bluuebunny/crossref_metadata_2025_split huggingface dataset:
RARE-04. INTELLECTUAL DEVELOPMENT IN CHILDREN WITH PEDIATRIC CRANIOPHARYNGIOMA AFTER TUMOR REMOVAL
Authors: Oyoshi Tatsuki , Fujio Shingo , Higa Nayuta , Yonezawa Hajime , Yoshimoto Koji 
Published: December 2020

Abstract

INTRODUCTION
Intellectual assessment in children with craniopharyngioma after tumor removal is still unknown. We assessed intellectual development in children who underwent microsurgical resection in our institute over the last twelve years.

MATERIALS AND METHODS
Ten children among 41 patients with craniopharyngioma treated and followed at Kagoshima University Hospital between 2007 and 2019 were reviewed. We also assessed intellectual development in 10 years or younger children with craniopharyngioma one year after tumor removal. Intelligence was assessed using the Wechsler Intelligence Scale for Children-Fourth Edition (WISC-Ⅳ).

RESULTS
Ten children underwent microsurgical tumor removal. The mean age at surgery was 5.8 (range 1–10) years. Transcranial approach was performed in 8 children, transsphenoidal approach in two children. The mean follow up period was 110 months. Gamma knife surgery (GKS) was performed in 6 children less than 6 months after first surgery. Regional recurrences occurred in 5 children, and additional GKS was performed in four children, second microsurgical removal in one child. Severe obesity with a transient electrolyte imbalance occurred in one child. Eight children with GH deficiency underwent GH replacement therapy. Eight children were assessed working memory index (WMI), processing speed index (PSI), Perceptual reasoning index (PRI), and verbal comprehension index (VCI) using WISC 4. Each mean value of WMI, PSI, and PRI was lower than VCI, except for 2 children with normal full scale intelligence quotient.

CONCLUSION
WMI, PSI and PRI in children with intellectual disabilities were lower tendency than VCI after surgical removal of craniopharyngiomas in the present study.
RARE-36. DYSEMBRYOPLASTIC NEUROEPITHELIAL TUMORS: A REVIEW OF CLINICAL AND MOLECULAR CHARACTERISTICS, AND OUTCOME IN A PEDIATRIC POPULATION AT A SINGLE CENTER
Authors: Flores Valerie Cruz , Geller Thomas , Gomez Ignacio Gonzalez , Rodriguez Luis , Quintana Javier , Stapleton Stacie 
Published: December 2020

Abstract

BACKGROUND
Neuronal and mixed neuro-glial tumors of the central nervous system (CNS) are relatively rare. Dysembryoplastic neuroepithelial tumor (DNET) is a benign, rare, slow-growing tumor, but in many cases is associated with intractable epilepsy.

OBJECTIVE
To report the experience with DNET at a single free-standing children’s institution.

METHODS
A retrospective chart review of 24 patients with confirmed DNET between 2001 and 2019 was performed. Data was collected on clinical characteristics, tumor location, surgical management, histopathological and molecular findings, and outcomes.

RESULTS
Mean age at diagnosis was 10 years (range 2 to 19 years), with female predominance (54.2%). Most common presenting symptoms were seizures (79.2%) and headaches (12.5%). Location of the tumor was temporal (29.2%), frontal (25.0%), parietal (16.7%), cerebellar (12.5%) and occipital (4.2%). A gross total resection was achieved in half the cases. Recurrence occurred in 4 patients (16.7%), all of whom had subtotal resections. The average follow up since diagnosis was 4.6 years (range 0.3 to 14 years). Nineteen patients presented with seizures, of which 63.2% were seizure free after surgery. The samples with molecular genetic testing (microarrays or FISH), were all normal except one patient positive for BRAF V600E mutation.

CONCLUSIONS
This is the first and largest review of pediatric DNETs in the last 10 years. Despite majority of patients having a favorable outcome after surgery, a subset of patients remains symptomatic. As molecular mechanisms in DNET remain unknown, future aim is to describe the molecular characteristics of our DNET population, and correlate with outcomes.
RARE-44. CLINICAL CHARACTERIZATION AND OUTCOME; OUR EXPERIENCE OF CHORDOMAS IN PEDIATRIC AND YOUNG ADULTS
Authors: Gibson Amber , Baig Muhammad , Raza Shaan , Grosshans David , Zaky Wafik 
Published: December 2020

