Epidemiology and Survival of Patients with Brainstem Gliomas: a Population-Based Study Using the SEER Database
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Central Nervous System Tumors General ~1% of Tumors in Adults, but ~25% of Malignancies in Children (Only 2Nd to Leukemia)
Last updated: 3/4/2021 Prepared by Kurt Schaberg Central Nervous System Tumors General ~1% of tumors in adults, but ~25% of malignancies in children (only 2nd to leukemia). Significant increase in incidence in primary brain tumors in elderly. Metastases to the brain far outnumber primary CNS tumors→ multiple cerebral tumors. One can develop a very good DDX by just location, age, and imaging. Differential Diagnosis by clinical information: Location Pediatric/Young Adult Older Adult Cerebral/ Ganglioglioma, DNET, PXA, Glioblastoma Multiforme (GBM) Supratentorial Ependymoma, AT/RT Infiltrating Astrocytoma (grades II-III), CNS Embryonal Neoplasms Oligodendroglioma, Metastases, Lymphoma, Infection Cerebellar/ PA, Medulloblastoma, Ependymoma, Metastases, Hemangioblastoma, Infratentorial/ Choroid plexus papilloma, AT/RT Choroid plexus papilloma, Subependymoma Fourth ventricle Brainstem PA, DMG Astrocytoma, Glioblastoma, DMG, Metastases Spinal cord Ependymoma, PA, DMG, MPE, Drop Ependymoma, Astrocytoma, DMG, MPE (filum), (intramedullary) metastases Paraganglioma (filum), Spinal cord Meningioma, Schwannoma, Schwannoma, Meningioma, (extramedullary) Metastases, Melanocytoma/melanoma Melanocytoma/melanoma, MPNST Spinal cord Bone tumor, Meningioma, Abscess, Herniated disk, Lymphoma, Abscess, (extradural) Vascular malformation, Metastases, Extra-axial/Dural/ Leukemia/lymphoma, Ewing Sarcoma, Meningioma, SFT, Metastases, Lymphoma, Leptomeningeal Rhabdomyosarcoma, Disseminated medulloblastoma, DLGNT, Sellar/infundibular Pituitary adenoma, Pituitary adenoma, -
The Role of PET in Supratentorial and Infratentorial Pediatric Brain Tumors
Review The Role of PET in Supratentorial and Infratentorial Pediatric Brain Tumors Angelina Cistaro 1,2 , Domenico Albano 3 , Pierpaolo Alongi 4,* , Riccardo Laudicella 5 , Daniele Antonio Pizzuto 6 , Giuseppe Formica 5, Cinzia Romagnolo 7, Federica Stracuzzi 5, Viviana Frantellizzi 8 , Arnoldo Piccardo 1,2 and Natale Quartuccio 2,9 1 Nuclear Medicine Department, Ospedali Galliera, 16128 Genova, Italy; [email protected] (A.C.); [email protected] (A.P.) 2 AIMN Pediatric Study Group, 20159 Milan, Italy; [email protected] 3 Department of Nuclear Medicine, University of Brescia and Spedali Civili Brescia, 25123 Brescia, Italy; [email protected] 4 Unit of Nuclear Medicine, Fondazione Istituto G. Giglio, 90015 Cefalù, Italy 5 Nuclear Medicine Unit, Department of Biomedical and Dental Sciences and of Morpho-Functional Imaging, A.O.U. Policlinico G. Martino, University of Messina, 98125 Messina, Italy; [email protected] (R.L.); [email protected] (G.F.); [email protected] (F.S.) 6 Department of Nuclear Medicine, University Hospital Zürich, 8091 Zürich, Switzerland; [email protected] 7 Nuclear Medicine Unit, Ospedali Riuniti, Torrette di Ancona, 60126 Ancona, Italy; [email protected] 8 Department of Radiological Sciences, Oncology and Anatomical Pathology, Sapienza University of Rome, 00161 Rome, Italy; [email protected] 9 Nuclear Medicine Unit, A.R.N.A.S. Ospedali Civico, Di Cristina e Benfratelli, 90127 Palermo, Italy * Correspondence: -
Adult Isocitrate Dehydrogenase–Mutant Brainstem Glioma: Illustrative Case
J Neurosurg Case Lessons 1(12):CASE2078, 2021 DOI: 10.3171/CASE2078 Adult isocitrate dehydrogenase–mutant brainstem glioma: illustrative case *Vincent C. Ye, MD,1 Alexander P. Landry, MD,1 Teresa Purzner, MD, PhD,1 Aristotelis Kalyvas, MD,1 Nilesh Mohan, MD,1 Philip J. O’Halloran, MD, PhD,1 Andrew Gao, MD,2 and Gelareh Zadeh, MD, PhD1,3 1Division of Neurosurgery, Department of Surgery, University of Toronto, Toronto, Ontario, Canada; 2Department of Pathology, University Health Network, Toronto, Ontario, Canada; and 3Arthur and Sonia Labatt Brain Tumour Research Center, The Hospital for Sick Children, Toronto, Ontario, Canada BACKGROUND Adult brainstem gliomas are rare entities that demonstrate heterogeneous biology and appear to be distinct from both their pediatric counterparts and adult supratentorial gliomas. Although the role of histone 3 mutations is being increasingly understood in this disease, the effectof isocitrate dehydrogenase (IDH) mutations remains unclear, largely because of limited