eMedicine Specialties > Radiology > Brain/Spine

Ganglioglioma

Author: Anil Khosla, MBBS, Assistant Professor, Department of Radiology, Section of Neuroradiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine, Veterans Affairs Medical Center of St Louis
Contributor Information and Disclosures

Updated: Mar 11, 2009

Introduction

Background

Ganglioglioma was first described by Courville in 1930 as a central nervous system neoplasm containing both astrocytic and neuronal components.1 Gangliogliomas may occur anywhere in the central nervous system but are not encountered commonly.

Axial MRI images (T1-weighted, T1-weighted with c...

Axial MRI images (T1-weighted, T1-weighted with contrast, and T2-weighted) demonstrate a left superior medial frontal mixed solid and cystic mass that is surgically proven and typical ganglioglioma. The solid anterior component is hypo-intense on the T1-weighted image, of intermediate intensity on the T2-weighted image, and reveals dense contrast enhancement. The cystic posterior component has low cerebrospinal fluidlike signal on the T1-weighted image and bright signal on the T2-weighted image with no contrast enhancement. Note that the tumor extends to a cortical surface, and no surrounding edema is seen.

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Axial MRI images (T1-weighted, T1-weighted with c...

Axial MRI images (T1-weighted, T1-weighted with contrast, and T2-weighted) demonstrate a left superior medial frontal mixed solid and cystic mass that is surgically proven and typical ganglioglioma. The solid anterior component is hypo-intense on the T1-weighted image, of intermediate intensity on the T2-weighted image, and reveals dense contrast enhancement. The cystic posterior component has low cerebrospinal fluidlike signal on the T1-weighted image and bright signal on the T2-weighted image with no contrast enhancement. Note that the tumor extends to a cortical surface, and no surrounding edema is seen.


Similar to other brain tumors, imaging techniques define the tumor location and its relationship to adjacent structures. Because of its relative rarity and nonspecific appearance, ganglioglioma is only infrequently considered a presurgical diagnosis.2,3

Pathophysiology

Gangliogliomas are firm grayish tumors that may have cystic components. Tumors are mild-to-moderately cellular and slightly pleomorphic with rare mitotic figures. On light microscopy, atypical dysplastic neurons, astrocytes, and fibrovascular stroma are observed. Infrequently, anaplastic degeneration occurs and involves the astrocytic component. Neoplastic ganglion cells are large and mature and reveal intense immunoreactivity with synaptophysin, show expression of the stem cell epitope CD34, and are often binucleated. Biologic behavior is not predicted by histology, since many histologically anaplastic gangliogliomas do not demonstrate clinically aggressive behavior.

Most gangliogliomas are observed in the brain. Temporal lobes and cerebellar hemispheres are the most common locations. Gangliogliomas have been reported in unusual locations, such as the pineal gland, basal ganglia, hypothalamus, and optic chiasm. Rarely, they may also develop in the spinal cord.

Frequency

United States

Gangliogliomas represent approximately 0.4% of central nervous neoplasms and 1.3% of brain tumors. Only 1% of intramedullary spinal neoplasms are histologically gangliogliomas. Approximately 10% of primary brain tumors in children are gangliogliomas.

Mortality/Morbidity

Gangliogliomas are generally well-demarcated and slowly growing tumors. Recurrence is rare following gross total resection of the tumor. Radiation treatment is not indicated following gross total resection. Complete tumor resection is generally curative. Metastatic spread is extremely rare. Only a few reported cases describe benign gangliogliomas progressing to malignant gliomas.

Sex

Male-to-female ratio is equal.

Age

Most gangliogliomas are observed in patients younger than 30 years. The age ranges from 2 months to 70 years.

Presentation

Presentation depends on patient age and the location and aggressiveness of the tumor. Most gangliogliomas are nonaggressive, and most patients present with long-standing progressive symptoms. Temporal lobe gangliogliomas usually present with temporal lobe seizures. Now that MR screening of seizure patients is more routine, these are increasingly identified on imaging.

