Management of Ganglioneuroma and Ganglioneuroblastoma Intermixed: A United Kingdom Children's Cancer and Leukaemia Group (UK CCLG) Nationwide Study Report.

Autor: Burnand KM; The Royal Marsden NHS foundation Trust, London, UK., Neville J; Department of Paediatric Oncology, Southampton Children's Hospital, Southampton, UK., Budzanowski A; Department of Paediatric Oncology, Great Ormond Street Hospital, London, UK., Okoye BO; The Royal Marsden NHS foundation Trust, London, UK., Cross K; Department of Paediatric Oncology, Great Ormond Street Hospital, London, UK., Wheeler K; Department of Paediatric Oncology, Oxford Children's Hospital, Oxford, UK., Gray J; Department of Paediatric Oncology, Southampton Children's Hospital, Southampton, UK., Hall N; Department of Paediatric Oncology, Southampton Children's Hospital, Southampton, UK., Ramanujakar R; Department of Paediatric Oncology, Southampton Children's Hospital, Southampton, UK., Reddy S; Department of Paediatric surgery, Birmingham Children's Hospital, Birmingham, UK., Pachl M; Department of Paediatric surgery, Birmingham Children's Hospital, Birmingham, UK., Kierulff C; Department of Paediatric Oncology, Royal Aberdeen Children's Hospital, Aberdeen, UK., Herd F; Department of Paediatric Oncology, Royal Aberdeen Children's Hospital, Aberdeen, UK., Makin G; Department of Paediatric Oncology, Royal Manchester Children's Hospital, Manchester, UK., Howell L; Department of Paediatric Oncology, Alder Hey Children's Hospital, Liverpool, UK., Pizer B; Department of Paediatric Oncology, Alder Hey Children's Hospital, Liverpool, UK., Rogers T; Department of Paediatric Surgery, Bristol Children's Hospital, Bristol, UK., Al-Khafaji N; Department of Paediatric Surgery, Bristol Children's Hospital, Bristol, UK., Tweddle D; Department of Paediatric Surgery, The Great North Children's Hospital, Newcastle Upon Tyne, UK.; Translational & Clinical Research Institute, Centre for Cancer, Newcastle University, Newcastle upon Tyne, UK., Carruthers VA; Department of Paediatric Surgery, The Great North Children's Hospital, Newcastle Upon Tyne, UK.; Translational & Clinical Research Institute, Centre for Cancer, Newcastle University, Newcastle upon Tyne, UK., Barone G; Department of Paediatric Oncology, Great Ormond Street Hospital, London, UK., Anderson J; Department of Paediatric Oncology, Great Ormond Street Hospital, London, UK., Vaidya S; The Royal Marsden NHS foundation Trust, London, UK., George S; The Royal Marsden NHS foundation Trust, London, UK., Braungart S; Department of Paediatric Surgery, Alder Hey Children's Hospital, University of Liverpool, Liverpool, UK., Kwok C; Department of Paediatric Surgery, Alder Hey Children's Hospital, University of Liverpool, Liverpool, UK., Jacovides M; Department of Paediatric Surgery, Royal Glasgow Children's Hospital, Glasgow, UK., Burke A; Department of Paediatric Oncology, Addenbrooke's Hospital, Cambridge, UK., Yeomanson D; Department of Paediatric Oncology, Sheffield Children's Hospital, Sheffield, UK., Murphy D; Department of Paediatric Surgery, Royal Glasgow Children's Hospital, Glasgow, UK., Losty PD; Institute of Systems Molecular and Integrative Biology, University of Liverpool, Liverpool, UK.; Department of Paediatric Surgery, Ramathibodi Hospital Mahidol University, Bangkok, Thailand., Angelini P; The Royal Marsden NHS foundation Trust, London, UK., Gabra H; Department of Paediatric Surgery, The Great North Children's Hospital, Newcastle Upon Tyne, UK.; Translational & Clinical Research Institute, Centre for Cancer, Newcastle University, Newcastle upon Tyne, UK.
Jazyk: angličtina
Zdroj: Pediatric blood & cancer [Pediatr Blood Cancer] 2024 Nov 24, pp. e31445. Date of Electronic Publication: 2024 Nov 24.
DOI: 10.1002/pbc.31445
Abstrakt: Background: Ganglioneuroblastoma intermixed (GNBi) and ganglioneuroma (GN) represent benign variants of peripheral neuroblastic tumours. While historically surgical resection was recommended, watchful active observation has become the accepted management for a subset of patients.
Objectives: To review clinical features, biology, natural history and management of a retrospective UK CCLG study cohort of GN and GNBi, and compare outcomes of patients treated with surgical resection or watchful active observation.
Methods: Retrospective review of histologically confirmed non-metastatic GN and GNBi diagnosed between 1990 and 2020.
Results: A total of 259 patients were identified (163 GN, 93 GNBi, median age 62 months). In all 201 (78%) had initial surgery and 58 (22%) were observed. Overall survival was 98%. Twenty-one of 58 observed (36%) required subsequent surgery due to progressive tumour growth (52%). Gross total resection (GTR) was achieved in 79% of patients with a 19% complication rate. GTR was obtained in a similar proportion of patients having initial surgery (65%) and delayed surgery (76%). Patients obtaining GTR were more likely to have complete symptom(s) control (43% vs. 24%). In 45 cases (39%), surgical pathology was different from pathology at biopsy, and in 14 (12%) the pathology changed from GN/GNBi to neuroblastoma/GNB nodular.
Conclusion: Watchful active observation can be a safe approach, with surgical resection reserved for symptomatic patients. However, a small proportion of patients in the observation group had their diagnosis revised to malignant at surgery. Careful assessment of surgical risk and expected benefits, after considering an initial period of observation, will allow clinicians to make optimal decisions for patients and their families.
(© 2024 The Author(s). Pediatric Blood & Cancer published by Wiley Periodicals LLC.)
Databáze: MEDLINE
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