Paediatric Oncology 2

Question 1

What is Mesna and what is it used for?

Answer 1

• Synthetic sulphydryl drug
• prevents haemorrhagic cystitis during chemo esp. with ifosfamide/cyclophosphamide
• helps detox metabolites such as acrolin

Question 2

Where are you most likely to get a primary in B-cell non-Hodgkin Lymphoma?

Answer 2

Intestine.

Question 3

Name high-risk syndromes for Wilm’s tumour:

Answer 3

High:

• Denys-Drash/other WT1 deletions
• Perlman syndrome
• Fanconi anaemia D1/Biallelic BRCA2 mutations
• Familial Wilm’s

Question 4

What tumours do we worry about in Costello?

Answer 4

• Rhabomyosarcoma
• Neuroblastoma
  (Most commonly get papillomas but these are benign)

Question 5

Name moderate-risk syndromes for Wilm’s tumour:

Answer 5

Moderate:
- Beckwith‐Wiedemann syndrome
- Simpson‐Golabi‐Behmel syndrome caused by GPC3
mutations/deletions
- Frasier syndrome

Question 6

When don’t we give MTX?

Answer 6

3rd space/effusions as it accumulates