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)

Epithelioid sarcomas are rare, morphologically distinct tumors that have a propensity to arise in the extremities. Brain metastasis from epithelioid sarcoma are a relatively rare occurrence. We report a case of brain metastasis in a 50-year-old man who was previously diagnosed with an epithelioid sarcoma arising in the elbow. Before the diagnosis of brain metastasis, he had developed an axillary lymph node metastasis. He presented with neurologic symptoms of progressively worsening headache and loss of vision on the right side. He underwent gross total resection of an occipital lobe mass. Histologically, the tumor was focally characterized by prominent perinecrotic pseudopalisading and demonstrated immunoreactivity with antibodies to cytokeratin AE1/3 and CAM5.2; the tumor did not stain with glial fibrillary acidic protein antibody. The literature is reviewed and the morphologic distinction between metastatic epithelioid sarcoma and other central nervous system neoplasms is discussed.
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PMID:Metastatic epithelioid sarcoma to the brain: palisaded necrosis mimicking glioblastoma multiforme. 1237 23

Recently, unusual examples of tumors of the mixed glioneuronal type have been reported, including the papillary glioneuronal tumor (PGNT). A 23-year-old woman with a 2-3 months history of headache and insomnia presented with a tumor. Neuroimaging showed a right temporal lobe cystic tumor with a mural nodule enhanced by contrast medium. She underwent gross total resection of the tumor. The tumor was histologically marked by a mixture of glial and neural components. A pseudopapillary component was comprised of highly hyalinized vessels surrounded by a single layer of thin spindle cells stained for glial fibrillary acidic protein. Specific abortive glial cells stained for vimentin/S-100 protein accompanied pseudopapillary structure. Intervening neural areas were occupied by neural cells of varying size, including neurocyte-like cell and ganglionic cells. Ganglionic cells demonstrated abnormal cluster, lack of normal polarity and epiperikaryal immunoreactivity for synaptophysin staining suggesting neural neoplastic nature. No mitotic activity or necrosis was noted. A MIB-1 labeling index was 1.8%. Our patient remains free of disease 33 months after surgical treatment.
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PMID:[Papillary glioneuronal tumor with highly degenerative pseudopapillary structure accompanied by specific abortive glial cells: a case report]. 1465 90

A 7-year-old boy was admitted to our hospital because of headache and frequent vomiting. The patient was noted to have papilloedema and mild palsy of the right abducent nerve. Magnetic resonance image (MRI) revealed a large tumor in the frontal base with tumoral hemorrhage. Angiography showed the tumor was fed by anterior meningeal arteries. At surgery, the tumor was arising in the dura mater at the frontal base, and was removed totally. Histological examination showed the tumor to be composed of small cells with uniform round nuclei and minimal cytoplasm. Immunohistochemical studies were positive for MIC-2, NSE, C-KIT, vimentin, Class III-beta tublin and glycogen, but negative for NFP, synaptophysin, chromogranin A and GFAP. MIB-1 labeling index was 40-50%. The tumor was histologically confirmed to be peripheral-type primitive neuroectodermal tumor(pPNET). Following surgery, he underwent whole brain, whole spine and local radiation therapy(30 Gy in total respectively) and received two 5-day cycles of chemotherapy, consisting of intravenous administration of cisplatin 20 mg/m2/day, etoposide 60 mg/m2/day and IFOS 900 mg/m2/day. After these therapies, follow-up radiological examination showed there was no recurrence of the tumor for 24 months. Intracranial pPNET is rare. Ewing sarcoma and pPNET(ES/pPNET) is the designation given to a family of small round cell tumor arising in bone or soft tissues. Intracranial PNETs are devided into central nervous system PNET(cPNET) and pPNET. It is necessary that intracranial PNETs are divided into two types of PNETs because of different prognosis between these tumors. MIC-2 is a specific marker for pPNET/ES family and is useful in the differential diagnosis of these two types of tumors.
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PMID:[A case of peripheral-type primitive neuroectodermal tumor arising in the dura mater at the frontal base]. 1511 48

