Paediatric CNS Tumours

2021 ◽  
pp. 373-394
Author(s):  
Prashanth Giridhar
Keyword(s):  
Cns Tumours

Postural sway in children and young adults, survivors of CNS tumours

2008 ◽  
Vol 53 (2) ◽  
Author(s):  
M Syczewska ◽  
B Dembowska-Bagińska ◽  
M Perek-Polnik ◽  
M Kalinowska ◽  
D Perek
Keyword(s):  
Young Adults ◽  
Postural Sway ◽  
Cns Tumours ◽  
Children And Young Adults

scholarly journals QOL-49. THE IMPACT OF OTOTOXICITY AND VISUAL IMPAIRMENT ON EDUCATION IN CHILDREN TREATED FOR CNS TUMOURS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii440-iii440
Author(s):  
Harriet Dulson ◽  
Rachel McAndrew ◽  
Mark Brougham
Keyword(s):  
Visual Impairment ◽  
Impaired Hearing ◽  
Cranial Radiotherapy ◽  
Radiotherapy Group ◽  
Platinum Based Chemotherapy ◽  
Cns Tumours ◽  
Group 3 ◽  
Group 2 ◽  
The Impact ◽  
Group 1

Abstract INTRODUCTION Children treated for CNS tumours experience a very high burden of adverse effects. Platinum-based chemotherapy and cranial radiotherapy can cause ototoxicity, which may be particularly problematic in patients who have impaired vision and cognition as a result of their tumour and associated treatment. This study assessed the prevalence of impaired hearing and vision and how this may impact upon education. METHODS 53 patients diagnosed with solid tumours in Edinburgh, UK between August 2013–2018 were included in the study. Patients were split into three groups according to treatment received: Group 1 – cisplatin-based chemotherapy and cranial radiotherapy; Group 2 - platinum-based chemotherapy, no cranial radiotherapy; Group 3 – benign brain tumours treated with surgery only. Data was collected retrospectively from patient notes. RESULTS Overall 69.5% of those treated with platinum-based chemotherapy experienced ototoxicity as assessed by Brock grading and 5.9% of patients had reduced visual acuity. Patients in Group 1 had the highest prevalence of both. 44.4% of patients in Group 1 needed increased educational support following treatment, either with extra support in the classroom or being unable to continue in mainstream school. 12.5% of Group 2 patients required such support and 31.3% in Group 3. CONCLUSIONS Children with CNS tumours frequently require support for future education but those treated with both platinum-based chemotherapy and cranial radiotherapy are at particular risk, which may be compounded by co-existent ototoxicity and visual impairment. It is essential to provide appropriate support for this patient cohort in order to maximise their educational potential.

scholarly journals PATH-17. INTRAGENIC COPY NUMBER BREAKPOINT ANALYSIS OF METHYLATION DATA FROM CNS TUMOURS IDENTIFIES NOVEL SUBGROUP-SPECIFIC CANDIDATE FUSION GENE ENRICHMENTS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii427-iii428
Author(s):  
Alan Mackay ◽  
Yura Grabovska ◽  
Matthew Clarke ◽  
Diana Carvalho ◽  
Sara Temelso ◽  
...  
Keyword(s):  
Copy Number ◽  
Fusion Gene ◽  
High Grade Glioma ◽  
Structural Variations ◽  
Methylation Array ◽  
Kinase Gene ◽  
Integrated Diagnosis ◽  
Cns Tumours ◽  
Breakpoint Analysis ◽  
Balanced Translocations

Abstract Methylation array-based molecular profiling has redefined the classification of brain tumours and now forms an important part of their integrated diagnosis, providing both subgroup assignment and genome wide DNA copy number profiles. These latter data can be used to identify intragenic breakpoints which are frequently associated with structural variations resulting in therapeutically targetable oncogenic fusion genes. To systematically assess the landscape of these alterations, we combined publicly available methylation datasets resulting in a total of 5660 CNS tumours, around half paediatric, and including >1000 high grade glioma and DIPG. These were analysed by standard methodology (MNP, conumee), and intragenic breakpoint enrichment was compared within methylation subgroups, superfamilies, and tumours with no high-scoring classification. Benchmarking included sequence-verified cases such as infant hemispheric gliomas (IHG) with ALK(15%) and ROS1(7%) fusions, and pathognomic alterations associated with specific entities such as RELA-EPN, MYB-LGG and HGNET-MN1. We identified previously unreported enrichments of well-recognised fusion targets such as NTRK2in GBM_MID and NTRK3in DMG_K27 (both 5%), METin A_IDH / A_IDH_HG (3–5%), and FGFR1/3in GBM_G34 (8–9%). Novel recurrent kinase gene candidates to be verified and explored further include IGF1Rin 2–12% cases spanning glioma subgroups, and TIE1in poorly classified tumours. This latter ‘NOS’ group were also enriched in various transcription factor targets of breakpoints, including TCF4and PLAGL2. Despite limitations due to sample quality, resolution or balanced translocations, breakpoint analysis of methylation copy number profiles provides simple screening for structural rearrangements which may directly influence targeted therapy in paediatric CNS tumours.

