CH12 - Kidney and Urinary Tract Pathology Flashcards Preview

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Flashcards in CH12 - Kidney and Urinary Tract Pathology Deck (238)
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1

What is the most common congenital renal anomaly?

Horseshoe kidney - Conjoined kidneys usually connected at the lower pole

2

In horseshoe kidney where is it located?

Kidney is abnormally located in the lower abdomen

3

What happens in horseshoe kidney?

The kidney gets caught on the inferior mesenteric artery root during its ascent from the pelvis to the abdomen

4

What is renal agenesis?

Absent kidney formation; may be unilateral or bilateral

5

What does unilateral renal agenesis lead to?

hypertrophy of the existing kidney; hyperfiltration increases risk of renal failure later in life.

6

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

7

What is dysplastic kidney?

Non-inherited, congenital malformation of the renal parenchyma characterized by cysts and abnormal tissue (e.g., cartilage)

8

Is dysplastic kidney unilateral or bilateral?

Usually unilateral; when bilateral, must be distinguished from inherited polycystic kidney disease

9

What is polycystic kidney disease?

Inherited defect leading to bilateral enlarged kidneys with cysts in the renal cortex and medulla

10

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

11

What is the autosomal recessive form of polycystic kidney disease associated with?

congenital hepatic fibrosis (leads to portal hypertension) and hepatic cysts

12

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

13

What is the autosomal dominant form of polycystic kidney disease due to?

A mutation in the APKD1 or APKD2 gene; cysts develop over time.

14

What is the autosomal dominant form of polycystic kidney disease associated with?

berry aneurysm, hepatic cysts, and mitral valve prolapse

15

What is medullary cystic kidney disease?

Inherited (autosomal dominant) defect leading to cysts in the medullary collecting ducts

16

What does parenchymal fibrosis result in?

shrunken kidneys and worsening renal failure

17

What is acute renal failure?

Acute, severe decrease in renal function (develops within days)

18

What is the hallmark of acute renal failure?

Azotemia, (increased BUN and creatinine) often with oliguria

19

What is acute renal failure divided into?

Its divided into prerenal, postrenal, and intrarenal azotemia and is based on etiology

20

What is prerenal azotemia?

Due to decreased blood flow to kidneys (e.g., cardiac failure)

21

Prerenal azotemia is a common cause of what?

ARF (acute renal failure)

22

What does decreased blood flow result in?

decreased GFR, azotemia, and oliguria.

23

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)

24

What is postrenal azotemia due to?

obstruction of urinary tract downstream from the kidney (e.g., ureters)

25

In postrenal azotemia what does the decreased outflow result in?

decreased GFR, azotemia, and oliguria

26

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).

27

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)

28

What is acute tubular necrosis?

It is injury and necrosis of tubular epithelial cells

29

What is the most common cause of acute renal failure?

Acute tubular necrosis (intrarenal azotemia)

30

In acute tubular necrosis, what happens?

Necrotic cells plug tubules; obstruction decreases GFR

31

In acute tubular necrosis, what is seen in the urine?

Brown, granular casts are seen in the urine

32

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).

33

What is the etiology for acute tubular necrosis?

may be ischemic or nephrotoxic,

34

What happens with ischemia and acute tubular necrosis?

Decreased blood supply results in necrosis of tubules.

35

With ischemia causing acute tubular necrosis, what is it often preceded by?

prerenal azotemia

36

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

37

What is another cause, aside from ischemia that results in acute tubular necrosis?

its nephrotoxic and toxic agents result in necrosis of tubules

38

In nephrotoxic damage leading to acute tubular necrosis, what parts of the nephron are particularly susceptible to damage?

Proximal tubule

39

What is the most common cause of nephrotoxic damage leading to acute tubular necrosis?

aminoglycosides

40

In nephrotoxic damage leading to acute tubular necrosis, what are the causes?

They include aminoglycosides, heavy metals, myoglobinuria, ethylene glycol, radiocontrast dye, and urate

41

What is an example of heavy metals leading to acute tubular necrosis?

lead

42

What causes myoglobinuria leading to acute tubular necrosis?

and example is from crush injury to muscle

43

What is ethylene glycol leading to acute tubular necrosis associated with?

oxalate crystals in urine

44

What is an example of urate leading to acute tubular necrosis?

tumor lysis syndrome

45

In nephrotoxicity leading to acute tubular necrosis what are used prior to initiation of chemotherapy?

Hydration and allopurinol

46

Why is hydration and allopurinol used prior to initiation of chemotherapy?

to decrease risk of urate-induced ATN.

