Updated: Jun 30, 2009
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
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.
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.
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.
Some reports have suggested a slight male preponderance, whereas others have failed to observe any sex predilection.
| Arteriovenous Malformations | Meningioma |
| Ependymoma | Metastatic Disease to the Brain |
| Glioblastoma Multiforme | Neurosarcoidosis |
| Low-Grade Astrocytoma | Tolosa-Hunt Syndrome |
| Medulloblastoma |
Hemangioblastoma
MRI
CT scan
Arteriography occasionally is useful in differentiating vascular lesions, including tumors, from gliomas.
The histopathology is variable; most gliomas in the brain stem are fibrillary, pilocytic astrocytomas, or the more malignant glioblastoma multiforme. Hemorrhage and necrosis are associated with the more malignant forms. Cysts may be seen with either the high- or low-grade forms.
Surgical resection is performed in conjunction with radiation and/or chemotherapeutic agents.
The goals of pharmacotherapy are to reduce morbidity and prevent complications.
These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.
Can be used to reduce tumor- and radiotherapy-associated cerebral edema.
24 mg IV bolus followed by maintenance dose of 4 mg PO tid/qid; for intracranial neoplasms, higher doses may be required in patients with severe cerebral edema or herniation syndrome secondary to tumor
Not established
Coadministration with warfarin or heparin increases risk of bleeding
Documented hypersensitivity; systemic fungal infections; documented hypersensitivity
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Increases risks of multiple complications, including severe infections; monitor adrenal insufficiency when tapering drug; abrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections are possible complications
Typically, patients are monitored for worsening signs/symptoms. Admission to the hospital may be required to enable therapeutic intervention and stabilization.
Follow-up neuroimaging with MRI (unless contraindicated) is recommended within 72 hours after surgery and every 2-3 months to monitor response to therapy and progression of disease. This should be considered standard care for these patients.
Patients and families of patients acquire information from multiple sources, including, but not limited to, physician, patients, support groups, pharmaceutical companies, and the Internet. Physicians should be aware of this and have an open, informative relationship with their patients, empowering patients to become active members of the team with regard to the decision-making process involving their care.
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brainstem tumors, pontine lesions, tectal lesions, hydrocephalus, cervicomedullary lesions, neurofibromatosis, intrinsic pontine gliomas, tectal gliomas, cervicomedullary gliomas, intracranial tumors
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
Anita Venkataramana, MBBS, Clinical Instructor, Department of Neurology, Division of Neuroimmunology/HIV, Johns Hopkins University
Disclosure: Nothing to disclose.
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, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine 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
Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.
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 Honoraria Consulting; Teva Marion Honoraria Consulting; Boeringer-Ingelheim Honoraria Speaking and teaching
Clinical guidelines
ACR Appropriateness Criteria® ataxia.
American College of Radiology - Medical Specialty Society. 1999 (revised 2006). 10 pages. NGC:005547
Long-term follow-up guidelines for survivors of childhood, adolescent, and young adult cancers. Sections 38-91: radiation.
Children's Oncology Group - Medical Specialty Society. 2003 Sep (revised 2006 Mar). 74 pages. NGC:005599
Clinical trials
Immunotherapy for Patients With Brain Stem Glioma and Glioblastoma
DNA Analysis of Tumor Tissue Samples From Patients With Diffuse Brain Stem Glioma
A Study of Bevacizumab Therapy in Patients With Newly Diagnosed High-Grade Gliomas and Diffuse Intrinsic Pontine Gliomas
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