CH12 - Kidney and Urinary Tract Pathology Flashcards
What is the most common congenital renal anomaly?
Horseshoe kidney - Conjoined kidneys usually connected at the lower pole
In horseshoe kidney where is it located?
Kidney is abnormally located in the lower abdomen
What happens in horseshoe kidney?
The kidney gets caught on the inferior mesenteric artery root during its ascent from the pelvis to the abdomen
What is renal agenesis?
Absent kidney formation; may be unilateral or bilateral
What does unilateral renal agenesis lead to?
hypertrophy of the existing kidney; hyperfiltration increases risk of renal failure later in life.
What does bilateral agenesis lead to?
oligohydramnios with lung hypoplasia, flat face with low set ears, and developmental defects of the extremities (Potter sequence) incompatible with life
What is dysplastic kidney?
Non-inherited, congenital malformation of the renal parenchyma characterized by cysts and abnormal tissue (e.g., cartilage)
Is dysplastic kidney unilateral or bilateral?
Usually unilateral; when bilateral, must be distinguished from inherited polycystic kidney disease
What is polycystic kidney disease?
Inherited defect leading to bilateral enlarged kidneys with cysts in the renal cortex and medulla
How does the autosomal recessive form of polycystic kidney disease present?
in infants as worsening renal failure and hypertension; newborns may present with Potter sequence
What is the autosomal recessive form of polycystic kidney disease associated with?
congenital hepatic fibrosis (leads to portal hypertension) and hepatic cysts
How does the autosomal dominant form of polycystic kidney disease present?
in young adults as hypertension (due to increased renin), hematuria, and worsening renal failure
What is the autosomal dominant form of polycystic kidney disease due to?
A mutation in the APKD1 or APKD2 gene; cysts develop over time.
What is the autosomal dominant form of polycystic kidney disease associated with?
berry aneurysm, hepatic cysts, and mitral valve prolapse
What is medullary cystic kidney disease?
Inherited (autosomal dominant) defect leading to cysts in the medullary collecting ducts
What does parenchymal fibrosis result in?
shrunken kidneys and worsening renal failure
What is acute renal failure?
Acute, severe decrease in renal function (develops within days)
What is the hallmark of acute renal failure?
Azotemia, (increased BUN and creatinine) often with oliguria
What is acute renal failure divided into?
Its divided into prerenal, postrenal, and intrarenal azotemia and is based on etiology
What is prerenal azotemia?
Due to decreased blood flow to kidneys (e.g., cardiac failure)
Prerenal azotemia is a common cause of what?
ARF (acute renal failure)
What does decreased blood flow result in?
decreased GFR, azotemia, and oliguria.
In prerenal azotemia what happens to the ability to absorb fluid?
reabsorption of fluid and BUN ensues (serum BUN:Cr ratio > 15) tubular function remains intact (fractional excretion of sodium [FENa] < 1% and urine osmolality [osm] > 500 mOsm/kg)
What is postrenal azotemia due to?
obstruction of urinary tract downstream from the kidney (e.g., ureters)
In postrenal azotemia what does the decreased outflow result in?
decreased GFR, azotemia, and oliguria
In postrenal azotemia, what happens during the early stage of obstruction?
There is increased tubular pressure which forces BUN into the blood (serum BUN;Cr ratio > 15); tubular function remains intact (FENa < 1% and urine osm > 500 mOsm/kg).
In postrenal azotemia, what happens with long-standing obstruction?
tubular damage ensues, resulting in decreased reabsorption of BUN (serum BUN:Cr ratio < 15), decreased reabsorption of sodium (FENa > 2%), and inability to concentrate urine (urine osm < 500 mOsm/kg)
What is acute tubular necrosis?
It is injury and necrosis of tubular epithelial cells
What is the most common cause of acute renal failure?
Acute tubular necrosis (intrarenal azotemia)
In acute tubular necrosis, what happens?
Necrotic cells plug tubules; obstruction decreases GFR
In acute tubular necrosis, what is seen in the urine?
