bluuebunny/crossref_metadata_2025_split huggingface dataset:
P16.02.A FRENCH NATIONAL STUDY ON THE EPIDEMIOLOGY OF CHILDHOOD BRAIN TUMORS
Authors: Khettab M , Chirpaz E , Zouaoui S , Ah-pine F , Rigau V , Rolland A , Darlix A , Ng S , Roujeau T , Bauchet L 
Published: October 2024

Abstract

BACKGROUND
Primary central nervous system tumors (PCNST) in children aged 0 to 19 years are a heterogeneous group of pathological entities that continue to present significant challenges. This abstract provides exhaustive data on histologically confirmed pediatric PCNST cases in France.

MATERIAL AND METHODS
Patients were identified from the French Brain Tumor Database, a comprehensive national database. The study included children (0 to 19 years) who were diagnosed with histologically confirmed PCNST between 2006 and 2015. For each histological subtype, including rare variants, data such as the number of cases, incidence rates, gender distribution, and surgical approach (resection or biopsy).

RESULTS
Between 2006 and 2015, a total of 6,691 cases of newly diagnosed and histologically confirmed primary central nervous system tumors (PCNST) were recorded in France, with a gender distribution of 3,692 males and 2,999 females. The principal histological categories included 4,863 neuroepithelial tumors (72.68%) including 2,829 gliomas (42.29%), 819 neuronal and mixed neuronal-glial tumors (12.24%), and 1,005 embryonal tumors (15.02%). Additionally, there were 746 tumors of the meninges (11.15%), which included 140 meningiomas (2.09%), 551 mesenchymal tumors (8.23%), and 55 other types (0.82%). Other categories reported were 251 tumors of cranial and paraspinal nerves (3.75%), 223 germ cell tumors (3.33%), 284 craniopharyngiomas (4.24%), and 324 other types (4.84%).The overall crude incidence rate (CR) was 4.334 per 100,000 person-years, with age-specific rates of 4.418 per 100,000 for ages 0-4 years, 4.184 per 100,000 for ages 0-14 years, and 4.789 per 100,000 for ages 15-19 years. Age-standardized incidence rates (adjusted to European, US, and world standard populations), demographic characteristics, along with other relevant data, will be further elaborated in the presentation. In addition, specific attributes for the age groups of 0-4 and 15-19 years, relating to histological subtypes and sex predominance, will be provided in detail. For example, anaplastic ependymomas constituted 101 out of 171 cases (59.06%) in the 0-4 years age group, compared to 16 out of 99 cases (16.16%) in the 15-19 years age group. Embryonal tumors represented 422 out of 1,715 cases (24.61%) in the 0-4 years age group, and 100 out of 1,835 cases (5.45%) in the 15-19 years age group. Among mesenchymal tumors, lipomas accounted for 98 cases in the 0-4 years age group and 10 cases in the 15-19 years age group.

CONCLUSION
To our knowledge, this constitutes the most extensive report of histologically confirmed PCNST cases diagnosed in children and adolescents within the French population. The report meticulously details all histological subtypes along with their respective frequencies and distributions. The findings hold significant implications for epidemiology, clinical practice, and future research.
P16.05.A PEDIATRIC NEUROFIBROMATOSIS 1-RELATED AND NOT-RELATED OPTIC PATHWAY GLIOMA TREATED WITH FIRST LINE CHEMOTHERAPY: A SINGLE-CENTER EXPERIENCE
Authors: Castelli B , Fonte C , Tellini M , Di Nicola M , Guidi M , Giunti L , Marzano C , Tirinnanzi B , La Torre C E , Buccoliero A M , Giordano F , Marchiori V , Maimone F , D’Incerti L , Bacci G M , Scagnet M , Genitori L , Sardi I 
Published: October 2024

Abstract

BACKGROUND
Optic pathway gliomas (OPG) are low-grade tumors (LGG) that affect the precortical visual pathway, representing 3-5% of pediatric brain tumors, more often diagnosed before the age of 5 years. Although histologically benign, due to their location and thus their unresectability, they carry considerable morbidity, bringing to progressive visual loss. In up to 20% of cases they are detected in patients with neurofibromatosis type 1 (NF1). Consolidated chemotherapy regimen with or without biopsy or debulking, if feasible, remains so far the standard management, moving to new personalized frontiers.

MATERIAL AND METHODS
In Meyer Children’s Hospital IRCCS Neuro-oncology Unit in Florence (Italy), from February 2013 to April 2024, for visual impairment at diagnosis or for disease progression, thirty-seven OPG patients (female/male ratio 1.3, median age at onset 4 years) completed the first line LGG chemotherapy (carboplatin-etoposide scheme adopted in our institution, built upon the INT Milan strategy and the standard SIOP LGG trial). Fourteen patients (37.8%) presented NF1-related OPG diagnosis, mostly made on a clinical/neuroradiological data. Response was assessed according to RAPNO criteria.

