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PubMed1990

Neoplasms of the peripheral nervous system.

Neoplasms of the peripheral nervous system arise from the cellular sheath surrounding the nerve trunks, that is, the pluripotential Schwann cells and related cells, and rarely affect the feet. When present, they are most frequently associated with the autosomal dominantly inherited neurofibromatosis 1. This condition has been related to chromosome 17, and it appears, from in vitro experiments, to involve defects in tyrosine metabolism. Hence, the most common neoplasm is the neurofibroma. Distinct criteria have been established for a diagnosis of neurofibromatosis 1, so that a single pedal neurofibroma may not represent this complex. However, if the complex is present, it is necessary to consider the possibility of malignant transformation to a neurofibrosarcoma. Although malignant peripheral nerve neoplasms are extremely rare in the feet, they may arise in the context of neurofibromatosis 1, or independently. Other benign or malignant schwannomas may (rarely) also arise in the feet. Surgical excision of benign lesions, according to established standards for tumor surgery, is usually curative, but a detailed personal and familial history, along with adjunctive radiologic procedures and biopsy, is necessary to determine the nature of the lesion. Since (endocrine and) other abnormalities may complicate neurofibromatosis 1, surgical procedures must not be undertaken until the patient has been medically cleared and is carefully monitored. A high mortality rate is associated with malignant peripheral neurogenic tumors, especially those arising in the context of neurofibromatosis 1. It should be recalled that neurologic manifestations in the foot may represent non-neoplastic conditions as well as peripheral nerve tumors (or other tumors involving those nerves) that are proximal to the foot and ankle area.

Adult↗
PubMed1992

p53 protein expression in central nervous system neoplasms.

AIMS: To demonstrate, immunohistochemically, p53 protein expression in a selection of central nervous system tumours; to investigate the relation between p53 expression and that of the proliferation related antigen, PCNA. METHODS: Surgical specimens from 86 central nervous system tumours were routinely fixed, paraffin wax embedded, and immunostained with a monoclonal (PAb 1801) and a policlonal antibody (CM1) p53 protein and a monoclonal antibody against PCNA (PC10). Normal brain samples obtained at necropsy and 10 surgically obtained samples of gliotic brain parenchyma were also immunostained. RESULTS: p53 protein expression was observed in 35 of 86 brain tumours, suggesting frequent p53 gene mutation. p53 protein alterations were associated with all grades of malignancy in tumours displaying solely astrocytic differentiation, with the exception of pilocytic astrocytomas. In those showing oligodendroglial or ependymal differentiation they appeared to be restricted almost to only high grade lesions. No p53 immunoreactivity was observed in normal or gliotic brain tissue; p53 altered expression was not related to the percentage of PCNA labelled cells. CONCLUSIONS: The use of sophisticated gene amplification techniques or highly sensitive immunohistochemical methods might be useful in distinguishing between reactive and neoplastic astrocytic lesions, and in the identification of malignant progression in other non-astrocytic glial tumours. Tumours with very similar histogenetic differentiation features might actually be a genetically heterogeneous group with possible different clinical courses.

Antibodies, Monoclonal↗
PubMed1988

International mortality from primary nervous system neoplasms: distribution and trends.

International comparisons of average annual age-adjusted mortality rates for primary tumours of the nervous system for 1951 through 1958 and 1967 through 1973 showed marked geographical variation for both study periods. For the majority of countries, the mortality rates increased by at least 40% in the in the intervening 15-year period, while in 20% of the countries the rates increased by over 100%. The percentage increases varied from 12.2 to 345.5. The improvement in the diagnosis of these tumours, particularly among elderly individuals (who have the highest age-specific incidence rates for these neoplasms), presumably accounts for most of this change. However, it is possible that the increase may not be fully accounted for by these factors alone. It is therefore suggested that further epidemiological studies are undertaken to determine more accurately the causes and implications of this worldwide increase in mortality rates from primary tumours of the nervous system.

