Ganglioglioma of conus medullaris: A case report

A case of a ganglioglioma of the conus medullaris extending between T-12 and L2 segments is reported. The tumor was succesfully removed by third stage operation. Ganglioglioma located in the conus medullaris is extremely rare. The best treatment of spinal cord ganglioglioma is totally tumor excision even when multiple stage operations are necessary.     

Ganglioglioma presenting as a meningioma: case report and review of the literature

OBJECTIVE AND IMPORTANCE: Gangliogliomas are intra-axial, avascular masses located predominantly in the temporal lobe. A ganglioglioma that mimics a meningioma in that it is extra-axial and has a significant extracranial vascular supply has not been reported previously. CLINICAL PRESENTATION: A 12-year-old girl presented with a right temporoparietal mass. A neurological examination revealed nothing abnormal, and the girl's symptoms were limited to headaches. INTERVENTION: Magnetic resonance imaging revealed an extra-axial mass, and cerebral catheter angiography revealed a blood supply mainly from the posterior division of the right middle meningeal artery. Intraoperative findings confirmed the extra-axial location of the tumor, and histological analysis revealed that the tumor was a ganglioglioma. CONCLUSION: This report confirms that gangliogliomas can present as extra-axial, vascular masses that are similar to meningiomas.     

Ganglioglioma of the optic pathway. A case report.

A case of ganglioglioma of the optic pathway associated with congenital exophthalmos and strabismus is presented. Since the tumor extended from the right optic nerve to the right geniculate body, it was diagnosed as an optic glioma before operation. However, optic nerve biopsy showed that the lesion was a ganglioglioma. Although a literature review yielded two previous cases of ganglioglioma of the optic pathway, this is the first case in which the tumor involved the whole optic pathway.     

Ganglioglioma of conus medullaris

Summary Gangliogliomas are glioneuronal tumours of the young, and occur more frequently supratentorially. Among those uncommon cases in the spinal cord, the conus medullaris is an extremely rare site. We reviewed the literature of such cases and report another case of a ganglioglioma of the conus in a 13-year-old boy with an insidious sensory sensitive and motor deficit of the right lower limb. Subtotal resection was accomplished with no recurrence after two years of follow-up. As for the supratentorial locations, the extent of surgical resection is the main prognostic factor for gangliogliomas of the spinal cord, and they should be considered in the differential diagnosis of intramedullar neoplasm.     

Prognostic Factors in Supratentorial Ganglioglioma

Summary  Although gangliogliomas are often associated with long survival, efforts to identify specific prognostic factors in these tumors have been largely unsuccessful. To identify factors associated with long survival, we retrospectively reviewed 42 cases of supratentorial ganglioglioma surgically treated at our institution since 1985. Data analysis included Kaplan-Meier survival curves and log-rank tests of the effects of individual variables. The Cox proportional hazards method was used to fit a model incorporating several variables simultaneously.  The 42 cases included 21 male and 21 female patients with an average age at surgery of 31 years. Length of follow-up averaged 48 months. Ten patients died an average of 38 months after surgery. Factors found to have a significant correlation with mortality were older age at diagnosis (P=0.012), male gender (P=0.034), and malignant glial features (P=0.020). Presenting symptoms, location of tumor, adjuvant radiation therapy, and extent of surgical resection were not significantly related to survival.  These results are the first to demonstrate an association between prognostic factors and outcome in patients with supratentorial ganglioglioma.     

Ganglioglioma of the medulla oblongata: case report and review of the literature

The authors present a pediatric case of ganglioglioma occurring in the medulla oblongata. A 7-year-old boy was referred to our hospital with complaints of ataxia, seizure and sleep apnea. MRI of the brain disclosed a large tumor occupying the medulla oblongata, and the upper portion of the cervical spinal cord was also involved. The patient underwent midline suboccipital craniotomy and laminectomy of C1 to attempt radical resection of the tumor, which resulted only in partial removal of the tumor due to severe bradycardia during the operation. The histological diagnosis was ganglioglioma, WHO grade 2. Although both radiotherapy and chemotherapy were performed following the operation, the tumor remained unchanged. The patient died of respiratory arrest five months after the operation. Gangliogliomas usually occur in the supratentorial region, which permits easy surgical access and good prognosis. Only 3% of gangliogliomas occur in the brain stem, and its management can be challenging because of the difficulty of radical resection and poor response to both radiotherapy and chemotherapy.     

