bluuebunny/crossref_metadata_2025_split huggingface dataset:
RARE-62. VISUAL FUNCTION IN CHILDREN WITH CRANIOPHARYNGIOMA AT DIAGNOSIS: A SYSTEMATIC REVIEW
Authors: Nuijts Myrthe , Veldhuis Nienke , Stegeman Inge , van Santen Hanneke , Porro Giorgio , Imhof Saskia , van Meeteren Antoinette Schouten 
Published: December 2020

Abstract
Childhood craniopharyngioma is a rare and slow growing brain tumour, often located in the sellar and suprasellar region. It commonly manifests with visual impairment, increased intracranial pressure and hypothalamic and/or pituitary deficiencies. Visual impairment in childhood adversely affects a child’s daily functioning and quality of life. We systematically reviewed the literature to provide an extensive overview of the visual function in children with craniopharyngioma at diagnosis in order to estimate the diversity, magnitude and relevance of the problem of visual impairment. Of the 543 potentially relevant articles, 84 studies met our inclusion criteria. Visual impairment at diagnosis was reported in 1041 of 2071 children (50.3%), decreased visual acuity was reported in 546 of 1321 children (41.3%) and visual field defects were reported in 426 of 1111 children (38.3%). Other ophthalmological findings described were fundoscopic (32.5%) and orthoptic abnormalities (12.5%). Variations in ophthalmological testing methods and ophthalmological definitions precluded a meta-analysis. The results of this review confirm the importance of ophthalmological examination in children with craniopharyngioma at diagnosis in order to detect visual impairment and provide adequate support. Future studies should focus on long-term visual follow-up of childhood craniopharyngioma in response to different treatment strategies to provide insight in risks and ways to prevent further loss of vision.
RONC-01. PROTON BEAM THERAPY IN THE MULTIDISCIPLINARY THERAPY FOR PEDIATRIC BRAIN AND SPINAL TUMOR AT KOBE CHILDREN’S HOSPITAL WITH KOBE PROTON CENTER
Authors: Kawamura Atsufumi , Koyama Junji , Akutsu Nobuyuki , Demizu Yuske , Fukumitsu Nobuyoshi , Soejima Toshinori , Kosaka Yoshiyuki 
Published: December 2020

Abstract
It could be remarked that Radiotherapy (RT) has the important role for the multidisciplinary therapy to Malignant Pediatric Central Nervous System tumor. And recently among RT, Proton Beam Therapy (PBT) is expected to be effective and decrease serious late effects after RT in malignant pediatric tumor. PBT could be controlled precisely the dose and depth and spare the normal structures outside the target. Thus, PBT becomes applicable for pediatric solid tumor to insurance in April, 2016 in Japan. We have worked in closer cooperation with Hyogo Prefectural Ion Beam Medical Center and started PBT from April 2015. And from December 2017, our PBT has transferred to adjacent new medical center (Kobe Proton Center) which has the only institute that equipped the exclusive gantry for children in our country. The treated cases are 28 boys and 35 girls (age average 8.2 years oid). They are 15 Germ cell tumor, 14 Ependymoma, 13 Medulloblastoma, 4 Chordoma, 4 Atypical teratoid/rhabdoid tumor, 2 Craniopharyngioma and others. We have simulated the applications of not only broad beam but also scanned beam to limit the dose distribution and prepare for the cranio-spinal irradiation. All cases underwent magnetic resonance imaging to evaluate the results at out clinic and also the complications are assessed after treatments. The effect of PBT in this series is similar to our experience of traditional RT. There are a few controllable complications such as conventional RT. Farther more follow up is necessary to evaluate the advantage of PBT which could reduce delayed complications of RT.
RONC-04. RE-IRRADIATION AFTER TREATMENT OF MEDULLOBLASTOMA; RELAPSED CASES AND SECOND CANCER CASES
Authors: Soejima Toshinori , Fukumitsu Nobutoshi , Demizu Yusuke , Mima Masayuki , Suzuki Takeshi , Kawamura Atsufumi , Kosaka Yoshiyuki 
Published: December 2020

Abstract

PURPOSE
Late complications such as brainstem necrosis are great concern of re-irradiation for brain tumor. Proton beam therapy can reduce radiation dose of organs at risk such as brainstem, so is expected to reduce late complications.

PATIENTS AND METHODS
Patients with medulloblastoma treated with re-irradiation from January 2015 to February 2019 at the Kobe Children’s Hospital and the Kobe Proton Center were reviewed. There were three cases of relapsed medulloblastoma and three cases of second cancer (glioblastomas).