scholarly journals 55 Malignant primary brain and other central nervous system tumours diagnosed in the Canadian population from 2009 to 2013

2018 ◽  
Vol 45 (S3) ◽  
pp. S16-S17
Author(s):  
EV Walker ◽  
F Davis ◽  
Keyword(s):  
United States ◽  
Central Nervous System ◽  
Nervous System ◽  
Brain Tumour ◽  
Age Distribution ◽  
Paediatric Population ◽  
The United States ◽  
Incidence Rates ◽  
Canadian Population ◽  
Cns Tumours

The Canadian Brain Tumour Registry (CBTR) project was established in 2016 with the aim of enhancing infrastructure for surveillance and clinical research to improve health outcomes for brain tumour patients in Canada. We present a national surveillance report on malignant primary brain and central nervous system (CNS) tumours diagnosed in the Canadian population from 2009-2013. Patients were identified through the Canadian Cancer Registry (CCR); an administrative dataset that includes cancer incidence data from all provinces/territories in Canada. Cancer diagnoses are coded using the ICD-O3 system. Tumour types were classified by site and histology using The Central Brain Tumour Registry of the United States definitions. Incidence rates (IR) and 95% confidence intervals (CI) were calculated per 100,000 person-years and standardized to the 2011 census population age-distribution. Overall, 12,115 malignant brain and CNS tumours were diagnosed in the Canadian population from 2009-2013 (IR:8.43;95%CI:8.28,8.58). Of these, 6,845 were diagnosed in males (IR:9.72;95%CI:9.49,9.95) and 5,270 in females (IR:7.20;95%CI:7.00,7.39). The most common histology overall was glioblastoma (IR:4.06;95%CI:3.95,4.16). Among those aged 0-19 years, 1,130 malignant brain and CNS tumours were diagnosed from 2009-2013 (IR:3.36;95%CI:3.16,3.56). Of these, 625 were diagnosed in males (IR:3.32;95%CI:3.34,3.92) and 505 in females (IR:3.08;95%CI:2.81,3.36). The most common histology among the paediatric population was pilocytic astrocytoma (IR:0.73;95%CI:0.64,0.83). The presentation will include: IRs for other histologies, the geographic distribution of cases and a comparison between Canada and the United States.

scholarly journals A case of metastatic adenocarcinoma of the prostate arising in a meningioma

2013 ◽  
Vol 3 (3) ◽  
pp. 4 ◽  
Author(s):  
Devin Pugsley ◽  
Greg Bailly ◽  
Rekha Gupta ◽  
Derek Wilke ◽  
Lori Wood
Keyword(s):  
Prostate Cancer ◽  
Central Nervous System ◽  
Nervous System ◽  
Prostate Carcinoma ◽  
Metastatic Adenocarcinoma ◽  
Cns Metastases ◽  
Diagnostic Uncertainty ◽  
Sont Encore ◽  
Adenocarcinoma Of The Prostate ◽  
Cns Tumours

We present the case of a 70-year-old man who had a prostate adenocarcinomathat metastatized to a previously unknown cranialmeningioma. Central nervous system (CNS) metastases are veryuncommon in patients with prostate cancer, and metastases topre-existing primary CNS tumours are even more uncommon. Rareevents like this can cause diagnostic uncertainty, as shown by thiscase. This case is a reminder for clinicians to consider prostatemetastases in patients with known prostate carcinoma and focalneurological symptoms.Nous décrivons le cas d'un homme de 70 ans présentant un adénocarcinomemétastatique de la prostate secondaire à un méningiomeintra-crânien non diagnostiqué auparavant. Les métastasesau niveau du SNC sont très rares chez les patients atteints de cancerde la prostate et les métastases sont encore plus rares dansle cas de tumeurs primitives pré-existantes au niveau du SNC.La rareté de ces cas peut soulever des incertitudes au niveau dudiagnostic, comme nous le montrerons. Ce cas permet de rappeleraux cliniciens d'envisager la possibilité de métastases chezles patients atteints d'un carcinome prostatique confirmé et présentantdes signes neurologiques focaux.

scholarly journals Hearing loss and quality of life in survivors of paediatric CNS tumours and other cancers

2018 ◽  
Vol 28 (2) ◽  
pp. 515-521 ◽  
Author(s):  
Annette Weiss ◽  
◽  
Grit Sommer ◽  
Christina Schindera ◽  
Laura Wengenroth ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Hearing Loss ◽  
Cns Tumours

scholarly journals Raised cardiovascular disease mortality after central nervous system tumour diagnosis: analysis of 171 926 patients from UK and USA

2021 ◽  
Author(s):  
KAI JIN ◽  
Michael TC Poon ◽  
Paul M Brennan ◽  
Catherine LM Sudlow ◽  
Jonine D Figueroa
Keyword(s):  
Cardiovascular Disease ◽  
Central Nervous System ◽  
Nervous System ◽  
General Population ◽  
Mortality Risk ◽  
Cox Regression ◽  
Malignant Tumours ◽  
Targeted Interventions ◽  
Cns Tumours ◽  
Cvd Mortality

