Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of unexpected death in persons with epileptic seizures due to a brain tumor are presented which encompassed an astrocytoma WHO grade II and an anaplastic astrocytoma WHO grade III. A 35-year-old man was found somnolent and disoriented at home. A computed tomography (CT) scan revealed a tumor of the right frontal lobe suggestive for an oligodendroglioma. During an angiographic examination the patient experienced an epileptic seizure. Some weeks later, the man was found dead in front of his house with a fresh bite mark of the tongue. Neuropathological examination revealed an astrocytoma WHO grade II of the right frontal lobe. A 47-year-old man plunged into a swimming-pool and was found submerged some minutes later. After resuscitation he survived comatose for 8 days but finally died due to severe hypoxic brain damage. He had been operated on a brain tumor of the temporal lobe 1 year before the accident. Neuropathological examination revealed residual tumor tissue at the operation site corresponding to an anaplastic astrocytoma WHO grade III. Although rare, death in persons with epileptic seizures due to brain tumors is an important mechanism of death encountered by the forensic pathologist.
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PMID:Unexpected death in persons with symptomatic epilepsy due to glial brain tumors: a report of two cases and review of the literature. 1035 81

Astrocytic tumors, particularly gliosarcoma, may contain epithelial features in the form of trabecular, adenoid, papillary arrangement, and squamous metaplasia. A case of gliosarcoma with unusual epithelial feature is described. The patient was a 60-year-old male with frequent seizures. The mass was 4 cm and in the left frontal lobe. Trabecular or rarely adenoid arrangement of neoplastic astrocytes was present in the mucinous stroma, and there was a distinctive transition between the trabecular area and typical anaplastic astrocytoma. The tumor cells in the trabecular area showed positive immunostain for glial fibrillary acidic protein, but did not react with various kinds of cytokeratin. The sarcomatous area was undifferentiated and was not labeled by factor-VIII, desmin, and anti-smooth muscle actin. Occurrence and histogenesis of epithelial features in gliosarcoma are reviewed. The importance to recognize the existence of epithelial feature in malignant astrocytic tumor is emphasized.
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PMID:Gliosarcoma: a case with unusual epithelial feature. 1040 83

The preoperative history and postoperative course of histologically verified 348 low-grade and 383 anaplastic astrocytomas have been reviewed. In 71.2 and 48.0% of patients epilepsy was the initial symptom of a suspected astrocytoma, and the history was longer than 3 years in 28.1 and 19.5% of cases, respectively. Before the advent of CT, angiography was performed because of a suspected tumour 2-9 years before surgery in 34 cases. The second angiography years later demonstrated the tumour which in 18 cases at surgery proved to be low-grade astrocytoma or anaplastic astrocytoma in 16 cases. During the last 10 years CT or MRI demonstrated a low-density lesion in 21 patients years before surgery. Operation was postponed for different reasons. Ten tumours appeared at 'delayed' surgery as low-grade, but 11 as anaplastic astrocytoma. In the same period 29 further patients were operated on after a history of seizures, longer than 3 years. Histology showed anaplastic astrocytoma in 10 cases also. Malignant transformation occurred nearly in half of the patients during the observation period. This strongly suggests that dedifferentiation is a spontaneous process, an intrinsic feature of astrocytomas and does not depend on any kind of external stimulus. Another 51 patients' surgery was performed following a shorter (1-24 months) history of epilepsy. The 5-year survival rate was 44 and 39.5% Median survival times (53.5 and 51 months) did not show a significant difference between the two groups, but the total survival, including second survivals after reoperation displayed a significant difference (57.5 vs 67.5 months) in favour of patients with a shorter history of seizures. These experiences confirm the difficulties in decision of the time of surgery. Considering the frequent malignant transformation among patients with a long history of seizures, followed by a relatively shorter survival, it may be supposed that an early radical removal in suitable cases might prevent the late dedifferentiation and recurrence.
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PMID:Preoperative history and postoperative survival of supratentorial low-grade astrocytomas. 1056 42