RESULTS
In relapsed cases, all three cases treated with 12 Gy in 8 fractions cranio-spinal irradiation followed by gamma knife radiosurgery (one) or 28.8 Gy (RBE) in 16 fractions of proton beam therapy (two). Follow-up periods were 8 to 19 months (median 12 months) and all three cases survived without relapse. In second cancer cases, all three cases were treated with 40.05 Gy per 15 fractions of radiation therapy (2 cases were treated with photon and one case with proton). However, all cases relapsed and two cases died of disease.

CONCLUSION
Twelve Gy in 8 fractions cranio-spinal irradiation followed by 28.8 Gy (RBE) in 16 fractions of proton beam therapy is thought to be useful for the relapsed case. Re-irradiation for second cancer was disappointing and further study is warranted.
RONC-13. RADIATION INDUCED BRAIN STEM GLIOMA AFTER RADIATION THERAPY FOR MIXED GERM CELL TUMOR
Authors: Yamamura Natsumi , Nonaka Masahiro , Asai Akio 
Published: December 2020

Abstract
We report a case of radiation-induced glioma in the pons after radiation therapy for germ cell tumor. A 17-year-old man was diagnosed as HCG and AFP secreting germ cell tumor at the age of 9. The tumor was located in the suprasellar region, which filled up most part of the third ventricle. Five courses of chemotherapy with cisplatin, etoposide, and cyclophosphamide, and whole ventricle plus local radiation therapy (total 51.2 Gy / 32Fr) were performed. After the treatment, most part of the tumor was regressed, and only small enhanced lesion remained. Six years after the treatment, he started to be ataxic, and worsened. An MRI revealed an enhanced lesion in the pons. Lesion biopsy was performed via the right cerebellar peduncle. Histopathological diagnosis confirmed the lesion was high grade glioma. He underwent extended local radiation therapy (50.4 Gy / 28 Fr) and administered temozolomide. Later, bevacizumab was added, and 3 months after treatment started, the size of the tumor was reduced and his symptoms were improving. There is no established treatment for radiation induced glioma. However, additional radiation therapy, temozolomide and bevacizumab appears to be useful to reduce tumor size and resolve the symptoms, even if it is transient.
RONC-17. STEREOTACTIC RADIOSURGERY FOR SPINE METASTASES IN PEDIATRIC MALIGNANCIES
Authors: Woodhouse Kristina , Alvarez Victor Albornoz , Boyce David , Li Jing , Yeboa Debra , Grosshans David , Briere Tina , Tatsui Claudio , Rhines Laurence , Behrang Amini , McGovern Susan , Paulino Arnold , McAleer Mary Frances , Ghia Amol 
Published: December 2020

Abstract

BACKGROUND
Spine stereotactic radiosurgery (SSRS) is a non-invasive technique that delivers ablative radiotherapy for optimal control of bony disease. While SSRS is known to provide excellent local control (LC) and minimal toxicity in adults, the role of SSRS in pediatrics is less clear.

PURPOSE
To evaluate SSRS in pediatric patients with spinal metastases.

METHODS
A retrospective review of patients (&lt;18 yrs) treated with SSRS at MDACC was performed after IRB approval. Descriptive statistics were utilized for analysis.

RESULTS
From 2011–2019, 12 metastatic osseous sites (3 cervical, 4 thoracic, 5 lumbar-sacral) in 9 patients were treated. Median follow-up was 9 months (range 2–41). Six males (67%) and 3 females (33%) all KPS ≥70, received radiation to ≤3 contiguous vertebral bodies. Median age was 16 yrs (range 8–18). No patients required sedation. Histologies included 7 osteosarcomas, one rhabdomyosarcoma and one Ewing’s sarcoma. Metastatic epidural spinal cord compression scores ranged from 0 (6), 1b (3) and 3 (3). No sites had surgery prior to SSRS and one site received prior conventional radiation. SSRS doses included 24 Gy in 1 fraction (7), 24–27 Gy in 3 fractions (4) and 50 Gy in 5 fractions (1). Six-month LC was 83% with one local failure following 27 Gy. OS at 6 and 12 mo were 55% and 23%. There was no grade ≥3 acute toxicity, no radiation myelopathy or vertebral compression fractures.

