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CPR Exam 3 > Renal Pathology Pt. 4 > Flashcards

Renal Pathology Pt. 4 Flashcards

1
Q

what is renal agenesis?

A

bilateral is incompatible with life

unilateral - single kidney hypertrophies to compensate

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2
Q

what is renal hypoplasia?

A

failure of the kidneys to develop to normal size

bilateral is possible but unilateral is more likely

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3
Q

what is an ectopic kidney?

A

kidney not present at the usual paravertebral retroperitoneal site but present somewhere along the normal path of the ureter

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4
Q

what is a potential complication of ectopic kidney?

A

infection due to obstruction

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5
Q

what is a horseshoe kidney?

A

fusion of the lower or upper poles of the kidneys

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6
Q

what are the cystic diseases of the kidney?

A 
multicystic renal dysplasia
polycystic kidney disease
medullary cystic disease
acquired cystic disease
localized renal cysts
hereditary malformation syndromes
glomerulocystic disease
extra-parenchymal renal cysts
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7
Q

what is adult/ AD polycystic kidney disease?

A

AD mutation in PKD1 (85%) or PKD2 (15%) which encodes polycystin

results in chronic renal failure beginning at age 40-60

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8
Q

what morphological changes are associated with adult polycystic kidney disease?

A

large, multicystic kidneys
liver cysts
berry aneurysms

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9
Q

what are the clinical features of adult polycystic kidney disease?

A 
hematuria
flank pain
UTI
renal stones
HTN
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10
Q

what is infant/ AR polycystic kidney disease?

A

AR mutation in PKHD1 encoding fibrocystin

may result in early death

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11
Q

what are the morphological changes associated with AR polycystic kidney disease?

A

enlarged, cystic kidneys at birth

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12
Q

what are the four clinical subtypes of AR polycystic kidney disease?

A

Perinatal * most common - death several hours after birth
Neonatal - death several months after birth
Infantile - death in early childhood
Juvenile - death during adolescence

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13
Q

what is medullary sponge kidney?

A

benign medullary cysts on excretory urography

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14
Q

what are the clinical features of medullary sponge kidney?

A

hematuria
UTI
recurrent renal stones

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15
Q

what is juvenile nephronophthisis?

A

AR disorder

presents as progressive renal failure in childhood

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16
Q

what are the morphological changes associated with juvenile nephronophthisis?

A

corticomedullary cysts

shrunken kidneys

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17
Q

what are the clinical features of juvenile nephronophthisis?

A

salt wasting
polyuria
growth retardation

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18
Q

what is adult-onset nephronophthisis?

A

AD

presents as chronic renal failure in adulthood

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19
Q

what are the morphological changes associated with adult-onset nephronophthisis?

A

corticomedullary cysts

shrunken kidneys

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20
Q

what are the clinical features of adult-onset nephronophthisis?

A

salt wasting

polyuria

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21
Q

what are simple cysts?

A

benign cystic lesions within the kidneys causing microscopic hematuria

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22
Q

what is acquired renal cystic disease?

A

cystic degeneration in end-stage kidney disease resulting in dialysis dependence

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23
Q

what are the clinical features of acquired renal cystic disease?

A

hemorrhage
erythrocytosis
neoplasia

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24
Q

what is multicystic renal dysplasia?

A

irregular kidneys with various sized cysts

associated with other renal anomalies

results in renal failure if bilateral
surgically curable if unilateral

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25
Q

what are the major clinical features of AR polycystic kidney disease?

A

cystic collecting ducts

hepatic fibrosis

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26
Q

what renal anomalies are commonly seen with multicystic renal dysplasia?

A

ureter agenesis
ureteropelvic obstruction
perinatal mass

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27
Q

what is nephrolithiasis?

A

kidney stones

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28
Q

who is prone to developing nephrolithiasis?

A

men between age 20-30

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29
Q

what is the clinical presentation of nephrolithiasis?

A

renal colic
ulceration and bleeding of ureter (hematuria)
obstruction of urinary flow (anuria/oliguria)

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30
Q

which size of stone is most hazardous: small or large?

A

small because they can enter the ureter

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31
Q

what is the most common type of stone?

A

calcium oxalate and phosphate

32
Q

what predisposing condition is associated with calcium oxalate and phosphate stones?

A

idiopathic hypercalciuria

33
Q

what factors predispose a patient to kidney stone formation?

A

increased concentration of stone constitutes
changes in urine pH
decreased urine volume
presence of bacteria

34
Q

generally, what is the clinical presentation of benign renal neoplasms?

A

rarely cause clinical problems and are usually discovered as incidental findings on imaging studies

35
Q

what is the most common benign kidney neoplasm?

A

renal papillary adenoma

36
Q

are renal papillary adenomas always benign?

A

no, once they are greater than 1 cm they are classified as low-grade renal cell carcinoma

37
Q

what is seen histologically in renal papillary adenoma?

A

acidophilic cytoplasm (not clear)
papillae
thin fibrovascular cores

38
Q

what is the origin of renal oncocytoma?

A

arises from intercalated cells of the renal cortical collecting ducts

39
Q

what is the clinical presentation of renal oncocytoma?

