BACKGROUND: Modern WT management consist of ample chemotherapy, nephron-sparing surgery, and, when indicated, radiotherapy. Survivors may develop renal failure or secondary tumors due to anticancer treatment. We analyzed long-term outcome (follow-up >5 years) after bilateral Wilms tumor (BWT) treatment with respect to survival, renal function, and secondary malignancies. METHODS: From 41 patients (23 females, 28 synchronous tumors) diagnosed with BWT between 1967 and 2007, 25 (18 females, 14 synchronous) with a follow-up >5 years could be included. Of this subgroup, median age at diagnosis was 1.64 years (range 0.27-5.35), and at maximum follow-up 14.99 years (range 5.40-33.99). Data were retrospectively collected and analyzed. RESULTS: One patient (4%) died 17.75 years after diagnosis, five (20%) had renal transplants: 3/5 after bilateral nephrectomy for Denys-Drash syndrome (DDS), and 2/5 for ESRD after an interval of 7 and 18 years, respectively. All transplanted patients remained in CR. Another three patients developed mild renal insufficiency (creatinine levels 1.3, 1.8, and 2.8 mg/100 ml, respectively; N = 0.5-1.2), combined with hypertension in 1; neither of them was transplanted. Sixteen (64%) had normal renal function and were in CR. Long-term renal function appeared significantly better after bilateral nephron sparing surgery (NSS) then after other surgical procedures (P < 0.0001). Seven secondary tumors were found in five (20%) patients, one of whom had a DDS. CONCLUSION: Long-term 10-year overall survival was 78%. There was significant morbidity (13/25, 52%), in terms of renal failure (8/25, 32%) including renal transplantation (5/25, 20%), and secondary tumors (5/25). These findings necessitate long-term follow-up beyond childhood. Future work should be directed at reducing the harmful effects of treatment, including the increased use of NSS.
BACKGROUND: Modern WT management consist of ample chemotherapy, nephron-sparing surgery, and, when indicated, radiotherapy. Survivors may develop renal failure or secondary tumors due to anticancer treatment. We analyzed long-term outcome (follow-up >5 years) after bilateral Wilms tumor (BWT) treatment with respect to survival, renal function, and secondary malignancies. METHODS: From 41 patients (23 females, 28 synchronous tumors) diagnosed with BWT between 1967 and 2007, 25 (18 females, 14 synchronous) with a follow-up >5 years could be included. Of this subgroup, median age at diagnosis was 1.64 years (range 0.27-5.35), and at maximum follow-up 14.99 years (range 5.40-33.99). Data were retrospectively collected and analyzed. RESULTS: One patient (4%) died 17.75 years after diagnosis, five (20%) had renal transplants: 3/5 after bilateral nephrectomy for Denys-Drash syndrome (DDS), and 2/5 for ESRD after an interval of 7 and 18 years, respectively. All transplanted patients remained in CR. Another three patients developed mild renal insufficiency (creatinine levels 1.3, 1.8, and 2.8 mg/100 ml, respectively; N = 0.5-1.2), combined with hypertension in 1; neither of them was transplanted. Sixteen (64%) had normal renal function and were in CR. Long-term renal function appeared significantly better after bilateral nephron sparing surgery (NSS) then after other surgical procedures (P < 0.0001). Seven secondary tumors were found in five (20%) patients, one of whom had a DDS. CONCLUSION: Long-term 10-year overall survival was 78%. There was significant morbidity (13/25, 52%), in terms of renal failure (8/25, 32%) including renal transplantation (5/25, 20%), and secondary tumors (5/25). These findings necessitate long-term follow-up beyond childhood. Future work should be directed at reducing the harmful effects of treatment, including the increased use of NSS.
Authors: Marry M van den Heuvel-Eibrink; Janna A Hol; Kathy Pritchard-Jones; Harm van Tinteren; Rhoikos Furtwängler; Arnauld C Verschuur; Gordan M Vujanic; Ivo Leuschner; Jesper Brok; Christian Rübe; Anne M Smets; Geert O Janssens; Jan Godzinski; Gema L Ramírez-Villar; Beatriz de Camargo; Heidi Segers; Paola Collini; Manfred Gessler; Christophe Bergeron; Filippo Spreafico; Norbert Graf Journal: Nat Rev Urol Date: 2017-10-31 Impact factor: 14.432
Authors: E Sieswerda; R L Mulder; I W E M van Dijk; E C van Dalen; S L Knijnenburg; H J H van der Pal; M S Mud; R C Heinen; H N Caron; L C M Kremer Journal: J Cancer Surviv Date: 2013-04-30 Impact factor: 4.442
Authors: Andrew C Dietz; Kristy Seidel; Wendy M Leisenring; Daniel A Mulrooney; Jean M Tersak; Richard D Glick; Cathy A Burnweit; Daniel M Green; Lisa R Diller; Susan A Smith; Rebecca M Howell; Marilyn Stovall; Gregory T Armstrong; Kevin C Oeffinger; Leslie L Robison; Amanda M Termuhlen Journal: Lancet Oncol Date: 2019-08-27 Impact factor: 41.316
Authors: Esmee Cm Kooijmans; Arend Bökenkamp; Nic S Tjahjadi; Jesse M Tettero; Eline van Dulmen-den Broeder; Helena Jh van der Pal; Margreet A Veening Journal: Cochrane Database Syst Rev Date: 2019-03-11
Authors: Sophie E van Peer; Janna A Hol; Alida F W van der Steeg; Martine van Grotel; Godelieve A M Tytgat; Annelies M C Mavinkurve-Groothuis; Geert O R Janssens; Annemieke S Littooij; Ronald R de Krijger; Marjolijn C J Jongmans; Marc R Lilien; Jarno Drost; Roland P Kuiper; Harm van Tinteren; Marc H W A Wijnen; Marry M van den Heuvel-Eibrink Journal: J Clin Med Date: 2021-11-26 Impact factor: 4.241