Brown, granular casts are seen in the urine
In acute tubular necrosis what does dysfunctional tubular epithelium result in?
decreased reabsorption of BUN (serum BUN:Cr ratio < 15), decreased reabsorption of sodium (FENa > 2%), and inability to concentrate urine (urine osm < 500 mOsm/kg).
What is the etiology for acute tubular necrosis?
may be ischemic or nephrotoxic,
What happens with ischemia and acute tubular necrosis?
Decreased blood supply results in necrosis of tubules.
With ischemia causing acute tubular necrosis, what is it often preceded by?
prerenal azotemia
With ischemia causing acute tubular necrosis, what parts of the nephron are particularly susceptible to ischemic damage?
proximal tubule and medullary segment of the thick ascending limb
What is another cause, aside from ischemia that results in acute tubular necrosis?
its nephrotoxic and toxic agents result in necrosis of tubules
In nephrotoxic damage leading to acute tubular necrosis, what parts of the nephron are particularly susceptible to damage?
Proximal tubule
What is the most common cause of nephrotoxic damage leading to acute tubular necrosis?
aminoglycosides
In nephrotoxic damage leading to acute tubular necrosis, what are the causes?
They include aminoglycosides, heavy metals, myoglobinuria, ethylene glycol, radiocontrast dye, and urate
What is an example of heavy metals leading to acute tubular necrosis?
lead
What causes myoglobinuria leading to acute tubular necrosis?
and example is from crush injury to muscle
What is ethylene glycol leading to acute tubular necrosis associated with?
oxalate crystals in urine
What is an example of urate leading to acute tubular necrosis?
tumor lysis syndrome
In nephrotoxicity leading to acute tubular necrosis what are used prior to initiation of chemotherapy?
Hydration and allopurinol
Why is hydration and allopurinol used prior to initiation of chemotherapy?
to decrease risk of urate-induced ATN.
What are the clinical features for acute tubular necrosis?
1) Oliguria with brown, granular casts 2) Elevated BUN and creatinine 3) Hyperkalemia (due to decreased renal excretion) with metabolic acidosis
Why is there hyperkalemia in acute tubular necrosis?
its due to decreased renal excretion
Is acute tubular necrosis reversible?
Yes, but often requires supportive dialysis since electrolyte imbalances can be fatal
In acute tubular necrosis, for how long can oliguria persist?
for 2 -3 weeks before recovery
In acute tubular necrosis, why is that oliguria can persist for 2-3 weeks?
tubular cells (stable cells) take time to reenter the cell cycle and regenerate
What is acute interstitial nephritis?
Drug-induced hypersensitivity involving the interstitium and tubules
What does acute interstitial nephritis result in?
acute renal failure (intrarenal azotemia)
What are the causes of acute interstitial nephritis?
They include NSAIDs, penicillin, and diuretics
What does acute interstitial nephritis present as?
oliguria, fever, and rash days to weeks after starting a drug; eosinophils may be seen in urine.
How does acute interstitial nephritis resolve?
with cessation of drug
What may acute interstitial nephritis progress to?
renal papillary necrosis
What is renal papillary necrosis?
Necrosis of renal papillae
What does renal papillary necrosis present with?
gross hematuria and flank pain
What are the causes for renal papillary necrosis?
- Chronic analgesic abuse (e.g., long-term phenacetin or aspirin use) 2. Diabetes mellitus 3. Sickle cell trait or disease 4. Severe acute pyelonephritis
What is nephrotic syndrome?
Glomerular disorders characterized by proteinuria (> 3.5 g/day)
What does nephrotic syndrome result in?
- Hypoalbuminemia 2. Hypogammaglobulinemia 3. Hypercoagulable state 4. Hvperlipidemia and hypercholesterolemia
In nephrotic syndrome, what is involved with hypoalbuminemia being the cause?
pitting edema
In nephrotic syndrome caused y hypogammaglobulinemia, what is there an increased risk of?
infection
In nephrotic syndrome caused by a hypercoagulable state, what is that state due to?
loss of antithrombin III
With hyperlipidemia and hypercholesterolemia causing nephrotic syndrome, what may result?
This may result in fatty casts in urine
What is the most common cause of nephrotic syndrome in children?
Minimal change disease (MCD)
What is the etiology for MCD?
