Glial Central Nervous System Tumours of Childhood

2020 ◽  
pp. 179-187
Author(s):  
Dannis van Vuurden ◽  
Darren Hargrave ◽  
Dominik Sturm ◽  
David T.W. Jones
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
World Health ◽  
High Grade ◽  
Dismal Prognosis ◽  
Central Nervous System Tumours ◽  
Current Therapies ◽  
Cns Tumours ◽  
Health Organization ◽  
Future Outcomes

Gliomas are the largest group of paediatric central nervous system (CNS) tumours and can affect any age and location. Currently, they are divided into low and high grade using the World Health Organization (WHO) classification but, increasingly, biology is being used to classify and stratify therapy, and this division may be challenged in the future. Outcomes vary greatly with some low-grade tumours being very indolent, requiring nothing more than careful observation, compared to aggressive high-grade gliomas of the brainstem that have a dismal prognosis. Current therapies are based on surgery, radiotherapy, and cytotoxic chemotherapy but, increasingly, biologically targeted therapies are being explored in an attempt to increase survival and decrease late effects and the burden of treatment.

scholarly journals Oncology Referral Concerning Paediatric Neurosurgery? Analysis of the Situation between 2008-2013 in the North of Portugal

2016 ◽  
Vol 29 (1) ◽  
pp. 15
Author(s):  
Ana Batista ◽  
Luís Nogueira-Silva ◽  
Rui Vaz ◽  
Joana Oliveira
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Survival Rates ◽  
Population Based ◽  
World Health ◽  
The North ◽  
Central Nervous System Tumours ◽  
Significant Difference ◽  
Health Organization ◽  
Tumour Characteristics

<p><strong>Introduction:</strong> Primary central nervous system tumours represent the second most frequent neoplasm and are the leading cause of death from solid tumours in children. Our study aimed to provide the most up-to-date epidemiological analysis of primary central nervous system tumours in children living in the north of Portugal and to explore a possible effect of the centralisation of care on outcomes.<br /><strong>Material and Methods:</strong> This population-based study included all newly diagnosed and histologically confirmed primary central nervous system tumours in people below 18 years old, living in the north of Portugal, from January 2008 to December 2013. Data were collected through access to clinical files. We described children and tumour characteristics, and analysed patient: paediatric neurosurgeon and histological tumour type: paediatric neurosurgeon ratios.<br /><strong>Results:</strong> A total of 147 cases was recorded. Mean age at diagnosis of primary central nervous system tumours was 8.0 ± 5.2 years. Astrocytomas were the most common histological type (34%). We found that World Health Organization Tumour Grade was the only variable of statistically significant influence in mortality (p = 0.001). 1-, 3- and 5-year survivals related to 2008-2013 period were statistically higher than 2005 - 2007. Astrocytomas have the highest survival rate, with statistically significant difference from other tumours (p = 0.015). We estimated a mean of 12.25 treated cases per year by each paediatric neurosurgeon at our institution.<br /><strong>Discussion:</strong> Our results are globally consistent with those published all over the world, but we reported the highest survival rates. The number of cases treated per year by each neurosurgeon is accordant to the recommended.<br /><strong>Conclusion:</strong> We showed an improvement when comparing to data previous to 2008 and better results than those from previous studies, namely higher survival at 1-, 3- and 5-years from the diagnosis, with low complication and relapsing rates. Therefore, the referral of primary central nervous system tumours cases to centres with larger experience can be invaluable for the achievement of the best outcomes.</p>

Long-term survival in a child with a central nervous system medulloepithelioma

2008 ◽  
Vol 2 (5) ◽  
pp. 339-345 ◽  
Author(s):  
Parham Moftakhar ◽  
Xuemo Fan ◽  
Carole H. Hurvitz ◽  
Keith L. Black ◽  
Moise Danielpour
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Treatment Strategies ◽  
High Dose Chemotherapy ◽  
World Health ◽  
High Dose ◽  
Term Survival ◽  
Stem Cell Rescue ◽  
Dismal Prognosis ◽  
Health Organization

Central nervous system medulloepitheliomas are extremely rare and malignant (World Health Organization Grade IV) primitive neuroectodermal tumors (PNETs) that arise in childhood. Unlike other PNETs, medulloepitheliomas have a dismal prognosis, with only 2 reported cases in the literature in which the patient survived beyond 5 years after treatment. The authors report on the third known case of a child who survived longer than 5 years. A review of all the published cases of medulloepithelioma is also presented, and alternative treatment strategies for PNET tumors, including high-dose chemotherapy with stem-cell rescue, are discussed.

scholarly journals Oncology Referral Concerning Paediatric Neurosurgery? Analysis of the Situation between 2008-2013 in the North of Portugal

2016 ◽  
Vol 29 (1) ◽  
pp. 15
Author(s):  
Ana Batista ◽  
Luís Nogueira-Silva ◽  
Rui Vaz ◽  
Joana Oliveira
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Survival Rates ◽  
Population Based ◽  
World Health ◽  
The North ◽  
Central Nervous System Tumours ◽  
Significant Difference ◽  
Health Organization ◽  
Tumour Characteristics

