Hong Cheng Song1, Ning Sun2, Wei Ping Zhang1, LeJian He3, Libing Fu3, ChengRu Huang1. 1. Department of Urology, Beijing Children's Hospital of Capital Medical University, Beijing 100045, China. 2. Department of Urology, Beijing Children's Hospital of Capital Medical University, Beijing 100045, China. Electronic address: Pro_sn@126.com. 3. Department of Pathology, Beijing Children's Hospital of Capital Medical University, Beijing 100045, China.
Abstract
PURPOSE: To investigate the clinical features of pediatric Xp11.2 translocation renal cell carcinoma (RCC). METHODS: A retrospective review of 22 cases over 35 years. RESULTS: Xp11.2 translocation RCCs were identified in 13 boys and 9 girls with a median age of 10.5 years (range: 2.5-16 years). RCC presented with hematuria in 17, abdominal mass in 1, abdominal masses with hematuria in 2, abdominal pain with hematuria in 1, and as an incidental finding in 1 patient. Ten patients were classified stage I, 10 were stage III, and two were stage IV. Of the 10 patients with stage I RCCs, 3 patients with tumor measuring less than 7 cm had nephron-sparing surgery (NSS) and 17 patients underwent simple nephrectomy. A 15-cm tumor was incompletely removed in one patient and another patient with a 25-cm × 18-cm × 15-cm tumor had gross residual. Of the 15 patients followed up between 6 months and 35 years, 13 were still living and 2 had died after surgery. CONCLUSIONS: Xp11.2 translocation RCC is the predominant form of pediatric RCC, associated with advanced stage at presentation. Nephrectomy is the usual treatment for RCC but NSS is an option for patients with tumors measuring<7 cm. Patients with N+M0 maintained a favorable prognosis following surgery alone.
PURPOSE: To investigate the clinical features of pediatric Xp11.2 translocation renal cell carcinoma (RCC). METHODS: A retrospective review of 22 cases over 35 years. RESULTS: Xp11.2 translocation RCCs were identified in 13 boys and 9 girls with a median age of 10.5 years (range: 2.5-16 years). RCC presented with hematuria in 17, abdominal mass in 1, abdominal masses with hematuria in 2, abdominal pain with hematuria in 1, and as an incidental finding in 1 patient. Ten patients were classified stage I, 10 were stage III, and two were stage IV. Of the 10 patients with stage I RCCs, 3 patients with tumor measuring less than 7 cm had nephron-sparing surgery (NSS) and 17 patients underwent simple nephrectomy. A 15-cm tumor was incompletely removed in one patient and another patient with a 25-cm × 18-cm × 15-cm tumor had gross residual. Of the 15 patients followed up between 6 months and 35 years, 13 were still living and 2 had died after surgery. CONCLUSIONS: Xp11.2 translocation RCC is the predominant form of pediatric RCC, associated with advanced stage at presentation. Nephrectomy is the usual treatment for RCC but NSS is an option for patients with tumors measuring<7 cm. Patients with N+M0 maintained a favorable prognosis following surgery alone.
Authors: Camilla Calandrini; Frans Schutgens; Rurika Oka; Thanasis Margaritis; Tito Candelli; Luka Mathijsen; Carola Ammerlaan; Ravian L van Ineveld; Sepide Derakhshan; Sanne de Haan; Emmy Dolman; Philip Lijnzaad; Lars Custers; Harry Begthel; Hindrik H D Kerstens; Lindy L Visser; Maarten Rookmaaker; Marianne Verhaar; Godelieve A M Tytgat; Patrick Kemmeren; Ronald R de Krijger; Reem Al-Saadi; Kathy Pritchard-Jones; Marcel Kool; Anne C Rios; Marry M van den Heuvel-Eibrink; Jan J Molenaar; Ruben van Boxtel; Frank C P Holstege; Hans Clevers; Jarno Drost Journal: Nat Commun Date: 2020-03-11 Impact factor: 14.919