Cases reported "Meningeal Neoplasms"

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1/17. Case of the month: March 1999--A 26 year old hiv positive male with dura based masses.

    A 26-year-old male with AIDS presented with a chief complaint of headaches and neck pain. An MRI revealed two enhancing extra-axial dura based masses, one in the area of the left sphenoid wing and one at the level of C2-3. In both cases, microscopic sections showed actin positive spindle cell neoplasms with long slender nuclei, consistent with leiomyomas. Both tumors were positive for Epstein Barr virus by in situ hybridization. This case report serves to emphasize the importance of considering soft tissue tumors such as leiomyoma in the differential diagnosis of mass lesions that occur in the central nervous system in AIDS and discusses the role of EBV in tumorigenesis.
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2/17. An isochromosome 6p in a primary meningeal malignant melanoma.

    The rearrangement of chromosome 6, particularly the deletion of 6q, has been observed in human malignant melanoma with or without brain metastases. The isochromosome 6p has also been described. In this study, we report the cytogenetic analysis of a primary malignant melanoma of the central nervous system. Its dominating karyotype was 47,XX, i(6)(p10). fluorescence in situ hybridization (FISH), using a 6p chromosome arm probe, confirmed the structure of the isochromosome. To our knowledge, this is the first report of this type of chromosomal aberration in an uncommon neoplasm of leptomeningeal melanocytic origin.
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3/17. Deletion of the NF2 region in both meningioma and juxtaposed meningioangiomatosis: case report supporting a neoplastic relationship.

    We report a case of juxtaposed atypical meningioma and meningioangiomatosis (MA) in an 8-year-old boy with no clinical stigmata or family history of neurofibromatosis. We studied the proliferative activity and genetic changes in the two lesions in an attempt to define their biologic and pathogenetic relationships. The MIB-1 index was 11% in the meningioma and <1% in the MA, indicating increased proliferative activity in the meningioma. fluorescence in situ hybridization was done for two chromosomal regions commonly deleted in meningiomas. There was loss of the neurofibromatosis 2 locus (22q12) in both the meningioma and MA. Conversely, the region of 1p32 was not deleted. Our results indicate that both the meningioma and MA arose from the same clonal process, with the meningioma probably undergoing additional, but undefined, genetic alterations that confer upon it a more proliferative potential. This loss of 22q12 in the MA raises doubt about the presumed hamartomatous nature of MA.
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4/17. Primary peripheral PNET/Ewing's sarcoma of the dura: a clinicopathologic entity distinct from central PNET.

    We describe two cases of peripheral primitive neuroectodermal tumor-Ewing's sarcoma (PNET-ES) arising intracranially in the leptomeninges. Both tumors exhibited a primitive undifferentiated round-cell morphology. Immunohistochemical stains revealed strong membrane expression of CD99 in both cases. A t(11;22)(q24;q12) could be demonstrated with reverse transcriptase-polymerase chain reaction in one case, whereas fluorescence in situ hybridization analysis performed in the second case showed a rearrangement of the EWS gene. The occurrence of PNET-ES at this site is very unusual. Immunophenotypical as well as genetic analysis play a key role in the diagnosis and the distinction from central PNET.
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5/17. Biphasic malignant meningioma: a comparative genomic hybridization study.

    To ascertain if a carcinoma-like component within a fibroblastic meningioma represented a metastatic carcinoma to a meningioma or malignant progression, we employed traditional immunohistochemical methods as well as comparative genomic hybridization (CGH) which compares chromosomal alterations. vimentin and epithelial membrane antigen were strongly immunoreactive in both the fibroblastic and carcinoma-like components. The CGH profile in both components had similar chromosomal alterations, including losses of 1p, 14, 16p13-->p10 and 22. However, the CGH profiles from the fibroblastic component showed losses of 4p, 10q23-->q24 and 18, along with gains of 1q, 6q25-->qter and 13q32-->qter. The profile of the carcinoma-like component showed losses of chromosome 4, in addition to gains of 3p12-->q13.11, 5q14.3-->q23.2, 6pter-->p23, and 13q14.2-->qter. CGH analysis of a biphasic malignant meningioma confirmed that the disparate histologic components were genetically related and likely derivative from a common precursor, demonstrating genetic instability and clonal expansion. Furthermore, CGH showed that the histologically appearing low-grade fibroblastic component had not solely the characteristic alterations of a benign meningioma but had already progressed to an atypical meningioma.
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6/17. Malignant rhabdoid meningioma arising in the setting of preexisting ganglioglioma: a diagnosis supported by fluorescence in situ hybridization. Case report.

    A highly malignant brain neoplasm with rhabdoid morphological features emerged in the bed of a subtotally resected ganglioglioma in a 54-year-old retired nuclear submarine officer. A combined application of neuroimaging, immunohistochemical studies, electron microscopy, and fluorescence in situ hybridization (FISH) was used to establish the morphological identity of the tumor. The rhabdoid appearance of the tumor cells indicated either an especially malignant variant of rhabdoid meningioma or an atypical teratoid/rhabdoid tumor with an unusually late onset. Whereas immunohistochemical studies and electron microscopy could only be used to narrow down the differential diagnosis, FISH revealed loss of one copy of NF2 with preservation of the INI1 region on 22q, thus establishing the identity of the tumor.
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7/17. Histologically benign metastatic meningioma: morphological and cytogenetic study. Case report.

