Management of Ganglioneuroma and Ganglioneuroblastoma Intermixed A
UNited Kingdom Childrens cancer and Leukaemia Group (UKCCLG) Nationwide
Study REPORT
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.