47

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

48

Why is there hyperkalemia in acute tubular necrosis?

its due to decreased renal excretion

49

Is acute tubular necrosis reversible?

Yes, but often requires supportive dialysis since electrolyte imbalances can be fatal

50

In acute tubular necrosis, for how long can oliguria persist?

for 2 -3 weeks before recovery

51

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

52

What is acute interstitial nephritis?

Drug-induced hypersensitivity involving the interstitium and tubules

53

What does acute interstitial nephritis result in?

acute renal failure (intrarenal azotemia)

54

What are the causes of acute interstitial nephritis?

They include NSAIDs, penicillin, and diuretics

55

What does acute interstitial nephritis present as?

oliguria, fever, and rash days to weeks after starting a drug; eosinophils may be seen in urine.

56

How does acute interstitial nephritis resolve?

with cessation of drug

57

What may acute interstitial nephritis progress to?

renal papillary necrosis

58

What is renal papillary necrosis?

Necrosis of renal papillae

59

What does renal papillary necrosis present with?

gross hematuria and flank pain

60

What are the causes for renal papillary necrosis?

1. Chronic analgesic abuse (e.g., long-term phenacetin or aspirin use) 2. Diabetes mellitus 3. Sickle cell trait or disease 4. Severe acute pyelonephritis

61

What is nephrotic syndrome?

Glomerular disorders characterized by proteinuria (> 3.5 g/day)

62

What does nephrotic syndrome result in?

1. Hypoalbuminemia 2. Hypogammaglobulinemia 3. Hypercoagulable state 4. Hvperlipidemia and hypercholesterolemia

63

In nephrotic syndrome, what is involved with hypoalbuminemia being the cause?

pitting edema

64

In nephrotic syndrome caused y hypogammaglobulinemia, what is there an increased risk of?

infection

65

In nephrotic syndrome caused by a hypercoagulable state, what is that state due to?

loss of antithrombin III

66

With hyperlipidemia and hypercholesterolemia causing nephrotic syndrome, what may result?

This may result in fatty casts in urine

67

What is the most common cause of nephrotic syndrome in children?

Minimal change disease (MCD)

68

What is the etiology for MCD?

For minimal change disease, it is usually idiopathic and may be associated with Hodgkin lymphoma

69

How does MCD appear on H&E stain?

Normal glomeruli on H&E stain, lipid may be seen in proximal tubule cells

70

In MCD, what is seen on electron microscopy?

effacement of foot processes

71

In MCD what is seen on immunofluorescence and why?

negative immunofluorescence (IF) because there are no immune complex deposits

72

In MCD, what happens in terms of proteinuria?

There is selective proteinuria where there is loss of albumin, but not immunoglobulin

73

What is the treatment for MCD?

It has an excellent response to steroids

74

How is the damage in MCD mediated?

damage is mediated by cytokines from T cells

75

What is the most common cause of nephrotic syndrome in Hispanics and African Americans?

Focal segmental glomerulosclerosis (FSGS)

76

What is the etiology for focal segmental glomerulosclerosis?

It is usually idiopathic, maybe associated with HIV, heroin use, and sickle cell disease

77

What is focal segmental glomerulosclerosis?

Focal (some glomeruli) and segmental (involving only part of the glomerulus) sclerosis on H&E stain

78

What is seen on electron microscopy in FSGS?

In focal segmental glomerulosclerosis, there is effacement of foot processes on EM

79

For focal segmental glomerulosclerosis, what is seen on IF?

Negative immunofluorescence due to no immune complex deposits

80

How does FSGS respond to steroids and what may it progress to?

Poor response to steroids and may progress to chronic renal failure

81

What is the most common cause of nephrotic syndrome in Caucasian adults?

Membranous nephropathy

82

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)

83

For membranous nephropathy, what is seen on H&E stain?

Thick glomerular basement membrane

84

What is the appearance of membranous nephropathy on electron microscopy?

subepithelial deposits with spike and dome appearance on EM

85

For membranous nephropathy, what is its appearance on EM due to?

immune complex deposition

86

How does membranous nephropathy respond to steroids and what might it progress to?

Poor response to steroids; progresses to chronic renal failure

87

What is membranoproliferative glomerulonephritis?

Thick glomerular basement membrane on H&E stain, often with tram-track appearance

88

What is membranoproliferative glomerulonephritis due to?

immune complex deposition (granular IF)

89

What is membranoproliferative glomerulonephritis divided into?

two types based on location of deposits

90

What is membranoproliferative glomerulonephritis - Type I?

subendothelial associated with HBV and HCV

91

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)

92

How does membranoproliferative glomerulonephritis respond to steroids and what might it progress to?

Poor response to steroids; progresses to chronic renal failure

93

How is diabetes mellitus related to nephrotic syndrome?

high serum glucose leads to nonenzymatic glycosylation of the vascular basement membrane resulting in hyaline arteriolosclerosis.

94

With diabetes mellitus leading to nephrotic syndrome what happens to the glomerular filtration pressure?

There is high glomerular filtration pressure

95

With diabetes mellitus leading to nephrotic syndrome why is there high glomerular filtration pressure?

glomerular efferent arteriole is more affected than the afferent arteriole

96

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

97

What does diabetes mellitus relating to kidney?s eventually progress to?

nephrotic syndrome

98

What is diabetes mellitus leading to nephrotic syndrome characterized by?

sclerosis of the mesangium with formation of Kimmelstiel-Wilson nodules

99

In diabetes mellitus leading to nephrotic syndrome what slows the progression of hyperfiltration-induced damage?

ACE inhibitors

100

What is the most commonly involved organ in systemic amyloidosis?

The kidney

101

How does systemic amyloidosis lead to nephrotic syndrome?

Amyloid deposits in the mesangium, resulting in nephrotic syndrome

102

How is systemic amyloidosis characterized?

by apple-green birefringence under polarized light after staining with Congo red

103

What is nephritic syndrome?

Glomerular disorders characterized by glomerular inflammation and bleeding

104

What is seen in nephritic syndrome?

1. 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

105

In nephritic syndrome what is seen in biopsy?

it reveals hypercellular, inflamed glomeruli

106

In nephritic syndrome, what mediates damage?

Immune-complex deposition activates complement; C5a attracts neutrophils, which mediate damage,

107

What is poststreptococcal glomerulonephritis (PSGN)?

Nephritic syndrome that arises alter group A beta-hemolytic streptococcal infection of the skin (impetigo) or pharynx

108

In PSGN, what does it occur with?

nephritogenic strains (which carry the M protein virulence factor)

109

PSGN may occur after infection with what organisms?

nonstreptococcal organisms as well as nephritogenic strains

110

How does PSGN present?

2-3 weeks after infection as hematuria (cola-colored urine), oliguria, hypertension, and periorbital edema

111

PSGN is usually seen in whom?

children, but may occur in adults

112

In PSGN what is seen on H&E stain?

Its hypercellular, inflamed glomeruli on H&E

113

In PSGN, what is seen on electron microscopy?

subepithelial humps on EM, and is mediated by immune complex deposition - granular IF

114

What is the treatment for PSGN?

Treatment is supportive.

115

What is the chance for renal failure in PSGN?

Children rarely (1%) progress to renal failure

116

In PSGN, what might some adults develop?

25% might develop rapidly progressive glomerulonephritis (RPGN)

117

What is rapidly progressive glomerulonephritis?

Nephritic syndrome that progresses to renal failure in weeks to months

118

What is rapidly progressive glomerulonephritis characterized by?

crescents in Bowman space (of glomeruli) on H&E stain; crescents are comprised of fibrin and macrophages

119

For rapidly progressive glomerulonephritis how is the etiology resolved?

clinical picture and IF help resolve etiology

120

In rapidly progressive glomerulonephritis, the immunofluorescence pattern of linear (anti-basement membrane antibody) refers to what disease?

Goodpasture syndrome

121

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

122

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

123

In rapidly progressive glomerulonephritis, the immunofluorescence pattern of granular (immune complex deposition) refers to what disease?

PSGN (most common) or diffuse proliferative glomerulonephritis

124

What is diffuse proliterative glomerulonephritis due to?

diffuse antigen - antibody complex deposition, usually sub-endothelial; most common type of renal disease in SLE

125

In rapidly progressive glomerulonephritis, the immunofluorescence pattern of negative IF (pauci-immune) refers to what disease?

Wegener granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome

126

For the immunofluorescence pattern of negative IF (pacui-immune) what disease is associated with c-ANCA?

Wegener granulomatosis

127

For the immunofluorescence pattern of negative IF (pacui-immune) what disease is associated with p-ANCA?

microscopic polyangiitis and Churg-Strauss

128

For the immunofluorescence pattern of negative IF (pacui-immune) what distinguishes Churg-Strauss from microscopic polyangiitis?

Granulomatous inflammation, eosinophilia, and asthma

129

What is IgA nephropathy?

Berger disease - IgA immune complex deposition in mesangium of glomeruli; most common nephropathy worldwide

130

What is the most common nephropathy worldwide?

Berger disease ? IgA nephropathy

131

When does Berger disease present?

during childhood

132

In childhood what does Berger disease present as?

Presents as episodic gross or microscopic hematuria with RBC casts, usually following mucosal infections (eg. gastroenteritis)

133

In Berger disease what is increased during infection?

IgA production

134

In Berger disease what is seen on IF?

IgA immune complex deposition in the mesangium

135

What may Berger disease slowly progress to?

renal failure

136

What is alport syndrome?

Its an inherited defect in type IV collagen; most commonly X-linked

137

What does Alport Syndrome result in?

the thinning and splitting of the glomerular basement membrane

138

What does Alport syndrome presents as?

isolated hematuria, sensory hearing loss, and ocular disturbances

139

What is a urinary tract infection?

Infection of urethra, bladder, or kidney

140

A urinary tract infection most commonly arises from what?

due to ascending infection; increased incidence in females

141

What are the risk factors for urinary tract infection?

they include sexual intercourse, urinary stasis, and catheters,

142

What is cystitis?

Infection of the bladder

143

What does cystitis present as?

dysuria, urinary frequency, urgency, and suprapubic pain; systemic signs (e.g., fever) are usually absent.

144

What are the laboratory tests for cystitis?

1) Urinalysis 2) Dipstick 3) Culture

145

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)

146

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

147

In cystitis, if there is alkaline urine with ammonia scent, what is the etiology?

Proteus mirabilis

148

In cystitis, what is sterile pyuria?

it is the presence of pyuria (> 10 WBCs/hpf and leukocyte esterase) with a negative urine culture.

149

In cystitis, what does sterile pyuria suggest and what is it due to?

urethritis due to Chlamydia trachomatis or Neisseria gonorrhoeae

150

What is the dominant presenting sign of urethritis?

it is dysuria)

151

What is pyelonephritis?

Infection of the kidney

152

What is pyelonephritis usually due to?

ascending infection; increased risk with vesicoureteral reflux

153

What does pyelonephritis present with?

fever, flank pain, WBC casts, and leukocytosis in addition to symptoms of cystitis

154

For pyelonephritis, what are the most common pathogens?

1) E coli (90%) 2) Klebsiella species 3) Enterococcus faecalis

155

What is chronic pyelonephritis?

Interstitial fibrosis and atrophy of tubules due to multiple bouts of acute pyelonephritis

156

What is chronic pyelonephritis due to?

vesicoureteral reflux (children) or obstruction (e.g., BPH or cervical carcinoma)

157

What does chronic pyelonephritis lead to?

cortical scarring with blunted calyces

158

In chronic pyelonephritis, what is characteristic of vesicoureteral reflux?

scarring at upper and lower poles

159

In chronic pyelonephritis, what is thyroidization of the kidney?s?

Atrophic tubules containing eosinophilic proteinaceous material resemble thyroid follicles

160

In chronic pyelonephritis, what may be seen in urine for thyroidization of the kidney?s?

waxy casts

161

What is nephrolithiasis?

Precipitation of a urinary solute as a stone

162

What are the risk factors for nephrolithiasis?

they include high concentration of solute in the urinary filtrate and low urine volume

163

What does nephrolithiasis present as?

colicky pain with hematuria and unilateral flank tenderness

164

In nephrolithiasis, what happens to the stone?

it is usually passed within hours; if not, surgical intervention may be required.

165

What is chronic renal failure?

End-stage kidney failure

166

What may chronic renal failure result from?

glomerular, tubular, inflammatory, or vascular insults

167

What are the most common causes of chronic renal failure?

diabetes mellitus, hypertension, and glomerular disease,

168

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

169

What is asterixis?

Tremor of the hand when wrist is extended

170

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,

171

In chronic renal failure, what is anemia due to?

decreased erythropoietin production by renal peritubular interstitial cells

172

In chronic renal failure, what is hypocalcemia due to?

decreased l-alpha-hydroxylation of vitamin D by proximal renal tubule cells and hyperphosphatemia

173

In chronic renal failure, what is renal osteodystrophy due to?

secondary hyperparathyroidism, osteomalacia, and osteoporosis

174

In chronic renal failure, what is the treatment?

involves dialysis or renal transplant.

175

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.

176

What is the composition for the most common type of nephrolithiasis?

Calcium oxalate and/ or calcium phosphate, usually seen in adults

177

What is the most common cause for the most common type of nephrolithiasis?

It is idiopathic

178

In what disease might the most common cause of nephrolithiasis be seen?

with Crohn disease

179

For the most common type of nephrolithiasis what is the treatment?

Hydrochlorothiazide (caicium-sparing diuretic).

180

For the second most common type of nephrolithiasis what is the composition?