    The authors report on a 75-year-old man with histologically benign fibroblastic meningioma metastasizing to the lung, liver, spleen, and kidney. The original tumor exhibited a complex karyotype involving different structural and numerical anomalies associated with monosomy of chromosome 22. The implication of chromosome 1p36 was confirmed by fluorescence in situ hybridization in most interphase nuclei. Metastases occurred 4 months after incomplete resection with prior therapeutic embolization. The recurrent tumor in turn displayed anaplastic features and an increased Ki-67 labeling index. Genetic alterations in such morphologically benign meningiomas have been implicated in the malignant development and progression of these tumors.
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8/17. Primitive neuroectodermal tumor in the cerebellopontine angle with isochromosome 17q presenting as meningioma in a woman 26 years of age.

    An unusual posterior fossa neoplasm in a 26-year-old woman with short history of the cerebellar symptoms is presented. CT and MR images showed the tumor within the cerebellopontine angle, suspected as meningioma. At surgery, the tumor was dura-attached and did not infiltrate the arachnoid. Histologically, the neoplasm was a small blue cell tumor with solid and microcystic pattern, consistent with primitive neuroectodermal tumor (PNET). Immunohistochemically the cells were strongly positive for NCAM and GFAP. fluorescence in situ hybridization (FISH) was performed with the cosmids G9 and F7 (flanking EWSR1/22q12 region) dna probes and dual-color spectrum-orange LSI HER-2/neu (17q11.2)/spectrum green CEP17 (17p11.1-q11.1) DNA probe. The presence of isochromosome 17q within neoplastic cells was found. The tumor was classified as a medulloblastoma. We demonstrate the utility of a multidisciplinary approach to nervous system tumor diagnosis. The clinical features together with histological, immunohistochemical, and characteristic molecular alteration allowed classification of the presented case.
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9/17. Melanocytic colonization of a meningothelial meningioma: histopathological and ultrastructural findings with immunohistochemical and genetic correlation: case report.

    OBJECTIVE AND IMPORTANCE: Melanocytic colonization of nonpigmented extracranial tumors has been reported in adenocarcinomas, squamous cell carcinomas, skin appendage tumors, and dermatofibrosarcoma protuberans. To our knowledge, melanocytic colonization of a meningioma has not previously been described. CLINICAL PRESENTATION: We report an unusual case of a 70-year-old African-American woman who presented with a large frontoparietal meningioma that extended through the calvarium. INTERVENTION: craniotomy with gross total resection of the tumor was performed. Histochemistry, immunocytochemistry, ultrastructural analysis, and molecular genetic study via fluorescence in situ hybridization confirmed melanocytic colonization of a meningothelial meningioma. CONCLUSION: With the inclusion of meningothelial meningioma, the spectrum of tumors affected by melanocytic colonization continues to expand.
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10/17. cavernous sinus and leptomeningeal metastases arising from a squamous cell carcinoma of the face: case report.

    OBJECTIVE AND IMPORTANCE: Invasion of trigeminal and facial perineural spaces is a recognized complication of cutaneous malignancies. Centripetal spread along the trigeminal nerve axis and into the cavernous sinus and the gasserian ganglion is rare. Metastasis to the leptomeninges and cauda equina has not been reported. We report a unique case of perineural spread and central dissemination from an epithelial squamous cell carcinoma (SCC) associated with a tumor biomarker. CLINICAL PRESENTATION: After excision of multiple cutaneous SCCs and basal cell carcinomas of the head and neck, a 70-year-old male patient developed successive, right-side, V1 and V2 trigeminal neuropathies and complete right cavernous sinus syndrome during a 5-year period. Concurrently, the right face became paralyzed. Left facial paresis developed during the latter half of this period. Two months before admission, subacute left lower-extremity radicular weakness resulted in falls. Serial magnetic resonance imaging scans obtained in the previous 4 years were unrevealing. At the time of admission, enhancing masses were found in the 1) right cavernous sinus and dura, foramina ovale and rotundum, and Meckel's cave, 2) right subtemporal region and orbital rectus muscles, and 3) cauda equina. cerebrospinal fluid analysis demonstrated mild pleocytosis and rare carcinoma cells. INTERVENTION: biopsy of the right cavernous sinus mass confirmed moderately differentiated, metastatic SCC. Immunohistochemical staining and fluorescence in situ hybridization revealed epidermal growth factor receptor overexpression and genomic amplification. CONCLUSION: The indolent progression of cranial nerve palsy among patients with resected cutaneous SCCs of the head and neck must raise clinical suspicion of perineural spread, even in the absence of radiological changes. Biomarkers predicting aggressive SCC behavior, illustrated here by epidermal growth factor receptor amplification and central invasion, have the potential to guide early therapy.
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