Cases reported "Ependymoma"

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1/82. Familial gliomas : a case report.

    Two non-twin brothers were found to have intracranial malignant neoplasms. The age of presentation was third and fourth decade but the onset was simultaneous, at the same time. diagnosis in each of them was made by computed tomography and confirmed by histopathology. Elder among them had cellular ependymoma and the younger had oligodendroglioma. Both the brothers received radiotherapy post operatively and were surviving asymptomatically without any neurological deficit, leading active life as police constable, 12 months after surgical treatment.
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keywords = neoplasm
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2/82. Signet-ring cell ependymoma: case report with implications for pathogenesis and differential diagnosis.

    We describe light microscopic, immunohistochemical and ultrastructural features of a signet-ring cell ependymoma (WHO grade II) identified in a surgically resected left cerebellar cystic tumor from a 64-year-old man. Part of the tumor showed clear-cell differentiation. Immunohistochemical coexpression of glial fibrillary acidic protein and epithelial membrane antigen, characteristic of ependymoma, was detected in both components. Sinuous intermediate junctions, cytoplasmic lumina, and scant astroglial filaments were demonstrated by electron microscopy. Signet-ring cell change was shown to be induced by disproportionate cavitation of either microvillus-bearing cytoplasmic lumina or microrosettes. The staining qualities of clear cells were mainly due to paucity and degeneration of subcellular organelles. Therefore, signet-ring cell ependymomas represent a unique anomaly of intra- and extracellular compartmentalization to be distinguished from various unrelated forms of cytoplasmic volume increase, resulting in an optically similar "empty" appearance of tumor cells. As a clinically relevant consequence, signet-ring cell ependymoma must be included in the differential diagnosis of primary or metastatic neoplasms of the central nervous system, having in common a phenotype characterized by overdeveloped optically lucent cell bodies.
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ranking = 1
keywords = neoplasm
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3/82. MKM-guided resection of diffuse brainstem neoplasms.

    OBJECTIVES: Some primary brainstem tumors, when extensive, are considered inoperable. We wanted to assess the value of robotic image-guided microscopic surgery in the resection of these tumors and to improve survival and quality of life for these patients. methods: Two patients with extensive brainstem tumors were evaluated at our center. They previously underwent several biopsies, attempted partial resections, radiotherapy and shunting. They presented with progressive neurological deterioration, 'coma vigil' for several months, and required life-supporting measures prior to surgery. Both patients underwent frameless stereotactic craniotomy using a MKM robotic microscope, intraoperative neurophysiological monitoring, and extensive resection of their recurrent brainstem tumors. RESULTS: In the immediate weeks after surgery, both patients became interactive and regained major motor and cranial nerve deficits present prior to surgery. Nine months after surgery, 1 patient succumbed to pneumonia. At 2 years after the operation, 1 patient has maintained his neurological status and showed no recurrence on imaging studies. CONCLUSIONS: Image-guided surgery with an MKM microscope allows surgical outlines to be injected in the microscope viewer and facilitates resection of extensive brainstem tumors previously considered inoperable.
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ranking = 4
keywords = neoplasm
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4/82. A dramatic loss of non-verbal intelligence following a right parietal ependymoma: brief case report.

    Virtually all reports on the effects of focal brain lesions upon specific neuropsychological functions are based upon estimates of cognitive loss in persons following a lesion, without proof of premorbid capacity. This report presents the case of a 19-year-old left-handed male with assessment of Verbal and Performance I.Q. testing 1 year prior to a subsequent brain tumor for reasons unrelated to the neoplasm. The patient was then re-tested 23 months later, following the diagnosis and treatment of a supratentorial ependymoma in the right parietal region. His multi-modal treatment regimen included a partial surgical resection of the tumor, cranio-spinal irradiation with focal boost to the primary site, and chemotherapy. Results demonstrate a striking 58-point decline in the Wechsler adult intelligence Scale - Revised (WAIS - R) Performance I. Q. with no significant change in Verbal I.Q. These findings clearly document the important cognitive functions associated with the right parietal lobe.
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keywords = neoplasm
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5/82. Primary intradural extramedullary ependymoma: case report and review of the literature.

