Am J Psychiatry (subscription) - May 1, 2008 We report the case of a teenager who presented with short-lasting episodes resembling panic attacks secondary to a dorsal anterior cingulate ganglioglioma. ... |
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Am J Psychiatry (subscription) - May 1, 2008 We report the case of a teenager who presented with short-lasting episodes resembling panic attacks secondary to a dorsal anterior cingulate ganglioglioma. ... |
Am J Psychiatry (subscription) - May 1, 2008 We report the case of a teenager who presented with short-lasting episodes resembling panic attacks secondary to a dorsal anterior cingulate ganglioglioma. ... |
Am J Psychiatry (subscription) - May 1, 2008 We report the case of a teenager who presented with short-lasting episodes resembling panic attacks secondary to a dorsal anterior cingulate ganglioglioma. ... |
![]() | Medicexchange, UK - Apr 29, 2008 ... medulloblastoma, atypical teratoid rhabdoid tumor, primitive neuroectodermal tumor, ganglioglioma, hemangioblastoma and craniopharyngioma, n=16). ... |
Newswise (press release) - 12 hours ago ... medulloblastoma, atypical teratoid rhabdoid tumor, primitive neuroectodermal tumor, ganglioglioma, hemangioblastoma and craniopharyngioma, n=16). ... |
Newswise (press release) - Apr 28, 2008 ... medulloblastoma, atypical teratoid rhabdoid tumor, primitive neuroectodermal tumor, ganglioglioma, hemangioblastoma and craniopharyngioma, n=16). ... |
| Related Articles |
Anaplastic ganglioglioma arising from a Lhermitte-Duclos-like lesion.
J Neurosurg. 2007 Aug;107(2):137-142
Authors: Takei H, Dauser R, Su J, Chintagumpala M, Bhattacharjee MB, Jones J, Adesina AM
check markThe authors report the case of a 7-year-old boy with a history of developmental delay who presented with aggressive behavior. A magnetic resonance (MR) image showed a mass lesion originating from the cerebellar vermis with an atypical folial pattern and contrast enhancement. Histologically, the subtotally resected specimen consisted mostly of neuropil with nodular foci of ganglion cells. Lhermitte-Duclos disease (LDD) was diagnosed in the patient. A retrospective review of the tissue sections showed a nidus of associated astrocytic proliferation, suggesting a diagnosis of ganglioglioma. Five years later, the patient experienced an altered mental state and a facial droop. An MR image revealed a cerebellar mass with cystic areas and an enhancing nodule. The resected tissue specimen consisted primarily of a mixed proliferation of glial and ganglion cells consistent with a ganglioglioma. Two years later, a third craniectomy was performed in the patient for worsening headache and ataxia. Histologically, the tumor showed progressive anaplasia and was most accurately classified as an anaplastic ganglioglioma. Immunohistochemically, most of the tumor cells were immunoreactive for anti-phospho-mammalian target of rapamycin (mTOR) and phospho-S6 ribosomal protein antibodies. In contrast, the subpopulation of neoplastic ganglion cells in the tissue, particularly from the first surgery, did not express phosphatase and tensin homolog deleted from chromosome 10 (PTEN). This immunohistochemical pattern suggests that the large dysplastic ganglion cells (the gangliocytomatous component) forming the greater part of the lesion were associated with activation of the phosphatidylinositol 3-kinase-PTEN/Akt/mTOR signaling pathway, a feature previously reported in LDD. This case represents the first report of an anaplastic ganglioglioma arising in an LDD-like lesion.
PMID: 18459885 [PubMed - as supplied by publisher]
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MRI of long-term epilepsy-associated tumors.
