TY - JOUR
T1 - Management of Ganglioneuroma and Ganglioneuroblastoma Intermixed
T2 - A United Kingdom Children's Cancer and Leukaemia Group (UK CCLG) Nationwide Study Report
AU - Burnand, Katherine M.
AU - Neville, Jonathan
AU - Budzanowski, Annita
AU - Okoye, Bruce O.
AU - Cross, Kate
AU - Wheeler, Kate
AU - Gray, Juliet
AU - Hall, Nigel
AU - Ramanujakar, Ramya
AU - Reddy, Snigdha
AU - Pachl, Max
AU - Kierulff, Carla
AU - Herd, Fiona
AU - Makin, Guy
AU - Howell, Lisa
AU - Pizer, Barry
AU - Rogers, Timothy
AU - Al-Khafaji, Nadeem
AU - Tweddle, Deborah
AU - Carruthers, Vicky Anne
AU - Barone, Giuseppe
AU - Anderson, John
AU - Vaidya, Sucheta
AU - George, Sally
AU - Braungart, Sarah
AU - Kwok, Chun
AU - Jacovides, Michael
AU - Burke, Amos
AU - Yeomanson, Dan
AU - Murphy, Dermot
AU - Losty, Paul D.
AU - Angelini, Paola
AU - Gabra, Hany
N1 - Copyright:
© 2024 The Author(s). Pediatric Blood & Cancer published by Wiley Periodicals LLC.
PY - 2024/11/24
Y1 - 2024/11/24
N2 - 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.
AB - 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.
KW - ganglioneuroblastoma
KW - ganglioneuroma
KW - observation
KW - surgery
KW - surveillance
UR - http://www.scopus.com/inward/record.url?scp=85210035733&partnerID=8YFLogxK
U2 - 10.1002/pbc.31445
DO - 10.1002/pbc.31445
M3 - Article
AN - SCOPUS:85210035733
SN - 1545-5009
JO - Pediatric Blood and Cancer
JF - Pediatric Blood and Cancer
M1 - e31445
ER -
