1/7. radiation therapy approach in gliomatosis cerebri--case reports and literature review.Gliomatosis cerebri is defined as a remarkably diffuse glioma, characterized by widespread infiltration of the central nervous system. Clinicopathologic characteristics and imaging findings have been published but valid classification remains controversial. Few reports exist regarding therapeutic options in gliomatosis cerebri. Here we review data on 17 patients treated with radiation therapy extracted from the literature, in which we focus our attention on available details of irradiation and clinical outcome and present the results of three additional patients treated at our two institutions. Radiologic-pathologic correlation in gliomatosis cerebri indicates that tumor delineation should be based on T2-weighted MRI. radiation therapy in gliomatosis cerebri is associated with a temporary improvement in or stabilization of clinical symptoms in the majority of cases. Duration of improvement was > or = 6 months in 50% of treated patients. survival from onset of symptoms was 23.8 months (range 8-42). Considerable variation in the natural course of the disease precludes conclusions regarding the impact of radiation therapy on survival.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/7. Primary diffuse leptomeningeal gliomatosis.Primary diffuse leptomeningeal gliomatosis is a rare central nervous system neoplasm in which focal or diffuse evidence of gliomatous tissue is identified in the subarachnoid space with no evidence of a primary tumour. A case is presented and the differential diagnosis and management are discussed.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/7. Gliomatosis cerebri: post-mortem molecular and immunohistochemical analyses in a case treated with thalidomide.Gliomatosis cerebri (GC) is a rare tumor of the central nervous system (CNS) characterized by widespread diffuse infiltration of the brain and spinal cord by neoplastic glial cells. We report the case of a 17-year-old boy with a bioptically diagnosed fibrillary astrocytoma. The administration of thalidomide, which was suggested to be beneficial in the treatment of human cancers, had no substantial clinical effect on our patient. autopsy studies revealed a diffuse infiltration of the frontal and temporal lobes of the right hemisphere, brainstem, and the leptomeninges covering the whole spinal cord by an astrocytic tumor, which showed features both of low-grade astrocytoma and glioblastoma multiforme. No mutations in the p53 and PTEN tumor suppressor genes were found; immunoreactivities for p53, PTEN, and EGFR could not be detected.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/7. Gliomatosis of the brain and spinal cord masquerading as infective lesions.BACKGROUND: Gliomatosis of the brain or spinal cord is an infiltrating glial neoplasm that shows widespread invasion of the central nervous system with relative sparing of the underlying cytoarchitecture. Acceptance of the idea that the condition represents a distinct entity remains controversial in the absence of conclusive pathogenetic data. The clinico-pathological problems and difficulties in the ante-mortem diagnosis as well as the clinical and pathological similarities to infective lesions are evaluated. methods AND RESULTS: Three cases of cerebral and spinal gliomatosis are presented that clinically mimicked infective lesions and were diagnosed and treated as such. The correct diagnosis in each case was only made at post-mortem examination. The ante-mortem diagnosis of this rare tumor remains difficult owing to poor correlation of clinical, neuroradiological, and neuropathological findings. CONCLUSION: Gliomatosis of the brain and spinal cord may simulate infective lesions owing to difficulty in ante-mortem diagnosis because of vagueness of physical, radiological, and pathological findings. It is a diagnostic pitfall particularly in our setting where there is a high incidence of hiv/AIDS and patients often present with opportunistic infections such as mycobacterial, fungal, and/or viral infections, which show an atypical clinical picture and radiological findings. Multifocal neurologic deficit with noncontrast enhancing lesions that show diffuse contiguous involvement with overall preservation of the spinal or cerebral architecture and do not respond to infective treatment could suggest a diagnosis of gliomatosis cerebri.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/7. Malignant lymphoma of the central nervous system: difficult histologic diagnosis after glucocorticoid therapy prior to biopsy.Four cases of central nervous system (CNS) lymphoma are reported which presented obstacles in diagnosis due to steroid treatment prior to biopsy. Reliable diagnoses were provided by molecular analysis. Malignant lymphoma of the CNS may be indistinguishable from other conditions, even in view of the gravity of the diagnosis. All patients had a previous history of glucocorticoid injection, for 2-18 days prior to stereotactic brain biopsy. The pathologic examination revealed in all cases axonal destruction and reactive gliosis with a variable infiltration of B- or T lymphocytes and macrophages. Characteristically, scattered degenerating small round cells with pyknotic or fragmented nuclei were also observed. However, the molecular assessment of paraffin-embedded tissues revealed the monoclonal IgH gene rearrangement, which allowed the confident diagnosis of B cell lymphoma. The histopathological findings of the present cases suggest that the tumor cells might be selectively destroyed by steroid treatment, which may render diagnosis impossible. Thus, molecular genetic investigation constitutes an important tool for establishing a diagnosis of CNS lymphoma obscured by steroid administration. This is especially true in cases where a paucity of tumor cells is observed or when monoclonality fails to be demonstrated by immunohistochemical tests.- - - - - - - - - - ranking = 5keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/7. Gliomatosis cerebri angiographically mimicking central nervous system angiitis: case report.OBJECTIVE: The diagnosis of isolated central nervous system angiitis often requires both cerebral angiography and biopsy for diagnosis. CLINICAL PRESENTATION: We present a case of gliomatosis cerebri with demonstrated angiographic changes suggestive of a central nervous system vasculitis. INTERVENTION: A 47-year-old woman presented with a 2-week history of worsening headache and progressive drowsiness. magnetic resonance imaging scans revealed confluent nonenhancing T2-weighted hyperintensity involving the frontal white matter bilaterally, hypothalamus, and both thalami. A four-vessel angiogram revealed diffuse arterial beading with stenoses of a right frontal middle cerebral artery branch and the distal posterior cerebral artery and anterior cerebral artery branches, bilaterally. The biopsy revealed a Grade 2 fibrillary astrocytoma consistent with gliomatosis cerebri. CONCLUSION: Gliomatosis cerebri can mimic central nervous system vasculitis angiographically. Perivascular infiltration by tumor cells may be responsible for these changes.- - - - - - - - - - ranking = 7keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/7. Polar spongioblastoma of the spinal cord: a case report."Rhythmic palisading" is a striking histologic pattern infrequently encountered in a variety of central nervous system (CNS) tumors. We present the case of an infant with a large spinal cord lesion wherein all sampled tissue showed columnar arrangements of palisaded cells, typical of polar spongioblastoma. The tumor was briskly proliferative, focally necrotic, and variably expressed S100, glial fibrillary acidic protein, neuron specific enolase, and p53 by immunohistochemistry. fluorescence in situ hybridization failed to reveal isochromosome 17q, EGFR amplification, or deletions of 1p, 19q, 22q11.2, 10q, or p16. Despite chemotherapy and decadron, he developed lesional necrosis and intracranial metastases and died less than 1 mo from presentation. This case illustrates polar spongioblastoma as a distinctive histologic pattern that can occur in embryonal CNS tumors. Discrimination of these rare aggressive lesions from other CNS tumors with focal palisaded architecture is crucial as the treatment and prognosis of the latter may differ significantly.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |