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CPR II Exam #2 > Renal Pathology III > Flashcards

Renal Pathology III Flashcards

1
Q

Can someone live with unilateral renal agenesis?

What change occurs in the sole kidney?

Rarely, some patients may develop…

A

It can be compatible with life if no other abnormality exists.

The sole kidney will hypertrophy.

Some patients may develop progressive glomerular sclerosis in the sole kidney.

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2
Q

Can someone live with bilateral renal agenesis?

A

No, it is not compatible with life.

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3
Q

Renal hypoplasia typically occurs only…

A

Unilaterally. It is very rare.

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4
Q

Ectopic kidney(s) can develop…

What location is most common?

Associated ureteral abnormalities may be associated with…

A

Anywhere along the path of the ureter, not at the usualy T10-L2 retroperitoneal location.

Most common location is just above the pelvic brim or within the pelvis.

Infection and obstruction.

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5
Q

Horseshoe kidney usually affects which pole?

A

Upper poles - 90%

Lower poles - 10%

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6
Q

ADPKD

Genetics:

Pathological features:

Clinical features:

Typical outcome:

Kidney size:

A

Genetics: AD

Pathological features: large multicystic kidneys, liver cysts and aneurysms.

Clinical features: Hematuria, flank pain, UTI, stones, HTN.

Typical outcome: CRF beginning at 40-60 y/o.

Kidney size: Large

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7
Q

Adult-onset Nephrolithiasis

Genetics:

Pathological features:

Clinical features:

Typical outcome:

Kidney size:

A

Genetics: AD

Pathological features: Corticomedullary cysts, shrunken kidneys.

Clinical features: Salt wasting, polyuria.

Typical outcome: Progressive RF onset in adulthood.

Kidney size: Small

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8
Q

Familial Juvenile Nephrolithiasis

Genetics:

Pathological features:

Clinical features:

Typical outcome:

Kidney size:

A

Genetics: AR

Pathological features: Corticomedullary cysts, shrunken kidneys.

Clinical features: Salt wasting, polyuria, growth retardation.

Typical outcome: Progressive RF onset in childhood.

Kidney size: Small

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9
Q

Childhood Polycystic Kidney Disease

Genetics:

Pathological features:

Clinical features:

Typical outcome:

Kidney size:

A

Genetics: AR

Pathological features: Enlarged cystic kidneys at birth.

Clinical features: Hepatic fibrosis.

Typical outcome: Variable, but death in infancy or childhood.

Kidney size: Large

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10
Q

What occurs to the kidney in ADPKD?

A

Multiple expanding cysts destroys the renal parenchyma and leads to RF.

The cysts eventually replace all of the functional cortex, but some scattered remnants of nephrons can be seen.

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11
Q

ADPKD is always…

A

BL

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12
Q

What population is most likely to develop ADPKD?

Manifestation of ADPKD requires…

A

Northern Europeans

Requires mutation of both alleles of either PKD gene

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13
Q

85% of ADPKD have what defective gene? On what chromosome?

What does this gene code for?

A

PKD1 gene, on chr. 16p13.3

Polycystin-1, an integral membrane glycoprotein. It is believed to have a role in cell-cell or cell-matrix interactions.

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14
Q

15% of ADPKD have what defective gene? On what chromosome?

What does this gene code for?

A

PKD2 gene, on chr. 4q13-p23

Polycystin-2, a Ca++ cation channel

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15
Q

Which genetic profile has a better prognosis in ADPKD?

A

Patients with PKD2 mutations

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16
Q

Presentation of ADPKD occurs at what age?

What are the symptoms?

Which patients may have a more aggressive course?

A

4-6th decades.

Renal insufficiency (HTN, azotemia), abdominal pain, possible hematuria.

AAs (sickle-cell correlation), males, patients with concomitant HTN.

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17
Q

Extra-renal manifestations of ADPKD include… (4)

A

40% have hepatic cysts
4-10% die from subarachnoid hemorrhage due to berry aneurysms
25% have MV prolapse
82% have diverticular disease of the colon

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18
Q

Gross appearance of kidneys in ARPKD

A

Slightly enlarged with many small linear/radial-arrayed cysts derived from dilated collecting ducts.

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19
Q

Several major clinical subtypes exist in ARPKD, but 2 are of main concern:

What are the main features of both subtypes?

A

Perinatal: >90% of CDs are cystic; minimal hepatic fibrosis. Babies live for only a few hours, and death is due to hypoplastic lungs, usually.

Neonatal: about 60% of CDs are cystic; mild hepatic fibrosis. Babies live for a few months, but then die fron renal failure.

