Unusual evolution of Wilms tumor in a patient with Wiedemann-Beckwith syndrome.

A very unusual case of Wilms tumor in a child with Wiedemann-Beckwith syndrome is presented. The patient had his first metastasis after a disease-free interval longer than three years. Later, he developed spinal epidural involvement. Both data emphasize the oncogenic potential of Wiedemann-Beckwith syndrome in children and support the contention that Wiedemann-Beckwith syndrome worsens the prognosis of Wilms tumor. Patients with Wilms tumor associated to Wiedemann-Beckwith syndrome should receive intensive therapy as well as close and prolonged follow-up in spite of apparent tumor remission.