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Ascertainment of the Incidence of Beckwith-Wiedemann Syndrome in the National Wilms Tumor Study Group

Institute of Child HealthUniversity of BirminghamBirmingham, United Kingdom
Institute of Child HealthLondon, United Kingdom

To the Editor:In the study by Porteus et al published in the May 2000 issue of the Journal of Clinical Oncology,¹ the authors assume a correct diagnosis of Beckwith-Wiedemann syndrome (BWS) from National Wilms Tumor Study Group (NWSTG) data sheets completed by registering physicians at the time of diagnosis. Apparently, there was no systematic attempt to confirm the diagnosis of BWS—a crucial point because several other rare but important "overgrowth" syndromes, notably Perlman syndrome² and Simpson-Golabi-Behmel syndrome, are associated with Wilms’ tumor and clinical distinction can be difficult. To emphasize the point, careful clinical examination of an unselected group of children with Wilms’ tumor presenting to one of our institutions revealed a higher-than-expected level of congenital and developmental abnormalities, including isolated hemihypertrophy.³

If our supposition about the data collection is correct, it is likely that the NWTSG survey has underestimated the number of patients with overgrowth syndromes and/or incorrectly ascribed all such cases to BWS. The data provided by Porteus et al are, therefore, potentially valuable, but some "fine tuning" is needed before they can be regarded as accurate.

REFERENCES

1. Porteus MW, Narkool P, Neuberg D, et al: Characteristics and outcome of children with Beckwith-Wiedemann syndrome and Wilms’ tumor: A report from the National Wilms Tumor Study Group. J Clin Oncol 18: 2026-2031, 2000[Abstract/Free Full Text]

2. Grundy RG, Pritchard J, Baraister M, et al: Perlman and Wiedemann-Beckwith syndromes: Two distinct conditions associated with Wilms tumour. Eur J Pediatr 151: 895-898, 1992[Medline]

3. Grundy RG, Blacklay A, Cole T, et al: A review of 60 consecutive cases of Wilms tumour with documentation of the dysmorphic syndromes and associated clinical features. Med Pediatr Oncol 35: 126-130, 2000[Medline]

Response

Dana-Farber Cancer InstituteBoston, MA

In Reply:We agree with Drs Grundy and Pritchard that one must analyze the data presented in our report¹ in light of the methodology used. They are correct that central review of the cases, in terms of the presence or absence of overgrowth syndromes, was not performed. Patients were classified as having Beckwith-Wiedemann syndrome (BWS) if they were reported as such by their primary treating oncologist. This could have resulted in misclassification and an overestimate or underestimate of the number of BWS patients in the data set. Clinical examination of the patients suspected of having BWS was beyond the scope of our study. One must interpret the data regarding Wilms’ tumor and BWS keeping these limitations in mind. A possible strength of this method is that patients were classified as having BWS if their clinical oncologist thought they had BWS. This may be an imprecise but clinically relevant way of looking at the problem.

REFERENCES

1. Porteus MH, Narkool P, Neuberg D, et al: Characteristics and outcome of children with Beckwith-Wiedemann syndrome and Wilms’ tumor: A report from the National Wilms Tumor Study Group. J Clin Oncol 18: 2026-2031, 2000

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