Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

According to the WHO grading system, myxopapillary ependymomas are assigned to WHO Grade I. However, the clinico-pathological spectrum might be very heterogenous. Herein, we report 4 cases exhibiting lumbar tumor masses, 1 causing muscular atrophy over a 30-year period, 3 displaying clinical history of persisting lumbar pain for only several weeks. All tumors were crooked with dura and spinal roots resulting in incomplete resection in three cases. On histological examination, two tumors were almost acellular and showed polycyclic hyaline and fibrotic extracellular matrix leading to differential diagnoses of chordoma, meningioma, fibrolipoma and ependymoma. Finally, together with the immunohistochemical investigations, electron microscopy led to the diagnosis of myxopapillary ependymoma, WHO Grade I, with massive degenerative changes. The other 2 cases presented with the typical neuropathology of myxopapillary ependymomas but showed local recurrence within 1 and 13 years throughout the whole neuraxis, and in 1 case additional metastases of the 3rd ventricle. Although the morphological feature of these myxopapillary ependymomas was benign, the presented cases showed that the biological behavior of myxopapillary tumors might differ greatly and that these tumors present a serious operative and diagnostic challenge. Myxopapillary ependymomas occur most often in the lumbosacral region. Due to the anatomic complexity of the cauda equina, a complete resection can be technically challenging in this region. However, a gross total resection at the primary surgery is the most predictive factor for the outcome.
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PMID:The clinico-surgico-pathological spectrum of myxopapillary ependymomas--report of four unusal cases and review of the literature. 1825 71

The objective of this study was to identify differentially expressed and prognostically important genes in pediatric medulloblastoma and pediatric ependymoma by Affymetrix microarray analysis. Among the most discriminative genes, three members of the SOX transcription factor family were differentially expressed. Both SOX4 and SOX11 were significantly overexpressed in medulloblastoma (median, 11-fold and 5-fold, respectively) compared with ependymoma and normal cerebellum. SOX9 had greater expression in ependymoma (median, 16-fold) compared with normal cerebellum and medulloblastoma (p<0.001 for all comparisons). The differential expression of the SOX genes was confirmed at the protein level by immunohistochemical analysis. Survival analysis of the most discriminative probe sets for each subgroup showed that 35 and 13 probe sets were predictive of survival in patients with medulloblastoma and ependymoma, respectively. There was a trend toward better survival with increasing SOX4 expression in medulloblastoma. SOX9 expression was predictive for favorable outcome in ependymoma. The mRNA levels of BCAT1, a mediator of amino acid breakdown, were higher (median, 15-fold) in medulloblastoma patients with metastases compared with those without metastasized disease (p<0.01). However, the correlation between BCAT1 expression and metastatic medulloblastoma could not be confirmed at the protein level. The potential prognostic effect of the genes associated with outcome should be evaluated in ongoing studies using larger groups of patients. Furthermore, our findings support further analysis of the functional properties of the selected genes, especially SOX4 and BCAT1 for medulloblastoma and SOX9 for ependymoma, to evaluate the use of these genes as potential tumor markers, prognostic markers, and drug targets in pediatric brain tumors.
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PMID:Differential expression and prognostic significance of SOX genes in pediatric medulloblastoma and ependymoma identified by microarray analysis. 1857 62

