bluuebunny/crossref_metadata_2025_split huggingface dataset:
LINC-08. INCREASED TREATMENT TOXICITIES AND INFERIOR OUTCOMES IN UNDERNOURISHED CHILDREN WITH BRAIN TUMOURS
Authors: Prasad Maya , Chheda Ekta , Chinnaswamy Girish , Gupta Tejpal , Krishnatry Rahul , Vora Tushar , Godashastri Jayant 
Published: December 2020

Abstract

BACKGROUND
Children on treatment for brain tumours are known to be at high risk of undernutrition, the impact on outcome and toxicity is not well understood.

METHODS
Retrospective audit of children(&lt;18 years)diagnosed January 2017-December 2018 with embryonal brain tumours (medulloblastoma, primitive neuro-ectodermal tumors, pinealoblastoma, atypical teratoid/rhabdoid tumour) and treated at our centre. Data was retrieved from case records and electronic medical records. Nutritional status(NS) was defined as per World Health Organization (WHO) into severe malnutrition (SAM),moderate malnutrition (MAM),well nourished (WN) and overweight. Undernutrition(UN) was defined as SAM/MAM.Toxicity was documented till end of treatment,defined as treatment delay&gt;1week,significant infection or toxic death.

RESULTS
Of 124 eligible patients who received entire chemotherapy at our centre, NS data was available in 73 at diagnosis and 58 at follow-up. At diagnosis-29,16,26 and 2 and at follow-up-20,16,22 and 0 were SAM,MAM,WN and overweight. During treatment, weight gain was documented in 26%, stable weight in 55% and weight loss in 19%. Those UN at diagnosis had worse outcomes at follow-up with 70% alive in remission compared to 88% of WN(p-0.14). There was increased toxicity in UN group(50%) compared to WN(24%),p-0.04.All 3 toxic deaths were in UN. Those who lost weight during treatment had higher toxicities(70%) compared to those with stable weight (30%)or weight gain(20%),p-0.02.

CONCLUSIONS
In spite of nutritional intervention, children on treatment for brain tumours tend to lose weight. Increased treatment toxicities and inferior outcomes in undernourished children with brain tumours necessitates proactive and aggressive nutritional monitoring and intervention.
LINC-11. NEUROPATHOLOGY REVIEW OF LATIN AMERICAN CHILDHOOD AND ADOLESCENT BRAIN TUMOR PATIENTS: A MULTI-NATIONAL, MULTI-DISCIPLINARY PEDIATRIC NEURO-ONCOLOGY TELECONFERENCE EXPERIENCE
Authors: Osorio Diana S , Pierson Christopher R , Lassaletta Alvaro , la Madrid Andres Morales , Qaddoumi Ibrahim , Bartels Ute , Boue’ Daniel R , Finlay Jonathan L 
Published: December 2020

Abstract

BACKGROUND
Pediatric brain tumor classification has undergone significant evolution over the last decade requiring a high-level of expertise and diagnostic techniques. Such advances have created challenges for pathologists particularly in low-to-middle income countries (LMIC). We conduct weekly pediatric neuro-oncology teleconferences linking global pediatric neuro-oncologists from high-income countries (HIC) to review patients with pediatric subspecialists from Latin America.

METHODS
Three to five patients are discussed weekly and second neuropathology review is offered when a high-level of suspicion emerges of a questionable diagnosis based on clinical and radiographical information. Nationwide Children’s Hospital (NCH) provides second neuropathology review at no cost to institutions in Latin America that fulfill these criteria.

RESULTS
From July 2015 to December 2019 NCH reviewed 54 pathology samples from eleven Latin American countries. Of these, 33 (61.1%) cases resulted in diagnostic changes, of which 28 (51.8%) were significant, impacting treatment plans and overall patient outcomes. The remaining 21 (38.9%) confirmed institutional diagnosis; however, in eight of these 21 cases additional molecular information and/or further tumor subtyping unavailable in their home country at the time (eg: BRAF, RELA-fusion, medulloblastoma subtyping) was provided.

