1. Wilms Tumour PI Wilms #1 Protocol
Dr Jane Skeen

2. chemotherapy (+ / - radiotherapy stages III/IV)
Wilms Tumour
mostly unilateral painless abdominal mass + / - haematuria
treatment
surgery
chemotherapy (+ / - radiotherapy stages III/IV)
80% OS

3. Stages
I Confined to kidney, completely resected, renal capsule intact II Tumour completely resected, tumour extends beyond kidney (penetration capsule, invasion renal sinus) III Spread beyond kidney but confined to abdomen IV Haematogenous metastases (lungs, liver, bone, brain) V Bilateral renal involvement by tumour

4. Starship Blood and Cancer Centre
Histology
Stage
10yr OS %
Favourable 95% I 96 II 93
III 89 IV 81 V 78
Anaplastic
(Unfavourable) 5%
II-III 49 18
Starship Blood and Cancer Centre

5. Solid tumour kidney- arises from immature kidney cells (nephrogenic rest)
4th most common childhood cancer
M=F
Usually < 8 years (also adult Wilms)
1 in ,000 children
Genetic predisposition in a few (1.5% familial)
Associated conditions (congenital abnormalities)

6. Syndromes
WAGR (wilms,aniridia,genitourinary/mental retardation
Denys-Drash
Beckwith Wiedemann (overgrowth disorder)
Hemihypertrophy
Association with Assisted ReproductiveTechnology
Bilateral in 5-10% cases – multiple tumours

7. Two approaches
COG (AREN)- based on NWTS- surgery up front unless inoperable
SIOP
- Pre-and post-op chemotherapy
No biopsy at diagnosis
Surgery around week 6

8. TREATMENT PLAN- Fiji
Complete investigations/ workup as per protocol. Check BP Discuss with Chch team
Radiological (CT chest/abdomen) diagnosis of Wilms –biopsy not indicated
Ensure appropriate chemotherapy in country
Pre-operative chemotherapy VCR/ACT-D
Reimage week 5 then proceed to surgery –ideally at week 6
Histology review (NZ)
Post surgery chemotherapy VCR/ACT-D +/- DOXO, dependent on histology and stage, at CWMH or Lautoka

9. TREATMENT PLAN- Tonga and Samoa
On imaging suspicion of Wilms tumour, image and arrange transfer to Starship, Auckland, NZ as soon as feasible.
Decision will then be made whether nephrectomy possible.
If up front nephrectomy, post operative chemotherapy dependent on stage.
If inoperable pre-operative chemotherapy (VCR/ACT-D/DOXO) given, then reimage week 5 with surgery at week 6.
To return to island of referral, once clinically stable from nephrectomy and initial chemotherapy (either VCR/ACT-D or VCR/ACT-D/DOXO) and
completion of radiotherapy (if indicated)
Chemotherapy to return with patient

10. PI WILMS #1 Protocol (Source NWTS 4)
STAGE I and II FAVORABLE HISTOLOGY REGIMEN EE-4A WEEK A A A A A A A V V VV V V V VVV V* V* V* A - DACTINOMYCIN (45 MCG/KG, IV) V - VINCRISTINE (0.05 MG/KG, IV) V* - VINCRISTINE (0.067 MG/KG, IV)

11. PI WILMS #1 Protocol STAGE III and IV
FAVORABLE HISTOLOGY OR FOCAL ANAPLASIA
REGIMEN DD-4A
WEEK
A D+ A D A D* A D* A
V V V V V V V V V V V* V* V* V* V*  
XRT
A DACTINOMYCIN (45 MCG/KG, IV)
D* - DOXORUBICIN (1.0 MG/KG, IV)
D+ - DOXORUBICIN (1.5 MG/KG, IV)
V VINCRISTINE (0.05 MG/KG, IV)
V* - VINCRISTINE (0.067 MG/KG, IV)
XRT - RADIATION THERAPY

12. Tumour ?Wilms thought to be operable. Repeat CT scan done TTM PF
August/September 2011: presented to surgeons TTM, Samoa, with an increasing abdominal mass of 2 months duration causing respiratory compromise
Imaged with CT scan
November: CT scan discussed at weekly Starship Paediatric Oncology MDM conference- no rupture seen
Tumour ?Wilms thought to be operable. Repeat CT scan done TTM
24 November: Surgery Samoa - L)nephrectomy/splenectomy (Mr James Hamill, Starship)
Histology :Wilms Stage III FH

14. PF December 2011 –funding approved
December- arrived NZ, immunised (Hib, Men C, PCV 13,quad meningo) then commenced chemotherapy as per PI Wilms #1 protocol (3 drugs)
January 2012 whole abdomen RT
December-February 10 weeks chemotherapy
29 February 2011 returned to Samoa
7 March- ongoing chemotherapy TTM ( 3 weekly x 5 till week 24)

15. PI WILMS #1 Protocol STAGE III and IV
FAVORABLE HISTOLOGY OR FOCAL ANAPLASIA
REGIMEN DD-4A
WEEK
A D+ A D A D* A D* A
V V V V V V V V V V V* V* V* V* V*  
XRT
A DACTINOMYCIN (45 MCG/KG, IV)
D* - DOXORUBICIN (1.0 MG/KG, IV)
D+ - DOXORUBICIN (1.5 MG/KG, IV)
V VINCRISTINE (0.05 MG/KG, IV)
V* - VINCRISTINE (0.067 MG/KG, IV)
XRT - RADIATION THERAPY

16. Late Effects
Anthracycline exposure: Doxorubicin dose 3.5mg/kg (equivalent to 105mg/m2)-Recommend 5 yearly ECHO
Radiation: Whole abdomen 10.5 gy in 8 fractions 09/01-17/01/2012.
Single right kidney- urine dipstik
Splenectomy- reimmunised+ prophylactic Amoxil
Annual clinical reviews- imaging not indicated

17. July 2016 (5 years off treatment)