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 pleomorphic xanthoastrocytoma (P X A) of young subjects (Kepes et al., 1979) are reported. Case 1 arose in 15-year-old boy admitted to the hospital with the complaint of severe headaches associated with nausea and vomiting of 1 month's duration. Computed tomographic scans showed a large well-defined low density area in the left temporo-parietal region of which an anterior portion was enhanced by contrast medium. Craniotomy revealed a large superficial and cystic tumor with a mural nodule. Histological and immunohistochemical features were those of a P X A confirmed by an electron microscopic study. No radiotherapy was given. The patient made a complete recovery, and 32 months later was asymptomatic. Case 2, a 17-year-old boy was admitted to the hospital in 1977. He presented with seizures that started 18 months prior to surgery. Carotid and humeral angiograms and air studies indicated the presence of a right, internal temporal mass with herniation. The craniotomy revealed a firm superficial tumor with an infratentorial, extraparenchymal extension. The histological diagnosis was giant cell glioblastoma or gliosarcoma. The patient received post-operative radiation of 5.500 rads and chemotherapy (CCNU and VM 26). He died on the 7th post-operative month. In this 2nd case, the diagnosis of P X A was made retrospectively based upon histological and immunohistochemical observations similar to case 1. We are aware of 24 P X A in the literature. In their clinical and histological features these neoplasms resemble closely each other. P X A are superficial, supratentorial astrocytomas occurring in youngs subjects (ages 3 to 32). Their typical microscopic structure include a marked cellular pleomorphism with bizarre giant cells, some mitotic figures and no necrosis. Many cells contain lipid and hyalin droplets in their cytoplasm. Characteristically, the tumoral stroma contain a very rich reticulin fiber network. Immunoperoxidase technique reveal glial fibrillary acidic protein in the tumor cells. Electron microscopic studies demonstrate abundant intracytoplasmic glial filaments. Individual cells or group of cells are surrounded by a prominent basal lamina. Some hemidesmosomes or primitive attachments are seen at the margins of the tumor cells. The biological behaviour of PXA with or without radiotherapy is relatively favorable. Long survival times (up to 25 years) are reported but in 5 cases, P X A follow a less favorable course with malignant transformation and death. Morphologic and immunohistochemical studies support the subpial astrocytic origin of P X A.
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PMID:[Xanthoastrocytoma inf young subjects. Review of the literature apropos of 2 cases with discordant courses]. 389 48

This series reports a pilot study of chemotherapy conducted for newly diagnosed and relapsing patients with oligodendrogliomas and oligoastrocytomas. Twenty-three patients with clinically or histologically aggressive tumours were eligible to receive procarbazine CCNU and vincristine (PCV) chemotherapy followed by radiotherapy in previously non irradiated patients. Sixteen (69%) responded to chemotherapy with complete responses in two patients and partial responses in 14. Previously irradiated patients were as likely to respond to PCV as those previously non irradiated. An over 1-year history of seizures was the main clinical prognostic factor of response. All toxicities were manageable and no treatment related deaths occurred. Chemotherapy is an effective treatment in aggressive oligodendrogliomas. Further studies must assess the role of chemotherapy in the multidisciplinary management of oligodendroglioma.
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PMID:Chemotherapy for aggressive or anaplastic high grade oligodendrogliomas and oligoastrocytomas: better than a salvage treatment. 1101 83

Low-grade gliomas are uncommon primary brain tumors classified as histologic grades I or II in the World Health Organization (WHO) classification. The most common variants are pilocytic and low-grade astrocytomas, oligodendrogliomas, and mixed oligo-astrocytomas located in the cerebral hemispheres. Prognostic factors that predict progression-free and overall survival include young age, pilocytic histology, good Karnofsky performance status, gross total resection, lack of enhancement on imaging, and small preoperative tumor volumes. Edema and vasogenic effects are typically managed with corticosteroids. Dexamethasone is given at an initial dosage of 4 mg given four times daily. Anticonvulsants are given prophylactically after resection and for patients who present with seizures. The rationale for open craniotomy depends on the need for immediate palliation of symptoms by reduction of intracranial pressure or focal mass effect, and/or improved oncologic control. Gross total resection of tumor is generally defined as the absence of residual enhancement on contrast-enhanced postoperative MRI scan. Most retrospective studies suggest that patients who have undergone a gross total resection of tumor have improved survival. Depending upon the proximity of the tumor to eloquent brain, gross total resection may or may not be possible. In these cases a stereotactic biopsy is required to provide the histologic diagnosis. Adjuvant radiotherapy is recommended for patients with incompletely resected grade II tumors or for patients older than age 40 regardless of extent of resection. It may be considered for any pilocytic astrocytoma from which a biopsy has been performed. Phase III randomized prospective trials have shown statistically significantly improved progression-free survival at 5 years with the addition of radiotherapy, though overall survival does not appear different. Based on prospective randomized phase III trials, 50.4 Gy to 54 Gy of conventionally fractionated radiotherapy appears to be a safe and effective regimen with minimal neurotoxicity; 45 Gy may be adequate for biopsied pilocytic astrocytomas. Currently, RTOG trial 98-02 is investigating the efficacy of postradiation PCV chemotherapy (procarbazine, CCNU, and vincristine) in the treatment of newly diagnosed unfavorable low-grade gliomas. Other areas of investigation include Temozolomide chemotherapy and the association of 1p and 19q chromosomal deletions with prolonged survival in oligodendrogliomas and sensitivity to PCV chemotherapy. Radiosurgery and/or experimental chemotherapy may provide some measure of local control in the recurrent disease setting.
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PMID:Low-grade gliomas. 1205 95

