Nephron Sparing Surgery for Unilateral Wilms Tumor in Children with Predisposing Syndromes: Single Center Experience Over 10 Years
2012; Lippincott Williams & Wilkins; Volume: 188; Issue: 4S Linguagem: Inglês
10.1016/j.juro.2012.02.034
ISSN1527-3792
AutoresRodrigo Romao, João L. Pippi Salle, Cheryl Shuman, Rosanna Weksberg, Víctor Figueroa, Bryce Weber, Darius Bägli, Walid A. Farhat, Ronald Grant, J. Ted Gerstle, Armando J. Lorenzo,
Tópico(s)Childhood Cancer Survivors' Quality of Life
ResumoNo AccessJournal of UrologyOncology and Transplant1 Oct 2012Nephron Sparing Surgery for Unilateral Wilms Tumor in Children with Predisposing Syndromes: Single Center Experience Over 10 Years Rodrigo L.P. Romão, João L. Pippi Salle, Cheryl Shuman, Rosanna Weksberg, Victor Figueroa, Bryce Weber, Darius J. Bägli, Walid A. Farhat, Ronald Grant, J. Ted Gerstle, and Armando J. Lorenzo Rodrigo L.P. RomãoRodrigo L.P. Romão Division of Urology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author , João L. Pippi SalleJoão L. Pippi Salle Division of Urology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author , Cheryl ShumanCheryl Shuman Division of Clinical and Metabolic Genetics, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author , Rosanna WeksbergRosanna Weksberg Division of Clinical and Metabolic Genetics, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author , Victor FigueroaVictor Figueroa Division of Urology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author , Bryce WeberBryce Weber Division of Urology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author , Darius J. BägliDarius J. Bägli Division of Urology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author , Walid A. FarhatWalid A. Farhat Division of Urology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author , Ronald GrantRonald Grant Department of Pediatrics, Division of Hematology Oncology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author , J. Ted GerstleJ. Ted Gerstle Division of General and Thoracic Surgery, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author , and Armando J. LorenzoArmando J. Lorenzo Division of Urology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2012.02.034AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract Purpose: Unilateral Wilms tumors associated with predisposing syndromes are treated with preoperative chemotherapy followed by surgical resection. We describe our experience with nephron sparing surgery for Wilms tumor in this population at risk for metachronous lesions. Materials and Methods: We conducted a retrospective review of all children with a predisposing syndrome who underwent nephrectomy for malignancy during a 10-year period (2000 to 2010). Data collected included age, mode of detection, tumor size, treatment, pathology results, followup time and recurrence episodes. Results: From 2000 to 2010, 13 of 75 (19%) patients treated for Wilms tumor were diagnosed with predisposing syndrome(s). Eight patients with unilateral tumors were treated and had a mean age at diagnosis of 27 months (range 7 months to 9 years). Beckwith-Wiedemann syndrome, isolated hemihyperplasia, WAGR (Wilms tumor, Aniridia, Genitourinary abnormalities, mental Retardation) syndrome and isolated 11p13 deletion were the underlying diagnoses in 3, 2, 2 and 1 patient, respectively. All but 2 patients were diagnosed by screening ultrasound and 5 underwent preoperative chemotherapy. Median tumor size at surgery was 2.5 cm (range 1 to 13). Nephron sparing surgery was performed in 6 of 8 patients. Pathological study showed favorable histology Wilms tumor and nephrogenic rests in 6 and 2 patients, respectively. After a mean followup of 36 months (range 6 to 72) no recurrences were documented and all children had normal creatinine levels. Conclusions: Nephron sparing surgery appears safe for patients with unilateral Wilms tumor associated with predisposing syndrome(s), allowing for the preservation of renal function and good oncologic outcomes for the available followup time. If more studies confirm our observation, current recommendations for the surgical treatment of Wilms tumor may need to reemphasize the value of attempting nephron sparing surgery in this patient population. References 1 : Current management of Wilms' tumor. Curr Urol Rep2010; 11: 58. Google Scholar 2 : Beckwith-Wiedemann syndrome. Eur J Hum Genet2010; 18: 8. Google Scholar 3 : Diagnostic criteria and tumor screening for individuals with isolated hemihyperplasia. Genet Med2009; 11: 220. Google Scholar 4 : WAGR syndrome: a clinical review of 54 cases. Pediatrics2005; 116: 984. Google Scholar 5 : Educational paper: screening in cancer predisposition syndromes: guidelines for the general pediatrician. Eur J Pediatr2011; 170: 285. 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Google Scholar © 2012 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetailsCited byKhondker A, Jain A, Groff M, Brzezinski J, Lorenzo A and Zappitelli M (2021) Late Kidney Effects of Nephron-Sparing vs Radical Nephrectomy for Wilms Tumor: A Systematic Review and Meta-AnalysisJournal of Urology, VOL. 207, NO. 3, (513-523), Online publication date: 1-Mar-2022.Wang H, Abern M, Cost N, Chu D, Ross S, Wiener J and Routh J (2014) Use of Nephron Sparing Surgery and Impact on Survival in Children with Wilms Tumor: A SEER AnalysisJournal of Urology, VOL. 192, NO. 4, (1196-1202), Online publication date: 1-Oct-2014. Volume 188Issue 4SOctober 2012Page: 1493-1499 Advertisement Copyright & Permissions© 2012 by American Urological Association Education and Research, Inc.KeywordsWilms tumordisease susceptibilitynephrectomychildMetricsAuthor Information Rodrigo L.P. Romão Division of Urology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author João L. Pippi Salle Division of Urology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author Cheryl Shuman Division of Clinical and Metabolic Genetics, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author Rosanna Weksberg Division of Clinical and Metabolic Genetics, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author Victor Figueroa Division of Urology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author Bryce Weber Division of Urology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author Darius J. Bägli Division of Urology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author Walid A. Farhat Division of Urology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author Ronald Grant Department of Pediatrics, Division of Hematology Oncology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author J. Ted Gerstle Division of General and Thoracic Surgery, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author Armando J. Lorenzo Division of Urology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada More articles by this author Expand All Advertisement PDF downloadLoading ...
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