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

Renal neoplasia 12/21 Flashcards

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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
RCC. mass effect less common metastases more commonly detected than primary tumor
.
26
RCC. IVC obstruction presentation?
symetric bilateral lower extremity edema + collateral veins in the abdominal wall
27
RCC. IVC obstuction -> collateral veins on the abdominal walls (be cia chronic obstuction)
.
28
RCC. hypercalcemia 2 mechanisms?
Production of PTHrP or lytic bone metastases
29
RCC. loss of what gene?
Loss of WHL (3p) tumor suppressor gene
30
Loss of WHL (3p) tumor suppressor gene?
RCC.
31
RCC.Loss of WHL (3p) tumor suppressor gene -> leads to increased what (2)?
Increased of IGF-1 (promotes growth) and increased HIF transcription factor (increases VEGF and PDGF).
32
RCC. Increased of IGF-1 (promotes -->
Increased of IGF-1 (promotes growth)
33
RCC.i ncreased HIF transcription factor (increases --> ?
increased HIF transcription factor (increases VEGF and PDGF).
34
RCC. Sporadic. Population and location?
Adult males (>60) + smoking. Occurs in upper pole of the kidney
35
RCC. Hereditary. population and location i kidney?
younger adults. Bilateral
36
RCC. Hereditary. what disease?
Von Hippel-Lindau
37
Von Hippel-Lindau inheritance?
AD
38
Von Hippel-Lindau assoc with what mutation?
Inactivation of WHL gene leading to increased risk for hemangioblastoma of the cerebellum and RCC.
39
Von Hippel-Lindau assoc with what mutation -> Inactivation of WHL gene. risk for what?
hemangioblastoma of the cerebellum and RCC.
40
RCC. staging. T? based on what
based on the size and involvement of the renal vein (occurs commonly and increases risk of hematogenous spread to the lung and bone
41
RCC. staging. N. Spreads to what lymph nodes?
retroperitoneal lymph nodes
42
WT. Comprises of what?
malignant kidney tumor comprised of blastema (immature kidney mesenchyme), primitive glomeruli and tubules and stromal cells.
43
malignant kidney tumor comprised of blastema (immature kidney mesenchyme), primitive glomeruli and tubules and stromal cells.?
WT
44
WT population?
Most common malignant renal tumor in children. average age 3 y/o
45
WT. presentation?
large, unilateral flank mass with hematuria and hypertension (due to renin secretion)
46
WT. side?
unilateral
47
WT. why hypertension?
due to renin secretion
48
WT. most common what?
sporadic
49
WT. if non sporadic, but syndromic. What 3 syndomes?
WAGR syndrome Denys-Drash syndrome Beckwith-Wiedemann syndrome
50
WAGR syndrome. Presentation?
Wilms tumor; Aniridia; Genital abnormalities; mental and motor Retardation (WAGR).
51
WAGR syndrome. what deletion?
DELETION of WT1 tumor suppressor gene (located at 11p13)
52
Denys Drash syndrome. presentation?
Wilms tumor; progressive renal (glomerular) disease, and male pseudohermaphroditism
53
Denys Drash syndrome. what mutation?
MUTATIONS OF WT1
54
Beckwith-Wiedemann syndrome. presentation?
Wilms tumor; neonatal hypoglycemia, muscular hemihyperthrophy, and organomegaly (including tongue).
55
Beckwith-Wiedemann syndrome. assoc with what mutation?
Mutations in WT2 gene cluster (imprinted genes at 11p15.5), particularly IGF-2
56
Wilms tumor; Aniridia; Genital abnormalities; mental and motor Retardation?
WAGR syndrome.
57
Wilms tumor; progressive renal (glomerular) disease, and male pseudohermaphroditism
Denys Drash syndrome.
57
DELETION of WT1 tumor suppressor gene (located at 11p13)
WAGR syndrome.
58
MUTATIONS OF WT1
Denys Drash syndrome.
59
Wilms tumor; neonatal hypoglycemia, muscular hemihyperthrophy, and organomegaly (including tongue).
Beckwith-Wiedemann syndrome
60
Mutations in WT2 gene cluster (imprinted genes at 11p15.5), particularly IGF-2
Beckwith-Wiedemann syndrome

1 STEP Renal pathology (12 decks)

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