Wilms' tumor and gonadal dysgenesis in a child with the 2q37.1 deletion syndrome.
Here we report Wilms' tumor, gonadal dysgenesis and a bifid uterus in an 18-month-old female with a terminal deletion of the long arm of chromosome 2 [46,XX,del(2)(q37.1)]. Since Wilms' tumor has been previously reported in the 2q37 deletion syndrome, the present observation raises the question of whether a tumor susceptibility gene maps to chromosome 2q37 and suggests giving consideration to the possible occurrence of Wilms' tumor in the course of disease.
Viot-Szoboszlai
G
G
Département de Génétique et Unité INSERM 393, Hôpital Necker-Enfants Malades, Paris, France.
Amiel
J
J
Doz
F
F
Prieur
M
M
Couturier
J
J
Zucker
J N
JN
Henry
I
I
Munnich
A
A
Vekemans
M
M
Lyonnet
S
S
D000015:Abnormalities, Multiple / Q000235:genetics*; D002872:Chromosome Deletion; D002889:Chromosomes, Human, Pair 2; D004268:DNA-Binding Proteins / Q000235:genetics; D005260:Female; D016162:Genes, Wilms Tumor; D006059:Gonadal Dysgenesis / Q000235:genetics*; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D007223:Infant; D013577:Syndrome; D014157:Transcription Factors / Q000235:genetics; D014599:Uterus / Q000002:abnormalities; D025721:WT1 Proteins; D009396:Wilms Tumor / Q000235:genetics*
D004268:DNA-Binding Proteins; D014157:Transcription Factors; D025721:WT1 Proteins