data. OBSERVATIONS The authors present the case of a 29-year-old male with an IDH1-mutant, World Health Organization grade III anaplastic astrocytoma in the dorsal medulla, and they provide a review of the available literature on adult IDH-mutant brainstem glioma. The authors have amassed a cohort of 15 such patients, 7 of whom have survival data available. Median survival is 56 months in this small cohort, which is similar to that for IDH wild-type adult brainstem gliomas. LESSONS The authors’ work reenforces previous literature suggesting that the role of IDH mutation in glioma differs between brainstem and supratentorial lesions. Therefore, the authors advocate that adult brainstem gliomas be studied in terms of major molecular subgroups (including IDH mutant) because these gliomas may exhibit fundamental differences from each other, from pediatric brainstem gliomas, and from adult supratentorial gliomas. -
Malignant CNS Solid Tumor Rules
Malignant CNS and Peripheral Nerves Equivalent Terms and Definitions C470-C479, C700, C701, C709, C710-C719, C720-C725, C728, C729, C751-C753 (Excludes lymphoma and leukemia M9590 – M9992 and Kaposi sarcoma M9140) Introduction Note 1: This section includes the following primary sites: Peripheral nerves C470-C479; cerebral meninges C700; spinal meninges C701; meninges NOS C709; brain C710-C719; spinal cord C720; cauda equina C721; olfactory nerve C722; optic nerve C723; acoustic nerve C724; cranial nerve NOS C725; overlapping lesion of brain and central nervous system C728; nervous system NOS C729; pituitary gland C751; craniopharyngeal duct C752; pineal gland C753. Note 2: Non-malignant intracranial and CNS tumors have a separate set of rules. Note 3: 2007 MPH Rules and 2018 Solid Tumor Rules are used based on date of diagnosis. • Tumors diagnosed 01/01/2007 through 12/31/2017: Use 2007 MPH Rules • Tumors diagnosed 01/01/2018 and later: Use 2018 Solid Tumor Rules • The original tumor diagnosed before 1/1/2018 and a subsequent tumor diagnosed 1/1/2018 or later in the same primary site: Use the 2018 Solid Tumor Rules. Note 4: There must be a histologic, cytologic, radiographic, or clinical diagnosis of a malignant neoplasm /3. Note 5: Tumors from a number of primary sites metastasize to the brain. Do not use these rules for tumors described as metastases; report metastatic tumors using the rules for that primary site. Note 6: Pilocytic astrocytoma/juvenile pilocytic astrocytoma is reportable in North America as a malignant neoplasm 9421/3. • See the Non-malignant CNS Rules when the primary site is optic nerve and the diagnosis is either optic glioma or pilocytic astrocytoma. -
Recent Advances in the Treatment of Gliomas – Comprehensive Brain Tumor Center
RECENT ADVANCES IN NEUROSURGERY Recent Advances in the Treatment of Gliomas – Comprehensive Brain Tumor Center STEVEN A. TOMS, MD, MPH; NIKOLAOS TAPINOS, MD, PhD ABSTRACT development of electric current loco-regional antimitotic Gliomas are a class of primary brain tumors arising from therapy (“tumor-treating fields”) led to the first reported the supporting structures of the brain, the astrocytes and survivals exceeding 20 months7. oligodendrocytes, which range from benign lesions to In the United States alone, 12,000 new cases of GBM are its most malignant form, the glioblastoma. Treatment diagnosed each year8. One reason cited for the failure to for these lesions includes maximal surgical resection, improve survival has been the presence of a robust blood- radiotherapy, and chemotherapy. Recently, novel thera- brain barrier within the tumor, which impedes delivery of pies such as immune modulatory therapies and electrical traditional cytotoxic and novel molecular therapies9. Most field treatment of the most malignant form, the glioblas- chemotherapeutic agents are hydrophilic, and do not pene- toma, have shown promise in improving survival. We trate the blood brain barrier well. Attempts to deliver che- will review recent advances in clinical trials, explore the motherapeutic molecules into the brain have included both role of multimodal care in brain tumor therapy, as well osmotic, chemical, and ultrasound mediated opening of the as explore advances in molecular biology and nanotech- blood brain barrier to improve drug delivery, but none have nology which offer new hope for treatment of this class improved clinical outcomes10. A novel method to bypass of disease. this barrier, (i.e., convection enhanced delivery), met with KEYWORDS: glioblastoma, immunotherapy, tumor success in delivering high drug concentrations of hydro- treating fields, nanotechnology, drug delivery philic drugs to brain tumors and led to several clinical trials. -
