Part 10: Peds Cancers- Wilms Tumor

Wilms Tumor aka

1. nephroblastoma

2. embryonal kidney tumor

Which cancer was the first one to be treated using the multi-modal approach?

Wilms Tumor

Wilms tumor is a cancer that arises from what cells?

embryonal kidney cells

Wilms Tumor Epidemiology

• ~500 cases/ year

• Avg. Age: 3

• Almost all before age: 7

Risk Factors:

1. 1/3 of patients that develop sporadic aniridia will develop a Wilms tumor

2. genetics

3. multiple genitourinary abnormalities

Wilms Tumor Etiology

Wilms Tumor Gene- deletion on chromosome 11p13

Presentation of Wilms tumor

Palpable abdominal mass

• Usually painless

• Painful = advanced disease
  
  

Found:

At home by parent

or pediatrician visit

How is Wilms tumor diagnosed

1. Abdominal Ultrasound

2. CT scan for:

   1. primary tumor

   2. lymph nodes

   3. possible lung mets

True or False; Wilms Tumors are unilateral

False, can be bilateral

Wilms Tumor staging

National Wilms Tumor Study System (NWTS)

• Stages I-V

• Stage V: bilateral kidney involvement

Invasion pattern & mets for Wilms tumors

1. tends to mets to lungs

2. The following lymph nodes are usually positive at diagnosis:

   1. Renal Hilar nodes

   2. Para-aortic nodes

Treatment for Wilms Tumors:

1. Surgery

2. RTT

3. Chemo

Surgery : Nephrectomy

RTT

Chemo: VACA

Stage I: Nephrectomy

Stage II: Nephrectomy + VACA
Stage III: Nephrectomy + VACA (sometimes RTT- not best A)

Stage IV: Nephrectomy + VACA + RTT

Stage V: Nephrectomy + VACA + RTT

Which treatment modality is the definitive modality when treating a Wilms tumor?

Surgery- nephrectomy

What type of surgery is used for treating wilms tumor?

1. nephrectomy (on stages I-V)

2. Second look: not common anymore

RTT for Wilms Tumor

1. What stages is it used on

2. Field Arrangement

3. Organs to avoid

4. Stuff in the field

5. Dosage

1. What stages is it used on: IV & V sometimes

2. Field Arrangement: Parallel Opposed APPA

3. Organs to avoid: APPA to avoid contralateral kidney

4. Stuff in the field:

   1. tumor bed

   2. entire width of vertebral body + 1-2 cm

   3. Para-aortic nodes

5. Dosage: low dose tx→ 1080-2000cGy

   1. 180 cGy/ Day starting at day 9

   2. Day 0: surgery

Wilms tumor Chemo:

VACA what is it

combination chemo

V: Vincristine

A: Adriamycin (doxorubicin)

C: Cytoxan (cyclophosphamide)

A: Actinomycin-D

With combination chemo, even metastatic disease can be cured more than ___% of the time

70

5 year prognosis for Wilms tumor

80% younger is better