RESULTS
Non-NF1 OPG patients debuted at a lower median age (3 years) and mostly in a chiasmatic-hypothalamic location, predominantly with a pilocytic astrocytoma histology and manifested with nystagmus, secondarily with visual impairment. Despite the most frequent pre-chiasmatic involvement, during follow-up NF1 patients surprisingly presented a mean and median higher thickness (μm) of the retinal nerve fiber layer (RNFL) on optic coherence tomography (OCT) compared to non-NF1 patients, tested in the majority of patients (mean right RNFL 74.4 versus 58.0, median 66.0 versus 50.0, mean left RNFL 77.2 versus 55.3, median 73.0 versus 49.5). Four non-NF1 patients progressed during treatment or immediately afterwards. Considering the whole follow-up from the stop therapy (median time 5 years), non-NF1 patients progressed by far much more (15/23, 65.2% with one death versus 2/14, 14.3%). Overall survival was 97.3%.

CONCLUSION
NF1-related and not related pediatric OPG represent two distinct subgroups, with different clinical and prognostic characteristics. In agreement to other series our data show that non-NF1 OPG are less chemotherapy responsive than NF1-related OPG, surely due to the molecular landscape at the basis of tumor growth, warranting the needing of precision medicine directed to targets also in first- line treatment. A worse OCT outcome was found in the former cohort.
P16.10.A UNRAVELING THE CLINICAL IMPLICATIONS OF TRIM24::NTRK2 FUSION GENE IN PEDIATRIC HIGH-GRADE GLIOMA: A CASE STUDY
Authors: Karlsson I , Keane S , Deland L , Lindlöf A , Kling T , Olsson Bontell T , Fagman H , Nilsson J , Nilsson D , Sabel M , Carén H , Abel F 
Published: October 2024

Abstract

BACKGROUND
High-grade gliomas are among the deadliest of all childhood cancers and the leading cause of cancer-related mortality in children. The standard treatment is resection followed by chemotherapy, most often temozolomide and radiation therapy. Unfortunately, there is no evidence that temozolomide influences survival in pediatric patients and only a limited number of new drugs have been identified for specific subgroups of tumors. The identification of fusion genes as possible drivers of the disease offers an opportunity for targeted therapies. We present a case of a six-year-old patient with a novel TRIM24::NTRK2 fusion positive epithelioid glioblastoma with subsequent relapses.

METHODS
We investigated the ramification of this fusion and evolution of the tumor through whole genome sequencing, RNA-sequencing and DNA methylation profiling, and studied the effect of the patient’s treatment using patient-derived cell cultures.

RESULTS
Our studies show that the TRIM24::NTRK2 fusion has oncogenic abilities but loses its importance as the tumor evolves. In addition, we demonstrate an increased drug resistance over time, further indicating the diminished driver function of the fusion.

CONCLUSION
The use of resequencing revealed parallel development of tumor subclones with different genomic alterations, highlighting the importance of genomic profiling from various parts of the tumor.

FUNDING
This work was supported by the Swedish Research Council, the Swedish Childhood Cancer Foundation, and the Swedish Cancer Society
P17.01.A ANAMNESTIC RADIOLOGICAL METASTASES OUTCOME SURGICAL SCORE (ARMO-S). A PURPOSE OF A PREDICTIVE SCORING SYSTEM FOR SURGICAL BRAIN METASTASES
Authors: Armocida D , Ius T , Cofano F , Tartara F , Salvati M , Frati A , Garbossa D 
Published: October 2024

Abstract

BACKGROUND
Several risk stratification scores have been suggested to aid prognostication and guide treatment strategies for brain metastases (BMs). However, the current scores do not focus on the specific neurosurgical population, therefore not predicting short-term mortality and postoperative performance status.

MATERIAL AND METHODS
This retrospective observational study of 362 consecutive patients treated with surgery for BMs aims to identify the factors associated with post-surgical outcomes and propose a surgery-specific prognostic score for patients with BMs candidate for open surgery.