    STUDY DESIGN: The authors report the ninth case in the literature of a primary intradural extramedullary ependymoma of the spinal cord. OBJECTIVE: To discuss surgical treatment and the physiopathologic hypothesis of this localization on the basis of the results of the present study and a review of the literature. SUMMARY OF BACKGROUND DATA: ependymoma is a glial tumor known to arise in the central nervous system. Intradural extramedullary location of this neoplasm has been exceptionally described previously. methods: A 43-year-old woman was admitted to the authors' institution with an history of progressive paraplegia. neurologic examination showed sensory loss below T1 and bladder disturbances. magnetic resonance imaging revealed an enhanced thoracic intradural extramedullary tumor, extending from T1-T8. No other lesion in the central nervous system was found. Emergency surgical resection was performed. RESULTS: Surgery gave confirmation of an encapsulated extramedullary tumor without attachment to the spinal cord or to the dura mater. Total removal was achieved under microscope. The postoperative course was uneventful, with complete neurologic recovery 3 months later. The patient has been well for 24 months of follow-up evaluation, without evidence of recurrence on magnetic resonance images. Histologic examination revealed the tumor as a benign ependymoma. CONCLUSION: The encapsulated feature, the lack of attachment to the central nervous system, and the absence of other neoplastic processes within the brain or the spinal cord suggested that this lesion is a primary tumor developed from ectopic ependymal cells.
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keywords = neoplasm
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6/82. Double ependymoma of the filum terminale.

    Myxopapillary ependymoma of the filum terminale and conus medullaris are relatively common spinal intradural neoplasms in adults, only 8-14% affecting children. We describe a case of a 15-year-old girl with two myxopapillary ependymomas of the filum terminale. Although cases of two such tumours have been described in adults, we have not found any similar paediatric cases in the literature.
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ranking = 1
keywords = neoplasm
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7/82. Supratentorial giant cell ependymoma.

    Ependymomas account for 3 to 9% of all neuroepithelial tumors and, although occurring most often within the ventricular system, they may arise from the extraventricular parenchyma as well. Several histologic patterns of these neoplasm are well known, but little attention has been devoted to a variant composed of giant elements. We describe the case of a 13-year-old girl experiencing a 3-month history of partial seizures in whom cranial magnetic resonance imaging showed an extraventricular, right parietotemporal neoplasm, adherent to the overlying dura mater. Histologic, immunohistochemical and ultrastructural findings were consistent with those of a high-grade ependymoma. The tumor was characterized by the presence of a major component of pleomorphic giant cells, as also seen in pleomorphic xantoastrocytoma, subependymal giant cell astrocytoma and glioblastoma multiforme. Similar elements have been described in two filum terminale and one supratentorial, intraventricular ependymoma, respectively. Histologic and evolutional data of those and of our own case suggest that isolated giant cells are not necessarily linked to a bad prognosis in ependymomas.
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ranking = 2
keywords = neoplasm
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8/82. Intraoperative diagnosis of tanycytic ependymoma: pitfalls and differential diagnosis.

    Smear preparations have become increasingly popular in the intraoperative assessment of central nervous system pathology. The cytological features of a histologically proven tanycytic ependymoma are presented with the pitfalls and differential diagnosis. The smear preparation showed a glial neoplasm composed of cells with long, bipolar glial processes and oval to spindle-shaped nuclei resembling those seen in pilocytic astrocytoma smears. The smear characteristics of an ependymoma usually show remarkably uniform round-to-oval nuclei, fluffy glial processes, and a perivascular nuclear-free zone (pseudorosetting). None of these features were present in our case. The accompanying frozen section showed a fascicular spindle-cell tumor that resembled a schwanomma, a commonly reported misinterpretation of the histology of tanycytic ependymomas on frozen sections. Careful attention to the radiological findings, the surgeon's impression, and the intraoperative smear preparation details should allow one to include this uncommon entity in the differential diagnosis of spinal neoplasms.
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ranking = 2
keywords = neoplasm
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9/82. Primary ependymoma of the ovary, in which long-term oral etoposide (VP-16) was effective in prolonging disease-free survival.

    BACKGROUND: Ovarian ependymoma is an extremely unusual teratoma of the ovary with only eight cases previously reported in the literature worldwide. CASE: A 26-year-old woman presented in 1992 with a sensation of abdominal fullness. The laparotomy revealed ovarian cancer (stage III), which proved to be an ependymoma pathologically. Three courses of the PVP regimen (cisplatin, vinblastine, peplomycin) and pelvic irradiation were administered postoperatively. Oral administration of etoposide (VP-16) was initiated after the residual tumor began to proliferate, and the tumor decreased in size and never regrew during etoposide administration for a total of 5 years and 8 months. The recurrent tumor was observed soon after the drug was discontinued. CONCLUSION: Oral administration of etoposide was effective in prolonging disease-free survival.
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ranking = 0.017816219173443
keywords = cancer
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10/82. 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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ranking = 4
keywords = neoplasm
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