Imported: Renal_Quiz2_Leonard : Renal Tumors

Front 1

Wilms Tumor (Nephroblastoma) is pediatric or adult?

Back 1

ped

blast=immature

Front 2

Malignant pediatric tumor of the kidneys comprised of embryonal nephrogenic elements

Back 2

Wilms Tumor (Nephroblastoma)

Front 3

Most common malignant pediatric kidney tumor?

Back 3

Wilms Tumor (Nephroblastoma)

Front 4

why is a Wilms tumor called triphasic?

Back 4

has 3 types of tissue

Blastema: small densely packed cells with little cytoplasm; grow in nests or cords (trabeculae)
Epithelium: abortive tubules and glomeruli
Stroma: spindle cells of varying differentiation (Undifferentiated; skeletal muscle (most common), cartilage)

Front 5

a triphasic neoplasm in kids =

Back 5

Wilms Tumor (Nephroblastoma)

Front 6

Blastema =?

seen in?

Back 6

small densely packed cells with little cytoplasm; grow in nests or cords (trabeculae)

Wilms Tumor

Front 7

Blastema, epithelium, and stomal cells are seen in what?

Back 7

Wilms tumor

Front 8

unilateral abdominal tumor in a child =

Back 8

Wilms tumor

Front 9

What is the rule of 5 in tumors? what population is it found in? what specific tumor is this with?

Back 9

Accounts for ~ 5% of childhood cancers
5% bilateral
5% multicentric
5% anaplastic (histopathologic feature) – worse prognosis


in kids

Wilms tumor

Front 10

85% of pediatric renal neoplasms =

Back 10

Wilms tumor

Front 11

Wilms tumors metastasize to where?

Back 11

Lung

Front 12

What are the 3 associated symptoms with Wilms tumor?

Back 12

Beckwith-Wiedeman (organomegaly, hemihypertrophy)

WAGR syndrome (Wilms-aniridia-genital anomaly-retardation)

Denys-Drash syndrome (Glomerulonephritis, male gonadal dysgenesis)

1st two are most common

Front 13

Prognosis for Wilms tumor?

Back 13

if Anaplasia is present--> poor prognostic

yet 80-90% overall cure rate

Front 14

tx for wilms tumor?

Back 14

nephrectomy plus chemo

Front 15

Pathogenesis of Wilms tumor?

Back 15

WT1 gene protein regulates transcription of growth-promoting genes (e.g., IGF-2)

WT2 gene product also interacts with growth promoting factors

both on chromosome 11 and do growth promoting factors

Front 16

classic histology in Wilms tumor?

Back 16

triphasic microscopic appearance:

Primitive tubules (blue arrows)
Blastema (red arrows)
Stroma (green arrows)

Front 17

immature islands of tissue and small tubules of tissue and spindle areas =

Back 17

triphasic microscopic appearance found in

Wilms tumor

Front 18

Most common renal tumor in INFANCY (less than 1 year)

Back 18

Congenital Mesoblastic Nephroma

Front 19

palpable mass, spindle cells, and immature tissues found in an infant =

Back 19

Congenital Mesoblastic Nephroma

Front 20

discuss metastases in Congenital Mesoblastic Nephroma

Back 20

they are RARE

Front 21

majority of adult renal neoplasms are derived from where?

Back 21

epithelial components

aka tubular cells

Front 22

what are the 3 benign neoplasms we need to know

Back 22

papillary adenoma

Renal oncocytoma

Angiomyolipoma

Front 23

Key diagnostic feature of a papillary adenoma?

****

Back 23

SIZE at time of diagnosis

≤0.5 cm = adenoma; >0.5 cm = papillary renal cell carcinoma

Front 24

how do you define the difference btw a benign and malignant papillary neoplasm

Back 24

.5 cm < =Papillary adenoma--> benign

.5 cm> = Papillary carcinoma -->aggressive

Front 25

Most common renal tubular epithelial neoplasm?

Back 25

Renal papillary adenoma

Front 26

what is normally found in acquired cystic renal disease?

Back 26

Renal papillary adenoma

Front 27

What can be a long term result of hemodialysis?

Back 27

acquired cystic renal disease --> renal papillary adenoma

Front 28

Higher power view: fibrovascular cores lined by bland neoplastic cells with uniform, round to oval nucli =

Back 28

papillary adenoma

Front 29

Well circumscribed, tan/yellow/mahogany brown =

Back 29

Renal Oncocytoma

Front 30

Central stellate scar =

Back 30

Renal Oncocytoma

Front 31

chromophobe variant is a DD for ?

