5,192 results match your criteria Primitive Neuroectodermal Tumors of the Central Nervous System


Next-generation whole exome sequencing of glioblastoma with a primitive neuronal component.

Brain Tumor Pathol 2019 Feb 4. Epub 2019 Feb 4.

Department of Pathology and Laboratory Medicine, North Shore University Hospital and Long Island Jewish Medical Center, Lake Success, 11042, NY, USA.

Glioblastoma with a primitive neuronal component (GBM-PN) was renamed from glioblastoma with primitive neuroectodermal tumor-like component (GBM-PNET) in the new WHO classification of tumors of the central nervous system in 2016. GBM-PN is a rare variant of glioblastoma. There were not so many publications on the investigation of GBM-PN. Read More

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http://link.springer.com/10.1007/s10014-019-00334-1
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http://dx.doi.org/10.1007/s10014-019-00334-1DOI Listing
February 2019
5 Reads

Sleeping Beauty insertional mutagenesis reveals important genetic drivers of central nervous system embryonal tumors.

Cancer Res 2019 Jan 23. Epub 2019 Jan 23.

Department of Pediatrics, University of Minnesota

Medulloblastoma and central nervous system primitive neuroectodermal tumors (CNS-PNET) are aggressive, poorly differentiated brain tumors with limited effective therapies. Using Sleeping Beauty (SB) transposon mutagenesis, we identified novel genetic drivers of medulloblastoma and CNS-PNET. Cross-species gene expression analyses classified SB-driven tumors into distinct medulloblastoma and CNS-PNET subgroups, indicating they resemble human SHH and group 3 and 4 medulloblastoma and CNS neuroblastoma with FOXR2 activation. Read More

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http://cancerres.aacrjournals.org/lookup/doi/10.1158/0008-54
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http://dx.doi.org/10.1158/0008-5472.CAN-18-1261DOI Listing
January 2019
5 Reads

Primary spinal primitive neuroectodermal tumor: A single center series with literature review.

J Spinal Cord Med 2018 Dec 18:1-9. Epub 2018 Dec 18.

a Department of Neurosurgery , Beijing Tiantan Hospital affiliated to Capital Medical University , Beijing , People's Republic of China.

Context: Primary spinal primitive neuroectodermal tumor (PNET) of the central nervous system has a low incidence. The intraspinal case is very rare. Around 30 cases have been reported so far. Read More

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https://www.tandfonline.com/doi/full/10.1080/10790268.2018.1
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http://dx.doi.org/10.1080/10790268.2018.1547862DOI Listing
December 2018
11 Reads
1.878 Impact Factor

Neuroglial Differentiation and Neoplasms in Testicular Germ Cell Tumors Lack Immunohistochemical Evidence of Alterations Characteristic of Their CNS Counterparts: A Study of 13 Cases.

Am J Surg Pathol 2019 Mar;43(3):422-431

Departments of Pathology.

Overgrowth of neuroglial tissue is rare in testicular germ cell tumors and mostly reported as isolated cases. We retrospectively reviewed 13 cases of testicular germ cell tumors from 2 institutions from 1995 to 2018. Hematoxylin and eosin slides were collected and reviewed. Read More

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http://dx.doi.org/10.1097/PAS.0000000000001206DOI Listing
March 2019
3 Reads

Silencing the ACAT1 Gene in Human SH-SY5Y Neuroblastoma Cells Inhibits the Expression of Cyclo-Oxygenase 2 (COX2) and Reduces β-Amyloid-Induced Toxicity Due to Activation of Protein Kinase C (PKC) and ERK.

Med Sci Monit 2018 Dec 12;24:9007-9018. Epub 2018 Dec 12.

Department of Anatomy, Histology and Embryology, School of Basic Medical Sciences, Fudan University, Shanghai, China (mainland).

BACKGROUND Acyl-coenzymeA: cholesterol acyltransferase (ACAT) 1, a key enzyme converting excess free cholesterol to cholesterol esters, has been demonstrated to be associated with the pathogenesis of Alzheimer disease (AD). However, the mechanism underlying the protective role of ACAT1 blockage in AD progression remains elusive. MATERIAL AND METHODS Human neuroblastoma SH-SY5Y cells were treated for 24 h with increasing concentrations of aggregated Aβ₂₅₋₃₅ (5, 15, 25, and 45 μmol) with or without the ACAT1 siRNA pretreatment. Read More

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http://dx.doi.org/10.12659/MSM.912862DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6299791PMC
December 2018
1 Read

Effects of Long-Term Treatment with a Blend of Highly Purified Olive Secoiridoids on Cognition and Brain ATP Levels in Aged NMRI Mice.

Oxid Med Cell Longev 2018 30;2018:4070935. Epub 2018 Oct 30.

Institute of Nutritional Sciences, Laboratory for Nutrition in Prevention and Therapy, Justus-Liebig-University of Giessen, Biomedical Research Center Seltersberg (BFS), Schubertstr. 81, 35390 Giessen, Germany.