CONCLUSION
In this initial report, SSRS represents a promising modality that is well tolerated and provides excellent LC. However, further follow-up is warranted in the pediatric setting.
RONC-18. ANALYSIS OF BRAIN TUMOR INDUCED BY IRRADIATION IN CHILDHOOD - A SINGLE INSTITUTIONAL ANALYSIS
Authors: Sano Takashi , Tamura Kaoru , Kuroha Masae , Sumita Kazutaka , Arai Yukika , Sugawara Takashi , Inaji Motoki , Tanaka Yoji , Nariai Tadashi , Maehara Taketoshi 
Published: December 2020

Abstract

BACKGROUND
Radiation-induced brain tumors are rare tumors that appear during long-term follow-up after radiation therapy. Children are at greater risk for radiation -induced brain tumors than adults. The clinical characteristics of radiation-induced brain tumor treated at our hospital were retrospectively examined.

PATIENTS AND METHODS
Clinical characteristics of seven radiation-induced brain tumors that developed in 6 patients irradiated in their childhood at our hospital were analyzed. The background disease, age at irradiation, irradiation dose, period from irradiation to onset, pathological diagnosis, and treatment for radiation-induced brain tumor were examined.

RESULTS
Background diseases for irradiation were leukemia in 3 patients, germinoma in 2, medulloblastoma in 1, and the average cranial irradiation dose was 23.2 Gy. The patients tended to be young at irradiation (2–17 yeays; median:4 years old). The time between irradiation and the onset of radiation-induced brain tumors ranged from 9.5 to 39.1 years (median:28 years). Radiation-induced brain tumors comprised 6 meningioma（grade I:5, grade II:1）and 1 high-grade gliomas. All patients underwent surgical removal of the radiation-induced brain tumors and 2 received additional irradiation. During a median of 5.3 years of follow-up after the diagnosis of radiation-induced brain tumors, 2 underwent second surgery, while the remaining 4 have no recurrence. DISCUSSION: In most cases, radiation-induced brain tumors occur for a long time after irradiation in childhood. Monitoring of radiation-induced brain tumors as well as primary tumor recurrence was considered important.
RONC-19. TWO CASES OF RE-IRRADIATION FOR LATE RECURRENT OR RADIATION-INDUCED TUMOR AFTER RADIATION THERAPY FOR PEDIATRIC BRAIN TUMORS
Authors: Mori Takashi , Yamaguchi Shigeru , Onimaru Rikiya , Hashimoto Takayuki , Aoyama Hidefumi 
Published: December 2020

Abstract

BACKGROUND
As the outcome of pediatric brain tumors improves, late recurrence and radiation-induced tumor cases are more likely to occur, and the number of cases requiring re-irradiation is expected to increase. Here we report two cases performed intracranial re-irradiation after radiotherapy for pediatric brain tumors. CASE 1: 21-year-old male. He was diagnosed with craniopharyngioma at eight years old and underwent a tumor resection. At 10 years old, the local recurrence of suprasellar region was treated with 50.4 Gy/28 fr of stereotactic radiotherapy (SRT). After that, other recurrent lesions appeared in the left cerebellopontine angle, and he received surgery three times. The tumor was gross totally resected and re-irradiation with 40 Gy/20 fr of SRT was performed. We have found no recurrence or late effects during the one year follow-up. CASE 2: 15-year-old female. At three years old, she received 18 Gy/10 fr of craniospinal irradiation and 36 Gy/20 fr of boost to the posterior fossa as postoperative irradiation for anaplastic ependymoma and cured. However, a anaplastic meningioma appeared on the left side of the skull base at the age of 15, and 50 Gy/25 fr of postoperative intensity-modulated radiation therapy was performed. Two years later, another meningioma developed in the right cerebellar tent, and 54 Gy/27 fr of SRT was performed. Thirty-three months after re-irradiation, MRI showed a slight increase of the lesion, but no late toxicities are observed.

CONCLUSION
The follow-up periods are short, however intracranial re-irradiation after radiotherapy for pediatric brain tumors were feasible and effective.
RONC-31. ADVANCED ECHOCARDIOGRAPHY WITH MYOCARDIAL-STRAIN-ANALYSIS DESCRIBES SUBCLINICAL CARDIAC DYSFUNCTION AFTER CRANIOSPINAL IRRADIATION (CSI) IN PEDIATRIC AND YOUNG ADULT PATIENTS WITH CENTRAL NERVOUS SYSTEM (CNS) TUMORS
Authors: Martinez Hugo , Salloum Ralph , Wright Erin , Khoury Philip , Tretter Justin , Ryan Thomas 
Published: December 2020

