bluuebunny/crossref_metadata_2025_split huggingface dataset:
EPID-13. A POPULATION-BASED ANALYSIS OF CNS TUMOR DIAGNOSES, TREATMENT, AND SURVIVAL IN CONGENITAL AND INFANT AGE GROUPS
Authors: Hart Muriel , Mellies Amy , Beltrami Alina , Gilani Ahmed , Green Adam 
Published: December 2020

Abstract

BACKGROUND
Congenital (&lt;3 months) and infant (3 to 11 months) brain tumors are biologically different from tumors in older children, but epidemiology of these tumors has not been studied comprehensively. Insight into epidemiological differences could help tailor treatment recommendations by age and increase overall survival (OS).

METHODS
Population-based data from the SEER 18 registries was obtained for 14,493 0-19-year-olds diagnosed with CNS tumors between 1990 and 2015. Incidence, treatment, and survival were analyzed using Chi-square and Kaplan-Meier analyses.

RESULTS
Between the &lt;3 month, 3–5 month, 6–11 month, and 1–19 year age groups, tumor type distribution differed significantly (p&lt;0.001); high-grade glioma (HGG) was most common in the &lt;3-month-olds, while low-grade glioma (LGG) was most common in the other groups. 5-year OS for all tumors was 36.7% (&lt;3 months), 56.0% (&lt;3–5 months), 63.8% (6–11 months), and 74.7% (1–19 years) (log rank p&lt;0.001). OS by tumor type was worst for &lt;3-month-olds with LGG, medulloblastoma, and other embryonal tumors; OS was worst for 3-5-month-olds with ependymoma, &lt;1-year-olds collectively with atypical teratoid-rhabdoid tumor, and 1-19-year-olds with HGG (log rank p&lt;0.02 for all tumor types). &lt;3-month-olds were least likely to receive any treatment for each tumor type and least likely to undergo surgery for all except HGG. &lt;1-year-olds were far less likely than 1-19-year-olds to undergo radiation for embryonal tumors, as expected, but were also less likely to undergo chemotherapy.

CONCLUSIONS
Congenital/infant CNS tumors differ pathologically, therapeutically, and prognostically from those in older children. Treatment changes could help address poorer outcomes for these young patients.
EPID-14. GABRIELLA MILLER KIDS FIRST DATA RESOURCE CENTER: COLLABORATIVE PLATFORMS FOR ACCELERATING RESEARCH IN PEDIATRIC CANCERS & STRUCTURAL BIRTH DEFECTS
Authors: Heath Allison P , Zhu Yuankun , Mattioni Michele , Farrow Bailey , Lilly Jena , Guo Yiran , Rokita Jo Lynne , Storm Phillip B , Volchenboum Samuel L , Nazarian Javad , Vasilevsky Nicole , DiGiovanna Jack , Haendel Melissa , Grossman Robert L , Davis-Dusenbery Brandi , Taylor Deanne M , Ferretti Vincent , Resnick Adam 
Published: December 2020

Abstract
Since launching to the public in September 2018, the Gabriella Miller Kids First Data Resource Center (DRC) has made an increasing number of pediatric genomic studies available to the research community. Currently, 1.3 PBs of genomic and clinical data drawn from 12,000 participants are available across a variety of pediatric cancer and structural birth defect studies. The DRC has architected a secure, cloud-based platform with over 1,300 users that supports the ability of researchers to not only find, access, and reuse data, but also integrate, collaborate, and analyze data quickly at scale. Users can use integrations with platforms such as Cavatica for bioinformatics workflows and PedcBioPortal for cancer genomic visualizations. Additionally, a set of framework services, powered by Gen3, provide a foundation for interoperability with other large-scale data sources, platforms, and a growing ecosystem of analysis and visualization applications. These integrations allow users to search across both TARGET and Kids First clinical data in one location while allowing data governance to be maintained by the original approvers. The new “explore data” feature allows users to search across all studies in order to identify virtual cohorts. Within the portal, these cohorts can be saved and shared with collaborators for iterative refinement and analysis. With appropriate approvals, the associated genomic data can be accessed and analyzed seamlessly in Cavatica or other platforms with interoperable framework services. Additionally, gene searching capabilities will be available in 2020. Data is free to download and cloud credits are available for analysis support.
EPID-15. THE INTERNATIONAL DIFFUSE INTRINSIC PONTINE GLIOMA (DIPG)/DIFFUSE MIDLINE GLIOMA (DMG) REGISTRY AND REPOSITORY (IDIPGR) EXPANSION
Authors: Torontali Marianne , Doughman Renee , Chaney Brooklyn , Black Katie , Asher Anthony , Rupert Andrew , Fuller Christine , Leach James , Jones Blaise , Fouladi Maryam , DeWire Mariko 
Published: December 2020

