References include Oxford handbook of Urology (3rd Edition) Smith`s Urology (18th Edition) EAU Guidelines 2017

1. BY
DR. MUHAMMAD SAIFULLAH
REGISTRAR UROLOGY
MADINAH TEACHING HOSPITAL, FAISALABAD.

2. GERMAN PATHOLOGIST
AND SURGEON

3.  Most common SOLID RENAL tumour of childhood is
Nephroblastoma (5%)
 Third year of life
 Unicentric tumour (Single centre of Origin)
 Right side = Left side
 20% familial cases

4.  1 in 10,000 children
 Males = females
 5% are bilateral

5. FAMILY HISTORY
ONE PREZYGOTIC
MUTATION
ONE POSTZYGOTIC
MUTATION
FAMILIAL FORM
NO FAMILY HISTORY
TWO POSTZYGOTIC
MUTATIONS
SPORADIC FORM
TWO HIT HYPOTHESIS

6. ANIRIDIA

7.  10% Cases
 WAGR Syndrome
1. Wilms Tumour
2.Aniridia
3.Genitourinary malformations
4.Retarded (Mantel Retardation)

9.  BECKWITH-WIEDEMANN SYNDROME
Wilms Tumour
Hemihypertropy
Macroglossia

10.  DENYS-DRASH SYNDROME
Wilms Tumour
Gonadal dysgenesis
Nephropathy

11.  PERLMAN`S SYNDROME
Wilms Tumour
Fetal overgrowth

12.  Precursor Wilms Tumour lesions 
NR (Nephrogenic Rests)
 TYPES
1. Perilobar
2. Intralobar
 NR  Remain dormant for years 
INVOLUTION & SCLEROSIS 
WILMS TUMOUR

13. COMPONENTS
1. Blastemal (Metanephric blastema)
2. Epithelial (Primitive renal tubular
epithelium)
3. Stromal (Connective tissue)

16. ANAPLASTIC CELLS
or
CLEAR CELL SARCOMA
OF KIDNEY
or
RHABDOID TUMOUR OF
THE KIDNEY
UNFAVOURABLE TUMOUR
NO ANAPLASIA
FAVOURABLE TUMOUR
(WELL DIFFERENTIATED)

17. SARCOMA
 Mesenchymal Origin
(Mucous connective
tissue or mucoid
connective tissue in
the embryo is
MESENCHYME)
 MESODERM
 Epithelial Origin
 (Lines the inner or
outer surfaces of the
body)
 ECTODERM or
ENDODERM
CARCINOMA

18. Extreme nuclear atypia
Hyperdioploidy
Numerous complex translocations
Increases with advancing age
AFRICAN-AMERICAN race
p53 association

19.  Large & Multilobulated
 Gray/Tan colour
 Focal areas of hemorrhage / necrosis
 Ocasional fibrous pseudocapsule
 Direct extension through renal capsule
 Renal vein & IVC
 Lymphatics

21.  25% Lymph Node Spread
 15% Hematogenous spread
 LUNGS 85-95%
 LIVER 10-15%

22.  STAGE I  Limited to kidney & completely excised
 STAGE II  Extending beyond the kidney but
completely excised
 STAGE III  Unresectable tumour / Regional lymph
nodes (Non-hematogenous metastatic / Residual
tumour confined to abdomen)
 STAGE IV  Metastasis to LLBB / non-Regional LNs
 STAGE V  Bilateral Renal Involvement

23.  Asymptomatic Mass (90%)
 Abdominal Pain (33%)
 Hematuria (30-50%)
 HYPERTENSION (50% due to Renin secretion)
 Abdominal distension, Anorexia, Nausea.

24.  Mesoblastic nephroma - Most common renal
tumor in the first month of life
 Neuroblastoma
 Renal cell carcinoma
 Clear cell sarcoma of the kidney
 Rhabdoid tumor of the kidney
 Nonmalignant mass

25.  Hydronephrosis
 Multicystic kidney disease
 Renal cyst
 Renal thrombosis
 Dysplastic kidney
 Renal hemorrhage

26.  Benign
 Hamartoma (Hamartia means Defect)
 tumour like malformation made up
of abnormal mixture of cells which
resembles the tissue of its origin
 Distinguished post-operatively by
histopathology after Nephrectomy

27. NEPHROBLASTOMA
 Confined to one side
 Intrarenal (Kidney
doesn`t change axis)
 Late metastasis
 Decreased calcifications
 VMA not increased
 Crosses midline
 Outward and downward
displaced kidney
 Early metastasis
 More Calcifications
 VMA may be increased
NEUROBLASTOMA

