Abstract
PurposeThis study investigated relationships between neuroblastomas (NBs) imaging phenotypes, tumor genomic profile and patient outcome.Patients and methodsThis IRB-approved retrospective observational study included 133 NB patients (73 M, 60 F; median age 15 months, range 0–151) treated in a single institution between 1998 and 2012. A consensus review of imaging (CT-scan, MRI) categorized tumors according to both the primarily involved compartment (i.e., neck, chest, abdomen or pelvis) and the sympathetic anatomical structure the tumors rose from (i.e., cervical, paravertebral or periarterial chains, or adrenal gland). Tumor shape, volume and image-defined surgical risk factors (IDRFs) at diagnosis were recorded. Genomic profiles were assessed using array-based comparative genomic hybridization and divided into three groups: “numerical-only chromosome alterations” (NCA), “segmental chromosome alterations” (SCA) and “MYCN amplification” (MNA). Statistical analyses included Kruskal–Wallis, Chi2 and Fisher’s exact tests and the Kaplan-Meier method with log-rank tests and Cox model for univariate and multivariate survival analyses.ResultsA significant association between the sympathetic structure origin of tumors and genomic profiles was demonstrated. NBs arising from cervical sympathetic chains were all NCA. Paravertebral NBs were NCA or SCA in 75% and 25%, respectively and none were MNA. Periarterial NBs were NCA, SCA or MNA in 33%, 56% and 11%, respectively. Adrenal NBs were NCA, SCA or MNA in 16%, 36% and 48%, respectively. Among MNA NBs, 92% originated from the adrenal gland. The sympathetic anatomical classification was significantly better correlated to overall survival than the compartmental classification (P < .0003). The tumor volume of MNA NBs was significantly higher than NCA or SCA NBs (P < .0001). Patients with initial volume less than 160 mL had significantly better overall survival (P < .009). A “single mass” pattern was significantly more frequent in NCA NBs (P = .0003). The number of IDRFs was significantly higher in MNA NBs (P < .0001).ConclusionImaging phenotypes of neuroblastomas, including tumor origin along the sympathetic system, correlate with tumor genomic profile and patient outcome.
Highlights
Neuroblastomas (NBs) are the most common extracranial solid tumors in children
NBs arising from cervical sympathetic chains were all numerical-only chromosome alterations” (NCA)
The sympathetic anatomical classification was significantly better correlated to overall survival than the compartmental classification (P < .0003)
Summary
Neuroblastomas (NBs) are the most common extracranial solid tumors in children. NBs derive from the sympathetic nervous system originating from neural crest cells. These tumors may theoretically arise from any migratory pathway [1, 2]. NBs mostly arise from the abdomen (adrenal gland 48%, extra-adrenal retroperitoneum 25%), less frequently from the chest (16%) and rarely from the pelvis (3%) or the neck (3%)[3]. NBs are associated with remarkable biological heterogeneity and outcome. Some tumors undergo spontaneous regression, some are cured by surgery alone or after chemo-reduction, while other exhibit extremely aggressive behavior
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