Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 48-year-old woman was admitted to the Neurosurgery Department for a large frontal lobe tumor revealed by partial seizures. The patient was conscious and alert. Neurological examination was normal. MRI study showed a right frontal lobe tumor compounded of an anterior solid mass strongly enhanced after gadolinium injection and a posterior voluminous cyst with important mass effect. The cerebral blood volume (CBV) map showed no area of elevated CBV within the tumor consistent with a low-grade tumor. The patient was operated on with a presumed diagnosis of anaplastic oligodendroglioma. Postoperative course was uneventful. Histopathological examination was consistent with a benign ganglioglioma. The patient did not undergo an additional treatment. One year later, the patient was healthy and neurological and neuropsychological examination were normal. MRI study did not show any recurrence. This case emphazises the relevance of perfusion MR imaging in the preoperative workup of glioneuronal and glial tumors.
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PMID:[Contribution of perfusion magnetic resonance imaging in a patient with cerebral ganglioglioma]. 1248 21

Gangliogliomas are tumors of mixed glial and neuronal phenotype that usually have a benign clinical course. Rare cases display anaplastic features at the time of first presentation or progress to anaplastic gliomas over extended times. We report on a ganglioglioma of the spinal cord that recurred as a malignant glioma one and a half years after resection. The initial neoplasm was composed of a mixture of well-differentiated ganglionic and astrocytic cells. The recurrent tumor was an anaplastic small-cell glioma. The sole unusual aspect in the initial neoplasm was an abundance of small vessels with calcified walls, which mimicked a vascular malformation.
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PMID:Malignant transformation of a spinal cord ganglioglioma--case report and review of the literature. 1558 15

Doublecortin (DCX) is required for neuroblastic migration during the development of the cerebral cortex. DCX is a microtubule-associated protein that plays a role in cellular motility. These facts led us to hypothesize that DCX is increased in invasive brain tumors. DCX expression was assessed in 69 paraffin-embedded brain tumors of neuroepithelial origin. In addition, mouse brain sections of the subventricular zone and dentate gyrus were used as positive controls for immunostaining, and specificity of antibody staining was demonstrated by peptide neutralization. DCX was highly expressed in both high-grade invasive tumors (glioblastoma, n=11; anaplastic astrocytoma/oligoastrocytoma, n=7; and medulloblastoma/PNET, n=6) and low-grade invasive tumors (oligodendroglioma, n=3; and astrocytoma/oligoastrocytoma, n=5). However, DCX was less intensely expressed in the circumscribed group of tumors (pilocytic astrocytoma, n=6; ependymoma/subependymoma, n=7; dysembryoplastic neuroepithelial tumor, n=4; ganglioglioma, n=2; meningioma, n=9; and schwannoma, n=9). By the Cochran-Mantel-Haenszel statistical test, the circumscribed group was significantly different from both the high-grade invasive group (P<0.0001) and the low-grade invasive group (P<0.0001). We conclude that DCX is preferentially expressed in invasive brain tumors. In addition, DCX immunostaining was stronger at the margin of the tumor than at the center. For a subset of these tumors, we also detected DCX mRNA and protein by Northern and Western blotting. DCX mRNA and protein was detected in glioma cell lines by Northern blotting, immunofluorescence microscopy and Western blotting. Collectively, the immunohistochemistry, Western blots and Northern blots conclusively demonstrate expression of DCX by human brain tumors.
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PMID:Doublecortin is preferentially expressed in invasive human brain tumors. 1619 16

Desmoplastic infantile ganglioglioma is a supratentorial tumor that typically occurs in infants below the age of 24 months. Rare tumors with the same radiological and histological characteristics have been described in older subjects. We report a case of desmoplastic ganglioglioma in a 12-year-old girl with a 13 years follow-up. The patient presented with an inaugural generalized seizure. CT scan demonstrated a large superficial parieto-occipital mass, attached to the dura with solid and cystic components. Surgical resection was macroscopically complete. No adjuvant treatment was given. Thirteen years after surgery, the patient is symptom free. Histological examination revealed a pleomorphic tumor involving the meningeal space and the cortex. Meningeal portion was made of neoplastic astrocytes enmeshed in a dense network of connective tissue. The cortical component showed abnormal neurons, tumoral astrocytes and small foci of poorly differentiated cells with rare mitoses. Our observation and the seven others found in the literature indicate that desmoplastic gangliogliomas can occur in children and even in young adults. Despite some worrisome radiological and histological features, these tumors should not be misdiagnosed as malignant glioma. Like infantile cases, non-infantile desmoplastic gangliogliomas seem to have a favorable prognosis without additional therapy, if a total surgical resection can be performed.
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PMID:A report of a desmoplastic ganglioglioma in a 12-year-old girl with review of the literature. 1620 62