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20
Q

2 major Medullary Cystic Diseases

A

Medullary sponge kidney: benign findings on imaging.

Nephronophthisi cystic disease (medullary cystic disease): cysts localized to corticomedullary junction and medulla.

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21
Q

Which disorders are Acquired Renal Cystic diseases?

A

Simple cysts
Acquired renal cystic disease
Unilateral multicystic renal dysplasia

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22
Q

Acquired renal cystic disease

Morphology:

Clinical complications:

Typical outcome:

A

Morphology: cystic degenration in end-stage disease.

Clinical complications: Hemorrhage, erthrocytosis, neoplasia.

Typical outcome: Dialysis dependent.

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23
Q

Unilateral multicystic renal dysplasia

Morphology:

Clinical complications:

Typical outcome:

A

Morphology: Multiple large cysts and cartilage.

Clinical complications: Abdominal mass

Typical outcome: Normal life expectancy

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24
Q

Where do simple cysts usually occur? Filled with?

A

On the renal cortical surface and are clear fluid-filled

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25
Q

What are acquired renal cysts associated with?

A

RCC, and 7% of patients will need dialysis for more than 10 yrs.

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26
Q

Multicystic Renal Dysplasia

Most cases have other abnormalities, like…

What does it look like on pathology?

A

Ureteral agenesis, uretopelvic abstruction and/or other GU abnormalities.

Variably-sized cysts with intervening mesenchyme, often with cartilage formation and immature CDs.
It appears very disorganized on staining.

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27
Q

Nephrolithiasis is usually…

Who gets it more? (M or F?)

When is the peak age of onset?

A

UL in 80%

Men get it more

Peak age is in 3rd decade (20-30 y/o)

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28
Q

When do patients with nephrolithiasis develop symptoms?

A

Once the stone enters the ureters and causes renal colic and ulceration of the ureteral mucosa and obstruction.

29
Q

Most common stone type that develops nephrolithiasis?

Avg. stone size:

A

Calcium oxalate and phosphate (70%) - idiopathic hypercalcemia is main cause

0.2-0.3 cm

30
Q

What are the predisposing conditions to developing a kidney stone? (4)

A

Increased stone constituent conc.
Changes in urinary pH
Low urine volume
Presence of bacteria

31
Q

Renal Papillary Adenoma is the most common…

What is it defined by? Can they become malignant?

A

Benign kidney neoplasm.

Defined by size (must be <1 cm). They are capable of malignancy.

32
Q

How does Renal Papillary Adenoma look grossly?

A

Cortical, discrete, yellow-gray and multiple

33
Q

What does Renal Papillary Adenoma look like on histology? (3)

A

Very similar to RCC

  • acidophilic cytoplasm
  • papillae (maybe Psammoma bodies)
  • thin fibrovascular cores
34
Q

Renal Oncocytoma represents how many cases of primary renal epithelial neoplasms?

What cell type makes it up?

A

5-15%

Arises from type A intercalated cell of cortical CDs with high degree of mitochondria

35
Q

When does Renal Oncocytoma present?

How often does it metastasize/invade?

What does it look like on histology?

What does it look like grossly?

A

Presents in adulthood

Rarely invades/mets

Abundant acidophilia, granular cytoplasm, packed w/ mitochondria

Grossly, mahogany-brown with central stellate star

36
Q

Renal Oncocytoma closely resembles…

A

Chromophobe variant of RCC

37
Q

Renal Angiomyolipoma has a strong association with…

Who is most likely to get it?

Loss of what genes is linked?

A

Tuberous sclerosis

Middle-aged adults, F > M

Loss of TSC1 or TSC2 with AD inheritance

38
Q

What is the clinical significance of Renal Angiomyolipoma?

How does it look on histology?

A

It should be on a DDx with RCC; occasionally it may rupture with massive hemorrhage

39
Q

When do patients develop RCC? Who gets it more frequently, males or females?

A

6th-8th decades, men > women

40
Q

Risk factors associated with RCC

A 
Smoking (2x risk)
HTN
Obesity
Estrogens
Asbestos
CRD
Tuberous sclerosis
Acquired cystic disease
41
Q

Most instances of RCC are… but…

A

Sporadic, but 4% are hereditary with an AD pattern and happens in younger patients

42
Q

3 subtypes of RCC

A

CCC
Papillary carcinoma
Chromophobe renal carcinoma

43
Q

Clear cell carcinoma makes up what percentage of RCC?

How does it look on histology?

Is it sporadic, inherited, etc.?