Subependymal giant cell astrocytoma (SEGA) is a benign, slowly growing tumor typically occurring in the setting of tuberous sclerosis complex (TSC). However there are several reported cases in which patients with a solitary SEGA had no other stigmata of TSC. We describe a case of SEGA in a 75-year-old woman representing the oldest patient reported to-date. The patient had a history of radical vulvectomy for malignant melanoma (MM), and died of autopsy-confirmed widespread systemic metastasis. Postmortem examination of the brain revealed a single 2.1 x 1.0 x 0.8 cm intraventricular nodule in the lateral ventricle. Histologically, it was composed of interlacing bundles of spindle-shaped tumor cells with thin delicate processes admixed with relatively large pleomorphic cells with abundant glassy eosinophilic cytoplasm, as seen in a SEGA. Immunohistochemically, GFAP, S-100 protein, and neuron specific enolase were positive, and synaptophysin labeled a few tumor cells. Also noted were rare isolated MM cells within the tumor (i.e., tumor-to-tumor metastasis). Autopsy showed no manifestations of TSC systemically or intracranially. The histopathological differential diagnosis was limited and included giant cell ependymoma and, much less likely, giant cell glioblastoma and pleomorphic xanthoastrocytoma. This case illustrates that SEGA can be found incidentally in an elderly individual with no associated symptoms and also indicates that SEGA can occur outside the setting of TSC. Tumor metastasis to an occult SEGA is extremely rare.
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PMID:Solitary subependymal giant cell astrocytoma incidentally found at autopsy in an elderly woman without tuberous sclerosis complex. 1867 43

We report a divergent ependymal tumor of the posterior fossa (ependymoblastoma/anaplastic ependymoma) observed in an 8-year-old boy. The tumor showed the histological pattern typical of an ependymoblastoma (tubular-papillary fetaloid architecture with stratification of the tumor cells) next to areas in which findings typical of an anaplastic ependymoma were detected. The immunohistochemical study confirmed our diagnostic suspicion, allowing us to establish a differential diagnosis with other entities such as medulloblastoma, medulloepithelioma, atypical rhabdoid/teratoid tumor, or metastases.
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PMID:Divergent ependymal tumor (ependymoblastoma/anaplastic ependymoma) of the posterior fossa: an uncommon case observed in a child. 1882 70

Nonresectable ependymomas are associated with poor prognosis despite intensive radiochemotherapy and radiation. The molecular pathogenesis of ependymoma initiation and progression is largely unknown. We here present a case of therapy-refractory, progressive ependymoma with cerebrospinal as well as extraneural metastases, which allowed us for the first time to follow the stepwise accumulation of chromosome aberrations during disease progression. Genome-wide DNA copy-number analysis showed sequential deletions on chromosomes 1, 9, and 14 as well as a homozygous deletion of the CDKN2A locus, underscoring its role in tumor progression. Gradual loss at 1p36 was associated with loss of protein expression of the putative tumor suppressor gene AJAP1/SHREW1. In summary, this is the first report on acquired genomic aberrations in ependymoma over time pointing to novel candidate tumor suppressor genes. This analysis provides molecular insights into the chronology of genetic events in this case from initial localized tumor to widespread metastasized disease.
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PMID:Stepwise accumulation of distinct genomic aberrations in a patient with progressively metastasizing ependymoma. 1902 95

A 50-year-old female complained of back pain. Computed tomography showed a well-defined spindle-shaped mass 3.4 x 1.0 cm in size in the left paravertebral posterior mediastinum at the Th 3-4 level. The tumor was completely resected via thoracoscopic surgery, which showed no invasion into the surrounding tissue. It consisted of a cyst with a tiny mural solid element. By means of histological and immunohistochemical examinations, we diagnosed the tumor as ependymoma, and the patient remains alive 59 months after resection. As far as we know, 8 reported cases with primary mediastinal ependymoma have been reported. They had the following specific characteristics: (1) All patients were adult females; (2) The tumors were usually located at the paravertebral upper mediastinum; (3) Most of the tumors consisted of cystic and solid elements; (4) The tumors usually made no invasion into the surrounding tissues, though 2 cases had lymph-node metastases.
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PMID:A case of primary mediastinal ependymoma. 1990 89

