CANCER IN CHILDREN

NEPHROBLASTOMA

Nephroblastoma (Wilm’s Tumor) is the commonest renal tumor in children

comprising 90% of renal cancer in this group.

Survival of Wilm’s 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 anomaliesAniridia,hemihyperthrophy and genitourinary anomalies ( cryptorchidism, hypospadias, horseshoe kidney). Specific syndrome such as Backwith-Widemann, Denys-Drash and WAGR (Wilm’s 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.

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