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Table of Contents    
Year : 2016  |  Volume : 64  |  Issue : 2  |  Page : 349-351

Ganglioglioma of the posterior third ventricle region: An unusual pathology in an uncommon location

1 Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India
2 Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India

Date of Web Publication3-Mar-2016

Correspondence Address:
Arivazhagan Arimappamagan
Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.177605

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How to cite this article:
Maiti TK, Arimappamagan A, Mahadevan A, Pandey P. Ganglioglioma of the posterior third ventricle region: An unusual pathology in an uncommon location. Neurol India 2016;64:349-51

How to cite this URL:
Maiti TK, Arimappamagan A, Mahadevan A, Pandey P. Ganglioglioma of the posterior third ventricle region: An unusual pathology in an uncommon location. Neurol India [serial online] 2016 [cited 2022 Nov 26];64:349-51. Available from: https://www.neurologyindia.com/text.asp?2016/64/2/349/177605


Gangliogliomas are infrequently occurring tumors in the central nervous system, accounting for approximately 1% of all brain tumors.[1] They usually present in the temporal or frontal lobe in children and young adults with epilepsy. Other unusual deep parenchymal locations are rarely documented. We report a case of ganglioglioma in the posterior third ventricle region and describe its management. A 20-year-old man presented with a 3-month history of headache, vomiting, and progressive loss of vision. Examination revealed bilateral papilledema. CT of the brain revealed an isodense lesion in the posterior third ventricle region, with specks of hyperdensity on plain images and mild enhancement on contrast, causing obstructive hydrocephalus [Figure 1]a,[Figure 1]b,[Figure 1]c. On MRI, the lesion was well defined, isointense on T1-weighted images, hyperintense on T2-weighted images, and enhancing uniformly and well on contrast, with no areas of bleed, and causing obstructive hydrocephalus. Serum and ventricular cerebrospinal fluid (CSF) [collected by ventricular tap] analyses for markers (beta human chorionic gonadotropin and alpha fetoprotein levels) were normal. He underwent a right-sided Poppen's approach and gross total resection of the tumor. The lesion was heterogenous, soft and suckable, with few calcified areas, and was relatively avascular. Postoperative contrast-enhanced CT scan of the brain showed no residual lesion [Figure 1]d and [Figure 1]e.
Figure 1: (a-c) CT scan of the head of a 20-year-old man showed an ill-defined isodense lesion in the posterior third ventricle region, with small areas of hyperdensity within (a); there was mild enhancement with contrast (b and c). Postoperative contrast-enhanced CT scan showed complete excision of the lesion with the presence of a pneumoventricle (d). A contrast-enhanced MRI of the brain at 3-month follow-up showed no residual lesion (e). (f and j) Histopathology revealed glial component with piloid astrocytes arranged in tight fascicles with Rosenthal fibres (f) and diffuse glial fibrillary acidic protein [GFAP] (g) labeling. Within this are clusters of large dysplastic ganglion cells with a large nuclei and prominent nucleoli (h, arrows). Some of these exhibit cytoplasmic and membrane immunoreactivity for synaptophysin (i, arrows) or phosphorylated neurofilament (j, arrows) (f: Hematoxylin–eosin [H and E], ×20; g: GFAP immunoperoxidase, ×20; h: H and E, ×40; i: Synaptophysin, ×40; j: Phosphorylated neurofilament, ×40)

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Histopathology showed features of ganglioglioma having the characteristic biphasic morphology with the glial component having a pilocytic morphology, with sheets of oligodendroglia-like cells in a fibrillary stroma, and the presence of eosinophilic granular bodies [Figure 1]f. Degenerative atypia and small multinucleate cells were seen. Entrapped large ganglion cells with dystrophic changes [Figure 1]h and perivascular lymphocytic infiltrates were seen. No calcification was noted. The glial zones were diffusely positive for GFAP [Figure 1]g. Synaptophysin and phosphorylated neurofilament was seen accumulating within the dysplastic ganglionic element [Figure 1]i and [Figure 1]j. MIB-1 labeling was low. A histological diagnosis of ganglioglioma (WHO Grade I) was made. A contrast-enhanced brain MRI of the patient at 3-month follow-up showed no residual lesion. Hydrocephalus had resolved without any need for a ventriculoperitoneal shunt [Figure 1]e. The patient was on follow-up and asymptomatic at 12 months after surgery.

