Cases reported "Glioma"

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1/26. A case history of glioma progression.

    Low-grade diffuse astrocytomas have an intrinsic tendency for malignant progression but the factors determining the kinetics of this process are still poorly understood. We report here the case of a male patient who developed a fibrillary astrocytoma at the age of 33 years and who underwent six surgical interventions over a period of 17 years without radiotherapy or chemotherapy. The first three biopsies spanned a period of 11 years and led to the diagnosis of low-grade, diffuse astrocytoma (WHO grade II), with a growth fraction (MIB-1 labeling index) of 2.3-3.7%. The fourth to sixth biopsies showed histological features of anaplastic astrocytoma (WHO grade III), with growth fractions between 5.0 and 10.5%. The fraction of gemistocytic neoplastic astrocytes also increased, from 0.3% in the first biopsy to 17.5% in the last biopsy and preceded the increase in proliferative activity and transition to anaplastic astrocytoma. The fraction of tumor cells immunoreactive to BCL-2 increased from 0.3% to 8.2%. A p53 mutation in codon 273 (CGT-->TGT, Arg-->Cys) was identified in the first biopsy and persisted throughout the course of the disease. However, the fraction of cells with p53 protein accumulation increased significantly during progression, from 3.2% in the first biopsy to 13.7% in the last. The absence of additional genetic alterations (PTEN mutations, loss of chromosome 10 and 19q) may be responsible for the slow progression and lack of glioblastoma features even after a 17-year disease duration.
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2/26. Demyelinating pseudotumor.

    Demyelinating disease presenting as a solitary contrast-enhancing mass poses a diagnostic challenge for both radiologists and surgical pathologists. We report the cases of two female patients, aged 23 and 37 years, who exhibited the clinical and radiologic features of a space-occupying mass strongly suggestive of neoplasia. In both patients, magnetic resonance imaging showed a ring-enhancing parietal lesion. Intraoperative frozen sections in both patients displayed histologic features strongly suggestive of a glial neoplasm, including marked hypercellularity, a prominent astrocytic component, and easily identifiable mitotic figures. However, permanent sections showed additional and helpful histologic findings that included Creutzfeldt astrocytes and granular mitoses. Subsequent immunostaining showed that the hypercellularity was principally caused by macrophage infiltration (HAM-56 and CD68) and an associated reactive astrocytosis (glial fibrillary acidic protein). Additional confirmatory tests included special stains for myelin (Luxol-fast-blue), which demonstrated focal, sharply marginated loss of myelin, and for axons (silver stain for axons and neurofilament protein immunohistochemistry), which showed relative preservation of axons in areas of myelin loss. Together, the special stains confirmed the demyelinating nature of the lesions. The keys to avoiding misdiagnosing a demyelinating pseudotumor as a diffuse glioma include a general awareness of this potential pitfall, including the radiologic appearance of demyelinating pseudotumors as contrast-enhancing solitary masses that mimic tumor; knowledge of the characteristic histologic features, including Creutzfeldt astrocytes and granular mitoses; and a high index of suspicion for macrophage infiltration combined with a willingness to use appropriate confirmatory immunohistochemical studies in suspicious or uncertain cases. This approach will minimize the chance of misdiagnosis and subsequent use of inappropriate and deleterious therapies.
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3/26. Progressive multifocal leukoencephalopathy and gliomas in a hiv-negative patient.

    A case of progressive multifocal leukoencephalopathy (PML) is reported, detected at autopsy of a 30-year-old patient. The clinical picture was characterized by a progressive course of mental deterioration and ingravescent neurological symptoms. The patient was hiv-negative. He died of bronchopneumonia, after a clinical course of 13 months. autopsy disclosed pulmonary tuberculosis with involvement of regional lymph nodes. In the brain, besides numerous PML-foci of varying age and structure, a pleomorphic astrocytoma was found in the white matter of the right parietal lobe. In the brain stem glial proliferation resembling diffuse gliomatosis was also present. in situ hybridization revealed Papova-virus (JCV) in oligoglial nuclei, but not in neoplastic astrocytes. This is the third report on the concomitant occurrence of PML and glioma in man.
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4/26. Primary leptomeningeal glioma: ultrastructural and laminin immunohistochemical studies.

    We studied a case of primary leptomeningeal glioma (PLG) on the left parietal lobe of a 74-year-old woman and compared the tissue with heterotopic glial tissue from another case. The PLG tumor consisted of spindle-shaped cells with marked nuclear atypism, which tended to be arranged in a fascicular pattern, and the majority of its cells were positive for glial fibrillary acidic protein. Ultrastructural examination demonstrated that most of the tumor cells contained intermediate filaments and often junctional complexes were present on their plasma membranes. Frequently, basal lamina-like structures surrounding the tumor cell surfaces were observed. laminin immunohistochemistry clearly demonstrated a fine network of linear positive staining around the cytoplasm and processes of the tumor cells. The ultrastructure of the heterotopic glial tissue consisted of many astrocytes partially surrounded by basal lamina. These findings strongly suggest that PLG is a distinct tumor, which arises from the heterotopic astrocytes within the subarachnoid space.
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5/26. Nasal glial heterotopy in childhood: a case report highlighting the morphological and immunohistochemical features.