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

We collected 6 case-reports of symptomatric non removable low grade fibrillary astrocytoma of adults treated with a procarbazine-CCNU-vincristine chemotherapy regimen. All patients had drug-resistant epilepsy but brain imaging was stable. Total gross resection was rejected because of Volume or tumor location. After 4 to 7 cycles of chemotherapy, 2 patients had partial response and one minor response on brain MRI. All of them were seizure-free. Progression free survival was not reached at 5 Years. Up-front chemotherapy for low-grade astrocytomas may be useful and has to be prospectively evaluated.
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PMID:[Neoadjuvant chemotherapy for symptomatic non operable grade II fibrillary astrocytoma in adults]. 1526 67

Making treatment decisions for patients with infiltrating low-grade gliomas (LGGs) is challenging. Patients frequently present with seizures and usually have little or no neurologic deficit. In this younger and relatively well patient population, despite the potential for significant morbidity, we believe that surgical resection, radiation therapy, and chemotherapy each play an important role in the optimal management of these tumors. Randomized clinical trials have begun to address some of the many questions about prognosis, natural history, and treatment, but most questions have yet to be answered. We believe that, when possible, a maximal surgical resection consistent with preservation of neurologic function should be performed, even though it is likely that no randomized clinical trial will ever be done to demonstrate a survival advantage for this approach. External beam radiation therapy is most often given to a total dose of 50.4 or 54 Gy in 1.8-Gy fractions. The role of chemotherapy is less certain, but a growing body of evidence suggests that temozolomide, a generally well-tolerated drug, is active in the treatment of LGGs. In recent years, loss of heterozygosity of chromosome 1p and 19q, as well as silencing of the MGMT gene, have been identified as promising predictors of response to adjuvant therapy in gliomas. Although randomized trials have not yet shown a survival benefit for early radiation therapy or chemotherapy, one study by the European Organisation for Research and Treatment of Cancer did show an improvement in time to tumor progression with the earlier use of radiation therapy. In addition, a trial by the Radiation Therapy Oncology Group (soon to be analyzed and reported) is comparing radiation alone with radiation followed by a year (six cycles) of standard-dose PCV chemotherapy (procarbazine, CCNU, and vincristine); this trial may shed light on the use of chemotherapy in conjunction with radiation therapy for the initial treatment of LGGs. Because patients remain at risk for tumor progression for the remainder of their lives, we recommend lifelong follow-up with MRI scans, even for patients without documented tumor regrowth over long intervals. To give clinicians a more solid basis for guiding therapy recommendations, we encourage participation in large cooperative group clinical trials.
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PMID:Low-grade gliomas in adults. 1857 14

A 9 year-old, neutered, male French Bulldog showing cluster seizures was diagnosed with a glioma in the right piriform cortex by MRI. Hypofractionated radiation therapy (RT) was performed using a linear accelerator. Although the lesion had involuted significantly at 2 months after RT, recurrence was observed at 4 months after RT. Chemotherapy was started using CCNU (60 mg/m(2) every 6-9 weeks) and was continued for one year. Follow-up MRI revealed involution of the lesion and the intervals of CCNU were increased to every 9-14 weeks. Two years after the first presentation, the dog suffered status epilepticus, followed by deficits of left sided postural reaction with cognitive dysfunction. The dog died on day 910, and histopathological diagnosis confirmed anaplastic oligodendroglioma.
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PMID:Long-term survival in a dog with anaplastic oligodendroglioma treated with radiation therapy and CCNU. 2278 44