Abstract
Pediatric chordomas are exceedingly rare and there are limited data to guide treatment decisions. We report a retrospective analysis of 19 patients with chordomas who received treatment at our institution from 2001–2020. Of the 19 patients, 15 had clival (79%), 3 cervical and 1 sacral chordoma. There were 9 males (47%). Median age at diagnosis of 10.6 years. Eight patients had gross total (42%) and 11 (58%) had sub-total resection. As front line therapy 15 patients (79%) underwent surgery followed by radiation (1 photon and 14 proton), 3 patients (16%) received surgery and chemotherapy (anaplastic histology) and 1 patient received only surgery. For patients treated with radiation therapy the average prescribed dose was 70 Gy (range: 52–74). Post-surgery and radiation, 14 of 15 patients remained in remission. Five (26%) patients had progressive disease (PD) with median time to progression of 13 months of whom 3 died of disease at median of 18 months. Treatment of PD consisted of chemotherapy and radiation for 3, re-resection with radiation for 1 and chemotherapy alone for 1 patient. The patients with metastatic and anaplastic disease mean survival is 21 month versus 45 for the rest of the cohort. In summary, post-operative adjuvant radiation provided an overall good outcome in majority of patients. Patients with anaplastic pathology and metastasis at diagnosis had worse outcome. Those who relapsed, subsequent treatment was palliative at best with short survival. Molecular analysis is warranted in future for better disease stratification.
RARE-63. CYST WALL OF ADAMANTINOMATOUS CRANIOPHARYNGIOMA CONTAINS TUMOR CELLS THAT COULD LEAD TO RECURRENCE AFTER SURGERY
Authors: Zhao Chuan , Wang Ye , Liu Hongxing , Qi Xueling , Zhou Zhongqing , Lau Ching , Lin Zhixiong 
Published: December 2020

Abstract

BACKGROUND
Adamantinomatous craniopharyngioma (ACP) is the primary subtype of craniopharyngioma in children, frequently with mutations in exon 3 of the CTNNB1 gene. Most ACP consists of both a solid tumor and one or more cysts. Despite surgical resection of the solid tumor followed by radiation, recurrence involving the cystic component is common, suggesting that the cyst wall contains tumor cells. We present here conclusive molecular pathology evidence of the presence of tumor cells in the cyst wall similar to those in the solid tumor.

METHODS
We used standard H&E staining and immunohistochemistry (IHC) to compare the histopathology characteristics between the matched cyst wall and solid tumor of 11 cases of ACP as well as their CTNNB1 expression and exon 3 mutation.

RESULTS
Samples of the cyst wall and solid tumor were collected separately during the operation of 11 cases of ACP through careful dissection. The cyst wall showed the nested cell clusters and peripheral palisading epithelium which are identical to those in the solid tumor. The cyst wall and solid tumor both showed Ki67 and nuclear β-catenin expression by IHC. There is no difference in the transcription level of CTNNB1 between the cyst wall and the solid tumor, both being significantly higher than that in normal brain tissue. Exon 3 mutations of the CTNNB1 gene of the cyst wall and the solid tumor are identical in each case.

CONCLUSION
Follow-up of clinical cases suggests that tumor cells in residual cyst wall may be the cause of recurrence after surgery.
RONC-09. PSEUDOPROGRESSION AFTER PROTON THERAPY OF PEDIATRIC SPINAL PILOCYTIC ASTROCYTOMA AND MYXOPAPILLARY EPENDYMOMA
Authors: McGovern Susan , Johnson Jason , Kralik Stephen , Grosshans David , McAleer Mary Frances , Zaky Wafik , Baxter Patricia , Lin Frank , Chintagumpala Murali , Paulino Arnold 
Published: December 2020

Abstract

BACKGROUND
Pseudoprogression after proton therapy of CNS tumors is a challenging clinical situation. The rate of pseudoprogression after proton therapy of pediatric spinal tumors is unknown.

METHODS
Records of pediatric patients with spinal pilocytic astrocytoma (sPA; n = 9) or myxopapillary ependymoma (MPE; n = 6) with gross disease treated with proton therapy with at least 6 months of follow up from completion of proton therapy were retrospectively reviewed for demographics, treatment characteristics, and occurrence of pseudoprogression. Pseudoprogression was defined as a post-radiation increase in tumor size with subsequent decrease in size without additional tumor-directed therapy.

RESULTS
The median age at radiation for sPA patients was 10.1y (range, 7.0 – 16.2y) and 12.7y (range, 7.9 – 14.4y) for MPE patients. The median prescribed dose was 45 GyRBE (range, 39.6 – 50.4 GyRBE) for sPA patients and 50.4 GyRBE (range, 45 – 54 GyRBE) for MPE patients. One sPA patient received concurrent vincristine. Median follow up after proton therapy was 44 months (range, 9 – 99 months). Six of nine sPA patients (67%) had pseudoprogression occurring at a median of 81 days (range, 34 – 136 days) after proton therapy; no MPE patients developed pseudoprogression (0%; p &lt; 0.03). Two sPA patients with pseudoprogression were symptomatic and improved with medical therapy.

