Brainstem Gliomas 

  • Author: Joseph Landolfi, DO; Chief Editor: Tarakad S Ramachandran, MBBS, FRCP(C), FACP   more...
 
Updated: Jul 22, 2011
 

Background

Brainstem gliomas are tumors that occur in the region of the brain referred to as the brain stem, which is the area between the aqueduct of Sylvius and the fourth ventricle. Although various systems are used to classify these tumors, the authors have divided brainstem gliomas into 3 distinct anatomic locations—diffuse intrinsic pontine,[1] tectal, and cervicomedullary. Intrinsic pontine gliomas carry a grave prognosis. Longer survival is associated with the tectal and cervicomedullary gliomas. Tumors also are characterized on the basis of site of origin, focality, direction and extent of tumor growth, degree of brainstem enlargement, degree of exophytic growth, and presence or absence of cysts, necrosis, hemorrhage, and hydrocephalus.[2]

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Pathophysiology

These tumors have a predilection to originate from the left side. Most are located in the pons; however, medulla and midbrain may be involved as well. Brainstem gliomas are highly aggressive brain tumors. Anatomic location determines the pathophysiological manifestation of the tumor. With tectal lesions, hydrocephalus may occur as a result of fourth ventricular compression. With pontine and cervicomedullary lesions, cranial nerve or long tract signs are observed commonly.

Histopathologically, brainstem gliomas can range from WHO Grade 1 to 4. Grade 1 is the juvenile pilocytic astrocytoma, Grade 2 is the diffuse astrocytoma, Grade 3 is the anaplastic astrocytoma, and grade 4 is the glioblastoma multiforme. The grading is based on the presence of nuclear atypia, vascular proliferation, mitoses, and necrosis. Typically, the necrosis is seen in Grade 4 (glioblastoma multiforme).

Although biopsy or resection is not typically performed on brainstem gliomas, vascular endothelial growth factor (VEGF) receptors are an important pathway in the invasion and growth of supratentorial glioblastomas by promoting the growth of new blood vessels. Epidermal growth factor receptors (EGFR) are present in 25% of glioblastomas and are important in the growth of these neoplasms as well. The presence of these receptors may aid in the response to various targeted therapies, as is discussed in Medical Care.

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Epidemiology

Frequency

United States

Brainstem gliomas have been reported to make up 2.4% of all intracranial tumors in adults and 9.4% of intracranial tumors in children. Brainstem gliomas account for approximately 10-20% of all childhood brain tumors. The incidence in adults is lower than that in children younger than 16 years. A tendency for brainstem gliomas to follow a more indolent course in adults than in children has been noted; in adults, these tumors are more likely to be low grade and remain localized.

Mortality/Morbidity

  • Morbidity is due to the location of the space-occupying lesion and compression of surrounding structures; because these structures regulate basic body functions of blood pressure, respiration, and swallowing as well as motor and sensory functions, compression can produce substantial neurological disability.
  • Sudden death can result from increased intracranial pressure and subsequent cerebral herniation. This may be a consequence either of edema induced by the tumor or of hemorrhage into the neoplasm.

Race

CNS tumors vary in incidence by age, sex, ethnic group, and country, and also over time. How much of this variation is due to artifactual influences or etiologic differences has been the subject of many debates.

Sex

Some reports have suggested a slight male preponderance, whereas others have failed to observe any sex predilection.

Age

  • Bimodal age distribution has been noted, with a peak incidence in the latter half of the first decade of life and a second peak in the fourth decade.
  • Approximately three fourths of patients are younger than 20 years.
  • Neoplasms of the brain stem have been identified in children younger than 1 year.
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Contributor Information and Disclosures
Author

Joseph Landolfi, DO  Director of Neuro-oncology, New Jersey Neuroscience Institute Brain Tumor Center; Medical Director, Gamma Knife Program, JFK Medical Center; Associate Professor of Neurology, Seton Hall University School of Graduate Medical Education

Joseph Landolfi, DO is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Schering-Plough Honoraria Speaking and teaching; Genetech Honoraria Speaking and teaching

Coauthor(s)

Anita Venkataramana, MBBS  Clinical Instructor, Department of Neurology, Division of Neuroimmunology/HIV, Johns Hopkins University

Disclosure: Nothing to disclose.

Specialty Editor Board

Edward L Hogan, MD  Professor, Department of Neurology, Medical College of Georgia; Emeritus Professor and Chair, Department of Neurology, Medical University of South Carolina

Edward L Hogan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Neurological Association, American Society for Biochemistry and Molecular Biology, Phi Beta Kappa, Sigma Xi, Society for Neuroscience, and Southern Clinical Neurological Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Jorge Kattah, MD  Head, Program Director, Professor, Department of Neurology, University of Illinois College of Medicine at Peoria

Jorge Kattah, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, and New York Academy of Sciences

Disclosure: Biogen Honoraria Consulting; Bayer Corporation Honoraria Consulting

Chief Editor

Tarakad S Ramachandran, MBBS, FRCP(C), FACP  Professor of Neurology, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Chair, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, FRCP(C), FACP is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners, American College of International Physicians, American College of Managed Care Medicine, American College of Physicians, American Heart Association, American Stroke Association, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, and Royal Society of Medicine

Disclosure: Abbott Labs None None; Teva Marion None None; Boeringer-Ingelheim Honoraria Speaking and teaching

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T2-weighted image of a diffuse intrinsic pontine glioma.
T2-weighted image of a right tectal glioma.
 
 
 
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