Cases reported "Ependymoma"

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1/38. 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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2/38. 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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3/38. Combined treatment of fourth ventricle ependymomas: report of 26 cases.

    BACKGROUND: This study investigated the relevance of prognostic factors and the impact of histological features in posterior fossa ependymoma. methods: The charts of 26 patients (aged 1-59 years, mean 20.6 years; 11 adults) with posterior fossa ependymoma operated on between January 1983 and December 1994 were reviewed and patients followed up (mean: 93 months). RESULTS: Gross total resection was performed in 18 patients (69%), subtotal in seven patients (27%), biopsy in one patient (4%). One patient (3.8%) developed respiratory complications and died. All patients underwent posterior fossa radiotherapy (5000 cGy) after surgery. Four children first received chemotherapy and then radiotherapy only when at least 3 years old. Eleven patients (42%) received radiotherapy and subsequently chemotherapy. The 5-year survival rate was 90% for adults and 40% for children (review suggests that a) younger patients (prognosis; b) the microanatomical location of the tumor (lateral recess, roof, and floor) influences the extent of tumor removal (p < 0.05); c) longer survivals are associated with complete removal (p < 0.05); d) the histological feature most often related to a poor prognosis is a high mitotic index (p < 0.05), whereas vascular proliferation (p = 0.149), necrosis (p = 0.215), nuclear atypia (p = 0.384) and high cellularity (p = 0.786) do not affect survival; e) histological classification (WHO) does not reflect different survival rates between ependymomas and anaplastic ependymomas (p = 0.082).
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4/38. Disseminated spinal and cerebral ependymoma with unusual histological pattern: clinicopathological study of a case with retrograde tumor spread.

    The subject of this study is a case of anaplastic ependymoma originally arising from the central canal of the lower spinal cord followed by the 13 years history of events of upper spinal dissemination and retrograde intracranial spread. The specimens from four subsequent surgeries generally displayed the same microscopic features of neoplastic tissue and were consistent with the diagnosis of anaplastic ependymoma. The histological diagnosis was based upon the high cellularity, considerable nuclear atypia and pleomorphism, brisk mitotic activity, focally exhibited vascular endothelial proliferation and extensive necrosis. Apart from the typical pattern of ependymoma, the tumors contained areas composed almost entirely of large, uniform clear cells or pseudogemistocytes indicating the morphological heterogeneity of neoplastic cells population. The surgical specimens from four surgical resections shared light microscopic similarities suggesting spinal and intracranial dissemination from the primary spinal tumor. Since the retrograde spread via the cerebrospinal fluid (CSF) pathway is extremely rare, the authors of this study discuss the mechanism of such way of tumor metastases.
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5/38. 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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6/38. 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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7/38. recurrence of infantile supratentorial ependymoma after 23-year remission following surgical removal and radiation therapy.

    We report on a patient with ependymoma who had a recurrence after long-term remission. The patient developed frontoparietal ependymoma at the age of one year and ten months. The tumor was radically removed and postoperative radiation therapy was performed. A calcified area adjacent to the area of surgical removal remained unchanged until the patient was 18 years old. The patient was healthy except for mild hemiparesis until an MRI scan performed when he was 25 years old showed regrowth of the tumor. The patient underwent surgery with additional radiation therapy and was discharged. The 23-year interval until tumor recurrence in this case is far beyond the so-called risk period of "Collins' law". Immunohistochemical study with MIB-1 and anti-p53 antibody showed a high proliferative potential of the primary and recurrent tumors and possible p53 mutation in the primary tumor. This is the first report to describe the detailed clinical course and histological features of a recurrent infantile ependymoma that progressed after Collins' risk period. It seems that follow-up of ependymoma patients after initial treatment should be performed regularly for a longer period in cases showing radiological evidence of a residual lesion.
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8/38. Parasagittal ependymoma resembling falcine meningioma.

    Presentation, diagnosis, and management of an unusual parasagittal ependymoma, radiographically resembling a falcine meningioma, are described. Despite its radiographic appearance, pathologic evaluation revealed classic features of an ependymoma. The radiographic and pathologic characteristics of this unusual lesion are briefly examined, and the literature is reviewed. Although extraaxial ependymomas are rare, they should be considered in the radiographic differential diagnosis of dural-based lesions, especially for patients within the first 3 decades of life.
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9/38. 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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10/38. Fetal ependymoma associated with Down's syndrome.

    Down's syndrome (DS) is the most common multiple malformation syndrome occurring in humans and is associated with an increased risk of childhood malignancies, in particular leukemias. However, solid tumors seem to be underrepresented in DS patients and brain tumors occurring in association with DS have so far been limited to sporadic case reports, predominantly in children and young adults. We present the first case of an ependymoma associated with DS. This is also the youngest patient with DS suffering from a brain tumor as well as the first case in the English literature of a fetal ependymoma. The tumor was discovered incidentally at autopsy in a female fetus with DS after amniocentesis and termination of pregnancy in the 19th gestational week. Neuropathological examination revealed a tumor of 3x2x1 mm attached to the medulla oblongata rostrally of the floor of the fourth ventricle. Histologically, the ependymoma was moderately cellular and well demarcated with numerous perivascular pseudorosettes. immunohistochemistry showed strong reactivity for GFAP, in particular of the fibrillary elements of the pseudorosettes, while the neuronal marker synaptophysin was not expressed. The Ki67/MIB-1 proliferation index was 0.5%.
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