CONCLUSIONS
This study highlights the importance of centralized pathology review by institutions with the proper equipment, infrastructure and expertise in pediatric neuropathology. Furthermore, this documents the beneficial impact of teleconferencing for subspecialists in LMIC who must treat a wide variety of pediatric cancers with few resources and support. Additionally, our findings underscore the need for pediatric subspecialty training in LMIC.
LINC-14. TREATMENT OF PEDIATRIC CNS TUMORS IN ARMENIA. 10 YEARS OF EXPERIENCE IN A 29 YEARS OLD RESOURCE-LIMITED SETTING
Authors: Harutyunyan Martin , Sargsyan Lilit , Iskanyan Samvel , Hakobyan Lusine , Papyan Ruzanna , Tamamyan Gevorg , Bedirian Karen , Danielyan Samvel , Tananyan Armen , Avagyan Manushak , Karapetyan Nune , Bardakhchyan Samvel 
Published: December 2020

Abstract

BACKGROUND
Pediatric CNS tumors are the most common solid childhood malignancies with many challenges facing optimal outcome due to multimodality complex therapies, abandonment, and long-term morbidity. In our three-decades young, country the field of neuro-oncology is in its infancy. MATERIALS: The aim of our study is to assess incidence, epidemiology and treatment outcomes of children diagnosed and treated with CNS tumors within the last 10 years (2009–2019) in the Chemotherapy Clinic of “Muratsan” Hospital Complex of Yerevan State Medical University.

RESULTS
During these periods 20 patients with CNS tumors were treated in our clinic. 13 patients (65%) were diagnosed with medulloblastoma (2 patients were infants), two patients (10%) with optic pathway glioma, and 5 patients each with pilocytic astrocytoma, ATRT, ETANTR, DIPG, and glioblastoma. Five patients (3 patients with medulloblastoma, 1 patient with pilocytic astrocytoma, 1 patient with ATRT) had metastatic disease at the time of diagnosis. Seventeen patients (80%) had undergone surgery, 8 patients with medulloblastoma received chemo-RT with vincristine. Median follow up time was 15.5 months (range 5–94). Twelve patients (60%) are alive without evidence of disease. 5 patients had disease progression and three patients relapsed. From them, 3 patients died. Long-term survivors are mainly standard risk medulloblastoma patients. All medulloblastoma patients were treated according to HIT-MED guidelines.

CONCLUSION
Here we report about the pediatric brain tumors of one of the main pediatric oncology units in Armenia for a period of 10 years. The numbers are quite small for firm conclusions, but it shows the emerging need for further research.
LINC-17. SIROLIMUS AS AN ALTERNATIVE TO SURGICAL RESECTION OF PEDIATRIC TUBEROUS SCLEROSIS COMPLEX-ASSOCIATED BILATERAL SUBEPENDYMAL GIANT CELL ASTROCYTOMAS: AN AFFORDABLE OPTION FOR PATIENTS FROM LOW-MIDDLE INCOME COUNTRIES
Authors: Orduña Patricia 
Published: December 2020