Clear-cell meningioma (CCM) is a rare subtype of meningioma which occurs at a younger age and has a higher recurrence rate than other subtypes (WHO grade II). CCM usually occur in younger patients and is located in the posterior fossa and spine. The authors report the first case of olfactory groove mixed clear-cell meningioma. A 66-year-old woman was admitted to the SS. Annunziata Hospital (Taranto, Italy) in January 2007 with a two-year history of subtle changes in personality and mental function. On neurologic examination she presented a loss of sense of smell. The magnetic resonance imaging (MRI) showed an olfactory groove meningioma. The computed tomography (CT) and MRI features of CCM are not different from those of common meningiomas. The tumor was totally removed by frontolateral approach on January 24, 2007). Histological examination showed that the tumor was composed of sheet-like uniform and polygonal cells, with abundant clear cytoplasm, and small and bland nuclei. The cytoplasm was heavily laden with granular periodic acid Schiff-positive and diastase-sensitive material representing glycogen. There were no rich vascular networks but scattered collagen bundles within the tumour, little foci areas of necrosis and whorls of meningothelial cells. The neoplastic cells were positive for epithelial membrane antigen (EMA) and vimentin, and negative for glial fibrillary acidic protein (GFAP)? S-100?chromogranin A; Ki-67 labelling showed an index of 1%. The final diagnosis was mixed clear-cell meningioma. Until now only 38 intracranial CCM cases had been reported in English language literature. Different diagnoses for CCM include lesions with clear cell appearance such metastases of renal cell carcinoma and sarcoma, hemangioblastoma, ependymoma, oligodendroglioma, germinoma, chordoma, pleomorphic xanthoastrocytoma, lipid-rich glioblastoma, microcystic and lipomatous meningioma.
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PMID:Clear-cell meningioma of the anterior cranial fossa. Case report and review of the literature. 2007 23

Magnetic resonance imaging is the current imaging modality of choice in the evaluation of patients presenting with myelopathic symptoms in the search for spinal cord lesions. It is important for the radiologist to recognize and differentiate nonneoplastic from the neoplastic process of the spinal cord as the differentiation of the 2 entities is extremely crucial to the neurosurgeon. This article presents a broad spectrum of benign intramedullary spinal abnormalities including syrinx, contusion, abscess, infarction, myelitis, multiple sclerosis, sarcoid, cavernoma, and arteriovenous malformation. Rare intramedullary neoplasms including dermoid tumor, astrocytoma, ependymoma, hemangioblastoma, lymphoma, ganglioneuroblastoma, and metastases are also illustrated. The clinical presentation and magnetic resonance signal characteristics as well as the differential diagnosis of the intramedullary lesions are discussed. The potential pitfalls in the differentiation of tumors from nonneoplastic disease of the spinal cord are also elucidated.
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PMID:Magnetic resonance imaging of intramedullary spinal cord lesions: a pictorial review. 2051 Jul 54

Swelling of the spinal cord and/or enhancement after intravenous gadolinium administration are not always specific features of intramedullary tumour. These may also be seen in association with several diseases of inflammatory, infectious, granulomatous or vascular origin. A tumour is characterized by its sagittal location, axial topography: central, lateral or exophytic, its size and size of the spinal canal, macroscopic components: calcium, fat, methemoglobin, melanin, hemosiderin, vascular pedicle, cystic component, enhancement after intravenous gadolinium administration, effect on the spinal cord tracts and edema. Characteristics: astrocytoma is lateral and infiltrative, ependymoma is central with white matter tract displacement and hemosiderin cap, hemangioblastoma is postero-lateral and shows enhancement with a vascular pedicle, metastases are very edematous or leptomeningeal in location.
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PMID:[Intramedullary tumours and pseudotumours]. 2081 90

Anaplastic ependymoma is a malignant glial tumor thought to arise from radial glial cells of the ventricular zone. Because ependymoma is frequently encountered within ventricular spaces, they are prone to leptomeningeal dissemination. Metastatic extracranial ependymoma has been reported, but in the context of progressive intracranial disease. We report on a boy who developed isolated extracranial recurrence of his anaplastic ependymoma, initially at the scalp and later metastases to cervical lymph nodes. The location of tumor recurrence proximate to the surgical site suggested surgical seeding. This case demonstrates an unusual site of recurrence of anaplastic ependymoma and highlights a previously underappreciated surgical complication.
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PMID:Isolated extracranial recurrence of anaplastic ependymoma. 2083 Jul 72


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