Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe the case of a 31-year-old woman who was first treated for a pigmented choroid plexus papilloma of the fourth ventricle. Ten year later, she developed a new tumor in the region of the cauda equina. This second neoplasm contained areas of papillary ependymoma that displayed phosphotungstic acid hematoxylin-positive glial fibers and immunoreactivity for glial fibrillary acidic and S-100 proteins. Areas of ependymoma merged with others that displayed the appearance of a paraganglioma, including lobules and nests of chief cells immunoreactive for neuron-specific enolase, synaptophysin, chromogranin, and serotonin. Satellite cells, but not chief cells, stained for glial fibrillary acidic and S-100 proteins. Electron microscopy showed features of both ependymal and paraganglionic differentiation, including intercellular lumina with microvilli, junctional complexes, cell processes with closely packed filaments, and dense core granules. Our case represents a rare example of a cauda equina neoplasm with simultaneous ependymal and paraganglionic differentiation. To our knowledge, this is the first described example of a tumor of this region showing features of both ependymoma and paraganglioma.
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PMID:Cauda equina tumor with ependymal and paraganglionic differentiation. 161 83

Three cases of periventricular neurocytomas are presented. All patients had a large but well-circumscribed, hyperdense tumor with insignificant contrast enhancement in the lateral and third ventricle, causing hydrocephalus. Calcification was present in one patient. Angiography revealed a blush tumor enhancement in two cases. Surgical removal was complete in two patients and incomplete in one. Light microscopy showed a cell pattern that resembled either ependymoma or oligodendroglioma. However, in all cases the tumor was confirmed to be a neurocytoma by immunocytochemical analysis that showed reactivity for synaptophysin and/or neuron-specific enolase in a high percentage of neoplastic cells. With respect to the literature it is concluded that neurocytomas represent an individual pathological entity of supratentorial midline tumors. Complete surgical removal without irradiation is the recommended treatment.
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PMID:Periventricular neurocytoma: a pathological entity. 161 72

Hemicorporectomy (HCP) is infrequently used, but its effects can devastate the patient's body image, autonomic function, and physical abilities even more than a spinal cord injury of comparable level. Interdisciplinary management is of the utmost importance. This report describes the 2 year course, including four separate rehabilitation admissions, of a patient who was initially paraplegic, and then underwent a HCP for complications secondary to a cauda equina ependymoma. The patient's expectations for functional independence were established by his successful initial spinal cord rehabilitation. The HCP was performed 6 months after initial discharge secondary to infected Harrington rods and rapid spread of the tumor. Extensive use of the interdisciplinary team approach allowed comprehensive analysis and treatment of the patient's comfort, mobility, skin tolerance, and upper extremity functional abilities. Four successive prostheses were developed and modified, until all concerns were successfully addressed. The patient ultimately became completely independent at the wheelchair level. The evaluations, treatment plan, and emphasis of each discipline, including physical therapy, occupational therapy, rehabilitation nursing, therapeutic recreation, social work, vocational rehabilitation, and physiatry are summarized. Differences between the patient's course and standard spinal cord rehabilitation are detailed.
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PMID:Interdisciplinary management of hemicorporectomy after spinal cord injury. 162 24

Although ependymomas comprise 5-10% of pediatric brain tumors, consistent cytogenetic aberrations have not been identified in these neoplasms. We report karyotypes for two ependymomas. A predominantly well-differentiated ependymoma contained several numerical chromosome aberrations, including monosomy 22. In contrast, an anaplastic ependymoma had a more complex karyotype that included loss of one chromosome 22 homologue and a balanced translocation at q13.3 in the remaining 22 homologue. These findings suggest the location of an ependymoma tumor suppressor gene on the long arm of chromosome 22.
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PMID:Cytogenetic evidence for a chromosome 22 tumor suppressor gene in ependymoma. 163 2

A rare case of myxopapillary ependymoma is reported. The tumor occurred in the cerebral hemisphere of an 8-year-old girl and had no relationship to the lateral ventricles. Microscopically, it showed abundant mucin production around papillary or reticular structures. Immunohistochemically, these tumor cells were weakly positive, with glial fibrillary acidic protein demonstrated in part of the tumor and vimentin strongly demonstrated throughout the tumor. The results may indicate the poorly differentiated nature of this tumor. This is the second reported case of intracranial myxopapillary ependymoma.
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PMID:Cerebral myxopapillary ependymoma. 164 42

