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Acta Neurochir (Wien) 2019 Apr 13. Epub 2019 Apr 13.

Department of Neurosurgery, University Hospital Münster, Germany Albert-Schweitzer-Campus 1, Building A1, 48149, Munster, Germany.

Background: 5-Aminolevulinic acid (5-ALA)-guided resection of gliomas in adults enables better differentiation between tumor and normal brain tissue, allowing a higher degree of resection, and improves patient outcomes. In recent years, several reports have emerged regarding the use of 5-ALA in other brain tumor entities, including pediatric brains tumors. Since gross total resection (GTR) of many brain tumors in children is crucial and the role of 5-ALA-guided resection of these tumors is not clear, we sought to perform a comprehensive literature review on this topic. Read More

Medicine (Baltimore) 2019 Apr;98(14):e15019

Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, Sichuan, P. R. China.

Rationale: Cerebellopontine angle (CPA) ependymomas are atypical kind of ependymomas that characteristically occur in the pediatric age group. Therefore, finding a case of CPA ependymoma in a young male adult is not a common occurrence.

Patient Concerns: We present a case of a 28-year-old male who was involved in road traffic accident with suspected mild head injury. Read More

Asian J Neurosurg 2019 Jan-Mar;14(1):275-279

Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, Bengaluru, Karnataka, India.

It is often intriguing to suspect and confirm the diagnosis of primary malignant melanoma (PMM) in the brain without any evidence of neurocutaneous melanosis. We report a 16-year-old male patient with malignant melanoma which intraoperatively was small sized, soft, fleshy, hemorrhagic in appearance resembling hematoma. Interestingly, the histopathology showed prominent papillary architecture with a differential diagnosis of papillary meningioma and ependymoma and perplexed the tissue diagnosis. Read More

Brain Tumor Pathol 2019 Mar 30. Epub 2019 Mar 30.

Department of Neuro-Oncology/Neurosurgery, Saitama Medical University International Medical Center, Saitama, Japan.

We focused on histological and immunohistochemical characteristics of ependymoma (EPN) with molecular profiles to develop more reproducible criteria of the diagnosis. Three expert neuropathologists reviewed the pathology of 130 samples from the Japan Pediatric Molecular Neuro-Oncology Group study. Confirmed cases were assessed for histology, surrogate markers, molecular subgrouping, and survival data. Read More

Neuro Oncol 2019 Mar;21(4):537-546

Division of Hematology/Oncology, Ann & Robert H. Lurie Children's Hospital of Chicago and Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.

Background: Gene-mediated cytotoxic immunotherapy (GMCI) is a tumor-specific immune stimulatory strategy implemented through local delivery of aglatimagene besadenovec (AdV-tk) followed by anti-herpetic prodrug. GMCI induces T-cell dependent tumor immunity and synergizes with radiotherapy. Clinical trials in adult malignant gliomas demonstrated safety and potential efficacy. Read More

Oncologist 2019 Mar 8. Epub 2019 Mar 8.

Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

Background: Data on frequency, clinical presentation, and outcome of primary metastatic intracranial ependymoma in children are scarce.

Patients And Methods: Prospective data on patients younger than 21 years with metastatic intracranial ependymoma at first diagnosis, registered from 2001 to 2014 in the HIT-2000 trial and the HIT-2000 Interim Registry, were analyzed.

Results: Of 453 registered patients with intracranial ependymoma and central neuropathology review, initial staging included spinal magnetic resonance imaging in all patients and lumbar cerebrospinal fluid (CSF) analysis in 402 patients. Read More

J Neurosurg Pediatr 2019 Mar;23(3):261-273

Brain tumors are the most common solid tumors in children, and, unfortunately, many subtypes continue to have a suboptimal long-term outcome. During the last several years, however, remarkable advances in our understanding of the molecular underpinnings of these tumors have occurred as a result of high-resolution genomic, epigenetic, and transcriptomic profiling, which have provided insights for improved tumor categorization and molecularly directed therapies. While tumors such as medulloblastomas have been historically grouped into standard- and high-risk categories, it is now recognized that these tumors encompass four or more molecular subsets with distinct clinical and molecular characteristics. Read More

Radiother Oncol 2019 Mar 20;132:1-7. Epub 2018 Dec 20.