Aging represents a major risk factor for developing neurodegenerative diseases such as Alzheimer's disease (AD). As components of the Mediterranean diet, olive polyphenols may play a crucial role in the prevention of AD. Since mitochondrial dysfunction acts as a final pathway in both brain aging and AD, respectively, the effects of a mixture of highly purified olive secoiridoids were tested on cognition and ATP levels in a commonly used mouse model for brain aging. Read More

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http://dx.doi.org/10.1155/2018/4070935DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6232801PMC
January 2019

Experience with 102 Frameless Stereotactic Biopsies Using the neuromate Robotic Device.

World Neurosurg 2018 Nov 27. Epub 2018 Nov 27.

Department of Neurosurgery, Bethel Clinic, Bielefeld, Germany. Electronic address:

Objective: Stereotactic biopsy is a standard procedure in neurosurgery. In addition to or even replacing frame-based stereotaxy, some centers also use frameless imaging-based techniques and more recently robotic systems. Here we report a retrospective analysis of our experience with 102 consecutive biopsies performed in our institution using the neuromate robotic device. Read More

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http://dx.doi.org/10.1016/j.wneu.2018.11.187DOI Listing
November 2018
4 Reads

Sellar Embryonal Tumor: A Case Report and Review of the Literature.

Asian J Neurosurg 2018 Oct-Dec;13(4):1197-1201

Department of Neurosurgery, Ankara University, Ankara, Turkey.

Primitive neuroectodermal tumors (PNETs) are aggressive, poorly differentiated tumors in children and young adults. However, the embryonal tumor group did not include the central nervous system (CNS) PNET title and ependymoblastoma subtitle in the 2016 World Health Organization CNS tumor classification. Here, we report the case of a 6-year-old boy with a sellar embryonal tumor and present a review of the related literature. Read More

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http://dx.doi.org/10.4103/ajns.AJNS_30_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6208192PMC
November 2018
9 Reads

Melanotic Neuroectodermal Tumor of Infancy.

Arch Pathol Lab Med 2018 Nov;142(11):1358-1363

From the Divisions of Anatomic and Clinical Pathology (Dr Soles) and Anatomic Pathology (Drs Wilson and Lucas), Department of Pathology, Michigan Medicine, University of Michigan, Ann Arbor.

Context.—: Melanotic neuroectodermal tumor of infancy, albeit rare and generally regarded as benign, is an important tumor to recognize because of its rapid growth, potential for local recurrence, and small round blue cell morphology, which can lead to misdiagnosis of a malignant neoplasm.

Objective. Read More

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http://www.archivesofpathology.org/doi/10.5858/arpa.2018-024
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http://dx.doi.org/10.5858/arpa.2018-0241-RADOI Listing
November 2018
8 Reads

Quantitative MRI in post-operative paediatric cerebellar mutism syndrome.

Eur J Radiol 2018 Nov 10;108:43-51. Epub 2018 Sep 10.

Developmental Imaging and Biophysics Section, UCL GOS Institute of Child Health, 30 Guilford Street, London WC1N 1EH, United Kingdom. Electronic address:

Post-operative paediatric cerebellar mutism syndrome (pCMS) occurs in around 25% of children undergoing surgery for cerebellar and fourth ventricular tumours. Reversible mutism is the hallmark of a syndrome which comprises severe motor, cognitive and linguistic deficits. Recent evidence from advanced neuroimaging studies has led to the current theoretical understanding of the condition as a form of diaschisis contingent on damage to efferent cerebellar circuitry. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S0720048X183031
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http://dx.doi.org/10.1016/j.ejrad.2018.09.007DOI Listing
November 2018
15 Reads

Volumetric voxelwise apparent diffusion coefficient histogram analysis for differentiation of the fourth ventricular tumors.

Neuroradiol J 2018 Dec 19;31(6):554-564. Epub 2018 Sep 19.

1 Department of Radiology and Biomedical Imaging, Yale School of Medicine, New Haven, USA.

Purpose: We applied voxelwise apparent diffusion coefficient (ADC) histogram analysis in addition to structural magnetic resonance imaging (MRI) findings and patients' age for differentiation of intraaxial posterior fossa tumors involving the fourth ventricle.

Participants And Methods: Pretreatment MRIs of 74 patients with intraaxial brain neoplasm involving the fourth ventricle, from January 1, 2004 to December 31, 2015, were reviewed. The tumor solid components were segmented and voxelwise ADC histogram variables were determined. Read More

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http://dx.doi.org/10.1177/1971400918800803DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6243467PMC
December 2018
8 Reads

Intracranial Peripheral Primitive Neuroectodermal Tumor Arising from the Clivus with Intracranial Metastasis in an Elderly Woman: Case Report and Review of the Literature.

World Neurosurg 2018 Nov 23;119:331-334. Epub 2018 Aug 23.

Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.

Background: Intracranial peripheral primitive neuroectodermal tumors (pPNETs) are rare lesions, accounting for only 1% of all pPNETs, and usually arise from bone and soft tissue. These tumors primarily affect young patients (<30 years old) and rarely affect older patients. The common intracranial locations of these lesions are frontal dura and tentorium. Read More

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http://dx.doi.org/10.1016/j.wneu.2018.08.066DOI Listing
November 2018
3 Reads
2.420 Impact Factor

Rapid discrimination of pediatric brain tumors by mass spectrometry imaging.

J Neurooncol 2018 Nov 20;140(2):269-279. Epub 2018 Aug 20.

Winship Cancer Institute, Emory University School of Medicine, 1365C Clifton Road, Suite C5086, Atlanta, GA, 30322, USA.