The Historical Change of Brainstem Glioma Diagnosis and Treatment
Zhang et al. Chinese Neurosurgical Journal (2015) 1:4 DOI 10.1186/s41016-015-0006-3 REVIEW Open Access The historical change of brainstem glioma diagnosis and treatment: from imaging to molecular pathology and then molecular imaging Liwei Zhang1,2,3*, Chang-cun Pan1,2,3 and Deling Li1,2 Abstract Understanding a process from shallow to deep is necessary for controlling and even curing diseases. The history of diagnosis and treatment of brainstem gliomas vividly reflects this process. The development of neuroimaging plays a great role in tumor treatment at different periods, including the period when brainstem gliomas were regarded as an homogenous incurable disease, and currently it is considered as an entity with high heterogeneity. Presently, it is not enough to just rely on the conventional neuroimaging techniques to determine the anatomic location of a tumor and its relationship with normal tissues. The development of molecular genetics and molecular imaging further promotes the progress of individualized and precision diagnosis and treatment in brainstem gliomas. In this paper, we summarize the evolution of brainstem glioma radiological classification mainly focusing on the aspects of imaging and surgical treatment. In the meanwhile, we reviewed the recent progresses in the fields of molecular genetics and molecular imaging. Keywords: Brainstem gliomas, Microsurgery, Radiological classification, Molecular genetics, Molecular imaging Introduction caused by hydrocephalus and failure to thrive. After total The breakthrough of the “no man’s land” resection, patients can achieve a relatively long survival In the 1960s, the brainstem was still the forbidden region without routine postoperative radiotherapy, and most re- for surgery, and the mortality rate of operation on brain- sidual tumors are stable. -
Primary Intracranial Neoplasms in Man Comprise from 2 to 5 Per Cent of All Tumors in the Body
THE AMERICAN JOURNAL OF PATHOLOGY VOLUME XXXI JANUARY-FEBRUARY, I955 NUMBER x THE NATURE OF GLIOMAS AS REVEALED BY ANIMAL EXPERIMENTATION * H. M. ZnMMERMAN, M.D. From the Laboratory Division, Montej ore Hospital, New York 67, N.Y. Primary intracranial neoplasms in man comprise from 2 to 5 per cent of all tumors in the body. Of these neoplasms, somewhat less than half belong to the glioma group. The latter comprises at least seven universally recognized, distinctive types as well as a number of related subtypes. The major types are: astrocytoma, astroblas- toma, ependymoma, gioblastoma multiforme, medulloblastoma, oligo- dendroglioma, and spongioblastoma polare. To the subtypes belong, among others, the ependymoblastomas, ganglioneuromas, and me- dullo-epitheliomas. The problem of identification and classification of tumors of the glioma variety has not been simple, mainly for two reasons. One is that neurosurgical pathology is still in that early developmental stage which is preoccupied with descriptive morphology and with finding new tumor types to classify. Thus it is still considered somewhat of an achievement to have divided the astrocytoma into the piloid, fibril- lary, and protoplasmic types, even though the histogenesis and biologic behavior of this tumor does not seem to warrant such subclassification. The other is that a vastly complicated terminology has developed which has greatly discouraged students in this field. The concept is also current that a battery of complicated and difficult staining techniques is essential for the identification of the various gliomas. The general pathologist has shunned the field of neurosurgical pathology because of his belief in the almost insurmountable complexity of the intra- cranial neoplasms. -
Involvement of the Olfactory Apparatus by Gliomas
