1/38. Secondary supratentorial anaplastic astrocytoma following treatment of medulloblastoma.The development of secondary tumours is a rare but well known late effect of radiation therapy of lesions in the central nervous system. Most radiation-induced tumours are of mesenchymal origin, but on rare occasions gliomas can occur. We describe a patient in whom a supratentorial anaplastic astrocytoma developed 15 years after surgery and radiotherapy for a childhood posterior fossa medulloblastoma. A concise review of the pertinent literature is given.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/38. Use of a portable CT scanner during resection of subcortical supratentorial astrocytomas of childhood.The development of intraoperative imaging has made it possible to visualize shifting brain structures during surgery, and may allow greater intraoperative discrimination of normal and abnormal tissue. This may provide greater confidence to the neurosurgeon to proceed with a more extensive resection while decreasing postoperative morbidity. We investigated the intraoperative use of a portable CT scanner in the resection of 4 cases of supratentorial, subcortical astrocytomas of childhood to assess its usefulness in determining the endpoint of the dissection. We operated on 4 patients, ages 3-17, with astrocytomas. Three were thalamic, and 1 was based primarily in the caudate nucleus. The approach to the basal ganglia was transcallosal in 3 and transtemporal in 1. Specific observations on the intraoperative use of the portable CT scanner included its overall facility, any additional operative time required, the overall quality of the images, intraoperative decisions made based on the images and problems associated with its use. These observations are presented with a review of intraoperative imaging as it pertains to deep pediatric brain tumors. The CT scanner was helpful in limiting the dissection of the hypothalamic and midbrain regions and in localizing remaining abnormal tissue. The scans allowed informed decisions about leaving margins of the tumor which were adjacent to vital structures, but dit not prove to be a decisive factor in providing a complete resection. The following observations are worth noting: (1) average imaging time was 20 min per scan; (2) the extent and location of residual enhancing tumor was easily identified despite other materials in the surgical bed; (3) air/tissue interfaces limit resolution; (4) tumors retain contrast long enough to obtain multiple scans without additional dye, and (5) the cost profile of a mobile CT scanner is superior to that of a fixed intraoperative CT scanner.- - - - - - - - - - ranking = 0.70505384085021keywords = brain (Clic here for more details about this article) |
3/38. High F-18 FDG uptake in a low-grade supratentorial ganglioma: a positron emission tomography case report.PURPOSE: Positron emission tomography (PET) with fluorine-18 fluorodeoxyglucose (F-18 FDG) is used for the noninvasive monitoring and grading of primary brain tumors. Here the FDG uptake is positively correlated with the malignant extent of the lesion and thereby negatively correlated with patient survival. Little is known about the FDG PET features of primary brain tumors in children, such as mixed neuronal-glial tumors. methods: The authors describe a 13-year-old boy who had partial complex seizures since early childhood caused by a brain tumor in the left temporal lobe. RESULTS: Magnetic resonance and computed tomographic examinations yielded uncharacteristic results: mixed density, marked calcifications, little contrast enhancement, a nearly absent mass effect, and no edema. The FDG PET scan revealed a large hypermetabolic tumor, with a tumor: contralateral gray matter FDG uptake ratio of 1.45. In contrast to this intense hypermetabolism, the pathologic analysis after gross total resection revealed a low-grade ganglioglioma (WHO grade 1), which is usually associated with an excellent prognosis. CONCLUSIONS: Mixed neuronal-glial tumors such as gangliogliomas must be considered in making differential diagnoses by judging hypermetabolic FDG PET scans in young patients with brain tumors in the presence of uncharacteristic imaging features.- - - - - - - - - - ranking = 0.94007178780028keywords = brain (Clic here for more details about this article) |
4/38. Desmoplastic neuroepithelial tumor of infancy in the nevus sebaceus syndrome: report of a unique constellation and review of the literature.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.- - - - - - - - - - ranking = 1.4700358939001keywords = central nervous system, brain, nervous system (Clic here for more details about this article) |
