Clinical and biological features of neuroblastic tumors: A comparison of neuroblastoma and ganglioneuroblastoma
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Wen-Guang He1,*, Yu Yan3,*, Wen Tang1,*, Rong Cai2 and Gang Ren1
1Department of Radiology, Xinhua Hospital, Shanghai Jiaotong University Medical School, Shanghai 200092, China
2Department of Radiotherapy, Ruijin Hospital, Shanghai Jiaotong University Medical School, Shanghai 200025, China
3Department of Pathology, Xinhua Hospital, Shanghai Jiaotong University Medical School, Shanghai 200092, China
*These authors have contributed equally to this work
Gang Ren, email: [email protected]
Rong Cai, email: [email protected]
Keywords: neuroblastoma, ganglioneuroblastoma, clinical and biological features
Received: June 21, 2016 Accepted: March 27, 2017 Published: April 17, 2017
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.
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