

most common benign tumor of kidney; common complication of hemorrhage

Question 14

What is a wilms tumor?

Answer 14

amost always a pediatric tumor; contains a variety fo cell and tissue components all derived from mesoderm

Question 15

What is undulated lesion: polypoid and papillary cystitis?

Answer 15

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

Question 16

What is nephrogenic adenoma most likely to present in?

Answer 16

61% of cases following GU surgery
often associated with chronic cystitis/longstanding infection
benign proliferation of tubules

Question 17

What is staging of TNM?

Answer 17

key determinant is whether muscularis propia is invaded; non-invasion leads to conservative management

Question 18

What are the urothelial papilloma histology?

Answer 18

minimally branching delicate apillae with fibrovasccular core
urothelium of normal thickness and polarity and no significant cytologic atypia

Question 19

What are the morphology and histology signs of flat intra-urothelial neoplasia?

Answer 19

loss of polarity
nuclear clustering-touch each other
increased nuclear size
nuclear pleomorphism
increased chromatin granularity
scattered nucleoli

Question 20

What are the signs of upper urothelial tract urothelial carcinoma?

Answer 20

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

Question 21

What is the structure of glomerular endothelial cells?

Answer 21

fenestrated
negatively charged surface
form initial filtration barrier
synthesize and maintain GBM

Question 22

What is the glomerular basement membrane?

Answer 22

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

Question 23

What are the visceral epithelial cells?

Answer 23

podocytes
synthesize and maintain GBM
cytoplasmic foot processes form filtration slit
podocytes are negatively charged

Question 24

What are mesangial cells?

Answer 24

cell cytoplasm contains myosin filaments
surrounded by GBM like matrix
provides structural support
modulate glomerular filtration

Question 25

What is a horseshoe kidney associated with?

Answer 25

turner syndrome

increased risk of infection and kidney stones

Question 26

What is ARPKD associated with?

Answer 26

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

Question 27

What are the main extrarenal manifestation ARPKD?

Answer 27

hepatic fibrosis
cholangitis
portal hypertension

Question 28

What are the morphology associated with ARPKD?

Answer 28

smooth kidney with numerous small cysts
cut section-cylindrical cysts extending radially through cortex
microscopically-cysts lined by cuboidal epithelium
glomeruli normal

Question 29

What is the cyst formation in autosomal dominant cyst formation?

Answer 29

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

Question 30

What are the clinical manifestations of ADPKD?

Answer 30

hematuria and mild proteinuria
HTN
progressive renal failure
infections
stones
pain

Question 31

What are ADPKD have extrarenal manifestations?

Answer 31

hepatic cysts
intracranial aneurysms
berry aneurysm due to HTN

Question 32

What are the ADPKD diagnosis?

Answer 32

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

Question 33

What are hte ADPKD for treatment goals?

Answer 33

slow progression to ESRD
-control BP
-Tx Infxns
Identify and manage extrarenal manifestations
control pain
renal replacement therapy when necessary

Question 34

What are the signs of medullary sponge kidney?

Answer 34

no inheritance pattern
dilated medullary and papillary collecting ducts
spongy appearance on pyelogram
complicated by:
recurrent urinary tract infections
kidney stones
hematuria