Oncology Radiotherapy Institut de Cancérologie de Lorraine, Vandoeuvre les Nancy, France.

Purpose: This study aimed to evaluate retrospectively the clinical results of re-irradiation for children with a locally recurrent brain ependymoma.

Methods: 33 full-dose re-irradiations were delivered to 31 children with a recurrent brain ependymoma after a standard treatment. Each child was followed up with clinical and MRI examinations. Read More

Wiad Lek 2019;72(1):129-123

Department of Nervous Diseases with Neurosurgery and Medical Genetics, Ukrainian Medical Stomatological Academy, Poltava, Ukraine.

Objective: Introduction: The article describes a clinical case of a malignant tumor of the brain and spinal cord with metastasis, which remained undetected for many years and was treated as syringomyelia. Long-term exhausting examinations of the brain and spinal cord, dynamic follow-up of medical specialists, and repeated surgical interventions on the spine helped to differentiate this process and make the correct diagnosis. The aim: The objectives of the present paper are to analyze the existing classifications of syringomyelia; to examine its etiology, pathogenesis, diagnostic approaches and treatment tactics; to present a clinical case of a malignant tumor of the brain and spinal cord with metastasis, which was misdiagnosed as syringomyelia. Read More

Acta Neuropathol Commun 2019 Feb 20;7(1):24. Epub 2019 Feb 20.

Division of Neuropathology, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square, London, WC1N 3BG, UK.

The introduction of the classification of brain tumours based on their DNA methylation profile has significantly changed the diagnostic approach for cases with ambiguous histology, non-informative or contradictory molecular profiles or for entities where methylation profiling provides useful information for patient risk stratification, for example in medulloblastoma and ependymoma. We present our experience that combines a conventional molecular diagnostic approach with the complementary use of a DNA methylation-based classification tool, for adult brain tumours originating from local as well as national referrals. We report the frequency of IDH mutations in a large cohort of nearly 1550 patients, EGFR amplifications in almost 1900 IDH-wildtype glioblastomas, and histone mutations in 70 adult gliomas. Read More

Cancer 2019 Feb 15. Epub 2019 Feb 15.

Division of Haematology/Oncology, Hospital for Sick Children, Toronto, Ontario, Canada.

Background: Posterior fossa ependymoma (PFE) comprises 2 groups, PF group A (PFA) and PF group B (PFB), with stark differences in outcome. However, to the authors' knowledge, the long-term outcomes of PFA ependymoma have not been described fully. The objective of the current study was to identify predictors of survival and neurocognitive outcome in a large consecutive cohort of subgrouped patients with PFE over 30 years. Read More

BMC Bioinformatics 2019 Jan 31;20(1):60. Epub 2019 Jan 31.

Department of Medicine, Division of Medical Genetics, University of California San Diego (UCSD), San Diego, USA.

Background: High-throughput technologies for analyzing chromosome conformation at a genome scale have revealed that chromatin is organized in topologically associated domains (TADs). While TADs are relatively stable across cell types, intra-TAD activities are cell type specific. Epigenetic profiling of different tissues and cell-types has identified a large number of non-coding epigenetic regulatory elements ('enhancers') that can be located far away from coding genes. Read More

Pediatr Neurosurg 2019 30;54(2):98-107. Epub 2019 Jan 30.

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China,

Objective: The purpose of this study was to explore the clinical features and risk factors of outcomes in pediatric posterior cranial fossa ependymoma. We aim to provide evidence-based recommendations for the improvement of prognoses.

Patients And Methods: The clinical data, treatment modalities, approaches performed, recurrence rates and times, as well as the outcomes of 94 cases were analyzed retrospectively. Read More

Acta Neurochir (Wien) 2019 Mar 29;161(3):535-543. Epub 2019 Jan 29.

Department of Neurosurgery, Goethe University Hospital, Schleusenweg 2-16, 60528, Frankfurt am Main, Germany.

