Management of Local-Regional Failure in Children With High-Risk Neuroblastoma: A Single Institution Experience

Abstract

all patient and tumor factors, adults were associated with less chance of receiving RT (pZ0.002) but not surgery (pZ0.084). The median and 5 year OS of adults continued to be significantly worse than those of pediatrics (p < 0.0001) which could be partially secondary to a lower chance of having RT in adults. Conclusion: Adult patients with PMS had inferior survival as compared to pediatric patients even following propensity matching of all patient and tumor factors. Adults had less odds of receiving RT which could be partially responsible for the poor outcomes. Further studies on adults adopting pediatric treatment regimens are warranted. TX Purpose/Objective(s): Radiation plays a pivotal role in the treatment of pediatric brain tumors. For such patients, proton radiation (PRT) has been utilized in an attempt to prevent neurocognitive deficits by sparing normal tissues. Recent studies suggest that minimizing dose to the hippocampus or subventricular zone (SVZ), may lessen the negative impact of radiation on neurocognition. We evaluated the relationship between change in neurocognitive scores over time and PRT dose to the hippocampus and SVZ. Materials/Methods: Pediatric brain tumor patients treated with partial brain PRT underwent serial neurocognitive testing. IQ, working memory (WMI), and processing speed scores (PSI) were examined. Hippocampal and SVZ were delineated, and dosimetric data compiled. In general linear mixed models controlling for tumor volume, associations were examined between IQ, WMI, and PSI score trajectories and: 1) median hippocampal and SVZ doses, and 2) percentage of hippocampus and SVZ receiving 50 (V50), 30 (V30) and 10 (V10) CGE. Results: Data were available for 15 of a total 44 planned patients (9 males, 6 females) receiving PRT between 2007 and 2015. The median age at PRT was 9 years (range 1.5 to 13 y). Patients were 4.8 years post-PRT at last testing on average (range 1.2-9.0 y). Eleven patients had supratentorial tumors (3 craniopharyngiomas, 1 ependymoma, 1 germinoma, 2 low grade gliomas, 1 desmoplastic ganglion cell tumor, 1 optic pathway, 1 ganglioblastoma, 1 DNET) and 4 patients had infratentorial tumors (1 ependymoma, 1 medulloblastoma, 1 desmoplastic ganglion, 1 tectal glioma). The median prescription dose was 50.4 Gy (RBE) (range 45 e 50.4) with median tumor volume of 7.7cm 3 (range 2.1 e 67.6cm 3 ). Significant decline in PSI scores over time-from-PRTwas observed (pZ0.03), while IQ and WMI scores were stable. There were no significant associations between change in scores over time and any clinical variables (age at RT, gross tumor volume (GTV), gender, and tumor location). Also, there were no significant associations between change in scores over time and any dosimetric variables in the models adjusting for tumor volume. There was a trend between PSI decline and GTV (p Z 0.057), and right hippocampal V50 (p Z 0.08). A trend was also seen between change in WMI scores and left SVZ V10 (p Z 0.07). Conclusion: Preliminary findings suggest processing speed is most vulnerable t...

Evaluating the RIST Molecular-Targeted Regimen in a Three-Dimensional Neuroblastoma Spheroid Cell Culture Model

“…NB exhibits diverse clinical behavior, from spontaneous remission [ 5 ] to a widespread metastatic disease with poor outcomes and event-free survival rates below 50% [ 2 , 6 , 7 ]. Despite intensive treatment regimens, the prognosis for patients with high-risk relapsed or treatment-refractory NB remains poor and novel treatment strategies are urgently needed [ 8 , 9 ]. The pre-treatment risk stratification for this cancer relies on the International Neuroblastoma Risk Group (INRG) classification system, including age at diagnosis, histologic category, grade of tumor differentiation, tumor cell ploidy, 11q aberration and status of the oncogene MycN [ 2 , 10 , 11 ].…”

Novel Therapies for Relapsed and Refractory Neuroblastoma

“…Recent data presented by Modak and colleagues and Manole and colleagues indicate that patients with isolated local recurrence can be treated successfully with surgical tumor resection and/or radiation therapy at sites of active disease [ 6 , 7 ]. These authors further recommend using chemotherapy regimens with demonstrated activity in children with recurrent neuroblastoma, rather than experimental therapy for localized relapses.…”