For minimal change disease, it is usually idiopathic and may be associated with Hodgkin lymphoma
How does MCD appear on H&E stain?
Normal glomeruli on H&E stain, lipid may be seen in proximal tubule cells
In MCD, what is seen on electron microscopy?
effacement of foot processes
In MCD what is seen on immunofluorescence and why?
negative immunofluorescence (IF) because there are no immune complex deposits
In MCD, what happens in terms of proteinuria?
There is selective proteinuria where there is loss of albumin, but not immunoglobulin
What is the treatment for MCD?
It has an excellent response to steroids
How is the damage in MCD mediated?
damage is mediated by cytokines from T cells
What is the most common cause of nephrotic syndrome in Hispanics and African Americans?
Focal segmental glomerulosclerosis (FSGS)
What is the etiology for focal segmental glomerulosclerosis?
It is usually idiopathic, maybe associated with HIV, heroin use, and sickle cell disease
What is focal segmental glomerulosclerosis?
Focal (some glomeruli) and segmental (involving only part of the glomerulus) sclerosis on H&E stain
What is seen on electron microscopy in FSGS?
In focal segmental glomerulosclerosis, there is effacement of foot processes on EM
For focal segmental glomerulosclerosis, what is seen on IF?
Negative immunofluorescence due to no immune complex deposits
How does FSGS respond to steroids and what may it progress to?
Poor response to steroids and may progress to chronic renal failure
What is the most common cause of nephrotic syndrome in Caucasian adults?
Membranous nephropathy
What is the etiology for membranous nephropathy?
It is usually idiopathic, it may be associated with hepatitis B or C, solid tumors, SLE, or drugs (e.g., NSAIDs and penicillamine)
For membranous nephropathy, what is seen on H&E stain?
Thick glomerular basement membrane
What is the appearance of membranous nephropathy on electron microscopy?
subepithelial deposits with spike and dome appearance on EM
For membranous nephropathy, what is its appearance on EM due to?
immune complex deposition
How does membranous nephropathy respond to steroids and what might it progress to?
Poor response to steroids; progresses to chronic renal failure
What is membranoproliferative glomerulonephritis?
Thick glomerular basement membrane on H&E stain, often with tram-track appearance
What is membranoproliferative glomerulonephritis due to?
immune complex deposition (granular IF)
What is membranoproliferative glomerulonephritis divided into?
two types based on location of deposits
What is membranoproliferative glomerulonephritis - Type I?
subendothelial associated with HBV and HCV
What is membranoproliferative glomerulonephritis - Type II?
(dense deposit disease) intramembranous; associated with C3 nephritic factor (autoantibody that stabilizes C3 convertase, leading to overactivation of complement, inflammation, and low levels of circulating C3)
How does membranoproliferative glomerulonephritis respond to steroids and what might it progress to?
Poor response to steroids; progresses to chronic renal failure
How is diabetes mellitus related to nephrotic syndrome?
high serum glucose leads to nonenzymatic glycosylation of the vascular basement membrane resulting in hyaline arteriolosclerosis.
With diabetes mellitus leading to nephrotic syndrome what happens to the glomerular filtration pressure?
There is high glomerular filtration pressure
With diabetes mellitus leading to nephrotic syndrome why is there high glomerular filtration pressure?
glomerular efferent arteriole is more affected than the afferent arteriole
With diabetes mellitus leading to nephrotic syndrome resulting in high glomerular filtration pressure, what does this lead to?
Leads to hyperfiltration injury leading to microalbuminuria
What does diabetes mellitus relating to kidney?s eventually progress to?
nephrotic syndrome
What is diabetes mellitus leading to nephrotic syndrome characterized by?
sclerosis of the mesangium with formation of Kimmelstiel-Wilson nodules
In diabetes mellitus leading to nephrotic syndrome what slows the progression of hyperfiltration-induced damage?
ACE inhibitors
What is the most commonly involved organ in systemic amyloidosis?
The kidney
How does systemic amyloidosis lead to nephrotic syndrome?
Amyloid deposits in the mesangium, resulting in nephrotic syndrome
How is systemic amyloidosis characterized?
by apple-green birefringence under polarized light after staining with Congo red
What is nephritic syndrome?