<p><strong>Introduction:</strong> Primary central nervous system tumours represent the second most frequent neoplasm and are the leading cause of death from solid tumours in children. Our study aimed to provide the most up-to-date epidemiological analysis of primary central nervous system tumours in children living in the north of Portugal and to explore a possible effect of the centralisation of care on outcomes.<br /><strong>Material and Methods:</strong> This population-based study included all newly diagnosed and histologically confirmed primary central nervous system tumours in people below 18 years old, living in the north of Portugal, from January 2008 to December 2013. Data were collected through access to clinical files. We described children and tumour characteristics, and analysed patient: paediatric neurosurgeon and histological tumour type: paediatric neurosurgeon ratios.<br /><strong>Results:</strong> A total of 147 cases was recorded. Mean age at diagnosis of primary central nervous system tumours was 8.0 ± 5.2 years. Astrocytomas were the most common histological type (34%). We found that World Health Organization Tumour Grade was the only variable of statistically significant influence in mortality (p = 0.001). 1-, 3- and 5-year survivals related to 2008-2013 period were statistically higher than 2005 - 2007. Astrocytomas have the highest survival rate, with statistically significant difference from other tumours (p = 0.015). We estimated a mean of 12.25 treated cases per year by each paediatric neurosurgeon at our institution.<br /><strong>Discussion:</strong> Our results are globally consistent with those published all over the world, but we reported the highest survival rates. The number of cases treated per year by each neurosurgeon is accordant to the recommended.<br /><strong>Conclusion:</strong> We showed an improvement when comparing to data previous to 2008 and better results than those from previous studies, namely higher survival at 1-, 3- and 5-years from the diagnosis, with low complication and relapsing rates. Therefore, the referral of primary central nervous system tumours cases to centres with larger experience can be invaluable for the achievement of the best outcomes.</p>

scholarly journals Clinicopathological Study of Primary Central Nervous System Tumours - A Descriptive Study in a Tertiary Care Center Hospital of Dakshina Kannada

2021 ◽  
Vol 10 (36) ◽  
pp. 3109-3114
Author(s):  
Deepak Panasseril Jayapradeep ◽  
Muktha R. Pai
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Clinical Features ◽  
Who Classification ◽  
The Body ◽  
World Health ◽  
Research Centre ◽  
Central Nervous System Tumours ◽  
The Central Nervous System ◽  
Cns Tumours

BACKGROUND The central nervous system (CNS) consisting of brain and spinal cord is a delicate and a complex organ. Even a minor lesion within the central nervous system can significantly affect the higher functions and the voluntary and involuntary systems of the body. The CNS tumours have become one among the leading cause of cancer death in the present days. Early diagnosis and proper grading of these tumours can significantly improve the patient outcome. This study was conducted with an objective of correlating the clinical features with histomorphological characteristics of the primary CNS tumours and to grade the primary CNS tumors based on World Health Organization (WHO) classification. METHODS This was a descriptive observational study. A total of 117 cases of primary CNS tumours were collected from January 2012 to June 2017 at the central diagnostic laboratory of A.J Institute of Medical Sciences and Research Centre, Mangalore belonging to the Dakshina Kannada district of Karnataka, India. Out of 117 cases, 35 cases were retrieved from the files and remaining 82 were fresh cases. The tissues were fixed in 10 % buffered formalin and routinely processed. The tissue sections were stained with haematoxylin and eosin and were classified based on WHO classification. Special stain like reticulin was done in selected cases to establish the diagnosis. Patient details including the complete clinical history was collected to correlate with the histological findings. RESULTS Meningeal tumours were the maximum (37.6 %) among the central nervous system tumours in present study. Clinically, most of them (40.9 %) presented with headache and seizures. The commonest clinical presentation of central nervous system tumours observed in the present study was seizures (31.6 %). Middle cranial fossa was the preferred site for the CNS tumours (35 %). The study showed a female preponderance for CNS tumour with a male to female ratio of 1 : 1.3. The mean age for primary CNS tumours observed in the present study was 43.94 years. CONCLUSIONS In the present study, a systematic analysis of primary central nervous system tumour has been done giving due importance to the clinical features. The present study also showed a significant correlation with that of other studies. Despite of having modern imaging technique, the histopathological examination remains as the gold standard in diagnosing CNS tumours. KEY WORDS CNS Tumours, Neuroepithelial Tumours, Meningioma, Astrocytoma, Schwannoma.

scholarly journals High-grade astrocytoma with piloid features (HGAP): the Charité experience with a new central nervous system tumor entity

2021 ◽  
Author(s):  
Katja Bender ◽  
Eilís Perez ◽  
Mihaela Chirica ◽  
Julia Onken ◽  
Johannes Kahn ◽  
...  
Keyword(s):  
Dna Methylation ◽  
Central Nervous System ◽  
Nervous System ◽  
World Health ◽  
Central Nervous System Tumor ◽  
High Grade ◽  
Thoracic Spinal Cord ◽  
High Grade Astrocytoma ◽  
Tumor Entity