CONCLUSION
Preliminary analysis suggests that pseudoprogression occurs frequently within 6 months after proton therapy for sPA and infrequently after proton therapy for MPE.
RONC-12. TREATMENT AGE AND NEUROCOGNITIVE OUTCOMES FOLLOWING PROTON BEAM RADIOTHERAPY FOR PEDIATRIC LOW GRADE GLIOMA
Authors: Heitzer Andrew , Kahalley Lisa , Grosshans David , Okcu M Fatih , Raghubar Kimberly , Gragert Marsha , McCurdy Mark , Warren Emily , Ris M Douglas , Paulino Arnold , Chintagumpala Murali 
Published: December 2020

Abstract

INTRODUCTION
Younger age at radiotherapy increases cognitive risk for patients with pediatric low grade glioma (LGG). We examined the impact of age at treatment on cognitive trajectories in LGG patients treated with proton radiotherapy (PRT) compared to patients treated without radiotherapy (surgery only; SO).

METHODS
We examined cognitive scores of 48 LGG patients on a prospective, longitudinal study. General linear mixed models evaluated change in cognitive scores over time.

RESULTS
The sample included 16 patients treated with PRT and 32 with SO (median follow-up=3.1 years, range 0.9–6.1). Median age of PRT patients was 8.2 years at diagnosis (range 1.0–14.4) and 9.4 years at PRT (range 4.2–16.7). 13 PRT patients also received surgery: 53.8% biopsy, 30.8% subtotal resection, 15.4% gross total resection. Tumor sites included: 31.2% hypothalamic/suprasellar, 25.0% optic pathway, 18.8% temporal, 25.0% other. Median age of SO patients was 8.2 years at diagnosis (range 2.9–18.6). Surgical outcomes were: 75.0% gross total resection, 21.9% biopsy/other. There were no group differences in diagnosis age, tumor volume, or shunt history (all p&gt;0.05). Both PRT and SO groups displayed stable cognitive functioning over time (all p&gt;0.1). Slopes (i.e., change in scores over time) did not differ between groups (all p&gt;0.1). Age at treatment was not associated with slope or performance at last follow-up in either group (all p&gt;0.05).

CONCLUSIONS
We observed stable cognitive functioning, independent of age at treatment, following PRT for LGG. Outcomes were similar to patients receiving surgery only. Further examination in a larger sample is warranted.
RONC-16. PROTON BEAM THERAPY FOR PATIENTS WITH INTRACRANIAL EPENDYMOMA UNDER 3 YEARS OLD: INITIAL CLINICAL OUTCOMES
Authors: Hashimoto Takayuki , Yamaguchi Shigeru , Mori Takashi , Iguchi Akihiro , Ishi Yukitomo , Motegi Hiroaki , Onimaru Rikiya , Manabe Atsushi , Shimizu Shinichi , Aoyama Hidefumi 
Published: December 2020

Abstract

BACKGROUND
Proton beam therapy (PBT) provides dosimetric benefits in sparing normal tissue when treating pediatric patients with brain tumors. We report the preliminary clinical outcomes of surgery and adjuvant PBT for patients under 3 years old diagnosed as intracranial ependymoma at our institute.

METHODS
This is a retrospective review of the medical records for 3 children with ependymoma in the fourth ventricle, diagnosed between March 2013 and September 2019. PBT was performed after tumor resection in all the patients.

RESULTS
Gross total resection was achieved in 2 males and 1 female patients with fourth ventricle WHO grade II to III ependymoma at 15, 18, and 37 months old. All the patients received adjuvant PBT (54.0 GyE/30 fractions) to the postoperative tumor bed under general anesthesia or sedation. PBT was acutely well tolerated, with mostly mild alopecia and skin reactions at the irradiated sites. At a median follow-up of 54 months (4–59 months) after irradiation, all the patients are alive without recurrence. No serious late adverse events were observed in any of the patients.