A

70-80%

Clear cytoplasm

95% sporadic, but of the familial, 98% have a chr. 3 short arm translocation/deletions (VHL tumor suppressor gene)

44
Q

Papillary carcinoma makes up what percentage of RCC?

What genetic alterations is it associated with?

A

10-15%

Trisomy 7, 16, 17; lost Y (MET proto-oncogenes)

45
Q

How do Chromophobe renal carcinomas look on histology?

Where is it concentrated?

What do they arise from?

A

Pale eosinophilia, nuclear halos.

Near BVs.

Type B intercalated cells of the renal cortex CDs.

46
Q

What symptoms occur in RCC?

A

Classic triad: hematuria (50%), costovertebral pain (20%), palpable flank mass (10%)

47
Q

What makes treating RCC difficult?

A

Tends to reach large size and metastasize before local signs/symptoms appear, then it presents with generalized complaints.
25% have mets at initial Dx.

48
Q

How is RCC spread?

A

Hematologenous spread via the renal v.

49
Q

What is the histological appearance of CCC?

A

Clear cytoplasm with sharply delineated CM.

Clear cytoplasm is from glycogen and lipid accumulation - “hypernephroma”.

50
Q

What is the histological appearance of Papillary carcinoma?

A

Papillae and foamy macrophages in stalk

51
Q

Which RCCs have the best prognosis? Worst?

A

Best: Chromophobe renal carcinoma

Worst: Sarcomatoid and CD carcinomas

52
Q

What is Sarcomatoid RCC composed of?

A

Spindle cells stimulating a mesenchymal neoplasm.

It can arise in any RCC and indicates a poor prognosis.

53
Q

What is CD carcinoma composed of?

A

Branching tubules lined by atypical cuboidal cells. Suggests poor prognosis.

54
Q

Urothelial Carcinoma of the Kidney begins where?

What other tumor is it associated with?

What disorders is it associated with?

A

In the urothelium of the renal pelvis.

Bladder tumors.

Analgesic and Balkan (tubule-interstitial) nephropathy.

55
Q

Symptoms of Urothelial Carcinoma (3)

Where can it infiltrate?

Prognosis?

A

Hematuria
Blockage of urinary outflow
Flank pain

Infiltrate into pelvis/calyces is common

Poor prognosis

56
Q

Wilms tumor is most common in which race?

A

Asian > White > Black

57
Q

When do Wilms tumors present?

Presenting SX: (4)

UL or BL?

A

Ages 2-5 y/o

Abdominal mass, with pain, microscopic hematuria and HTN

Most are UL, but 5% are BL

58
Q

WAGR syndrome

A

Undescended testes in males and streak ovaries in females. 33-40% have a Wilms tumor.

59
Q

Denys-Drash syndrome

A

Gonadal and renal tumors. 90% have Wilms tumor.

60
Q

Beckwith-Wiedemann syndrome

A

Hemihypertrophy and macroglossia

61
Q

What is the typical precursor lesion of a Wims tumor?

A

Nephrogenic rests

62
Q

What is Triphasic histomorphology?

A

It is favorable in a Wilms tumor.

Mimics germinal development of a normal kidney with 3 cell types: blastemal, epithelial, and stromal cells.

No significant anaplasia.

63
Q

What is Anaplastic histomorphology?

What is it associated with?

A

It is unfavorable in a Wilms tumor.

Focal or diffuse pleomorphism and atypia (focal not always bad, but diffuse is).

p53 mutations and resistance to chemotherapy.

64
Q

What genetic alteration suggests a worse prognosis in a Wilms tumor?

A

1q gain

65
Q

Prognosis of a Wilms tumor:

Which patients have a better prognosis?

What is “the most critical prognostic element”?

A

90% will be cured after 4 yrs.

Older children have better prognosis.

“Presence or absence of diffuse anaplasia”.

66
Q

Metastasis to the kidneys is…

What cancers might metastasize to the kidney? (5)

A

Uncommon, but terminal if it occurs. Frequently multifocal and BL.

Lung, melanoma, breast, GI and pancreas.

67
Q

Cytogenetics and genetics of sporadic papillary carcinoma

A

Cyto - trisomy 7, 17; loss of Y

Genetics - mutated, activated MET

68
Q

Cytogenetics and genetics of hereditary papillary carcinoma

A

Cyto - trisomy 7

Genetics - mutated, activated MET

69
Q

Cytogenetics and genetics of sporadic and hereditary CCC

A

Cyto - deletions on chr 3

Genetics - loss of VHL, inactivated/mutated VHL, hypermethylation of VHL

CPR II Exam #2 (20 decks)

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