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Characteristics and management of ganglioneuroma and ganglioneuroblastoma‐intermixed in children and adolescents
Author(s) -
Alexander Natasha,
Sullivan Katie,
Shaikh Furqan,
Irwin Meredith S.
Publication year - 2018
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.26964
Subject(s) - ganglioneuroblastoma , medicine , ganglioneuroma , pathological , neuroblastoma , natural history , retrospective cohort study , surgery , genetics , biology , cell culture
Background Ganglioneuromas (GNs) usually demonstrate favorable histological and clinical features. Surgery is often performed due to clinical symptoms and/or theoretical concerns that GN may transform into neuroblastoma (NB); however, several studies have identified significant GN‐surgical morbidities. Objectives We compared the natural history, biological and clinical features of GN and ganglioneuroblastoma‐intermixed (GNB‐I) managed by surgery or observation to inform management and surveillance. Procedures This retrospective study includes patients (n = 67) with histological diagnosis of GN (50/67) and GNB‐I (17/67) at the Hospital for Sick Children between 1990 and 2014. Clinical, pathological features, tumor dimensions, and management were recorded. Results Median age and maximal tumor diameter were 6 years (1.3–17.8) and 6.3 cm (1.4–16.9), respectively. Of the 67 patients, 46 (69%) had upfront surgery and 21 (31%) were observed. Of the 21 observed patients 4 later underwent resection. There were post‐operative complications in 15 of the 50 (30%) surgical patients. The presence of imaging‐defined risk factors correlated with complications ( P = 0.005). Observed patients were older (median 8.4 vs. 5.3 years) and diagnosed more recently. Median growth was 0.3 cm/year and 6 of 21 had progressive disease (PD). At median follow‐up of 2.2 years (0.2–14.3), all patients were alive and for those with evaluable imaging there were 27 complete and 10 partial responses, 19 stable and 6 PD. Pathology classification changed at resection for three cases, but no GN was reclassified to NB. Conclusions GN and GNB‐I have a slow growth rate and resection can be associated with significant morbidity. Watch and wait approaches should be considered for some GN and GNB‐I.