1/147. 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.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/147. ependymoma of the fourth ventricle presenting with hemifacial spasm. Report of a case.According to Gardner's hypothesis (1962) later confirmed by Jannetta (1982, 1985), hemifacial spasm can usually be related to a "vascular conflict" which takes place inside the cerebellopontine angle (CPA). Occasionally, the causative lesion can be identified as a mass encasing the facial nerve at its root exit zone (REZ) from the brain stem. The hemifacial spasm has been rarely reported in presence of a contralateral CPA mass ("false localising sign"). hemifacial spasm in patients with masses in anatomical regions other than the CPA has to be considered exceptional. The case of an adult man harboring an ependymoma of the fourth ventricle whose only neurological sign was a left hemifacial spasm is reported. The rarity of such a condition prompted us to review the literature. Particular attention has been paid to the possible pathogenetic mechanisms and their therapeutic implications.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
3/147. Sacrococcygeal ependymoma: case report and review of the literature.Ependymomas are uncommon glial cell tumors found within the brain, spinal cord, cauda equina, or filum terminale. Ependymomas originating in the parasacral soft tissues are exceedingly rare. The authors report the case of a 7-year-old boy with a primary ependymoma of the sacrococcygeal area and review the world literature of this tumor at this location.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
4/147. 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.- - - - - - - - - - ranking = 6.460852963296keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/147. 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.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
6/147. The tanycytic ependymoma of the lateral ventricle: case report.The tanycytic ependymoma is an extremely rare, primitive neuroectodermal tumor, arising from the ependymoglial cells or tanycytes. Such cells are generally seen in the primitive nervous system instead of the mature ependymal cells. The tanycytic ependymoma described in this report was found in a 42-year-old man. Histological analysis strongly suggested that this tumor originated from a primitive progenitor cell, the ependymoglia or the tanycyte in the lateral ventricle.- - - - - - - - - - ranking = 1.5447460221968keywords = nervous system (Clic here for more details about this article) |
7/147. Sacrococcygeal extraspinal ependymomas: the role of coccygectomy.BACKGROUND: Ependymomas, the common glial tumors of the spinal cord, occur occasionally outside the central nervous system and are called exstraspinal ependymomas (EEP). EEPs are found primarily in sacrococcygeal region during childhood. The pathogenesis and the treatment of the sacrococcygeal (SC) ependymomas are still controversial. Therefore, we present our case with metaanalysis of other case reports to determine the optimal treatment modality for SC EEPs. methods: A metaanalysis of case reports of SC EEPs, including the current case, was conducted. Also all available case reports of EEPs, without age limit, were analyzed to determine the distribution of EEPs localization. RESULTS: EEPs usually are found in teratoma localizations such as the SC area, ovary, paraovarian structures, and medastinum. The distribution of EEPs localization differs with age. Local recurrence rate of EEPs after coccyx excision is zero, however, it increases to 71% when the coccyx was left behind. CONCLUSION: The identical clinical characteristics of the SC teratomas and EEPs imply that the SC EEPs may be monophasic teratomas as their ovarian counterparts are named. coccyx excision is an important part of the surgical treatment of these tumors, with an apparent decrease in the recurrence rate.- - - - - - - - - - ranking = 6.460852963296keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/147. 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.- - - - - - - - - - ranking = 8keywords = brain (Clic here for more details about this article) |
9/147. 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.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
10/147. 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.- - - - - - - - - - ranking = 20.382558889888keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
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