Renal Tumors

Front 1

Wilms Tumor

incidence and etiology

Back 1

varies by race
10% associated with syndromes - aniridiaisolated hemihypertrophy, Denys-Drash syndrome (nephropathy, renal failure, male pseudohermaphroditism, wilms tumor)
beckwith wiedemann syndrome (visceromegaly, macroglossia, omphalocele, hyperinsulinemic hypoglycemia in infancy
the WAGR complex (wilms, aniridia, GU malformations, mental retardation) all suggesting a genetic predisposition
simpson golabi behmel syndrome (overgrowth syndrome)

Front 2

genetic origins

WT1 & WT2 do not have prognostic significance (contrast to MYCN gene in neuroblastoma)

LOH loss of heterozgosity on 16q associated with a 3.3x greater incidence relapse and 12x greater mortality

Back 2

chromosmal deletion of band p13 of chromosome 11
aniridia gene PAX 6 and Wilm's tumor suppressor gene (WT1)
(none of the familia cases of aniridia lacked the WT1 site.
if you have sporadic aniridia risk of WT is 67x normal.
45-57% of WAGR will develop WT.
WHEN YOU DELETE THE BAND p13 of CHROMOSOME 11, YOU DELETE WILM'S TUMOR SUPPRESSOR GENE WT1

Hint 2

protein product of WT1 gene is transcriptional factor of the zinc-finger family which regulates expression of other genes - insulin like growth factor 2, platelet derived growth factor a. the suppresion of these growth associated genes may explain the tumor suppresor role of WT1.

Front 3

what is unique about wilms' tumor in patients with WAGR

Back 3

younger and more often bilateral

Front 4

what is unique about beckwith wiedemann syndrome?

Back 4

dysregulation of p15 chromosome 11
children with hemihypertrophy greater risk for malignancy - 40%

Front 5

recommendations for children with syndromes associated with Wilms

Back 5

US every 3 months 0-5 years of age.

Front 6

pathologic precursors
nephrogenic rests - NR - perilobular & intralobular



mutlicystic dysplastic kidneys

Back 6

Tumor development associated with persistence of NR after the 36th week of gestation

Hint 6

aniridia and deny drash ~ Intralobular

hemihypertrophy and BWs ~ perilobular

Front 7

nephroblastomatosis

Back 7

presence of multiple NRs

Front 8

classification of NR

Back 8

incipient/dormant - blastemal; sharp margins

regressing /sclerosing - maturation of cellular elements progress to obsolescent rests

hyperplastic - problematic - hard to distinguish from small Wilm's tumors - diffuse proliferation of components throughout the rest - need margin with normal tumor to distinguish from actual WT - hyperplastics rests lack a capsule; WT have a pseudocapsule

Hint 8

NR are often associated with WT
41% of unilateral WT

99% of bilateral WT

MRI helpful to monitor nephroblastomatosis

Front 9

Diffuse hyperplastic perilobar neprhoblastomatosis is a distinct entity that MUST be distinguished from Wilm's tumor.

Back 9

nephrectomy is not indicated
chemo can control the proliferative element
classic xray - massively enlarged kidney; renal configuration preserved in NR ; in WT kidney is distorted.

Front 10

multicystic dysplastic kidneys

Back 10

risk of WT is low and not enough to justify prophylactic nephrectomy

Front 11

Wilms Tumor

incidence and etiology

Back 11

varies by race
10% associated with syndromes - aniridiaisolated hemihypertrophy, Denys-Drash syndrome (nephropathy, renal failure, male pseudohermaphroditism, wilms tumor)
beckwith wiedemann syndrome (visceromegaly, macroglossia, omphalocele, hyperinsulinemic hypoglycemia in infancy
the WAGR complex (wilms, aniridia, GU malformations, mental retardation) all suggesting a genetic predisposition
simpson golabi behmel syndrome (overgrowth syndrome)

Front 12

genetic origins

WT1 & WT2 do not have prognostic significance (contrast to MYCN gene in neuroblastoma)

LOH loss of heterozgosity on 16q associated with a 3.3x greater incidence relapse and 12x greater mortality

