Clinical and biological features of neuroblastic tumors: A comparison of neuroblastoma and ganglioneuroblastoma

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.

“Re-educating” Tumor Associated Macrophages as a Novel Immunotherapy Strategy for Neuroblastoma

“…Accounting for more than 15% of all childhood cancer deaths, neuroblastoma is a malignant pediatric cancer arising from neural crest cells of the sympathetic nervous system ( 1 , 2 ). Risk stratification for patients with neuroblastoma have evolved over time, and currently utilize the International Neuroblastoma Risk Group consensus criteria, which includes age at diagnosis, disease stage, tumor pathologic features, including histology and tumor cell ploidy, and tumor genetic characteristics, such as MYCN amplification and chromosomal aberrations ( 3 – 6 ).…”

Immune Escape Mechanisms and Future Prospects for Immunotherapy in Neuroblastoma

“…Neuroblastoma (NB) is the most common extracranial solid tumor of early childhood, accounting for about 6% of all childhood cancers, with an incidence of 1/70,000 in children younger than 15 years [ 1 ]. It is a neuroblastic tumor arising from deregulation of the signaling pathways governing primitive sympathetic ganglion cell development that also include ganglioneuroblastoma and ganglioneuroma [ 2 ]. NB patients are subdivided into low-, intermediate-, and high-risk groups based on clinical stage, age at diagnosis, tumor histology, MYCN oncogene amplification, histology, and chromosomal ploidy.…”

“…High-risk NB has a high recurrence rate. The most common sites for metastasis are bone marrow (BM), bone, lymph nodes, and liver [ 2 ]. The 5-year survival rate of high-risk patients remains around 40%, even after the use of multimodal intensive treatment [ 3 ].…”

Targeting the Tumor Microenvironment in Neuroblastoma: Recent Advances and Future Directions

“…The overall incidence of neuroblastoma is 1 patient per 100,000 children and every year 600-700 new cases are diagnosed in the United States of America [3]. NB patients are stratified into low-, intermediate-and high-risk groups, based on different parameters, including tumor histology, clinical stage, tumor cell ploidy, and MYCN oncogene amplification [4]. In approximately, 50% of children with this disease, tumors lack amplification of MYCN oncogene and these patients show overall survival more than 90%.…”