RESULTS
Factors significantly associated with OS and performance status in multivariate analysis were age, KPS, surgical site, synchronous debut of BM, number, tumor volume, seizure, extra-cranial metastases, and deep-seated location. The variables were incorporated into the Anamnestic Radiological Metastases Outcome Surgical score (ARMO-S). The values range between 0 and 10. Patients were divided into two groups (low-risk and high-risk) based on each significant subgroup’s median survival and performance status with an optimal cutoff value determined as 4. The two groups have significant differences in OS (9.6 versus 14 months, p = 0.0048) postoperative KPS (90 versus 70, p=0.012) and KPS at last follow-up evaluation (75 versus 30, p&lt;0.001)

CONCLUSION
ARMO-S is a simple and comprehensive score for BM patients selected for neurosurgery, as it incorporates the main factors of the most important prognostic scores, implementing them with more surgery-specific predictive elements such as tumor location and volume, presence of seizures at onset, and involvement of eloquent brain areas.
P17.04.B CIRCULATING TUMOR CELL NUMBERS IN CSF HAVE ADDED PROGNOSTIC VALUE IN PATIENTS WITH LEPTOMENINGEAL METASTASES
Authors: van Kessel E , Woerdman N , Pluim D , Milojkovic B , van den Broek D , Brandsma D 
Published: October 2024

Abstract

BACKGROUND
Approximately 10% of patients with solid tumors develop leptomeningeal metastases (LM). Median survival rates are low in patients with LM but can vary significantly and are dependent on various patient-, tumor-, and treatment-related factors. Advanced techniques for circulating tumor cell enumeration in cerebrospinal fluid (CSF) can help in diagnosing patients with LM, but they may also have value in prognostication. Our aim was to investigate whether circulating tumor cells (CTCs) in CSF are of added value in survival prediction in LM patients.

MATERIAL AND METHODS
We performed a single-center retrospective study in a cohort of patients with LM from an epithelial tumor, diagnosed at the Netherlands Cancer Institute - Antoni van Leeuwenhoek, a tertiary comprehensive cancer center. CTCs in CSF were enumerated by an Epithelial Cell Adhesion Molecule (EpCAM - based immunoflow cytometry method) at the diagnosis of LM. The added prognostic value of CTCs in CSF on top of known clinical prognostic factors for LM, was assessed. We compared Cox proportional hazards regression models with and without CTCs by means of loglikelihood ratio tests (LRT) and discriminative performance measures.

RESULTS
We included 103 eligible patients who were diagnosed with either possible (n=10), probable (n=7), or definitive LM (n=86) according to EANO-ESMO guidelines, with a median survival of 129 days. All patients with possible LM at diagnosis, were found to actually have LM during clinical and radiological follow-up. CTCs in CSF were of significant added prognostic value upon the following known predictors of LM; gender, age, WHO performance score, brain metastases, primary tumor type, targetable driver mutation, systemic progression at diagnosis of LM and switch of systemic therapy. This was indicated by a loglikelihood ratio test with a p-value of ≤ 0.01 and an Area Under the Curve (AUC) of 0.723 (with CTC) versus 0.710 (without CTC). Sensitivity analyses in subgroups with only patients with definitive or probable LM showed similar results. Additionally, the median amount of CTCs differed significantly between the following survival groups: &lt; 6 weeks: 103.9 CTCs/ml; 6 weeks to 6 months: 41.3 CTCs/ml; &gt; 6 months: 5.8 CTCs/ml, with ANOVA p-value =0.031.

CONCLUSION
Our findings indicate that the number of CTCs in CSF, as measured by EpCAM immunoflow cytometry, is of additional prognostic value to other well-known predictors of survival in LM. The number of CTCs in CSF should be taken in account when assessing the prognosis of patients with LM of solid tumors.
P17.06.B CSF DRAINAGE IN LEPTOMENINGEAL DISEASE: SURGICAL AND SURVIVAL OUTCOMES IN VENTRICULO-PERITONEAL SHUNT VS. RICKHAM RESERVOIR PATIENTS
Authors: Alhalabi O T , Klein L , Büsken C , Mellal A , Wasilewski D , Buszello C , Cossu G , Eyüpoglu I Y , Unterberg A W , Vajkoczy P , Messerer M , Misch M , Krieg S M , Juratli T A , Younsi A 
Published: October 2024

Abstract

BACKGROUND
Leptomeningeal disease (LMD) usually represents a terminal condition that can cause symptomatic hydrocephalus. With improved survival rates under systemic therapy, the role of cerebrospinal fluid (CSF) drainage via ventriculo-peritoneal shunt (VPS) or Rickham reservoir placement for symptom control is gaining more relevance. This study aimed to describe surgical and survival outcomes of hydrocephalic LMD patients after surgical drainage in a contemporary cohort.

