Abstract
Neuroblastoma (NB), ganglioneuroblastoma intermixed (GNBi) and ganglioneuroblastoma nodular (GNBn) are neuroblastic tumors that present with a wide range of symptoms and variable prognoses. We retrospectively reviewed the pretreatment clinical (age, sex and tumor stage) and biological (MYCN amplification; and levels of lactate dehydrogenase, ferritin and neuron-specific enolase) characteristics of 279 patients who were diagnosed with pathologically confirmed NB and GNB from January 2005 to December 2015. The median age at diagnosis increased with grade of differentiation (NB: 28.9 months; GNBn: 38.4 months; GNBi: 47.5 months; p < 0.01). NB patients were more frequently diagnosed with adrenal tumors and had a higher prevalence of abnormal serum ferritin at the time of diagnosis (60.0% vs. 40.0% vs. 12.0%, P<0.001), NSE (96.0% vs. 93.0% vs. 81.0%, P=0.013) when compared with GNBn and GNBi patients. The prevalence rates of disseminated tumors and MYCN amplified tumors were lower in the GNBi group than in the GNBn and NB groups (13.0% vs. 25.0% vs. 44.0%, P=0.002; 0 vs. 14.0% vs. 26.0%, P=0.032, respectively). The overall survival (OS) of patients with GNB was significantly better than that of patients with NB (GNBi: 100%, GNBn: 74.5±11.4%, NB: 50.8±4.5%, respectively; P<0.01). Our study revealed that both NB and GNB have a wide range of presentations, and clinicians should be aware of both typical and atypical symptoms and signs. Children with GNB (especially GNBi) were more likely to present favorable prognostic factors than their NB counterparts, which consequently lead to better outcomes and longer survival for these patients.
Highlights
Neuroblastoma (NB); ganglioneuroblastoma, intermixed (GNBi); ganglioneuroma (GN); and ganglioneuroblastoma, nodular (GNBn) represent a spectrum of neuroblastic tumors arising from primitive sympathetic ganglion cells; these solid extra-cranial tumors are frequently identified in children [1, 2]
Twelve (26.0%) of the 47 tumors analyzed in patients with NB were MYCN amplified, while only 1 case of ganglioneuroblastoma nodular (GNBn) had MYCN amplification
NB patients were more likely to have disseminated disease compared with GNBn and ganglioneuroblastoma intermixed (GNBi) patients (44.0% vs. 25.0% vs. 13.0%, p=.0.002), who most frequently had localized disease (75.0%, 87.0% respectively)
Summary
Neuroblastoma (NB); ganglioneuroblastoma, intermixed (GNBi); ganglioneuroma (GN); and ganglioneuroblastoma, nodular (GNBn) represent a spectrum of neuroblastic tumors arising from primitive sympathetic ganglion cells; these solid extra-cranial tumors are frequently identified in children [1, 2]. These tumors vary in their relative proportions of Schwann cells and neuroblasts and can be distinguished by their degree of cellular maturation and differentiation. A recent study found that GNB with nodular tumors represent a small group of pNT and are associated with heterogenous outcomes [6]. We retrospectively evaluated the clinical (age, sex and tumor stage) and biological (MYCN amplification; levels of lactate dehydrogenase, ferritin, vanillylmandelic and neuron-specific enolase) features of NB, GNBn and GNBi to provide more evidence to inform clinical diagnoses and therapeutic approaches
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