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

Research output: Contribution to journalArticle

25 Citations (Scopus)

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
StatePublished - Jan 1999
Externally publishedYes

Fingerprint

Beckwith-Wiedemann Syndrome
Wilms Tumor
Kidney
Magnetic Resonance Imaging
Macroglossia
Nephrocalcinosis
Incidence
Nephrons
Abdominal Wall
Nephrectomy
Medical Records
Cysts
Tomography
Drug Therapy

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

Renal findings on radiological followup of patients with Beckwith- Wiedemann syndrome. / Borer, Joseph G.; Kaefer, Martin; Barnewolt, Carol E.; Elias, Ellen R.; Hobbs, Nedda; Retik, Alan B.; Peters, Craig A.

In: Journal of Urology, Vol. 161, No. 1, 01.1999, p. 235-239.

Research output: Contribution to journalArticle

Borer, JG, Kaefer, M, Barnewolt, CE, Elias, ER, Hobbs, N, Retik, AB & Peters, CA 1999, 'Renal findings on radiological followup of patients with Beckwith- Wiedemann syndrome', Journal of Urology, vol. 161, no. 1, pp. 235-239. https://doi.org/10.1016/S0022-5347(01)62118-9
Borer, Joseph G. ; Kaefer, Martin ; Barnewolt, Carol E. ; Elias, Ellen R. ; Hobbs, Nedda ; Retik, Alan B. ; Peters, Craig A. / Renal findings on radiological followup of patients with Beckwith- Wiedemann syndrome. In: Journal of Urology. 1999 ; Vol. 161, No. 1. pp. 235-239.
@article{3552e6718ede44fe9cd3acff597169b2,
title = "Renal findings on radiological followup of patients with Beckwith- Wiedemann syndrome",
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.",
keywords = "Beckwith-Wiedemann syndrome, Kidney, Nephroblastoma",
author = "Borer, {Joseph G.} and Martin Kaefer and Barnewolt, {Carol E.} and Elias, {Ellen R.} and Nedda Hobbs and Retik, {Alan B.} and Peters, {Craig A.}",
year = "1999",
month = "1",
doi = "10.1016/S0022-5347(01)62118-9",
language = "English (US)",
volume = "161",
pages = "235--239",
journal = "Journal of Urology",
issn = "0022-5347",
publisher = "Elsevier Inc.",
number = "1",

}

TY - JOUR

T1 - Renal findings on radiological followup of patients with Beckwith- Wiedemann syndrome

AU - Borer, Joseph G.

AU - Kaefer, Martin

AU - Barnewolt, Carol E.

AU - Elias, Ellen R.

AU - Hobbs, Nedda

AU - Retik, Alan B.

AU - Peters, Craig A.

PY - 1999/1

Y1 - 1999/1

N2 - 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.

AB - 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.

KW - Beckwith-Wiedemann syndrome

KW - Kidney

KW - Nephroblastoma

UR - http://www.scopus.com/inward/record.url?scp=0032877567&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0032877567&partnerID=8YFLogxK

U2 - 10.1016/S0022-5347(01)62118-9

DO - 10.1016/S0022-5347(01)62118-9

M3 - Article

VL - 161

SP - 235

EP - 239

JO - Journal of Urology

JF - Journal of Urology

SN - 0022-5347

IS - 1

ER -