Feasibility of partial nephrectomy for Wilms' tumor in children with Beckwith-Wiedemann syndrome who have been screened with abdominal ultrasonography.
BACKGROUND
Children with Beckwith-Wiedemann syndrome (BWS), a congenital syndrome associated with Wilms' tumor commonly are screened with abdominal sonography resulting in detection of tumor at a lower stage. Wilms' tumors have been traditionally treated with complete nephrectomy; however, smaller tumors are amenable to nephron-sparing surgery. Because Wilms' tumors may be metachronous and nonmalignant disease may compromise renal function in BWS, nephron-sparing approaches may be desirable as the first option.


METHODS
Seven patients with BWS and Wilms' tumor underwent nephrectomy. The preoperative computed tomography (CT) or ultrasound scan were evaluated by a pediatric surgeon to assess whether partial nephrectomy would have been feasible. The determining criteria included tumor involving one third or less of the kidney and no involvement of either hilar or vascular structures.


RESULTS
Seven patients underwent complete nephrectomies. The remaining patient, who had undergone a left nephrectomy before the initiation of screening had salvage chemotherapy after biopsy results showed right kidney involvement with Wilms' tumor.


CONCLUSIONS
Nephron-sparing surgery is reasonable to consider in children with Beckwith-Wiedemann syndrome who are screened at intervals of 4 months or less. The relative benefits of partial nephrectomy for children with Wilms' tumor-predisposing conditions only can be assessed in the setting of a cooperative clinical trial.
McNeil
D Elizabeth
DE
Division of Cancer Epidemiology and Genetics, Genetic Epidemiology Branch, National Institutes of Health, Bethesda, Maryland, USA.
Langer
Jacob C
JC
Choyke
Peter
P
DeBaun
Michael R
MR
D001506:Beckwith-Wiedemann Syndrome / Q000150:complications*; D002675:Child, Preschool; D005240:Feasibility Studies; D006801:Humans; D007223:Infant; D007680:Kidney Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D009392:Nephrectomy / Q000379:methods*; D014463:Ultrasonography; D009396:Wilms Tumor / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*