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IS NEPHRON SPARING SURGERY JUSTIFIED IN WILMS TUMOR WITH
BECKWITH-WIEDEMANN SYNDROME OR ISOLATED HEMI-HYPERTROPHY ?
Aurélien SCALABRE
1
, Christophe BERGERON
2
, Frederic BRIOUDE
3
, Linda DAINESE
4
, Claire CROPET
5
, Aurore COULOMB
L'HERMINE
4
, Laurence BRUGIÈRES
6
, Frederic AUBER
7
, Arnauld VERSCHUUR
8
, Jean MICHON
9
, Georges AUDRY
10
and
Sabine IRTAN
10
1) Hôpital Femme Mère Enfant, Paediatric surgery, Bron, FRANCE - 2) Centre Léon Berard, Paediatric oncology, Lyon,
FRANCE - 3) Hôpital Armand Trousseau, Genetic, Paris, FRANCE - 4) Hôpital Armand Trousseau, Pathology, Paris,
FRANCE - 5) Centre Léon Berard, Biostatistics, Lyon, FRANCE - 6) Institut Gustave Roussy, Paediatric oncology, Villejuif,
FRANCE - 7) Centre Hospitalier Régional Universitaire Hôpital Jean Minjoz, Paediatric surgery, Besançon, FRANCE - 8)
Hôpital de la Timone, Paediatric oncology, Marseille, FRANCE - 9) Institut Curie, Paediatric oncology, Paris, FRANCE -
10) Hôpital Armand Trousseau, Paediatric surgery, Paris, FRANCE
PURPOSE
To delineate the indications of Nephron Sparing Surgery (NSS) in patients with Beckwith-Wiedemann syndrome (BWS)
or isolated Hemi-Hypertrophy (HH) treated for Wilms Tumor (WT) and/or nephroblastomatosis and to evaluate their
outcome.
MATERIAL AND METHODS
All cases of BWS or HH operated on in France for a WT and treated according to the SIOP protocols from 1980 to 2013
were retrospectively reviewed. Patients were divided into two groups: isolated unilateral tumor (group 1) and bilateral
tumor (group 2) with two subgroups: bilateral tumor suspected of malignancy (group 2a), unilateral tumor suspected of
malignancy with contralateral nephroblastomatosis (group 2b).
RESULTS
46 patients were included: 34 in group 1, 3 in group 2a, and 9 in group 2b. Median follow up was 62 months (2m-29y).
Nine NSS and 25 total nephrectomies were performed in group 1, 2 bilateral NSS and one NSS with contralateral total
nephrectomy in group 2a and 8 NSS and one total nephrectomy in group 2b. The 3-year event-free survival was 92.3%
(IC95% [77.9%-97.5%]). One death occurred after a local relapse following a total nephrectomy for a stade III stromal
WT in group 1, and another after a combined local and distant relapse following a NSS for a stade I diffuse anaplasic WT
in group 2b. There were 2 metachronous WT (4%), 3 years after a total nephrectomy (group 1) and 12 years after a
NSS (group 2b).
CONCLUSIONS
NSS is recommended in bilateral WT, and could be safely performed in BWS and HH patients with good oncological
outcomes.