Cases reported "Ependymoma"

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1/14. ganglioglioma with a tanycytic ependymoma as the glial component.

    We studied a cystic ganglioglioma (GG) located in the right frontal lobe of the brain. Interestingly, the fibrillary spindle glial cells were often arranged in a fascicular pattern, and the generally uniform, round-to-oval delicate nuclei appeared to resemble those of ependymoma; and the neoplastic neurons often contained neurofibrillary tangles (NFTs). The glial component was positive for glial fibrillary acidic protein and occasionally contained granular or microvesicular structures positive for epithelial membrane antigen. Ultrastructural investigation revealed that the glial cells were ependymal in nature; intracytoplasmic lumina and intercellular microrosettes lined with cilia and microvilli, as well as long zonulae adherentes, were evident. In addition, chromogranin a-positive granular staining, neurosecretory-granule-like structures, and parallel arrays of microtubules were sometimes associated with the blood vessels. We considered the present case to be an unusual example of GG with an ependymoma, more precisely a tanycytic ependymoma, as the glial component; to our knowledge, the existence of ependymoma as the main glial component of this particular tumor has not been described before. The occurrence of NFTs, which has been reported in several cases of GG, was an additional, unusual feature.
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2/14. Suprasellar monomorphous pilomyxoid neoplasm: an ultastructural analysis.

    The authors report 3 patients, 2 children and 1 adult, each of whom presented with an unusual myxoid lesion reminiscent of pilocytic astrocytoma but with other features of myxopapillary ependymoma. The magnetic resonance imaging findings in all cases showed a diffusely contrast-enhancing suprasellar mass focally extending into the third ventricle. Involvement of adjacent structures was more extensive in both infants. By light microscopy, all were composed of a monotonous population of cells with delicate piloid-like processes, loosely arranged within a prominent myxoid background. Focally, the neoplastic cells converged upon small blood vessels in pseudorosette-like formations. These histomorphologic features are identical to those of the recently described astrocytoma with monomorphous pilomyxoid features. In addition, the individual tumor cells showed strong cytoplasmic immunoreactivity with antibodies to glial fibrillary acidic protein (GFAP) and vimentin, as well as nuclear and cytoplasmic staining with S-100. All stained positive for synaptophysin and negative for chromogranin. By electron microscopy, the tumor cells were bipolar with elongated processes and apical surfaces displaying microvilli, cytoplasmic blebs and rare cilia. Vesicles and coated pits were seen, as were occasional synaptoid complexes. The current study serves to expand our clincopathologic experience with this rare and enigmatic lesion, with particular attention given to the ultrastructural characteristics.
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3/14. Differentiation between recurrent tumor and radiation necrosis in a child with anaplastic ependymoma after chemotherapy and radiation therapy.

    BACKGROUND: In patients after treatment for malignant brain tumors, a clear distinction between tumor recurrence and radiation necrosis can be challenging. This case report describes the diagnostic workup in a child with anaplastic ependymoma and inconclusive MRI (magnetic resonance imaging) and PET (positron emission tomography) findings. CASE REPORT: 1.5 years after resection, hyperfractionated radiotherapy and chemotherapy of an anaplastic ependymoma in the right parietal region, the cranial MRI of an 11-year-old girl showed multiple small contrast-enhanced lesions in the frontal cortex. In the following months, these lesions increased in number and size and neurologic symptoms developed. Diagnostic workup included repeated MRI scans, PET with an (18)F-amino acid and (18)F-fluorodeoxyglucose (FDG), as well as a brain biopsy. RESULTS: Amino acid PET, performed when the lesions were still small, showed multiple small areas of mild uptake in close correlation to the MRI lesions. Although not typical, this result was suspicious of tumor seeding, the more since the lesions appeared in gray matter areas outside the high-dose-rate irradiation field. A biopsy, performed 6 months later when the clinical appearance worsened, showed no tumor tissue. FDG PET, performed after the size and number of the lesions had increased, showed no intensely increased glucose metabolism, a high-grade recurrent tumor was therefore very unlikely. In the following months, the clinical picture stabilized. CONCLUSION: The final interpretation of the lesions was multiple focal radiation necrosis based on perfusion abnormalities after chemotherapy and conformal hyperfractionated radiotherapy, probably due to an individually enhanced vulnerability of the cerebral vessels.
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4/14. November 2004: intradural mass of the cauda equina in a woman in her early 60s.

    November 2004. A 63-year-old woman presented with slowly aggravating lower back pain and recent urinary urge incontinence. MRI revealed a sharply-delineated, partly cystic intradural mass with inhomogenous contrast-enhancement and ectatic vessels at the upper pole. An ependymoma was suspected, and the tumor was resected in toto. Histologically, at first glance, the tumor strongly resembled an ependymoma, showing a monomorphic cellular pattern, perivascular pseudorosettes and ependymal canal-like structures. However, the finding of a delicate collagen capsule, compartmentation of tumor cells into zellballen and the presence of ganglionic cells were untypical. These features were indicative of a paraganglioma with a gangliocytic component. Immunoreactivity of the tumor cells for neuroendocrine antigens, the detection of GFAP-positive sustentacular cells and the ultrastructural confirmation of neurosecretory granules substantiated this diagnosis. The clinical, radiological and morphological similarity between ependymomas, which are far more common in the cauda equina region than paragangliomas, has led to substantial diagnostic confusion in the past.
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5/14. Myxopapillary ependymoma of the fourth ventricle.