Glomerular disorders characterized by glomerular inflammation and bleeding
What is seen in nephritic syndrome?
- Limited proteinuria (< 3.5 g/day) 2. Oliguria and azotemia 3. Salt retention with periorbital edema and hypertension 4. RBC casts and dysmorphic RBCs in urine
In nephritic syndrome what is seen in biopsy?
it reveals hypercellular, inflamed glomeruli
In nephritic syndrome, what mediates damage?
Immune-complex deposition activates complement; C5a attracts neutrophils, which mediate damage,
What is poststreptococcal glomerulonephritis (PSGN)?
Nephritic syndrome that arises alter group A beta-hemolytic streptococcal infection of the skin (impetigo) or pharynx
In PSGN, what does it occur with?
nephritogenic strains (which carry the M protein virulence factor)
PSGN may occur after infection with what organisms?
nonstreptococcal organisms as well as nephritogenic strains
How does PSGN present?
2-3 weeks after infection as hematuria (cola-colored urine), oliguria, hypertension, and periorbital edema
PSGN is usually seen in whom?
children, but may occur in adults
In PSGN what is seen on H&E stain?
Its hypercellular, inflamed glomeruli on H&E
In PSGN, what is seen on electron microscopy?
subepithelial humps on EM, and is mediated by immune complex deposition - granular IF
What is the treatment for PSGN?
Treatment is supportive.
What is the chance for renal failure in PSGN?
Children rarely (1%) progress to renal failure
In PSGN, what might some adults develop?
25% might develop rapidly progressive glomerulonephritis (RPGN)
What is rapidly progressive glomerulonephritis?
Nephritic syndrome that progresses to renal failure in weeks to months
What is rapidly progressive glomerulonephritis characterized by?
crescents in Bowman space (of glomeruli) on H&E stain; crescents are comprised of fibrin and macrophages
For rapidly progressive glomerulonephritis how is the etiology resolved?
clinical picture and IF help resolve etiology
In rapidly progressive glomerulonephritis, the immunofluorescence pattern of linear (anti-basement membrane antibody) refers to what disease?
Goodpasture syndrome
In rapidly progressive glomerulonephritis, the immunofluorescence pattern of linear (anti-basement membrane antibody), the antibody is against what?
Antibody against collagen in glomerular and alveolar basement membranes
In rapidly progressive glomerulonephritis, the immunofluorescence pattern of linear (anti-basement membrane antibody), how does is present?
as hematuria and hemoptysis, classically in young, adult males
In rapidly progressive glomerulonephritis, the immunofluorescence pattern of granular (immune complex deposition) refers to what disease?
PSGN (most common) or diffuse proliferative glomerulonephritis
What is diffuse proliterative glomerulonephritis due to?
diffuse antigen - antibody complex deposition, usually sub-endothelial; most common type of renal disease in SLE
In rapidly progressive glomerulonephritis, the immunofluorescence pattern of negative IF (pauci-immune) refers to what disease?
Wegener granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome
For the immunofluorescence pattern of negative IF (pacui-immune) what disease is associated with c-ANCA?
Wegener granulomatosis
For the immunofluorescence pattern of negative IF (pacui-immune) what disease is associated with p-ANCA?
microscopic polyangiitis and Churg-Strauss
For the immunofluorescence pattern of negative IF (pacui-immune) what distinguishes Churg-Strauss from microscopic polyangiitis?
Granulomatous inflammation, eosinophilia, and asthma
What is IgA nephropathy?
Berger disease - IgA immune complex deposition in mesangium of glomeruli; most common nephropathy worldwide
What is the most common nephropathy worldwide?
Berger disease ? IgA nephropathy
When does Berger disease present?
during childhood
In childhood what does Berger disease present as?
Presents as episodic gross or microscopic hematuria with RBC casts, usually following mucosal infections (eg. gastroenteritis)
In Berger disease what is increased during infection?
IgA production
In Berger disease what is seen on IF?
IgA immune complex deposition in the mesangium
What may Berger disease slowly progress to?
renal failure
What is alport syndrome?