    We present a case of a 5 months old infant who presented with difficulty in breathing and a visible intranasal swelling. Clinical examination revealed a reddish mass medial to the middle turbinate in the left nasal cavity. MRI was done and a provisional diagnosis of nasal glioma was suggested. The mass was surgically excised and sent for histopathology which showed it to be comprising of astrocytes and neuroglial fibers intermixed with a fibrovascular connective tissue stroma. The presence of Glial fibrillary acid protein (GFAP) confirmed the presence of glial tissue.
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6/26. diagnosis and management of gliomatosis cerebri: recent trends.

    Gliomatosis cerebri (GC) describes a diffuse overgrowth of the neuraxis by neoplastic glial cells with relative preservation of the underlying cytoarchitecture. Ten cases of GC are presented, followed by discussions of its radiological and pathological characteristics. The diagnosis of GC was formerly made only at autopsy. However, recent improvements in imaging and biopsy now allow for antemortem diagnosis. Immunocytochemistry for S-100 protein and glial fibrillary acidic protein (GFAP) in three open biopsies of early primary GC revealed diffuse proliferation of S-100 immunoreactive and GFAP-resistant small astrocytes. magnetic resonance imaging appears to be the imaging modality of choice. T2-weighted images generally show contiguous high intensity signal in affected areas. biopsy, either open or stereotactic, remains necessary for antemortem diagnosis. No treatment has proven effective for GC, the prognosis being poor. The authors also review theories of tumorigenesis of GC in an effort to explain its biology.
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7/26. Gliomatosis cerebri: a case report.

    We report a case of gliomatosis cerebri in a 46-year-old woman with five-year history of seizures and psychiatric disturbance. There were also two episodes of lethargy, disorientation, and headache which cleared promptly with mannitol. A 3rd episode terminated in her death. Remarkably, between the episodes of presumed increased intracranial pressure, the neurologic examination was normal except for the patient's denial of her illness. Postmortem examination revealed the entire right cerebral hemisphere to be enlarged and infiltrated by cells resembling astrocytes. The clinical signs, symptoms, and controversial histopathologic features of this rare entity are discussed.
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8/26. Gliomatosis cerebri: bioptical approach and neuropathological verification.

    Gliomatosis cerebri is rarely encountered and its intra vitam diagnosis has remained difficult. We present biopsy and autopsy findings in three cases that are representative of diffuse glioma, gliomatosis cerebri and diffuse glioblastosis, a modification of the subclassification proposed by Zulch31. Stereotactic biopsy in conjunction with nuclear magnetic resonance tomography (MRT) is recommended as the diagnostic procedure in suspected cases. Immunohistochemical examination with a panel of neuroectodermal markers is helpful in the differential diagnosis but has to take into account that reactive astrocytes may be closely intermingled with the neoplastic glial cells.
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9/26. Assessment of proliferative potential in gliomatosis cerebri.

    The proliferative potential of neoplastic cells in two cases of gliomatosis cerebri was investigated by a combined staining technique, a one-step silver colloid method for nucleolar organizer region-associated protein (AgNOR) and immunohistochemistry for fibrillary acidic protein (GFAP). The neoplastic cells in the two cases had an abnormal shape and showed positive GFAP immunostaining in their cytoplasm. The numbers of AgNORs were counted in central and peripheral lesions of the neoplastic field in each case. The mean AgNOR scores in neoplastic cells were almost the same as those of nonneoplastic astrocytes in both the central and the peripheral lesions. These values were almost equal to the AgNOR score of low-grade gliomas. These findings indicate that gliomatosis cerebri has an invasive character in the central nervous system and often shows a malignant tendency, but its proliferative potential is significantly lower than that of high-grade gliomas.
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10/26. Reactive glioma in intracranial sarcoma: a form of mixed sarcoma and glioma ("sarcoglioma"): report of eight cases.

    The clinicopathologic features of eight new cases of combined intracranial sarcoma and glioma are described. This type of mixed cerebral tumor is histologically characterized by a peripheral distribution of the gliomatous elements in relation to a more centrally situated meningeal or intracerebral sarcoma, and by the frequent presence of gradual transitions from reactive to frankly neoplastic astrocytes. In six of the eight cases, the additional development of either infiltrating astrocytoma or frank glioblastoma in the adjacent brain was demonstrated; this was interpreted as a further expression of malignant glial reaction. It is suggested that these tumors be termed "sarcogliomas" to distinguish them from the type of mixed glioma and sarcoma that has recently been redesignated "gliosarcoma."
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