28.  CBC  Anemia
 Urinalysis  Hematuria
 LFTs  Liver metastasis (Increased)

29.  ULTRASOUND KUB  CT-Scan KUB
with IV contrast
(Tumour Extension,
Contralateral kidney,
Regional Lymph nodes)
 MRI  Vascular Invasion

31.  CXR (PA view)  Lung metastasis
 CT-chest  In high risk cases

32.  If the tumour is too large for safe
primary resection and patient needs
neoadjuvant chemotherapy /
radiotherapy
 Bilateral tumours

33.  HIGHEST POSSIBLE CURE with
LOWEST Treatment Related
MORBIDITY

34.  Radical nephrectomy Via a Transabdominal
Incision for Stage I-IV + Avoidance of
spillage.
 Lymph Node dissection not recommended
 Tumour extending into the Vena Cava
Should be removed unless there are signs of
total obstruction.

35.  Stage V  Neoadjuvant Chemotherapy
followed by Nephron Sparing Surgery.

36. FAVOURABLE
 Stage I,II  Chemotherapy
(Vincristine & Dactinomycin)
 Stage III,IV  Chemotherapy
(Vincristine + Dactinomycin +
Doxorubicin) + Radiotherapy
 Stage V  Neoadjuvant
Chemotherapy + Surgery
NOT FAVOURABLE
 Stage I  Chemotherapy
(Vincristine & Dactinomycin)
 Stage II-IV  Chemotherapy
(Vincristine +Dactinomycin +
Cyclophosphamide + Etoposide)
+ Radiotherapy
 Stage V  Neoadjuvant
Chemotherapy + Surgery
HISTOPATHOLOGY

37.  Stage III,IV with favourable histology
 Stage II-IV with unfavourable histology.

38.  Multimodality approach has improved outcomes
 4-year survival of patients with favourable histology
Wilms tumour now approaches 90%
 2-year survival with Clear cell sarcomas is 60-90%
with addition of doxorubicin
 Poor prognosis of Rhabdoid and Anaplastic tumours.
 Bilateral Wilms tumour have a 3-year survival of 82%.

40. NEPHROBLASTOMA

41. Tumour thrombus transected?

42.  STAGE I  Limited to kidney & completely excised
 STAGE II  Extending beyond the kidney but
completely excised
 STAGE III  Unresectable tumour / Regional lymph
nodes (Non-hematogenous metastatic / Residual
tumour confined to abdomen)
 STAGE IV  Metastasis to LLBB / non-Regional LNs
 STAGE V  Bilateral Renal Involvement

44. Biopsy proven Nephroblastoma confined
to kidney?

45.  STAGE I  Limited to kidney & completely excised
 STAGE II  Extending beyond the kidney but
completely excised
 STAGE III  Unresectable tumour / Regional lymph
nodes (Non-hematogenous metastatic / Residual
tumour confined to abdomen)
 STAGE IV  Metastasis to LLBB / non-Regional LNs
 STAGE V  Bilateral Renal Involvement

47. Tumour confined to the kidney which
spilled locally during resection?

48.  STAGE I  Limited to kidney & completely excised
 STAGE II  Extending beyond the kidney but
completely excised
 STAGE III  Unresectable tumour / Regional lymph
nodes (Non-hematogenous metastatic / Residual
tumour confined to abdomen)
 STAGE IV  Metastasis to LLBB / non-Regional LNs
 STAGE V  Bilateral Renal Involvement

50. Tumour infiltrated local vitals
structures?

51.  STAGE I  Limited to kidney & completely excised
 STAGE II  Extending beyond the kidney but
completely excised
 STAGE III  Unresectable tumour / Regional lymph
nodes (Non-hematogenous metastatic / Residual
tumour confined to abdomen)
 STAGE IV  Metastasis to LLBB / non-Regional LNs
 STAGE V  Bilateral Renal Involvement

53. Tumour invaded the renal sinus vessels?

54.  STAGE I  Limited to kidney & completely excised
 STAGE II  Extending beyond the kidney but
completely excised
 STAGE III  Unresectable tumour / Regional lymph
nodes (Non-hematogenous metastatic / Residual
tumour confined to abdomen)
 STAGE IV  Metastasis to LLBB / non-Regional LNs
 STAGE V  Bilateral Renal Involvement

56. Tumour confined to kidney and spills in
the peritoneal cavity during resection?

57.  STAGE I  Limited to kidney & completely excised
 STAGE II  Extending beyond the kidney but
completely excised
 STAGE III  Unresectable tumour / Regional lymph
nodes (Non-hematogenous metastatic / Residual
tumour confined to abdomen)
 STAGE IV  Metastasis to LLBB / non-Regional LNs
 STAGE V  Bilateral Renal Involvement