Its an inherited defect in type IV collagen; most commonly X-linked
What does Alport Syndrome result in?
the thinning and splitting of the glomerular basement membrane
What does Alport syndrome presents as?
isolated hematuria, sensory hearing loss, and ocular disturbances
What is a urinary tract infection?
Infection of urethra, bladder, or kidney
A urinary tract infection most commonly arises from what?
due to ascending infection; increased incidence in females
What are the risk factors for urinary tract infection?
they include sexual intercourse, urinary stasis, and catheters,
What is cystitis?
Infection of the bladder
What does cystitis present as?
dysuria, urinary frequency, urgency, and suprapubic pain; systemic signs (e.g., fever) are usually absent.
What are the laboratory tests for cystitis?
1) Urinalysis 2) Dipstick 3) Culture
What are the laboratory findings for cystitis?
1) Urinalysis?cloudy urine with > 10 WBCs/high power field (hpf) 2) Dipstick?Positive leukocyte esterase (due to pyuria) and nitrites (bacteria convert nitrates to nitrites) 3) Culture?greater than 100,000 colony forming units (gold standard)
What is the etiology for cystitis?
1) E. coli (80%) 2) Staphylococcus saprophytics increased incidence in young, sexually active women (but E. coli is still more common in this population) 3) Klebsiella pneumoniae 4) Proteus mirabilis 5) Enterococcus faecalis
In cystitis, if there is alkaline urine with ammonia scent, what is the etiology?
Proteus mirabilis
In cystitis, what is sterile pyuria?
it is the presence of pyuria (> 10 WBCs/hpf and leukocyte esterase) with a negative urine culture.
In cystitis, what does sterile pyuria suggest and what is it due to?
urethritis due to Chlamydia trachomatis or Neisseria gonorrhoeae
What is the dominant presenting sign of urethritis?
it is dysuria)
What is pyelonephritis?
Infection of the kidney
What is pyelonephritis usually due to?
ascending infection; increased risk with vesicoureteral reflux
What does pyelonephritis present with?
fever, flank pain, WBC casts, and leukocytosis in addition to symptoms of cystitis
For pyelonephritis, what are the most common pathogens?
1) E coli (90%) 2) Klebsiella species 3) Enterococcus faecalis
What is chronic pyelonephritis?
Interstitial fibrosis and atrophy of tubules due to multiple bouts of acute pyelonephritis
What is chronic pyelonephritis due to?
vesicoureteral reflux (children) or obstruction (e.g., BPH or cervical carcinoma)
What does chronic pyelonephritis lead to?
cortical scarring with blunted calyces
In chronic pyelonephritis, what is characteristic of vesicoureteral reflux?
scarring at upper and lower poles
In chronic pyelonephritis, what is thyroidization of the kidney?s?
Atrophic tubules containing eosinophilic proteinaceous material resemble thyroid follicles
In chronic pyelonephritis, what may be seen in urine for thyroidization of the kidney?s?
waxy casts
What is nephrolithiasis?
Precipitation of a urinary solute as a stone
What are the risk factors for nephrolithiasis?
they include high concentration of solute in the urinary filtrate and low urine volume
What does nephrolithiasis present as?
colicky pain with hematuria and unilateral flank tenderness
In nephrolithiasis, what happens to the stone?
it is usually passed within hours; if not, surgical intervention may be required.
What is chronic renal failure?
End-stage kidney failure
What may chronic renal failure result from?
glomerular, tubular, inflammatory, or vascular insults
What are the most common causes of chronic renal failure?
diabetes mellitus, hypertension, and glomerular disease,
What are the clinical features for chronic renal failure?
1) uremia 2) salt and water retention with resultant hypertension 3) hyperkalemia with metabolic acidosis 4) anemia 5) hypocalcemia 6) renal osteodystrophy
What is asterixis?