Abstract
Established in April 2012, the mission of the IDIPGR is to provide secure integrated data sets including clinical, pathologic, radiologic and molecular genomics to the research community to promote hypothesis driven research. Over 600 data points per patient are securely stored on a CCHMC constructed web resource and domain using the open-source data mart development framework Harvest (PMID:24303304) (‘Links’). Genomic data is stored in the cloud-enabled VIVA platform and accessed through cross-platform integration and standardization algorithms for comparison across datasets. Features include source identification, data wrangling, and standardization of molecular and phenotypic data (2017), a web-enabled data mart that provides phenotype-genotype query/exploration, along with raw and processed data file downloads to authorized investigators (Harvest, 2017), additional tools for filtering and analysis of genomic datasets at the level of a phenotype, sample, gene, and variant (VIVA, 2017–2018), and uploaded digitized slides (Aperio, 2019). The IDIPGR Repository stores abstracted datasets for &gt;1020 patients with DIPG/DMG, of whom 366 have tumor tissue available through biopsy and/or autopsy, and centrally reviewed and digitized specimens from 124 patients. The Repository contains &gt;5000 radiology studies from &gt;700 patients, with &gt;550 patients centrally reviewed, and genomics data from 80 patients. Currently 27 IDIPGR approved projects utilize these datasets. The DIPG/DMG Registry constructed a robust database platform and integration system that provides the infrastructure to promote highly collaborative, international, hypothesis-driven research. Broadening collaboration among investigators for hypothesis-driven research studies will lead to better classification and more effective treatment of patients with DIPG and DMG.
ETMR-11. A CASE OF PRIMARY DIFFUSE LEPTOMENINGEAL PRIMITIVE NEUROECTODERMAL TUMOR
Authors: Sugawa Masahiro , Terashima Keita , Matsukawa Yukihiro , Mizuno Takanori , Isshiki Kyohei , Tsujimoto Shinichi , Uchiyama Meri , Sakamoto Kenichi , Gocho Yoshihiro , Osumi Tomoo , Shioda Yoko , Kiyotani Chikako , Kato Motohiro , Tomizawa Daisuke , Usami Kenichi , Ogiwara Hideki , Tsutsumi Yoshiaki , Nakano Masayuki , Yoshioka Takako , Matsumoto Kimikazu 
Published: December 2020

Abstract

BACKGROUND
Primary diffuse leptomeningeal primitive neuroectodermal tumor (PDL PNET) is a rare embryonal brain tumor which arises primarily in the meninges without an intraparenchymal mass. Few previous reports of this condition exist, and the clinical outcomes are poor. We herein report a case of a child with PDL PNET and present a cursory review of the literature. CASE: A 3-year-old female patient was seen at a local clinic due to vomiting, headaches, and seizures. As a head MRI revealed hydrocephalus but no mass, acute encephalopathy was initially diagnosed. She received steroid pulse therapy, but the symptoms progressed to hallucination and lethargy. Another MRI at the 1-month follow-up revealed diffuse leptomeningeal enhancement. Thereafter she was transferred to our hospital. A spine MRI revealed spinal dissemination. She underwent a dura mater biopsy, and the pathological analysis led to the diagnosis of PDL PNET. She received chemotherapy consisting of vincristine, cyclophosphamide, etoposide, cisplatin, and intrathecal methotrexate injections two months after the initial presentation. The progressive hydrocephalus was managed with external ventricular drainage. Two weeks after the first cycle of chemotherapy the hydrocephalus resolved, and the external ventricular drainage was removed. A follow-up MRI showed that the leptomeningeal enhancement decreased during the four cycles of chemotherapy without radiotherapy. The patient is scheduled to receive high-dose chemotherapy as consolidation therapy.