Abstract
Subependymal giant cell astrocytomas (SEGA) may lead to significant neurological morbidity in children diagnosed with tuberous sclerosis complex (TSC). Surgical resection is warranted for SEGAs demonstrating continuous growth, causing hydrocephalus and increased intracranial pressure. mTOR inhibitors (sirolimus and everolimus) are alternatives to surgery and have shown efficacy in stabilizing and shrinking SEGAs. Everolimus showed stronger evidence in efficacy, but its cost poses a limitation for this treatment among patients from low-middle income countries. We explored sirolimus as a potentially more cost-effective alternative in our setting. We present a 10-year-old Filipino child with TSC admitted due to headache, vomiting, and increased sleeping time. Neuroimaging revealed large bilateral SEGAs involving the frontal horns and foramina of Monro, causing moderate obstructive hydrocephalus. Surgical excision was offered, but parents opted for medical treatment. Bilateral posterior parietal ventriculoperitoneal shunts were inserted to decrease intracranial pressure. Due to the cost of everolimus, the patient was started on sirolimus at 1mg/m2/day. Imaging done 6 months after initiating therapy demonstrated significant decrease in size of both SEGAs (right: 82.5%, left: 64.1%). Sirolimus levels were maintained at 15.7ng/ml and minimal elevations on cholesterol and triglyceride levels were observed and treated with simvastatin. Results of this case and review of related data suggest that sirolimus can be used as a conservative approach in inducing regression of large bilateral SEGAs, and an affordable alternative to everolimus for pediatric TSC patients from low-middle income countries. Prospective studies and clinical trials are needed to further establish its efficacy, safety and cost-effectiveness in our setting.
LINC-24. CHARACTERISTICS OF PEDIATRIC BRAIN TUMORS AT DEPARTMENT OF CHILD HEALTH FACULTY OF MEDICINE UNIVERSITAS INDONESIA-DR. CIPTO MANGUNKUSUMO TERTIARY GENERAL HOSPITAL, JAKARTA, INDONESIA
Authors: Widodo Dwi Putro , Mangunatmadja Irawan , Siregar Marsintauli , Pusponegoro Hardiono , Handryastuti Setyo , Soebadi Amanda , Rafli Achmad 
Published: December 2020

Abstract
Brain tumors are still the second leading cause of death among cancers in children. Based on data from National Brain Tumor Society (2019), in United States, there are 28.000 children living with brain tumor with varied clinical, radiological, and histopathological features. The most prevalent children’s brain tumor types in US are gliomas (ependymal tumors, pilocytic astrocytomas) and embroyonal tumors, including medulloblastoma. From 1993–1994 at Department of Child Health Faculty of Medicine Universitas Indonesia-Dr. Cipto Mangunkusumo Tertiary General Hospital, Jakarta, Indonesia, there are 19 patients with brain tumors hospitalized with most of patients with astrocytoma 8 patients (42%), 4 patients (21%) medulloblastoma, 2 patients (11%) neuroblastoma, 2 patients (11%) ependymoma, 2 patients (11%) craniopharyngioma, and one patient (4%) meningioma. Retrospective cohort study (2010–2015) with subjects 100 children revealed that based on the radiographs, the brain tumors were located mostly in the cerebellum (24%) and the suprasellar region (10%); based on the histopathology, the most common types of brain tumor were astrocytomas (18%), medulloblastoma (21%), and gliomas (17%). The most common symptom of brain tumors was headache and impaired vision. Survival patients with brain tumors for 5 years in the age group aged 3 years and above was better than that in children aged under 3 years (60% vs 55% and 17% vs 14%). This report can serve as one of basic data for profile children’s brain tumors in Indonesia. Keywords: brain; tumor; children; survival; Indonesia
LINC-25. BRAF ABERRATIONS IN PEDIATRIC PILOCYTIC ASTROCYTOMAS (PCAs): PREVALENCE AND IMPACT ON CLINICAL OUTCOME
Authors: Ramanathan Subramaniam , Prasad Maya , Vora Tushar , Gurav Mamta , Sahay Ayushi , Sahu Arpita , Moiyadi Aliasgar , Shetty Prakash , Goda Jayant Sastri , Krishnatry Rahul , Gupta Tejpal , Epari Sridhar , Chinnaswamy Girish 
Published: December 2020

Abstract

BACKGROUND
Increasing knowledge on pilocytic astrocytoma (PCA) biology now points towards an aberration in BRAF/MAPK/ERK pathway which has both diagnostic and therapeutic implications. This study was done to note the impact of BRAF aberrations on clinical outcome in childhood PCA.