Semin Ultrasound CT MR. 2008 Feb;29(1):40-6
Authors: Urbach H
In 20 to 30% of patients with long-term drug-resistant epilepsy neuroepithelial tumors, usually glioneuronal tumors are found. Gangliogliomas and dysembryoplastic neuroepithelial tumors (DNTs) are well characterized, both clinically and on MRI. Both tumor types are located in the cortex or in the cortex and subcortical white matter, gangliogliomas most commonly in the mesial temporal lobe ("around the collateral sulcus"). Both tumor types have typical imaging features, and from both, location and imaging features, they can be usually distinguished from glial tumors. This distinction is important since more than 70% of patients with drug resistant temporal lobe epilepsy caused by gangliogliomas and DNTs get seizure free following extended lesionectomy.
PMID: 18383906 [PubMed - indexed for MEDLINE]
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Role of perfusion-weighted imaging at 3 Tesla in the assessment of malignancy of cerebral gliomas.
Radiol Med (Torino). 2008 Feb;113(1):134-43
Authors: Di Costanzo A, Pollice S, Trojsi F, Giannatempo GM, Popolizio T, Canalis L, Armillotta M, Maggialetti A, Carriero A, Tedeschi G, Scarabino T
PURPOSE: This study was performed to clarify the role of perfusion-weighted imaging (PWI) at 3 Tesla in the characterisation of haemodynamic heterogeneity within gliomas and surrounding tissues and in the differentiation of high-grade from low-grade gliomas. MATERIALS AND METHODS: We examined 36 patients with histologically verified gliomas (25 with high-grade and 11 with low-grade gliomas). PWI was performed by first-pass gadopentetate dimeglumine T2*-weighted echo-planar images, and cerebral blood volume (CBV) maps were computed with a nondiffusible tracer model. Relative CBV (rCBV) was calculated by dividing CBV in pathological areas by that in contralateral white matter. RESULTS: In high-grade gliomas, rCBV were markedly increased in mass [mean+/-standard deviation (SD), 4.3+/-1.2] and margins (4.0+/-1.1) and reduced in necrotic areas (0.3+/-0.3). Oedematous-appearing areas were divided in two groups according to signal intensity on T2-weighted images: tumour with lower (nearly isointense to grey matter) and oedema with higher (scarcely isointense to cerebrospinal fluid) signal intensity. Tumour showed significantly higher rCBV than did oedema (1.8+/-0.5 vs. 0.5+/-0.2; p<0.001) areas. In low-grade gliomas, mass (2.0+/-1.5) and margin (2.2+/-1.2) rCBV were significantly lower than in high-grade gliomas (p<0.001). CONCLUSIONS: Three-Tesla PWI helps to distinguish necrosis from tumour mass, infiltrating tumour from oedema and high-grade from low-grade gliomas. It enhances the magnetic resonance (MR) assessment of cerebral gliomas and provides useful information for planning surgical and radiation treatment.
PMID: 18338133 [PubMed - indexed for MEDLINE]
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Microsurgical anatomy of the supracerebellar transtentorial approach to the posterior mediobasal temporal region: technical considerations with a case illustration.
Neurosurgery. 2008 Mar;62(3 Suppl 1):1-7; discussion 7-8
Authors: Moftakhar R, Izci Y, Başkaya MK
OBJECTIVE: Surgical access to the posterior portion of the mediobasal temporal lobe presents a formidable challenge to neurosurgeons, and much controversy still exists regarding the selection of the surgical approach to this region. The supracerebellar transtentorial (SCTT) approach to the posterior mediobasal temporal region can be used as an alternative to the subtemporal or transtemporal approaches. The aim of this study was to demonstrate the surgical anatomy of the SCTT approach and review the gyral, sulcal, and vascular anatomy of the posterior mediobasal temporal lobe. The use of this approach in the resection of a ganglioglioma located in the left posterior parahippocampal gyrus is illustrated. METHODS: The SCTT approach to the posterior parahippocampal gyrus was performed on three silicone-injected cadaveric heads. The gyral, sulcal, and arterial anatomy of the posterior mediobasal temporal lobe was studied in six formalin-fixed injected hemispheres. RESULTS: The SCTT approach provided a direct path to the posterior mediobasal temporal lobe and exposed the posterior parahippocampal gyrus as well as the adjacent gyri in all of the cadaveric specimens. Through this approach, gross total resection of the ganglioglioma was possible in our patient. CONCLUSION: The SCTT approach provided a viable surgical route to the posterior mediobasal temporal lobe in the cadaveric studies. This approach provides an advantage over the subtemporal and transtemporal routes in that there is less temporal lobe retraction.
