Screening for Wilms tumor in children with Beckwith-Wiedemann syndrome or idiopathic hemihypertrophy

Choyke, Peter L.; Siegel, Marilyn J.; Craft, Alan; Green, Daniel M.; DeBaun, Mph Michael R.

doi:10.1002/(sici)1096-911x(199903)32:3<196::aid-mpo6>3.0.co;2-9

Cited by 144 publications

(66 citation statements)

References 16 publications

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“…Regarding screening for Wilms tumor, ultrasound tests obtained less frequently than every 3-4 months may not help decrease tumor stage secondary to their rapid rate of growth [28,63]. Given the low incidence of Wilms tumor, the frequent testing needed to reduce tumor stage, and the lack of evidence for improvement in mortality rates with monitoring, it is our opinion that routine screening for Wilms tumor is not supported by the present data.…”

Section: Ultrasoundcontrasting

confidence: 60%

Management and etiology of the unilateral multicystic dysplastic kidney: a review

et al. 2009

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In children, unilateral multicystic dysplastic kidney (MCDK) is one of the most frequently identified urinary tract abnormalities. A variety of proposed etiologies has been associated with the underlying pathogenesis of MCDK. These include genetic disturbances, teratogens, in utero infections, and urinary outflow tract obstruction. From 5-43% of the time, MCDK has associated genito-urinary anomalies, both structural and functional in nature. A review of the literature reveals that involution rates are reported to be 19-73%, compensatory hypertrophy of the contralateral kidney occurs from 24-81% of the time, and estimated glomerular filtration rates (GFRs) (by the Schwartz formula) range from 86-122 ml/min per 1.73 m(2) body surface area. Most authors suggest serial ultrasonography to monitor contralateral growth, routine blood pressure monitoring, and a serum creatinine monitoring algorithm. The risk of hypertension in those with MCDKs does not appear to be greater than that of the general population, and the rates of malignant transformation of MCDK are small, if at all increased, in comparison with those in the general population. If the patient develops a urinary tract infection or has abnormalities of the contralateral kidney, shown on ultrasound, a voiding cystourethrogram is recommended. Finally, the body of literature does not support the routine surgical removal of MCDKs.

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Section: Ultrasoundcontrasting

confidence: 60%

Management and etiology of the unilateral multicystic dysplastic kidney: a review

et al. 2009

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“…Beckwith-Wiedemann and Denys-Drash syndrome; see text on nephroblastomatosis) [1,3,6,9,12,39,40,41,41,43,46,47,48,49,50]. Ultrasound screening for Wilms' tumour is suggested in all risk populations until (and throughout) school age (at approximately 6-to 12-month intervals) and based on the hypothesis that the detection of tumour at an early stage may decrease morbidity and permit curative nephron-sparing surgery [42,51]. Additionally, there is a high risk of renal failure in some conditions such as Denys-Drash syndrome and Wilms' tumour aniridia syndromes [47,52].…”

Section: Malignant Tumoursmentioning

confidence: 99%

Imaging of renal tumours in infancy and childhood

Riccabona

2003

Eur Radiol

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There is a large range of benign and malignant renal neoplasms. This article lists the most benign, indeterminate and malignant renal tumours that occur during infancy and childhood. It briefly describes the most important entities, including their imaging features, and important complications. Differential diagnosis and pitfalls are discussed, and a brief suggestion for a standardised imaging algorithm is proposed. Although modern imaging techniques, including colour Doppler sonography, helical or multidetector CT, and MR have significantly improved imaging potential, the definite diagnosis on tumour entity still is established only by histology.

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“…The nephromegaly is probably 'benign', as the kidney has retained its reniform configuration on CECT and normal isotope distribution on the DMSA scan. Given the increased risk of abdominal tumours with HH and reports as late as 8 years of age, it has been recommended that all children with HH be screened at 4-monthly intervals with ultrasound and followed throughout childhood [9]. The National Wilms Tumor Study Group has reported more stage 1 and small diameter tumours in those detected at radiological screening [7].…”

Section: Discussionmentioning

confidence: 99%

“…The National Wilms Tumor Study Group has reported more stage 1 and small diameter tumours in those detected at radiological screening [7]. However, one is cautioned against false positives in screening, and nephrectomy for benign disease [9] and histological confirmation should precede ablative surgical intervention.…”

Section: Discussionmentioning

confidence: 99%

Hemihypertrophy, renal dysplasia and benign nephromegaly

et al. 2005

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Hemihypertrophy is associated with malignant visceral abdominal neoplasms in childhood. Benign nephromegaly and nephroblastomas are both known to occur with hemihypertrophy; however, association with renal dysplasia has not previously reported. We describe an infant presenting with recurrent haematuria who had segmental hemihypertrophy, ipsilateral renal dysplasia and contralateral benign nephromegaly. Although debated, renal dysplasia may predispose to and predate malignant change. Rational management and optimal surveillance of renal dysplasia and benign nephromegaly in hemihypertrophy is discussed.

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Screening for Wilms tumor in children with Beckwith-Wiedemann syndrome or idiopathic hemihypertrophy

Cited by 144 publications

References 16 publications

Management and etiology of the unilateral multicystic dysplastic kidney: a review

Management and etiology of the unilateral multicystic dysplastic kidney: a review

Imaging of renal tumours in infancy and childhood

Hemihypertrophy, renal dysplasia and benign nephromegaly

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