5/38. Heavily T2 weighted MR assessment of fornical injury after anterior interhemispheric approach for large suprasellar tumors.Fornical injury in transforaminal approach is well known. Its injury in the anterior interhemispheric approach (AIA) has been rarely highlighted. We report 2 cases with a large suprasellar tumor who underwent AIA. Postoperative heavily T2 weighted reversed (T2R) MR images demonstrated its unilateral injury. The clinical significance of symptom-free fornical injury after AIA is discussed. Cases 1 and 2 were a 15 year-old girl with a meningioma and a 49-year-old woman with a craniopharyngioma, respectively. They underwent AIA. Postoperative T2R images revealed unilateral fornical crus atrophy. They did not present associated memory deficits. Case 1 had the injury of both fornical column and anterior commissure. They were speculatively torn by intra-operative lateral retraction of the frontal lobes. Case 2 had unilateral atrophy of the mammillary body and postcommissural fornix, which were probably caused by ischemic damage related to surgical manipulation, since case 2 had an associated anterior thalamic infarct. During the operation for large suprasellar tumors, excessive laterally directed brain retraction should be avoided, since such manipulation may easily tear the overstretched anterior commissure and fornical column. Once we notice or suspect fornical injury on MR studies in cases of re-operation, we have to choose a surgical approach and operative manipulation to preserve an intact fornix. The MR evaluation of fornix should be included in the perioperative radiological assessment, since patients with unilateral fornical injury were free of memory disturbance, and T2R imaging is a useful MR sequence for depicting the anatomy related to the fornix.- - - - - - - - - - ranking = 0.23501794695007keywords = brain (Clic here for more details about this article) |
6/38. Application of the orbito-cranial approach in pediatric neurosurgery.OBJECTIVE: This study evaluates the benefits of and indications for the orbito-cranial approach (OCA) in pediatric patients. methods AND RESULTS: The authors report their recent experience of using the OCA in 9 pediatric patients, 6 boys and 3 girls. The patients' ages ranged from 3 to 17 years (mean 9.6 /-5.16 years). Follow-up periods varied between 6 and 21 months (mean 12.6 /-5.9 months). Five patients were operated on for craniopharyngiomas, 2 for chiasmatic-hypothalamic astrocytomas, 1 for a recurrent hypothalamic gangliocytoma, and 1 for a hypothalamic hamartoma. In 7 cases a neuronavigation system (BrainLab) was utilized. The lesions were removed totally in 5 patients, near-totally in 1, subtotally in 2, and partially in 1 patient. An average increase of 30% in the area of vertical exposure significantly decreased the need for brain retraction. There was no mortality in this series. The only complications connected with the surgical approach were transient subgaleal cerebro-spinal fluid collections in 7 of 9 children and a subgaleal-peritoneal shunt placement in another patient. CONCLUSIONS: Our experience with this series of patients suggests that the OCA is as safe and beneficial in pediatric patients as it is in adults. It facilitates tumor removal by providing shorter access to and better exposure of the suprasellar area, thereby minimizing brain retraction.- - - - - - - - - - ranking = 0.47003589390014keywords = brain (Clic here for more details about this article) |
7/38. Rhabdoid glioblastoma.Rhabdoid phenotypic change has been described in a number of different neoplasms from diverse organ sites. These tumors share common light and electron-microscopic features, display a polyphenotypic immunohistochemical profile and often show cytogenetic abnormalities of chromosome 22. In the central nervous system (CNS), most rhabdoid tumors occur in the posterior fossa of very young children and are associated with a primitive neuroectodermal tumor (PNET) component and are designated atypical teratoid/rhabdoid tumors. Infrequently, other rhabdoid tumors of the CNS have been described, including rhabdoid meningiomas and malignant rhabdoid tumors of uncertain histogenesis. Several examples of conventional gliomas displaying significant areas with rhabdoid morphology were also presented in an abstract by Kepes and Moral [1991], although never published in final manuscript form. We now detail the case of an 18-year-old male with an aggressive, supratentorial CNS rhabdoid tumor that was associated with an epithelioid glioblastoma and apparently arose from areas of low-grade glioma. The rhabdoid tumor component was present in the original tumor but became more predominant with each of 3 successive resections. No areas of PNET were identified. Electron microscopy and immunohistochemistry showed features classic for rhabdoid tumors and cytogenetic studies demonstrated multiple tumor clones with monosomy 22. This case documents progressive rhabdoid transformation of a glioma, expands the spectrum of CNS tumor types that can display a rhabdoid phenotype and highlights the diagnostic and therapeutic challenges with this type of tumor.