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      Synchronous Bilateral Wilms Tumor: Five-Year Single-Center Experience with Assessment of Quality of Life

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          Abstract

          Context:

          Synchronous Bilateral Wilms tumor (sBWT).

          Aims:

          This study aimed to assess the outcome of patients with sBWT treated on SIOP protocol.

          Settings and Design:

          Retrospective and prospective randomized study.

          Subjects and Methods:

          SIOP 93-01 protocol was used to study nine patients of sBWT in a single center and followed up over a period from 2 to 5 years.

          Statistical Analysis Used:

          Unpaired t-test and Mann–Whitney U-test were used for analysis.

          Results:

          Of nine patients, six were included in the study as three patients lost to follow-up. Among the six patients, there were four girls and two boys with a median age of 2 years. Mean regression in the size of tumor was 87% in four out of six patients. Tumor with unfavorable histology showed 32% response (ratio of favorable: unfavorable histology 2:1). Event-free survival rate was 81.3% and overall survival was 90% over 2–5 years. Recurrence was seen in two patients of whom one had Denys–Drash syndrome. Mean DTPA glomerular filtration rate was 91.4/ml/min/1.73 m 2 preoperatively and that of 3 months after completion of treatment was 84/ml/min/1.73 m 2. Health-related quality of life (HRQOL) using Pediatric Quality of Life Inventory and Lansky Play Performance Scale revealed significant improvement results of all functioning domains such as physical, social, emotional, and school subscales with P < 0.05 and performance scale ( P < 0.04).

          Conclusions:

          We suggest SIOP protocol for sBWT and bilateral nephron-sparing surgery in two stages. However, long-term follow-up is required to assess the ultimate renal function outcome. HRQOL is an essential guide in improving the conditions of pediatric cancer survivors.

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          Most cited references23

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          Renal function after nephron-sparing surgery versus radical nephrectomy: results from EORTC randomized trial 30904.

          In the European Organization for Research and Treatment of Cancer (EORTC) randomized trial 30904, nephron-sparing surgery (NSS) was associated with reduced overall survival compared with radical nephrectomy (RN) over a median follow-up of 9.3 yr (hazard ratio: 1.50; 95% confidence interval [CI], 1.03-2.16). To examine the impact of NSS relative to RN on kidney function in EORTC 30904. This phase 3 international randomized trial was conducted in patients with a small (≤5 cm) renal mass and normal contralateral kidney who were enrolled from March 1992 to January 2003. Patients were randomized to RN (n=273) or NSS (n=268). Follow-up estimated glomerular filtration rates (eGFR; milliliters per minute per 1.73 m(2)) were recorded for 259 subjects in the RN arm and 255 subjects in the NSS arm. Percentages of subjects developing at least moderate renal dysfunction (eGFR <60), advanced kidney disease (eGFR <30), or kidney failure (eGFR <15) were calculated for each treatment arm based on the lowest recorded follow-up eGFR (intent-to-treat analysis). With a median follow-up of 6.7 yr, eGFR <60 was reached by 85.7% with RN and 64.7% with NSS, with a difference of 21.0% (95% CI, 13.8-28.3); eGFR <30 was reached by 10.0% with RN and 6.3% with NSS, with a difference of 3.7% (95% CI, -1.0 to 8.5); and eGFR <15 was reached by 1.5% with RN and 1.6% with NSS, with a difference of -0.1% (95% CI, -2.2 to 2.1). Lack of longer follow-up for eGFR is a limitation of these analyses. Compared with RN, NSS substantially reduced the incidence of at least moderate renal dysfunction (eGFR <60), although with available follow-up the incidence of advanced kidney disease (eGFR <30) was relatively similar in the two treatment arms, and the incidence of kidney failure (eGFR <15) was nearly identical. The beneficial impact of NSS on eGFR did not result in improved survival in this study population. EORTC trial 30904; ClinicalTrials.gov identifier NCT00002473. Copyright © 2013 European Association of Urology. Published by Elsevier B.V. All rights reserved.
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            Management of Wilms' tumor: NWTS vs SIOP

            With the availability of several protocols in the management of Wilms' tumor, there is dilemma in the minds of the treating oncologists or pediatric onco-surgeons as to whether the child should receive upfront chemotherapy or should be operated upon primarily. It is necessary for us to understand why do we follow either of the protocols, NWTS which follows the upfront surgery principle or the SIOP which follows the upfront chemotherapy principle in all stages of the disease. While deciding which protocol to follow, it is imperative to know the pros and cons of the treatment strategies and also to study the outcome patterns in both the treatment regimes which is what this article highlights. In an attempt to compare all the differences in both the major protocols, it was realized that most of our patients in the Indian scenario present with advanced disease and thus poorer outcomes if intensive and appropriate treatment strategies are not utilized. Hence, it is imperative that we should study our own patients through the Indian Wilms' tumor study group and adopt the policies which improve the overall event free survival on a nationwide basis.
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              Revised Kuppuswamy and B G Prasad socio-economic scales for 2016

              Background: Socio-economic scales are integral part in the assessment of social class of an individual/family, which can have an influence on disease causing factors. It is also vital for consideration when customising health education to the target audience. Socio-economic scales are dependent on evaluation of income and need to be updated with changing consumer price index. The objective of the study was to revise the socio-economic scales of Kuppuswamy and B G Prasad, taking into consideration the revision in consumer price index (industrial workers). Methods: The socio-economic scales were revised on the basis of updated CPI (IW) for the month of August 2016, using an online tool. Results: The updated Kuppuswamy and B G Prasad scales for the year 2016 were obtained using revised CPI (IW) and a simple online tool. Conclusions: Regular updating of socio-economic scales which depend on income and are therefore susceptible to changes, due to revisions in consumer price index, is necessary to maintain the validity of these scales, which are used widely in community surveys and social studies. 
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                Author and article information

                Journal
                J Indian Assoc Pediatr Surg
                J Indian Assoc Pediatr Surg
                JIAPS
                Journal of Indian Association of Pediatric Surgeons
                Medknow Publications & Media Pvt Ltd (India )
                0971-9261
                1998-3891
                Jan-Mar 2019
                : 24
                : 1
                : 52-60
                Affiliations
                [1]Department of Paediatric Surgery, Nil Ratan Sircar Medical College and Hospital, Kolkata, West Bengal, India
                [1 ]Department of Pathology, SSKM and IPGMER Medical College and Hospital, Kolkata, West Bengal, India
                Author notes
                Address for correspondence: Dr. Dipak Ghosh, Orbit Sky View, 1/1A, Umakanta Sen Lane, Block 4, Flat-4B, Kolkata - 700 030, West Bengal, India. E-mail: drdipakghosh45@ 123456gmail.com
                Article
                JIAPS-24-52
                10.4103/jiaps.JIAPS_42_18
                6322171
                80c05264-9534-4ce2-a095-5049bd4ecf45
                Copyright: © 2018 Journal of Indian Association of Pediatric Surgeons

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                : March 2018
                : August 2018
                Categories
                Original Article

                Surgery
                nephron-sparing surgery,preoperative chemotherapy,synchronous bilateral wilms tumor

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