1/131. central nervous system atypical teratoid tumor/rhabdoid tumor: response to intensive therapy and review of the literature.central nervous system atypical teratoid/rhabdoid tumor (ATT/RT) of infancy and childhood is a unique histologic entity with an extremely aggressive natural history. Standard therapy for infant and childhood medulloblastoma, for which this entity is often mistaken, has been ineffective; most children survive less than 12 months after diagnosis. Intensified therapy has been recently used for children with this disease, with promising results [1,2]. We report four cases of ATT/RT in young children; all had subtotal resections and localized disease at diagnosis. One child treated prior to bone marrow transplant availability died of progressive disease 9 months after diagnosis. Another child, treated with high-dose chemotherapy and radiotherapy in preparation for bone marrow transplant, had a recurrence and died 20 months after diagnosis, without undergoing the transplant. Two children received high-dose chemotherapy and autologous bone-marrow transplant and had a good response to treatment; one survived 19 months, the other child is free of disease 46 months from diagnosis. Intensified therapy has altered the natural history of central nervous system ATT/RT.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/131. Atypical teratoid/rhabdoid tumor of the CNS: cytopathology and immunohistochemistry of insulin-like growth factor-II, insulin-like growth factor receptor type 1, cathepsin d, and Ki-67.insulin-like growth factor (IGF)-II is a potent growth factor, normally controlled by a number of other factors, including IGF binding proteins and IGF binding protein proteases. In general, the latter increase the bioavailability of IGF by cleaving IGF binding proteins. cathepsin d (an IGF binding protein protease) was also implicated in tumor invasion. Although IGF-II was implicated in the pathogeneses of various childhood neoplasms, its significance in the pathogenesis of atypical teratoid/rhabdoid tumor of the central nervous system (ATRT-CNS) was not studied to date. We present clinicopathologic features of two cases of ATRT-CNS. In addition, formalin-fixed, paraffin-embedded tissue sections were stained immunohistochemically for IGF-II, IGF receptor type 1, cathepsin d, and Ki-67. Both tumors demonstrated diffuse strong cytoplasmic positivity for IGF-II, diffuse cytoplasmic and focal membranous positivity for IGF receptor type I, and diffuse cytoplasmic positivity for cathepsin d. The Ki-67 labeling indices were 10.0% and 1.4%. We conclude that ATRT-CNS cells express both IGF-II and IGF receptor type 1, supporting the hypothesis that autocrine/paracrine stimulation of cell growth by IGF-II might be one mechanism involved in ATRT-CNS tumorigenesis. cathepsin d expressed by the tumor cells might also be involved in both tumor cell invasion and growth. The exact pathogenesis of ATRT-CNS remains to be elucidated.- - - - - - - - - - ranking = 0.45360624254611keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/131. Antenatal sonographic diagnosis of epignathus at 15 weeks of pregnancy.Epignathus is a rare, benign, congenital teratoma of the hard palate. Most of these teratomas are unidirectional and protrude through the mouth. Hence, the prognosis depends on the size of the tumor and the degree of face distortion and airway obstruction that it causes. However, some epignathi protrude bidirectionally, involving and destroying the brain tissue, resulting in a poor prognosis. This report presents a case of ultrasonographic detection of a bidirectional epignathus at 15 weeks of pregnancy.- - - - - - - - - - ranking = 0.074257767589204keywords = brain (Clic here for more details about this article) |
4/131. glioblastoma multiforme in a mature ovarian teratoma with recurring brain tumours.AIMS: We report a case study to highlight the occurrence of glioblastoma multiforme in an ovarian teratoma. methods AND RESULTS: A 10-year-old girl presented with a left frontal lobe primitive neuroectodermal tumour which was successfully treated. After 6 uneventful years, she developed glioblastoma multiforme located posterior to the site of the initial tumour. Six years later, she presented with a mature cystic teratoma containing glioblastoma multiforme. CONCLUSIONS: glioblastoma in an ovarian teratoma is an exceptional event, which might have an initial clinical presentation as a metastatic brain tumour. Alternatively, recurring glial tumours may occur in a genetically predisposed person; the role of radiation and chemotherapy in this context remains to be elucidated.- - - - - - - - - - ranking = 0.37128883794602keywords = brain (Clic here for more details about this article) |