Desmoplastic infantile ganglioglioma is a rare intracranial tumor of infancy, characterized by solid and cystic component, voluminous size and supratentorial location. These tumors are diagnosed usually below the age of 2 years. We report 1 case of desmoplastic ganglioglioma in 13-year-old male. Computed tomography and magnetic resonance imaging diagnosed supratentorial mixed cystic and solid tumor, which presented as a large cystic component with intense contrast enhancement of a mural nodule. The tumor was surgically removed, and histology revealed desmoplastic ganglioglioma. The patient had a good follow up. This observation emphasizes the possibility of desmoplastic ganglioglioma in older infants. It mustn't be considered as a specific entity of very young age infant and must be recognized in older infant because it may be misdiagnosed as malignant glioma. Despite the pseudo malignant appearance, these tumors have a good prognosis after surgery and when excision is complete they don't led to recurrences.
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PMID:[Magnetic resonance imaging features of desmoplastic cerebral ganglioglioma of infancy: report of 1 case]. 1636 14

We describe the cases of 2 patients with a congenital malignant glioma that responded to chemotherapy. In the first case, a 2-month-old boy had a conjugate deviation to the right side and nystagmus. A T(1)-weighted gadolinium-enhanced magnetic resonance image showed a large tumor in his right frontal lobe. The tumor was partially resected, and the histological diagnosis was malignant ganglioglioma. The child then underwent 6 cycles of chemotherapy (mainly carboplatin and etoposide), and the residual tumor shrank. The tumor was then partially resected during a second operation, after which the patient underwent 5 cycles of chemotherapy (a combination of carboplatin, etoposide, vincristine, ifosfamide, cisplatin and cyclophosphamide). The tumor has not recurred in more than 8.5 years. In the second case, a 2-month-old boy had bulging of the anterior fontanel. The T(1)-weighted gadolinium-enhanced magnetic resonance image showed a large suprasellar tumor. The tumor was partially resected, and the histological diagnosis was anaplastic astrocytoma. The patient underwent 8 cycles of chemotherapy (MCNU, carboplatin and etoposide) and the tumor has not recurred in more than 7.5 years. Our experience indicates that, if surgical removal and chemotherapy are done aggressively for malignant gliomas in neonates and infants, long-term survival is possible.
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PMID:Successful chemotherapy for congenital malignant gliomas: a report of two cases. 1671 66

Ganglioglioma is a tumour containing both astrocytic and neuronal components. Most gangliogliomas are observed in the brain, but may also manifest as a nasal glioma. Approximately 250 cases of nasal gliomas have been described in the literature. Gliomas are classified as heterotopias of glia tissue. In the paper we describe the case of nasal ganglioglioma and the diagnostic difficulties. The differences between ganglioglioma, nasal glioma and other congenital midline nasal masses are discussed.
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PMID:Nasal ganglioglioma--difficulties in radiological imaging. 1809 65

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.
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PMID:MRI of long-term epilepsy-associated tumors. 1838 6

Epilepsy surgery has been proposed as a safe alternative treatment for intractable epilepsy in children, especially for patients with structural brain abnormalities. We studied 24 consecutive children who underwent surgery for intractable epilepsy. There were 12 males and 12 females. The mean age was 6.5 years. The seizures' duration ranged from 6 months to 2 years. The histopathological examination of the resected lesions revealed in 12 cases the presence of a ganglioglioma, in 7 cases dysembryoplastic neuroepithelial tumor, in 1 case a low grade glioma, in 2 cases cortical dysplasia, and in 2 cases cavernous malformations. In 18 cases, the lesions were located in the temporal lobe and in 6 cases the lesions were extratemporal. After a mean follow-up period of 4.4 years, 79% (19/24) of patients were seizure free. There were no permanent neurological deficits or deaths. Surgery for focal epilepsy in children is a safe procedure with favorable results.
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PMID:Benign lesions accompanied by intractable epilepsy in children. 1928 94

After successfully using cisplatin (30 mg/m(2)/day) and etoposide (150 mg/m(2)/day) in ten three-day courses for progressive low-grade gliomas, a subsequent protocol reduced the daily doses of cisplatin (to 25 mg) and etoposide (to 100 mg), with the objective of achieving the same response and three-year PFS rates with lower neurotoxicity and myelotoxicity. We treated 37 patients (median age 6 years); 23 had optochiasmatic tumours and nine were metastatic cases. Diagnoses were clinical in 13 cases and histological in 24, and comprised: pilocytic astrocytoma (17), ganglioglioma (3), pilomyxoid astrocytoma (2), and fibrillary astrocytoma (2). Treatment was prompted by radiological evidence of progression and/or clinical deterioration a median 18 months after the first diagnosis. After initial MRI staging, neurological and clinical examinations were performed before each chemotherapy cycle, with MRI after the first three courses and every three months thereafter. After a median 48 months, a volume reduction was appreciable in 24 cases (65%) and response was maximum 12 months after starting treatment. The three-year EFS and OS rates were 65 and 97%, respectively. Clinical, neurological, or functional improvements were seen in 26/37 cases. No children had a WBC nadir below 2,000/mm(3). Audiological toxicity caused damage in 4/34 cases. The previous protocol had achieved volume reductions in 70% of cases, causing audiological damage (data updated) in 11/31 (P = 0.023), with three-year PFS and OS rates of 70 and 100%, respectively. Lower doses of cisplatin/etoposide are still effective in progressive low-grade glioma, with less acute and persistent morbidity.
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PMID:A lower-dose, lower-toxicity cisplatin-etoposide regimen for childhood progressive low-grade glioma. 2015 Nov 74


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