Epidemiology, clinical features and outcomes of ganglioneuroblastoma: An analysis of the National Cancer Database.

Abstract

e22013 Background: Ganglioneuroblastoma (GNB) is a rare tumor of the sympathetic nervous system composed of both immature neuroblasts and mature ganglion cells. It has an intermediate malignant potential that lies between a low grade ganglioneuroma and a high-grade neuroblastoma and has the capability to transform into a neuroblastoma. While more information is known regarding pediatric neuroblastoma, little is known regarding the epidemiology and clinical features of GNB. To our knowledge, this is the first analysis of ganglioneuroblastoma using the National Cancer Database (NCDB) and potentially the largest and most comprehensive studies of this subtype of tumor to date. Methods: Using the National Cancer Database (NCDB), 273 patients diagnosed with ganglioneuroblastoma (ICD-0 9490/3) from 2004-2018 were identified. Kaplan-Meir analysis was used to estimate survival and log-rank tests were used to compare survival between stage groups. Results: The median age of diagnosis for patients with ganglioneuroblastoma was found to be 4 years. Girls were affected more commonly than boys, with a male to female ratio of 1.6/1. A majority of patients were white (77.7%) and African American (13.6%), followed by minor representations from multiple other races. The most common primary site of ganglioneuroblastoma was the thorax (40.5%), followed by the abdomen (35.7%). A minority of patients (14%) had distant metastases at diagnosis. At the time of disease presentation, 56% of patients were classified as stage I, 9.2% as stage II, 18.4% as stage III and 16.3% as stage IV. Stages I, II, and III conferred an excellent prognosis with a 99.2% survival rate as compared to patients with stage IV disease who had a survival rate of 65.2% (p < 0.001). Nearly all patients underwent surgery of the primary site, with the most common approach being local excision. Around one quarter of patients received chemotherapy while very few patients required radiation or immunotherapy. Conclusions: A number of unique findings help to distinguish ganglioneuroblastoma from the more common variant neuroblastoma. GNB seems to affect slightly older children (median age of 4 years) compared to a median age of 22 months in neuroblastoma. Interestingly, GNB was found to occur significantly more frequently in girls. On the contrary, neuroblastoma has been reported to be slightly more common in males than females. Additionally, the majority of ganglioneuroblastomas arise in the thorax. This is in comparison to neuroblastomas, of which the large majority arise in the abdomen, specifically in the adrenal glands. Stage IV GNB carries a significantly worse prognosis than stages I-III, with a survival rate of 62.5% which is similar to stage IV neuroblastoma.