Gross total removal is the best chance for a cure. In a study of 8 pediatric patients, aged 0-17 years, with confirmed anaplastic ganglioglioma, Karremann et al found that gross total tumor resection resulted in increased survival over nontotal resection.4,5

Cerebellar lesions present with ataxia, headache, and hydrocephalus. Patients presenting with seizures have a better prognosis, since they are diagnosed and treated early.

Preferred Examination

MRI with contrast enhancement is more sensitive and specific than other imaging techniques in determining the presence and location of lesions and in characterizing the lesion's cystic and/or solid components.6

Limitations of Techniques

Imaging appearances are nonspecific, and the diagnosis usually is established by histology and immunohistochemistry.

Differential Diagnoses

Oligodendroglioma

Other Problems to Be Considered

Gangliocytoma
Low-grade gliomas
Dysembryonic neuroepithelial tumor
Oligodendroglioma (tumors heterogeneous in appearance; small samples may not include diagnostic ganglion cells)
Ganglionic cells may be mistaken for gemistocytic astrocytes (immunohistochemistry helps diagnosis)

More on Ganglioglioma

Overview: Ganglioglioma
Imaging: Ganglioglioma
Follow-up: Ganglioglioma
Multimedia: Ganglioglioma
References
Further Reading
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References

  1. Courville CB. Ganglioglioma tumor of the central nervous system: review of the literature and report of two cases. Arch Neurol Psych. 1930;24:439-91.

  2. Park SH, Kim E, Son EI. Cerebellar ganglioglioma. J Korean Neurosurg Soc. Mar 2008;43(3):165-8. [Medline].

  3. Mpairamidis E, Alexiou GA, Stefanaki K, Sfakianos G, Prodromou N. Brainstem ganglioglioma. J Child Neurol. Dec 2008;23(12):1481-3. [Medline].

  4. Adachi Y, Yagishita A. Gangliogliomas: Characteristic imaging findings and role in the temporal lobe epilepsy. Neuroradiology. Oct 2008;50(10):829-34. [Medline].

  5. Karremann M, Pietsch T, Janssen G, Kramm CM, Wolff JE. Anaplastic ganglioglioma in children. J Neurooncol. Apr 2009;92(2):157-63. [Medline].

  6. Balaji R, Ramachandran K. Imaging of desmoplastic infantile ganglioglioma: a spectroscopic viewpoint. Childs Nerv Syst. Jan 13 2009;[Medline].

  7. Urbach H. MRI of long-term epilepsy-associated tumors. Semin Ultrasound CT MR. Feb 2008;29(1):40-6. [Medline].

  8. Kikuchi T, Kumabe T, Higano S, Watanabe M, Tominaga T. Minimum apparent diffusion coefficient for the differential diagnosis of ganglioglioma. Neurol Res. Jan 9 2009;[Medline].

  9. Castillo M. Gangliogliomas: ubiquitous or not?. AJNR Am J Neuroradiol. May 1998;19(5):807-9. [Medline].

  10. Castillo M, Davis PC, Takei Y, Hoffman JC Jr. Intracranial ganglioglioma: MR, CT, and clinical findings in 18 patients. AJNR Am J Neuroradiol. Jan-Feb 1990;11(1):109-14. [Medline].

  11. Di Patre PL, Payer M, Brunea M, et al. Malignant transformation of a spinal cord ganglioglioma--case report and review of the literature. Clin Neuropathol. Nov-Dec 2004;23(6):298-303. [Medline].

  12. Hamburger C, Buttner A, Weis S. Ganglioglioma of the spinal cord: report of two rare cases and review of the literature. Neurosurgery. Dec 1997;41(6):1410-5; discussion 1415-6. [Medline].

  13. Im SH, Chung CK, Cho BK, et al. Intracranial ganglioglioma: preoperative characteristics and oncologic outcome after surgery. J Neurooncol. Sep 2002;59(2):173-83. [Medline].

  14. Jallo GI, Freed D, Epstein FJ. Spinal cord gangliogliomas: a review of 56 patients. J Neurooncol. May 2004;68(1):71-7. [Medline].

