Wilm's Tumour

Question 2

What is Wilms’ Tumour also known as?

Answer 2

Nephroblastoma

Named after Dr. Max Wilms

Question 3

What type of cancer is Wilms’ Tumour?

Answer 3

Malignant embryonal cancer of the kidney

Question 4

What is the median age of diagnosis for Wilms’ Tumour?

Answer 4

3-4 years old

Question 5

What percentage of Wilms’ Tumour cases occur before the age of 5?

Answer 5

75%

Question 6

What are the two prognostic groups for Wilms’ Tumour based on pathology?

Answer 6

• Favourable histology (FH)
• Unfavourable histology (UFH)

Question 7

What characterizes Favourable Histology (FH) in Wilms’ Tumour?

Answer 7

Contains intermediate cellular differentiation and occurs in 90% of patients

Question 8

What characterizes Unfavourable Histology (UFH) in Wilms’ Tumour?

Answer 8

Highly anaplastic (poor cell differentiation) and occurs in 10% of patients

Question 9

What are some subtypes of Unfavourable Histologies?

Answer 9

• Focal anaplasia
• Diffuse anaplasia
• Clear cell sarcoma
• Rhabdoid sarcoma

Question 10

What are common presentations of Wilms’ Tumour in children?

Answer 10

• Painless abdominal swelling
• Smooth, firm, non-tender mass on one side of abdomen
• Gross hematuria
• Non-specific symptoms like fever and malaise

Question 11

What imaging techniques are used for diagnosing Wilms’ Tumour?

Answer 11

• CT of abdomen and chest
• MRI
• Ultrasound
• X-ray films

Question 12

What is the most common site of metastasis for Wilms’ Tumour?

Answer 12

Lungs

Question 13

What does the National Wilms’ Tumour Study Staging System focus on?

Answer 13

• Tumour size
• Tumour spillage
• Post-operative residual disease
• Potential metastasis

Question 14

What are poor prognostic factors for Wilms’ Tumour?

Answer 14

• Extensive tumours with poor histology
• Lymph node involvement
• Non-Diploid tumours

Question 15

What is the initial treatment approach for Wilms’ Tumour in North America?

Answer 15

An up-front nephrectomy at the initial presentation

Question 16

What are the indications for postoperative radiotherapy (RT) in Wilms’ Tumour?

Answer 16

• All cases of Stage I Unfavourable Histology
• Stage II Unfavourable Histology
• Stage III with macroscopic residual disease confined to flank

Question 17

What is the typical radiation dose for Stage III Unfavourable Histology?

Answer 17

1980 cGy to flank

Question 18

Fill in the blank: The acronym ‘WAGR’ stands for _______.

Answer 18

Wilms’ tumour, aniridia, genitourinary malformations, mental deficits

Question 19

What are late effects associated with Wilms’ Tumour treatment?

Answer 19

• Skeletal effects
• Increased incidence of scoliosis
• Secondary malignancies
• Intestinal obstruction

Question 20

What percentage of Wilms’ Tumour patients experience a relapse?

Answer 20

10%-15%

Question 21

What are favorable prognostic features after relapse in Wilms’ Tumour?

Answer 21

• Relapse within 12 months after initial diagnosis
• FH tumour pathology
• Early disease stage at initial diagnosis (Stage I or II)