Ganglioglioma: a clinical study with long-term follow-up

Gangliogliomas are uncommon tumors of mixed neoplastic glial and neuronal elements. Because of their low incidence, few large series exist that fully describe the clinical characteristics of patients afflicted with this tumor. We have reviewed the medical records of 20 patients at Duke University Medical Center with histologically proven gangliogliomas. These patients typically presented within the first three decades of life and their most common presenting symptom was seizures. Therapies included surgical resection, either partial or total, radiation therapy, and/or chemotherapy. Long-term follow-up was achieved by chart review and by telephone interview. Patients who underwent gross total resection alone seemed to fare the best when comparing all treatment groups, and we therefore recommend this as the main form of treatment.     

Ganglioglioma in a patient with Turcot syndrome. Case report

A 33-year-old woman with Turcot syndrome harbored a brain tumor and colon cancer and had a familial history of this syndrome. On histological examination, the brain tumor was found to have large and diffusely scattered ganglion cells within a diffuse background of astrocytic cells in a fibrillary matrix. The tumor was diagnosed as a ganglioglioma. No germline mutation in the adenomatous polyposis coli gene was detected using a protein truncation assay. These findings indicate that this patient had brain tumor-polyposis syndrome Type 1 of Turcot syndrome. This is the first report of a ganglioglioma related to Turcot syndrome.     

Ganglioglioma of the medulla oblongata.

A patient with signs and symptoms suggestive of symptomatic Arnold-Chiari malformation was encountered. Neuroradiologic investigation revealed the presence of a medullary tumor, which later proved to be a ganglioglioma. A review of the literature suggests that this presentation is typical of medullary gangliogliomas. It is proposed that the pathophysiology of this lesion renders it amenable to decompressive therapy via suboccipital craniotomy.     

鞍区节细胞胶质瘤1例

正患者女,50岁,因2年前无明显诱因出现双眼视力下降,近3个月头晕入院。查体:神清语明,双侧瞳孔等大、等圆,直径约3.0mm,对光反射灵敏;术前视力右眼0.8、左眼0.6;右眼视野下方及上方暗点,左眼配合不佳。垂体MR平扫及增强(图1):蝶鞍扩大,鞍底下陷,鞍区见类圆形异常信号,约1.8cm×1.4cm×2.0cm,T1WI呈稍低信号,T2WI呈稍高及等信号,增强扫描呈不均匀强化,程度低于正常垂体,垂体柄右     

Ganglioglioma: 13 years of experience.

A 13-year retrospective review of 17 patients with gangliogliomas treated at the University of Iowa was conducted to investigate the association between tumor location, extent of resection, pathological findings, and patient prognosis. Thirteen were in the cerebral hemispheres and 4 in the midline. The mean ages at diagnosis and symptom onset were 16 and 8.8 years, respectively. The most common presenting symptom was seizures (11 patients); focal neurological deficit was seen in 5 patients and headache in 1. Patients with hemispheric tumors had an older age at time of diagnosis and a longer duration of symptoms. Pathologically, they had more microcalcifications, lymphocytic infiltration, microcystic degeneration, and eosinophilic bodies. Cerebral hemispheric tumors were more amenable to total resection than midline neoplasms, 77% versus 25%, respectively. In the patients with hemispheric tumors, 10 patients were tumor-free after total resection. Two of the 3 patients with partial resection had stable residual tumors. The third died of tumor progression. In the 4 patients with midline neoplasms, one was tumor-free after total resection. The other 3 had subtotal resection and radiation therapy. Two died within 2 years; the third is still alive with progressive tumor. Five of 9 patients who had seizure disorders and who underwent total tumor removal were seizure-free postoperatively. The other 4 patients and the 2 with partial surgical resection continued to have seizures.     

