This study investigated relationships between neuroblastomas (NBs) imaging phenotypes, tumor genomic profile and patient outcome.
This 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.
A 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).
Imaging phenotypes of neuroblastomas, including tumor origin along the sympathetic system, correlate with tumor genomic profile and patient outcome.
PloS one. 2017 Sep 25*** epublish ***
Hervé J Brisse, Thomas Blanc, Gudrun Schleiermacher, Véronique Mosseri, Pascale Philippe-Chomette, Isabelle Janoueix-Lerosey, Gaelle Pierron, Eve Lapouble, Michel Peuchmaur, Paul Fréneaux, Louise Galmiche, Nathalie Algret, Matthieu Peycelon, Jean Michon, Olivier Delattre, Sabine Sarnacki
Imaging Department, Institut Curie, Paris, France., Department of Pediatric Surgery, Necker-Enfants-Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France., Paris Sciences et Lettres Research University, Paris, France., Department of Pediatric Surgery and Urology, Robert Debré University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France., Department of Pediatric Oncology, Institut Curie, Paris, France., Paris-Diderot Sorbonne-Paris-Cité University, Paris, France., Paris-Descartes Sorbonne-Paris-Cité University, Paris, France., Department of Biostatistics, Institut Curie, Paris, France.