PMID: 18424961 [PubMed - in process]
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Reversible deficit affecting the perception of tone of a human voice after tumour resection from the right auditory cortex.
Acta Neurochir (Wien). 2008 Apr 25;
Authors: Benzagmout M, Chaoui ME, Duffau H
We report on a young woman operated for a ganglioglioma involving the right auditory cortex (AC), presenting with auditory seizures. Despite a normal pre-operative examination, a specific post-operative disorder affecting the perception of a human voice occurred. The patient was unable to recognise the tone of familiar voices while she recognised the expressed content. A temporal lobectomy for recurrence was performed two years later. The patient recovered from the voice perception deficit. This report shows that (1) a discrete site within the AC is specifically involved in the perception of tone of the human voice (2) functional compensation is possible.
PMID: 18431530 [PubMed - as supplied by publisher]
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Gene expression profile analysis of epilepsy-associated gangliogliomas.
Neuroscience. 2008 Jan 2;151(1):272-92
Authors: Aronica E, Boer K, Becker A, Redeker S, Spliet WG, van Rijen PC, Wittink F, Breit T, Wadman WJ, Lopes da Silva FH, Troost D, Gorter JA
Gangliogliomas (GG) constitute the most frequent tumor entity in young patients undergoing surgery for intractable epilepsy. The histological composition of GG, with the presence of dysplastic neurons, corroborates their maldevelopmental origin. However, their histogenesis, the pathogenetic relationship with other developmental lesions, and the molecular alterations underlying the epileptogenicity of these tumors remain largely unknown. We performed gene expression analysis using the Affymetrix Gene Chip System (U133 plus 2.0 array). We used GENMAPP and the Gene Ontology database to identify global trends in gene expression data. Our analysis has identified various interesting genes and processes that are differentially expressed in GG when compared with normal tissue. The immune and inflammatory responses were the most prominent processes expressed in GG. Several genes involved in the complement pathway displayed a high level of expression compared with control expression levels. Higher expression was also observed for genes involved in cell adhesion, extracellular matrix and proliferation processes. We observed differential expression of genes as cyclin D1 and cyclin-dependent kinases, essential for neuronal cell cycle regulation and differentiation. Synaptic transmission, including GABA receptor signaling was an under-expressed process compared with control tissue. These data provide some suggestions for the molecular pathogenesis of GG. Furthermore, they indicate possible targets that may be investigated in order to dissect the mechanisms of epileptogenesis and possibly counteract the epileptogenic process in these developmental lesions.
PMID: 18093740 [PubMed - indexed for MEDLINE]
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Primary anaplastic ganglioglioma with a small-cell glioblastoma component.
Clin Neuropathol. 2008 Mar-Apr;27(2):91-5
Authors: Schittenhelm J, Reifenberger G, Ritz R, Nägele T, Weller M, Pantazis G, Capper D, Beschorner R, Meyermann R, Mittelbronn M
Gangliogliomas usually present as benign tumors corresponding to World Health Organization (WHO) Grade I. Very rarely, gangliogliomas show histological features of malignancy and are then classified as anaplastic gangliogliomas of WHO Grade III or IV. In most cases, anaplastic gangliogliomas developed after radiation therapy or progression from a pre-existing low-grade ganglioglioma. Here, we report the case of a 77-year-old male patient who was operated on a primary ganglioglioma with a highly anaplastic glial component corresponding to a small-cell glioblastoma. To our knowledge, this is the first reported case of a primary anaplastic ganglioglioma with a small-cell glioblastoma component.
