Wilm's Tumor (Nephroblastoma)

Bharat R Agarwal
Nephroblastoma - Staging
The National Wilm's tumor study group has given a clinicopathologic staging system. The pediatric surgeon determines the clinical stage in the operation theatre and pathological staging is confirmed by the pathologist.

Stage I: Tumor is limited to the kidney and is completely excised. It is seen in 43% of patients.

Stage II: Tumor extends beyond the kidney through the perirenal capsule and is incompletely excised. It is seen in 23 % of the patients.

Stage III: Residual tumor confined to the abdomen. No hematogenous involvement. It is seen in 23 % of the patients.

Stage IV: Hematogenous metastases to lung, liver, bone, brain, or combination of these sites. It is seen in 10 % of the patients.

Stage V: Bilateral renal involvement at the time of initial diagnosis. It is seen in 5 % of the patients. Each site should be staged according to the above criteria on the basis of the extent of the tumor prior to biopsy.

Though Wilm's tumor is curable in almost 90 % of the patients, the prognosis is related to the stage of the disease at diagnosis, the histologic features, age of the patients and the tumor size.

Treatment of Wilm's tumor consists of a combination of surgery (nephrectomy) followed by chemotherapy and in some patients, radiation therapy. It all depends on the histology and stage of the disease.

National Wilm's Tumor Study IV Recommendations are:
Stage I Favorable history or anaplastic: Nephrectomy + Chemotherapy (vincristine + Actinomycin D) x 6 months. No Radiotherapy

Stage II Favorable histology: Nephrectomy + chemotherapy (vincristine + Actinomycin D) x 15 months. No Radiotherapy.

Stage II (Anaplastic),III & IV: Nephrectomy + Radiotherapy + chemotherapy (vincristine + Actinomycin D + Adriamycin) x 15 months.

Clear cell sarcoma (stage I to V): Nephrectomy + Radiotherapy + chemotherapy (vincristine + Actinomycin D + Adriamycin) x 15 months

As stated earlier, abdominal ultrasound is done once in 6 months for two years and then yearly for an additional 1 to 3 years. Chest X ray should also be repeated at the same time. The patient with pulmonary disease at diagnosis should have a repeat CT scan every 3-6 monthly for 2 years and then yearly for 1-3 years.

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