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/38. A novel case of a CAT to AAT transversion in codon 179 of the p53 gene in a supratentorial primitive neuroectodermal tumor harbored by a young girl. Case report and review of the literature.BACKGROUND: The most common cytogenetic abnormality encountered in primitive neuroectodermal tumors (PNET)/medulloblastoma is loss of heterozygosity in the region of the short arm of chromosome 17. There is some evidence that supratentorial PNET has different cytogenetic markers than infratentorial PNET/medulloblastoma. Particularly, loss of 17p is more frequent in the latter than in the former. We describe a young girl diagnosed with supratentorial PNET (SPNET). Analysis of the tumor suppressor gene p53 in the tumorous tissue revealed a rare transversion mutational event of CAT to AAT in position 179 of exon 5. To the best of our knowledge, this is the first case of such a transversion at codon 179 in the p53 gene in SPNET. CASE REPORT: A 12-year-old girl was admitted with nausea, headache and vision disturbances. MRI of the brain showed a large space- occupying lesion in the right frontal lobe. Histological examination of the macroscopic resection of the tumor revealed PNET of the brain. polymerase chain reaction-single strand conformation polymorphism analysis of all p53 exons was performed, and a unique variant of a transversion at codon 179 of exon 5 was revealed. Therapy was started according to the Children's Cancer group protocol (CCG-99702) designated for treatment of high-risk central nervous system embryonal tumors. She received an initial course of chemotherapy, consisting of cyclophosphamide and vincristine for mobilizing and harvesting peripheral blood stem cells (PBSCs). Then she was given craniospinal irradiation (3,600 cGy) with a boost to the tumor bed (1,980 cGy) and three consecutive courses of high-dose chemotherapy with carboplatin, vincristine and thiotepa/cyclophosphamide, with PBSCs support after each course. RESULTS: The patient is in complete remission 17 months after diagnosis, based on the results of physical examination and imaging studies. DISCUSSION: The mutation results in an alteration of the amino acid HIS to ASN. The amino acids surrounding position 175 play an important role in stabilizing the p53/dna complex. There are only 12 known mutations of the reported type, and the finding of such a rare mutational event in a low-incidence p53 mutation tumor, such as SPNET, might add additional insight into the p53-SPNET relationship in tumorigenesis. Although not widely accepted, it is possible that different mutations of the p53 gene in patients with brain tumors may imply a different ultimate prognosis. In our case, we cannot exclude the fact that transversion of CAT to AAT in position 179 of exon 5 may explain prolonged survival of a patient with good response to therapy.- - - - - - - - - - ranking = 1.7050538408502keywords = central nervous system, brain, nervous system (Clic here for more details about this article) |
9/38. Supratentorial hemangioblastoma: a case report and review of the literature.central nervous system hemangioblastoma is a histologically benign tumor that usually occurs in the cerebellum. Supratentorial hemangioblastomas are exceedingly rare tumors. We present a case of cerebral hemangioblastoma, review the literature on supratentorial hemangioblastoma, and discuss the histologic characteristics and diagnostic difficulties associated with such lesions.- - - - - - - - - - ranking = 0.21880170374601keywords = nervous system (Clic here for more details about this article) |
10/38. Primary nodular meningeal glioma mimicking metastatic tumor of the cerebellum with diffuse infra- and supratentorial leptomeningeal spread.A 62-year-old man presented with acute headache, blurred and double vision, nausea, and ataxia. magnetic resonance imaging showed an enhancing mass on the inferior aspect of the right cerebellar hemisphere as well as a thin, widespread leptomeningeal enhancement and T2-weighted hyperintense lesions in the left occipital lobe and both thalami. Lumbar puncture revealed clusters of anaplastic cells. Therefore, metastatic tumor of unknown origin was suggested. Despite whole brain irradiation and intrathecal chemotherapy the patient deteriorated gradually and died four months later. Post-mortem examination of the brain revealed a nodular, high-grade astrocytic tumor within the subarachnoid space on the lower portion of the right cerebellar hemisphere. Diffuse leptomeningeal spread was noted, but cerebellar parenchyma was not infiltrated.- - - - - - - - - - ranking = 0.47003589390014keywords = brain (Clic here for more details about this article) |
| | Next -> |