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Management of Ganglioneuroma and Ganglioneuroblastoma Intermixed A UNited Kingdom Childrens cancer and Leukaemia Group (UKCCLG) Nationwide Study REPORT
  • +30
  • Hany Gabra,
  • Katherine Burnand,
  • Jonathan Neville J,
  • Annita Budzanowski,
  • Bruce Okoye,
  • Kate Cross,
  • Kate Wheeler,
  • Juliet Gray C,
  • Nigel Hall,
  • Ramya Ramanujachar,
  • Snigdha Reddy,
  • Maximilian Pachl,
  • Carla Kierulff,
  • Fiona Herd,
  • Guy Makin,
  • Lisa Howell,
  • Barry Pizer,
  • Timothy Rogers,
  • Nadeem Alkhafaji,
  • Deborah Tweddle,
  • Vickyanne Carruthers,
  • Giuseppe Barone,
  • John Anderson,
  • Sucheta Vaidya,
  • Sally George,
  • Sarah Braungart,
  • Chun Kwok,
  • Michael Jacovides,
  • G Amos Burke,
  • Dan Yeomanson,
  • Dermot Murphy,
  • Paul Losty,
  • Paola Angelini
Hany Gabra
Newcastle University Centre for Cancer

Corresponding Author:[email protected]

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Katherine Burnand
The Royal Marsden NHS Foundation Trust
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Jonathan Neville J
University Hospital Southampton NHS Foundation Trust
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Annita Budzanowski
Great Ormond Street Hospital for Children NHS Foundation Trust
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Bruce Okoye
The Royal Marsden NHS Foundation Trust
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Kate Cross
Great Ormond Street Hospital for Children NHS Foundation Trust
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Kate Wheeler
Oxford University Hospitals NHS Foundation Trust
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Juliet Gray C
University Hospital Southampton NHS Foundation Trust
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Nigel Hall
University Hospital Southampton NHS Foundation Trust
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Ramya Ramanujachar
University Hospital Southampton NHS Foundation Trust
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Snigdha Reddy
Birmingham Children's Hospital
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Maximilian Pachl
Birmingham Children's Hospital
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Carla Kierulff
Royal Aberdeen Children's Hospital
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Fiona Herd
Royal Aberdeen Children's Hospital
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Guy Makin
Manchester Royal Infirmary
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Lisa Howell
Alder Hey Children's Hospital
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Barry Pizer
Alder Hey Children's Hospital
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Timothy Rogers
Bristol Royal Hospital for Children
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Nadeem Alkhafaji
Bristol Royal Hospital for Children
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Deborah Tweddle
Newcastle University Centre for Cancer
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Vickyanne Carruthers
Newcastle University Centre for Cancer
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Giuseppe Barone
Great Ormond Street Hospital for Children NHS Foundation Trust
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John Anderson
Great Ormond Street Hospital for Children NHS Foundation Trust
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Sucheta Vaidya
The Royal Marsden NHS Foundation Trust
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Sally George
The Royal Marsden NHS Foundation Trust
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Sarah Braungart
Alder Hey Children's Hospital
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Chun Kwok
Alder Hey Children's Hospital
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Michael Jacovides
Royal Hospital for Children
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G Amos Burke
Cambridge University Hospitals NHS Foundation Trust
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Dan Yeomanson
Sheffield Children's NHS Foundation Trust
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Dermot Murphy
Royal Hospital for Children
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Paul Losty
Alder Hey Children's Hospital
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Paola Angelini
The Royal Marsden NHS Foundation Trust
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Abstract

Background Ganglioneuroblastoma intermixed (GNBi) and ganglioneuroma (GN) are benign subtypes of neuroblastic tumors. Primary observation has become accepted management for some patients with surgical operative strategies evolving to be less aggressive. Objectives Our study examines evolving management in a UK cohort investigating natural history, biology and clinical features of GN and ganglioneuroblastoma-intermixed (GNBi) in those having observation or surgery. Methods Retrospective review of histologically confirmed GN and GNBi managed over a 30 year period. Clinical, pathological features, tumor dimensions, management and outcomes are all recorded. Results A total of 259 patients were identified (GN= 163, GNBi = 93, median age = 62 months). 201(78%) had upfront surgery and 58 (22%) were actively observed. Of the 58 observed - 21 (36%) later required surgery due to progressive tumour growth (52%). Gross total resection was achieved in 79% of patients with a 19% complication rate. Presence of image defined risk factors and large tumour size correlated with incomplete resection (p < 0.05 in both). Forty-five index cases (39%) had change in pathology between biopsy and surgery with 14 patients (12%) altered from ‘favourable‘ to ‘unfavourable’. Conclusion Our findings show surveillance alone may be considered a safe approach. However, a significant number of index patients may eventually require operative surgery with development of symptoms. Extent of surgical resection did not impact overall survival (OS); however it improved symptom(s) resolution.
06 Mar 2024Submission Checks Completed
06 Mar 2024Assigned to Editor
06 Mar 2024Submitted to Pediatric Blood & Cancer
07 Mar 2024Review(s) Completed, Editorial Evaluation Pending
15 Mar 2024Reviewer(s) Assigned
23 Apr 2024Editorial Decision: Revise Major
10 Jun 2024Reviewer(s) Assigned
21 Jul 2024Assigned to Editor
21 Jul 2024Submission Checks Completed
21 Jul 20242nd Revision Received
22 Jul 2024Review(s) Completed, Editorial Evaluation Pending
28 Jul 2024Editorial Decision: Revise Major
08 Oct 2024Submission Checks Completed
08 Oct 2024Assigned to Editor
08 Oct 20243rd Revision Received
09 Oct 2024Review(s) Completed, Editorial Evaluation Pending
30 Oct 2024Editorial Decision: Accept