• Journal of Korean Neurosurgical Society
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• v.55 no.3
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• pp.164-166
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• 2014

Ganglioglioma is an infrequent tumor of the central nervous system (CNS); mostly supratentorial region. But, they can occur anywhere in the central nervous system such as brainstem, cerebellopontine angle (CPA), thalamus, optic nerve and spinal cord. Although it occurs rarely, ganglioglioma should be included in the differential diagnosis of a posterior fossa mass because early recognition is important for treatment and patient counseling.

• Journal of Korean Neurosurgical Society
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• v.29 no.5
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• pp.688-692
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• 2000

The authors report a case of ganglioglioma in unusual site, the hypothalamus. The tumor was completely removed and no recurrence was noted on the follow up. Ganglioglioma usually occurs in the temporal and frontal lobe, but is rarely found on the hypothalamus. We report this case with pertinent literatual reviews.

• Journal of Korean Neurosurgical Society
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• v.42 no.1
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• pp.53-55
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• 2007

Gangliogliomas could be found anywhere throughout the central nervous system and mainly affect children and young adults during the first three decades of life. Cerebellar gangliogliomas may be rarely found, especially in old ages. Here, we present a case of ganglioglioma of the cerebellum in an old patient. The cystic cerebellar mass was associated with calcifications, intratumoral hemorrhage without ng edema. When a cystic cerebellar mass is associated with calcifications and intratumoral hemorrhage, ganglioglioma should be included in differential diagnosis. Gangliogliomas usually have good prognoses. Radiation therapy should be deferred even in subtotally removed cases.

• Journal of Yeungnam Medical Science
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• v.14 no.2
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• pp.451-458
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• 1997

The desmoplastic infantile ganglioglioma is very rare cerebral tumor. It has been known to be characterized by its voluminious size, intense desmoplasia and the frequent presence of astrocytic and ganglioglionic differentiation. Also, It is usually presented in infantile period and predilected in the frontal and parietal lobes. We treated a huge desmoplastic infantile ganglioglioma($8{\times}7{\times}6cm$) on the right frontotemporo-parietal areas of with only gross total resection. It includes solid mass & several cysts and shows the areas of the proliferation of spindle cells exhibiting storiform pattern in dense desmoplastic stroma and the areas composed of spindle shaped glial component.

• Journal of Korean Neurosurgical Society
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• v.43 no.3
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• pp.165-168
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• 2008

The location of ganglioglioma (GG) within the infratentorial compartment is unusual. The authors report a rare case of GG in the cerebellar hemisphere. A 12-year-old boy suffered from headache and gait disturbance. Neuroimaging studies demonstrated a large enhancing cerebellar mass with cystic components compressing the forth ventricle. After complete resection of the tumor, the patient became symptom free. Histological examination on the tumor disclosed glial cells and dysplastic ganglion cells. Although it is a rare tumor, in the appropriate clinical setting, a GG should be considered in the presence of a cerebellar mass with both solid and cystic components on magnetic resonance images in children.

• Journal of Korean Neurosurgical Society
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• v.30 no.2
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• pp.239-243
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• 2001

Gangliogliomas are benign tumors, composed of neoplastic astrocytes and nerve cells. They are rare, account for 0.4-6.25% of all primary central nervous system neoplasms. Gangliogliomas affect predominantly infants or young adults. Gangliogliomas are preferentially encountered supratentorially, predominantly affecting the temporal lobe. Spinal gangliogliomas affect predominantly the cervical spinal cord. We report a case of cervical ganglioglioma which was successfully removed surgically, with a review of literature.

• Journal of Korean Neurosurgical Society
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• v.59 no.2
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• pp.158-160
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• 2016

Ganglion cell tumors (GCT) are divided into two subtypes : gangliocytoma and ganglioglioma. Intramedullary gangliocytomas are extremely rare. A 20-year-old male patient with pain of neck, who also had a previously known neuroendocrine tumor of lung, was operated for mass found in the cervicomedullary junction with a presumptive diagnosis of metastases. Only partial resection could be performed. Pathological diagnosis had been reported as gangliocytoma. Only ten cases of intramedullary gangliocytoma have been reported in the literature. Although association with scoliosis and Von Recklinghausen;s disease were previously reported in the literature, no gangliocytoma case concomitant with endocrine tumor of lung have been published. Pathological study is the most important diagnostic method for gangliocytomas. Surgical excision is the primary treatment, but difficulty in total surgical tumor resection is the most important problem.