Background: Data about the influence of pregnancy on progression-free survival and overall survival of glioma patients are sparse and controversial. We aimed at providing further evidence on this relation.

Methods: The course of 18 glioma patients giving birth to 23 children after tumor surgery was reviewed and compared to the course of 18 nulliparous female patients matched for tumor diagnosis including molecular markers, extent of resection, and tumor location. Read More

J Pediatr Hematol Oncol 2018 Oct 23. Epub 2018 Oct 23.

Departments of Neurosciences.

Successful use of immune checkpoint inhibitors in a variety of cancers has generated interest in using this approach in pediatric brain tumors. We performed a retrospective review of 10 consecutive children (6 boys, 4 girls; ages, 2 to 17 y), with recurrent or refractory pediatric brain tumors (5 high-grade glioma, 1 low-grade glioma, pineoblastoma, medulloblastoma, ependymoma, and CNS embryonal tumor, NOS) treated at Rady Children's Hospital San Diego from 2015 to 2017 with the immune checkpoint inhibitor nivolumab (3 mg/kg every 2 wk). Eight of 10 patients received prior chemotherapy and 9 radiation therapy. Read More

Turk Neurosurg 2018 06 30. Epub 2018 Jun 30.

The First Hospital of Jilin University.

Aim: Objective: To identify ependymoma (EPN) driver genes and key pathways, and also to illuminate the connection between prognosis of EPN patients and expression levels of driver genes.

Material And Methods: Methods: The gene expression profiles of GSE50161, GSE66354, GSE74195, and GSE86574 were analyzed to figure out the differentially expressed genes (DEGs) between tissue of EPN and normal brain samples. To harvest the enrichment functions, pathways and hub genes, the Gene ontology (GO), Kyoto Encyclopedia of Genes and Genomes (KEGG) analysis, and protein-protein interaction (PPI) network analysis were made. Read More

Childs Nerv Syst 2019 Apr 10;35(4):689-694. Epub 2019 Jan 10.

Departments of Pediatrics, Ribeirão Preto Medical of School, University of São Paulo, Ribeirão Preto, SP, Brazil.

Background: Ependymoma (EPN) is the third most common central nervous system tumor in childhood. Recent advances in the molecular classification of EPN revealed a supratentorial (ST) ependymoma subgroup characterized by C11orf95-RELA fusion.

Case Report: We describe a novel RELA-fusion composed by a chimeric transcript C11orf95-LOC-RELA in a supratentorial WHO grade II EPN occurring in a 4-year-old child. Read More

Neuro Oncol 2019 Jan 8. Epub 2019 Jan 8.

Department of Radiation Oncology, St. Jude Children's Research Hospital.

Background: Pediatric patients with brain tumors who are treated with radiation therapy (RT) are at risk for neurocognitive and psychosocial late effects. Research to date has primarily examined these outcomes at a group level and in isolation. Advanced statistical techniques allow for person-centered analyses, as well as examination of relationships between domain-specific trajectories. Read More

Oncotarget 2018 Nov 23;9(92):36530-36541. Epub 2018 Nov 23.

Children's Brain Tumour Research Centre, School of Medicine, University of Nottingham, Nottingham, UK.

Background: Epigenetic modifications have been shown to play an important role in the classification and pathogenesis of the pediatric brain tumor ependymoma, suggesting they are a potential therapeutic target.

Results: Agents targeting epigenetic modifications inhibited the growth and induced the death of ependymoma cells with variable efficiency. However, this was often not at clinically achievable doses. Read More

Childs Nerv Syst 2019 Mar 15;35(3):411-420. Epub 2018 Dec 15.

Children's Brain Tumour Research Centre, Academic Division of Child Health, Queen's Medical Centre, University of Nottingham, Nottingham, NG7 2RD, UK.

Purpose: This is the first UK multi-centre case-controlled study with follow-up in excess of 10 years to report the neurocognitive, academic and psychological outcomes of individuals diagnosed with a brain tumour in early childhood. Children enrolled into the UKCCSG CNS 9204 trial, diagnosed with intracranial ependymoma when aged ≤ 36 months old, who received a primary chemotherapy strategy to defer or avoid radiotherapy, were recruited.