Female↗
PubMed1990

Synaptophysin: a sensitive and specific marker for ganglion cells in central nervous system neoplasms.

Synaptophysin, a 38-kilodalton glycoprotein found in synaptic vesicle membranes, has been shown to be a sensitive marker of neuroendocrine differentiation in non-central nervous system (CNS) tumors. We analyzed the patterns of synaptophysin immunoreactivity in CNS neoplasms in comparison with various normal CNS sites in biopsies. Normal gray matter structures all showed a diffuse punctate granular pattern of neuropil staining without staining of neuronal cell bodies. In contrast, neoplastic ganglion cells in 18 of 18 gangliogliomas/gangliocytomas showed intense immunoreactivity outlinging the borders of the cell bodies. Focal staining was also seen in five of 16 primitive neuroectodermal tumors and in one of three central neurocytomas, but these tumors had a finely granular neuropil pattern of immunoreactivity more like that of normal gray matter than like that of the gangliogliomas. All 35 examples of pure gliomas of various types showed no immunoreactivity. Our data highlight synaptophysin as a sensitive and specific marker of both neuronal lineage and neoplastic character in gangliogliomas.

Antibodies, Monoclonal↗
PubMed1990

Immunolocalization of cathepsin D in the human central nervous system and central nervous system neoplasms.

The cellular distribution of the lysosomal proteinase cathepsin D was studied in a series of 76 neoplasms and 18 non-neoplastic tissues from the human central nervous system, using a well-characterized polyclonal antibody in a peroxidase-antiperoxidase technique. In the normal and developing brain, cathepsin D is confined to neurons and choroid plexus epithelium. Strong granular cytoplasmic staining was present in neuronal and choroid plexus neoplasms, and in reactive macrophages. A large variety of other neoplasms also exhibited positive cytoplasmic staining, albeit usually of a weaker diffuse type. Cathepsin D cannot be considered a specific marker for neuronal or choroid plexus neoplasms, but the antiserum used in this study may be of value in antibody panels for the investigation of these tumours. Its localization may also be of value in embryological studies, particularly in the cerebellum, and in investigations of steroid hormone receptor-associated proteins in meningiomas and Schwannomas.

Brain Neoplasms↗
PubMed1990

Synaptophysin: a sensitive and specific marker for ganglion cells in central nervous system neoplasms.

Synaptophysin, a 38-kilodalton glycoprotein found in synaptic vesicle membranes, has been shown to be a sensitive marker of neuroendocrine differentiation in non-central nervous system (CNS) tumors. We analyzed the patterns of synaptophysin immunoreactivity in CNS neoplasms in comparison with various normal CNS sites in biopsies. Normal gray matter structures all showed a diffuse punctate granular pattern of neuropil staining without staining of neuronal cell bodies. In contrast, neoplastic ganglion cells in 18 of 18 gangliogliomas/gangliocytomas showed intense immunoreactivity outlining the borders of the cell bodies. Focal staining was also seen in five of 16 primitive neuroectodermal tumors and in one of three central neurocytomas, but these tumors had a finely granular neuropil pattern of immunoreactivity more like that of normal gray matter than like that of the gangliogliomas. All 35 examples of pure gliomas of various types showed no immunoreactivity. Our data highlight synaptophysin as a sensitive and specific marker of both neuronal lineage and neoplastic character in gangliogliomas.

Biomarkers, Tumor↗
PubMed1984

Cancer in relatives of children with central-nervous-system neoplasms.