Purpose: Medulloblastoma, the most common primary pediatric malignant brain tumor, originates in the posterior fossa of the brain. Pineoblastoma, which originates within the pineal gland, is a rarer malignancy that also presents in the pediatric population. Medulloblastoma and pineoblastoma exhibit overlapping clinical features and have similar histopathological characteristics. Read More

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http://dx.doi.org/10.1007/s11060-018-2978-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6244779PMC
November 2018
3 Reads

Micro-RNA-137 Inhibits Tau Hyperphosphorylation in Alzheimer's Disease and Targets the CACNA1C Gene in Transgenic Mice and Human Neuroblastoma SH-SY5Y Cells.

Med Sci Monit 2018 Aug 13;24:5635-5644. Epub 2018 Aug 13.

Department of Neurology, The Fourth Affiliated Hospital of China Medical University, Shenyang, Liaoning, China (mainland).

BACKGROUND Alzheimer's disease (AD) results in cognitive impairment. The calcium voltage-gated channel subunit alpha-1 C CACNA1C gene encodes an alpha-1 C subunit of L-type calcium channel (LTCC). The aim of this study was to investigate the role of micro-RNA-137 (miR-137) and the CACNA1C gene in APPswe/PS1ΔE9 (APP/PS1) double-transgenic AD mice and in human neuroblastoma SH-SY5Y cells. Read More

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http://dx.doi.org/10.12659/MSM.908765DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6104547PMC
August 2018
5 Reads

Congenital medulloblastoma: Fetal and postnatal longitudinal observation with quantitative MRI.

Clin Imaging 2018 Nov - Dec;52:172-176. Epub 2018 Jun 7.

Department of Radiology, University of Washington, Seattle, WA, USA; Research Institute of Biology and Biophysics, National Research Tomsk State University, Tomsk, Russian Federation.

Congenital medulloblastoma is extremely rare. MRI appearance of this tumor in the fetal brain has not been described. A case of congenital medulloblastoma initially observed by antenatal MRI with postnatal follow-up and treatment is presented. Read More

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http://dx.doi.org/10.1016/j.clinimag.2018.06.001DOI Listing
January 2019
3 Reads

Staging and following common pediatric malignancies: MRI versus CT versus functional imaging.

Authors:
Stephan D Voss

Pediatr Radiol 2018 08 4;48(9):1324-1336. Epub 2018 Aug 4.

Department of Radiology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Ave., Boston, MA, 02115, USA.

Most pediatric malignancies require some form of cross-sectional imaging, either for staging or response assessment. The majority of these are solid tumors and this review addresses the role of MRI, as well as other cross-sectional and functional imaging techniques, for evaluating the most common pediatric solid tumors. The primary emphasis is on neuroblastoma, hepatoblastoma and Wilms tumor, three of the most common non-central-nervous-system (CNS) pediatric solid tumors encountered in young children. Read More

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http://dx.doi.org/10.1007/s00247-018-4162-4DOI Listing
August 2018
5 Reads

Differential gene expression analysis tools exhibit substandard performance for long non-coding RNA-sequencing data.

Genome Biol 2018 07 24;19(1):96. Epub 2018 Jul 24.

Department of Pediatrics and Medical Genetics, Ghent University, Ghent, Belgium.

Background: Long non-coding RNAs (lncRNAs) are typically expressed at low levels and are inherently highly variable. This is a fundamental challenge for differential expression (DE) analysis. In this study, the performance of 25 pipelines for testing DE in RNA-seq data is comprehensively evaluated, with a particular focus on lncRNAs and low-abundance mRNAs. Read More

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http://dx.doi.org/10.1186/s13059-018-1466-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6058388PMC
July 2018
3 Reads

Genetically engineered cerebral organoids model brain tumor formation.

Nat Methods 2018 Aug 23;15(8):631-639. Epub 2018 Jul 23.

Institute of Molecular Biotechnology of the Austrian Academy of Sciences (IMBA), Vienna, Austria.

Brain tumors are among the most lethal and devastating cancers. Their study is limited by genetic heterogeneity and the incompleteness of available laboratory models. Three-dimensional organoid culture models offer innovative possibilities for the modeling of human disease. Read More

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http://dx.doi.org/10.1038/s41592-018-0070-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6071863PMC
August 2018
18 Reads

Detection of cavernous malformations after whole-brain radiotherapy in primitive neuroectodermal tumor patients-comparing susceptibility-weighted imaging and T2 gradient-echo sequences.

Neuroradiology 2018 Sep 20;60(9):913-919. Epub 2018 Jul 20.

Department of Diagnostic Imaging, Sheba Medical Center, Derech Sheba 2, Ramat Gan, Israel.

Purpose: The aim of this retrospective study is to investigate the value of the susceptibility-weighted imaging (SWI) sequence compared to gradient echo (GRE) in the detection and follow-up of cavernous malformations in patients who underwent whole-brain irradiation as part of their medulloblastoma treatment.

Methods: We retrospectively examined MRI studies of 28 subjects (16 males, 12 females) who received whole-brain irradiation as part of their treatment. Ages at irradiation ranged from 2 to 38 years. Read More

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http://dx.doi.org/10.1007/s00234-018-2055-8DOI Listing
September 2018
2 Reads

Recurrent homozygous deletion of DROSHA and microduplication of PDE4DIP in pineoblastoma.