Methods: Outcomes of those who relapsed and subsequently received radiotherapy (n = 13) were compared to those enrolled who did not relapse (n = 16), age-matched controls-diagnosed with solid non-central nervous system (SN-CNS; n = 15) tumours or low-grade posterior fossa pilocytic astrocytoma (PFPA; n = 15), and normative data. Read More

Childs Nerv Syst 2019 Feb 4;35(2):267-275. Epub 2018 Dec 4.

Department of Radiotherapy, The Maria Sklodowska-Curie Institute - Oncology Center, Gliwice Branch, Ul. Wybrzeże AK 15, 44-101, Gliwice, Poland.

Purpose: To assess the results and tolerance of radiosurgery/hypofractionated stereotactic radiotherapy performed after craniospinal irradiation for recurrent tumor.

Methods: Fourteen patients aged 3-46 years, diagnosed with medulloblastoma (10), anaplastic ependymoma (3), and primitive neuroectodermal tumor (1). All patients had craniospinal irradiation (CSI) with the total dose of 30. Read More

Acta Neuropathol Commun 2018 12 4;6(1):134. Epub 2018 Dec 4.

Division of Brain Tumor Translational Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.

Extensive molecular analyses of ependymal tumors have revealed that supratentorial and posterior fossa ependymomas have distinct molecular profiles and are likely to be different diseases. The presence of C11orf95-RELA fusion genes in a subset of supratentorial ependymomas (ST-EPN) indicated the existence of molecular subgroups. However, the pathogenesis of RELA fusion-negative ependymomas remains elusive. Read More

Surg Neurol Int 2018 23;9:211. Epub 2018 Oct 23.

Department of Anesthesiology, Advocate Illinois Masonic Medical Center, Chicago, Illinois, USA.

Background: Ependymomas represent 50-60% of all brain and central nervous system tumors. Previous lumbar spine surgery for resection of an ependymoma should not be considered a relative contraindication for the administration of epidural/subarachnoid anesthesia to patients in labor.

Case Description: A 34-year-old G1P0, who underwent resection of an L1-L3 intramedullary ependymoma 8 years previously, presented in active labor with residual left leg numbness and tingling. Read More

J Neurooncol 2019 Jan 20;141(2):449-457. Epub 2018 Nov 20.

Developmental and Stem Cell Biology Program, The Arthur and Sonia Labatt Brain Tumour Center, Division of Neurosurgery, Hospital for Sick children, Toronto, ON, Canada.

Background: DNA methylation inhibitors are logical therapeutic candidates for ependymomas originating in the posterior fossa of the brain. Our objective was to test the safety of infusing 5-Azacytidine (5-AZA), a DNA methylation inhibitor, directly into cerebrospinal fluid (CSF) spaces of the fourth ventricle or tumor resection cavity in children with recurrent ependymoma originating in the posterior fossa.

Materials And Methods: In patients with recurrent ependymoma whose disease originated in the posterior fossa, a maximal safe subtotal tumor resection was performed. Read More

Med J Armed Forces India 2018 Oct 5;74(4):407-409. Epub 2017 Oct 5.

Classified Specialist (ENT), Command Hospital (Central Command), Lucknow, India.

Neurofibromatosis is a neurocutaneous, tumor predisposing, inheritable disorder characterized by tumors of the brain and spine and the presence of skin lesions. The most important tumors associated with neurofibromatosis are vestibular nerve schwannomas, with others being meningiomas and ependymomas. The cord is also affected by tumors, ependymoma being the commonest, besides meningiomas and schwannomas. Read More

World Neurosurg 2019 Feb 16;122:e1300-e1304. Epub 2018 Nov 16.

Department of Radiology, Stanford University School of Medicine, Stanford, California, USA.

Background: Current standard of care for children with infratentorial ependymoma includes maximal safe resection and local radiation of 54-59 Gray. High-dose local radiation has been associated with declines in multiple cognitive domains. The anatomic and physiologic correlates of this cognitive decline remain undefined, and there have been no radiographic studies on the long-term effects of this treatment paradigm. Read More

BMJ 2018 Nov 16;363:k4844. Epub 2018 Nov 16.

