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The varied spectrum of nephroblastomatosis,nephrogenic rests,and Wilms tumors: Review of current definitions and challenges of the field
Authors:Elizabeth Fialkowski  Hélène Sudour-Bonnange  Gordan M Vujanic  Robert C Shamberger  Tanzina Chowdhury  Jennifer H Aldrink  Jonathan Davick  Jesse Sandberg  Rhoikos Furtwaengler  Elizabeth Mullen
Institution:1. Division of Pediatric Surgery, Department of Surgery, Oregon Health and Science University, Portland, Oregon, USA;2. Paediatric and AYA Unit, Oscar Lambret Center, Lille, France;3. Department of Pathology, Sidra Medicine and Weill Cornell Medicine, Doha, Qatar;4. Boston Children's Hospital and Dana Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts, USA;5. Department of Paediatric Oncology, Great Ormond Street Hospital for Children, London, UK;6. Division of Pediatric Surgery, Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, Ohio, USA;7. University of Iowa Hospitals and Clinics/Stead Family Children's Hospital, Iowa City, Iowa, USA;8. Department of Pediatric Radiology, Lucile Packard Children's Hospital, Stanford University, Stanford, California, USA;9. Paediatric Haematology and Oncology, Saarland University Hospital, Homburg, Germany;10. Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, Massachusetts, USA
Abstract:The diagnosis of multiple or diffuse renal lesions in a child is challenging by imaging and/or pathology. Optimal management requires distinguishing benign lesions such as nephrogenic rests from cancerous lesions such as Wilms tumor, but this is often difficult or impossible. This difficulty is compounded by the overlapping nature of our current radiologic and pathologic definitions of lesions along the spectrum of nephrogenic rests/nephroblastomatosis. We provide a review of these issues, as a collaborative effort between the Children's Oncology Group Renal Tumor Committee and International Society of Pediatric Oncology Renal Tumor Study Group. Our aim is to discuss current challenges in diagnosis and management of these renal lesions, encouraging future work toward consensus definitions for research and patient care.
Keywords:nephroblastomatosis  nephrogenic rests  Wilms tumors
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