PMID: 18402388 [PubMed - in process]
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Papillary glioneuronal tumor--contribution to a new tumor entity and literature review.
Clin Neuropathol. 2008 Mar-Apr;27(2):72-7
Authors: Guo SP, Zhang F, Li QL, Li Q, Wang WL, Li FF
Papillary glioneuronal tumor (PGNT) is a recently identified low-grade mixed glial-neuronal neoplasm of juvenile and young adult patients. The WHO classification does not categorize this tumor as a separate entity, but rather considers it as a variant of ganglioglioma. We present a new case of this rare entity, representing the 3rd case of this lesion in Chinese patients and review the findings in 34 patients from different case reports found in the international literature. This report describes a histologically similar-appearing lesion arising in the left frontoparietal lobe of a 23-year-old man. Its salient morphological characteristics are the presence of pseudopapillary structures composed of blood vessels, often hyalinized, lined by uniform small astrocytes and a proliferation of neurocytic cells which eventually admixed with ganglioid and ganglion cells. Sporadic Rosenthal fibers, foci of calcification, areas of hemosiderin deposition were identified. The mean Ki67 labeling index remained below 1%. Signs of anaplasia, in particular mitotic figures, endothelial proliferation or necrosis were consistently lacking. It is important that every new case of PGNT is reported to allow its recognition and classification. We perceive PGNT as a clinically and morphologically well-delineated subgroup of extraventricular low malignant potential neoplasm, whose presentation may allow for consideration as an entity.
PMID: 18402385 [PubMed - in process]
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Genetic alterations in a papillary glioneuronal tumor.
J Neurosurg Pediatrics. 2008 Jan;1(1):99-102
Authors: Faria C, Miguéns J, Antunes JL, Barroso C, Pimentel J, Martins Mdo C, Moura-Nunes V, Roque L
Papillary glioneuronal tumors (PGNTs) are rare lesions of the central nervous system, and no information exists on the genetic alterations in these neoplasms. The authors report on such a case in a child. Genetic studies revealed that the tumor was characterized by gains and structural alterations involving only chromosome 7 with breakpoints at 7p22. By using comparative genomic hybridization, the authors observed a high-level amplification region at 7p14~q12. Fluorescence in situ hybridization with a probe for EGFR revealed that this gene was not amplified. Similar to other patients with PGNTs, the patient in the present case fared well. From a genetic point of view the data in the present case are in accordance with previous findings of EGFR amplifications as uncommon in low-grade gliomas and gangliogliomas. Recurrent rearrangements of chromosome 7 have been noted in other mixed glioneuronal tumors. The data in this case suggest that genes located at chromosome 7 can also be involved in the pathogenesis of PGNT. In clinical terms it will be especially important to corroborate, through the analysis of further cases, the involvement of the chromosome 7p22 locus, a region where glial and neuronal linked genes (RAC1 and NXPH1) are known to be located.
PMID: 18352813 [PubMed - indexed for MEDLINE]
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[Multiple intracranial ganglioglioma.]
Neurochirurgie. 2008 Mar 18;
Authors: Lakhdar F, Laghmari M, Gana R, Maaqili R, Bellakhdar F
Multiple intracranial ganglioglioma (GG) is an extremely rare condition, generally associated with van Recklinghausen neurofibromatosis. We report the case of a 26-year-old woman who presented with progressive tetraparesis, raised intracranial pressure and visual loss. The neuroradiologic workup demonstrated a multiple lesion involving the temporal lobe, the diencephalomesencephalon and the optical pathways. Computed tomography-guided stereotactic biopsy of a right temporal lesion led to the diagnosis of GG. In light of this case and the literature review, we discuss morphologic aspects, differential diagnosis and therapeutic options of GG. In patients with multiple cerebral tumors, search for multiple ganglioglioma is particularly important because of the therapeutic implications and the good prognosis of this tumor.
