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GIR Test 4 > B5.081 Prework 1: Renal Tumors > Flashcards

B5.081 Prework 1: Renal Tumors Flashcards

1
Q

most common kidney neoplasms

A
  1. RCC (3 subtypes)
  2. wilms tumor
  3. urothelial tumors of renal pelvis
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2
Q

epidemiology of RCC

A

30,000 new cases/year in US
typically older patients (60s-70s)
2:1 M:F

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3
Q

where does RCC arise from

A

arise from renal tubular epithelium

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4
Q

risks factors for RCC

A 
tobacco**
obesity, HTN, asbestos, petroleum, heavy metals
renal failure (long term dialysis)
tuberous sclerosis
vHL
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5
Q

rare familial variants of RCC

A

autosomal dominant
von Hippel Lindau (vHL)
hereditary papillary carcinoma

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6
Q

von Hippel Lindau

A

RCC occurs in 2/3 of patients with vHL
often bilateral
loss of vHL tumor suppressor gene

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7
Q

hereditary papillary carcinoma

A

multiple bilateral tumors

mutated MET proto-oncogene

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8
Q

most common type of RCC

A

clear cell carcinoma

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9
Q

description of clear cell carcinoma

A

70-80% of renal cell cancers
proximal tubule origin
clear or granular cytoplasm
95% are sporadic

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10
Q

gross appearance of clear cell carcinoma

A

well circumscribed, tan mass

arises in parenchyma

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11
Q

histo appearance of clear cell carcinoma

A

multiple small cells with clear cytoplasm

blood vessels scattered throughout

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12
Q

cytogenetics associated with clear cell carcinoma

A

loss of sequences on short arm of chromosome 3 OR unbalanced translocation with loss of part of 3p

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13
Q

description of papillary carcinoma

A

10-15% of renal cell cancer
distal convoluted tubule origin
most common subtype found in dialysis patients

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14
Q

histo appearance of papillary carcinoma

A 
papillary growth (tubular formations with blood vessels in the middle)
cells with pink cytoplasm
interstitial foam cells (macrophages)
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15
Q

genetics associated with papillary carcinoma

A

familial and sporadic
associated with trisomy of 7 and 17 and loss of Y
chromosome 7: MET locus (proto-oncogene) mediates cell growth, invasion

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16
Q

description of chromophobe renal cell carcinoma

A

5% of renal cell cancer
intercalated cell of collecting duct origin
excellent prognosis

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17
Q

histo appearance of chromophobe renal cell carcinoma

A

cells with prominent cell membranes and pale cytoplasm “vegetable cells”
halo around nucleus

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18
Q

genetics associated with chromophobe RCC

A

multiple chromosome losses and hypodiploidy

no characteristic abnormalities

19
Q

clinical presentation of RCC

A

classic triad: costovertebral (flank) pain, palpable mass, hematuria
increased incidental discovery due to imaging for other reasons
produced a variety of systemic symptoms: polycythemia, hypertension, hypercalcemia

20
Q

severity of RCC

A

often metastasizes before any symptoms: lungs, bones most common
tends to invade renal vein, can grow continuously and reach vena cava/ right heart

21
Q

prognosis and treatment of RCC

A 
5 year survival average : 70 %
if localized : 95%
renal vein invasion or perinephric fat invasion : 60%
nephrectomy or partial nephrectomy
chemo if metastatic
22
Q

gross description of urothelial carcinoma of the renal pelvis

A

well circumscribed mass confined to renal pelvis

23
Q

description of UCC renal pelvis

A

5-10% of primary renal tumors
present earlier than RCC due to location: hematuria, urinary obstruction
50% have previous or concurrent bladder tumor

24
Q

risk factors for UCC renal pelvis

A

analgesic nephropathy

Lynch Syndrome

25
Q

prognosis of UCC renal pelvis

A

50-100% 5 year survival with low grade non-invasion

10% 5 year survival with invasion

26
Q

epidemiology of wilms tumor

A

most common primary pediatric renal tumor
incidence: 1/10,000
2-5 years old
5-10% bilateral

27
Q

prognosis of wilms tumor

A

90% 5 year survival
anaplastic histology key determinant
survivors at increased risk for secondary tumors (sarcoma, leukemia, lymphoma)

28
Q

gross appearance of wilms tumor

A

well circumscribed, bulging tumor

“squishes” out normal kidney

29
Q

genetic associations with wilms tumor

A

sporadic much more common BUT 10% are syndromic

30
Q

syndromes associated with wilms tumor

A

WAGR
Denys-Drash Syndrome
Beckwith Wiedemann Syndrome

31
Q

what is WAGR

A

wilms (33% chance)
aniridia
genital anomalies
mental retardation

32
Q

genetic mutation in WAGR

A

germline deletion of 11p13
WT1 gene
PAX6 gene

33
Q

what is the WT1 protein

A

critical for normal renal and gonadal development
encodes DNA binding transcription factors
tumor suppressor gene

34
Q

what is Denys Drash Syndrome

A 
90% risk for wilms
gonadal dysgenesis
early onset nephropathy
diffuse mesangial sclerosis
increases risk of gonadoblastoma
35
Q

genetic mutation in Denys-Drash

A

germline mutations in WT1

missense mutation in region that affect DNA binding properties

36
Q

what is Beckwith-Wiedemann syndrome

A

organomegaly
macroglossia
omphalocele
adrenal cytomegaly

37
Q

other tumors that appear in Beckwith-Wiedemann syndrome

A 
wilms
hepatoblastoma
pancreatoblastoma
adrenal cortical tumors
rhabdomyosarcomas
38
Q

genetic mutation associated with Beckwith-Wiedemann syndrome

A

genomic imprinting in 11p15.5, WT2 gene
loss of imprinting (re-expression of normally silenced maternal IGF2 allele)
OR
deletion of imprinted maternal allele/supplication of active paternal allele

39
Q

histology of wilms tumor

A

triphasic: blastemal cells, stromal cells, epithelial cells
possible anaplasia in 5% of tumors
-large pleomorphic cells with abnormal mitoses
-p53 mutations, resistant to chemo = worse prognosis

40
Q

appearance of blasternal cells in wilms

A

small, blue cells

form clumps together

41
Q

appearance of stromal cells in wilms

A

fibrous or myxoid
spindle cells
skeletal muscle

42
Q

appearance of epithelial cells in wilms

A

abortive tubules or glomeruli (gland looking)

43
Q

precursor lesions to wilms

A

nephrogenic rests seen in renal parenchyma adjacent to tumor in 25-40% of cases
share genetic abnormalities with adjacent tumor
increased risk of wilms in other kidney if present

44
Q

clinical presentation of wilms

A 
large abdominal mass (often felt by parent)
hematuria
abdominal pain
intestinal obstruction
HTN
pulmonary mets

GIR Test 4 (20 decks)

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