Kidney

Question 1

Which two pediatric tumors have a t(12;15) translocation?

Answer 1

Congenital mesoblastic nephroma and fibrosarcoma

Question 2

BQ! What disease is this EM showing?

Answer 2

Fabry’s Disease: X-linked lysosomal storage disease that is caused by deficient activity of lysosomal enzyme α -galactosidase A. Abnormal storage product, “zebra
bodies” are seen in a podocyte.

Question 3

What is the syndrome associated with WT1? WT2?

Answer 3

WAGR syndrome (WT-1)

– Wilms tumor

– Aniridia

– Genital anomalies

– Mental Retardation

Beckwith-Wiedemann syndrome (WT-2)

• Wilms tumor
• Hepatoblastoma
• Congenital anomalies

– Macroglossia

– Macrosomia

– Midline abdominal wall defects

• Ear abnormalities
• Neonatal hypoglycemia