Tremor of the hand when wrist is extended
In chronic renal failure, what happens in uremia?
increased nitrogenous waste products in blood (azotemia) result in nausea, anorexia, pericarditis, platelet dysfunction, encephalopathy with asterixis, and deposition of urea crystals in skin,
In chronic renal failure, what is anemia due to?
decreased erythropoietin production by renal peritubular interstitial cells
In chronic renal failure, what is hypocalcemia due to?
decreased l-alpha-hydroxylation of vitamin D by proximal renal tubule cells and hyperphosphatemia
In chronic renal failure, what is renal osteodystrophy due to?
secondary hyperparathyroidism, osteomalacia, and osteoporosis
In chronic renal failure, what is the treatment?
involves dialysis or renal transplant.
In chronic renal failure, what often develops during dialysis and what does this increase the risk for?
cysts within shrunken end-stage kidneys during dialysis increasing risk for renal cell carcinoma.
What is the composition for the most common type of nephrolithiasis?
Calcium oxalate and/ or calcium phosphate, usually seen in adults
What is the most common cause for the most common type of nephrolithiasis?
It is idiopathic
In what disease might the most common cause of nephrolithiasis be seen?
with Crohn disease
For the most common type of nephrolithiasis what is the treatment?
Hydrochlorothiazide (caicium-sparing diuretic).
For the second most common type of nephrolithiasis what is the composition?
Ammonium magnesium phosphate
What is the most common cause for the second most common type of nephrolithiasis?
Infection with urease-positive organisms (e.g Proteus vulgaris or Klebsiella);
For the second most common type of nephrolithiasis what leads to the formation of the stone?
alkaline urine
What does the second most common type of nephrolithiasis classically result in?
staghorn calculi in renal calyces, which act as a nidus for urinary tract infections
For the second most common type of nephrolithiasis what does treatment involve?
surgical removal of stone (due to size) and eradication of pathogen (to prevent recurrence)
What is the composition for the third most common type of nephrolithiasis?
Uric acid (5%)
How is the nephrolithiasis that is comoposed of uric acid different from the other stones?
Its radiolucent (as opposed to other types of stones which are radiopaque)
What are the risk factors for nephrolithisasis composed of uric acid?
they include hot, arid climates, low urine volume, and acidic pH.
What is the most common stone seen in patients with gout?
Nephrolithiasis composed of uric acid
For nephrolithiasis composed of uric acid what increases the risk?
hyperuricemia (e.g., in leukemia or myeloproliferative disorders)
For nephrolithisasis composed of uric acid, what does the treatment involve?
hydration and alkalinization of urine (potassium bicarbonate);
For the treatment of nephrolithiasis composed of uric acid, what is also given to patients with gout?
allopurinol is also administered in patients with gout (in addition to hydration and alkalinization of urine with potassium bicarbonate)
What form of nephrolithiasis is most commonly seen in children?
Cysteine, it is a rare cause of nephrolithiasis and is most commonly seen in children
What is associated with nephrolithiasis caused by cysteine?
cystinuria (a genetic defect of tubules that results in decreased reabsorption of cysteine)
Nephrolithiasis composed of cysteine may form what?
staghorn calculi
For nephrolithiasis composed of cysteine, what is involved in the treatment?
It involves hydration and alkalinization of urine
What are the types of renal neoplasia?
Angiomylipoma, renal cell carcinoma and wilms tumor
What is angiomyolipoma?
Hamartoma comprised of blood vessels, smooth muscle, and adipose tissue
In what is there an increased frequency of angiomyolipoma?
in tuberous sclerosis
What is renal cell carcinoma?
Malignant epithelial tumor arising from kidney tubules
How does renal cell carcinoma present?
with classic triad of hematuria, palpable mass, and flank pain, Fever, weight loss, or paraneoplastic syndrome (e.g EPO, renin, PTHrP, or ACTH) may also be present
For renal cell carcinoma what is the frequency of the symptoms?
All three symptoms rarely occur together; hematuria is the most common symptom.
For renal cell carcinoma what is the most common symptom?
Hematuria
What may renal cell carcinoma rarely present with?
left-sided varicocele
How might renal cell carcinoma lead to left sided varicocele?
Involvement of the left renal vein by carcinoma blocks drainage of the left spermatic vein leading to varicocele
For renal cell carcinoma why is right sided varicocele not seen?
Right spermatic vein drains directly into the IVC; hence, right-sided varicocele is not seen.