PMID: 18355879 [PubMed - as supplied by publisher]
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Desmoplastic infantile ganglioglioma with a malignant course.
J Neurosurg Pediatrics. 2008 Jan;1(1):95-8
Authors: Hoving EW, Kros JM, Groninger E, den Dunnen WF
Desmoplastic infantile gangliogliomas (DIGs) are rare supratentorial tumors that arise in infancy. Despite the large size of these lesions, the prognosis is generally considered favorable after gross-total resection (GTR); however, in incidental cases tumor progression has been described. The authors report on a child harboring a DIG with a high-grade primitive cell population and glial differentiation. The progressive clinical course was determined by this cell component in spite of GTR and adjuvant chemotherapy. The significance of the presence of a high-grade primitive tumor component in the context of DIG is discussed.
PMID: 18352812 [PubMed - in process]
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Gliomas in neurofibromatosis type 1: a clinicopathologic study of 100 patients.
J Neuropathol Exp Neurol. 2008 Mar;67(3):240-9
Authors: Rodriguez FJ, Perry A, Gutmann DH, O'Neill BP, Leonard J, Bryant S, Giannini C
There are few pathologic studies of gliomas in patients with neurofibromatosis type 1. We analyzed clinical and pathologic features of gliomas from 100 neurofibromatosis type 1 patients (57 men; 43 women). The median age at tumor diagnosis was 13 years (range, 4 months to 68 years). Most tumors were typical pilocytic astrocytoma (PA) (49%) or diffusely infiltrating astrocytoma (DA) (27%) that included World Health Organization Grades II (5%), III (15%), and IV (7%); others were designated as low-grade astrocytoma, subtype indeterminate (LGSI; 17%). Two pilomyxoid astrocytomas, 1 desmoplastic infantile ganglioglioma and 1 conventional ganglioglioma, were also identified. The tumors in 24 cases arose in the optic pathways and included PA (n = 14), LGSI (n = 4), DA (n = 4), pilomyxoid astrocytoma (n = 1), and ganglioglioma (n = 1). The prognoses of the PA and LGSI gliomas overall were generally favorable; there were no survival differences between PA and LGSI groups based on site, tumor size, mitotic activity, or MIB-1 labeling index. In the combined PA and LGSI group, age younger than 10 years and gross total resection were associated with an increased overall survival rate (p = 0.047 and 0.002, respectively). Compared with the combined group (PA + LGSI), patients with DA at all sites had decreased overall and recurrence-free survival times (p < 0.001 and p = 0.003, respectively). This study emphasizes the wide histologic spectrum of gliomas that occur in patients with neurofibromatosis type 1. Classic PA and LGSI are the most common, and most have favorable prognoses. By contrast, DAs are more aggressive, similar to those that arise sporadically.
PMID: 18344915 [PubMed - in process]
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Ganglioglioma Associated with Cerebral Cortical Dysplasia: An Unusual Case with Leptomeningeal Involvement.
Pediatr Dev Pathol. 2008 Feb 25;:1
Authors: Saad A, Jayarao M, Chin L, Delalle I
Ganglioglioma is a tumor occurring in children and young adults and characterized by a superficial cortical location and biphasic histologic differentiation encompassing neuronal and glial elements. Ganglioglioma may arise anywhere throughout the neuraxis including the optic nerve, brain stem, pineal gland, cerebellum, cerebrum and spinal cord; however, the majority of glioneuronal neoplasms involve the temporal lobe. Gangliogliomas may show focal leptomeningeal involvement but predominant leptomeningeal involvement by gangliogliomas is extremely rare; only two cases of ganglioglioma extensively involving the leptomeninges have been reported. In this report, we present an unusual case of a ganglioglioma predominantly present within the leptomeninges of a 15 year-old boy with history of seizures. Furthermore, the cerebral cortex beneath the tumor showed dysplastic changes. We report a very unusual case of ganglioglioma involving predominantly the cerebral leptomeninges and associated with adjacent cerebral cortical dysplasia. Histologic characteristics and diagnostic pitfalls are discussed.