A 51 year old caucasian male presented with headache, facial nerve paresis and continuing contraction of the visual field. CT scan revealed a singular intracerebral contrast enhancing lesion in the left frontal lobe. Intraoperatively the tumour was well demarcated. Frozen sections showed a high grade glioma. Paraffin sections revealed, in addition to the gliomatous component, some sharply demarcated nests of meningothelial cells. Immunohistochemistry with glial fibrillary acidic protein and epithelial membrane antigen confirmed a collision tumour consisting of a glioblastoma WHO-grade IV and a meningothelial meningioma WHO-grade I. The coincidence of these two different tumours at the same time and the same location leads us to the speculation, that the collision tumour might have been caused by malignant transformation of a reactive astrogliosis surrounding the meningioma.
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PMID:[Collisiontumour composed of glioblastoma and meningioma-a case report]. 1516 23

We present here an unusual case of papillary neuroepithelial tumor of the pineal region. The patient was a 29-year-old female who presented with headaches. A computed tomography scan revealed a tumorous lesion at the pineal region and hydrocephalus. The resected tumor was composed of columnar and cuboidal cells showing characteristics of papillary growth. The tumor cells exhibited diffuse and intense immunoreactivity to cytokeratins and neural cell adhesion molecule. The tumor expressed abundant levels of transthyretin (prealbumin) and appeared ependymal in nature, with numerous microlumens delineated by punctate and ring-like patterns in epithelial membrane antigen staining. Reactivity to synaptophysin and glial fibrillary acidic protein was observed only in the infiltrated non-neoplastic pineal parenchyma. These histological characteristics matched the description of the recently reported papillary tumor of the pineal region thought to originate from the specialized ependyma of the subcommissural organ (SCO). Transthyretin expression of the present case further supports the likelihood of SCO origin, as transthyretin is one of the proteins presumed to be secreted by human SCO.
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PMID:Papillary neuroepithelial tumor of the pineal region. A case report. 1522 40

We reported a rare case of supratentorial primitive neuroectodermal tumors (sPNET) and reviewed the literature. A 15-year-old girl presented with a one-month history of headache and vomiting. CT scans showed a huge, solid and cystic tumor with calcification, occupying the left anterior cranial fossa. The solid portion of the tumor was hypointense on T1-weighted images, slightly hyperintense on T2-weighted images, hyperintense on diffusion- weighted images, isointense on fluid-attenuated inversion recovery (FLAIR) images, and strongly enhanced after administration of contrast medium. The expansile tumor had a broad attachment to the dura matter of the anterior cranial fossa. The patient underwent an uneventful extirpation of the tumor. Microscopically, the solid tumor contained small, round poorly-differentiated cells with pleomorphic nuclei and brisk mitotic activity. The tumor cells were immunoreactive for synaptophysin and GFAP, whereas lack of MIC2 gene product expression was confirmed using the monoclonal antibody 12E7. The MIB-1-positive rate was 43%. The histological diagnosis was sPNET. Postoperatively, a dose of 30.6Gy was administrated to the whole brain and a boost of 19.6Gy to the T2-hyperintensity lesion. The patient successively received a dose of 30.4Gy to the whole spine and Linac stereotactic radiotherapy with a marginal dose of 16.8Gy at the tumor bed. Three months after radiotherapy the patient received chemotherapy using carboplatin and etoposide. Follow-up MR images showed no evidence of recurrent tumor 5 months after the radiochemotherapy. On the basis of MR findings on both diffusion-weighted and FLAIR images, preoperative diagnosis of sPNET may be important for choosing appropriate therapeutic strategies for this tumor.
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PMID:[Supratentorial primitive neuroectodermal tumor: report of a surgical case]. 1535 32

