Renal Tumors in Children- Overview (Quick review of renal tumors)

1. PEDIATRIC RENAL TUMORS
Dr. Vidhya T
MBBS, DNB, Fellowship in Pediatric Urology
Division of Pediatric Urology
Apollo children’s hospital

2. SPECTRUM OF PEDIATRIC RENAL TUMORS
NEONATES
Mesoblastic Nephroma
6months-5years
Wilms’ Tumor
Nephroblastomatosis
Medullary tumors
Clear cell sarcoma
Rhabdoid tumor of kidney
Multilocular cystic nephroma
>5yrs
Renal cell carcinoma
RCC variants

3. WILM’S TUMOR
Most common renal tumor in children (85%)
4th most common malignancy in childhood
5-7% of overall pediatric tumors
Malignant embryonal tumor originating from nephrogenic blastema,
Imitates the histology of developing kidney
Named after the German surgeon Max Wilms

4. WILM’S TUMOR
DEMOGRAPHY
Age – PRESCHOOL CHILDREN 3-4yrs
Sex- Slight female preponderence
STATISTICS
Majority- non syndromic/sporadic Wilm’s
15% associated with syndromes and congenital anomalies
1 - 2% familial
5-10% bilateral

5. WILMS’ TUMOR GENETICS
Chromosone 11, short arm- Wilm’s tumor gene
Chr. 11p13 WT1
Chr. 11p15 WT2
Others
CTNNB1
TP53
IGF2
MYCN
Gain of 1q

6. GUESS?
Syndrome 1
Syndrome 3
Syndrome 2

7. WT 1 WT2
SYNDROMES

8. SYNDROMES with WILM’S TUMOR RISK
Most common predisposition syndromes and conditions with different risk of Wilms
tumor include:
High risk (> 20%)
WAGR (Wilms tumor - aniridia - genitourinary anomalies - intellectual disability)
Denys-Drash syndrome
Moderate risk (5 - 20%)
Beckwith Wiedemann syndrome
Simpson-Golabi-Behmel syndrome
Frasier syndrome
Low risk (< 5%)
Bloom syndrome
DICER1 syndrome
Li-Fraumeni syndrome
Isolated hemihypertrophy

9. TUMOR PATHOLOGY
TRIPHASIC pattern- classic of Wilms’ tumor, favourable outcome
 Epithelial component
 Stromal component
 Blastemal component

10. ANAPLASTIC WILM’S
FEATURES- Enlarged nucleoli
Hyperchromasia
Increased mitotic figures
Focal anaplasia
Diffuse anaplasia
TP53mutations
Poor outcome

11. Nephrogenic rests are precursor lesions
Can be multiple or diffuse- Nephroblastomatosis
Nephrogenic rests are found in 40% of unilateral and > 90% of bilateral Wilms tumors
2 main types of nephrogenic rests:
Perilobar nephrogenic rests
Intralobar nephrogenic rests
Perilobar nephrogenic rests in children under 1 year of age are associated with a markedly
increased risk of developing a contralateral Wilms tumor
PATHOPHYSIOLOGY

12. PATHOPHYSIOLOGY

13. PRESENTATION
Abdomen mass(most common)
Most often found when dressing, bathing, or changing child’s diaper
Others (20-30%)
o Hematuria
o Abdomen pain
o Fever
o Hypertension
o Anemia
o Varicocele

14. LABS
Anemia
Polycythemia
Thrombocytenia
Acquired Von Willebrand defiency
Renal function- essential normal, renal insufficeincy in bilateral extensive wilm’s
Urine microscopy-:RBC+

15. IMAGING
Ultrasound
CECT Whole abdomen
Chest Xray
CT chest

16. COG ( NWTS+Pediatric oncology
group)
SIOP
ORIGIN USA, Canada
NWTSG- 5 trials
Europe
STRATEGY Surgery>>Chemo+/- Radiotherapy Chemo first>> surgery>> Adjuvant
Chemo+/-radiotherapy
STAGING Stage I-IV Localises (Stage I-III), Metastaic
(stage IV)
EXCEPTIONS Chemo>Surgery <6mon of age
PROS Avoid chemotherapy
In Benign lesions
In different histological type of
malignant tumor
Downsizing of tumor pre op
Surgery made simpler
Decreased riskmof tumor rupture
Avoid
Modification in tumor histology
Loss of staging information
Makes nephron sparing possible
REMEMBER UPFRONT NEPHRECTOMY NEOADJUVANT CHEMOTHERAPY
COG/SIOP

17. SURGERY- Radical nephroureterectomy with lymph node sampling
CHEMOTHERAPY- EE4A, DD4A, REGIMEN I, REGIMEN M
RADIOTHERAPY- localised, whole body irradiation
WILM’S TREATMENT

18. TUMOR THROMBUS

19. LYMPH NODE SAMPLING

20. COG STAGING

21. WILM’S CHEMOTHERAPY REGIMEN

22. RECOMMENDATIONS

23. RECOMMENDATIONS

24. SURVIVAL RATES

25. MESOBLASTIC NEPHROMA
Most common in neonatal period
Diagnosed before 3months of age while Wilms not before 6months
Frequently benign
Malignant variants do occur with mets to lung, liver, heart, brain
Can be diagnosed with prenatal ultrasound- Polyhydramnios, hydrops, preterm delivery
Rx RADICAL NEPHRECTOMY

26. CLEAR CELL SARCOMA OF KIDNEY
Uncommon
Presnts between1-4yrs as abdomen mass
BONE METS most common
Lung, abdomen, retroperitoneum, brain, liver

27. RHABDOID TUMOR OF KIDNEY
Rare, aggressive tumor of kidney
Deletions and mutations in SMARCB1/
Mets to Brain, lungs
Poor prognosis with current therapeutic protocols
NWTS-5 4y/ Overall survival
Stage 1 50%
Stage II/III 33%
Stage IV 21%

28. RENAL CELL CARCINOMA
Not common in childhood
Seen in children >10yrs of age
Prevalent during 2nd decade of life
Syndromic association- Von Hippel Lindau, Tuberous sclerosis, Hereditary leimyomatosis
Renal medullary Carcinoma- sickle cell trait
Most common subtype of children in RCC is-TRANSLOCATION TYPE RCC (TFE3gene)
Seen I childhood cancer survivors, as secondary malignancies in children post chemo/ transplant
RCC propensity to meatstatsize-Lungs, liver, bone
Nephrectomy
Chemo/Radio ??

29. THANK YOU!