Wilms

Question 1

Aka

Answer 1

Nephroblastoma

Question 2

Is a

Answer 2

Malignant tumor of the kidney that arises from embryonal cells

Question 3

Most cases of wilms are

Answer 3

Solitary lesions, 6% involve both kidneys

Question 4

Median age

Answer 4

3 to 4 years and 75% of cases occur by age 5

Question 5

Cause of wilms

Answer 5

Unknown

Question 6

Syndromes associated with higher risk

Answer 6

WAGR, denys drash

Question 7

LOH on chromosome 1 p and 16q

Answer 7

Associated with poorer outcomes

Question 8

10-15% of cases show unfavorable histology

Answer 8

Anaplastic wilms, clear cell, rhabdoid

Question 9

Clear cell tumors have a

Answer 9

Higher rate of bone Mets

Question 10

Rhabdoid are associated with

Answer 10

CNS neoplasms

Question 11

Presenting symptoms

Answer 11

Abdominal pain (37) hyper tension, hematuria, fever

Question 12

Typically presents as a

Answer 12

Painless abdominal mass.
Grows rapidly

Question 13

Symptoms often reflect the pressure put on

Answer 13

Abdominal organs.

Question 14

Wilms tumors have a tendency to

Answer 14

Rupture

Question 15

Unlike patients with Neuroblastoma

Answer 15

Children with wilms do not appear severely ill

Question 16

A differential diagnosis includes

Answer 16

Neuroblastoma
Lymphoma
Sarcoma
Liver tumor

Question 17

What is the first step in diagnosis?

Answer 17

Abdominal ultrasound

Question 18

CT scan of chest, abdomen, pelvis

Answer 18

To assess the extent of disease and evaluate lymph nodes and lungs

Question 19

What is the primary site of Mets

Answer 19

Lungs, must be assessed

Question 20

Biopsy is

Answer 20

Contraindicated because they do tend to rupture

Question 21

Can invade or Mets into

Answer 21

Liver and inferior vena cava

Question 22

Classified

Answer 22

Favorable (90)
Or unfavorable/ anaplastic (10%)

Question 23

Unfavorable histology

Answer 23

Focal anaplasia
Diffuse anaplasia

Question 24

Unfavorable histologies are associated with

Answer 24

Aggressive behavior and frequent Mets to brain bone.

Question 25

First step of treatment

Answer 25

Nephrectomy

Question 26

In patients with bilateral disease

Answer 26

Partial resection can be done on minimally affected size

Question 27

Wilms is managed using a

Answer 27

Trimodalitiyy approach Surgery, radiation, chemo. Increased survival rates

Question 28

Chemo therapy

Answer 28

Actinomycin, vincristine, doxurubicin Used for advanced stages led to cure rates of 80% to 100%

Question 29

Long term surgical rates for wilms are

Answer 29

Excellent 85% to 90% of patients with favorable histology can be cured

Question 30

Even patients with Mets can be cured more than

Answer 30

70%

Question 31

With doses of 3000 to 4000 of ortho

Answer 31

Atrophy of soft tissues on treated sight and scoliosis is common

Question 32

Doses must be kept below

Answer 32

1500 cgy to prevent diffuse lung fibrosis

Question 33

Breast development may be impaired if

Answer 33

Pre pubescent female

Question 34

One kidney

Answer 34

Utj must be handled right away