University of Kent, Chatham Maritime, Kent, UK.

Brain Pathol 2019 01;29(1):75-84

Department of Pathology, University of California, San Francisco, CA.

Myxopapillary ependymomas (MPE) are considered benign (World Health Organization (WHO) grade I) neoplasms with favorable prognosis. However, malignant behavior occurs in a small subset. To our knowledge, only five anaplastic MPEs have been reported without consensus on diagnostic criteria. Read More

J Clin Neurosci 2019 Jan 6;59:310-312. Epub 2018 Nov 6.

Department of Pathology and Molecular Medicine/Neuropathology, McMaster University, Hamilton, Ontario, Canada. Electronic address:

The diagnosis and management of ependymoma may be challenging when there is a comorbidity of ependymoma and Parkinson's disease (PD). We report the first case to demonstrate unusual clinical and pathological features of an ependymoma associated with PD. A 77-year-old male with a history of PD had brain magnetic resonance imaging (MRI) that showed signal abnormalities in the right temporal lobe, most consistent with a low-grade glioma. Read More

Cureus 2018 Jul 18;10(7):e3000. Epub 2018 Jul 18.

Department of Pathology, Osceola Regional Medical Center, Orlando, USA.

Medulloblastoma is the most common type of aggressive pediatric primary brain malignancy. This case describes a 45-year-old Hispanic male with no significant past medical history who presented to the emergency department (ED) complaining of 15 days of 10/10 intractable headaches with one day of lightheadedness, confusion, and loss of balance. An urgent magnetic resonance imaging (MRI) of the brain revealed a 4. Read More

Clin Oncol (R Coll Radiol) 2019 Mar 29;31(3):191-198. Epub 2018 Oct 29.

Radiation Medicine Program, Princess Margaret Cancer Centre, University Health Network, Toronto, Canada; Department of Radiation Oncology, University of Toronto, Toronto, Canada.

Despite best available therapy, many children with cancer develop recurrence after multimodal treatment, including initial radiation therapy. Re-irradiation is defined as the use of a second course of radiation therapy with a retreatment volume that overlaps substantially with that of a previously delivered course of radiation therapy. Re-irradiation is an important part of salvage treatment for patients with recurrent ependymoma, diffuse intrinsic pontine glioma, medulloblastoma and germinoma. Read More

World Neurosurg 2019 Feb 25;122:71-76. Epub 2018 Oct 25.

Department of Neurology and Neurosurgery, Montreal Neurological Institute and Hospital, McGill University, Montreal, Quebec, Canada. Electronic address:

Background: The occurrence of isolated fourth ventricle and injury to the Guillain-Mollaret triangle in the setting of posterior fossa ependymoma represents a new association. In this case report, we discuss the clinical, theoretical, and therapeutic aspects of this problem. We describe a lateral transcerebellar trajectory and shunt valve configuration for safe fourth ventricle shunting in a patient with prior posterior fossa surgery. Read More

World Neurosurg 2019 Feb 18;122:e427-e435. Epub 2018 Oct 18.

Department of Neurosurgery, IRCCS National Cancer Institute "Regina Elena", Rome, Italy.

Objective: To assess usefulness and limitations of flexible fiber carbon dioxide (CO) laser in the microsurgical treatment of intraventricular tumors.

Methods: We reviewed a series of 9 patients treated with microsurgical resection of intraventricular tumors using a flexible fiber CO laser. The lesions involved the third ventricle (8) and the frontal horn of the right lateral ventricle (1). Read More

Brain Pathol 2019 May 28;29(3):325-335. Epub 2018 Nov 28.

Department of Neuropathology, Sainte-Anne Hospital, Paris, France.

Ependymoma with RELA fusion has been defined as a novel entity of the revised World Health Organization 2016 classification of tumors of the central nervous system (CNS), characterized by fusion transcripts of the RELA gene and consequent pathological activation of the NFkB pathway. These tumors represent the majority of supratentorial ependymomas in children. The validation of diagnostic tools to identify this clinically relevant ependymoma entity is essential. Read More

Front Pharmacol 2018 27;9:00950. Epub 2018 Sep 27.