CONCLUSION
The number of patients in this study remains small for drawing any definite conclusion, however our preliminary results are still encouraging. Further studies of a large number of pediatric patients with long term follow-up are needed to more fully assess tumor control and late adverse events.
SURG-22. CERVICAL SPINE ANEURYSMAL BONE CYST OF A PEDIATRIC PATIENT
Authors: Almeida Carlos , Ventura Luis Marcelo , Previdelli Stephanie , Lamim Marina Lopes , Mançano Bruna Minniti , Cavalcante Carlos Bezerra , Lourenço Lucas Dias 
Published: December 2020

Abstract

BACKGROUND
Aneurysmal bone cysts (ABCs) are benign, expanding lesions that represent 15% of all primary spine tumors, and only 2% have been found at the cervical level. There are different therapeutic options; the most successful is complete surgical resection. Although not always possible, due to high blood loss that occurs during the procedure, a combination of surgery with other treatment modalities was used in 40% of the cases reported so far. We describe a pediatric patient that we managed with embolization plus surgery. CASE REPORT: A 5-year-old girl presented with painful torticollis associated with a left posterior cervical mass, without neurological impairment. Magnetic resonance imaging of the cervical spine showed a multiseptated bony lesion with multiple fluid levels, involving the posterior elements of C2, associated with diffuse soft tissue enhancement of the left paravertebral muscles. We proposed a multi-staged treatment with pre-operative arterial embolization followed by the posterior surgical approach. Super selective embolization of the left ascending cervical artery was performed. The right ascending cervical artery also contributed to the tumor blush, but due to its connection to the right vertebral artery and, therefore, associated with a high risk of neurological injury, we prefer not to embolize it. Two days later, we performed a posterior surgical approach, with a gross total resection of the tumor. Histological examination revealed an ABC.

CONCLUSION
An aneurysmal bone cyst is a rare cervical spine lesion that demands a multidisciplinary approach due to its locally aggressive behavior and the excessive blood loss related to surgery.
SURG-29. A SINGLE CENTRE EXPERIENCE OF USING INTRA-OPERATIVE MRI IN MANAGING PEDIATRIC CRANIAL NEURO-ONCOLOGY CASES
Authors: Ganesan Dharmendra , Bahuri Nor Faizal Ahmad , Rajagopal Revathi , PY Jasmine Loh , Mun Kein Seong , Aman Raja Rizal Azman Raja 
Published: December 2020

Abstract
The University of Malaya Medical Centre, Kuala Lumpur had acquired a intraoperative MRI (iMRI) brain suite via a public private initiative in September 2015. The MRI brain suite has a SIEMENS 1.5T system with NORAS coil system and NORAS head clamps in a two room solution. We would like to retrospectively review the cranial paediatric neuro-oncology cases that had surgery in this facility from September 2015 till December 2019. We would like to discuss our experience with regard to the clear benefits and the challenges in using such technology to aid in the surgery. The challenges include the physical setting up the paediatric case preoperatively, the preparation and performing the intraoperative scan, the interpretation of intraoperative images and making a decision and the utilisation of the new MRI data set to assist in the navigation to locate the residue safely. Also discuss the utility of the intraoperative images in the decision of subsequent adjuvant management. The use of iMRI also has other technical challenges such as ensuring the perimeter around the patient is free of ferromagnetic material, the process of transfer of the patient to the scanner and as a consequence increased duration of the surgery. CONCLUSION: Many elements in the use of iMRI has a learning curve and it improves with exposure and experience. In some areas only a high level of vigilance and SOP (Standard operating procedure) is required to minimize mishaps. Currently, the iMRI gives the best means of determining extent of resection before concluding the surgery.
THER-08. SGT53 – A NOVEL p53 NANOMEDICINE INDUCES SIGNIFICANT RESPONSES IN CHILDREN WITH RECURRENT MEDULLOBLASTOMA AND CHOROID PLEXUS CARCINOMA: A REPORT OF TWO CASES
Authors: Hwang Eugene , Kilburn Lindsay , Hancock Lauren , Pirollo Kathleen , Leung Chris , Packer Roger , Chang Esther 
Published: December 2020

Abstract

BACKGROUND
Abnormal p53 function commonly defines high-risk CNS tumors, but functional restoration has eluded investigators. SGT-53 is a targeted nanomedicine encapsulating a plasmid DNA encoding wild-type human p53 with a transferrin receptor-targeting scFv on the nanocomplex surface resulting in efficient delivery across the BBB and robust tumor binding/uptake. Data generated by collaborators demonstrated synergy with irradiation and chemotherapy. REPORT: Two children with recurrent CNS malignancies have been treated with SGT-53, each receiving greater than 50 infusions in combination with irradiation and chemotherapy with no grade 3/4 AEs positively attributed to SGT-53. The first was an 11yo male with recurrent disseminated p53+ SHH medulloblastoma, with bulky intracranial/thoracolumbar disease. He received irradiation followed by biweekly doses of SGT-53 and temozolomide, bevacizumab and irinotecan given one week out of four. This patient exhibited a complete response of all disease on his first follow-up scan 8 weeks after therapy initiation and remained in remission for 8 months. The second patient was a 3yo male with disseminated recurrent choroid plexus carcinoma. He received CSI with SGT-53, followed by SGT-53, temozolomide and irinotecan as described above, again with no related grade 3/4 AEs. He experienced a partial response to all sites of disease and completed therapy after six months, progressing 7.8 months after initiating treatment.

