Complicated and unusual neurosurgical problems.
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Adenoma, Chromophobe↗
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Results for “CEREBELLAR NEOPLASMS”
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Hemangioblastoma of the central nervous system: nature of the stromal cells as studied by the immunoperoxidase technique.
Ten hemangioblastomas of the central nervous system were examined by an immunoperoxidase technique for glial fibrillary acidic protein (GFAP), S-100 protein, and Factor VIII-related antigen to determine the origin of the stromal cells. In seven cases scattered stromal cells were positive for GFAP, and eight cases showed variable numbers of S-100 protein-positive cells. These positive cells were present throughout the tumors. S-100 protein-positive stromal cells corresponded to GFAP-positive cells, but the former were more numerous than the latter. Staining for Factor VIII-related antigen was negative in stromal cells, except in endothelial cells lining capillaries. It was suggested that stromal cells of capillary hemangioblastomas are composed by heterogeneous cell populations.
Adult↗
Duraplasty: our current experience.
BACKGROUND: A large variety of biologic and artificial materials have been suggested as dural substitutes. However, no ideal material for dural repair in neurosurgical procedures has been identified. The authors report their experience with Tutoplast processed dura and pericardium. METHODS: This study is designed to evaluate Tutoplast dura and pericardium. The study population was composed of 250 consecutive patients who underwent cerebral duraplasty with these homologous materials between 1996 and 1998. The average follow-up was 5.4 years. RESULTS: We have observed only four complications with uncertain relationship with the dural implant. These resulted in complete recovery. CONCLUSIONS: We support the efficacy and safety of this natural dural substitute treated with Tutoplast method.
Adult↗
Medulloblastoma and glioblastoma multiforme in a patient with Turcot syndrome: a case report.
BACKGROUND: Turcot syndrome (TS) or the glioma-polyposis syndrome, is a rare, heritable disorder thought by some authors to be a variant of familial adenomatous polyposis (FAP). It is characterized by central nervous system (CNS) neoplasms and gastrointestinal polyposis. METHODS: We present a case report of a patient who developed a medulloblastoma at age 5 years. Ten years later, she developed adenocarcinoma of the colon. Seven months after resection of this Dukes' C2 adenocarcinoma, she presented with a second primary CNS tumor, a glioblastoma multiforme. The patient's colonic adenocarcinoma and glioblastoma were evaluated histologically and cytogenetically. RESULTS: Cytogenetic analysis revealed the presence of chromosomal instability in both tumors. This unusual case of two primary CNS neoplasms in a patient with TS is presented with a review of the literature. CONCLUSIONS: The implications of the cytogenetic analysis are discussed in conjunction with the present knowledge of the molecular biology of TS.
Adenocarcinoma↗
Intracranial metastases as the first manifestation of prostate cancer.
We report on 2 patients with neurologic symptoms secondary to intracranial metastases from carcinoma of the prostate. In one of these patients, the intracranial lesion was the only clinically detectable metastasis. We have found only three other such cases reported in the English-language literature.
Adenocarcinoma↗
SENSITIVITY OF INTRACRANIAL GLIOMAS TO VINCRISTINE SULPHATE.
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Astrocytoma↗
Malignant brain tumours associated with adenylate kinase in cerebrospinal fluid.
Adenylate-kinase activity was measured in cerebrospinal fluid (C.S.F.) from 35 healthy control subjects and 11 patients with brain tumours, of which 9 were malignant and 2 benign. No adenylate kinase could be detected in C.S.F. from the controls or from the patients with benign brain tumours. The enzyme was consistently found in C.S.F. from patients with malignant brain tumours.
Adenylate Kinase↗
Chromosome arm 17p deletion analysis reveals molecular genetic heterogeneity in supratentorial and infratentorial primitive neuroectodermal tumors of the central nervous system.
The current World Health Organization (WHO) classification groups together both infratentorial neoplasms (medulloblastomas) and their supratentorial counterparts as primitive neuroectodermal tumors (PNETs), implying a common origin. Previous analyses of medulloblastoma have shown loss of chromosome arm 17p as the most frequent genetic abnormality: the molecular genetic constitution of supratentorial PNETS has not been systematically studied. We therefore examined 8 hemispheric PNETs and 35 medulloblastomas with 17p restriction fragment length polymorphism (RFLP) and microsatellite markers. We also examined the TP53 tumor suppressor gene by a combined polymerase chain reaction-denaturing gradient gel (PCR-DGGE) technique. Our results showed that all of the 17p markers tested were preserved in all of the supratentorial PNET specimens. In contrast, loss of distal chromosome arm 17p was detected in 37% of the medulloblastomas. Analysis of the TP53 gene showed 2 mutations in the medulloblastomas and no mutations in the supratentorial tumors. These results show that the most common molecular genetic abnormality in infratentorial PNETS is absent in their supratentorial counterparts and suggests that alternative pathways and genetic events may be involved in their etiology.
Adolescent↗
[Metastasis in leptomeninges discovered with PET-FDG].
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Adenocarcinoma↗
p73 gene transcripts in human brain tumors: overexpression and altered splicing in ependymomas.
