1. Copyright Prof. Dr. Graf, Homburg

2. Prognosis of Nephroblastoma since 1915
Copyright Prof. Dr. Graf, Homburg

3. Copyright Prof. Dr. Graf, Homburg

4. Copyright Prof. Dr. Graf, Homburg

5. Symptoms in Wilms´ Tumor
56 %
Pain
25 %
Haematuria
18 %
Fever
10 %
Urinary tract infection
6 %
Weight loss
5 %
Constipation
Enteritis
4 %
Vomiting
Other
19 % 0%
20%
40%
60%
80%
100%
symptomatic
other symptoms
U3 - U9 (no symptoms)
Symptoms in Wilms´ Tumor
Gutjahr P: Bundesweite Wilmstumor-Studie 1980 bis 1988.
Dt Ärztebl 87:B2130-B2134, 1990
Copyright Prof. Dr. Graf, Homburg

6. Copyright Prof. Dr. Graf, Homburg

7. Copyright Prof. Dr. Graf, Homburg

8. Differential Diagnosis
Other malignancies :
Neuroblastoma
Adenocarcinoma of kidney
Lymphoma
Langerhans Cell Histiocytosis
Teratoma
Benign diseases :
Abscess of the kidney
Fibroma of the kidney
Haematoma
Hydronephrosis
Cystadenoma
Ganglioneuroma
Hamartoma
Copyright Prof. Dr. Graf, Homburg

9. Copyright Prof. Dr. Graf, Homburg

10. Frequency of congenital Anomalities
Anomalia
NWTS [%]
SIOP [%]
CNS
0.47
1.66
Eye (all)
1.21
2.17
only Aniridia
0.84
Cardiopulmonal
1.52
Gastrointestinal
0.68
Urogenital (all)
4.61
4.41
Cryptorchism
2.78
Hypospadia
1.78
Hair, skin, nails
Hemihypertrophia
2.47
3.13
Neurofibromatosis
0.10
Bürger D, Feickert HJ, Mildenberger H: Current status of nephroblastoma treatment. Monogr Paediatr 18: , 1986
Copyright Prof. Dr. Graf, Homburg

11. Syndromes und congenital Anomalities in Association with Wilms´ Tumor
Copyright Prof. Dr. Graf, Homburg

12. Copyright Prof. Dr. Graf, Homburg

13. Nephroblastoma
Neuroblastoma
Copyright Prof. Dr. Graf, Homburg

14. Copyright Prof. Dr. Graf, Homburg

15. Sonography
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16. CT
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17. MRI
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18. Bilateral nephroblastoma
Copyright Prof. Dr. Graf, Homburg

19. Copyright Prof. Dr. Graf, Homburg

20. Copyright Prof. Dr. Graf, Homburg

21. Copyright Prof. Dr. Graf, Homburg

22. SIOP 9 and SIOP 93-01 / GPOH tumor volume
Copyright Prof. Dr. Graf, Homburg

23. SIOP 9 and SIOP 93-01 / GPOH low risk intermediate risk high risk
2000
1600
1200
800
400
after chemotherapy at diagnosis
Tumor volume [ml]
N = 51
470 85
mean ml ml ml
mean ml ml ml
P < p < n.s.
1200
800
400
N = 27
382 75
Copyright Prof. Dr. Graf, Homburg

24. SIOP 9 and SIOP 93-01 / GPOH low risk intermediate risk high risk
3000
P < 0.001
p < 0.001
mean ml ml ml
median ml ml ml
2000
1000
Tumor volume reduction after
preoperative chemotherapy [ml]
low risk intermediate risk high risk
-1000
-2000
N = 25
366 71
Copyright Prof. Dr. Graf, Homburg

25. Copyright Prof. Dr. Graf, Homburg

26. Operative Complications
Obstruction of the gut and ileus 6.9 %
Severe bleeding %
Wound infection %
Vessel injury %
Hernia %
Gut infarction %
Obstruction V. cava inferior 0.5 %
Spleen injury %
Gut injury %
Pneumothorax %
Chylous ascites %
ML Ritchey et al.: Surgical Complications after Nephrectomy for Wilms´ Tumor.
Surg Gynecol Obstet 175: , 1992
Copyright Prof. Dr. Graf, Homburg

27. Copyright Prof. Dr. Graf, Homburg

28. Copyright Prof. Dr. Graf, Homburg

29. Copyright Prof. Dr. Graf, Homburg

30. Copyright Prof. Dr. Graf, Homburg

31. Stockholm Working Classification of Renal Tumours of Childhood
Copyright Prof. Dr. Graf, Homburg

32. Tumor staging
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33. Copyright Prof. Dr. Graf, Homburg

34. SIOP 9 and SIOP 93-01 / GPOH preop. chemotherapy primary surgery %
Copyright Prof. Dr. Graf, Homburg

35. Treatment schedule of the study SIOP 93-01
Copyright Prof. Dr. Graf, Homburg

36. multivariate analysis
SIOP 9 / SIOP 93-01
multivariate analysis
Copyright Prof. Dr. Graf, Homburg

37. Tumour volume and local stage
SIOP 9 / SIOP 93-01
Tumour volume and local stage
Copyright Prof. Dr. Graf, Homburg

38. SIOP 9 / SIOP 93-01 Chisq= 10.9 on 1 degrees of freedom, p= 0.000941
Survival according to treatment - correct / incorrect
Chisq= on 1 degrees of freedom, p=
Copyright Prof. Dr. Graf, Homburg

39. SIOP 9 / SIOP 93-01
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40. SIOP 9 / SIOP 93-01
Copyright Prof. Dr. Graf, Homburg

41. SIOP 9 / SIOP 93-01
Copyright Prof. Dr. Graf, Homburg

42. SIOP 9 / SIOP 93-01
Copyright Prof. Dr. Graf, Homburg

43. SIOP 9 / SIOP 93-01
Copyright Prof. Dr. Graf, Homburg

44. Copyright Prof. Dr. Graf, Homburg

45. SIOP 9 / SIOP 93-01
Copyright Prof. Dr. Graf, Homburg

46. SIOP 9 / SIOP 93-01
Copyright Prof. Dr. Graf, Homburg

47. SIOP 9 / SIOP 93-01 Tumor volume after preoperative CT
Copyright Prof. Dr. Graf, Homburg

48. SIOP 9 / SIOP 93-01 Tumor volume at diagnosis
Copyright Prof. Dr. Graf, Homburg

49. SIOP 9 / SIOP 93-01 Mixed subtype, focal anaplasia
Copyright Prof. Dr. Graf, Homburg

50. SIOP 9 / SIOP 93-01
Copyright Prof. Dr. Graf, Homburg