title: Is Nephron Sparing Surgery Justified in Wilms Tumor With Beckwith-Wiedemann Syndrome or Isolated Hemihypertrophy?
abstract: Patients with Beckwith-Wiedemann syndrome (BWS) or isolated hemihypertrophy (HH) treated for a Wilms tumor (WT) carry an increased risk of developing metachronous lesion. There are no guidelines on precise indications for nephron sparing surgery (NSS) in unilateral WT (UWT). The objective of this retrospective study was to delineate the indications of NSS in patients with BWS/HH treated for WT and to evaluate their outcome.
  
  
  
  All cases of BWS/HH treated for a WT according to SIOP protocols from 1980 to 2013 were reviewed. Patients were divided into two groups (G): isolated UWT (G1) and bilateral lesions (G2) with two subgroups: bilateral tumors suspected of malignancy (G2a), and unilateral tumor suspected of malignancy with contralateral nephroblastomatosis (G2b).
  
  
  
  Forty-six patients were included (34 G1, three G2a, and nine G2b). Nine NSS and 25 total nephrectomies (TN) were performed in G1, two bilateral NSS and one NSS with contralateral TN in G2a, and eight NSS and one TN in G2b. The 3-year event-free survival was 92.3% (95% CI [77.9-97.5%]). One death occurred after a local relapse following a TN for a stage III stromal WT (G1) and another after a combined local and distant relapse following a NSS for a stage I diffuse anaplastic WT (G2b). There were two metachronous WT (4%), 3 years after a TN (G1) and 12 years after a NSS (G2b).
  
  
  
  NSS is recommended in bilateral WT and may be an option in selected UWT patients with BWS/HH because it was not associated with an increased risk of local relapse.
authors.affiliation: Sorbonne Universités, UPMC Univ Paris 06, APHP Hôpital Armand Trousseau, Pediatric Surgery, 75012 Paris, France.
authors.forename: Aurélien
authors.initials: A
authors.lastname: Scalabre
authors.affiliation: Institut d'Hématologie et d'Oncologie Pédiatrique, Department of Pediatrics, 690008 Lyon, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Bergeron
authors.affiliation: Universités, UPMC Univ Paris 06, Centre de Recherche St Antoine Inserm UMRS.938, APHP Hôpital Armand Trousseau Pediatric and Genetic Endocinology, 75012 Paris, France.
authors.forename: Frederic
authors.initials: F
authors.lastname: Brioude
authors.affiliation: Sorbonne Universités, UPMC Univ Paris 06, APHP Hôpital Armand Trousseau Pathology Department, Centre de Recherche St Antoine, Inserm UMRS.938, 75012 Paris, France.
authors.forename: Linda
authors.initials: L
authors.lastname: Dainese
authors.affiliation: Université de Lyon - Centre Léon Bérard, Department of Statistics, 69008 Lyon, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Cropet
authors.affiliation: Sorbonne Universités, UPMC Univ Paris 06, APHP Hôpital Armand Trousseau Pathology Department, Centre de Recherche St Antoine, Inserm UMRS.938, 75012 Paris, France.
authors.forename: Aurore
authors.initials: A
authors.lastname: Coulomb L'hermine
authors.affiliation: Gustave Roussy Cancer Campus, Pediatric Oncology, 94800 Villejuif, France.
authors.forename: Claudia
authors.initials: C
authors.lastname: Pasqualini
authors.affiliation: Université de Bourgogne Franche-Comté, Laboratoire de Nanomédecine, Imagerie et Thérapeutique EA 4662, CHRU Besançon, Service de Chirurgie Pédiatrique, 25030 Besançon, France.
authors.forename: Frederic
authors.initials: F
authors.lastname: Auber
authors.affiliation: Department of Pediatric Hematology and Oncology, Hôpital de la Timone Enfant, 13005 Marseille, France.
authors.forename: Arnauld
authors.initials: A
authors.lastname: Verschuur
authors.affiliation: Institut Curie, Paediatric oncology, 3 INSERM U830, Laboratoire de Génétique et Biologie des Cancers, 75005 Paris, France.
authors.forename: Gudrun
authors.initials: G
authors.lastname: Schleiermacher
authors.affiliation: Universités, UPMC Univ Paris 06, Centre de Recherche St Antoine Inserm UMRS.938, APHP Hôpital Armand Trousseau Pediatric and Genetic Endocinology, 75012 Paris, France.
authors.forename: Yves
authors.initials: Y
authors.lastname: Le Bouc
authors.affiliation: Sorbonne Universités, UPMC Univ Paris 06, APHP Hôpital Armand Trousseau, Pediatric Surgery, 75012 Paris, France.
authors.forename: Georges
authors.initials: G
authors.lastname: Audry
authors.affiliation: Sorbonne Universités, UPMC Univ Paris 06, APHP Hôpital Armand Trousseau Pediatric Surgery, Centre de Recherche St Antoine, Inserm UMRS.938, 75012 Paris, France.
authors.forename: Sabine
authors.initials: S
authors.lastname: Irtan
mesh_terms: D001506:Beckwith-Wiedemann Syndrome / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006965:Hyperplasia / Q000601:surgery*; D007223:Infant; D007680:Kidney Neoplasms / Q000601:surgery*; D008297:Male; D009399:Nephrons / Q000601:surgery*; D012189:Retrospective Studies; D009396:Wilms Tumor / Q000601:surgery*
keywords: Beckwith-Wiedemann syndrome; Wilms tumor; isolated hemihypertrophy; nephron sparing surgery