Astroblastoma is one of the very unusual type of tumors, whose histogenesis has not been clarified. It occurs mainly among children or young adults. Astroblastoma is grossly well-demarcated, and shows histologically characteristic perivascular pseudorosettes with frequent vascular hyalinization. Perivascular pseudorosettes in astroblastoma have short and thick cytoplasmic processes and blunt-ended foot plates. A 15-yr-old girl presented with headache and diplopia for one and a half year. A well-demarcated mass, 9.7 cm in diameter, was found in the right frontal lobe in brain MRI, and it was a well-enhanced inhomogenous mass. Cystic changes of various sizes were observed inside the tumor mass as well as in the posterior part of the mass, but no peritumoral edema was found. Histologically, this mass belongs to a typical astroblastoma, and no sign of anaplastic astrocytoma, gemistocytic astrocytoma or glioblastoma was found in any part of the tumor. Immunohistochemically, the tumor cells showed diffuse strong positivity for glial fibrillary acidic protein, S-100 protein, vimentin and neuron specific enolase, and focal positivity for epithelial membrane antigen and CAM 5.2, while showing negativity for synaptophysin, neurofilament protein, pan-cytokeratin and high molecular weight keratin.
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PMID:Astroblastoma: a case report. 1548 62

We describe a case of ependymoma with neuronal differentiation in form of neuropil-like islands. A 6-year-old boy presented at clinical examination for a short history of headaches and vomiting. Brain computed tomography showed a large, partially cystic, parieto-occipital lesion. The tumor was composed by glial fibrillary acidic protein-positive round cells with a perivascular arrangement and scattered neuropil-like islands, showing intense positivity for synaptophysin. Despite radiotherapy, the tumor recurred, showing frank features of anaplasia, but lacking the neuropil-like islands. The histological features of the tumor are discussed in the light of the concept that neuronal differentiation can occur occasionally in gliomas of different lineage without affecting the expected biological behavior.
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PMID:Ependymoma with neuropil-like islands: a case report with diagnostic and histogenetic implications. 1554 32

Medulloblastoma is a malignant invasive embryonal tumor of the cerebellum with preferential manifestation in children. The peak of occurrence is seven years of age. Seventy percent of medulloblastomas occur in individuals younger than 16. In adulthood, 80% of medulloblastomas arise in the 21-40 years age group. A 48-year-old male patient was admitted to the hospital with complains of headache, ataxia, morning vomiting and difficulty in speech was operated with the diagnosis of presence of mass of 4 x 7 cm size retaining a diffuse homogenous contrast in the posterior fossa. The diagnosis of desmoplastic medulloblastoma was given after histopathological examination. Immunohistochemical examination revealed that neoplastic cells showed staining with neuron-specific enolase and synaptophysin but not with glial fibrillary acidic protein. This lesion showed nodular, reticulin free-zones (pale islands) surrounded by densely packed, highly proliferative cells. The pale regions within the tumor did not contain reticulin fibers. Desmoplastic medulloblastoma is encountered especially in adulthood. This type of tumor rarely occurs beyond the fifth decade of life. We present a case of desmoplastic medulloblastoma in a 48-year-old male.
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PMID:Desmoplastic medulloblastoma in a 48-year-old male. 1557 57

An unusual case of glioblastoma with adenoid structures arising in a 30-year-old Japanese woman with neurofibromatosis type-1 (NF1) is reported. The patient was admitted to University of Miyazaki Hospital, complaining of headache, nausea and vomiting. From the neuroradiological findings the patient was diagnosed as having glioblastoma, and the tumor was surgically resected. Histologically, the tumor consisted mainly of dark basophilic cells showing prominent tubular or glandular structures surrounded by large eosinophilic cells, in addition to the typical glioblastoma features in the periphery of the tumor. Both cells showed strong stainability with glial fibrillary acidic protein (GFAP) and S-100 protein immunohistochemically, so that the tumor was classified as adenoid glioblastoma. Several cases of glioblastoma have been reported to reveal the adenoid or epithelioid differentiation. The patients with NF1 are prone to develop malignant tumors including glioblastoma, but no cases representing adenoid glioblastoma associated with NF1 have been reported. This report is considered to be the first case of adenoid glioblastoma arising in a patient with NF1. The recognition of the existence of epithelial features of glioblastoma would be important in differential diagnosis of epithelioid tumors of the brain including metastatic carcinomas.
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PMID:Adenoid glioblastoma arising in a patient with neurofibromatosis type-1. 1594 92


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