eMedicine Specialties > Neurology > Neuro-oncology
Brainstem Gliomas: Differential Diagnoses & Workup
Updated: Jun 30, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Differential Diagnoses
| Arteriovenous Malformations | Meningioma |
| Ependymoma | Metastatic Disease to the Brain |
| Glioblastoma Multiforme | Neurosarcoidosis |
| Low-Grade Astrocytoma | Tolosa-Hunt Syndrome |
| Medulloblastoma |
Other Problems to Be Considered
Hemangioblastoma
Workup
Laboratory Studies
- Lab studies of blood chemistry and related body fluids are not helpful as a rule, though cerebrospinal fluid (CSF) examination is often important for differential diagnosis. The protein content of CSF may be elevated. Because of the risk of increased intracranial pressure due to obstructive hydrocephalus, caution in clinical and imaging assessment prior to lumbar puncture is stressed.
- Tissue confirmation is frequently not feasible with infiltrating, expansile tumors unless an exophytic component exists, and even then, biopsy cannot always be obtained.
Imaging Studies
MRI
- MRI of the head is the diagnostic test of choice. MRI can differentiate vascular malformations and other processes that can be misdiagnosed as a brainstem glioma on CT scan.3
- The typical MRI appearance of a brainstem glioma is an expansile, infiltrative process with low-to-normal signal intensity on T1-weighted images and heterogeneous high-signal intensity on T2-weighted images, with or without contrast enhancement (see Media files 1-2).
- MR spectroscopy has been used to help distinguish between tumor and nontumor lesions in the brain. An elevated choline peak suggests neoplasm.
- MRI can delineate the extent of infiltration of the leptomeninges and the surrounding structures.
- High midbrain tumors, especially those arising in the tectum, are typically low-grade lesions by histologic criteria. They commonly appear hypointense on T1 and hyperintense on T2 images even without contrast enhancement.
- The occurrence of contrast enhancement in a tectal lesion should raise suspicion of a metastatic lesion, especially in an adult, with or without a known history of cancer.
CT scan
- Although CT imaging is an appropriate choice when MRI is not available, the appearance of brainstem gliomas is variable on CT scan, and the sensitivity of and characterization of tumors by CT are poorer.
- CT identifies calcifications, cystic changes, and displacement of the ventricular system; however, lower brainstem lesions are often not apparent on CT scan.
Other Tests
Arteriography occasionally is useful in differentiating vascular lesions, including tumors, from gliomas.
Procedures
- Patients with hydrocephalus may require ventriculostomy or ventriculoperitoneal shunting for symptomatic relief.
- Patients with difficulties in swallowing and diminished gag reflex may need feeding by gastrostomy such as the percutaneous esophagogastrostomy (PEG).
- Those patients who have had multiple upper respiratory infections, pneumonia, or altered voice may need postoperative ventilatory assistance.
Histologic Findings
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.
More on Brainstem Gliomas |
| Overview: Brainstem Gliomas |
Differential Diagnoses & Workup: Brainstem Gliomas |
| Treatment & Medication: Brainstem Gliomas |
| Follow-up: Brainstem Gliomas |
| Multimedia: Brainstem Gliomas |
| References |
| Further Reading |
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References
Frazier JL, Lee J, Thomale UW, Noggle JC, Cohen KJ, Jallo GI. Treatment of diffuse intrinsic brainstem gliomas: failed approaches and future strategies. J Neurosurg Pediatr. Apr 2009;3(4):259-69. [Medline].
Ueoka DI, Nogueira J, Campos JC, Maranhão Filho P, Ferman S, Lima MA. Brainstem gliomas--retrospective analysis of 86 patients. J Neurol Sci. Jun 15 2009;281(1-2):20-3. [Medline].
Grau SJ, Rachinger W, Holtmannspoetter M, Herms J, Tonn JC, Kreth FW. Serial Stereotactic Biopsy of Brainstem Lesions in Adults Improves Diagnostic Accuracy Compared to MRI Only. J Neurol Neurosurg Psychiatry. Jun 10 2009;[Medline].
Frappaz D, Schell M, Thiesse P et al. Preradiation chemotherapy may improve survival in pediatric diffuse intrinsic pontine gliomas: Final results of BSG 98 prospective trial. Neuro Oncol. Aug/2008;10(4):599-607. [Medline].
Raza S, Donach M. Bevacizumab in adult malignant brainstem gliomas. J Neurooncol. Jun 9 2009;[Medline].