CONCLUSION
PDL PNET is extremely rare, and its diagnosis is often delayed. Treatment of PDL PNET is very difficult due to its aggressive course, and surgical resection is impossible. Early diagnosis may help improve outcomes.
ETMR-20. IMPACT OF HIGH DOSE CHEMOTHERAPY WITH AND WITHOUT METHOTREXATE (MTX) ON OUTCOME OF PATIENTS WITH EMBRYONAL TUMORS WITH MULTI-LAYERED ROSETTES (ETMRs): A REPORT FROM CHILDREN’S ONCOLOGY GROUP PHASE III TRIAL ACNS0334
Authors: Mazewski Claire , Kang Guolian , Kellie Stewart , Gossett Jeffrey , Leary Sarah , Li Bryan , Aridgides Paul , Hayes Laura , Reddy Alyssa , Shaw Dennis , Burger Peter , Judkins Alexander , Geyer Jeffrey Russell , Fouladi Maryam , Huang Annie 
Published: December 2020

Abstract
Infant embryonal brain tumors comprise a spectrum of histologic and molecular entities including medulloblastoma (MB) and tumors collectively called CNS PNET’s, including supratentorial PNET (sPNET), pineoblastoma and other less common histologic entities. Non-MB embryonal tumors, historically considered high risk disease, were included in ACNS0334, A Children’s Oncology Group prospective phase III trial which compared efficacy of an induction regimen with and without methotrexate combined with high dose chemotherapy and stem cell rescue; no radiation was mandated. Molecular testing performed after ACNS0334 closure identified 14 patients with embryonal tumors with multi-layered rosettes (ETMRs), a new molecular entity previously classified under various diagnostic categories. ETMR patients made up 20% of the molecularly analyzed ACNS0334 cohort and were predominantly females. Tumors were largely non-metastatic (10/14 M0, 1 M1, 3 M2/M3) and originated in the cerebrum (8), cerebellum (3) and pineal gland (3). Gross total tumor resection was achieved in 5/11 patients with M0/M1 disease; 9/14 patients completed full treatment with 5 randomized to MTX induction and 9 to no-MTX. Five of 14 patients progressed on treatment, one had a toxic death. Disease progression was primarily local (88 %). No difference by methotrexate randomization was observed. Four patients are alive without progression 5–10+ years off therapy, none received radiation. No patients received radiation prior to progression. Four were irradiated after progression and died from disease within 3 to 13 months. Our study, a first report on ETMRs prospectively treated on a clinical trial, suggests high dose chemotherapy benefits a portion of ETMR patients.
GCT-08. PROTON BEAM RADIOTHERAPY FOR PEDIATRIC AND YOUNG-ADULT PATIENTS WITH INTRACRANIAL GERM CELL TUMOR
Authors: Sugiyama Minako , Hashimoto Takayuki , Mori Takashi , Hara Kazuya , Terashita Yukayo , Hirabayashi Shinsuke , Cho Yuko , Iguchi Akihiro , Okamoto Michinari , Ishi Yukitomo , Motegi Hiroaki , Yamaguchi Shigeru , Manabe Atsushi 
Published: December 2020

Abstract

BACKGROUND
To reduce treatment-related adverse events in pediatric and young-adult patients with brain tumors, proton beam radiotherapy (PBT) has recently been performed instead of conventional X-ray radiotherapy. However, whether PBT is as effective as X-ray radiotherapy has not been sufficiently investigated, especially in patients receiving whole-ventricular irradiation.