Nat Commun 2018 07 20;9(1):2868. Epub 2018 Jul 20.

Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, 10065, NY, USA.

Pineoblastoma is a rare and highly aggressive brain cancer of childhood, histologically belonging to the spectrum of primitive neuroectodermal tumors. Patients with germline mutations in DICER1, a ribonuclease involved in microRNA processing, have increased risk of pineoblastoma, but genetic drivers of sporadic pineoblastoma remain unknown. Here, we analyzed pediatric and adult pineoblastoma samples (n = 23) using a combination of genome-wide DNA methylation profiling and whole-exome sequencing or whole-genome sequencing. Read More

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http://dx.doi.org/10.1038/s41467-018-05029-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6054684PMC
July 2018
6 Reads

Intracranial immature teratoma invading the nasal cavity mimicking olfactory neuroblastoma: A case report.

Medicine (Baltimore) 2018 Jul;97(28):e11527

Department of Pathology, First Affiliated Hospital and College of Basic Medical Sciences of China Medical University.

Rationale: Primary intracranial immature teratoma accounts for majority of congenital central nervous system germ-cell tumors, but it is extremely rare in patients older than 15 years.

Patient Concerns: A 27-year-old woman was referred to our hospital for headache, nasal congestion, and decreased olfactory sensation. Imaging showed a mass measuring approximately 5 cm × 4 cm in the right frontal lobe, which also filled the right nasal cavity. Read More

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http://dx.doi.org/10.1097/MD.0000000000011527DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6076187PMC
July 2018
16 Reads
5.723 Impact Factor

Molecular Classification and Management of Rare Pediatric Embryonal Brain Tumors.

Curr Oncol Rep 2018 Jul 11;20(9):69. Epub 2018 Jul 11.

Arthur and Sonia Labatt Brain Tumour Research Centre, Division of Haematology/Oncology, Hospital for Sick Children, Toronto, Ontario, M5G1X8, Canada.

Purpose Of Review: Malignant embryonal brain tumors (EBTs) of childhood span a wide clinical spectrum but can share remarkably similar morphologic features. This overlap presents significant diagnostic challenges, particularly for tumor entities that are rarely encountered in clinical practice and for which diagnostic criteria were poorly defined. This review will provide an update on the evolving characterization and treatment of rare EBTs. Read More

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http://dx.doi.org/10.1007/s11912-018-0717-7DOI Listing
July 2018
12 Reads

Surgical Treatment of Intramedullary Spinal Metastasis in Medulloblastoma: Case Report and Review of the Literature.

World Neurosurg 2018 Oct 7;118:42-46. Epub 2018 Jul 7.

Department of Neurosurgery, Nicklaus Children's Hospital, Miami, Florida, USA; Department of Neurosurgery, University of Miami Miller School of Medicine, Miami, Florida, USA.

Background: Medulloblastomas are common childhood central nervous system tumors that are prone to leptomeningeal spread. Intramedullary dissemination is rare with very few case reports existing in the available literature.

Case Description: The authors here present a case of a 14-year-old boy with Li-Fraumeni syndrome and medulloblastoma who underwent surgical resection of spinal intramedullary spread. Read More

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http://dx.doi.org/10.1016/j.wneu.2018.06.250DOI Listing
October 2018
3 Reads

Intra-cavity stem cell therapy inhibits tumor progression in a novel murine model of medulloblastoma surgical resection.

PLoS One 2018 10;13(7):e0198596. Epub 2018 Jul 10.

Division of Pharmacoengineering and Molecular Pharmaceutics, UNC Eshelman School of Pharmacy, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States of America.

Background: Cytotoxic neural stem cells (NSCs) have emerged as a promising treatment for Medulloblastoma (MB), the most common malignant primary pediatric brain tumor. The lack of accurate pre-clinical models incorporating surgical resection and tumor recurrence limits advancement in post-surgical MB treatments. Using cell lines from two of the 5 distinct MB molecular sub-groups, in this study, we developed an image-guided mouse model of MB surgical resection and investigate intra-cavity NSC therapy for post-operative MB. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0198596PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6038981PMC
December 2018
8 Reads

Effects of diesel exhaust particles on the expression of tau and autophagy proteins in human neuroblastoma cells.

Environ Toxicol Pharmacol 2018 Sep 22;62:54-59. Epub 2018 Jun 22.

School of Respiratory Therapy, College of Medicine, Taipei Medical University, Taipei, Taiwan; Division of Pulmonary Medicine Department of Internal Medicine, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan; Division of Pulmonary Medicine, Department of Internal Medicine, Shuang Ho Hospital, Taipei Medical University, New Taipei City, Taiwan. Electronic address:

Particulate air pollution is recognized as a potential risk factor for neurological disorders; however, the underlying mechanisms of neurodegenerative diseases that occur due to particulate air pollution remain unclear. The objective of the present study was to evaluate the neurotoxic effects caused by diesel exhaust particles (DEPs). We determined the ability of DEPs and carbon black (CB) to induce neurotoxicity, oxidative stress and inflammation, and to disrupt the expression of tau and autophagy proteins in human neuroblastoma IMR-32 cells. Read More

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http://dx.doi.org/10.1016/j.etap.2018.06.007DOI Listing
September 2018
6 Reads
1.862 Impact Factor

A 15-Year-Old Girl With Sudden Onsent Reversible Neurologic Symptoms After Cranial Irradiation for Medulloblastoma.

