title: Unusual evolution of Wilms tumor in a patient with Wiedemann-Beckwith syndrome.
abstract: 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.
authors.affiliation: Department of Pediatrics, N.S. Covadonga Hospital, Asturias, Spain.
authors.forename: C
authors.initials: C
authors.lastname: Rey
authors.forename: A
authors.initials: A
authors.lastname: del Molino
authors.forename: F
authors.initials: F
authors.lastname: Santos
authors.forename: S
authors.initials: S
authors.lastname: Malaga
authors.forename: M
authors.initials: M
authors.lastname: Crespo
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001506:Beckwith-Wiedemann Syndrome / Q000150:complications*; D002675:Child, Preschool; D003131:Combined Modality Therapy; D006801:Humans; D007680:Kidney Neoplasms / Q000150:complications* / Q000473:pathology / Q000628:therapy; D008297:Male; D009362:Neoplasm Metastasis; D009392:Nephrectomy; D013997:Time Factors; D009396:Wilms Tumor / Q000150:complications* / Q000473:pathology / Q000628:therapy