PMID: 18338935 [PubMed - as supplied by publisher]
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Additional hippocampectomy in the surgical management of intractable temporal lobe epilepsy associated with glioneuronal tumor.
Neurol Res. 2007 Dec;29(8):807-15
Authors: Morioka T, Hashiguchi K, Nagata S, Miyagi Y, Yoshida F, Shono T, Mihara F, Koga H, Sasaki T
In surgery for epileptogenic glioneuronal tumor in the temporal lobe, whether additional hippocampectomy is needed remains in dispute. We retrospectively analysed clinical profile and seizure outcome in a consecutive series of six patients, paying special attention to pathophysiologic conditions in the ipsilateral hippocampus. Long-term video electroencephalography (EEG) monitoring showed attenuation of background activity, followed by ictal discharges in the ipsilateral temporal region in five cases. (18)Fluorodeoxyglucose-positron emission tomography (FDG-PET) in five cases showed hypometabolism in the ipsilateral medial temporal lobe. Intraoperative electrocorticography (ECoG) after removal of the tumor revealed frequent paroxysmal activity or electrographic seizure activity on the hippocampus in five cases. A high incidence of hippocampal pathology, such as hippocampal sclerosis in four cases and dysgenesis in one case, was demonstrated. Five patients who underwent additional hippocampectomy along with resection of the tumor became completely seizure-free. Our findings indicated a proclivity for the epileptogenic zone to encompass the medial structures and for hippocampal pathology to be present even when no direct medial tumor involvement was identified. Thus, it is conceivable that removal of the hippocampus with the guidance of pre- and intraoperative multimodal examinations, in addition to resection of the tumor, may be recommended to achieve 'complete' freedom from seizures.
PMID: 17601368 [PubMed - indexed for MEDLINE]
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Malignant cerebellar ganglioglioma.
J Clin Neurosci. 2008 Feb 28;
Authors: Harding M, Brophy B, Geake T
We present a 62-year-old man with a high-grade cerebellar ganglioglioma with ataxia. Gangliogliomas are rare tumours which usually occur in the first 3 decades of life. There have only been a small number of grade IV gangliogliomas reported in the literature.
PMID: 18313924 [PubMed - as supplied by publisher]
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Mechanisms of epileptogenesis in tuberous sclerosis complex and related malformations of cortical development with abnormal glioneuronal proliferation.
Epilepsia. 2008 Jan;49(1):8-21
Authors: Wong M
Malformations of cortical development (MCDs) are increasingly recognized as causes of medically intractable epilepsy. In order to develop more effective, rational therapies for refractory epilepsy related to MCDs, it is important to achieve a better understanding of the underlying mechanisms of epileptogenesis, but this is complicated by the wide variety of different radiographic, histopathological, and molecular features of these disorders. A subset of MCDs share a number of characteristic cellular and molecular abnormalities due to early defects in neuronal and glial proliferation and differentiation and have a particularly high incidence of epilepsy, suggesting that this category of MCDs with abnormal glioneuronal proliferation may also share a common set of primary mechanisms of epileptogenesis. This review critically analyzes both clinical and basic science evidence for overlapping mechanisms of epileptogenesis in this group of disorders, focusing on tuberous sclerosis complex, focal cortical dysplasia with balloon cells, and gangliogliomas. Specifically, the role of lesional versus perilesional regions, circuit versus cellular/molecular defects, and nonneuronal factors, such as astrocytes, in contributing to epileptogenesis in these MCDs is examined. An improved understanding of these various factors involved in epileptogenesis has direct clinical implications for optimizing current treatments or developing novel therapeutic approaches for epilepsy in these disorders.
PMID: 17727667 [PubMed - indexed for MEDLINE]