WILMS` TUMOUR
Clinical Features and Management
In Pakistan
Dr M. Shamvil Ashraf
Children Cancer Hospital &
National Institute of Child Health
Karachi, Pakistan
Children Cancer Hospital,Karachi
Children Cancer Hospital,
Karachi
A project of Children Cancer Foundation
Premier facility exclusively dedicated to children
suffering with cancer regardless of paying ability
80% are treated on support of Foundation while
only 20% pay for treatment.
200 new referral every year
National Institute Of Child
Health, Karachi
250 bedded Government Children Hospital
Tertiary care referral center
Oncology dept is a 10 bedded unit with
daycare and clinics
Funded by NGO Child Aid Association
225 new cases referral every year
WILMS` TUMOUR
Incidence
(Cases /106 children under 15 years of age/year
USA 7.8
UK (Manchester) 5.1
Australia (Queensland) 7.2
Sweden 8.3
The Netherland 6.5
Italy (Turin) 6.5
WILMS` TUMOUR
Incidence in Pakistan
No population based registry
Mainly Hospital based data
Wilm's Tumor In Karachi
(1953 cases)
Other
STS
6%
Wilms 7%
5%
Leuk
RB 40%
6%
Brain
3%
GCT
4% Bone
NB Lymp
5% 5% 19%
WILM`S TUMOUR
Age and Sex Distribution
80% presents under 5 years of age with
peak at 2 - 3 years
Sex ratio is equal
WILM`S TUMOUR
Age Distribution
30
25
20
15
10
5
0
1 2 3 4 5 6 7 8 9 10 11
WILMS` TUMOUR
Sex Ratio
45% Male
55% Female
WILMS` TUMOUR
Congenital Anomalies (97 cases)
Anomaly Karachi NWTS%
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Aniridia 3% 0.76
Hemihypertrophy 2% 3.2
Cryptorchidism - 3.2
Hypospadias - 2.2
Other genital -1% 0.7
CVS anomalies 2%
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WILMS` TUMOUR
Familial Versus Sporadic Cases
Familial origin is estimated in the range of 1% to
2.5%
The family history was positive in one family with
two maternal aunt dying with WT and one cousin
treated for Wilm's
In another family one child with WT had one
sibling dying with AML.
WILMS` TUMOUR
Clinical Presentation
Abdominal mass
Macroscopic haematuria
Hypertension
Clinical Presentation
Abdominal mass was the most frequent
presentation
Hypertension was seen in 5 % children
Haematuria noted in 6%
Clinical Presentation
Adverse factor
Late Presentation : There was a median time lag
of 2 months (range 2 wk –1and ½ year ) from
onset of symptoms to diagnosis
Poor nutritional status : Majority children were
malnourished at presentation
Inappropriate initial treatment
Wilm's Tumor
Problems in Management
Lack of multidisciplinary approach
Initially seen and treated by surgeons
No uniform protocol
Late referrals and advanced staged disease
Poor Performance status
Inability to afford treatment.
Lost to follow up
Multidisciplinary Approach
SURGEON PATHOLOGIST
RADIOTHERAPIST
ONCOLOGIST
Surgical Details
Notes available 23 / 36
Staging 20 / 36
Lymph node sampling 12 / 36
Pediatric surgeon 20
Adult Urologist 04
Adult General Surgeon 12
WILMS` TUMOUR
Histopathology
Lack of standardized reporting
Favorable and Unfavorable Features
mentioned only in less than 50% reports
Pre-Nephrectomy Work up
36 cases
Ultrasound abdomen 36 / 36
CT abdomen 18 / 36
Chest X-ray 25 / 36
CT chest 10 / 36
IVP 4 / 36
Radiotherapy
Radiotherapy centers are available in major
cities
Expertise particularly in children is not
available in every center.
STAGE
Stage I
Recurrent
4%
Stage V 12%
5%
Stage II
Stage IV
31%
9%
Stage III
39%
Lost to Follow up
Lost to Follow up 15%
Main reasons
Distance from treatment center
No place to stay in Karachi
Financial constraints
WILMS` TUMOUR
Treatment Approaches
NWTS Trials
Immediate Nephrectomy followed by
chemo, XRT.
Pre-op chemo only in selected cases.
SIOP Trials
Routine pre-op chemo followed by surgery
and further chemo and XRT
Patient Groups
Immediate Nephrectomy group 36
Delayed surgery group 41
Immediate Nephrectomy Group
Staging
Stage I 1
Stage II 13
Stage III 7
Stage V 2
Post nephrectomy treatment
27patients received post- nephrectomy
treatment based on UKWT2 protocol
–FH stage 1 Vinc x 10 weeks
–FH stage II Vinc + Act x 26 weeks
–FH stage III Vinc + Act + Dox x 26 weeks
–UH Stage I Vinc + Act x 26 weeks
–UH Stage II & III Vinc + Act + Dox x 26
weeks
XRT given to UH stage III
No standard protocols followed 18 –24 Gy
Post nephrectomy treatment
09 patients either received no treatment or
were treated inadequately and later
presented with relapsed disease.
Delayed surgical group
Total No 41
Biopsy FNAB or TRUCUT 41
Pre op chemo Vincristine and Actinomycin
for 4-6 wk
Surgery
Delayed surgical group
Post nephrectomy Treatment
Till2002 :
Stage 11 Vinc and Act x 26 week
Stage III Vinc + Act + Dox x 26 weeks
XRT
2003 onward
UK version of SIOP WT 2 based on
Pathological staging
Delayed surgical group
Staging
Stage I 02
Stage II 10
Stage III 20
Stage IV 07
Stage V 02
Outcome of Two Groups
Immed Delayed
Total Evaluable 32 33
Off Rx 18 25
On Rx 02 0
RIP 12 8
Survival 62 % 75%
Bilateral Disease
4 / 68
No uniform, treatment
2 immediate surgery
– Nephrectomy and partial nephrectomy
One died during pre-op chemotherapy
One had bilateral nephrectomy and is on
dialysis,waiting for transplant
Recurrent & Metastatic Disease
Pre-op chemo with Vinc,Act and Doxo
Surgery once metastatic disease under
control
Post op XRT followed by further chemo
Total duration 52 weeks
Outcome 2 / 8 are alive
Relapsed Wilms
Total No 5
Relapsed protocol
Based on Carbo + Etopo alternating with
Cyclo + Doxo
surgery
XRT and further chemo
Outcome Poor with no survival
Outcome according to Stage
Delayed Immed overall
Stage I&II 100% 100% 100%
Stage III 62% 60% 61%
Stage IV 0% 25% 16%
Stage V 50% 100% 75%
Mortality
Deaths 20/ 65 (30%) evaluable patients
Cause of death
– Progressive disease 13
– Sepsis 06
– Surgical Complication 01
Problems with Immediate
Nephrectomy
Pretreatment workup is incomplete
Surgical details are not available
Surgical staging is not done
Post surgery follow up is poor
Incomplete surgery
Operative complications
Delayed Surgery
More control of oncologist
More adherence to the protocol
Better compliance
Less surgical complications
Better survival?
Cost Issue
The treatment cost is not high as compared
to other pediatric cancer
Does not need high tech lab
Themain drugs vincristine, actinomycin
and doxorubicin are cheaper drug
The treatment is mainly out-patient
WILMS` TUMOUR
Conclusion
Wilms tumor is curable in majority even within
constrained resources
Multidisciplinary approach with good team work
of Surgeon, Pediatric Oncologist, pathologist and
Radiotherapist is essential
Adherence to a standard protocol
SIOP Approach may be more suitable for
developing countries?
THANK YOU