Wilms Tumor

Question 1

Most common malignant renaltumor of

childhood

Answer 1

Wilms tumor or Nephroblastoma

Question 2

Peak age group of Wilms tumor

Answer 2

Peak: 3-4yrs old

Question 3

The clinical syndromes associated with WILMS?

Answer 3

The clinical syndromes associated with
WT include WAGR syndrome (WT, Aniridia, Genitourinary mal-
formations, mental Retardation), Denys-Drash syndrome (pseu-
dohermaphroditism, mesangial sclerosis, renal failure, and
WT), and overgrowth syndromes like Beckwith-Wiedemann
syndrome (somatic gigantism, omphalocele, macroglossia,
genitourinary abnormalities, ear creases, hypoglycemia, hemihypertrophy, and a predisposition to WT and other malignan-
cies) and Simpson-Golabi-Behmel syndrome.

Question 4

_____ is tumor suppressor gene at chromosome 11p13 that was
isolated from a child with WAGR syndrome.____ is likely to
play a specific role in glomerular and gonadal development

Answer 4

WT1

Question 5

Beckwith-Wiedemann syndrome maps to chromosome 11p15.5; this locus is also referred to as ___.

Answer 5

WT2

Question 6

Patients with ______ have higher relapse and mortality rates.

Answer 6

loss of heterozygosity (LOH) at 16q and 1p

Question 7

The NWTS classifies all tumors as having either FH or unfavorable histology (UH).
The UH tumors include:

Answer 7

anaplastic tumors
clear cell sarcoma
rhabdoid tumor of kidney

Question 8

WT reflects the development of the normal kidney, consisting of
three components: ______

Answer 8

Blastemal, epithelial (tubules), and stromal

elements, in varying proportions

Question 9

The histologic feature of greatest clinical significance in WT is _____

Answer 9

anaplasia

Question 10

_____ is the most lethal renal neoplasm in children.

Answer 10

Rhabdoid tumor of the kidney or RTK

Question 11

classic presentation for WT is ?

Answer 11

abdominal swelling is discovered by the child’s mother or by a physician during a routine physical examination.

A smooth, firm, nontender mass on one side of the abdomen
is felt.

Question 12

differential diagnosis of WT includes _____

Answer 12

other malignant
childhood lesions of the kidney, neuroblastoma, and benign
conditions such as hydronephrosis, polycystic disease, and
splenomegaly in left-sided tumors.

Question 13

Excretory
urography (intravenous pyelography) was once the main-
stay of imaging in WT and now has largely been replaced by
___________ and _______

Answer 13

ultrasonography and computed tomography (CT) scanning

Question 14

The most common sites of metastases of WT

are in the _____

Answer 14

lungs (80%), lymph nodes, and liver.

Question 15

The NWTS has shown that although RT does not need to be
given immediately after surgery, a delay of ____ days after
surgery was associated with a significantly higher abdominal
relapse rate, particularly among patients with UH tumors.

Answer 15

≥10 days

Question 16

In the COG protocols, the dose is ____ Gy
for most indications except for stage III DA and stages I to
III RTK, where a higher dose of ____ Gy is recommended

Answer 16

In the COG protocols, the dose is 10 Gy
for most indications except for stage III DA and stages I to
III RTK, where a higher dose of 19.8 Gy is recommended

Question 17

____ Gy: HIGH Incidence of 54% (Washington Univ.) of scoliosis

Answer 17

> 30 Gy

Question 18

> __ Gy flank rt has higher pregnancy outcomes in the future

Answer 18

25 Gy

Question 19

Syndrome with WT with Highest ESRD?

Answer 19

Denys Drash syndrome (74)

Question 20

To decrease ______, shielding of femur and acetabulum is done

Answer 20

Slipped femoral capital epiphysis

Question 21

Inferior border of WLI is?

Answer 21

Costophrenic angles

Question 22

In NWTS2, what drug is added in dactino and vincistine that increased 2 year survival for stage II-IV tumors to 77%

Answer 22

Doxorubicin

Question 23

The COG staging and risk-group classification for treatment
assignment in the new generation of WT protocols This classification will, in addition to tumor stage, also consider the _____

Answer 23

eL-STAR

LOH  at  1p  and  16q
Stage
tumor weight
patient’s age
response  to chemotherapy in children with FH tumors and lung metastases.

Question 24

COG: ONLY those children

Answer 24

surgery-only therapy

Question 25

In clear cell, Multivariate analysis revealed

four independent prognostic factors for survival:

Answer 25

treatment
with doxorubicin, tumor stage, age at diagnosis, and tumor
necrosis.

Question 26

In recurrent WT, The favorable prognostic factors include:

Answer 26

no previous treatment with doxorubicin;

relapse more than 12 months after diagnosis; and

intra-abdominal relapse in a patient not previously treated with abdominal RT

Question 27

Wilms tumor (WT, nephroblastoma) is a highly curable child-
hood neoplasm. The prognosis of children with WT has
improved considerably from a very high mortality rate at
the beginning of the 20th century to the current cure rate
of ____%

Answer 27

> 90