Gangliogliomas are commonly noted in the temporal or frontal lobe, usually present with epilepsy and demonstrate an indolent course.[1] Histologically, they comprise of both neuronal and glial components. In the posterior third ventricle region, the pineocyte has been described as a multipotential cell that can differentiate into neuronal and glial cells, and is regarded as the possible cell of origin of a ganglioglioma.[2] Horrax and Bailey reported the first case of gangliocytoma in 1928.[3] Posterior third ventricle region gangliogliomas have been only reported in eight cases previously to the best of our knowledge [Table 1].[3],[4],[5],[6],[7],[8],[9],[10] As gangliogliomas are extremely rare, it is not possible to make a preoperative definitive diagnosis on imaging. In view of a well-defined lesion in the posterior third ventricular region, enhancing well on contrast, in a young patient, the close differential diagnosis includes a germinoma/germ cell tumor and pineocytoma. Hence, serum and ventricular CSF analysis is usually performed for tumor markers prior to considering resective surgery, as was done in our patient. Complete surgical resection should be considered when the intraoperative squash cytology suggests a benign tumor and provides a good outcome. Surgical approach may be influenced by the choice of the surgeon. We preferred the Poppen's approach because of better exposure of the pineal region anatomy. Radiotherapy has been rarely given for residual tumors.[5] Ganglioglioma, when occurring in the pineal region, presents with raised intracranial pressure. Complete tumor excision, as was done in the present case, should be performed to obtain a good neurological outcome. These lesions could be considered in the differential diagnosis when the intraoperative cytology suggests a pineocytoma/benign glial tumor.
Table 1: Reported cases of ganglioglioma of pineal region in literature

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 » References Top

Luyken C, Blümcke I, Fimmers R, Urbach H, Wiestler OD, Schramm J. Supratentorial gangliogliomas: Histopathologic grading and tumor recurrence in 184 patients with a median follow-up of 8 years. Cancer 2004;101:146-55.  Back to cited text no. 1
Rubinstein LJ, Okazaki H. Gangliogliomatous differentiation in a pineocytoma. J Pathol 1970;102:27-32.  Back to cited text no. 2
Horrax G, Bailey P. Pineal pathology: Further studies. Arch Neurol Psychiatr 1928;19:394-414.  Back to cited text no. 3
Ebina K, Suzuki S, Takahashi T, Iwabuchi T, Takei Y. Gangliocytoma of the pineal body. A case report and review of the literature. Acta Neurochir (Wien) 1985;74:134-40.  Back to cited text no. 4
Hunt SJ, Johnson PC. Melanotic ganglioglioma of the pineal region. Acta Neuropathol 1989;79:222-5.  Back to cited text no. 5
Tokoro K, Chiba Y, Ohtani T, Abe H, Yagishita S. Pineal ganglioglioma in a patient with familial basal ganglia calcification and elevated serum alpha-fetoprotein: Case report. Neurosurgery 1993;33:506-11.  Back to cited text no. 6
Faillot T, Sichez JP, Capelle L, Kujas M, Fohanno D. Ganglioglioma of the pineal region: Case report and review of the literature. Surg Neurol 1998;49:104-8.  Back to cited text no. 7
Chang YL, Lin SZ, Chiang YH, Liu MY, Lee WH. Pineal ganglioglioma with premature thelarche. Report of a case and review of the literature. Childs Nerv Syst 1996;12:103-6.  Back to cited text no. 8
Fagundes-Pereyra WJ, Sousa LD, Carvalho GT, Sousa AA. Ganglioglioma of the pineal region: Case report. Arq Neuropsiquiatr 2001;59:599-604.  Back to cited text no. 9
Tender GC, Smith RD. Pineal ganglioglioma in a young girl: A case report and review of the literature. J La State Med Soc 2004;156:316-8.  Back to cited text no. 10


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