21/569. 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. ( info) |
22/569. 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". ( info) |
23/569. ependymoma with extensive lipidization mimicking adipose tissue: a report of five cases. Lipomatous ependymoma is a recently described entity and only 3 cases of this variant have been reported in the literature. We report 5 cases of this rare variant of ependymoma. patients age ranged from 4 years to 45 years and, interestingly, all of them were males. Two tumors were supratentorial in location, 2 in the fourth ventricle and 1 was intramedullary. Microscopically all of them showed the classical histology of ependymoma along with lipomatous differentiation. The lipomatous component was composed of cells with a large clear vacuole pushing the nucleus to the periphery and giving a signet ring cell appearance. This component demonstrated positivity for GFAP and S-100 protein thereby confirming its glial lineage. Three of the 5 tumors were high grade (WHO-grade III), had a high MIB-1 labelling index (MIB-1 LI) and showed recurrence on follow-up. However, 2 were low grade (WHO grade II) and patients are free of disease till the last follow up. ( info) |
| We describe a patient with a recurrent spinal cord ependymoma who initially presented with symptoms of increased intracranial pressure rather than symptoms directly relating to involvement of the conus medullaris. brain magnetic resonance imaging with gadolinium showed communicating hydrocephalus. magnetic resonance imaging of the entire spinal cord with gadolinium revealed recurrent tumor. Postoperatively, a permanent ventriculoperitoneal shunt was placed. Recognition of the association between spinal cord ependymoma and hydrocephalus is important in the evaluation of patients with headache. ( info) |
25/569. Spinal seeding of anaplastic ependymoma mimicking fungal meningitis. A case report and review of the literature. BACKGROUND: The spinal seeding from brain tumors sometimes mimicks fungal meningitis on examination of cerebrospinal fluid. methods AND RESULTS: A 19-year-old woman gradually developed increased intracranial hypertension. MRI identified a mass in the right parieto-occipital area. It was totally removed and histologically diagnosed as an anaplastic ependymoma. radiation- and chemotherapy were administered postoperatively. The patient reported low back pain 5 months after the surgical treatment. MRI disclosed neither spinal dissemination nor tumor recurrence at the primary site. Lumbar puncture was performed and the cerebrospinal fluid (CSF) was found to have an extremely low glucose level (5 mg/dl); no tumor cells were identified. blood samples were obtained and a relative increase of WBC and CRP was noted. A slight degree of inflammation and low-grade fever were recorded. A tentative diagnosis of fungal meningitis was made and anti-fungal therapy was administered transventricularly and transvenously. However, her neurological condition continued to deteriorate gradually. Sequential CSF studies showed that the glucose level remained extremely low, it even decreased to 0 mg/dl Eight months after the surgical treatment, MRI with Gd-DTPA revealed marked subarachnoid enhancement in both intracranial and spinal areas. An open biopsy was performed and a histological diagnosis of intracranial and spinal seeding of the anaplastic ependymoma was returned. CONCLUSIONS: We report a patient with intracranial and spinal seeding of an anaplastic ependymoma that mimicked fungal meningitis. We discuss the difficulty of obtaining a differential diagnosis in this case and describe the mechanism of the decreased CSF glucose level. ( info) |
26/569. 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. ( info) |
27/569. 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). ( info) |
28/569. 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. ( info) |
29/569. 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. ( info) |
| Acute paraplegia is a rare presentation for a spinal cord ependymoma Among spinal cord tumors ependymomas are most commonly associated with subarachnoid hemorrhage and there is evidence that some have had intratumoral hemorrhage, but most of these bleedings pass without symptoms. In this report, a case of spinal cord ependymoma debutting with acute neurological deterioration due to tumoral bleeding is presented. We discuss the clinical and neuroradiological findings and review the literature related to this unusual presentation. ( info) |