    Myxopapillary ependymoma is a benign variant of ependymoma, occurring almost exclusively in the cauda equina region of the back. Here, we report an extremely rare case of primary myxopapillary ependymoma, and also present our review of the existing literature. This tumor occurred in the fourth ventricle of a 62-year-old woman. The patient had complained of dizziness and general weakness, persisting for the prior 6 months. The patient underwent surgical removal of the tumor. Imaging studies of the spine were normal. Microscopically, the cut sections of the tumor revealed papillary formation surrounding areas containing both hyalinized blood vessels and myxoid degeneration. The histological findings were consistent with those of tumors originating in the filum terminale. The patient's postoperative course was uneventful, and she was doing well on follow-up, 15 months after surgery. This is the second reported case of fourth ventricular myxopapillary ependymoma.
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6/14. An unusual case of proctalgia.

    We report the case of a 21-year-old man with the sudden onset of severe proctalgia followed by headaches and fever. The patient had a lumbar puncture consistent with subarachnoid hemorrhage. After a normal four-vessel cerebral arteriogram, a myelogram and computed tomography scan of the spine were performed and revealed a mass lesion of the L1 level. Pathologic evaluation after resection of the mass resulted in a diagnosis of hemorrhage from a myxopapillary ependymoma. These relatively benign tumors of the cauda equina-filum terminale respond well to treatment. The differential diagnosis of proctalgia is reviewed, and the need for emergency physicians to consider spinal subarachnoid hemorrhage when a patient presents with the acute onset of rectal pain is emphasized.
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7/14. Initial experience with MR-imaging of intracranial midline-lesions and lesions of the cervical spine at half Tesla.

    Fifty-two patients were examined both with computed tomography using a different third generation scanner and by magnetic resonance imaging (MRI) at half Tesla field strength (Philips Gyroscan 5 S). Excellent contrast and spatial resolution as well as initial comparative results of normal anatomy and also selected clinical cases were demonstrated with the spin-echo (SE) and/or inversion recovery (IR) technique. The clinical material included a residual prolactinoma after transphenoidal surgery, follow-up of a recurrent partly calcified solid and cystic intra-/supra-sellar craniopharyngioma, low-grade glioma under stereotactic-like conditions, suspected pinealoma, recurrent astrocytoma (II-III) and ganglioneuroma at the posterior aspect of the middle and/or lower brain stem, small scar after lower brain stem infarction, stenosing degenerative disease of the cervical spine and multicystic lesion with an underlying benign ependymoma of the cervical spinal cord. MRI--although duplicating some CT results--provided better two- or three-dimensional anatomical detail as well as display of relevant vessels without need of contrast agent. It also gave more specific information in suprasellar tumours containing fat, afforded uniquely specific diagnosis in a bleeding venous malformation of the midbrain and defined more sensitively extra-/intra-axial lesions of the brain stem and cervical spinal cord. Small bony structures (erosions) and punctate calcifications may be missed by MRI. Although ferromagnetic material distorts the MR image, compared with CT, it is not impaired by non-ferromagnetic clips. This is an advantage with respect to postoperative control examinations.
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8/14. ependymoma of the ovary: report of three cases.

    Three apparently pure ependymomas of the ovary occurred in young women 25 to 35 years of age who presented with abdominal swelling or pain. At operation one tumor was stage IC, and two were stage III. On microscopic examination the tumors contained cells with fibrillary cytoplasmic processes, often arranged around blood vessels to form perivascular pseudorosettes. Immunohistochemical staining for glial fibrillary acidic protein confirmed the nature of the tumor in each case. The patient with the stage IC tumor was alive and well five years postoperatively. One patient with stage III disease died of tumor six years postoperatively, and insufficient time elapsed after treatment of the second to allow meaningful follow-up data. These cases illustrate the rare occurrence of ovarian tumors resembling differentiated tumors of the central nervous system. Recognition and distinction of these tumors from common epithelial tumors, which they may resemble, have important prognostic and therapeutic implications.
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9/14. Myxopapillary ependymoma of the lateral ventricle. A study on the mechanism of its stromal myxoid change.

    Reported was the first case of myxopapillary ependymoma arising from the right lateral ventricle of a 29-year-old Japanese male. The histological and ultrastructural findings were identical to those from the filum terminale. It was suggested that insudation of plasma proteins found within hyaline-thickened blood vessels in the tumor stroma represented the presence of previous long-standing anoxia and thus, circulatory disturbance would be closely related to the formation of the stromal myxoid change.
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10/14. oligodendroglioma-like cells (clear cells) in ependymoma.

    A brain tumor of a 22-year-old man was composed mostly of round cells with perinuclear halos (clear cells), forming clusters intersected by small blood vessels. In some areas, the tumor cells showed perivascular arrangement and epithelial pattern. Phosphotungstic-acid hematoxylin stain and immunoperoxidase stain for glial fibrillary acidic protein (GFAP) technique failed to stain the clear cells. Electron microscopy of the clear cells revealed them to be classical ependymoma cells with well developed intercellular junctions, microvilli and cilia. As no reporters in the past showed the evidence to clarify the nature of the clear cells, this case is considered a good example to support the viewpoint that the clear cells (oligodendroglioma-like cells) commonly observed in ependymomas are in reality ependymoma cells. It is stressed that the diagnosis of "mixed glioma" or "oligoependymoma" should be made with sufficient caution despite the recent advances of GFAP technique.
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