CONCLUSIONS
SGT-53 was well tolerated in two heavily-pretreated patients despite aggressive combinatorial strategies. The majority of the AEs experienced were mild and manageable. Each patient had significant responses, suggesting that SGT-53 should be evaluated in a clinical trial for similar patients.
A remark on renormalization group theoretical perturbation in a class of ordinary differential equations
Authors: Kuniba Atsuo 
Published: December 2020

Abstract
We revisit the renormalization group (RG) theoretical perturbation theory on oscillator-type second-order ordinary differential equations. For a class of potentials, we show a simple functional relation among secular coefficients of the harmonics in the naive perturbation series. It leads to an inversion formula between bare and renormalized amplitudes and an elementary proof of the absence of secular terms in all orders of the RG series. The result covers nonautonomous as well as autonomous cases and refines earlier studies, including the classic examples of Van der Pol, Mathieu, Duffing, and Rayleigh equations.
Sex estimation from morphology in living animals and dinosaurs
Authors: Motani Ryosuke 
Published: December 2020

AbstractSexual dimorphism is a prevalent feature of sexually reproducing organisms yet its presence in dinosaurs has recently been questioned. However, the inferred absence of sexual dimorphism may be a methodological artefact, rooted in the lack of systematic knowledge concerning how sexual dimorphism of living animals behaves statistically. To start building such knowledge, I re-analysed published data of 139 species of living animals that are sexually dimorphic. The previous method used for dinosaurs recognized only 5% of the living species correctly as dimorphic. This low rate is largely caused by the tilting of ordinated multivariate space due to interactions between size and shape dimorphisms, low signal/noise ratios and inclusion of outliers. The rate can be improved to 50% by modifying the method but not further, unless the information on the sex of individual specimens is used. Such information is unavailable in dinosaurs, so sexual dimorphism probably cannot be established for a large proportion of sexually dimorphic dinosaurs. At the same time, about 32% of the 139 are strongly sexually dimorphic, and can be re-sexed from shape with misclassification rates below 0.05. A reassessment of dinosaurian data suggests that sexual dimorphism likely existed at least in some species, such as Allosaurus fragilis.
Physical Activity as a Predictor of Chronic Pain Following Pediatric Spinal Surgery
Authors: Rabbitts Jennifer A. , Holley Amy L. , Zhou Chuan , Chen Lucas 
Published: December 2020

Objectives:
(1) Characterize objective physical activity patterns via actigraphy over 4 months postspinal fusion surgery, and (2) examine associations between activity patterns at 2-week and chronic postsurgical pain (CPSP) status at 4 months.

Materials and Methods:
Data from 109 youth (10 to 18 y) who underwent spinal fusion surgery at a children’s hospital in the Northwestern United States were analyzed. Youth completed questionnaires and actigraphic assessment of physical activity presurgery, and 2 weeks and 4 months postsurgery.

Results:
Eighteen percent of youth developed CPSP at 4 months. Presurgery physical activity was similar for youth with and without CPSP. At 2 weeks postsurgery, daily activity levels were lower for youth who developed CPSP as compared with those who did not, including lower mean activity (168 vs. 212 counts/min, P=0.01), fewer activity bouts (n=1.7 vs. 2.6, P=0.02), and shorter bout duration (27 vs. 40 min, P=0.02). Differences in activity were maintained at 4 months such that youth with CPSP had lower mean activity (284 vs. 334 counts/min, P=0.03), as well as greater proportion time spent sedentary and lower proportion time in light activity than youth without CPSP. In adjusted models examining 2-week actigraphy as a predictor of 4-month pain status, mean activity (odds ratio=0.99, P=0.04) and number of activity bouts (odds ratio=0.79, P=0.02) were associated with subsequent CPSP status over and above 2-week pain intensity.

Discussion:
Lower activity engagement during the initial weeks following spinal fusion surgery was associated with development of CPSP, suggesting early physical activity limitations as a risk factor for CPSP in youth. Understanding recovery patterns is critical for identifying those at risk for chronic pain and implementing early interventions.