Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The case is described of a 23-year-old female patient presenting with unilateral proptosis, headaches, and transient epiphora. Surgery revealed an encapsulated tumour composed exclusively of spindle-shaped cells within a richly vascularised myxoid stroma. Immunohistochemical staining showed focal positivity for smooth muscle actin, vimentin, and glial fibrillary acidic protein. These combined findings are interpreted as providing evidence of a myoepithelioma, which may be regarded as a monomorphic adenoma consisting solely of myoepithelial cells. To our knowledge this is only the second report of such a tumour in the lacrimal gland.
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PMID:Myoepithelioma of the lacrimal gland: report of a case with spindle cell morphology. 132 39

We report a case of Rathke's cleft cyst in pituitary tumor. A 31-year-old woman admitted to our hospital complained of visual disturbance, headache, amenorrhea and galactorrhea. Serum prolactin level was 3,060 ng/ml. By means of CT scan and MRI, we found suprasellar-extending tumor to have cystic component in the center of the tumor. Transsphenoidal surgery revealed grayish jelly-like content in the cyst. Histologically tumor cells were composed of chromophobic, basophilic and eosinophilic cells. The cyst wall in the pituitary adenoma was composed of ciliated or non-ciliated columnar epithelium intermingled with goblet cells, being similar to Rathke's cleft cyst. Except for the cells of the cyst wall, most of the tumor cells were immunoreactive for prolactin. As for intermediate filament, tumor cells in both solid and cystic portions showed positive immunoreactivity for cytokeratin and GFAP, and not for vimentin and neurofilament. These results suggest that the nature of the intracytoplasmic filament in the pituitary tumor with Rathke's cleft cyst may be not only keratin but also GFAP.
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PMID:[A case of prolactinoma in close association with Rathke's cleft cyst]. 332 Aug 6

Malignant schwannomas are rare neoplasms that are seldom found in the head and neck. Few cases have been reported involving paranasal sinuses and none of them was of the "epithelioid" type. In this report, an unusual case of epithelioid malignant schwannoma involving the maxillary sinus, nasal cavity and orbit is presented. The patient was a 27-year-old male with a history of headache, nasal obstruction and epistaxis. Histologically, the tumour had a biphasic pattern with spindle and epithelioid elements which led to a differential diagnosis with malignant melanoma. It had also to be distinguished from other neoplasms, such as squamous cell carcinoma and olfactory neuroblastoma because of it location. Immunohistochemical positivity for S-100 protein, glial fibrillary acidic protein and vimentin together with negativity for HMB-45 and cytokeratins, as well as mesaxon formation detected with electron microscopy were conclusive in the diagnosis. The patient was treated with surgical excision and radiotherapy but local recurrence and metastases occurred, and he died within 1 year after initial diagnosis.
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PMID:Malignant sinonasal epithelioid schwannoma. 811 30

Granular cell tumours of neurohypophysis are rare. These tumours are more often encountered as incidental autopsy findings seen in up to 17% of unselected adult autopsy cases. There are few reports of parasellar granular cell tumours large enough to cause symptoms. We present three cases of neurohypophysis granular cell tumour and a review of the literature. In one patient, the asymptomatic granular cell tumour was incidentally discovered at surgical removal of a corticotroph microadenoma. The remaining 2 patients had a symptomatic tumour which caused neurological symptoms such as visual disturbance and headaches and endocrine disorders such as hypopituitarism or hyperprolactinaemia. In these 2 cases, computerized tomography showed a well-circumscribed, contrast-enhanced, intrasellar and suprasellar mass. Magnetic resonance imaging demonstrated an isointense gadolinium-enhanced mass in T1-weighted images. Transsphenoidal partial resection was performed and histology was interpreted as a granular cell tumour. The immunohistochemical study was positive for glial fibrillary acidic protein (GFAP) and neuron specific enolase (NSE) in 1 of the 2 tumours and positive for S100 protein and vimentin in both tumours but negative for CD68. The histogenesis of neurohypophysis granular cell tumours is still controversial but ultrastructural and immunohistochemical studies support the theory that they may arise from pituicytes, the glial cells of neurohypophysis. Management of these benign, slow-growing, tumours is based mainly on neurosurgical resection. Data from the literature do not support a beneficial effect of postoperative radiation therapy on postoperative recurrences.
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PMID:[Granular cell tumors. Rare tumors of the neurohypophysis]. 854 14

Teratoid/rhabdoid tumor is a recently introduced infantile brain neoplasm of uncertain origin clinically characterized by a most aggressive course. We describe the case of a 2-year-old boy and a 7-year-old girl, the former presenting with central fever and hemiparesis, while vomiting and headache were symptoms in the latter. A brain tumor in the right fronto-parieto-occipital and the left fronto-medial parenchyma, respectively, were found and removed by surgery. On histology, both lesions consisted of sheets of ovoid cells with prominent cytoplasm, vesicular nuclei and macronucleoli. In the second case, a spindle-cell component and bone metaplasia were also noted. Ultrastructurally, tumor cells contained whorls of intermediate filaments. Immunohistochemistry revealed a polyphenotypic expression profile including positivity for epithelial membrane antigen, vimentin and a-actin. There was no evidence of a germ-cell origin. Chromosomal translocation involving 22q11 was established cytogenetically in the first case. The teratoid/rhabdoid tumor shares many common traits with renal rhabdoid tumors and primitive neuroectodermal tumors as well as germ cell neoplasms; therefore its being an entity is debated.
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PMID:[Teratoid/rhabdoid tumor of the central nervous system]. 945 8

