NEPHROBLASTOMA (WILM'S TUMOUR)

Question 1

Nephroblastoma definition and peak incidence

Answer 1

*It is a malignant embryonal tumor of the kidney and the commonest malignancy of the urinary tract of children.
*peak incidence is 3 years

Question 2

Name 5 associated syndromes

Answer 2

*a. WAG: Wilms tumor, Aniridia, Genitourinary abnormalities (hypospadias, cryptorchidism) &Mental Retardation
*b. Denys-Drash syndrome: male pseudo hermaphrodism, early onset of renal failure and wilm’s tumor
*c. Beckwith Wiedemann: Omphalocele, Macroglossia and Visceromegaly
*d. Simpson Golabi Behemel Syndrome:
overgrowth, mental impairment, craniofacial anomalies.

Question 3

Which gene is associated with this disease

Answer 3

• Wilm’s tumor are WTI gene (located on 11p13) required for normal kidney and gonadal development.

Question 4

Clinical Features of nephroblastoma

Answer 4

• abdominal masses that rarely cross midline,
• abdominal pain,
• hematuria
• hypertension
• fever, nausea, vomiting, anorexia

Question 5

INVESTIGATIONS of nephroblastoma

Answer 5

** Intravenous Urogram (most important as ti shows the distorted calyces and pelvises)
* CT scan, Chest Xray (to check for metastasis to lungs),
*abdominal ultrasound
* Chromosomal o
analysis, Percutaneous needle biopsy

Question 6

Treatment

Answer 6

*Surgery,
*Chemotherapy
*Radiotherapy