Semin Pediatr Neurol 2018 07 1;26:124-127. Epub 2017 Apr 1.

Department of Neurology, Boston Children׳s Hospital, Boston, MA. Electronic address:

A 15-year-old girl with history of medulloblastoma was evaluated for headache and neurologic deficits 5 years after completion of initial radiation therapy and 3 years following completion of reirradiation. Neurologic examination was notable for new-onset left hemianopia, hemiparesis, and neglect. Magnetic resonance imaging showed extensive areas of cortical T2 prolongation and thickening involving the right parietal, occipital, and temporal lobes with associated extensive gyral enhancement. Read More

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http://dx.doi.org/10.1016/j.spen.2017.03.019DOI Listing

Outcomes in children with central nervous system tumors disseminated at presentation: a large single-center experience.

Childs Nerv Syst 2018 Jun 24. Epub 2018 Jun 24.

Department of Neurosurgery, Great Ormond Street Hospital for Children NHS Trust, London, WC1N 3JH, UK.

Purpose: Children with disseminated central nervous system (CNS) tumors have worse outcomes than those with solitary disease, but outcomes of disease dissemination at initial presentation have not been systematically studied and compared across tumor groups to date. We evaluated the impact of tumor dissemination at presentation on management and clinical outcomes in a cohort of consecutively treated children in a single neurosurgical unit over a 14-year period.

Methods: Method used was a retrospective review of data on children presenting to Great Ormond Street Hospital, London, UK, with medulloblastoma, primitive neuroectodermal tumor, atypical teratoid rhabdoid tumor, pilocytic astrocytoma, and ependymoma between 2003 and 2016 inclusive. Read More

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http://dx.doi.org/10.1007/s00381-018-3871-1DOI Listing
June 2018
10 Reads

Seizures with Migraine-like Attacks after Radiation Therapy (SMART): A new meaning of an old acronym.

Seizure 2018 Aug 18;60:94-95. Epub 2018 Jun 18.

Department of Medical and Surgical Sciences, Magna Graecia University, Viale Europa, Catanzaro, Italy; Regional Epilepsy Centre, Great Metropolitan Hospital, Via Melacrino, Reggio Calabria, Italy; Institute of Molecular Bioimaging and Physiology, National Research Council, Viale Europa, Catanzaro, Italy. Electronic address:

Stroke-like migraine attacks after radiation therapy (SMART) is a late-onset complication of cerebral irradiation, clinically characterized by headache, seizures and focal deficits. We describe two patients with SMART presenting with focal status epilepticus and headache. We believe that SMART is a misnomer that misjudge seizures among clinical features and we suggest to rename this entity as " Seizure with Migraine-like Attacks after Radiation Therapy". Read More

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http://dx.doi.org/10.1016/j.seizure.2018.06.014DOI Listing
August 2018
3 Reads

Epigenetic regulators Rbbp4 and Hdac1 are overexpressed in a zebrafish model of RB1 embryonal brain tumor, and are required for neural progenitor survival and proliferation.

Dis Model Mech 2018 06 15;11(6). Epub 2018 Jun 15.

Department of Genetics, Development and Cell Biology, Iowa State University, Ames, IA 50011, USA

In this study, we used comparative genomics and developmental genetics to identify epigenetic regulators driving oncogenesis in a zebrafish () somatic-targeting model of RB1 mutant embryonal brain tumors. Zebrafish brain tumors caused by TALEN or CRISPR targeting are histologically similar to human central nervous system primitive neuroectodermal tumors (CNS-PNETs). Like the human oligoneural CNS-PNET subtype, zebrafish tumors show elevated expression of neural progenitor transcription factors , , and the receptor tyrosine kinase oncogene. Read More

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http://dx.doi.org/10.1242/dmm.034124DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6031359PMC
June 2018
21 Reads

Cerebellar tumors.

Handb Clin Neurol 2018 ;155:289-299

Department of Oncology and Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland. Electronic address:

The cerebellum is the most common site of presentation of central nervous system tumors in children but exceedingly rare in adults. Children often present with acute symptoms related to increased intracranial pressure, requiring urgent surgical intervention. The differential diagnosis is broad and includes a variety of benign and malignant entities. Read More

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http://dx.doi.org/10.1016/B978-0-444-64189-2.00019-6DOI Listing
October 2018
2 Reads

Cerebellar mutism syndrome.

Handb Clin Neurol 2018 ;155:273-288

Department of Radiology, University of Tennessee Health Science Center and Department of Diagnostic Imaging, St. Jude Children's Research Hospital, Memphis, TN, United States.

Cerebellar mutism most commonly, but not exclusively, develops in children after surgery for midline cerebellar or intraventricular tumors in the posterior fossa, typically medulloblastoma. Cerebellar mutism syndrome (CMS) comprises a complex set of neurologic and neurocognitive signs and symptoms, the cardinal and central component of which is an initially profound but usually reversible speech disorder. As such, CMS is currently recognized as an extreme form of the so-called cerebellar cognitive affective syndrome (Schmahmann syndrome). Read More

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http://dx.doi.org/10.1016/B978-0-444-64189-2.00018-4DOI Listing
October 2018
3 Reads

Rethinking medulloblastoma from a targeted therapeutics perspective.