CLINICAL REPORT HEAD & NECK Involvement of the Olfactory Apparatus by Gliomas X. Wu, Y. Li, C.M. Glastonbury, and S. Cha ABSTRACT SUMMARY: The olfactory bulbs and tracts are central nervous system white matter tracts maintained by central neuroglia. Although rare, gliomas can originate from and progress to involve the olfactory apparatus. Through a Health Insurance Portability and Accountability Act–compliant retrospective review of the institutional teaching files and brain MR imaging reports spanning 10 years, we identified 12 cases of gliomas involving the olfactory bulbs and tracts, including 6 cases of glioblastoma, 2 cases of ana- plastic oligodendroglioma, and 1 case each of pilocytic astrocytoma, diffuse (grade II) astrocytoma, anaplastic astrocytoma (grade III), and diffuse midline glioma. All except the pilocytic astrocytoma occurred in patients with known primary glial tumors else- where. Imaging findings of olfactory tumor involvement ranged from well-demarcated enhancing masses to ill-defined enhancing infiltrative lesions to nonenhancing masslike FLAIR signal abnormality within the olfactory tracts. Familiarity with the imaging find- ings of glioma involvement of the olfactory nerves is important for timely diagnosis and treatment of recurrent gliomas and to dis- tinguish them from other disease processes. ABBREVIATIONS: GBM ¼ glioblastoma multiforme; TMZ ¼ temozolomide; EGFR ¼ epidermal growth factor receptor; IDH1 ¼ Isocitrate dehydrogenase 1; MGMT ¼ O6-methylguanine methyltransferase he olfactory bulbs and tracts are central nervous system white bulbs and tracts and to differentiate them from other masses of Tmatter tracts extending directly to the cerebrum, maintained the anterior cranial fossa. by a combination of specialized olfactory ensheathing cells and central neuroglia, including astrocytes and oligodendrocytes.1 As Case Series a result, gliomas can rarely originate from and progress to involve – the olfactory apparatus. -
Medulloblastoma Therapy Generates Risk of a Poorly-Prognostic H3 Wild-Type Subgroup of Diffuse Intrinsic Pontine Glioma: a Repor
Gits et al. Acta Neuropathologica Communications (2018) 6:67 https://doi.org/10.1186/s40478-018-0570-9 RESEARCH Open Access Medulloblastoma therapy generates risk of a poorly-prognostic H3 wild-type subgroup of diffuse intrinsic pontine glioma: a report from the International DIPG Registry Hunter C. Gits2, Maia Anderson2, Stefanie Stallard2, Drew Pratt1, Becky Zon2, Christopher Howell3, Chandan Kumar-Sinha1,4, Pankaj Vats1,4, Katayoon Kasaian5, Daniel Polan6, Martha Matuszak6, Daniel E. Spratt6, Marcia Leonard2, Tingting Qin7, Lili Zhao8, James Leach9, Brooklyn Chaney10, Nancy Yanez Escorza10, Jacob Hendershot11, Blaise Jones9, Christine Fuller12, Sarah Leary13, Ute Bartels14, Eric Bouffet14, Torunn I. Yock15, Patricia Robertson16, Rajen Mody2, Sriram Venneti1, Arul M. Chinnaiyan1,4, Maryam Fouladi17, Nicholas G. Gottardo4,18,19 and Carl Koschmann2* Abstract With improved survivorship in medulloblastoma, there has been an increasing incidence of late complications. To date, no studies have specifically addressed the risk of radiation-associated diffuse intrinsic pontine glioma (DIPG) in medulloblastoma survivors. Query of the International DIPG Registry identified six cases of DIPG with a history of medulloblastoma treated with radiotherapy. All patients underwent central radiologic review that confirmed a diagnosis of DIPG. Six additional cases were identified in reports from recent cooperative group medulloblastoma trials (total n = 12; ages 7 to 21 years). From these cases, molecular subgrouping of primary medulloblastomas with available tissue (n = 5) revealed only non-WNT, non-SHH subgroups (group 3 or 4). The estimated cumulative incidence of DIPG after post-treatment medulloblastoma ranged from 0.3–3.9%. Posterior fossa radiation exposure (including brainstem) was greater than 53.0 Gy in all cases with available details. -
Report of the Brain Tumor Progress Review Group
Progress Review Groups Report of the Brain Tumor Progress Review Group November 2000 From the Leadership: It is a great pleasurdfce to submit this Report of the Brain Tumor Progress Review Group (BT- PRG) to the Director and Advisory Committee to the Director of the National Cancer Institute (NCI), and to the Director and National Advisory Neurological Disorders and Stroke Council of the National Institute of Neurological Disorders and Stroke (NINDS). At the beginning of 1999, the BT-PRG accepted the charge of Dr. Richard Klausner, Director of the NCI, and Dr. Gerald Fischbach, Director of the NINDS, to develop a national plan for the next decade of brain tumor research. Although this is the 4th in the series of PRGs, it is the first to be sponsored by 2 institutes, reflecting the importance of both cancer biology and neurobiology to the brain tumor field. The expertise and efficiency