We compared the occurrence of cancer in parents, siblings, and offspring of 643 patients who had central-nervous-system tumors in childhood (cases), as recorded in the Connecticut Tumor Registry, with the occurrence in parents, siblings, and offspring of 360 controls selected according to birth certificate and matched for sex, birth date, and birthplace. Overall cancer incidence was comparable in the two groups. However, 11 nervous-system tumors occurred in relatives of cases, whereas none occurred in relatives of controls (P = 0.0005). Nine relatives of cases but no relatives of controls had cancer of the hematopoietic-lymphatic system (P = 0.003). Nine siblings of cases but only one sibling of a control had cancer as children. Medulloblastoma and glioblastoma multiforme were overrepresented in the group of children whose relatives had central-nervous-system tumors. We compared the actual number of cancers of the central nervous system or hematopoietic-lymphatic system in relatives of cases with the number expected on the basis of known incidence rates and found a fivefold excess. We conclude that the occurrence of a brain tumor in a child is a marker for an increased likelihood of central-nervous-system tumors, leukemia, and childhood tumors in the family.

Adolescent↗
PubMed1987

Central nervous system neoplasm in a young man with Martin-Bell syndrome--fra(X)-XLMR.

A 17-year-old retarded male developed unilateral leg weakness with foot drop, pain, and incontinence. Workup disclosed a cauda equina tumor which, on surgical exploration, was demonstrated to merge with the conus medullaris. Pathological examination of the subtotally resected tumor led to a diagnosis of malignant ganglioglioma. Further evaluation of the patient documented marginal macro-orchidism, and chromosome studies showed fragile X. Since some neoplasms are known to be associated with chromosomal deletions and other abnormalities, we suggest that the occurrence of this tumor in this patient indicates a more than coincidental relationship between the two diagnoses.

Adolescent↗
PubMed2006

Recruited leukocytes and local synthesis account for increased matrix metalloproteinase-9 activity in cerebrospinal fluid of dogs with central nervous system neoplasm.

Matrix metalloproteinases (MMP) 2 and 9 are enzymes known to degrade several protein components of the extracellular matrix. In humans, increased concentrations of these enzymes have been demonstrated in the cerebrospinal fluid (CSF) of subjects affected by many neurological conditions including brain tumours; nevertheless comparative data in dogs are completely lacking. Aim of this study was to investigate these molecules in CSF of dogs diagnosed with CNS neurological diseases. Higher activity of MMP 2 and 9 was revealed in dogs with space occupying lesions of likely neoplastic origin in comparison to dogs with idiopathic epilepsy. Statistical modelling reveals that increased MMP 9 activity is mainly due to leukocytes recruitment and local synthesis.

Animals↗
PubMed2001

Histological pattern of central nervous system neoplasms.

OBJECTIVE: To see in a large series of patients the histological pattern of CNS neoplasms and to provide a comprehensive data about the frequency of CNS neoplasms in Pakistani patients and to give a clearer idea about their prevalence. METHOD: Retrospective study of all consecutive cases of CNS neoplasms diagnosed at The Aga Khan University Hospital (AKUH) between 1st January, 1994 and 31st December, 1999. SETTING: The section of Histopathology, AKUH, Karachi. OBSERVATION: Our series included a large number of cases i.e., 1110. There are very few local studies on CNS tumours and none so large. Our data was correlated with published Western and Pakistani data. Glial tumours were the commonest primary CNS neoplasms followed by meningiomas. Among glial tumours, astrocytomas comprised the largest group and the majority were high grade (III and IV) astrocytomas. Percentages of anaplastic oligodendrogliomas and ependymomas were also significant. Male to female ratio was 1.6:1. However, meningiomas were more common in females. Almost half of the CNS tumours in patients 15 years or younger were in the posterior cranial fossa. Metastatic tumours were much lower as compared to the West. CONCLUSION: Except for the high percentages of anaplastic oligodendrogliomas and ependymomas and the low percentage of metastatic tumours, most of our findings roughly correspond with the published data.

Adolescent↗
PubMed1998

Lipomatous differentiation in ependymomas: a report of three cases and comparison with similar changes reported in other central nervous system neoplasms of neuroectodermal origin.