J Neurooncol 2018 Sep 5;139(3):713-720. Epub 2018 Jun 5.

Department of Neurosurgery, The University of Texas M.D. Anderson Cancer Center, Houston, TX, USA.

Introduction: Medulloblastoma is an aggressive but potentially curable central nervous system tumor that remains a treatment challenge. Analysis of therapeutic targets can provide opportunities for the selection of agents.

Methods: Using multiplatform analysis, 36 medulloblastomas were extensively profiled from 2009 to 2015. Read More

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http://dx.doi.org/10.1007/s11060-018-2917-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6132970PMC
September 2018
5 Reads
3.070 Impact Factor

Embryonal brain tumor with unknown primary lesion and massive cerebrospinal fluid dissemination: A case report.

J Clin Neurosci 2018 Aug 26;54:125-128. Epub 2018 May 26.

Department of Neurosurgery, Kobe University Graduate School of Medicine, 7-5-2, Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan.

The 2007 World Health Organization Classification of Tumors of the Central Nervous System (CNS) categorized embryonal tumors of the CNS into three classes: medulloblastoma, CNS primitive neuroectodermal tumor, and atypical teratoid/rhabdoid tumor. Due to the lack of specific histological features, it was sometimes difficult to accurately differentiate CNS embryonal tumors pathologically. Here, we report a case of a young man, who presented with headache. Read More

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http://dx.doi.org/10.1016/j.jocn.2018.04.046DOI Listing
August 2018
33 Reads

Protective Effects of Donepezil Against Alcohol-Induced Toxicity in Cell Culture: Role of Caspase-3.

Neurotox Res 2018 Oct 26;34(3):757-762. Epub 2018 May 26.

Department of Pharmacology, Howard University College Medicine, 520 W Street NW, Washington, DC, 20059, USA.

Ethanol (EtOH) is one of the most frequently abused drugs with heavy health, economic, and societal burdens. Although moderate to low EtOH may have some neuroprotective effects, heavy EtOH consumption associated with high blood alcohol level (BAL) can be quite detrimental. The brain is particularly vulnerable to the damaging effects of high BAL, leading to neuronal loss, cognitive, and behavioral deficits. Read More

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http://dx.doi.org/10.1007/s12640-018-9913-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5991078PMC
October 2018
4 Reads

Atypical teratoid rhabdoid tumor in a 65-year-old man presenting with disseminated leptomeningeal disease: A case report and review of the literature.

SAGE Open Med Case Rep 2018 14;6:2050313X18775298. Epub 2018 May 14.

Department of Neurology, Duke University Hospital, Durham, NC, USA.

Central nervous system atypical teratoid rhabdoid tumors are very rare aggressive tumor of childhood, primarily occurring at age of less than 3 years old. The prognosis of these tumors is very poor, with a reported median survival of 6-12 months in most cases. Treatment typically consists of aggressive chemotherapy and radiotherapy. Read More

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http://dx.doi.org/10.1177/2050313X18775298DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5954568PMC
May 2018
3 Reads

Infantile medulloepithelioma in the lateral ventricle and cerebellopontine angle: Two case reports.

Medicine (Baltimore) 2018 May;97(20):e10751

Department of Neurosurgery.

Rationale: Intracranial medulloepitheliomas are extremely rare and highly malignant. Intraventricular medulloepitheliomas are even rarely reported, and little is known about the clinical features.

Patient Concerns: In this article, we report two cases of intracranial medulloepitheliomas. Read More

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http://dx.doi.org/10.1097/MD.0000000000010751DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5976342PMC
May 2018
1 Read

The developmental origin of brain tumours: a cellular and molecular framework.

Development 2018 05 14;145(10). Epub 2018 May 14.

Department of Oncology, University of Cambridge, Hutchison/MRC Research Centre, Cambridge Biomedical Campus, Cambridge CB2 0XZ, UK

The development of the nervous system relies on the coordinated regulation of stem cell self-renewal and differentiation. The discovery that brain tumours contain a subpopulation of cells with stem/progenitor characteristics that are capable of sustaining tumour growth has emphasized the importance of understanding the cellular dynamics and the molecular pathways regulating neural stem cell behaviour. By focusing on recent work on glioma and medulloblastoma, we review how lineage tracing contributed to dissecting the embryonic origin of brain tumours and how lineage-specific mechanisms that regulate stem cell behaviour in the embryo may be subverted in cancer to achieve uncontrolled proliferation and suppression of differentiation. Read More

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http://dx.doi.org/10.1242/dev.162693DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6001369PMC

PHOX2B is a reliable immunomarker in distinguishing peripheral neuroblastic tumours from CNS embryonal tumours.

Histopathology 2018 Sep 4;73(3):483-491. Epub 2018 Jul 4.

Department of Pathology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.

