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PEDIATRICS Vol. 113 No. 4 April 2004, pp. e326-e329

ELECTRONIC ARTICLE

Wilms Tumor Screening Is Unnecessary in Klippel-Trenaunay Syndrome

Arin K. Greene, MD, MMSc^*,, Mark Kieran, MD, PhD^*,,#, Patricia E. Burrows, MD^*,||, John B. Mulliken, MD^*,, James Kasser, MD^*,¶ and Steven J. Fishman, MD^*,

^* Vascular Anomalies Center
the Departments of Surgery
Hematology/Oncology
^|| Radiology
^¶ Orthopedic Surgery, Children’s Hospital Boston, Boston, Massachusetts
^# Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts

Background. Children with hemihypertrophy are screened for Wilms tumor, because this condition is a risk factor for developing the neoplasm. Patients with Klippel-Trenaunay syndrome (KTS) are often considered potential candidates for Wilms tumor, because they have unilateral overgrowth of the lower limb. In our experience, however, an association between KTS and Wilms tumor has not been observed.

Methods. To determine whether KTS and Wilms tumor are associated, we reviewed our institutional experience for patients with both diagnoses and searched the Klippel-Trenaunay literature for patients with Wilms tumor. The National Wilms Tumor Study Group database also was studied to identify patients with KTS. Two-sided exact binomial tests were used to evaluate whether patients with 1 condition had an increased risk for the other. Ninety-five percent confidence intervals for these 2 risks were compared with the general population risks of Wilms tumor (1 in 10 000) and KTS (1 in 47 313).

Results. None of the 115 patients with KTS followed at our institution developed Wilms tumor. One case of Wilms tumor has been reported in 1363 patients with KTS in the literature, giving a confidence interval of (1/57 377) and (1/267). None of the 8614 patients in the National Wilms Tumor Study Group database had KTS, giving a confidence interval of (0, 1/2336). Because the risks of KTS and Wilms tumor in the population fall within these confidence intervals, one cannot conclude that the risks of KTS among Wilms tumor patients or Wilms tumor among KTS patients are any different from the corresponding risks in the general population.

Conclusions. Patients with KTS are not at increased risk for developing Wilms tumor and thus should not undergo routine ultrasonographic screening.

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Key Words: Klippel-Trenaunay syndrome • Wilms tumor • screening

Abbreviations: KTS, Klippel-Trenaunay syndrome • PWS, Parkes Weber syndrome • BWS, Beckwith-Wiedemann syndrome • NWTSG, National Wilms Tumor Study Group

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Received for publication Sep 10, 2003; Accepted Nov 24, 2003.

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