MATERIAL AND METHODS
A multi-center retrospective analysis of adult patients with VPS or Rickham reservoir placement for CSF drainage in the presence of LMD with malresorptive hydrocephalus between 2017 and 2022 was performed. Patients with occlusive hydrocephalus were excluded. Clinical, surgical and survival data were collected and compared.

RESULTS
A total of 119 patients with a median age of 53 (42-77) years (40 males, 79 females) were included. Of those, 64 underwent VPS (54%) and 55 (46%) Rickham reservoir placement. The most common underlying oncological conditions were breast cancer (n=67, 56%) and non-small cell lung cancer (n=25, 21%). The median time between primary tumor and LM diagnosis was 23 months (0 to 180 months) and 44 patients (37%) had end-stage disease at primary tumor diagnosis. Moreover, 79 (66%) patients showed solid intraparenchymal cranial metastases, for which only 20 patients (17%) had received surgical resection. Most patients (n=95, 80%) presented with symptoms of intracranial hypertension, which were relieved in 79% (n=51/64) after surgery. Compared to VPS placement, Rickham reservoirs were associated with lower complication (11% vs. 30%, p=0.01) and revision (9% vs. 23%, p=0.049) rates. Median overall survival from surgery was 90 (23 -148) days, and longer in VPS patients (107 days) compared to Rickham patients (83 days). However, this difference did not reach statistical significance (p=0.512).

CONCLUSION
While surgical CSF drainage in patients with LM disease relieves symptoms of intracranial hypertension in many cases, median survival remains expectedly low. Compared to Rickham reservoir placement, VPS bears higher rates of complications and revisions without a clear survival benefit. Nevertheless, decision-making regarding surgical CSF drainage and more importantly VPS or Rickham reservoir placement in LMD patients retains an individual and palliative nature.
P17.07.A DIRECT COMPARISON OF LUNG-GPA AND LABBM PROGNOSTIC SCORES FOR PATIENTS WITH LUNG CANCER BRAIN METASTASES
Authors: Furman O , Keret S , Limon D , Waissengrin B , Bittan A , Blumenthal D T , Bokstein F , Wolf I , Zach L 
Published: October 2024

Abstract

BACKGROUND
Brain metastases (BM) are the most common malignant CNS tumors in adults, associated with decreased survival and severe impairment to quality of life. The majority of BM originate from lung cancer (40-50%), where availability of new drug therapies calls for personalized risk-benefit calculations. While several prognostic tools were developed to support therapeutic decision-making in the specific context of BM detection, our experience in the clinic suggests none is currently used routinely.

MATERIAL AND METHODS
We compared the predictive capabilities of two different prognostic scores on a retrospective cohort of 90 lung cancer patients treated for BM at the Tel Aviv Sourasky Medical Center. Both scores were computed for each patient. Lung-GPA score is based on age, Karnofsy performance status (KPS), number of BM, number of extra-cranial metastases and molecular features when relevant (for NSCLC patients, EGFR/ALK mutations, PD-L1 expression). LabBM score is based on levels of hemoglobin, albumin, CRP, LDH and platelets in blood tests taken at BM diagnosis.

RESULTS
Median age at diagnosis was 67.1 (range 36-88), 43 patients were female, mean number of BM was four (range 1-10), 79 patients had NSCLC, of which 15 (18.9%) had confirmed EGFR/ALK mutations, and 11 had SCLC. Median survival was 6.8 mo (range 0.5mo-103mo). Survival rates at 3mo, 6mo, and 12mo were 73%, 51%, and 40%, respectively. Both scores were significantly predictive of OS at these times, and direct comparison between them using ROC analysis revealed no differences in predictive ability for survival at 3mo (0.7 for LabBM and 0.67 for Lung-GPA); at 6mo (0.7 for LabBM and 0.74 for Lung-GPA) and at 12mo (0.61 for LabBM and 0.71 for Lung-GPA).

CONCLUSION
In a real-world cohort of patients with lung cancer BM we find that Lung-GPA score and LabBM score both show a similar prognostic ability. Future studies should examine whether predictive scores could improve by use of patient data currently not captured by either score, such as treatment response or neuroimaging-based features.
P17.13.B NANO-LM: AN UPDATED SCORECARD FOR THE CLINICAL ASSESSMENT OF PATIENTS WITH LEPTOMENINGEAL METASTASES
Authors: Le Rhun E , Nayak L , Lim-Fat M , Rudà R , Pentsova E , Forsyth P , O’Brien B J , Preusser M , Kumthekar P , Brandsma D , Weller M 
Published: October 2024

Abstract

BACKGROUND
There are no validated tools for the clinical neurological assessment of patients with leptomeningeal metastases. However, clinical examination during the course of the disease guides medical management and is part of response assessment in clinical trials. Standardization of clinical neurological assessment in LM is therefore essential, also because MRI may not always be obtained owing to rapid clinical deterioration and itself subject to rater disagreement.