Aims: The PHOX2B gene regulates neuronal maturation in the brain stem nuclei associated with cardiorespiratory function and in the autonomic sympathetic and enteric nervous system. PHOX2B expression is a reliable immunomarker for peripheral neuroblastic tumours; however, no systematic evaluation of central nervous system (CNS) embryonal tumours was included in the studies. We encountered two cases in which the differential diagnosis included neuroblastoma and CNS embryonal tumour, and we hypothesised that PHOX2B immunostain would be helpful in establishing the diagnosis. Read More

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http://dx.doi.org/10.1111/his.13648DOI Listing
September 2018
3 Reads

PACAP and VIP regulate hypoxia-inducible factors in neuroblastoma cells exposed to hypoxia.

Neuropeptides 2018 Jun 17;69:84-91. Epub 2018 Apr 17.

Section of Human Anatomy and Histology, Department of Biomedical and Biotechnological Sciences, University of Catania, Catania, Italy. Electronic address:

Pituitary adenylate cyclase-activating polypeptide (PACAP) and vasoactive intestinal peptide (VIP) are two related peptides acting as neurotransmitters/neuromodulators in central and peripheral nervous system. They are also involved in cancer showing a controversial role. Particulary, they are implicated in neuroblastoma differentiation (NB). Read More

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http://dx.doi.org/10.1016/j.npep.2018.04.009DOI Listing
June 2018
16 Reads

Development and validation of a sensitive LC MS/MS method for the measurement of the checkpoint kinase 1 inhibitor prexasertib and its application in a cerebral microdialysis study.

J Pharm Biomed Anal 2018 Jul 17;156:97-103. Epub 2018 Apr 17.

Department of Pharmaceutical Sciences, St. Jude Children's Research Hospital, Memphis, TN, United States. Electronic address:

LC MS/MS methods to measure prexasertib in mouse plasma and Ringer's solution containing 0.5% BSA (Ringer's/BSA) were developed and validated. Liquid-liquid extraction with tert-butyl methyl ether was used to extract prexasertib from mouse plasma and Ringer's/BSA. Read More

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http://dx.doi.org/10.1016/j.jpba.2018.04.018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5984718PMC
July 2018
4 Reads

The long non-coding RNA promotes KAP1-dependent chromatin changes and regulates olfactory bulb neurogenesis.

EMBO J 2018 05 16;37(10). Epub 2018 Apr 16.

Department of Biology and Biochemistry, University of Bath, Bath, UK

Many long non-coding RNAs (lncRNAs) are expressed during central nervous system (CNS) development, yet their roles and mechanisms of action remain poorly understood. , a CNS-expressed lncRNA, controls neuroblastoma cell growth by binding and modulating the activity of transcriptional regulatory elements in a genome-wide manner. We show here that the lncRNA directly binds KAP1, an essential epigenetic regulatory protein, and thereby regulates the expression of shared target genes important for proliferation and neuronal differentiation. Read More

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http://dx.doi.org/10.15252/embj.201798219DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5978383PMC
May 2018
5 Reads

The association of NF2 (neurofibromin 2) gene polymorphism and the risk of medulloblastomas.

Neurol Sci 2018 Jul 10;39(7):1175-1183. Epub 2018 Apr 10.

Department of Neurology, Shenzhen Children's Hospital, Shenzhen, No. 7019, Yitian Road, Shenzhen, 518038, China.

To explore the relationship between NF2 promoter gene mutation and the risk of medulloblastomas (MBs). We collected tissues from 16 MB patients and 7 age-matched non-MB controls. Gene sequencing, qPCR (real-time quantitative polymerase chain reaction), IHC (immunohistochemistry), and WB (Western blot) were used to analyze the changes in the NF2 gene sequence and expression between patients and controls. Read More

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http://dx.doi.org/10.1007/s10072-018-3327-0DOI Listing
July 2018
3 Reads

Reduced hydroxymethylation characterizes medulloblastoma while TET and IDH genes are differentially expressed within molecular subgroups.

J Neurooncol 2018 Aug 26;139(1):33-42. Epub 2018 Mar 26.

Department of Paediatrics, University of São Paulo, 3900, Bandeirantes Avenue, Ribeirão Preto, SP, 14049-900, Brazil.

Introduction: Medulloblastoma (MB) is an embryonal tumour that originates from genetic deregulation of cerebellar developmental pathways and is classified into 4 molecular subgroups: SHH, WNT, group 3, and group 4. Hydroxymethylation levels progressively increases during cerebellum development suggesting a possibility of deregulation in MB pathogenesis. The aim of this study was to investigate global hydroxymethylation levels and changes in TET and IDH gene expression in MB samples compared to control cerebellum samples. Read More

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http://dx.doi.org/10.1007/s11060-018-2845-1DOI Listing
August 2018
3 Reads

Primary cervical ganglioneuroblastoma: A case report.

Medicine (Baltimore) 2018 Mar;97(12):e0090

Department of Otorhinolaryngology, Head & Neck Surgery, West China Hospital.

Rationale: Ganglioneuroblastoma is usually located in the adrenal gland, retroperitoneal ganglia, or posterior mediastinum, but rarely occurs in the parapharyngeal space.

Patient Concerns: A 4-year-old girl presented with complaint of progressive inspiratory dyspnea and dysphagia, accompanying left-side Horner's syndrome.

Diagnose: Computed tomography (CT) scan revealed a giant mass with irregular low density in left oropharyngeal and posterior pharyngeal wall. Read More

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http://dx.doi.org/10.1097/MD.0000000000010090DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5895351PMC
March 2018
7 Reads

Lateral cerebellum is preferentially sensitive to high sonic hedgehog signaling and medulloblastoma formation.