We report a surgical case of unusual anaplastic astrocytoma which was accompanied by an exuberant proliferation of abnormal blood vessels with features resembling those of capillary telangiectasis or cavernous angioma. The patient was a 39-year-old man, who presented with a generalized convulsive seizure, and neuroradiological examination revealed a tumor in the left frontal lobe. The resected tumor showed the features of an anaplastic astrocytoma, grade 3. The proliferation of abnormal blood vessels with dilated lumina and thin walls was seen throughout the tumor, and in the central area these vessels were densely packed and almost replaced the neoplastic astrocytic tissue. Although these dense vascular aggregates in the central area closely simulated capillary telangiectasis or cavernous angioma, they were considered to be of a reactive nature. The term "angiomatous astrocytoma", which is analogous to angiomatous meningioma, seemed to be the most appropriate for the present tumor.
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PMID:Astrocytoma with angiomatoid vascular proliferation ("angiomatous astrocytoma"). 1091 49

Between December 1996 and September 1998, 13 patients with advanced recurrent malignant brain tumors (9 with glioblastoma multiforme, 1 with gliosarcoma, and 3 with anaplastic astrocytoma) were treated with a single intratumoral injection of 2 x 10(9), 2 x 10(10), 2 x 10(11), or 2 x 10(12) vector particles (VP) of a replication-defective adenoviral vector bearing the herpes simplex virus thymidine kinase gene driven by the Rous sarcoma virus promoter (Adv.RSVtk), followed by ganciclovir (GCV) treatment. The VP to infectious unit ratio was 20:1. Our primary objective was to determine the safety of this treatment. Injection of Adv.RSVtk in doses <==2 x 10(11) VP, followed by GCV, was safely tolerated. Patients treated with the highest dose, 2 x 10(12) VP, exhibited central nervous system toxicity with confusion, hyponatremia, and seizures. One patient is living and stable 29.2 months after treatment. Two patients survived >25 months before succumbing to tumor progression. Ten patients died within 10 months of treatment, 9 from tumor progression and 1 with sepsis and endocarditis. Neuropathologic examination of postmortem tissue demonstrated cavitation at the injection site, intratumoral foci of coagulative necrosis, and variable infiltration of the residual tumor with macrophages and lymphocytes.
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PMID:Phase I study of adenoviral delivery of the HSV-tk gene and ganciclovir administration in patients with current malignant brain tumors. 1093 31

Mixed glioneuronal neoplasms are relatively uncommon tumors in the central nervous system. Recently, an unusual glioneuronal tumor arising in adults marked histologically by neuropil-like islands was described. We present a similar case arising in a 23-year-old woman who presented with headaches and seizures and on imaging studies was noted to have a frontal-temporal lobe mass. The patient underwent partial resection of the tumor, which histologically resembled anaplastic astrocytoma, and received a course of radiation therapy and chemotherapy. Increasing seizure frequency and expanding size on neuroimaging prompted a re-excision of the tumor. The second resection was marked by islands of tissue resembling gray matter with slightly atypical neuronal and glial cells situated in the white matter. These islands stained positively with synaptophysin and did not stain with glial fibrillary acid protein. Mild vascular proliferation and moderate nuclear pleomorphism also characterized the tumor. Areas of necrosis were not noted. A MIB-1 labeling index of 18.1% was noted. P53 immunoreactivity was observed in approximately 40% of tumor cell nuclei. This lesion is felt to represent a clinically aggressive glioneuronal neoplasm with an unusual and distinctive histologic phenotype. HUM PATHOL 31:1435-1438.
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PMID:Glioneuronal tumor with neuropil-like islands. 1111 23

The nevus sebaceus syndrome (NSS) is a neurocutaneous disorder characterized by unilateral hyperplasia of skin appendages and skeletal hemihypertrophy, hemimegalencephaly, or hemiatrophy along with disabling seizures. Despite the proneness of the dermal stigmata to eventually undergo neoplastic transformation, the malformative lesions of the central nervous system rarely evolve into frank tumors. We present the case of a 10-year-old girl with left-sided sebaceus nevi, ipsilateral enlargement of the skull, and a desmoplastic neuroepithelial tumor (DNET) in the right fronto-parietal area of the brain. The tumor was removed by surgery. Histologically, it corresponded to a mitotically active small-cell anaplastic astrocytoma with genuine desmoplasia. Investigative methods included immunohistochemical positivity for glial fibrillary acidic protein, lack of expression of neuronal markers, and ultrastructural documentation of sheaths of basal lamina and collagen around tumor cells. A survey of the literature of brain tumors associated with NSS revealed two cases of histologically verified pilocytic astrocytomas, and one each of a choroid plexus papilloma, a mixed glioma, and a meningioma, as well as a subependymal giant cell astrocytoma--the latter possibly in an overlap syndrome of NSS and tuberous sclerosis. We hypothesize that the tumor described herein, one involving both atypical differentiation and enhanced growth potential, is paradigmatic of neuropathological events to be expected in the NSS.
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PMID:Desmoplastic neuroepithelial tumor of infancy in the nevus sebaceus syndrome: report of a unique constellation and review of the literature. 1135 15