• Molecules and Cells
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• v.41 no.10
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• pp.881-888
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• 2018

During the cortical development, cells in the brain acquire somatic mutations that can be implicated in various neurodevelopmental disorders. There is increasing evidence that brain somatic mutations lead to sporadic form of epileptic disorders with previously unknown etiology. In particular, malformation of cortical developments (MCD), ganglioglioma (GG) associated with intractable epilepsy and non-lesional focal epilepsy (NLFE) are known to be attributable to brain somatic mutations in mTOR pathway genes and others. In order to identify such somatic mutations presenting as low-level in epileptic brain tissues, the mutated cells should be enriched and sequenced with high-depth coverage. Nevertheless, there are a lot of technical limitations to accurately detect low-level of somatic mutations. Also, it is important to validate whether identified somatic mutations are truly causative for epileptic seizures or not. Furthermore, it will be necessary to understand the molecular mechanism of how brain somatic mutations disturb neuronal circuitry since epilepsy is a typical example of neural network disorder. In this review, we overview current genetic techniques and experimental tools in neuroscience that can address the existence and significance of brain somatic mutations in epileptic disorders as well as their effect on neuronal circuitry.

• Journal of Korean Neurosurgical Society
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• v.62 no.3
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• pp.313-320
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• 2019

Brain tumors are the second most common type of structural brain lesion that causes chronic epilepsy. Patients with low-grade brain tumors often experience chronic drug-resistant epilepsy starting in childhood, which led to the concept of long-term epilepsy-associated tumors (LEATs). Dysembryoplastic neuroepithelial tumor and ganglioglioma are representative LEATs and are characterized by young age of onset, frequent temporal lobe location, benign tumor biology, and chronic epilepsy. Although highly relevant in clinical epileptology, the concept of LEATs has been criticized in the neuro-oncology field. Recent genomic and molecular studies have challenged traditional views on LEATs and low-grade gliomas. Molecular studies have revealed that low-grade gliomas can largely be divided into three groups : LEATs, pediatric-type diffuse low-grade glioma (DLGG; astrocytoma and oligodendroglioma), and adult-type DLGG. There is substantial overlap between conventional LEATs and pediatric-type DLGG in regard to clinical features, histology, and molecular characteristics. LEATs and pediatric-type DLGG are characterized by mutations in BRAF, FGFR1, and MYB/MYBL1, which converge on the RAS-RAF-MAPK pathway. Gene (mutation)-centered classification of epilepsy-associated tumors could provide new insight into these heterogeneous and diverse neoplasms and may lead to novel molecular targeted therapies for epilepsy in the near future.

• Journal of Korean Neurosurgical Society
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• v.49 no.6
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• pp.334-338
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• 2011

Objective : The incidence of leptomeningeal dissemination from malignant glioma is rare, so the clinical features of this are not well documented yet We attempted to determine the clinical features of leptomeningeal dissemination from malignant gliomas. Methods : We retrospectively analyzed 11 cases of leptomeningeal dissemination of malignant glioma, who were treated at our institution between 2006 and 2009. We investigated the clinical features of these patients by considering the following factors : tumor locations, the events of ventricular opening during surgery and the cerebrospinal fluid (CSF) profiles, including the cytology. Results : The group was composed of 9 males and 2 females. The histological diagnosis of their initial intracranial tumors were 4 primary glioblastoma, 3 anaplastic astrocytoma, 1 anaplastic oligoastrocytoma, 2 ganglioglioma and 1 pleomorphic xanthoastrocyotma with anaplastic features. The mean age of the patients at the time of the initial presentation was $42.8{\pm}10.3$ years. The mean time between surgery and the diagnosis of spinal dissemination was $12.3{\pm}7.9$ (3-28) months. The mean overall survival after dissemination was $2.7{\pm}1.3$ months. All our patients revealed a history of surgical opening of the ventricles. Elevated protein in the CSF was reported for eight patients who had their CSF profiles checked. Conclusion : We propose that in the malignant gliomas, the surgical opening of ventricles can cause the spinal leptomeningeal dissemination and the elevated protein content of CSF may be a candidate marker of leptomeningeal dissemination.