Proc Natl Acad Sci U S A 2018 03 12;115(13):3392-3397. Epub 2018 Mar 12.

Developmental Biology Program, Sloan Kettering Institute, New York, NY 10065;

The main cell of origin of the Sonic hedgehog (SHH) subgroup of medulloblastoma (MB) is granule cell precursors (GCPs), a SHH-dependent transient amplifying population in the developing cerebellum. SHH-MBs can be further subdivided based on molecular and clinical parameters, as well as location because SHH-MBs occur preferentially in the lateral cerebellum (hemispheres). Our analysis of adult patient data suggests that tumors with Smoothened () mutations form more specifically in the hemispheres than those with Patched 1 () mutations. Read More

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http://dx.doi.org/10.1073/pnas.1717815115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5879676PMC
March 2018
44 Reads

Embryonal Tumors of the Central Nervous System: From the Radiologic Pathology Archives.

Radiographics 2018 Mar-Apr;38(2):525-541

From the Department of Neuroradiology, American Institute for Radiologic Pathology, Silver Spring, Md (R.Y.S., K.K.K.); Uniformed Services University of the Health Sciences, Bethesda, Md (R.Y.S.); Department of Radiology, Walter Reed National Military Medical Center, Bethesda, Md (R.Y.S.); and Department of Radiology, Mayo Clinic, 200 First St SW, Rochester, MN 55905 (K.K.K.).

Embryonal tumors of the central nervous system (CNS) are highly malignant undifferentiated or poorly differentiated tumors of neuroepithelial origin and have been defined as a category in the World Health Organization (WHO) classification since the first edition of the "Blue Book" in 1979. This category has evolved over time to reflect our ever-improving understanding of tumor biology and behavior. With the most recent update in 2016, many previous histologic diagnoses incorporate molecular parameters for the first time (genetically defined entities). Read More

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http://dx.doi.org/10.1148/rg.2018170182DOI Listing
August 2018
56 Reads

Brain cancer genomics and epigenomics.

Handb Clin Neurol 2018 ;148:785-797

Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States; Broad Institute of Harvard and MIT, Cambridge, MA, United States. Electronic address:

Classically, brain cancers have been graded and diagnosed based on histology and risk stratified by clinical criteria. Recent advances in genomics and epigenomics have ushered in an era of defining cancers based on molecular criteria. These advances have increased our precision of identifying oncogenic driving events and, most importantly, increased our precision at predicting clinical outcome. Read More

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http://dx.doi.org/10.1016/B978-0-444-64076-5.00050-8DOI Listing
August 2018
4 Reads

Treatment of pediatric esthesioneuroblastoma with smell preservation.

Auris Nasus Larynx 2018 Oct 21;45(5):1107-1112. Epub 2018 Feb 21.

Clinic of Otolaryngology, Head and Neck Surgery, Department of Translational Surgery and Medicine, University of Florence, Italy.

Olfactory neuroblastoma is a rare malignant tumor of neuroectodermal origin and represents the most common cancer of the nasal cavity in pediatric age. The gold standard of treatment consists of en bloc resection, numerous studies have shown as the endoscopic approaches permit good control of the disease improving the quality of life after the treatment. Herein we describe the case of a 13-year-old patient referred to our outpatient clinic with a polypoid multi-lobed lesion occupying the left nasal cavity and imaging that confirmed a left-sided nasal mass without cribriform plate involvement (Kadish B). Read More

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http://dx.doi.org/10.1016/j.anl.2018.02.003DOI Listing
October 2018
6 Reads

Tau protein liquid-liquid phase separation can initiate tau aggregation.

EMBO J 2018 04 22;37(7). Epub 2018 Feb 22.

Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Charlestown, MA, USA

The transition between soluble intrinsically disordered tau protein and aggregated tau in neurofibrillary tangles in Alzheimer's disease is unknown. Here, we propose that soluble tau species can undergo liquid-liquid phase separation (LLPS) under cellular conditions and that phase-separated tau droplets can serve as an intermediate toward tau aggregate formation. We demonstrate that phosphorylated or mutant aggregation prone recombinant tau undergoes LLPS, as does high molecular weight soluble phospho-tau isolated from human Alzheimer brain. Read More

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http://dx.doi.org/10.15252/embj.201798049DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5881631PMC
April 2018
16 Reads

Definitive re-irradiation using intensity-modulated radiation therapy in cancers of the head and neck, focusing on rare tumors.

Acta Otolaryngol 2018 Aug 22;138(8):750-758. Epub 2018 Feb 22.

a Miyakojima IGRT Clinic , Miyakojimahondori, Miyakojima-ku , Osaka , Japan.

Purpose: To analyze the outcomes following re-irradiation for local recurrence of rare head and neck tumors.

Material And Methods: We retrospectively analyzed 11 patients who had received intensity-modulated radiation therapy (IMRT) for recurrent tumors in the head and neck except for laryngopharynx.

Results: Primary tumor sites included the maxillary sinus, nasal cavity, and external ear canal in six, three, and two patients, respectively. Read More

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http://dx.doi.org/10.1080/00016489.2018.1438662DOI Listing
August 2018
6 Reads