WT1 expression and hemihypertrophy in congenital mesoblastic nephroma.
Congenital mesoblastic nephroma (CMN) is a rare primary pediatric renal tumor occurring predominantly in infants. There is no known association between CMN and WT1 gene expression and the association of hemihypertrophy and CMN is not well known. We report an infant with isolated hemihypertrophy and WT1-positive CMN, and the results of WT1 immunostaining in 13 other patients with CMN diagnosed over 14 years at SickKids. Of the 14 total patients 3 had positive nuclear immunostaining for WT1. Two patients also expressed WT1 RNA by reverse transcription-polymerase chain reaction. In conclusion, contrary to previous reports, WT1 may be expressed in CMN and CMN can be associated with hemihypertrophy in the absence of Beckwith-Wiedemann syndrome.
Abosoudah
Ibraheem
I
Division of Pediatric Hematology/Oncology, The Hospital for Sick Children (SickKids), University of Toronto, Toronto, Ontario, Canada. ibraheem.abosoudah@sickkids.ca
Ngan
Bo-Yee
BY
Grant
Ronald
R
Weitzman
Sheila
S
D006801:Humans; D006984:Hypertrophy / Q000209:etiology*; D007223:Infant; D007866:Leg; D008297:Male; D018201:Nephroma, Mesoblastic / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D025721:WT1 Proteins / Q000032:analysis* / Q000235:genetics
D025721:WT1 Proteins