Background Patients with central nervous system (CNS) tumours have significant morbidity and mortality. Some studies showed CNS tumours patients may be at risk for cardiovascular disease (CVD) mortality. The magnitude of CVD risk among CNS tumours patients has not been comprehensively assessed. If CVD mortality is elevated in this population, there may be a potential for risk reduction to improve outcomes. We examined CVD mortality risk in patients with malignant and non-malignant CNS tumours. Methods We conducted analyses using UK (Wales)-based Secure Anonymised Information Linkage (SAIL) for 8743 CNS tumour patients diagnosed in 2000-2015 (54.9% of whom died) and US-based National Cancer Institutes Surveillance, Epidemiology, and End Results (SEER) for 163183 patients in 2005-2015 (39.6% of whom died). We calculated age-, sex-, and calendar-year- adjusted standardised mortality ratios (SMRs) for CVD death in CNS tumour patients compared to Welsh and US residents. We used multivariable cause-specific Cox regression models to examine factors associated with CVD mortality among CNS tumour patients. We stratified all analyses by malignancy and main histological types. Results CVD was the second commonest cause of death for CNS tumour patients in SAIL (UK) and SEER (US) (9.5% & 11.7%, respectively). Patients with CNS tumours had higher CVD mortality than the general population (SAIL SMR=2.64, 95% CI=2.39-2.90; SEER SMR=1.38, 95% CI=1.35-1.42). Malignant CNS tumour patients had over 2-fold higher CVD mortality risk in both US and UK cohorts. SMRs for non-malignant tumours were almost 2-fold higher in SAIL than in SEER (SAIL SMR=2.73, 95% CI=2.46-3.02; SEER SMR=1.30, 95% CI=1.26-1.33). The greatest magnitude of excess CVD mortality risk, particularly from cerebrovascular disease, was substantially greater in patients diagnosed at age younger than 50 years and within the first year after their cancer diagnosis (SAIL SMR=2.98, 95% CI=2.39-3.66; SEER SMR=2.14, 95% CI=2.03-2.25). Age, sex, race/ethnicity in USA, deprivation in UK and no surgery were associated with CVD mortality. Discussion CVD mortality is high among patients diagnosed with both malignant and non-malignant CNS tumours compared to the general population. Targeted interventions and risk stratification tools might improve survival.

scholarly journals Multinodular and Vacuolating Neuronal Tumor

2018 ◽  
Vol 6 (9) ◽  
pp. 1697-1698 ◽  
Author(s):  
Charmaine Zahra ◽  
Reuben Grech
Keyword(s):  
Case Report ◽  
Brain Imaging ◽  
Temporal Lobe ◽  
Benign Lesion ◽  
Occipital Lobe ◽  
Imaging Findings ◽  
Cns Tumours ◽  
The Right ◽  
Rare Diagnosis ◽  
Neuronal Tumor

BACKGROUND: Multinodular and Vacuolating Neuronal Tumor (MVNT) of the cerebrum is a benign lesion described recently in the WHO CNS tumours in 2016. Although this tumour is uncommon, clinicians should be acquainted with the possible presentation and imaging findings. CASE REPORT: We present a case of a young gentleman whose only symptom was absence seizures. Brain imaging showed lesions, compatible with this rare diagnosis. CONCLUSION: Our description of imaging findings on MRI highlights the characteristic cystic appearances of note in the right occipital lobe, in contrast to the temporal lobe as the predominant location found in previous cases.

Clinical importance of molecular markers of adult diffuse glioma

2019 ◽  
Vol 19 (5) ◽  
pp. 412-416 ◽  
Author(s):  
Emanuela Molinari ◽  
Olimpia E Curran ◽  
Robin Grant
Keyword(s):  
Molecular Markers ◽  
Molecular Genetic ◽  
Clinical Importance ◽  
Response To Treatment ◽  
Low Grade ◽  
Diffuse Glioma ◽  
Wild Type ◽  
Genetic Classification ◽  
Cns Tumours

In 2016, the WHO incorporated molecular markers, in addition to histology, into the diagnostic classification of central nervous system (CNS) tumours. This improves diagnostic accuracy and prognostication: oligo-astrocytoma no longer exists as a clinical entity; isocitrate dehydrogenase (IDH) mutant and 1p/19q co-deleted oligodendroglioma is a smaller category with better prognosis; IDH wild-type ‘low-grade’ glioma has a much poorer prognosis; and glioblastoma is divided into IDH mutant (with an better prognosis than pre-2016 glioblastoma) and IDH wild type (with a poorer prognosis). Previous advice based on phenotype alone will change with respect to median survival, best management plan and response to treatment. There are implications for routine neuropathology reporting and future trial design. Cases that are difficult to classify may need more advanced molecular genetic classification through DNA methylation-based classification of CNS tumours (Heidelberg Classifier). We discuss the practical implications.

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