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GIR Test 4 > B5.081 - Renal and Urinary Tumors part 1 > Flashcards

B5.081 - Renal and Urinary Tumors part 1 Flashcards

1
Q

3 major types of RCC

A

clear cell

papillary

chromophobe

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2
Q

types of kidney tumors

A

RCC

renal pelvis tumor

wilms tumor

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3
Q

most common kidney tumor

A

RCC

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4
Q
A

normal kidney

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5
Q

epidemiology of RCC and risks

A

typically older

M>F

arise from tubular epithelium

tobacco

obesity, HTN, asbestos, petroleium, heavy metals

renal failure, TB, vHL

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6
Q

rare familial variants seen in RCC

A

AD - vHL

hereditary papillary carcinoma - MET

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7
Q

describe clear cell carcinoma

A

proximal tubule origin

clear or granular cytoplasm

95% sporadic

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8
Q

most common subtype of RCC

A

clear cell

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9
Q
A

Clear cell carcinoma

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10
Q
A

clear cell carcinoma

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11
Q

cytogenentics of clear cell RCC

A

98% of clear cell RCC have a loss of sequence on short arm of chromosome 3, or unbalanced translocation with loss of part of 3p

3p12-326 is the location of the vHL gene

vHL is a tumor suppressor gene, loss stimulates growth and angiogenesis

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12
Q

describe papillary carcinoma

A

10-15% of renal cell cancer

distal convoluted tubule origin

most common in dialysis pts

papillary growth within interstitial foam cells

familial and sporadic

trisomy 7 and 17 loss of Y

* chromosome 7: MET locus

TK receptor for hepatoycyte GF

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13
Q
A

papillary carcinoma

MET mt

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14
Q

describe features of chromophobe renal cell carcinoma

A

5% of renal cancers

intercalated cells of colle

cting duct origin

tumor cells have prominent cell membranes adn pale cytoplasm “vegetable cells”

halo around nucleus

multiple chromosome losses and hypodiploidy

excellent prognosis

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15
Q
A

chromophobe carcinoma

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16
Q

presentation of RCC

A

classic triad:

  1. flank pain
  2. palpabel mass
  3. hematuria

“great mimicker” produces a variety of systemic sx, paraneoplastic syndrones (polycythemia, HTN, hypercaclemia)

17
Q

where do RCC metastasize/invade

A

lungs, bones

lymph nodes, liver, adrenal, brain

tend to invade renal vein and can crawl up to VC

18
Q

tx of RCC

A

nephrectomy or partial

chemo if metastatic

19
Q

UCC

A

renal pelvis or anywhere covered by urothelium

20
Q
A

UCC

confined to pelvis

21
Q

presentation of UCC

A

they present earlier bc of location

hematuria, urinary obstruction

50% have previous or concurrent bladder tumor

22
Q

invasion of UCC

A

commonly invade renal pelvis/calyces

23
Q

risks fo UCC

A

analgesic nephropathy, lynch

24
Q

wilms tumor features

A

most common primary pediatric renal tumor

nephroblastoma

2-5 yo

25
Q
A

wilms tumor

26
Q

WAGR

A

wilms syndrome type

Wilms

Aniridia

Genital anomalies

mental Retardation

Germline deletion 11p13 “first hit”

WT gene

PAX6 gene (for iris)

27
Q

WT1 protein

A

critical for normal renal and gonadal development

encodes DNA binding transcription factor

Tumor suppressor gene

28
Q

sporadic wilms tumor genetics

A

b-catenin seen in 10% sporadic, GOF

29
Q

denys drash syndrome

A

associated with wilms tumor

*90% risk

1. Gonadal dysgenesis

2. Early onset nephropathy

3. Diffuse mesangial sclerosis

germline mutation in WT1 - missense mutation in region of WT1 affecting DNA properties

30
Q

beckwith wiedemann syndrome

A

assocated with wilms tumor

  1. Organomegaly
  2. Macroglossia
  3. Omphalocele
  4. Adrenal cytomegaly

increased tumor incidence

31
Q

genetics of beckwith wiedemann syndrome

A

genomic imprinting

11p15.5 (WT2)

genes in this region normally expressed from only 1 of 2 parental alleles with imprinting/silencing the other via methylation

loss of imprinting causes tumorigenesis

32
Q

wilms histo

A

triphasic

* blastemal - small blue

*stromal - fibrous, myxoid, skeletal m

*epithelial - abortive tubules or glomeruli

33
Q

anaplasia

A

wilms histo

large atypical cells in some wilms tumor, implications for treatment

underlying p53 mutation means resistant to chemo

34
Q
A

wilms tumor

35
Q
A

wilms tumor

36
Q

precursor lesion for wilms

A

nephrogenic rest

in 100% of wilms

found in adjacent kidney, same genetic abnormalities meaning the other kidney has a high risk of getting it

37
Q

wilms presentation

A

large abdominal mass

hematuria

abdominal pain

intestinal obstruction

HTN

pulmonary mets

GIR Test 4 (8 decks)

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