Ammonium magnesium phosphate

181

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);

182

For the second most common type of nephrolithiasis what leads to the formation of the stone?

alkaline urine

183

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

184

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)

185

What is the composition for the third most common type of nephrolithiasis?

Uric acid (5%)

186

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)

187

What are the risk factors for nephrolithisasis composed of uric acid?

they include hot, arid climates, low urine volume, and acidic pH.

188

What is the most common stone seen in patients with gout?

Nephrolithiasis composed of uric acid

189

For nephrolithiasis composed of uric acid what increases the risk?

hyperuricemia (e.g., in leukemia or myeloproliferative disorders)

190

For nephrolithisasis composed of uric acid, what does the treatment involve?

hydration and alkalinization of urine (potassium bicarbonate);

191

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)

192

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

193

What is associated with nephrolithiasis caused by cysteine?

cystinuria (a genetic defect of tubules that results in decreased reabsorption of cysteine)

194

Nephrolithiasis composed of cysteine may form what?

staghorn calculi

195

For nephrolithiasis composed of cysteine, what is involved in the treatment?

It involves hydration and alkalinization of urine

196

What are the types of renal neoplasia?

Angiomylipoma, renal cell carcinoma and wilms tumor

197

What is angiomyolipoma?

Hamartoma comprised of blood vessels, smooth muscle, and adipose tissue

198

In what is there an increased frequency of angiomyolipoma?

in tuberous sclerosis

199

What is renal cell carcinoma?

Malignant epithelial tumor arising from kidney tubules

200

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

201

For renal cell carcinoma what is the frequency of the symptoms?

All three symptoms rarely occur together; hematuria is the most common symptom.

202

For renal cell carcinoma what is the most common symptom?

Hematuria

203

What may renal cell carcinoma rarely present with?

left-sided varicocele

204

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

205

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.

206

For renal cell carcinoma what does gross exam reveal?

a yellow mass,

207

For renal cell carcinoma what is seen microscopically for the most common variant?

It exhibits clear cytoplasm

208

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).

209

For renal cell carcinoma are the tumors hereditary or sporadic?

May be either

210

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

211

For renal cell carcinoma what is the major risk factor for sporadic tumors?

cigarette smoke

212

For renal cell carcinoma when do hereditary tumors arise?

in younger adults and are often bilateral,

213

What is Von Hippel-Lindau disease?

It is an autosomal dominant disorder

214

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.

215

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

216

What is Wilms tumor?

Malignant kidney tumor comprised of blastema (immature kidney mesenchyme) primitive glomeruli and tubules, and stromal cells

217

What is the most common malignant renal tumor in children?

Wilms tumor with an average age of 3 years

218

How does Wilm?s tumor present?

as a large, unilateral flank mass with hematuria and hypertension (due to renin secretion)

219

What is Wilm?s tumor associated with?

WT1 mutation, especially in syndromic cases

220

What is WAGR syndrome?

Wilms tumor, Aniridia, Genital abnormalities, and mental and motor Retardation

221

What is Beckwith-Wiedemann syndrome?

Wilms tumor, neonatal hypoglycemia, muscular hemihypertrophy, and organomegaly (including tongue)

222

What are the lower urinary tract carcinomas?

1) Urothelial (transitional cell) carcinoma 2) squamous cell carcinoma 3) adenocarcinoma

223

What is urothelial (transitional cell) carcinoma?

Malignant tumor arising from the urothelial lining of the renal pelvis, ureter, bladder, or urethra

224

What is the most common type of lower urinary tract cancer?

Urothelial (transitional cell) carcinoma

225

Where does urothelial (transitional cell) carcinoma?

it usually arises in the bladder

226

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

227

In whom is urothelial (transitional cell) carcinoma generally seen?

in older adults

228

How does urothelial (transitional cell) carcinoma classically present?

with painless hematuria

229

How does urothelial (transitional cell) carcinoma arise?

via two distinct pathways; flat and papillary

230

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

231

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

232

Describe the tumors for urothelial (transitional cell) carcinoma?

Tumors are often multifocal and recur (field defect)

233

What is squamous cell carcinoma?

Malignant proliferation of squamous cells, usually involving the bladder

234

When does squamous cell carcinoma arise?

It arises in a background of squamous metaplasia (normal bladder surface is not lined by squamous epithelium)

235

What are the risk factors for squamous cell carcinoma?

They include chronic cystitis (older woman). Schistosoma hematobium infection (Egyptian male), and long-standing nephrolithiasis.

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What is adenocarcinoma?

Malignant proliferation of glands, usually involving bladder

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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)

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What is exstrophy related to adenocarcinoma?

congenital failure to form the caudal portion of the anterior abdominal and bladder walls