4th Pediatric Infectious Diseases Conference
 
 
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Pedi Poll
Today's Poll
Should teicoplannin, colistin be used in case of neonatal sepsis where culture does not reveal any organism_?
No, it should be used only after drug sensitivity report
Yes, under guidance of an infectious disease expert
WILM'S TUMOR (NEPHROBLASTOMA)
Wilm's Tumor (Nephroblastoma)
Dr Ira Shah
M.D, DCH(Gold Medalist), FCPS, DNB
Edited by Dr. Bharat R Agarwal
Consultant Pediatric Hematologist and Oncologist.


Q: What is Wilm's tumor?

A: Wilm's tumor is the second most common abdominal tumor and it makes up to 6% of all childhood cancer.

Q: What are its clinical features?

A: Wilm's tumor commonly occurs as an abdominal or a flank mass with abdominal pain, hematuria, hypertension. There may be associated findings of fever, anorexia, vomiting and features suggestive of metastatic disease to the lung, liver and brain.

Q: How is Wilm's tumor diagnosed?

A: A child who presents with a flank mass may have any of the following conditions: -

  1. Neuroblastoma

  2. Hydronephrosis

  3. Multicystic kidney

  4. Abdominal lymphoma etc.
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-

  1. Children with nephrogenic rests in the resected kidney (if < 48 months of age at initial diagnosis ) - every 3 months for 6 years.

  2. Children with nephrogenic rests in the resected kidney (if > 48 month of age at initial diognosis) - every 3 months for 4 years.

  3. Other patients - every 6 months for 2 years and then yearly for an additional 1 to 3 years.

 
 
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