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2018 AANS Annual Scientific Meeting
722. Pediatric supratentorial ependymoma: surgical ...
722. Pediatric supratentorial ependymoma: surgical, clinical, and molecular analysis
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Video Transcription
Thank you. Our next presenter is Jock Lillard, discussing pediatric supratentorial ependymomas. Thank you, guys. They said, my name is Jock Lillard. I'm a fourth-year medical student from the University of Tennessee and soon to be incoming intern at that institution as well in July. And I want to talk to you a little bit about our experience with supratentorial ependymomas. This study was conducted largely at St. Jude Children's Research Hospital in Memphis. And what we did was we went back and performed a retrospective review of all supratentorial ependymomas starting from 1990. And we went through the year end of 2015. And we pulled 73 such patients. And we performed various comparisons looking at specifically outcome measures being progression-free survival and overall survival. And the groups that we were comparing are listed here at the bottom. There are four of them. We looked at patients either age younger than three at diagnosis or greater than three with grade two or grade three tumors, patients who either underwent gross total resection or less than gross total resection. And finally, we looked at molecular status of the tumors. So a little bit about our patient model here. We had 73 patients, 41 of them being female. And the median age of diagnosis was just under seven years of age. The majority of our patients were greater than three years of diagnosis. And the majority of the tumors were grade three. A vast majority of the patients in this study experienced a gross total resection. And then upon review of residual tumors by our staff neuroradiologists, we determined that we actually had no subtotal resections. All patients with residual had less than a cubic centimeter of tumor remaining. So we had either all gross total or near total resections. And there were 51 of the 73 patients who had a tissue that was available for molecular testing. So this is a bivariate analysis chart from our paper. But basically all of this goes to show that we, and I'll get to the highlighted portion in a moment, there was no statistical significance for age of diagnosis or tumor histology in terms of progression-free survival or overall survival. Interestingly, gross total or section approach statistical significance. However, it did fall just short at .061. And surprisingly, our RelA positive patients did statistically significantly better than those without either with a C11 ORF95 gene rearrangement or a double negative group. So kind of recapping what that prior chart just said. Age of diagnosis and tumor grade had no effect. GTR, while it trended towards statistical significance, did not quite reach threshold. And our RelA fusion patients actually did better. So we started to compare the three different molecular groups, and it was noteworthy that the mortality rates between the RelA fusion group, the C11 ORF95 rearrangement group, and the double negative groups were all fairly similar. So we started to, you know, ponder why these results occurred given that prior studies had found and certainly purported that the RelA fusion would be a negative prognostic indicator. So some of our thoughts on the matter were these as listed on the slide, that GTR could overcome some deleterious effects of the fusion. This would seem to be somewhat surprising given the oncogenic nature of the fusion. There was a discrepancy in GTR rate between our molecular groups. As you can see, the RelA fusion group did have a higher rate of GTR compared to the other controls, so to speak, but not overwhelmingly more. But then there was an imbalance in the number of tumors for which we had tissue in testing. We had 34 RelA tumors, and again, remember we only had 51 total patients with the tissue for testing. So the other 17, there were eight gene rearrangements and nine double negatives. So certainly it's RelA heavy in the molecular testing group. And there was a difference in follow-up for the two groups. We had a longer follow-up in the RelA group as compared to the other groups. So takeaways from this study, we certainly want to, you know, champion maximal safer section when possible. We looked at surgical morbidity through decades. Since we started in 1990, we looked at morbidity rates from 1990 to 2000, 2000 to 2010, and then 2010 to present, and found that permanent morbidity rates were falling steadily with, you know, the advent of improved imaging modalities, the increased availability of staph neuroradiologists, and so forth. So we believe that maximal safer section is certainly something that we certainly, you know, want to achieve. But in regards to the RelA fusion, I think we, you know, our results in our study at least begs the question, is there something in our study that, you know, allowed the RelA fusion patients to actually do better, whether it be the imbalance of patients in our cohort, or, you know, perhaps there's something about the RelA fusion that we don't quite yet fully understand and haven't elucidated. So we're looking for more studies to corroborate or refute our results. Thank you.
Video Summary
In this video, Jock Lillard, a fourth-year medical student from the University of Tennessee, discusses a study conducted at St. Jude Children's Research Hospital on pediatric supratentorial ependymomas. The study looked at 73 patients and compared various factors including age at diagnosis, tumor grade, extent of tumor resection, and molecular status of the tumors. The results showed that age at diagnosis and tumor grade did not have an impact on progression-free survival or overall survival. However, patients with a RelA fusion showed better outcomes compared to those with a C11 ORF95 gene rearrangement or with no identifiable molecular abnormality. The study suggests the need for further research to understand the reasons behind these findings.
Asset Caption
Jock Lillard
Keywords
pediatric supratentorial ependymomas
molecular status
progression-free survival
overall survival
RelA fusion
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