METHODS
FFPE tissues of all childhood PCA diagnosed during 2011–2017 were evaluated for BRAFV600E mutation by Sanger sequencing and KIAA1549 fusion transcripts (16–9;15–9;16-11) by reverse transcriptase polymerase chain reaction. Children undergoing gross tumor resection received no adjuvant treatment. Unresectable tumors (only biopsy) and NF-1 associated PCAs, were treated if clinically indicated. Only patients with documented therapy details/followup were included for analysis. STUDY

RESULTS
Ninety-eight patients (median age-7.7yrs; boy:girl ratio-1.4) were included. Major sites were: Cerebellum-37(38%), 3rd Ventricle-26(27%), Cerebrum-15(15%). While BRAFV600E mutation was noted in 7/89(8%) specimens, BRAF-fusions were found in 34/85(40%). Following surgery/biopsy, 23(24%) and 21(22%) received adjuvant chemotherapy and radiotherapy respectively. The 1-year/3-year/5-year-EFS of the overall cohort was 90.7%/81.3%/67.4% respectively. Cerebellar tumors did better vis-à-vis other sites(5yr-EFS:74.3% v/s 66.4%;p=0.403). The 5yr-EFS of BRAF-fusion positive tumors (34), tumors without any BRAF aberration (40) and BRAFV600E mutant tumors (7) was 84.8%/ 69.6%/ 42.9% (p=0.215).

CONCLUSIONS
BRAF-fusion and BRAFV600E mutation were associated with good and poor outcomes respectively. Lack of statistical significance could be attributed to use of radiation as planned therapy in patients from earlier years. Data on BRAF aberrations in PCAs aids decision making regarding adjuvant therapy and choosing appropriate salvage-therapy especially in relapsed/refractory PCAs.
LINC-29. IMPACT OF RELA FUSION ON OUTCOMES OF CHILDHOOD SUPRATENTORIAL EPENDYMOMAS (ST-EPEN)
Authors: Chinnaswamy Girish , Ramanathan Subramaniam , Prasad Maya , Vora Tushar , Sahay Ayushi , Gurav Mamta , Sahu Arpita , Moiyadi Aliasgar , Shetty Prakash , Goda Jayant Sastri , Krishnatry Rahul , Gupta Tejpal , Epari Sridhar 
Published: December 2020

Abstract

BACKGROUND
Ependymomas are heterogenous group of tumours with variable clinical course and diverse molecular features. RELA fusion status has been reported to have prognostic impact in ST-EPEN. Our retrospective study analysed the prevalence and clinical impact of RELA fusion in childhood ST-EPEN at our centre. STUDY

METHODS
FFPE tissues of all childhood ST-EPEN diagnosed during 2011–2017 were evaluated for RELA fusion 1/2 by RT-PCR. Children were treated as per guidelines by the Neuro-oncology multidisciplinary team. Outcomes were correlated with RELA fusion, histological features and immunohistochemical parameters(L1CAM expression and Mib-1 index). Only patients with therapy details were included.

RESULTS
A total of 37 patients(0–50 years) with ST-EPEN were included(median age-10.2 years; boy:girl ratio-1.4:1)for analysis. Histological grade II, II/III and III was seen in 4(11%),2(5%) and 31(84%) patients respectively. Mib-1 index was assessable in 33 patients of which, 9 patients (24%) had a Mib-1 index &gt;20%. RELA fusion was detected in 13(35%)tumors. The 3-year and 5-year EFS/OS of the overall cohort was 64.2%/83.6% and 60.1%/73.1% respectively. The 3-year/5-year EFS of RELA-positive tumors was inferior compared to RELA-negative tumours (53.8%/36% v/s 62.6%/53.6%; p=0.391). The 3-year/5-year EFS of tumors expressing L1CAM versus negative-expression was comparable (61.1%/55%v/s59.8%/47.9%;p=0.44). Presence of Mib-1&gt;20% correlated with inferior survival (5-year EFS:81.1%vs22.2%; p&lt;0.01).