Ganglioglioma of the brain stem. Case report

A case of brain-stem ganglioglioma is reported. A review of the literature revealed only 13 other reported cases. Brain-stem gangliogliomas usually become symptomatic in the first and second decades of life and involve the medulla and pons. Clinical improvement and prolonged survival have been reported after partial resection.     

Ganglioglioma in the third ventricle: report on two cases

Gangliogliomas are uncommon central nervous system (CNS) tumors composed of a mixture of glial and neuronal elements. Although these tumors can occur in any portion of the central nervous system, involvement of the ventricular system is rare. We herein report on two cases of gangliogliomas in the third ventricle in a 34-year-old woman and in a 52-year-old man. One patient presented only with headaches, and the other presented symptoms associated with panhypopituitarism and diabetes insipidus. In the first case the tumor in the middle portion of the third ventricle was successfully removed by a transcallosal subchoroidal approach. In the second case the hemorrhagic tumor was located in the anterior floor of the third ventricle and was removed by an anterior inter-hemispheric trans-lamina terminalis approach. To date, follow-ups of both patients have involved no adjuvant therapy, and there have been no signs of tumor recurrence on magnetic resonance images. The nature, radiological findings, and treatments of these tumors are discussed.     

Ganglioglioma, a malignant tumor? Correlation with flow deoxyribonucleic acid cytometric analysis

This study describes the flow cytometric deoxyribonucleic acid (DNA) analysis of a resected ganglioglioma. The initial histopathological analysis revealed a benign tumor characterized by a predominance of mature ganglion cells. The flow cytometric DNA analysis of the necrotic areas, however, demonstrated an aneuploid population of cells. Further examination by histological analysis of the tumor revealed both benign and atypical foci. The retrospective DNA analysis performed from paraffin sections of tissue with benign-histological findings demonstrated euploid populations of cells consistent with a benign, slow-growing lesion. In contrast, DNA analysis performed from tissue with atypical histological findings revealed aneuploid populations of cells consistent with a malignant phenotype. Our analysis provides additional data supporting the existence of tumor progression in some gangliogliomas. Results support the concept of tumor cell heterogeneity and the importance of adequate tumor sampling. The finding of aneuploid populations with unfavorable histology further supports the use of flow cytometry as an adjunct method in assessing tumor biology.     

Ganglioglioma: a correlative clinicopathological and radiological study of ten surgically treated cases with follow-up

Gangliogliomas are rare benign tumors of the central nervous system containing neoplastic ganglion and low grade glial cells. In studying 10 surgically treated cases, we evaluated the clinical, pathological, radiological, and immunocytochemical features, with follow-up. Ranging from 18 to 58 years in age, 7 patients were women, and 3 were men. The most common presenting symptom was seizure. Computed tomographic scan showed a low density enhancing mass in 8 and calcification in 5. Six had minimally abnormal vascularity on angiography. Seven patients had total and 3 had subtotal resections of the tumor. The temporal lobe was the location of the tumor in 6 cases. All of the cases met the histological criteria of Russell and Rubinstein for ganglioglioma. Four patients received postoperative radiotherapy because of subtotal resection or aggressive histological makeup. On follow-up, from 2.5 to 7 years, 8 patients are alive and tumor-free, and 7 are also seizure-free. Two died after operation: one immediately and the other of a glioblastoma that developed 5 years later. Our study confirms that ganglioglioma is a distinct histological entity, anatomically localized, with characteristic clinical and radiological findings and long term survival. Aggressive histological makeup is not a definite indication of malignant potential. The definitive role of follow-up radiotherapy for this tumor needs further study. Malignant evolution is rare, but warrants follow-up.     

Intramedullary Spinal Cord Ganglioglioma: A Report of Five Cases

We report five cases of ganglioglioma in various locations. The tumours were removed with gross total resection in most cases without any further neurological deterioration. Without any additional treatment, no evidence of recurrence or regrowth of tumours was observed in the mean follow-up period of 4.1 years. We suggest that intramedullary spinal cord gangliogliomas be resected totally due to the high risk of recurrence and regrowth rate of the tumours with subtotal resection in the long term considering especially that these tumours affect predominantly young patients.