Abstract
Mulibrey-Nanism (Muscle-liver-brain-eye nanism= dwarfism) is a rare
syndrome. The underlying TRIM37 mutation predisposes these children to
develop tumors frequently. Wilms tumor has been reported to be
associated with this syndrome. Here we report a 2-year-old boy with
Wilms tumor and Mulibrey-Nanism and review the published similar cases.
Our patient had associated atrial septal defect, atrial flutter and an
episode of cardiac arrest along with other features of Mulibrey Nanism.
We managed him successfully with chemotherapy and surgery. A total of 14
cases (including present case) of Wilms tumor have been reported in
Mulibrey-Nanism. Longest survivor has been 15-year-old.