5/131. Reversible paraneoplastic encephalomyelitis associated with a benign ovarian teratoma.BACKGROUND: Paraneoplastic encephalomyelitis (PEM) is a well characterized, and typically irreversible, paraneoplastic syndrome, usually associated with small cell lung cancer or other malignancy. We describe a case of a young woman with a benign ovarian teratoma who presented with a reversible PEM. CASE REPORT: A 24-year-old woman presented with a three week history of memory impairment, unusual behavior, personality changes, auditory hallucinations, hypersomnolence and binocular biplopia. On admission she was disoriented and inattentive with impaired short term memory. Small doses of lorazepam (1 mg), given for episodic agitation, repeatedly induced multidirectional bilateral nystagmus and a skew deviation, but her neurological examination was otherwise normal. A left-sided pelvic mass was palpable. brain MRI pre- and post-gadolinium was normal. There was a mild CSF pleocytosis and an EEG showed minimal bilateral background activity irregularities. There were no other laboratory abnormalities. Two weeks after admission, she clinically deteriorated developing central respiratory failure and a flaccid paraplegia. Repeat MRI showed an area of increased T2 weighted signal in the medulla and three similar areas in the spinal cord. Following removal of her tumor, treatment with high dose corticosteroids and intravenous immunoglobulin, she ultimately made a full recovery. pathology revealed the tumor to be a benign ovarian cystic teratoma. CONCLUSIONS: This is the first report of a reversible PEM seen in association with a benign tumor, in this case a mature ovarian teratoma. Presumably, an immune response directed against neural clements of the teratoma cross-reacted with normal brain, brainstem and spinal cord antigens to cause neurologic symptoms. Tumor removal was followed by neurologic recovery.- - - - - - - - - - ranking = 0.14851553517841keywords = brain (Clic here for more details about this article) |
6/131. Dysembryoplastic neuroepithelial tumor. A case report.Dysembryoplastic neuroepithelial tumor (DNT) is a rare, benign tumor encountered in the cortex. It is characterized by the presence of cells of different histogenesis. Due to its mixed nature (glial-neuronal), WHO histological classification of brain tumors included it into the group of neuronal and glial-neuronal mixed tumors. Case of tumor in a 19-year-old woman experiencing for three years seizure of temporal lobe epilepsy is presented. A cranial magnetic resonance imaging (MRI) showed "pseudocystic" tumor in temporal lobe. Histological and immunocytochemical examinations of the tumor fragment removed during surgery revealed large numbers of neuronalglial nodules occurring in the cerebral cortex. Columns of glial-neuronal structures crossing parallely to the cortex surface, surrounded by oligodendrocyte-like cells (OLC) were a characteristic feature of the tumor texture. In the tumor interstitium, "floating" maturated, dysplastic-free ganglionic cells were visible in numerous bright spaces. In addition, numerous lobuliform--structured areas consisted of oligodendrocyte-like cells. Oligodendrocyte-like cells were characterized by positive immunoreaction to the presence of S-100 protein and synaptophysin. Basing on clinical manifestation and histopathological findings dysembryoplastic neuroepithelial tumor was diagnosed.- - - - - - - - - - ranking = 0.074257767589204keywords = brain (Clic here for more details about this article) |
7/131. 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 = 0.45360624254611keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/131. Germline INI1 mutation in a patient with a central nervous system atypical teratoid tumor and renal rhabdoid tumor.We describe a four-month-old child who presented with an atypical teratoid/rhabdoid tumor of the brain and subsequently developed a renal rhabdoid tumor. Distinct histologic features, immunophenotypic profiles, and deletions of chromosome 22 were supportive of two primary tumors. An identical mutation in exon 7 of the INI1 rhabdoid tumor suppressor gene was identified in both tumors, as well as in normal kidney tissue. We propose that this germline INI1 mutation predisposed the child to the development of both malignancies. These findings lend support to the hypothesis that rhabdoid tumors in all sites have a common genetic etiology.- - - - - - - - - - ranking = 1.8886827377736keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
9/131. Spinal atypical teratoid/rhabdoid tumor in an infant.Atypical teratoid/rhabdoid tumor of the central nervous system in infancy and childhood was established as an entity based on histological, immunohistochemical, and cytogenetic studies. We report the case of a 7-month-old girl who presented with progressive paraplegia and hypesthesia of her legs. Imaging studies revealed a spinal cord mass occupying the entire spinal canal below the T(7) level. Through a T(12)-L(3) laminectomy, the intramedullary tumor was partially debulked. Histologically, the tumor specimen had rhabdoid cells, and immunostaining showed vimentin and cytokeratin positivity. No abnormality of chromosome 22q was detected with the fluorescence in situ hybridization method.- - - - - - - - - - ranking = 0.45360624254611keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/131. central nervous system atypical teratoid/rhabdoid tumour of infancy. CT and mr findings.In 1995, as a result of the observation of Rhabdoid elements among the other components of a Teratoid/rhabdoid tumor (TRT), a new nomenclature was introduced, Atypical Teratoid/rhabdoid tumor (ATRT) of infancy and childhood. We report the clinical history and radiological findings in a child affected by central nervous system (CNS) ATRT.- - - - - - - - - - ranking = 0.89072124850922keywords = central nervous system, nervous system (Clic here for more details about this article) |
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