METHODS
We report a retrospective observation of 15 patients with intracranial germ cell tumors (GCT), who received PBT at our institution from April 2014 to September 2019. We evaluated their clinical course, short-term adverse events, and prognosis. RESULTS/

CONCLUSION
Fifteen patients (9 males and 6 females; median age 13 years) who received PBT following induction chemotherapy were analyzed. Nine patients received 23.4–27.0 GyE of whole-ventricular irradiation due to GCT in the pituitary gland, pineal body, or hypothalamic area. Three patients received 23.4 GyE of whole-brain irradiation: one of them had boost irradiation for basal ganglia. Three patients received 30.6 GyE of craniospinal irradiation (CSI). Six of the 15 patients experienced nausea (grade 2, according to the CTCAE version 4.0). Four patients, including two who received CSI, showed myelosuppression: decrease in white blood cell count, lymphocyte cell count, and neutrophil count (grade 3). No other severe short-term adverse events of &gt;grade 2 was observed in any of the patients. At a median follow-up of 21 months (2-62 months) after irradiation. all patients are alive without recurrence. Our results may be encouraging and further investigations with a larger scale is warranted.
GCT-10. CAN HIGH LEVEL SERUM hCG-β BE CONSIDERED EQUIVALENT TO A DIAGNOSIS OF CHORIOCARCINOMA IN PRIMARY CENTRAL NERVOUS SYSTEM GERM-CELL TUMOR?
Authors: Motegi Hiroaki , Yamaguchi Shigeru , Ishi Yukitomo , Okamoto Michinari , Iguchi Akihiro , Cho Yuko , Sugiyama Minako , Manabe Atsushi , Houkin Kiyohiro 
Published: December 2020

Abstract

BACKGROUND
Primary central nervous system(CNS) choriocarcinoma(CC) is very rare and has the poorest prognosis among germ cell tumor (GCT). CC usually has extremely high level (HL) of serum beta-human chorionic gonadotropin (bhCG) over than 1,000 mIU/ml. Some studies assign HL bhCG cases to poor prognosis group even without biopsy. The purpose of this study was to find out if there was a good prognosis subset in the HL bh group.

MATERIALS AND METHODS
We analyzed 103 cases diagnosed with GCT from 1998 to 2019 in Hokkaido University Hospital and reviewed the literature of CNS CC and bhCG. Survival was assessed using Kaplan-Meier method and log-rank statistics between the group with CC component and that with no CC component but HL bhCG.

RESULTS
One out of 103 our cases was diagnosed as a mixed GCT with CC component and did not respond to treatment and died 9 months later. Two cases were treated as CC because of HL bhCG (1,226 and 2,739 mIU/ml) despite that the biopsy showed only germinomas and survived(105 and 37 months), that is, no CC component. Combining our cases with 69 cases in the literature, all 7 cases with no CC component but HL bhCG survived but the median survival of the other 65 cases with CC component was 38.2 months (P=0.02).

CONCLUSION
This study has a limitation of selection bias, however, it suggests that patients with no CC component but HL bhCG may have a better prognosis.
GCT-13. THE TREATMENT OUTCOMES OF INTRACRANIAL GERM CELL TUMORS WITH KSPNO PROTOCOL: SINGLE CENTER RETROSPECTIVE ANALYSIS
Authors: Go Kyeong-O , Ji Soyoung , Hwang Kihwan , Han Jung Ho , Choi Hyoung Soo , Kim You Jung , Choi Byung Se , Kim In Ah , Choe Gheeyoung , Jung Jin-Myung , Hong Yong-Kil , Kim Chae-Yong 
Published: December 2020

Abstract
Dho et al. (BTRT, 2017) reported that 1.1% (127/11,827) of primary brain tumors are intracranial germ cell tumors (iGCT) in Korea. We analyzed the epidemiology and treatment results of germ cell tumors in our institution. From 2004 to 2019, among 6494 patients with intracranial neoplasms the 61 (0.9%) patients with iGCTs were enrolled: histologically diagnosed in 50 patients and clinically in 11 respectively. Pediatric patients underwent treatment according to the KSPNO protocol, and adult patients were treated with bleomycin, etoposide, and cisplatin regimens. The median age was 20 years (range: 1–42) and the follow-up period was 7.7 months (range: 10.0–203.4 months), respectively. The tumors arise most frequently in the pineal area (n=30, 49.2%). There were no significant differences in outcomes between protocols, but in KSPNO protocol group showed lower tumor recurrence rate (11.5% vs. 20%, p=0.494) and mortality (0% vs. 5.2%, p=0.503). According to the pathological subtype, the outcomes showed statistically significant differences between germinoma and non-germinomatous germ cell tumor (NGGCT) groups. The 10-year progression-free survival was 93.2% and 67.1% in the germinoma and the NGGCT group, respectively (p=0.009). The NGGCT pathological type (p=0.021) was a significant recurrence associated factor in multivariate analysis. Significant adverse events (CTCAE version 5.0 grade≥3) were showed in 14 patients (7 patients in both KSPNO and other treatment protocol groups). Pure germinoma has a higher survival rate and a lower recurrence rate than NGGCT. And KSPNO protocol might be safe and effective. For appropriate treatment for iGTCs, a multidisciplinary approach might be needed.
GCT-14. SECOND-LOOK SURGERY FOR INTRACRANIAL GERM CELL TUMORS
Authors: Ogiwara Hideki 
Published: December 2020