Abstract Purpose High-grade astrocytoma with piloid features (HGAP) is a recently described brain tumor entity defined by a specific DNA methylation profile. HGAP has been proposed to be integrated in the upcoming World Health Organization classification of central nervous system tumors expected in 2021. In this series, we present the first single-center experience with this new entity. Methods During 2017 and 2020, six HGAP were identified. Clinical course, surgical procedure, histopathology, genome-wide DNA methylation analysis, imaging, and adjuvant therapy were collected. Results Tumors were localized in the brain stem (n = 1), cerebellar peduncle (n = 1), diencephalon (n = 1), mesencephalon (n = 1), cerebrum (n = 1) and the thoracic spinal cord (n = 2). The lesions typically presented as T1w hypo- to isointense and T2w hyperintense with inhomogeneous contrast enhancement on MRI. All patients underwent initial surgical intervention. Three patients received adjuvant radiochemotherapy, and one patient adjuvant radiotherapy alone. Four patients died of disease, with an overall survival of 1.8, 9.1, 14.8 and 18.1 months. One patient was alive at the time of last follow-up, 14.6 months after surgery, and one patient was lost to follow-up. Apart from one tumor, the lesions did not present with high grade histology, however patients showed poor clinical outcomes. Conclusions Here, we provide detailed clinical, neuroradiological, histological, and molecular pathological information which might aid in clinical decision making until larger case series are published. With the exception of one case, the tumors did not present with high-grade histology but patients still showed short intervals between diagnosis and tumor progression or death even after extensive multimodal therapy.

scholarly journals Pilomyxoid Astrocytoma Occurring in the Third Ventricle

2015 ◽  
Vol 5 ◽  
pp. 41
Author(s):  
Sanghyeon Kim ◽  
Myongjin Kang ◽  
Sunseob Choi ◽  
Dae Cheol Kim
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
World Health Organization ◽  
Third Ventricle ◽  
World Health ◽  
Central Nervous System Tumor ◽  
Pilomyxoid Astrocytoma ◽  
The Third ◽  
The Central Nervous System ◽  
Health Organization

Pilomyxoid astrocytoma (PMA) is a rare central nervous system tumor that has been included in the 2007 World Health Organization Classification of Tumors of the Central Nervous System. Due to its more aggressive behavior, PMA is classified as Grade II neoplasm by the World Health Organization. PMA predominantly affects the hypothalamic/chiasmatic region and occurs in children (mean age of occurrence = 10 months). We report a case of a 24-year-old man who presented with headache, nausea, and vomiting. Brain CT and MRI revealed a mass occupying only the third ventricle. We performed partial resection. Histological findings, including monophasic growth with a myxoid background, and absence of Rosenthal fibers or eosinophilic granular bodies, as well as the strong positivity for glial fibrillary acidic protein were consistent with PMA.

The 2016 World Health Organization classification of tumours of the central nervous system

2018 ◽  
Vol 47 (11-12) ◽  
pp. e187-e200 ◽  
Author(s):  
Chiara Villa ◽  
Catherine Miquel ◽  
Dominic Mosses ◽  
Michèle Bernier ◽  
Anna Luisa Di Stefano
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
World Health Organization ◽  
World Health ◽  
World Health Organization Classification ◽  
The Central Nervous System ◽  
Health Organization

Accuracy of Intraoperative Examination in Central Nervous System Lesions: A Study of 133 Cases

2019 ◽  
Vol 63 (3) ◽  
pp. 224-232
Author(s):  
Ludmila Barbosa de Souza Balsimelli ◽  
Jamille Costa de Oliveira ◽  
Flora Ávila Adorno ◽  
Clarissa Almeida Brites ◽  
Giuliano Stefanello Bublitz ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Frozen Section ◽  
Final Diagnosis ◽  
World Health ◽  
Histopathological Diagnosis ◽  
Low Grade ◽  
Predictive Values ◽  
Health Organization ◽  
Cns Lesions

Objective: Intraoperative examination is a highly valuable tool for the evaluation of central nervous system (CNS) lesions, helping the neurosurgeon to determine the best surgical management. This study aimed to evaluate the accuracy and to analyze the diagnostic disagreements and pitfalls of the intraoperative examinations through correlation with the final histopathological diagnosis in CNS lesions. Study Design: Retrospective analysis of intraoperative examination of CNS lesions and their final diagnosis obtained during 16 consecutive years. All diagnoses were reviewed and classified according to World Health Organization (WHO) grading for CNS tumors. Squash was performed in 119 cases, while frozen section and both methods were done in 7 cases each. Results: Among the 133 intraoperative examinations considered, 114 (85.7%) presented concordance and 19 (14.3%) diagnostic disagreement when compared with subsequent histopathological examinations. The sensitivity and specificity for the detection of neoplasia in intraoperative examination was 98 and 94%, respectively. The positive and negative predictive values were 99 and 88%, respectively. The accuracy for neoplastic and nonneoplastic disease was 85.7%. Disagreements were more frequent among low-grade (WHO grades I and II) neoplasms and nonmalignant cases. Conclusions: Our results showed good accuracy of the intraoperative assessments for diagnosis of CNS lesions, particularly in high-grade (grades III and IV) lesions and metastatic neoplasms.

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