MATERIAL AND METHODS
The RANO-LM group launched a 2-steps process, aiming at improving and homogenizing the clinical assessment in patients with leptomeningeal metastasis. We report here on the first step: the establishment of a consensus scorecard. The RANO-LM group had 9 virtual meetings to define general recommendations on neurological assessment and selected domains of interest that should be tested. Domains of interest were selected from the Neurological Assessment in Neuro-Oncology (NANO) scale and from the first RANO-LM proposal.

RESULTS
Fourteen domains in neurological assessment were selected: level of consciousness, cognition, nausea and vomiting, vision, eye movement, facial strength, hearing, swallowing, dysarthria, limb strength, ataxia, walking, cauda equina symptoms-bowels and cauda equina symptoms-bladder). For each item, a clear instruction on how to perform the assessment is provided with scoring criteria between 0 and 2. The general status of the patient and use of steroids, pain medications, and anti-emetics should be considered when assessing the patient. Neurological sequelae from previous brain metastases or treatment (neuropathy, post-radiotherapy toxicities) should be rated at the baseline evaluation; it should be specified in the comments when symptoms or signs may be related to a condition other than leptomeningeal metastasis. The evaluation should be declared as non-evaluable when neurological symptoms or signs that develop during the follow-up are related or suspected to be related to comorbidities or any other medical event. In these situations, specific details should be included in the comments. If these signs and symptoms are retrospectively interpreted as related to leptomeningeal metastasis and not to any comorbidity, the date of symptoms or signs appearance should be retrospectively back-dated to assess the response.

CONCLUSION
A NANO-LM consensus scorecard has been established. A prospective validation of the proposal is currently ongoing at various medical centers.
P17.14.A OPTIMISATION OF THE STEREOTACTIC RADIOSURGERY (SRS) SERVICE AT THE BEATSON, WEST OF SCOTLAND CANCER CENTRE (BWOSCC)
Authors: Walker S , Nowicki S , James A , Williamson A 
Published: October 2024

Abstract

BACKGROUND
Since 2017 HyperArc™ VMAT SRS has been offered at BWOSCC as a treatment option for patients with brain metastases. During this period, WBRT (whole brain radiotherapy) or PB(Partial Brain)RT for the treatment of brain metastases has correspondingly reduced. Given the reduction in side effects, improved local control and reduced visits with SRS over WB/PBRT, the dedicated neuro-oncology team want to ensure all patients eligible for SRS treatment are being offered it, identify barriers to referral and ways to optimise the service.

MATERIAL AND METHODS
57 patients treated with WB/PBRT for brain metastases were identified via the RT database from August 2022-September 2023. Patients with primary small cell lung cancer, post-neurosurgical resection site or leptomeningeal disease were excluded. Patient demographics; primary site; number of brain metastases; diagnostic imaging; geographical location; RT dose and fractionation and survival post-treatment were documented and analysed. Diagnostic imaging for each patient was peer-reviewed by two consultant clinical oncologists, a SABR clinical research fellow and consultant neuro-oncology radiographer to determine whether SRS treatment was technically deliverable. Where it was felt SRS treatment was an option, the patient’s electronic record was interrogated to identify reasoning as to why SRS was not offered.

RESULTS
The majority of patients had either primary breast(26) or lung(22) cancer: gynaecological(3); colorectal(2); oesophageal(2); CUP(1); urothelial(1). All patients except 2 were treated with 20gy/5# (otherwise 30Gy/10#). Most were treated with WBRT(43). 14 had single brain metastasis; 20: 2-4 metastases; 9: 5-10 metastases; 14 patients had ≥11 brain metastases. 27 had MRI imaging in addition to CT. Most patients’ geographical location was outside of Glasgow(35). In 16 of the 57 patients identified(28%), SRS was technically deliverable. Median survival was 145 days(4.8months): within the technically deliverable SRS group, median survival was also 145days(4.8months).In these 16 patients, 7 were not offered SRS due to widespread systemic disease (not necessarily a barrier to SRS). There was no identifiable rationale in the remaining 9.

CONCLUSION
From this work we have identified up to 16 potential patients (28%) who could have been offered SRS treatment. As an innovative and committed centre, we want to optimise this service further. Moving forward, we will survey the consultant body to ask what they believe the potential barriers to SRS referral are, and deliver tumour-site-specific and hospital-wide education sessions on SRS treatment and delivery. After these educational interventions, we will review a further 6 months of patient data and compare to this work.