Cases reported "Ependymoma"

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1/104. Malignant glial tumor arising from the site of a previous hamartoma/ganglioglioma: coincidence or malignant transformation?

    Gangliogliomas are generally considered benign tumors. Although more commonly found in the brain, spinal cord ganglioglioma is a well established, albeit infrequent, entity. We describe a 2-decade clinical course of a patient initially diagnosed with a thoracolumbar 'glial-neuronal hamartoma' at age 4. Seventeen years after his first operation, local recurrence was noted. Despite subsequent multiple gross total resections and adjuvant therapy, histologic features became increasingly ominous and ultimately proved fatal. This is an unusual report and histologic presentation of a resected spinal cord ganglioglioma recurring as an anaplastic ependymoma/astrocytoma and subsequently a glioblastoma. It is quite likely that the originally resected ganglioglioma was actually part of a primitive neuroectodermal tumor which had undergone extensive maturation.
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2/104. 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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3/104. 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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4/104. Supratentorial desmoplastic ependymoma with giant ependymal rosettes.

    We report a case of a 22-year-old female who presented with a solid tumor in the frontal lobe having no continuity with the wall of the lateral ventricle. The tumor was excised. The patient has been free from clinical symptoms and tumor recurrence for over nine years. Microscopically, the tumor was composed of extremely large ependymal true rosettes, resembling medulloepithelioma, and thick fibrous septa, which were surrounded by thick reactive gliosis. There were no histological signs of malignancy. In our case, the tumor is assumed to be a variant of gliofibroma for which we propose the term "desmoplastic ependymoma".
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5/104. Ovarian ependymoma. A case report.

    We present the case of a 30-year-old woman who was referred to our institution with an erroneous diagnosis of poorly differentiated carcinoma of the ovary. The patient presented pelvic pain for one year prior to surgery. A second laparotomy revealed a bilateral pure ovarian ependymoma that infiltrated the uterus and presented implants on the omentum. Differential diagnosis included mainly endometrioid and small cell carcinoma of the ovary. Presence of typical ependymal rosettes and positivity to GFAP confirmed the diagnosis of ependymoma. Other teratomatous elements were not observed. Ovarian ependymomas are rare tumors; only eight cases, to our knowledge, have been reported in the literature. They have a favorable prognosis; patients with advanced stage disease are reported alive and well after treatment with surgery and chemotherapy.
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6/104. So-called malignant and extra-ventricular neurocytomas: reality or wrong diagnosis? A critical review about two overdiagnosed cases.

    Central neurocytoma (CN) is described as a rare intra-ventricular benign neuronal tumor of the brain. Two primary tumors first diagnosed as malignant and extra-ventricular neurocytomas are reported here. Histologically, the tumor of the first patient, a forty-one-year-old man, consisted of monotonous cells with round nuclei, but no fibrillar background. The second tumor, in a nineteen-year-old girl, showed areas of moderately pleomorphic round cells, with numerous rosettes and ganglion cell differentiation, in an abundant fibrillary network. Both presented calcifications. Mitoses were more frequent in recurrences and spinal locations than in the primaries. All tumors stained strongly for synaptophysin, and GFAP was partly positive in the first case only. patients received post-surgical radiotherapy and were still alive eight and six years, respectively, after initial surgery. The interpretation of atypical cases, such as ours is not easy: the diagnoses finally retained were oligodendroglioma in the first case and ganglioneuroblastoma in the second case. Furthermore, neurocytomas atypical either by their unusual topographical or histological presentation or by their poor prognosis, have been frequently entitled in this way on synaptophysin positivity. So, we were prompted to reassess the entity of CN, seventeen years after the first description, to re-appreciate the reality of anatomo-clinical variants and to discuss the value of synaptophysin positivity in these tumors. In conclusion, it seems preferable to individualize true classical CN, which has a favorable outcome, from so-called extra-ventricular, atypical and anaplastic, clinically malignant neurocytomas for which complementary treatment is required.
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7/104. Intramedullary clear cell ependymoma in the cervical spinal cord: case report.

    OBJECTIVE AND IMPORTANCE: Clear cell ependymoma of the spinal cord has not been reported in the literature, although ependymoma in the cerebral and cerebellar hemispheres has been described. We present the first case report of this rare histological type of ependymoma arising in the cervical spinal cord and emphasize the importance of recognizing this histological entity. CLINICAL PRESENTATION: A 42-year-old woman presented with numbness in both upper limbs and spastic gait. magnetic resonance imaging revealed an intramedullary tumor at the C6-T1 level with syringomyelia. INTERVENTION: The tumor was totally removed. Histological analysis revealed that the tumor was composed of round cells with perinuclear halos similar to those observed in oligodendroglioma. However, we diagnosed clear cell ependymoma because these tumor cells exhibited epithelial features and ependymal rosettes under light microscopic examination. CONCLUSION: Histological diagnosis was crucial to our determining whether to perform postoperative adjuvant therapy in this patient. Neurosurgeons should be aware of the possibility of this histological entity among intramedullary spinal cord tumors.
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8/104. Spinal tanycytic ependymomas.

    Three cases of spinal tanycytic ependymoma are reported, a man aged 45 years and two women aged 36 and 55 years. Each patient developed gradual paraparesis over a few months prior to admission. magnetic resonance imaging showed an enhancing, well-circumscribed tumor in the spinal cord in each case. Histologically, the tumors consisted of monotonous proliferation of long spindle cells with markedly eosinophilic cell processes; focally forming perivascular pseudorosettes. The tumor cells were strongly immunopositive for glial fibrillary acidic protein, S-100 protein and vimentin. Ultrastructurally, in addition to massive intermediate filaments, many tumor cells showed abundant microtubules. Well-developed desmosomes and microvilli/cilia-lined microlumina were occasionally observed. The tumors were grossly totally removed and the patients remain recurrence free at 9, 9, and 2 years postoperatively. Reviewing reported cases including our three cases, tanycytic ependymoma may occur frequently in spinal cord, especially in the cervical region of the spinal cord. Since histologically it resembles pilocytic astrocytoma and schwannoma, tanycytic ependymoma should be included in the differential diagnosis of benign spindle cell tumors of the central nervous system.
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9/104. brain surface ependymoma.

    A case of brain surface ependymoma is reported here. A 41-year-old woman presented with weakness of the right upper limb. magnetic resonance imaging revealed a left fronto-parietal cystic tumor with a calcified mural nodule. Total removal of the tumor was performed. This tumor was located within the surface portion of the fronto-parietal parenchyma and was sharply demarcated from the surrounding brain tissue, and there was no continuity with the ventricles. Histological examination of this tumor showed monotonous proliferation of tumor cells with moderate cellularity and occasional mitoses, forming true ependymal rosettes and perivascular pseudorosettes with fibrillary background. A clear cell component resembling oligodendroglioma was partly noted. The tumor cells showed positive immunoreaction for glial fibrillary acid protein and vimentin.
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10/104. Pelvic ependymoma arising from the small bowel.

    A 37-year-old woman underwent resection of an abdominal tumour which was adherent to the wall of the ileum. The diagnosis of an ependymoma was supported by evidence of typical perivascular pseudorosettes which stained positive for glial fibrillary acidic protein and contained abundant intermediate filaments within the elongated processes by electron microscopy. Flow cytometric study showed a diploid population of tumour cells. This is the first case of an ependymoma arising from the small bowel without any connection to the genital tract, the omentum or with the sacroccygeal area. As is the case with other unusual and ectopic localisations of ependymomas, prognosis of this tumour is difficult to evaluate.
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