Department of Pediatric Oncology, La Timone Children's Hospital, Assistance Publique Hôpitaux de Marseille, Marseille, France.

Metronomic chemotherapy (MC) is defined as the frequent administration of chemotherapy at doses below the maximal tolerated dose and with no prolonged drug-free break. MC has shown its efficacy in adult tumor types such as breast and ovarian cancer and has to some extent been studied in pediatrics. To assess the anti-tumor activity and toxicity of a four-drug metronomic regimen in relapsing/refractory pediatric brain tumors (BT) with progression-free survival (PFS) after two cycles as primary endpoint. Read More

J Korean Med Sci 2018 Oct 7;33(41):e260. Epub 2018 Sep 7.

Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Background: By estimating the survival rates and exploring prognostic factors in pediatric patients with relapsed or progressed solid tumors, our purpose was to generate background data for future studies.

Methods: We reviewed the medical records of 258 patients with solid tumors who experienced relapse/progression and received subsequent salvage treatment between 1996 and 2016.

Results: A total of 60 patients remained progression-free during first-line salvage treatment, while the remaining 198 patients experienced relapse/progression again; 149 underwent second-line salvage treatment. Read More

Surg Neurol Int 2018 10;9:186. Epub 2018 Sep 10.

International Center for Neurosurgery, Henan Provincial People's Hospital, Zhengzhou, China.

Background: In this video abstract, we present an unedited microsurgical resection of a fourth ventricular ependymoma performed by a senior author (JH). Currently, the goal of a standard treatment of a fourth ventricular ependymoma is based on microsurgical resection followed by radiochemotherapy. Our aim is to demonstrate the efficiency and safety of our microsurgical technique in deep brain territories under the principle "simple, clean, and preserving the normal anatomy. Read More

Cancer 2018 Nov 26;124(21):4168-4180. Epub 2018 Sep 26.

Division of Neuro-Oncology, Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee.

Despite significant improvements in pediatric brain tumor therapy and outcome, too many children still die of disease, and too many survivors experience significant sequelae as a result of conventional therapies. The molecular characterization of pediatric brain tumors has afforded tremendous insight into the basic biology and clinical management of these deadly childhood diseases. Genomic, epigenomic, and transcriptional profiling have facilitated the identification of significant heterogeneity among previously uniform disease entities. Read More

Brain Pathol 2019 03 11;29(2):205-216. Epub 2018 Nov 11.

Institute of Neuropathology, University of Bonn Medical Center, Bonn, Germany.

Ependymoma with YAP1-MAMLD1 fusion is a rare, recently described supratentorial neoplasm of childhood, with few cases published so far. We report on 15 pediatric patients with ependymomas carrying YAP1-MAMLD1 fusions, with their characteristic histopathology, immunophenotype and molecular/cytogenetic, radiological and clinical features. The YAP1-MAMLD1 fusion was documented by RT-PCR/Sanger sequencing, and tumor genomes were studied by molecular inversion probe (MIP) analysis. Read More

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

World Neurosurg 2018 Dec 6;120:125-128. Epub 2018 Sep 6.

Division of Neurosurgery, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Tottori, Japan.

Background: Li-Fraumeni syndrome is a genetic disease that is caused by mutation of the tumor suppressor gene TP53. Patients with this syndrome may develop multiple malignant neoplasms including brain tumors. We herein report the first case of Li-Fraumeni syndrome in which development of supratentorial anaplastic ependymoma led to difficulty in terms of selecting the optimal postoperative therapeutic protocol. Read More

Clin Cancer Res 2018 Dec 31;24(24):6471-6482. Epub 2018 Aug 31.

Knight Cancer Institute, Oregon Health and Sciences University, Portland, Oregon.

Purpose: Gliomas, a genetically heterogeneous group of primary central nervous system tumors, continue to pose a significant clinical challenge. Discovery of chromosomal rearrangements involving kinase genes has enabled precision therapy, and improved outcomes in several malignancies.