Abstract
CSI is part of the treatment of CNS tumors and is associated with cardiovascular disease; data in pediatric/young-adult patients are limited. Myocardial-strain-analysis can reveal subclinical dysfunction. Retrospective, single-center study in CNS tumor patients managed with CSI from 1986–2018. Clinical details, and echocardiography including myocardial-strain-analysis were collected at T1=first echocardiogram after CSI, and T2=most recent echocardiogram. Data are mean±standard deviation. Echocardiograms were available in 44 patients (36%female, 14±8.0years) at T1 and 39 patients (38%female, 21.0±11.3years) at T2. Standard echocardiography was normal for all subjects. At T1, global longitudinal peak systolic strain (GLS) was -16.3%±3.7% in CSI vs. -21.6%±3.5% in controls (p&lt;0.0001); global radial peak systolic strain (GRS) was 21.5%±10.1% in CSI vs. 26.5%±7.4% in controls, and global circumferential peak systolic strain (GCS) was -19.5%±6.0% in CSI vs. -21.4%±3.4% in controls (p&lt;0.05, both comparisons). At T2, GLS was -15.8%±5.2% in CSI vs. -21.9±3.5% in controls (p&lt;0.0001); GRS was 22.6%±10.4% in CSI vs. 27.1±8.2% in controls (p&lt;0.05); GCS was -20.5%±6.9% in CSI vs. -21.8±3.5% in controls (p=0.10). For 17 patients with myocardial-strain-analysis available for both time points: difference in GLS was 0.06±7.2% (p&gt;0.95); GRS was 5.5±9.5% (p&lt;0.05); GCS was -3.4±4.9% (p&lt;0.05). Subclinical dysfunction is present at first echocardiogram after CSI. Myocardial impairment may recover with time, however further analysis is needed to identify risk factors and trends. These results argue for inclusion of baseline cardiovascular assessment and longitudinal follow-up in CNS tumor patients post CSI.
RONC-32. LOCAL CONTROL FOLLOWING PROTON THERAPY FOR PEDIATRIC CHORDOMA
Authors: Indelicato Daniel , Rotondo Ronny , Vega Raymond , Holtzman Adam , Looi Wen Shen , Morris Christopher , Sandler Eric , Aldana Philipp , Bradley Julie 
Published: December 2020

Abstract

BACKGROUND
Due to the location and high dose required for disease control, pediatric chordomas are theoretically well-suited for treatment with proton therapy, but their low incidence limits the clinical outcome data available in the literature. METHODS AND MATERIALS: Between 2008 and 2019, 29 patients with a median age of 14.8 years (range, 3.8–21.8) received proton therapy for non-metastatic chordoma at a single institution. Twenty-four tumors arose in the clivus/cervical spine region and 5 in the lumbosacral spine. Twenty-six tumors demonstrated well-differentiated histology and 3 were dedifferentiated or not otherwise specified (NOS). Approximately half of the tumors underwent specialized testing: 14 were brachyury-positive and 10 retained INI-1. Seventeen patients had gross disease at the time of radiation. The median radiation dose was 73.8 GyRBE.

RESULTS
With a median follow-up of 4.3 years (range, 1.0–10.7), the 5-year estimates of local control, progression-free survival, and overall survival rates were 85%, 82%, and 86%, respectively. Excluding 3 patients with dedifferentiated/NOS chordoma, the 5-year local control, progression-free survival, and overall survival rates were 92%, 92%, and 91%, respectively. Serious toxicities included 3 patients with hardware failure or related infection requiring revision surgery, 2 patients with hormone deficiency, and 2 patients with Eustachian tube dysfunction causing chronic otitis media.

CONCLUSION
In pediatric patients with chordoma, proton therapy is associated with a low risk of serious toxicity and high efficacy, particularly in well-differentiated tumors. Complete resection may be unnecessary for local control and destabilizing operations requiring instrumentation may result in additional complications following therapy.
SURG-04. THE APPLICATION OF EN BLOC RESECTION IN THE OPERATION OF PEDIATRIC POSTERIOR FOSSA TUMOR
Authors: Jie Ma , Mao Weiwei , Tian Shuaiwei , Wang Baocheng , Zhao Yang 
Published: December 2020

Abstract

OBJECTIVE
To explore the efficacy and safety of en bloc resection therapy on posterior fossa tumor in children.