Abstract

OBJECTIVE
The authors present their experiences of second-look surgery in patients with intracranial GCTs who showed less than complete response despite normalizing or decreasing tumor markers after chemotherapy.

METHODS
Retrospective review of 14 patients who underwent second-look surgery for an intracranial GCT was performed.

RESULTS
Of 40 consecutive patients with newly diagnosed intracranial GCTs treated between August 2003 and 2019, 14 patients (35%) underwent second-look surgery. The mean age was 9.2 years. The initial diagnoses were mixed germ cell tumor in 6, immature teratoma in 4, yolk sac tumor in 2, and germinoma 2. Second-look surgery was performed after 1–3 courses of chemotherapy. Magnetic resonance imaging (MRI) at the surgery demonstrated increasing residual tumor in 8 and stable residual tumor in 6. Tumor markers were normalized in 10 and nearly-normalized in 4. Gross total resection was achieved in 12 patients and near-total resection in 2. Histopatholgy at second-look surgery revealed mature teratoma in 6, immature teratoma in 3, fibrosis with atypical cells in 2, and fibrosis in 3. Eleven patients subsequently underwent additional chemo-radiation therapy according to the initial diagnosis. All patients are alive with no evidence of recurrence with a mean follow-up of 69 months.

CONCLUSIONS
Second-look surgery plays an important role in the treatment of intracranial GCTs. Surgery may be encouraged at a relatively early phase after chemotherapy when the residual tumor increases or does not change the size despite normalized or nearly-normalized tumor markers in order to achieve complete resection and improve the outcome.
GCT-16. LONG-TERM CLINICAL OUTCOMES OF GERM CELL TUMORS
Authors: Matsuda Kenichiro , Sakurada Kaori , Kayama Takamasa , Sonoda Yukihiko 
Published: December 2020

Abstract

BACKGROUND
Intracranial germ cell tumors (GCT) are mainly arising in adolescent term and treated with chemotherapy concomitant with radiation therapy. There is accumulating evidence that the progress of treatment. Besides, long-term outcome and adverse effects are major problem in treatment. So, we must grasp the influence of these outcomes on daily and social life. Then we investigated in clinical outcome in cases of GCT treated in our institution. METHOD: We reviewed the clinical features and outcomes of 52 cases of intracranial GCT in 1975 to 2019. Ages on diagnosis are 5–35 years old (median 14 years old), consisted with 44 male cases. The pathological distributions are these: pure germinoma: 40 cases, non-germinomatous germ cell tumor (NGGCT): 10 cases (mature teratoma: 4, mixed germ cell tumors: 3, and one cases of choriocarcinoma, embryonal carcinoma, yolk sac tumor), unidentified pathology: 2 cases. Almost all cases have biopsied and treated by chemotherapy and radiation therapy.

RESULTS
Chemotherapy with ICE regimen (ifosphamide, cisplatin, etoposide) or CARE regimen (carboplatin, etoposide) concomitant with radiation therapy (mainly, extended local irradiation) have done in almost cases by the era. Clinical outcomes are relatively well in our cases, but 10 cases experienced recurrence. 3 cases have dead. Some cases with suprasellar involvement have need hormone replacement in long term. There are 10 cases at work.