We report a case of a 44-years-old woman with relapsing-remitting and secondarily progressive form of multiple sclerosis (MS) since aged 24 years, who developed an anaplastic astrocytoma. The neurological manifestations of the tumor were misinterpreted as resulting from MS. Sequential MRI examination and seizures raised the possibility of another nature of her symptoms, besides MS. Her initial good response to high doses corticosteroids led to the initial assumption her symptoms were only exclusively due to the demyelinating process. She underwent craniotomy with radical excision of the lesion. Pathological examination disclosed anaplastic astrocytoma. Other cases of coincidental MS and primary CNS tumors are reviewed, as well as their possible relation.
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PMID:Glioma and multiple sclerosis: case report. 1213 52

Sodium phenylbutyrate is a biological-response modifier that acts as a dose-dependent inhibitor of glioma cell proliferation, migration, and invasiveness in vitro, possibly by inhibition of urokinase and c-myc pathways. Despite its biological activity in vitro, there have not been any prior reports of efficacy in the treatment of human malignant gliomas. We report a 44-year-old female with a recurrent, multicentric, malignant glioma who experienced a durable remission lasting more than four years. The patient initially presented with seizures caused by a biopsy-proven anaplastic astrocytoma of the frontal lobe. The patient was treated with radiation therapy and Procarbazine-CCNU-Vincristine (PCV). However, the tumor progressed and extended to the corpus callosum with midline shift, refractory to four cycles of continuous 72-h infusion of BCNU/Cisplatinum. Additional enhancing lesions appeared in the left frontal and left temporal lobes. The patient was started on sodium phenylbutyrate, 18 g daily in three divided oral doses, and reduced to 9 g/day and eventually to 4.5 g/day to eliminate mild, reversible side effects. Four years later, the patient has a KPS functional score of 100%. Phenylbutyrate is a well-tolerated, oral agent that shows potential for the treatment of malignant gliomas. Further studies should be considered to identify a subset of patients that have tumors sensitive to phenylbutyrate, either as a single agent or in combination with radiation therapy or other chemotherapeutic agents.
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PMID:Complete response of a recurrent, multicentric malignant glioma in a patient treated with phenylbutyrate. 1501 93

We describe a case of anaplastic astrocytoma in a 14-year-old boy arising at the site of a dysembryoplastic neuroepithelial tumor (DNT) 3 years after combined radiation and chemotherapy. The subtotally excised superficial right temporoparietal tumor was originally diagnosed as mixed oligoastrocytoma in 1974; the patient was treated with radiation therapy postoperatively. One year later he underwent a craniotomy to remove cyst fluid and no change was reported in the size of the residual tumor. Postoperatively, he received a 6-week course of chemotherapy (lovustine, CCNU). He remained clinically and radiographically stable until 3 years later, when seizure activity returned and imaging studies were consistent with tumor recurrence. He was lost to follow-up until 1986, when records showed that he had died. Review of the initial biopsy showed cortical fragments containing abundant calcifications and multinodular structures typical of the complex form of DNT, in addition to specific glioneuronal elements. The Ki-67 labeling index ranged from 0.1% to 3% focally. The specimen from the third surgery showed an anaplastic astrocytoma (Ki-67 up to 12%) and morphologic features characteristic of radiation effect. This is the first documented case of malignant transformation of DNT following radiation and adjuvant chemotherapy. The implications of malignant transformation in subtotally excised complex DNTs and the intriguing issue of the contribution of radiation/chemotherapy are discussed.
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PMID:Malignant transformation of a dysembryoplastic neuroepithelial tumor after radiation and chemotherapy. 1291 47


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