A

benign lesions without symptoms
typically present in adulthood
rarely become invasive/metastatic
ddx: renal cell carcinoma

40
Q

what are the morphological features of renal oncocytoma?

A

mahogany-brown lesion that is well-circumscribed with central stellate scar

41
Q

what are the histological features of renal oncocytoma?

A

abundant acidophilic, granular cytoplasm

may show alveolar, nesting, tubular or solid pattern

42
Q

what condition are renal angiomyolipomas strongly associated with?

A

tuberous sclerosis complex (AD)

LoF mutation in TSC1 or TSC2 tumor suppresor genes

43
Q

what is the clinical presentation of renal angiomyolipomas?

A

may occasionally rupture and cause massive hemorrhage

initial presentation may be hypovolemic shock

44
Q

what are the histological features of renal angiomyolipomas?

A

thick-walled blood vessels (angio)
smooth muscle (myo)
fat (lipo)

45
Q

what is the most common malignant tumor of the kidneys?

A

renal cell adenocarcinoma

46
Q

what risk factors are associated with RCC?

A 
males age 60-80
cigarette smoking (2x)
HTN
obesity
estrogens
asbestos
CKD
tuberous sclerosis
acquired cystic disease
47
Q

what hereditary conditions are associated with RCC?

A

Von Hippel-Lindau syndrome
Hereditary clear cell carcinoma
Hereditary papillary carcinoma

48
Q

what is the most common form of RCC?

A

sporadic clear cell (80%)

49
Q

hereditary clear cell carcinoma is associated with what mutations?

A

LoF VHL (tumor suppressor gene) due to deletion/translocation on short arm of Chr. 3

50
Q

what mutations are associated with sporadic and hereditary papillary renal cell carcinoma?

A

trisomy 7, 16 and 17

Loss of Y which results in activation of MET proto-oncogene

51
Q

what is the major morphological difference between sporadic and hereditary RCC?

A

hereditary forms have multiple lesions

52
Q

what is the classic triad of RCC presentation?

A

hematuria
costovertebral pain
palpable flank mass

53
Q

why is RCC considered one of the “great mimics”?

A

it has a tendency to cause systemic symptoms unrelated to the kidney

reaches large size and metastasizes before local symptoms occur

54
Q

what type of RCC has the best prognosis?

A

chromophobe RCC

55
Q

what mutation is associated with chromophobe RCC?

A

Xp11 translocation

56
Q

what is the typical mode of spread for RCC?

A

hematogenous

57
Q

what are the morphological changes associated with RCC?

A

yellow-ish, spherical neoplasm in one pole of the kidney

58
Q

what histological changes are associated with clear cell RCC?

A 
clear cytoplasm (collagen +lipid)
sharply delineated cell membrane
termed "hypernephroma"
59
Q

what histological changes are associated with papillary RCC?

A

papillae and foamy macrophages

60
Q

what histological changes are associated with chromophobe RCC?

A

pale, eosinophilic cells arranged in solid sheets
well-defined cell membranes
faintly granular cytoplasm with perinuclear clear halos
cell concentrate around blood vessels

61
Q

what is urothelial carcinoma of the kidney?

A

cancer that originates from the urothelium of the renal pelvis

associated with analgesic and tubulointerstitial nephropathy

poor prognosis

62
Q

what is the clinical presentation of urothelial carcinoma?

A

hematuria
may block urinary outflow ad cause hydronephrosis
50% of patients have concomitant bladder tumors

63
Q

what is the most common renal tumor in children?

A

Wilms Tumor

64
Q

what is the clinical presentation of a wilms tumor?

A

large abdominal mass
pain
microscopic hematuria
HTN

65
Q

what familial syndromes are associated with Wilms tumors?

A

WAGR
Denys Drash
Beckwith-Wiedemann

66
Q

which familial syndromes are associated with a LoF mutation to WT1?

A

WAGR and Denys-Drash

67
Q

what is the presentation of WAGR?

A

genitourinary malformation

Wilms tumor

68
Q

what is the presentation of Denys-Drash?

A

Gonadal tumor
gonadal dysgenesis
early-onset nephropathy
Wilms tumor

69
Q

what is the presentation of Beckwith-Wiedemann?

A 
macroglossia
organomegaly
hemihypertrophy
omphalocele
adrenal cytomegaly
wilms tumor
70
Q

what are the two histomorphologies of Wilms tumors?

A

typical triphasic

anaplastic

71
Q

which Wilms tumor histomorphology has a more favorable outcome?

A

typical triphasic

72
Q

what is seen on histology in a triphasic Wilms tumor?

A

spindle-shaped cells
immature tubules
densely packed small blue cells

73
Q

what morphological changes are associated with a wilms tumor?

A

well-circumscribed
tan-grey color
large, expansile tumor in the lower pole

74
Q

what is the most critical prognostic element for a wilms tumor?

A

presence or absence of diffuse anaplasia

75
Q

what primary cancers are known to metastasize to the kidneys?

A 
breast
lung
melanoma
GI
pancreas

CPR Exam 3 (15 decks)

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