METHODS
A retrospective analysis was conducted on the clinical data of 94 patients with posterior fossa tumor admitted to Department of Pediatric Neurosurgery, Xinhua Hospital Affiliated to Shanghai Jiaotong University School Of Medicine from January 2018 to December 2019. Among them, 35 cases were treated with traditional resection (control group) and 59 cases with en bloc resection (observation group). We counted the amount of blood loss and the time during tumor resection, We compare the symptoms and signs between the two groups and determine a extent of tumor resection based on microscopic observation and preoperative and postoperative imaging comparison. RESULT: The total tumor resection rate of the observation group (88.1%, 52 / 59) was significantly higher than that of the control group (62.85%, 22 / 35, P &lt; 0.05). The average bleeding volume of 90.8ml in the observation group was significantly smaller than that of the control group (113.3ml, P &lt; 0.05), and the average operation time of 38.6min in the observation group was shorter than that of the control group (57.4min, P &lt; 0.05) only for tumor resection procedure.

CONCLUSION
En bloc resection technique can effectively accelerate the resection time, reduce intraoperative bleeding and improve the total resection rate of tumors in children’s posterior cranial fossa.
SURG-05. AN AWAKE SURGERY FOR A CHILD SUFFERING FROM EPILEPSY DUE TO DYSEMBRYPLASTIC NEUROEPITHELIAL TUMOR LOCATED IN THE LEFT PARIETAL LOBE
Authors: Kiyokawa Juri , Yamamoto Shinji , Murota Yasuhiro , Ishikawa Mariko , Yamaoka Hiroto , Kaneko Satoshi , Yoshimura Masataka , Hirota Shin , Inaji Motoki , Maehara Taketoshi 
Published: December 2020

Abstract

BACKGROUND
An awake surgery is a useful measure to remove tumors located close to eloquent areas of the brain to reduce surgical complications and maximize the resection. However, it has some disadvantages compared to surgeries under general anesthesia. Generally speaking, applying it to a child under 15 years-old (y/o) is hesitating because of anxiety, poor tolerance, failure to cooperate in tasks and so forth. Here, we present a case of a 13y/o girl who underwent an awake surgery due to dysembryplastic neuroepithelial tumor (DNT) located in the left parietal lobe. CASE PRESENTATION: She consulted our hospital for epileptic seizures. MRI showed a multilocular mass lesion in the left parietal lobe. The tumor was located in or close to eloquent areas. The epilepsy was refractory even with multiple antiepileptic drugs (AEDs). A Wada examination revealed that her speech area is on the left hemisphere. The operations were performed in two stages. Prior to the operations, we had several thought-out simulations in the operating room and ICU with her, her parents, and our staff including nurses and lab technicians. The first operation was to perform tumor biopsy and place intracranial electrodes. The histological diagnosis was DNT. Video electroencephalogram showed that the epileptogenic lesion was around the tumor. The second operation resulted in total tumor resection and reduction of paroxysmal epileptic spikes without major complications. She is seizure free for more than three years with two AEDs.

CONCLUSION
Careful preparations may enable an awake surgery even for a child under 15y/o.
SURG-09. REACTIVATION OF HERPES SIMPLEX VIRUS AFTER NEUROLOGIC SURGERY
Authors: Almeida Carlos , Mançano Bruna Minniti , Prado Seila Israel , Almeida Gisele Caravina , Souza Fernanda Magalhães , Lourenço Lucas Dias 
Published: December 2020

Abstract

BACKGROUND
Herpes simplex virus encephalitis (HSVE) is a rare complication after neurosurgery, and its clinical picture mimics features of other most frequent infectious complications of bacterial origin. Probable triggering factors are manipulation and surgical stress, since most cases occur due to reactivation rather than primary infection. The main symptoms include fever and altered consciousness. DNA identification of HSV by PCR has accuracy. Even with adequate treatment, HSVE is associated with a mortality of 30%, and potential neurologic sequelae such as cognitive and motor. CASE REPORT: An 18-year-old male patient presented with loss of vision due to cystic craniopharyngioma. We inserted an Omaya catheter and drained the cyst. On the third day, he presented with fever, seizures, and decreased consciousness. Magnetic resonance imaging (MRI) showed high signal intensity on T2-weighted and FLAIR images in the left frontal and temporal lobe, cingulate gyrus, and corpus callosum, with mass effect. He was submitted to decompressive craniectomy and empirical antibiotic therapy. CSF and blood cultures were negative. Due to inexpressive clinical improvement after 48 hours, CSF was collected for polymerase chain reaction (PCR), and we performed a brain biopsy and started intravenous acyclovir. Histology and PCR confirmed HSVE type 1 and 2. He received antiviral for two weeks and was discharged after CSF PCR negative.

CONCLUSION
Clinical suspicion, CSF PCR, and imaging are of paramount importance for early diagnosis of HSVE, which should be considered in the differential diagnosis of recent postoperative neurologic surgery in cases of unexplained postoperative fever with altered consciousness.