Back 12

chromosmal deletion of band p13 of chromosome 11
aniridia gene PAX 6 and Wilm's tumor suppressor gene (WT1)
(none of the familia cases of aniridia lacked the WT1 site.
if you have sporadic aniridia risk of WT is 67x normal.
45-57% of WAGR will develop WT.
WHEN YOU DELETE THE BAND p13 of CHROMOSOME 11, YOU DELETE WILM'S TUMOR SUPPRESSOR GENE WT1

Hint 12

protein product of WT1 gene is transcriptional factor of the zinc-finger family which regulates expression of other genes - insulin like growth factor 2, platelet derived growth factor a. the suppresion of these growth associated genes may explain the tumor suppresor role of WT1.

Front 13

what is unique about wilms' tumor in patients with WAGR

Back 13

younger and more often bilateral

Front 14

what is unique about beckwith wiedemann syndrome?

Back 14

dysregulation of p15 chromosome 11
children with hemihypertrophy greater risk for malignancy - 40%

Front 15

recommendations for children with syndromes associated with Wilms

Back 15

US every 3 months 0-5 years of age.

Front 16

pathologic precursors
nephrogenic rests - NR - perilobular & intralobular



mutlicystic dysplastic kidneys

Back 16

Tumor development associated with persistence of NR after the 36th week of gestation

Hint 16

aniridia and deny drash ~ Intralobular

hemihypertrophy and BWs ~ perilobular

Front 17

nephroblastomatosis

Back 17

presence of multiple NRs

Front 18

classification of NR

Back 18

incipient/dormant - blastemal; sharp margins

regressing /sclerosing - maturation of cellular elements progress to obsolescent rests

hyperplastic - problematic - hard to distinguish from small Wilm's tumors - diffuse proliferation of components throughout the rest - need margin with normal tumor to distinguish from actual WT - hyperplastics rests lack a capsule; WT have a pseudocapsule

Hint 18

NR are often associated with WT
41% of unilateral WT

99% of bilateral WT

MRI helpful to monitor nephroblastomatosis

Front 19

Diffuse hyperplastic perilobar neprhoblastomatosis is a distinct entity that MUST be distinguished from Wilm's tumor.

Back 19

nephrectomy is not indicated
chemo can control the proliferative element
classic xray - massively enlarged kidney; renal configuration preserved in NR ; in WT kidney is distorted.

Front 20

multicystic dysplastic kidneys

Back 20

risk of WT is low and not enough to justify prophylactic nephrectomy

Front 21

Wilms Tumor

incidence and etiology

Back 21

varies by race
10% associated with syndromes - aniridiaisolated hemihypertrophy, Denys-Drash syndrome (nephropathy, renal failure, male pseudohermaphroditism, wilms tumor)
beckwith wiedemann syndrome (visceromegaly, macroglossia, omphalocele, hyperinsulinemic hypoglycemia in infancy
the WAGR complex (wilms, aniridia, GU malformations, mental retardation) all suggesting a genetic predisposition
simpson golabi behmel syndrome (overgrowth syndrome)

Front 22

genetic origins

WT1 & WT2 do not have prognostic significance (contrast to MYCN gene in neuroblastoma)

LOH loss of heterozgosity on 16q associated with a 3.3x greater incidence relapse and 12x greater mortality

Back 22

chromosmal deletion of band p13 of chromosome 11
aniridia gene PAX 6 and Wilm's tumor suppressor gene (WT1)
(none of the familia cases of aniridia lacked the WT1 site.
if you have sporadic aniridia risk of WT is 67x normal.
45-57% of WAGR will develop WT.
WHEN YOU DELETE THE BAND p13 of CHROMOSOME 11, YOU DELETE WILM'S TUMOR SUPPRESSOR GENE WT1

Hint 22

protein product of WT1 gene is transcriptional factor of the zinc-finger family which regulates expression of other genes - insulin like growth factor 2, platelet derived growth factor a. the suppresion of these growth associated genes may explain the tumor suppresor role of WT1.

Front 23

what is unique about wilms' tumor in patients with WAGR

Back 23

younger and more often bilateral

Front 24

what is unique about beckwith wiedemann syndrome?