Vesper J, Bölke B, Wille C, Gerber PA, Matuschek C, Peiper M, et al. Current concepts in stereotactic radiosurgery - a neurosurgical and radiooncological point of view. Eur J Med Res. Mar 17 2009;14(3):93-101. [Medline].
Kaplan AM, Albright AL, Zimmerman RA, Rorke LB, Li H, Boyett JM, et al. Brainstem gliomas in children. A Children's Cancer Group review of 119 cases. Pediatr Neurosurg. 1996;24(4):185-92. [Medline].
Squires LA, Allen JC, Abbott R, Epstein FJ. Focal tectal tumors: management and prognosis. Neurology. May 1994;44(5):953-6. [Medline].
Grigsby PW, Garcia DM, Simpson JR, et al. Prognostic factors and results of therapy for adult thalamic and brainstem tumors. Cancer. Jun 1 1989;63(11):2124-9. [Medline].
Landolfi JC, Thaler HT, DeAngelis LM. Adult brainstem gliomas. Neurology. Oct 1998;51(4):1136-9. [Medline].
Hamilton MG, Lauryssen C, Hagen N. Focal midbrain glioma: long term survival in a cohort of 16 patients and the implications for management. Can J Neurol Sci. Aug 1996;23(3):204-7. [Medline].
Kesari S, Kim RS, Markos V, Drappatz J, Wen PY, Pruitt AA. Prognostic factors in adult brainstem gliomas: a multicenter, retrospective analysis of 101 cases. J Neurooncol. Jun 2008;88(2):175-83. [Medline].
Abbott R, Shiminski-Maher T, Epstein FJ. Intrinsic tumors of the medulla: predicting outcome after surgery. Pediatr Neurosurg. Jul 1996;25(1):41-4. [Medline].
Albright AL, Guthkelch AN, Packer RJ, et al. Prognostic factors in pediatric brain-stem gliomas. J Neurosurg. Dec 1986;65(6):751-5. [Medline].
Barkovich AJ, Krischer J, Kun LE, et al. Brain stem gliomas: a classification system based on magnetic resonance imaging. Pediatr Neurosurg. 1990-91;16(2):73-83. [Medline].
Cohen ME, Duffner PK. 2nd ed. Brain Tumors in Children: Principles of Diagnosis and Treatment. New York: Raven Press; 1994.
Cohen ME, Duffner PK, Heffner RR, et al. Prognostic factors in brainstem gliomas. Neurology. May 1986;36(5):602-5. [Medline].
Dunkel IJ, O'Malley B, Finlay JL. Is there a role for high-dose chemotherapy with stem cell rescue for brain stem tumors of childhood?. Pediatr Neurosurg. 1996;24(5):263-6. [Medline].
Edwards MS, Wara WM, Urtasun RC, et al. Hyperfractionated radiation therapy for brain-stem glioma: a phase I-II trial. J Neurosurg. May 1989;70(5):691-700. [Medline].
Epstein F, Wisoff J. Intra-axial tumors of the cervicomedullary junction. J Neurosurg. Oct 1987;67(4):483-7. [Medline].
Fenichel Gerald M. Clinical Pediatric Neurology: A Signs and Symptoms Approach. 3rd ed. Philadelphia: WB Saunders Company; 1997.
Guiney MJ, Smith JG, Hughes P, et al. Contemporary management of adult and pediatric brain stem gliomas. Int J Radiat Oncol Biol Phys. Jan 15 1993;25(2):235-41. [Medline].
Jallo GI, Biser-Rohrbaugh A, Freed D. Brainstem gliomas. Childs Nerv Syst. Mar 2004;20(3):143-53. [Medline].
Kaye AH, Laws ER. Brain Tumors. New York: Churchill Livingstone; 1995.
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Rosenblum RK. Brain stem glioma: two case studies. J Pediatr Oncol Nurs. Mar-Apr 2005;22(2):114-8. [Medline].
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Further Reading
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
Related eMedicine topics
Glioblastoma Multiforme (Neurology)
Glioblastoma Multiforme (Radiology)
Hydrocephalus
Neurofibromatosis, Type 1
Neurofibromatosis, Type 2
Keywords
brainstem tumors, pontine lesions, tectal lesions, hydrocephalus, cervicomedullary lesions, neurofibromatosis, intrinsic pontine gliomas, tectal gliomas, cervicomedullary gliomas, intracranial tumors




Differential Diagnoses & Workup: Brainstem Gliomas