A surgical case of gliosarcoma in which the mesenchymal component showed angiosarcomatous features is reported. The neoplasm was extirpated from the right cerebral hemisphere of a 76-year-old man who presented with left hemiparesis and headache, and it was composed of both anaplastic astrocytomatous and sarcomatous elements. The sarcomatous element was composed of a proliferation of atypical endothelial cells that had pleomorphic, mitotically active nuclei and formed vascular lumina of irregular shapes or solid cellular sheets, and was accompanied by the deposition of collagenous matrix. The cytoplasm of some of the atypical endothelial cells was immunohistochemically positive for vimentin, factor VIII-related antigen, CD 34, and thrombomodulin. The MIB-1 labeling index of the sarcomatous component was higher than that of the astrocytomatous component.
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PMID:Gliosarcoma with angiosarcomatous features: a case report. 1032 47

The term "chordoid glioma" was recently introduced to denote a circumscribed, apparently low-grade neoplasm arising in or preferentially involving the third ventricle of middle-aged women. We report biopsy and postmortem findings in a 60-year-old woman with symptoms of forgetfulness, headache, and lethargy. Neuroimaging showed a contrast-enhancing third ventricular mass with obstructive hydrocephalus. The tumor was subtotally resected. Microscopically, it consisted of clusters and strands of epithelioid cells in a mucoid matrix. Its margins were remarkably discrete and showed little tendency to infiltrate surrounding brain parenchyma. The majority of neoplastic cells were glial fibrillary acidic protein (GFAP) and vimentin positive, whereas S100 protein labeled only individual cells. Stains for epithelial membrane antigen (EMA) and cytokeratin were nonreactive. There was no evidence of neuroendocrine differentiation or expression of estrogen and progesteron receptors. Lymphoplasmacellular infiltrates were noted throughout the lesion and at the tumor-brain interface. The MIB-1 labeling index averaged 1.5%. At present, chordoid glioma is considered a glial neoplasm of uncertain histogenesis with distinct clinicopathologic features.
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PMID:Chordoid glioma of the third ventricle: confirmatory report of a new entity. 1037 85

Pituicytoma is a rare, poorly characterized tumor of the sella and suprasellar region that is distinct morphologically from other local tumors and is thought to be derived from neurohypophyseal pituicytes. Clinical data, neuroimaging studies, and microsections were reviewed from nine such low-grade gliomas. Immunostains for glial, neuronal, and proliferation markers were performed on all nine tumors and six control neurohypophyses. Three tumors were studied ultrastructurally. Six men and three women, age 30 to 83 years (mean, 48 years), presented with visual symptoms, headache, or hypopituitarism. Magnetic resonance images showed solid, discrete, contrast-enhancing masses, four within the sella and five in the suprasellar space. The tumors consisted of sheets and/or fascicles of plump spindle cells with slightly fibrillar cytoplasm and slightly pleomorphic, oval-to-elongate nuclei with pinpoint nucleoli. Extracellular mucin was prominent in one tumor. Rosenthal fibers, granular bodies, and Herring bodies (granular axonal dilatations characteristic of the normal neurohypophysis) were lacking. Mitoses were rare or absent. MIB-1 labeling indices were low (0.5-2%). Tumor cells were strongly reactive for vimentin and S-100 protein, variably positive for glial fibrillary acidic protein, and nonreactive for synaptophysin and neurofilament protein. Cytoplasm varied in electron density and contained intermediate filaments. Neither meningothelial nor ependymal features were noted. Two tumors recurred at 20 and 26 months after subtotal resection, but none of the six completely resected tumors have done so. Pituicytomas are discrete, largely noninfiltrative low-grade gliomas of the sellar region that occur in adults. Their histologic appearance is distinct from pilocytic and ordinary, infiltrative astrocytomas. The distinction between pituicytoma and normal neurohypophysis is aided by the latter's content of axons, Herring bodies, and perivascular anucleate zones rich in axonal terminations. Although curable by total excision, subtotal resection can be associated with recurrence.
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PMID:Pituicytoma: a distinctive low-grade glioma of the neurohypophysis. 1071 49

Malignant rhabdoid tumor (MRT), described for the first time in 1978 in the kidney, has rarely been reported in other organs including the brain and has involved adults in only 3 cases. We described a case of MRT in a 32-year-old woman who presented with severe headache, nausea and sudden onset of visual disturbance. MRI showed a well-enhanced mass at the suprasellar region. Subtotal removal of the tumor was performed. However, tumor regrowth occurred after the operation (doubling time, 8.36 days) and spinal dissemination was detected. Therefore, chemotherapy and radiotherapy were administered focusing on the suprasellar lesion and the spinal cord. Pathologically, light micrographs showed rhabdoid cells with large, round, single or double nuclei with one prominent nucleolus and eosinophilic cytoplasmic inclusions. Electron micrographs were made of typical rhabdoid cells displaying bundles of intermediate filaments within the perikaryon. In immunohistochemical studies, EMA, vimentin, cytokeratin and SMA were positive. Pathological findings were consistent with those of MRT. Optimal treatment for this tumor has not been established. Our case may be useful in defining treatment for MRT.
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PMID:[Suprasellar primary malignant rhabdoid tumor in an adult: a case report]. 1076 34

A 50-year-old female was admitted with headache and visual disturbance. Neuroimaging demonstrated a well-demarcated large tumor attached to the falx cerebri. The tumor was totally removed by surgery. Histological examination showed that the tumor consisted of spindle cells with no pattern in the collagenous background. Staghorn-like blood vessels were common. Immunohistochemical study showed the tumor cells were strongly positive for CD34 and vimentin, but negative for epithelial membrane antigen. The diagnosis was solitary fibrous tumor arising from the falx cerebri. Solitary fibrous tumor is rare within the cranial cavity, and can be distinguished from meningioma and hemangiopericytoma by the histological, ultrastructural, and immunohistochemical findings.
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PMID:Solitary fibrous tumor arising from the falx cerebri--case report. 1115 98


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