CONCLUSIONS
ST-EPEN with RELA fusion had trend towards increased relapse/progression. High Mib-1 correlated with poor survival. RELA fusion status needs to be studied in a larger cohort prospectively to confirm its clinical impact.
LINC-32. REPORT OF AN INITIAL SITE VISIT TO DETERMINE FEASIBILITY AND IMPLEMENTATION OF A COMPREHENSIVE NEURO-ONCOLOGY PROGRAM IN KENYA
Authors: Armstrong Beth , Mbiro Mark , Magoha Michael , Okemwa Minda , Mwang’ombe Nimrod , Coven Scott 
Published: December 2020

Abstract

BACKGROUND
Pediatric central nervous system (CNS) tumors are the leading solid tumors in the United States, but vastly under-reported in the African population. There’s limited data on childhood brain tumors as well as the histopathological distribution in Kenya. This report surveys as an initial site visit to determine the feasibility of a comprehensive neuro-oncology program at Kenyatta National Hospital (KNH) in Nairobi, Kenya. DESIGN: This collaboration began with a visit from the director of neuropathology at KNH to our neuro-Oncology program at Riley Hospital for Children at Indiana University Health in May 2019. This report includes recommendations from the May 2019 trip, as well as a reciprocal site visit to Kenya in January 2020.

RESULTS
Building off the May 2019 trip, a brain tumor registry has been initiated and maintained. Additionally, the KNH program has many necessary components to forming a comprehensive neuro-oncology program, including capable neurosurgeons with a neurosurgical training program, radiology, intensive care unit, oncology ward, rehab, skilled nursing, and radiation oncology services. Currently, neurosurgery, radiology, and pathology meet weekly to review challenging cases.

CONCLUSION
Kenyatta National Hospital has the expertise to build a comprehensive neuro-oncology program. The program currently lacks a dedicated nurse coordinator and “specialist” in neuro-oncology. Ongoing discussions with local stakeholders are aimed to galvanize national support to improve awareness for children with brain tumors and to plan a multidisciplinary neuro-oncology symposium in 2021. In the meantime, telemedicine efforts can support nursing education and reiterate the multidisciplinary needs for children with brain tumors.
LINC-35. THE ST. JUDE GLOBAL ACADEMY NEURO-ONCOLOGY TRAINING SEMINAR: A MULTIDISCIPLINARY, INTERNATIONAL EDUCATION PROGRAM
Authors: Moreira Daniel , Patay Zoltan , Boop Frederick , Chiang Jason , Merchant Thomas , Santiago Teresa , Gajjar Amar , Rodriguez-Galindo Carlos , Qaddoumi Ibrahim 
Published: December 2020

Abstract
The success of the treatment of children with central nervous system (CNS) tumors relies on an effective multidisciplinary team, with up-to-date and broad knowledge and skills. The St. Jude Global Academy Neuro-Oncology Training Seminar was launched as course in globally applicable content in pediatric neuro-oncology with a focus on multidisciplinary teams in low- and middle-income countries (LMICs). To identify the content that is most relevant for the learners, a needs assessment survey that included evaluation of team dynamics, treatment capacity, existing knowledge, and educational goals was designed. Survey questions in 11 domains were answered by 24 sites in LMICs across the world. This information was used to create the course that consists of two components: a 9-week online course and a 10-day workshop at the St. Jude campus. 72 participants from 11 institutions enrolled in the online portion and 20 participants were selected based on grades to attend the workshop. A retrospective post-test evaluation established that learners improved their understanding of the barriers to care, possible solutions to improve care, understanding of diagnosis and treatment, and methodology to implement projects (p&lt;0.01). All participating teams developed projects that are locally implemented. Those present at the workshop formed a multidisciplinary, international collaborative group (Global Alliance in Pediatric Neuro-Oncology). This experience establishes that educational programs with systematically created curricula can not only improved knowledge but be a mechanism to share experiences and create collaborative networks. Ultimately, patient outcomes will be tracked to monitor the true impact of the course.
LINC-39. PERFORMANCE STATUS OF PEDIATRIC PATIENTS WITH CENTRAL NERVOUS SYSTEM TUMORS TREATED IN MEXICO, A SINGLE-CENTER EXPERIENCE
Authors: Madrigal-Avila Claudia , Perez-Bañuelos Alfonso , Ruvalcaba-Sanchez Rafael , Vega-Vega Lourdes , Escamilla-Asiain Gabriela 
Published: December 2020