We present a case of a 25-year-old patient at term pregnancy who presented with a bilateral ovarian neoplasm that was histologically and immunohistochemically indistinguishable from ependymoma of the central nervous system. Progesterone receptors were detected in primary and recurrent neoplasms by immunohistochemistry. This is the first case of this rare neoplasm to present during pregnancy as well as with bilateral ovarian involvement. Together with a previously reported case of recurrent ovarian ependymoma with estrogen and progesterone receptors, this case suggests that hormonal responsiveness of this rare neoplasm may be pathogenically significant.
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PMID:Progesterone receptors in bilateral ovarian ependymoma presenting in pregnancy. 164 43

For 100 operated patients with malignant gliomas of the brain (63 malignant gliomas WHO grade IV--including 50 glioblastoma multiforme- and 37 astrocytomas WHO grade III-IV) the average survival time is 332 days. The survival rates show 6, 12 and 24 months after operation 55%, 32% and 12% of the patients alive. In addition to therapy there are some favourable prognostic factors: Young patients age at the onset of illness, localization in the non-dominant hemisphere, minor preoperative neurological deficit. On the other side unfavourable prognostic factors are: parietal localization (average survival time = 120 days), tumorous infiltration of the brain stem (average survival time = 143 days), preoperative clouding of consciousness (average survival time = 185 days). These factors influence survival time. The most important prognostic factor of therapy is the extent of operation: In case of macroscopical total resection of the tumor the mean survival time amounts to 554 days as compared to 202 days in subtotal tumor resection. The well known positive influence of postoperative radiation therapy is demonstrated by the high mean survival time of 456 days. In case of recurrent operation of the tumor (28 patients), the average survival time amounts to 443 days. In contrast to the literature our own analysis shows no prognostic value of histological variants in case of malignant glioma WHO grade IV. Two patients, whose histological slide preparations were neuropathologically graded a second time independent from the first view (1 anaplastic ependymoma WHO grade III and 1 glioblastoma multiforme WHO grade IV), are still alive more than 8 years after operation without any neurological deficit.
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PMID:[Malignant brain glioma--a catamnestic study of 100 operated patients]. 165 65

The MR examinations in 25 patients with intramedullary tumors were analyzed. Seven patients were diagnosed with astrocytoma, 6 ependymoma, 2 unspecified glioma, 3 medulloblastoma, 2 metastasis, one neurinoma, and one teratoma. In 3 patients the diagnosis was uncertain. The tumors frequently involved a large portion of the cord and were often accompanied by intratumor necrosis, cystic degeneration, and edema, which was well demonstrated on MR. Gd-DTPA was used in 6 patients and was helpful in separating solid tumor components from cysts and edema. It was difficult to separate different kind of tumors based on morphologic and signal characteristics on MR. Some prominent features could, however, be distinguished. Complete cystic degeneration was more common in astrocytomas than in other tumors, and ependymomas frequently had a heterogeneous signal pattern on both T1- and T2-weighted sequences. The single teratoma had a characteristic content of fat and calcification, and the melanoma had a signal pattern consistent with blood. CSF pathway spread in cases of medulloblastoma was demonstrated by ill-defined contour of the cord and CSF or tumor nodules on the surface of cord and nerve roots.
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PMID:MR imaging of spinal intramedullary tumors. 166 Feb 97

There are few population-based studies of intracranial tumours in children. This study examines 93 brain tumours in children up to 18 months of age from over 800 childhood central nervous system tumours reported to the Manchester Children's Tumour Registry during the period 1953-1987. The incidence was 1 per 25,000 live births. Of these tumours, 85% were malignant. The three most common tumours were medulloblastoma, ependymoma and juvenile astrocytoma. Eighteen percent of the tumours were in the axial region. Twenty-five percent were connected to the ventricles. The tumour was supratentorial in two-thirds of the children under 6 months of age. A high incidence of tumours in the families of these children was found; tumours were noted in two sets of siblings.
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PMID:Intracranial tumours in the first 18 months of life. 166 43

We detected a germ-line mutation of the p53 gene in a patient with a malignant ependymoma of the posterior fossa. This mutation, which was found at codon 242, resulted in an amino acid substitution in a highly conserved site of exon 7 of the p53 gene; the same mutation was found in both the germ-line and the tumor tissue. This is the most common region of previously described somatic p53 mutations in tumor specimens and of the germ-line p53 mutations in patients with the Li-Fraumeni cancer syndrome. Evaluation of the patient's family revealed several direct maternal and paternal relatives who had died at a young age from different types of cancer. The association of a germ-line p53 mutation with an intracranial malignancy and a strong family history of cancer suggests that p53 gene mutations predispose a person to malignancy and, like retinoblastoma mutations, may be inherited.
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PMID:Identification of a germ-line mutation in the p53 gene in a patient with an intracranial ependymoma. 167 37


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