of the BT-PRG members and of the participants of the BT- PRG Roundtable Meeting have produced this exciting report in a ten-month period, reflecting the energy and enthusiasm of the clinical, research, industrial and advocacy communities for finding a cure for brain tumors. The Report of the Brain Tumor Progress Review Group highlights the scientific research priorities that represent the next steps toward understanding the biological basis of brain tumors, and toward developing effective therapies for brain tumors. We look forward to discussing these priorities with the leadership of the NCI and NINDS. Respectfully, David N. Louis, M.D. Jerome Posner, M.D. Thomas Jacobs, Ph.D. Richard Kaplan, M.D. -
Central Nervous System Tumors
Central Nervous System Tumors Central Nervous System Tumors Authors: Ayda G. Nambayan, DSN, RN, St. Jude Children’s Research Hospital Erin Gafford, Pediatric Oncology Education Student, St. Jude Children’s Research Hospital; Nursing Student, School of Nursing, Union University Content Reviewed by: Daniel H. Alderete, MD, Hospital Nacional de Pediatría J.P. Garrahan, Argentina Cure4Kids Release Date: 1 September 2006 Central nervous system (CNS) tumors are the second most common malignancy in children and the most common cause of cancer mortality in this population. CNS tumors comprise approximately 17% of all childhood malignancies; and the incidence has steadily increased over the last decade. The most common histologic types of pediatric brain tumors are the gliomas, which arise from the glial tissue of the brain. Gliomas include mainly astrocytomas, ependymomas. The other important group comprises embryonal tumors, such as medulloblastoma and supratentorial primitive neuroectodermal tumors ( PNETs). The diversity of the tumors requires distinct therapeutic strategies and multimodal therapies, which have significant long term residual and late effects. Risk Factors: Though the cause of CNS tumors is largely unknown, the occurrence is associated with certain (A – 1) familial and hereditary syndromes. Other identified risk factors include ionizing radiation (cranial radiation) and environmental agents (industrial and chemical toxins, exposure to paint, solvents and electromagnetic fields). CNS tumors are also associated with other malignancies (embryonal CNS tumors-Atypical teratoid/ rhabdoid tumor- with renal tumors). Classification System: Because of the histologic diversity of CNS tumors, several (A – 2) classification systems have been developed. One widely accepted classification system (Bailey and Cushing) is based on different cell types, their developmental stages, and the corresponding tumors that arise from them. -
Neuroimaging of Pediatric Brain Tumors
Journal of Child Neurology http://jcn.sagepub.com/ Neuroimaging of Pediatric Brain Tumors: From Basic to Advanced Magnetic Resonance Imaging (MRI) Ashok Panigrahy and Stefan Blüml J Child Neurol 2009 24: 1343 DOI: 10.1177/0883073809342129 The online version of this article can be found at: http://jcn.sagepub.com/content/24/11/1343 Published by: http://www.sagepublications.com Additional services and information for Journal of Child Neurology can be found at: Email Alerts: http://jcn.sagepub.com/cgi/alerts Subscriptions: http://jcn.sagepub.com/subscriptions Reprints: http://www.sagepub.com/journalsReprints.nav Permissions: http://www.sagepub.com/journalsPermissions.nav Citations: http://jcn.sagepub.com/content/24/11/1343.refs.html >> Version of Record - Oct 19, 2009 What is This? Downloaded from jcn.sagepub.com at CHILDRENS HOSPITAL on April 14, 2014 Special Issue Article Journal of Child Neurology Volume 24 Number 11 November 2009 1343-1365 # 2009 The Author(s) Neuroimaging of Pediatric Brain Tumors: 10.1177/0883073809342129 http://jcn.sagepub.com From Basic to Advanced Magnetic Resonance Imaging (MRI) Ashok Panigrahy, MD, and Stefan Blu¨ ml, PhD In this review, the basic magnetic resonance concepts used in imaging (MRI) techniques. The second part of this review the imaging approach of a pediatric brain tumor are described will provide an overview of the major advanced MRI tech- with respect to different factors including understanding the niques used in pediatric imaging, particularly, magnetic reso- significance of the patient’s age. Also discussed are other fac- nance diffusion, magnetic resonance spectroscopy, and tors directly related to the magnetic resonance scan itself magnetic resonance perfusion.