  15. Kincaid PK, El-Saden SM, Park SH, Goy BW. Cerebral gangliogliomas: preoperative grading using FDG-PET and 201Tl-SPECT. AJNR Am J Neuroradiol. May 1998;19(5):801-6. [Medline].

  16. Kjällman M, Hamne M, Furugård S, et al. [Treatment of fibromyalgia. Positive experiences with group education]. Lakartidningen. Jan 26 1994;91(4):265, 268. [Medline].

  17. Krouwer HG, Davis RL, McDermott MW, et al. Gangliogliomas: a clinicopathological study of 25 cases and review of the literature. J Neurooncol. Aug 1993;17(2):139-54. [Medline].

  18. Luyken C, Blümcke I, Fimmers R, et al. Supratentorial gangliogliomas: histopathologic grading and tumor recurrence in 184 patients with a median follow-up of 8 years. Cancer. Jul 1 2004;101(1):146-55. [Medline].

  19. Matsumoto K, Tamiya T, Ono Y, et al. Cerebral gangliogliomas: clinical characteristics, CT and MRI. Acta Neurochir (Wien). 1999;141(2):135-41. [Medline].

  20. Miller DC, Koslow M, Budzilovich GN, Burstein DE. Synaptophysin: a sensitive and specific marker for ganglion cells in central nervous system neoplasms. Hum Pathol. Jan 1990;21(1):93-8. [Medline].

  21. Miller DC, Lang FF, Epstein FJ. Central nervous system gangliogliomas. Part 1: Pathology. J Neurosurg. Dec 1993;79(6):859-66. [Medline].

  22. Shin JH, Lee HK, Khang SK, et al. Neuronal tumors of the central nervous system: radiologic findings and pathologic correlation. Radiographics. Sep-Oct 2002;22(5):1177-89. [Medline].

  23. Tien RD, Tuori SL, Pulkingham N, Burger PC. Ganglioglioma with leptomeningeal and subarachnoid spread: results of CT, MR, and PET imaging. AJR Am J Roentgenol. Aug 1992;159(2):391-3. [Medline].

  24. Varlet P, Soni D, Miquel C, et al. New variants of malignant glioneuronal tumors: a clinicopathological study of 40 cases. Neurosurgery. Dec 2004;55(6):1377-91: discussion 1391-2. [Medline].

  25. Zentner J, Wolf HK, Ostertun B, et al. Gangliogliomas: clinical, radiological, and histopathological findings in 51 patients. J Neurol Neurosurg Psychiatry. Dec 1994;57(12):1497-502. [Medline].

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Keywords

ganglioglioma, brain tumor, spinal cord tumor, neural tumor, CNS tumor, brain neoplasm, spinal cord neoplasm, neural neoplasm, CNS neoplasm, central nervous system tumor, central nervous system neoplasm

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Contributor Information and Disclosures

Author

Anil Khosla, MBBS, Assistant Professor, Department of Radiology, Section of Neuroradiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine, Veterans Affairs Medical Center of St Louis
Anil Khosla, MBBS is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Neuroradiology, North American Spine Society, and Radiological Society of North America
Disclosure: Nothing to disclose.

Medical Editor

Mahesh R Patel, MD, Chief of MRI, Department of Radiology, Santa Clara Valley Medical Center
Mahesh R Patel, MD is a member of the following medical societies: American Roentgen Ray Society, American Society of Neuroradiology, and Radiological Society of North America
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

C Douglas Phillips, MD, Professor, Departments of Radiology, Neurosurgery, and Otolaryngology, University of Virginia Health Sciences Center
C Douglas Phillips, MD is a member of the following medical societies: American College of Radiology, American Medical Association, American Society of Head and Neck Radiology, American Society of Neuroradiology, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

James G Smirniotopoulos, MD, Professor of Radiology, Neurology, and Biomedical Informatics, Chairman, Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences
James G Smirniotopoulos, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Head and Neck Radiology, American Society of Neuroradiology, American Society of Pediatric Neuroradiology, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.

 
 
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