Back 31

Renal Oncocytoma

Front 32

large tumor with lots of mitochondria that can be seen in other places =

Back 32

Oncocytoma

Front 33

solid, mesenchymal tumor that is benign =

Back 33

angiomyolipoma

Front 34

what are the 3 tissue types in a AML?

Back 34

blood

muscle

fat

Front 35

Tuberous sclerosis and von Recklinghausen disease (NF1) are associated with what?

Back 35

Angiomyolipoma


these are both HEREDITARY disorders

Front 36

although benign, fatality may result in Angiomyolipoma from.....

Back 36

massive hemorrhage

renal failure from significant loss of functional renal parenchyma

Front 37

if you see a multifocal Angiomyolipoma what should you be thinking

Back 37

underlying hereditary component---> tuberous sclerosis

Front 38

LM finding of mixture of mature adipose tissue, spindled cells with smooth muscle features, and thick-walled blood vessels

Back 38

Angiomyolipoma

Front 39

most common malignant renal neoplasm in adults =

Back 39

Renal cell carcinoma (RCC)

Front 40

most common Renal cell carcinoma (RCC)

Back 40

conventional

or clear cell

Front 41

malignant tumor made of transitional cells involving the collecting system

Back 41

RCC

Front 42

2/3 are men; >50 yo; only ~ 1% are bilateral

Bilaterally strongly suggestive of underlying syndrome

Back 42

RCC

Front 43

biggest risk factor for RCC?

Back 43

SMOKING

Front 44

von Hippel Lindau disease can be a risk factor for what?

Back 44

RCC

Front 45

von Hippel Lindau disease =

Back 45

found in RCC

Front 46

classic triad seen in RCC?

Back 46

Flank pain

palpable mass

hematuria

Front 47

Pt is in a car wreck and has suspected internal bleeding, upon imaging what tumor would likely be found?

Back 47

RCC

most commonly found incidentally

Front 48

25% of the time what is found at time of presentation for RCC?

Back 48

metastases

Front 49

5 year survival for RCC

Back 49

70%

Front 50

What can help determine overall 5 year survival? (2)

Back 50

tumor stage and nuclear grade

Front 51

How does chemo help in RCC?

Back 51

it doesn't really

Front 52

major tx of RCC?

Back 52

surgical resection

Front 53

genetics of RCC?

Back 53

VHL gene (3p25) – mutation at this locus seen in >90% of cases

Front 54

what does mutation of VHL do? in what cancer?

Back 54

VHL is part of the ubiquitin ligase complex which is used to target proteins for degradation.... so if you mess this up, you lose the ability to break down protein (aka lose tumor suppressant)

Clear Cell RCC

Front 55

high levels of VFEG is seen in what?

Back 55

clear RCC

because lack of VHL gene

will lead to a highly vascular tumor

Front 56

what is TNM in tumor staging?

Back 56

T – size of primary tumor; extent of invasion beyond kidney
N – presence of metastases to regional lymph nodes
M – distant metastasis

Front 57

cyctoplasm clear with glycogen =

Back 57

Clear cell RCC

Front 58

germline mutation in c-met gene =

Back 58

Papillary RCC

Front 59

Associated with ESRD (“dialysis kidneys”)

Back 59

Papillary RCC

Front 60

More often multiple than any other type of RCC

Back 60

Papillary RCC

Front 61

size of a Papillary RCC

Back 61

> 0.5 cm

Front 62

which has a better prognosis Papillary RCC or clear?

Back 62

Papillary RCC

Front 63

Rare RCC with worst prognosis of subtypes. Typically presents as painless gross hematuria

Back 63

Collecting Duct Carcinoma

Front 64

A Mediterranean man presents with sickle-cell trait and a renal mass. What does he have

Back 64

Renal Medullary Carcinoma

could be a black dude too

Front 65

Neoplasms of transitional cell epithelium (TCC) =

Back 65

Renal Urothelial Carcinomas

Front 66

this is multifocal in development and undergoes the carcinogenic "feild effect" from tobacco

Back 66

Renal Urothelial Carcinomas

Front 67

adult man presents, found to have blood in the urine (microscopic) that he didn't know about (aka incidental), he has some microcytic hemolytic anemia. What does he have?

Back 67

RCC

Front 68

adult man presents, found to have blood in the urine (microscopic) that he didn't know about (aka incidental), he has some microcytic hemolytic anemia. What would be a genetic finding?

Back 68

VHL mutation