Back 24

dysregulation of p15 chromosome 11
children with hemihypertrophy greater risk for malignancy - 40%

Front 25

recommendations for children with syndromes associated with Wilms

Back 25

US every 3 months 0-5 years of age.

Front 26

pathologic precursors
nephrogenic rests - NR - perilobular & intralobular



mutlicystic dysplastic kidneys

Back 26

Tumor development associated with persistence of NR after the 36th week of gestation

Hint 26

aniridia and deny drash ~ Intralobular

hemihypertrophy and BWs ~ perilobular

Front 27

nephroblastomatosis

Back 27

presence of multiple NRs

Front 28

classification of NR

Back 28

incipient/dormant - blastemal; sharp margins

regressing /sclerosing - maturation of cellular elements progress to obsolescent rests

hyperplastic - problematic - hard to distinguish from small Wilm's tumors - diffuse proliferation of components throughout the rest - need margin with normal tumor to distinguish from actual WT - hyperplastics rests lack a capsule; WT have a pseudocapsule

Hint 28

NR are often associated with WT
41% of unilateral WT

99% of bilateral WT

MRI helpful to monitor nephroblastomatosis

Front 29

Diffuse hyperplastic perilobar neprhoblastomatosis is a distinct entity that MUST be distinguished from Wilm's tumor.

Back 29

nephrectomy is not indicated
chemo can control the proliferative element
classic xray - massively enlarged kidney; renal configuration preserved in NR ; in WT kidney is distorted.

Front 30

multicystic dysplastic kidneys

Back 30

risk of WT is low and not enough to justify prophylactic nephrectomy

Front 31

Wilms Tumor

incidence and etiology

Back 31

varies by race
10% associated with syndromes - aniridiaisolated hemihypertrophy, Denys-Drash syndrome (nephropathy, renal failure, male pseudohermaphroditism, wilms tumor)
beckwith wiedemann syndrome (visceromegaly, macroglossia, omphalocele, hyperinsulinemic hypoglycemia in infancy
the WAGR complex (wilms, aniridia, GU malformations, mental retardation) all suggesting a genetic predisposition
simpson golabi behmel syndrome (overgrowth syndrome)

Front 32

genetic origins

WT1 & WT2 do not have prognostic significance (contrast to MYCN gene in neuroblastoma)

LOH loss of heterozgosity on 16q associated with a 3.3x greater incidence relapse and 12x greater mortality

Back 32

chromosmal deletion of band p13 of chromosome 11
aniridia gene PAX 6 and Wilm's tumor suppressor gene (WT1)
(none of the familia cases of aniridia lacked the WT1 site.
if you have sporadic aniridia risk of WT is 67x normal.
45-57% of WAGR will develop WT.
WHEN YOU DELETE THE BAND p13 of CHROMOSOME 11, YOU DELETE WILM'S TUMOR SUPPRESSOR GENE WT1

Hint 32

protein product of WT1 gene is transcriptional factor of the zinc-finger family which regulates expression of other genes - insulin like growth factor 2, platelet derived growth factor a. the suppresion of these growth associated genes may explain the tumor suppresor role of WT1.

Front 33

what is unique about wilms' tumor in patients with WAGR

Back 33

younger and more often bilateral

Front 34

what is unique about beckwith wiedemann syndrome?

Back 34

dysregulation of p15 chromosome 11
children with hemihypertrophy greater risk for malignancy - 40%

Front 35

recommendations for children with syndromes associated with Wilms

Back 35

US every 3 months 0-5 years of age.

Front 36

pathologic precursors
nephrogenic rests - NR - perilobular & intralobular



mutlicystic dysplastic kidneys

Back 36

Tumor development associated with persistence of NR after the 36th week of gestation

Hint 36

aniridia and deny drash ~ Intralobular

hemihypertrophy and BWs ~ perilobular

Front 37

nephroblastomatosis

Back 37

presence of multiple NRs

Front 38

classification of NR

Back 38

incipient/dormant - blastemal; sharp margins

regressing /sclerosing - maturation of cellular elements progress to obsolescent rests

hyperplastic - problematic - hard to distinguish from small Wilm's tumors - diffuse proliferation of components throughout the rest - need margin with normal tumor to distinguish from actual WT - hyperplastics rests lack a capsule; WT have a pseudocapsule

Hint 38

NR are often associated with WT
41% of unilateral WT

99% of bilateral WT

MRI helpful to monitor nephroblastomatosis

Front 39

Diffuse hyperplastic perilobar neprhoblastomatosis is a distinct entity that MUST be distinguished from Wilm's tumor.

