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 BackwithWidemann, 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

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