The p73 gene encodes a protein that shares structural and functional homologies with the p53 tumor suppressor protein. The p73 gene is monoallelically expressed in normal tissue, maps to chromosome 1p36 and is deleted in human neuroblastoma cell lines. Alternative splicing of exon 13 in p73 transcripts generates two isoforms, p73alpha and p73beta, that differ in their carboxy-terminus and in their ability to form homotypic interactions. In this study, we investigated, in 129 human central nervous system tumors of various histological types, the levels of p73 transcripts and the splicing characteristics of p73 mRNA. Whereas p73 mRNA content was consistently low in most tumoral types, especially in meningiomas, some glioblastomas, medulloblastomas and metastases exhibited elevated p73 mRNA content. However, ependymomas expressed consistently high amounts of p73 mRNA, significantly different from the other tumoral types. Whereas the short (p73beta) isoform accounted for 20-25% of the total p73 mRNA in most of the tumors, these splicing characteristics were altered in ependymomas (only 9% of p73beta) and in neurinomas (up to 53% of p73beta). These observations suggest tissular or tumoral differences in the control of p73 gene transcription and alternative splicing, and raise the problem of the role of p73 isoforms in the control of tumor growth, particularly in ependymomas.
Alternative Splicing↗
Reirradiation of primary CNS tumors.
PURPOSE: Primary central nervous system (CNS) tumors are seldom reirradiated due to toxicity concerns and sparse clinical data regarding efficacy. METHODS AND MATERIALS: We retrospectively reviewed 34 patients with primary brain tumors retreated with fractionated external beam irradiation at the University of California, San Francisco from 1977-1993. Tumors included 15 medulloblastomas, 10 high-grade gliomas, 7 low-grade gliomas, and 2 meningiomas. RESULTS: Initial course of radiation was radical in intent for all patients. Median age at initial diagnosis was 19.8 years (range: 3.6-67). Median interval between radiation courses was 16.3 months (range: 3.8-166). Median Karnofsky Performance Status (KPS) prior to reirradiation was 80 (range: 40-100). Reirradiation volumes overlapped previous treatment in 30 patients and were nonoverlapping in 4 patients. Fractionation schemes used were hyperfractionated in 17, conventionally fractionated in 9, and hypofractionated in 8. Cumulative maximum overlap dose within the CNS ranged from 43.2-111 Gy (median: 79.7 Gy). Retreatment was completed as planned in 27 out of 34 patients and modified or aborted in 7 (four tumor progression on retreatment, three patient request). As measured from the time of retreatment median progression free and overall survival was 3.3 and 8.3 months. Clinical and radiographic indices were stabilized or improved in about half of patients evaluable at a median of 3 months postretreatment. Complications (early or late) potentially attributable to retreatment were noted in 10 of 34 (29%) of patients. Overt necrosis was noted in 3 of 34 (9%) of patients and the actuarial risk of necrosis was 22% at 1 year following retreatment. CONCLUSIONS: Reirradiation of primary central nervous system tumors was associated with only modest palliative and survival benefits in this retrospective review. Difficulties separating toxicity due to retreatment vs. tumor progression and limited patient survival following retreatment preclude definite conclusions regarding the safety of this practice.
Adolescent↗
Potential role of proton therapy in the treatment of pediatric medulloblastoma/primitive neuro-ectodermal tumors: spinal theca irradiation.
PURPOSE: Conventional postoperative photon-beam radiotherapy to the spine in children with medulloblastoma/PNET is associated with severe late effects. This morbidity (growth and developmental) is related to the exit dose of the beams and is particularly severe in young children. With the purpose of reducing this toxicity, a dosimetric study was undertaken in which proton therapy was compared to standard megavoltage photon treatment. METHODS AND MATERIALS: The results of a comparative dosimetric study are presented in such a way that the dose distribution achievable with a posterior modulated 100 MeV proton beam (spot scanning method) is compared with that of a standard set of posterior 6 MV x-ray fields. The potential improvements with protons are evaluated, using dose-volume histograms to examine the coverage of the target as well as the dose to the vertebral bodies (growth plates), lungs, heart, and liver. RESULTS: The target (i.e., the spinal dural sac) received the full prescribed dose in both treatment plans. However, the proportions of the vertebral body volume receiving > or = 50% of the prescribed dose were 100 and 20% for 6 MV x-rays and protons, respectively. For 6 MV x-rays > 60% of the dose prescribed to the target was delivered to 44% of the heart volume, while the proton beam was able to completely avoid the heart, the liver, and in all likelihood the thyroid and gonads as well. CONCLUSION: The present study demonstrates a potential role of proton therapy in decreasing the dose (and toxicity) to the critical structures in the irradiation of the spinal neuraxis in medulloblastoma/PNET. The potential bone marrow and growth arrest sparing effects make this approach specially attractive for intensive chemotherapy protocols and for very young children. Sparing the thyroid gland, the posterior heart wall, and the gonads may be additional advantages in assuring a long-term posttreatment morbidity-free survival.
Brain Neoplasms↗
Impact of targeting deviations on outcome in medulloblastoma: study of the French Society of Pediatric Oncology (SFOP).
