CANCER IN CHILDREN

NEPHROBLASTOMA
Nephroblastoma (Wilms Tumor) is the
commonest renal tumor in children
comprising 90% of renal cancer in this
group.
Survival of Wilms tumor patients has
improved to 90%
 Epidemiology and genetic

Annual incidence: 7 permillion children < 16 years

WT is found early childhood, median of 3,5 years at
diagnosis
Frequency appear equal in males and females
WT Strongly associated wit congenital anomalies
Aniridia,hemihyperthrophy and genitourinary
anomalies ( cryptorchidism, hypospadias, horseshoe
kidney). Specific syndrome such as Backwith-
Widemann, Denys-Drash and WAGR (Wilms tumor,
aniridia ,genitourinary abnormalities, mental
retardation)
Clinical presenation
The most common is the presence of an asymptomatic
abdominal mass
Associated sign and symptoms include:
malaise;abdominal pain gross or microscopic haematuria,
fever, anorexia and hypertension.
Abdominal pain may be result of local distention, intra
lesion haemorrhage, tumor rupture.
Hypertension 30-60%, aetiology renin like substance
Differential diagnosis: neuroblastoma, hepatoblastoma,,
renal sarcoma, multicystic dysplastic kidney.
Diagnosis
Physical examination should note the presence of
congenital anomalies (aniridia, hemihypertrophy,
genitourinary abnormalities), location and size of the
primary tumor, and measurement of blood pressure.
Complete blood count: urinalysis and blood chemistry
(creatinine, ureum, alkali phosphastase ), screening
coagulation.
Imaging study to identifying intra or extra renal
tumor, presence of normal function, tumor
thrombus,inferior vena cava and heart, pulmonary
metastases.
USG, CT Scan, MR all have their particular
advantages.
 Staging ( NWTS ; SIOP)
I Tumor limited to the kidney and completely excised

II Tumor extending outside the kidney, complete excised. Invasion

beyond the capsule, perirenal/perihilar.
No Lympnone involvement
III Invasion beyond the capsule; incomplete excision. Preoperative
biopsi; pre operative ruptur; peritoneal metastase.
Invasion of para oartic lymph node
IV Distant metastases

V Bilateral renal tumor

Prognostic factor

Significant prognostic variable: disease at

diagnosis (stage) and tumor histology.
Age at diagnosis and tumor size associated
with: relapse and death rates
Genetic abnormality and tumor cell DNA content
associated with prognosis is controversial
Treatment
Combined modality strategies using ;
surgery; radiotherapy and chemotherapy are the key
success in WT
Surgery: transabdominal, transperitoneal,large incision.
Radiotherapy: WT is radioresponsive. For stage III
favourable histology. Stage I IV clear cell sarcoma, and
stage II-IV diffusely anaplastic.
Dose of radiation approximately 10 Gy for local control
of both favourable histology and clear cell sarcoma.
Chemotherapy: Actinomycin D; Vincristine,Adriamycin,
cyclophosphamide and letter cisplatin, iphosphmid,
etoposide and carboplatin as being effective in WT.
Thank you