Back 39

nephrectomy is not indicated
chemo can control the proliferative element
classic xray - massively enlarged kidney; renal configuration preserved in NR ; in WT kidney is distorted.

Front 40

multicystic dysplastic kidneys

Back 40

risk of WT is low and not enough to justify prophylactic nephrectomy

Front 41

current guidelines for radiation therapy

Back 41

no lung radiation if mets resolve by CT scan after 6 weeks of chemo

Hint 41

abdominal radiation for WT stage 3
increasing dose from 10 to 20 decreases flank relapse

Front 42

SURGICAL APPROACH

Back 42

attempt initial surgical resection

Hint 42

less likely to invade

Front 43

factors associated with increased risk of surtical complications

Back 43

resection of other organs
advaced state local disease intreavascular etension resection of other organs tumor diam > 10 cm, ooperation by general surgeon, urologist

Hint 43

tumor biopsy and regional nodes biopsy should be perforemed

Front 44

surgical details

Back 44

imaging is critical, - 2 functioning kidney, presence of contralateral tumor, vascular invasion

Hint 44

ct of the chest, lesions assumed as malignant, sample small lesions seen only ot CT

Front 45

adequate incision explore for liver mets vena cava

Back 45

no need to examine contralateral kidney if imaging does not suggest disease

Hint 45

no need to secure hilum vascular

Front 46

cystoscopy for hematuria

Back 46

critical to biopsy nodes in the renal hilum and along the cava and oarta

Hint 46

take adrenal for upper pole tumors

Front 47

increased risk of local recurrence

Back 47

stage 3 disease, UF histology tumor rupture during ooperation.

Hint 47

most tumor rupture happens mobiizing the posterior aspect stuck to the diaphragm

Front 48

resection of organs and massive WT discouraged

Back 48

be wary of acquired von Willebrands

Front 49

when is preoperative treatment indicated

Back 49

solitary kidney,
bilateral WT
horseshoe
intravascular extension above

Hint 49

above the intrahepatic vena cava

Front 50

percutaneuous biopsy

Back 50

horseshoe kidney increased incidence of urine leak and ureteral damage

Hint 50

focal segmental glomerulosclerosis reported with a unilateral kidney

Front 51

criterion for partial nephrectomy

Back 51

involvement of one pole and less that one third of kidney
a functioning kidney
no involvement of the collecting system or renal vein, clear margins between tumor and surrounding structures

Hint 51

contained retroperitoneal rupture may be reason for preop chemo

Front 52

bilateral wilms tumor

Back 52

seen in younger 25 vs 44 months

Hint 52

preliminary treatment after biopsy and staging
biopsy all tumors because of discordant pathology

Front 53

higher incidence of postive surgical margins and local tumor recurrence

Back 53

presence of rhabomyomatous histology associated with poor response to chemo but favorable prognosis

Front 54

intravascular extension

recommend preop chemo

Back 54

4% incidence

Hint 54

determined by preoperative radiographs
US most sensitive better than CT

Front 55

does not affect prognosis if completely resected

Back 55

bypass needed if extension into the atrium

Hint 55

children receiving preop chemo had better outcomes

Front 56

resection alone for some WT?

Back 56

small group
less tha 2 years
stage 1
tumors < than 550 g

Front 57

neonatal WT

Back 57

50% of newborns had mesoblastic nephroma
4 WT

Hint 57

mesoblastic nephroma

Front 58

renal cell ca

Back 58

radical nephrectomy with node dissection
mean age 14 yrs
do better than adults

Front 59

mesoblastic nephroma

Back 59

most common renal tumor in neonate
invades medially

Hint 59

complete resection necessary