PURPOSE: To correlate targeting deviation in external beam radiation therapy with site of relapse in a prospective study of 174 patients treated for medulloblastoma. METHODS AND MATERIALS: Between February 1992 and February 1998 the radiotherapy treatment records were reviewed by a panel of radiation oncologists for 174 children treated with radiation therapy for medulloblastoma. The review was done without knowledge of patient outcome. Patterns of relapse were correlated with the results of the quality control review. RESULTS: Among the 174 patients five relapsed before the start of radiotherapy. One hundred sixty-nine patients were evaluable for correlation between targeting deviation and site of relapse. Number of major deviations in radiation therapy treatment is strongly correlated with the risk of tumor relapse (67% [95% CI: 28-91] of 3-year relapse rate in patient group with 2 major deviations and 78% [95% CI: 35-96] with 3 major deviations). This is particularly correlated with relapse in the frontal region of the brain: 5 relapses occurred in the frontal region in patients with major deviation in this area. An erroneous choice of electron beam energy is also linked with craniospinal fluid (CSF) relapse (3-year relapse rate of 68% [95% CI: 42-86]). Minor deviations in therapy technique are slightly associated with an increased risk of relapse in the same range as the group with only one major deviation. CONCLUSION: The quality of medulloblastoma radiation therapy technique is strongly correlated with outcome. Pretreatment central quality assurance review or standardized computer-designed blocks would improve survival to an extent equivalent to that attributed to adjuvant chemotherapy.
Adolescent↗
Paraneoplastic neurologic syndromes.
Cancer can affect the nervous system through many metastatic and nonmetastatic mechanisms, including side effects of cancer treatment, infections, coagulopathy, and metabolic or nutritional deficits. Paraneoplastic neurologic disorders (PND) are an extensive group of syndromes that cannot be explained by any of these complications and may affect any part of the nervous system. PND often develop before the presence of a cancer is known and their recognition may lead to the tumor diagnosis.
Brain Neoplasms↗
[Value of an indicator of the health status of children suffering from a severe illness: exemplified by brain tumors].
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Aftercare↗
The evolution of spinal growth after irradiation.
Spinal growth has been monitored in 13 children treated with craniospinal irradiation for brain tumours. Six were irradiated prepubertally and seven peripubertally, all with 30 Gy to the whole spine over four weeks. The change in (subischial leg length (SILL)--sitting height (SH)) standard deviation score (SDS) was used as an index of disproportionate segmental growth, which allowed the influence of growth hormone deficiency on growth to be discounted. Spinal growth over the prepubertal years was relatively normal: annual change in (SILL-SH) SDS was +0.07 SD 0.2. However, over puberty, annual change in (SILL-SH) SDS had increased significantly to +0.41 SD 0.14 (P = 0.03). If irradiated peripubertally, annual change in (SILL-SH) SDS to final height was +0.22 SD 0.23, not significantly different from the change over puberty in the prepubertal group. Relatively normal spinal growth after irradiation in the prepubertal child should be interpreted with caution, as it is during puberty that skeletal disproportion manifests itself.
Adolescent↗
Expression of Apo-3 and Apo-3L in primitive neuroectodermal tumours of the central and peripheral nervous system.
Deregulation of apoptosis has been implicated in the pathogenesis, spontaneous regression and treatment resistance of neuroblastoma. A newly recognised member of the tumour necrosis factor (TNF)-family of death receptors known as Apo-3 has been mapped to human chromosome 1p36.3, a region commonly deleted in aggressive neuroblastoma. Based on its localisation and function, Apo-3 is a candidate for the putative neuroblastoma tumour suppressor gene. Therefore we analysed mRNA expression of the Apo-3 receptor/ligand (Apo-3/Apo-3L) system in a representative panel of 18 neuroblastoma cell lines, 41 primary neuroblastoma and 13 ganglioneuromas/ganglioneuroblastomas by semi-quantitative RT-PCR. We compared the level of expression with the well-established prognostic factors age, stage, histology, MYCN-amplification and TrkA expression, as well as outcome. For comparison, we studied Apo-3/Apo-3L expression in 27 central nervous system (CNS) primitive neuroectodermal tumours/medulloblastomas (PNET/medulloblastoma) and in six normal brain samples. Neuroblastoma cell lines with 1p deletion and MYCN-amplification expressed significantly lower levels of Apo-3 (P=0.009 and P=0.03, respectively) compared with neuroblastoma cell lines without 1p deletion or MYCN-amplification. The mean expression level of Apo-3L was significantly higher in ganglioneuromas/ganglioneuroblastomas compared with neuroblastomas (P=0.001) and in normal brain compared with PNET/medulloblastoma (P<0.0001). Expression of Apo-3L was significantly associated with survival in neuroblastomas (P<0.049) and in PNET/medulloblastomas (P=0.01). Expression of Apo-3 was significantly associated with survival in PNET/medulloblastomas (P=0.03). Thus, the Apo-3 receptor/ligand system might be involved in the regulation of apoptosis in neuroblastomas and PNET.
Adolescent↗