For renal cell carcinoma what does gross exam reveal?
a yellow mass,
For renal cell carcinoma what is seen microscopically for the most common variant?
It exhibits clear cytoplasm
For renal cell carcinoma what does the pathogenesis involve?
loss of VHL (3p) tumor suppressor gene, which leads to increased IGF-l (promotes growth) and increased HIF transcription factor (increases VEGF and PDGF).
For renal cell carcinoma are the tumors hereditary or sporadic?
May be either
For renal cell carcinoma when do sporadic tumors classically arise?
Classically in adult males (average age is 60 years) as a single tumor in the upper pole of the kidney
For renal cell carcinoma what is the major risk factor for sporadic tumors?
cigarette smoke
For renal cell carcinoma when do hereditary tumors arise?
in younger adults and are often bilateral,
What is Von Hippel-Lindau disease?
It is an autosomal dominant disorder
What is Von Hippel-Lindau disease associated with?
inactivation of the VHL gene leading to increased risk for hemangioblastoma of the cerebellum and renal cell carcinoma.
What is the staging for renal cell carcinoma?
1) T - based on size and involvement of the renal vein (occurs commonly and increases risk of hematogenous spread to the lungs and bone) 2) N - spread to retroperitoneal lymph nodes
What is Wilms tumor?
Malignant kidney tumor comprised of blastema (immature kidney mesenchyme) primitive glomeruli and tubules, and stromal cells
What is the most common malignant renal tumor in children?
Wilms tumor with an average age of 3 years
How does Wilm?s tumor present?
as a large, unilateral flank mass with hematuria and hypertension (due to renin secretion)
What is Wilm?s tumor associated with?
WT1 mutation, especially in syndromic cases
What is WAGR syndrome?
Wilms tumor, Aniridia, Genital abnormalities, and mental and motor Retardation
What is Beckwith-Wiedemann syndrome?
Wilms tumor, neonatal hypoglycemia, muscular hemihypertrophy, and organomegaly (including tongue)
What are the lower urinary tract carcinomas?
1) Urothelial (transitional cell) carcinoma 2) squamous cell carcinoma 3) adenocarcinoma
What is urothelial (transitional cell) carcinoma?
Malignant tumor arising from the urothelial lining of the renal pelvis, ureter, bladder, or urethra
What is the most common type of lower urinary tract cancer?
Urothelial (transitional cell) carcinoma
Where does urothelial (transitional cell) carcinoma?
it usually arises in the bladder
For urothelial (transitional cell) carcinoma what are the major risk factors?
it is cigarette smoke; additional risk factors are naphthylamine, azo dyes, and long-term cyclophosphamide or phenacetin use
In whom is urothelial (transitional cell) carcinoma generally seen?
in older adults
How does urothelial (transitional cell) carcinoma classically present?
with painless hematuria
How does urothelial (transitional cell) carcinoma arise?
via two distinct pathways; flat and papillary
How does flat urothelial (transitional cell) carcinoma develop and what is it associated with?
as a high-grade flat tumor and then invades; associated with early p53 mutations
How does papillary urothelial (transitional cell) carcinoma develop?
as a low-grade papillary tumor that progresses to a high grade papillary tumor and then invades; not associated with early p53 mutations
Describe the tumors for urothelial (transitional cell) carcinoma?
Tumors are often multifocal and recur (field defect)
What is squamous cell carcinoma?
Malignant proliferation of squamous cells, usually involving the bladder
When does squamous cell carcinoma arise?
It arises in a background of squamous metaplasia (normal bladder surface is not lined by squamous epithelium)
What are the risk factors for squamous cell carcinoma?
They include chronic cystitis (older woman). Schistosoma hematobium infection (Egyptian male), and long-standing nephrolithiasis.
What is adenocarcinoma?
Malignant proliferation of glands, usually involving bladder
What does adenocarcinoma arise from?
a urachal remnant (tumor develops at the dome of the bladder), cystitis glandularis, or exstrophy (congenital failure to form the caudal portion of the anterior abdominal and bladder walls)
What is exstrophy related to adenocarcinoma?
congenital failure to form the caudal portion of the anterior abdominal and bladder walls