Abstract

BACKGROUND
Central nervous system (CNS) tumors are the most common solid neoplasms in the pediatric age, they comprise about a quarter of all cancers at this age. Little is known about the specific epidemiology of this group in Mexico and there are no reports of results focused on the Performance Status of patients who are treated in a multidisciplinary setting.

OBJECTIVE
To describe the Performance Status of CNS pediatric patients after being treated with a multidisciplinary approach in a tertiary center.

METHODS
We report a retrospective chart review of all pediatric patients who presented to the Neuro-Oncology Clinic at Teleton Pediatric Oncology Hospital in Queretaro, Mexico, from December 2014 to January 2020. We analyzed age, gender, the extent of surgical resection and histopathology. Performance Status was assessed using ECOG and Karnofsky/Lansky scores during every patient’s last follow-up visit.

RESULTS
A total of 56 patients were treated, epidemiology and histopathology variants are similar to those described in the international literature. With a median follow-up of 33 months, 35 patients are alive (62.5%), 28 of them (74.2%) have an excellent Performance Status (ECOG score 0 or Lansky/Karnofsky ≥ 90), 5 (14.2%) scored ECOG 1–2 and only 4 (11.4%) scored ECOG 3–4.

CONCLUSIONS
A multidisciplinary approach with a focus on Performance Status and the potential for neurological recovery is essential in the management of pediatric patients with CNS tumors. Efforts should be aimed at reducing post-surgical morbidity and early rehabilitation to reintegrate patients into society in the long term.
LINC-41. TREATMENT OF RECURRENT MEDULLOBLASTOMA IN CHILDREN IN LOW INCOME SETTINGS
Authors: Kizyma Roman , Zapotochna Khrystyna , Romanyshyn Bogdan , Kizyma Zoryana , Sobko Roman 
Published: December 2020

Abstract

INTRODUCTION
Children with recurrent medulloblastoma after initial therapy have very poor prognosis due to limited second line treatment options and significant treatment-related morbidity.

METHODS
A retrospective chart review of 18 children with recurrent or progressive medulloblastoma, treated initially with risk-adapted therapy in Western Ukrainian Specialized Pediatric Medical Centre from 2012 to 2019, was performed.

RESULTS
All patients received first line multimodal treatment: surgery, distant beam radiotherapy and chemotherapy. Recurrent disease in 11 patients presented with metastatic dissemination and in 7 patients as local relapse. The median time to recurrence was 10 months. The median follow-up after diagnosis of recurrent disease diagnosed was 2 years and 2 months. Second line therapy included re-surgery (5 cases), radiation therapy (10 cases) and various cytostatic agents as monotherapy or combination - carboplatin, cisplatin, cyclophosphamide, etoposide, methotrexate, temozolomide, lomustine. Patients treated with radiotherapy for salvage had prolonged local control compared to those that received chemotherapy only. On follow-up 8 children are currently alive.

CONCLUSION
Recurrent and progressive medulloblastoma had a poor prognosis with a 2-year overall survival (OS) of 28% on different salvage therapy. The variety in the treatment of all patients experiencing recurrence was observed due to low income country settings. The factors that influenced higher survival after recurrence of medulloblastoma were longer time to relapse, and local pattern of relapse/progression.