Renal findings on radiological followup of patients with Beckwith- Wiedemann syndrome

Joseph G. Borer, Martin Kaefer, Carol E. Barnewolt, Ellen R. Elias, Nedda Hobbs, Alan B. Retik, Craig A Peters

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Abstract

Purpose: The Beckwith-Wiedemann syndrome is most commonly characterized by macroglossia and abdominal wall defect(s), and it carries a predisposition to embryonal tumors, including Wilms tumor. We report our experience with the character and incidence of renal disease in patients with the Beckwith- Wiedemann syndrome, and discuss the role of radiological followup. Materials and Methods: We reviewed the medical records of all patients diagnosed with the Beckwith-Wiedemann syndrome who were treated at our institution between March 1979 and February 1998. Radiological followup consisted of renal ultrasound at approximately 3 to 6-month intervals with the addition of computerized tomography or magnetic resonance imaging (MRI) in patients with an indeterminate lesion(s) or nephrogenic rest(s). Results: A total of 29 patients were identified. Of these cases renal ultrasound showed normal kidneys bilaterally in 19 (70%), simple cysts in 5 (19%), indeterminate lesion(s) in 2 (7%) and nephrocalcinosis in 1 (4%). Nephrogenic rests were followed with MRI in 1 patient, and 1 in whom a 2 cm. mass was revealed by followup MRI underwent partial nephrectomy and chemotherapy for stage I Wilms tumor. Conclusions: The 3.7% incidence of Wilms tumor in our patients with the Beckwith-Wiedemann syndrome is similar to that in previously published reports. Aggressive followup by a sensitive radiological technique is warranted in cases of the Beckwith-Wiedemann syndrome, and associated hemihypertrophy and/or nephromegaly with or without evidence of a Wilms tumor precursor. The detection of suspected malignant disease at an early stage may permit curative nephron sparing surgery.

Original languageEnglish (US)
Pages (from-to)235-239
Number of pages5
JournalJournal of Urology
Volume161
Issue number1
DOIs
Publication statusPublished - Jan 1 1999

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Keywords

  • Beckwith-Wiedemann syndrome
  • Kidney
  • Nephroblastoma

ASJC Scopus subject areas

  • Urology

Cite this

Borer, J. G., Kaefer, M., Barnewolt, C. E., Elias, E. R., Hobbs, N., Retik, A. B., & Peters, C. A. (1999). Renal findings on radiological followup of patients with Beckwith- Wiedemann syndrome. Journal of Urology, 161(1), 235-239. https://doi.org/10.1016/S0022-5347(01)62118-9