Experimental Design: Positing that similar benefit could be accomplished for patients with brain cancer, we evaluated The Cancer Genome Atlas (TCGA) glioblastoma dataset. Read More

Mymensingh Med J 2018 Jul;27(3):665-668

Dr Kohinoor Jahan Shyamaly, Resident Phase-B, Department of General Paediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh.

Primitive neuroectodermal tumors are malignant tumor of pluripotent cells of neural crest. It has diverse clinical presentation and aggressive clinical behaviour. Clinical features may provide some clue but imaging studies such as MRI of brain; tissue histopathology, immune histochemistry and cytogenetic are essential to confirm the diagnosis. Read More

Zh Vopr Neirokhir Im N N Burdenko 2018;82(4):81-86

Burdenko Neurosurgical Institute, Moscow, Russia.

One of the main manifestations of posterior cranial fossa (PCF) tumors is the development of hydrocephalus (HC) symptoms that in most cases are the reason for examination and diagnosis [1, 2]. According to the literature, the rate of shunt surgery after removal of PCF tumors in the children's population is 18-40% [3-9]. Hydrocephalus remaining after removal of PCF tumor and requiring further treatment is called persistent hydrocephalus. Read More

No Shinkei Geka 2018 Aug;46(8):707-711

Department of Neurosurgery, Faculty of Medicine, Yamagata University.

Introduction: The draining veins of the brain stem and cerebellum commonly drain into the petrosal vein and sigmoid sinus, and often drain into the marginal sinus in the caudal part of the posterior fossa. Here, we report a rare case of anaplastic ependymoma involving a bridging vein that drained directly into the occipital sinus.

Case Description: A 6-year-old boy was admitted to our hospital with a 1-month history of nausea, headache, and dizziness. Read More

J Appl Clin Med Phys 2018 Sep 19;19(5):632-639. Epub 2018 Aug 19.

Department of Radiotherapy, Regensburg University Medical Center, Regensburg, Germany.

Pediatric patients suffering from ependymoma are usually treated with cranial or craniospinal three-dimensional (3D) conformal radiotherapy (3DCRT). Intensity-modulated techniques spare dose to the surrounding tissue, but the risk for second malignancies may be increased due to the increase in low-dose volume. The aim of this study is to investigate if the flattening filter free (FFF) mode allows reducing the risk for second malignancies compared to the mode with flattening filter (FF) for intensity-modulated techniques and to 3DCRT. Read More

Pediatr Radiol 2018 12 17;48(13):1955-1963. Epub 2018 Aug 17.

Lysholm Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, London, UK.

Central nervous system neoplasms are the most common solid tumours that develop in children, with the greatest proportion located in the infratentorium. The 2016 World Health Organization Central Nervous System tumour classification evolved from the 2007 edition with the integration of molecular and genetic profiling into the diagnosis, the addition of new entities and the removal of others. Radiology can assist with the subtyping of tumours from certain characteristics described below to provide prognostic information and guide further management. Read More

J Clin Oncol 2018 Oct 17;36(28):2854-2862. Epub 2018 Aug 17.

Ralph E. Vatner, Andrzej Niemierko, Madhusmita Misra, Elizabeth A. Weyman, Claire P. Goebel, David H. Ebb, Robin M. Jones, Mary S. Huang, Takara Stanley, Shannon M. MacDonald, Nancy J. Tarbell, and Torunn I. Yock, Massachusetts General Hospital, Boston, MA; Ralph E. Vatner, University of Cincinnati College of Medicine and Cincinnati Children's Hospital Medical Center, Cincinnati, OH; Anita Mahajan, Mayo Clinic, Rochester, MN; and David R. Grosshans and Arnold C. Paulino, MD Anderson Cancer Center, Houston, TX.

Purpose: There are sparse data defining the dose response of radiation therapy (RT) to the hypothalamus and pituitary in pediatric and young adult patients with brain tumors. We examined the correlation between RT dose to these structures and development of endocrine dysfunction in this population.

Materials And Methods: Dosimetric and clinical data were collected from children and young adults (< 26 years of age) with brain tumors treated with proton RT on three prospective studies (2003 to 2016). Read More