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1 STEP Renal pathology > Renal neoplasia 12/21 > Flashcards

Renal neoplasia 12/21 Flashcards

1
Q

what is angiomyolipoma?

A

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

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2
Q

angiomyolipoma in what diseases is increased frequency?

A

tuberous sclerosis

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3
Q

renal cell carcinoma, definition?

A

malignant EPITHELIAL tumor arising from kidney TUBULES

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4
Q

RCC. Presentation?3

A

painless hematuria, flank pain, palpable abdominal mass (all three rarely occur together)

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5
Q

RCC. most common of 3 symptom?

A

hematuria

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6
Q

RCC. what other presentations apart those 3 symtoms?

A

general - fever, weight loss
PARANEOPLASTIC SYNDROME

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7
Q

RCC. What PARANEOPLASTIC SYNDROMEs?

A

focal EPO -> polycytemia
PTHrP –> hypercalcemia
hormone production: ACTH, renin

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8
Q

RCC. risk factors?
what toxin exposure?

A

smoking, hypertension, obesity

heavy metal, petroleum by-products

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9
Q

RCC. what may be rare complication?

A

Left-sided varicocele

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10
Q

RCC. Left-sided varicocele what vessel?

A

LEFT renal vein by carcinoma is blocked -> impaired drainage of the left spermatic vein leading to varicocele.

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11
Q

RCC. why varicocele is not present on right?

A

Right spermatic vein drains directly into the IVC; hence, right-sided varicocele is not seen

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12
Q

RCC. GROSS appearance?

A

Golden yellow tissue/mass (due to high lipid content)

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13
Q

RCC. gross. how mass defined/look like?

A

Spherical mass, often with invasion of the renal vein

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14
Q

RCC. Invades what?

A

Renal vein –> IVC

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15
Q

RCC. histopathology?

A

Rounded, polyglonal or cuboidal cells
Abundant CLEAR or yellow cytoplasm

CLEAR CELLS

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16
Q

RCC. metastasis sites?

A

lung (cannonball metastasis) - most common site
bone (osteolytic)

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17
Q

RCC. bone metastasis what?

A

bone (osteolytic)

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18
Q

RCC. originates in what part of kidney?

A

CORTEX

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19
Q

RCC. what subtypes?

A

clear cell carcinoma (CCC)

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20
Q

RCC. clear cell carcinoma (CCC) originates from what?

A

from epithelium of proximal convoluted tubules (PCT)

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21
Q

RCC. gross - yellow mass, what’s more?

A

necrosis and hemorrhage

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22
Q

RCC. why cells are clear?

A

copious amounts of cytoplasmic/intracellular glycogen and lipids –> STANDARTD tissues fixation dissolves glycogen and lipid -> clear spaces

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23
Q

RCC. apart from all three symptoms, what is the most common presentation?

A

ASYMPTOMATIC until the disease is advanced - tumors detected incidentally

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24
Q

RCC. If IVC obtucrtion -> leads to what?

A

THROMBUS FORMATION

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25
Q

RCC. mass effect less common
metastases more commonly detected than primary tumor

A

.

26
Q

RCC. IVC obstruction presentation?

A

symetric bilateral lower extremity edema + collateral veins in the abdominal wall

27
Q

RCC. IVC obstuction -> collateral veins on the abdominal walls (be cia chronic obstuction)

A

.

28
Q

RCC. hypercalcemia 2 mechanisms?

A

Production of PTHrP or lytic bone metastases

29
Q

RCC. loss of what gene?

A

Loss of WHL (3p) tumor suppressor gene

30
Q

Loss of WHL (3p) tumor suppressor gene?

A

RCC.

31
Q

RCC.Loss of WHL (3p) tumor suppressor gene -> leads to increased what (2)?

A

Increased of IGF-1 (promotes growth) and increased HIF transcription factor (increases VEGF and PDGF).

32
Q

RCC. Increased of IGF-1 (promotes –>

A

Increased of IGF-1 (promotes growth)

33
Q

RCC.i ncreased HIF transcription factor (increases –> ?

A

increased HIF transcription factor (increases VEGF and PDGF).

34
Q

RCC. Sporadic. Population and location?

A

Adult males (>60) + smoking.
Occurs in upper pole of the kidney

35
Q

RCC. Hereditary. population and location i kidney?

A

younger adults. Bilateral

36
Q

RCC. Hereditary. what disease?

A

Von Hippel-Lindau

37
Q

Von Hippel-Lindau inheritance?

A

AD

38
Q

Von Hippel-Lindau assoc with what mutation?

A

Inactivation of WHL gene leading to increased risk for hemangioblastoma of the cerebellum and RCC.

39
Q

Von Hippel-Lindau assoc with what mutation -> Inactivation of WHL gene. risk for what?

A

hemangioblastoma of the cerebellum and RCC.

40
Q

RCC. staging. T? based on what

A

based on the size and involvement of the renal vein (occurs commonly and increases risk of hematogenous spread to the lung and bone

41
Q

RCC. staging. N. Spreads to what lymph nodes?

A

retroperitoneal lymph nodes

42
Q

WT. Comprises of what?

A

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

43
Q

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

A

WT

44
Q

WT population?

A

Most common malignant renal tumor in children. average age 3 y/o

45
Q

WT. presentation?

A

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

46
Q

WT. side?

A

unilateral

47
Q

WT. why hypertension?

A

due to renin secretion

48
Q

WT. most common what?

A

sporadic

49
Q

WT. if non sporadic, but syndromic. What 3 syndomes?

A

WAGR syndrome
Denys-Drash syndrome
Beckwith-Wiedemann syndrome

50
Q

WAGR syndrome. Presentation?

A

Wilms tumor; Aniridia; Genital abnormalities; mental and motor Retardation (WAGR).

51
Q

WAGR syndrome. what deletion?

A

DELETION of WT1 tumor suppressor gene (located at 11p13)

52
Q

Denys Drash syndrome. presentation?

A

Wilms tumor; progressive renal (glomerular) disease, and male pseudohermaphroditism

53
Q

Denys Drash syndrome. what mutation?

A

MUTATIONS OF WT1

54
Q

Beckwith-Wiedemann syndrome. presentation?

A

Wilms tumor; neonatal hypoglycemia, muscular hemihyperthrophy, and organomegaly (including tongue).

55
Q

Beckwith-Wiedemann syndrome. assoc with what mutation?

A

Mutations in WT2 gene cluster (imprinted genes at 11p15.5), particularly IGF-2

56
Q

Wilms tumor; Aniridia; Genital abnormalities; mental and motor Retardation?

A

WAGR syndrome.

57
Q

Wilms tumor; progressive renal (glomerular) disease, and male pseudohermaphroditism

A

Denys Drash syndrome.

57
Q

DELETION of WT1 tumor suppressor gene (located at 11p13)

A

WAGR syndrome.

58
Q

MUTATIONS OF WT1

A

Denys Drash syndrome.

59
Q

Wilms tumor; neonatal hypoglycemia, muscular hemihyperthrophy, and organomegaly (including tongue).

A

Beckwith-Wiedemann syndrome

60
Q

Mutations in WT2 gene cluster (imprinted genes at 11p15.5), particularly IGF-2

A

Beckwith-Wiedemann syndrome

1 STEP Renal pathology (12 decks)

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