CONCLUSION
Almost cases have gained better outcome and ADL. But there is slightly lower rate in work or marriage. Serial evaluation in outcome, and higher brain functions should be performed in follow up.
GCT-17. WHAT IS THE CLINICAL OUTCOME OF PROTON BEAM THERAPY FOR PATIENTS WITH INTRACRANIAL GERM CELL TUMOR IN KOREA?
Authors: Youn Sang Hee , Kim Joo-Young 
Published: December 2020

Abstract

PURPOSE
To evaluate the clinical outcome of patients with intracranial germ cell tumor treated with proton beam therapy (PBT).

MATERIALS AND METHODS
Fifty-seven patients with intracranial germ cell tumor treated with PBT between 2009 and 2016 were retrospectively analyzed.

RESULTS
Median follow-up duration was 63.7 months (range, 5.6–204.5). Thirty-seven patients (64.9%) were pure germinoma and 20 patients (35.1%) were non-germinomatous germ cell tumor (NGGCT). All patients except 2 patients received chemotherapy before PBT. Twenty-one patients (36.8%) of localized germinoma were treated with whole ventricle irradiation (WVI), while 36 (63.2%) patients who were diagnosed as disseminated germinoma or NGGCT received cranio-spinal irradiation (CSI). Two patients with pure germinoma in basal ganglia showed disease relapse at 3.0 and 6.9 years after PBT at the primary site and pituitary gland, respectively. There was one patient with NGGCT who died of chemotherapy-related mortality at 4.7 years after PBT while her disease was complete remission. The 7-year progression-free survival and overall survival were 70.8% and 100% for focal germinoma, 100% and 100% for disseminated germinoma, 100% and 100% for focal NGGCTs, and 100% and 80.0% for disseminated NGGCTs, respectively.

CONCLUSIONS
PBT of pure germinoma resulted in comparable clinical outcomes to that with photon radiotherapy. Our result for NGGCT is also excellent compared to other reports. Failure patterns of germ cell tumors originating in basal ganglia needs to be assessed in large pooled data.
GCT-20. EVALUATION OF NEURORADIOLOGICAL RESPONSE TO INDUCTION CHEMOTHERAPY FOR PATIENTS WITH LOCALISED GERMINOMA IN THE SIOP CNS GCT II TRIAL
Authors: Bison Brigitte , Morana Giovanni , Mitra Dipayan , Brisse Herve , Faure-Conter Cecile , Ajithkumar Thankamma , Alapetite Claire , Timmermann Beate , Nicholson James , Calaminus Gabriele , Murray Matthew 
Published: December 2020

Abstract

INTRODUCTION
The SIOP-CNS-GCT-96 trial demonstrated excellent survival for patients with germinoma. Localised patients received either craniospinal irradiation (CSI) 24Gy plus tumour-bed-boost 16 Gy or 2xcarboPEI chemotherapy (carboplatin/etoposide alternating with etoposide/ifosfamide) and focal-radiotherapy 40 Gy. Following trial closure, whole-ventricular-irradiation (WVI) was delivered with focal-radiotherapy to avoid ventricular relapse. Accordingly, current research priorities focus on reducing treatment burden and long-term neurocognitive sequelae.

METHODS
SIOP-CNS-GCT-II employed national central radiological review to assess whether dropping the 16Gy boost was safe for localized germinoma in complete-remission (CR) following 2xcarboPEI: i.e. no disease on clinical/marker/radiological assessment. Any abnormal thickening/enhancement after chemotherapy was to be classified as partial-remission (PR). Patients with less than CR after chemotherapy received a boost.

RESULTS
Shortly before trial closure (2018), it was noted that national CR rates were discrepant across the largest recruiting countries. For German patients, CR rates were ~80%, compared with ~30–40% for UK and France. A formal neuroradiology review was therefore convened. A total of 59 cases were randomly selected (UK, n=32; France, n=14 and Germany, n=13), including those deemed to be in CR and PR. Cases included those with disease at pituitary, pineal and bifocal sites. Both diagnostic scan and scan after induction chemotherapy were used for assessment. Detailed analysis is ongoing and will be presented.

CONCLUSION
Residual changes at both pituitary and pineal sites of uncertain significance may remain after chemotherapy. This process should facilitate consensus to define the best response criteria allowing treatment reduction for CNS germinoma for future clinical trials.