2. It is also known as NEPHROBLASTOMA.
It is the most common primary malignant renal tumor of childhood.
It is the 2nd m.c intra-abdominal tumor in childhood.
Peak age-2-5 yrs(1% in neonate and occoasionally in adulthood).
MOSTLY unilateral ,in 7% cases bilateral.
MOSTLY sporadic,1-2%familial(Familial is highly bilateral,aggressive).
MALE:FEMALE RATIO IS 0.7:1
6. WT arises from Nephrogenic rests(undifferentiated mesenchymal cell)
IT displaces intrinsically and deforms the pelvical system , no intraspinal extension.
GROSSLY-large,soft,solitary,rounded homogenous multinodular
mass with occasionally hemmorrhage, cyst formation and
necrosis.
HISTOLOGY-classic triphasic combination-
• 1-Blastemal component-sheets of small blue cells.
• 2-Epithelial component-tubules or glomeruli.
• 3-Stromal component-Fibrocystic/myxoid/skeletal muscle
Anaplasia seen in 5% patients.
7. :-
Homozygous mutation of WT1 gene,located in 11p13,is seen in
10-15% of tumour resulting in loss of function of encoded zinc
finger transcripition factor.mostly these aresomatic
mutation,germline mutation is assosciated with WAGR
syndrome.
Wnt signaling pathway play a crucial role in differentiation in fetal
kidney.2 proteins i)CTNNB1 $ii)WTX protein play important role
in this pathway.15% case,CTNNB1 mutation seen and in
20%case,WTX protein mutation seen.
Somatic mutation of p53 gene,TP53,is seen in 5% tumour
assosciated with anaplastic tumour.
70% cases of wt,loss of heterozygosity or loss of imprinting at
8.
9.
10. • 1-Asymptomatic large Abdominal mass(m.c presentation)
• 2-Hypertension(25%)due to high renin
• 3-Abdominal pain, gross hematuria , fever
• 4-Anaemia and otherhematological
abnormalities(vWD,Thrombocytosis)
• 5-WT thrombous extend into inferior venacavain4-10%case and rarely
into right atrium,dislodgement ofintravascular tumor lead to pulmonary
embolism
• 6-Lack of appetite
• 7-Nausea
• 8-Constipation
-
• Sites-3L-lungs,liver,lymph node.
• Bony metastasis only in clear cell sarcoma histology.
11. -
• USg abdomen differentiate solid from cystic masses
• Ct scan is most useful to define extent of disease,metastasis and contralateral kidney
involvment.May sometimes show stippled appearance.
• MRI done to know about vascular extension and to distinguish wt from nephrogenic rests.
• PET IS USEFUL as it is metabolically active.
• BIOPSY avoided as it can upstage disease.Core needle biopsy is done in case of unusual
presentations(>10 yrs ,signs of infection,inflammation)or unusual imaging findings(significant
adenopathy,intratumoral classification,no renal parenchyma seen).
• Histology is determined after surgical removal
12.
13.
14.
15.
16. TWO REGIMEN:-
1-COG(Children’s Oncology Group) protocol:-upfront surgery
prior to initiating tratment as early surgery provides accurate
diagnosis and staging and facilitate RISK ADAPTED THERAPY.
2-SIOP(International society of paediatric) protocol:-
recommends preoperative chemotherapy as preoperative chemo
therapy can make surgery easier and reduces the risk of
intraoperative tumour rupture and hemorrhage.
Each approach has some benefits and limitation but both have
same outcome.
17. RADICAL NEPHRECTOMY is needed in all patients.
PARTIAL NEPHRECTOMY is perfomed in patients with bilateral disease or withunilateral wilms tumour and
predisposing syndrome.
NEPHRECTOMY ALONE is indicated in <2 yrs of age with stage 1 disease and a tumour weighing <550
GMS.
Patient with stage l and ll :-Chemotherapy with 2 drugs ,Vincristine and Actinomycin D(RegimenEE4A)every
1-3 wk for a total of 18 wks.
Patient with stage lll or lV disease:-Chemotherapy with 3 drugs Vincristine,doxorubicin and ActinomycinD
(RegimenDD4A) EVERY 1-3 wk for total of 24 wks +Radiotherapy
Radiation therapy is indicated in metastasis,residual disease after surgery or tumour rupture
.
Patient with diffuse anaplasia are treated with intensive chemo therapy regimens that include
vincristine,cyclophospamide,doxorubicin,etoposide,carboplatinand ifosfamide ina addition to radiotherapy.
18. :-
Relapse occour early within 2 yrs of diangosis.
Seen in 15%of favorable histology and 50% of anaplastic histology.
FACTORS A/W FAVORABLE OUTCOME AFTER RELAPSE:-
• i-low stage at diagnosis,
• ii-T/t with vincristin and actinomycinD only,
• iii-no prior radiotherapy
• iv-favorable histology
• v-relapse to lung only
AGENTSused in recurrent disease;-
doxorubicin,carboplatin,cyclophospamide,ifosfamide and topotecan.
:-
• Survival of children with wilms tumour exceeds 90%.