Three cases of surgically removed ependymomas with lipomatous transformation of tumor cells are reported. Patients' ages were 13, 16, and 48 years at the time of operation. One patient's tumor was located in the third ventricle; the other two occupied paraventricular hemispheric white matter. Histologically all three cases fulfilled the criteria of ependymomas. In case 1, electron microscopy also confirmed this diagnosis, and preoperative radiologic studies (scans) suggested large amounts of lipids to be present in the tumor. Histologically, in all three cases many tumor cells contained fat droplets coalescing into a single large droplet, resulting in an appearance indistinguishable from adipocytes by conventional stains, but maintaining immunohistological positivity for glial fibrillary acidic protein and neuron-specific enolase in the cytoplasmic rims of the affected cells, attesting to their glial nature as opposed to being true adipocytes in a mixed glial/mesenchymal hamartoma. The alterations were also different from the "xanthomatous" changes seen in some gliomas. Lipomatous transformation of neuroectodermal tumor cells has been previously observed in neurocytomas, medulloblastomas, cerebellar and spinal cord astrocytomas, and primitive neuroectodermal tumors. Our three cases represent the first reported ependymomas with such changes. In medulloblastomas of adults, lipomatous changes have been found to signal relatively benign biologic behavior. So far, all three of our patients are doing well, but only more extended follow-up will show whether such benign behavior applies to lipomatous ependymomas as well.

Adolescent↗
PubMed1994

Staging and surveillance of children with central nervous system neoplasms: recommendations of the Neurology and Tumor Imaging Committees of the Children's Cancer Group.

Standardization of the baseline and surveillance evaluation of children with central nervous system (CNS) tumors could contribute to understanding tumor behavior, establishing prognoses, directing treatment, and interpreting multi-institutional cooperative study data. A tumor categorization schedule, based on recognized patterns of CNS tumor behavior involving 'aggressiveness' as assessed by the likelihood for neuraxis dissemination, relative time to progression and pattern of disease recurrence is presented. Included are the most common histologic subtypes of pediatric CNS neoplasms. No attempt has been made to include all histologic subtypes of pediatric CNS neoplasms. Using this categorization as a framework, recommendations are suggested for baseline evaluation at diagnosis and in surveillance.

Adolescent↗
PubMedDate not supplied

Differential uptake of tritiated digoxin in benign and malignant central nervous system neoplasms.

The purpose of this study was to measure uptake of tritiated digoxin by neoplastic tissues known to have differential contents of sodium-potassium adenosine triphosphatase (Na + K + ATPase), the presumed receptor for digoxin. Tumor samples were removed at the time of craniotomy in seven patients with meningiomas (Group 1) and seven patients with more malignant central nervous system tumors (Group 2) (three astrocytomas, three glioblastomas, one meduloblastoma). Patients with meningiomas were found to have a significantly higher digoxin uptake (21.8 +/- 7.3 ng/gm tumor versus 5.7 +/- 5.2 ng/gm tumor; (p less than 0.01) and a significantly greater tissue/serum ratio (13.9 +/- 11.7 versus 3.26 +/- 3.7, p less than 0.0). This study provides the first demonstration of increased uptake of digoxin by noncardiac pathologic tissues. The results are most likely due to differences in the number of digoxin receptor sites.

Adenosine Triphosphatases↗
PubMed1988

Metastases of central nervous system neoplasms. Case report.

Tumor-to-tumor metastasis is a rare occurrence. Fewer than 100 cases have been reported, many being metastases from carcinomas to benign intracranial neoplasms, most often meningiomas. A case is presented of carcinoma metastatic to a glioma. The patient, who presented for evaluation of bifrontal headache, was found on computerized tomography to have a partially calcified right frontal mass. Craniotomy revealed an oligodendroglioma containing foci of adenocarcinoma. Further work-up disclosed an infiltrative ductal adenocarcinoma of the breast. It has been suggested that tumors of the central nervous system may provide a fertile substrate or an immunological "haven" for metastases.

Adenocarcinoma↗