Wilm's Tumor (Nephroblastoma)

Bharat R Agarwal
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Nephroblastoma - Diagnosis
A child who presents with a flank mass may have any of the following conditions:
- Neuroblastoma
- Hydronephrosis
- Multicystic kidney
- Abdominal lymphoma
On clinical suspicion, the following investigations should be done to confirm the diagnosis:

- Ultrasound of abdomen: to find out the tumor extent, calcification, lymph node, liver and opposite kidney status.
Abdominal CT scan: for evidence of bilateral involvement, involvement of blood vessels with tumor.
Other investigations which are required are Biochemical profile, Chest X- ray, CBC, bone marrow aspiration (to rule out clear cell carcinoma of the kidney).

Also, since the Wilm's tumor gene, WT-1 is located on the short arm of chromosome 11(band 11p13), it may be associated with congenital anomalies like aniridia, hemihypertrophy and Beckwith- Wiedmann syndrome.

Infact, it is now recommended that patients with aniridia or hemihypertrophy should be screened with Ultrasound every 3months until the are 6 years of age. Also, children with Beckwith-Wiedemann syndrome should be screened with ultrasound every 3 months until they are 7years old.

Hereditary Wilm's tumor (presents as either bilateral tumor or with a family history of the neoplasm) is uncommon with only 4 to 5 % of patients having bilateral tumor and 1 to 2 % having positive family history of Wilm's tumor. The chance of bilateral Wilm's tumor occurring is higher in children in whom the original unilateral Wilm's tumor was diagnosed at less than 12 month of age or in whom the resected kidney contained nephrogenic rests.

Hence it is recommended that for early detection of metachronous bilateral Wilm's tumor, periodic abdominal ultrasound should be done in following patients:
- Children with nephrogenic rests in the resected kidney (if < 48 months of age at initial diagnosis) - every 3 months for 6 years.
- Children with nephrogenic rests in the resected kidney (if > 48 month of age at initial diagnosis) - every 3 months for 4 years.
- Other patients - every 6 months for 2 years and then yearly for an additional 1 to 3 years.

Histopathology
Although most patients with a histopathological diagnosis of Wilm's tumor do well with treatment, there are 2 categories of unfavorable histology:
- Anaplasia
- Sarcomatous changes (includes clear cell sarcoma of the kidney and malignant rhabdoid tumor of the kidney.)


Wilm's Tumor (Nephroblastoma) Wilm's Tumor (Nephroblastoma) 01/04/2001
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