[Unusual presentation of nephroblastomatosis].
BACKGROUND
Nephrogenic rests generally constitute precursor lesions of Wilms' tumor. We report a case of right nephroblastomatosis with dysmorphic features.


METHODS
An enlargement of the right kidney was incidentally discovered in a 1-year-old girl with dysmorphic features but normal psychomotor development. Combined ultrasonography and computerized tomography (CT) scan showed right cortical nephroblastomatosis. Chemotherapy using actinomycin D and vincristin was successful; however, an hyperechogenic nodule was subsequently found, necessitating a right nephrectomy.


CONCLUSIONS
The relationship between nephroblastomatosis and Wilms' tumor is discussed. This case report reminds us of the importance of a long-term follow-up including echography and CT scan in cases of nephroblastomatosis.
Service de pédiatrie, Centre Hospitalier, Albertville, France.
J
J
Santiago
R
R
Bouvier
J M
JM
Pouillaude
P
P
Thiesse
C
C
Bergeron
J P
JP
Chapuis
P
P
Cochat
J
J
Lespinasse
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007223:Infant; D007680:Kidney Neoplasms / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000628:therapy; D009392:Nephrectomy; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography; D009396:Wilms Tumor / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000628:therapy
Néphroblastomatose de présentation inhabituelle.