Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0268596 (EMA)
 2,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In this study, a clinicopathologically and immunophenotypically diverse group of T-cell neoplasms were evaluated by one- and two-color flow cytometry and/or immunohistochemistry for the presence of eight antigens (T10, T9, IL2-R, EMA, HLA-DR, LeuM1, Ki-1, and LeuM5) which are expressed in a hierarchical manner by phytohemagglutinin (PHA)-activated benign T cells. We found that 70 of the 72 T-cell neoplasms (97%) expressed at least one of these eight T-cell activation-associated antigens (T-AAgs) and that the number and type of T-AAgs expressed by the neoplastic T cells varied according to the clinicopathologic category of T-cell neoplasia. All 5 T-cell lymphoblastic malignancies expressed T10 and T9; 2 also expressed LeuM1. Twelve of 14 (86%) T cell chronic lymphocytic leukemias (T-CLL) expressed two to four T-AAgs, most frequently T10 (86%) and HLA-DR (79%). The 26 cutaneous T-cell lymphomas (CTCL) expressed between 2 and 5 T-AAgs, most commonly T9 (92%) and HLA-DR (92%), and least often T10 (12%) and EMA (15%). Twenty-six of 27 (96%) peripheral T-cell lymphomas (PTCL) expressed more than 4 T-AAgs. Each of the T-AAgs were expressed by between 22% (LeuM5) and 85% (T9) of the PTCLs. Some T-AAgs were preferentially expressed by the PTCLs in association with other T-AAgs, such as EMA in association with IL2-R and Ki-1. In addition, LeuM5 was preferentially expressed by CD4- CD8+ T-cell neoplasms. However, only 19 of the 72 (26%) T-cell neoplasms (3/5 lymphoblastic malignancies, 3/14 CLLs, 0/26 CTCLs, 13/27 PTCLs) expressed T-AAg immunophenotypic profiles paralleling those expressed by normal peripheral blood T cells activated in vitro with PHA. These results suggest that T-AAg expression by neoplastic T cells does not often mirror the hierarchical order of expression by activated benign T cells, implying that neoplastic T cells do not usually represent the precise malignant counterpart of activated benign, normal T cells.
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PMID:T-cell activation-associated antigen expression by neoplastic T-cells. 142 41

Ectopic adrenal cortical neoplasms are extremely rare, and only a few have involved the CNS. We report the first case of an intramedullary oncocytic adrenal cortical neoplasm of the spinal cord with immunohistochemical (IMHC) confirmation. A 27-year-old man presented with progressive lower extremity weakness, spastic paraparesis, decreased reflexes, and hypoesthesia below T10. A spinal myelogram showed cauda equina blockade and obliteration of sacral nerve roots. This prompted emergent surgical intervention. A well-circumscribed, approximately 3 x 2 cm, light brown to tan, intramedullary tumor was identified at the level of the conus medullaris. Histologically, the tumor showed sheets and nests of plump, cytologically bland polygonal cells with abundant eosinophilic cytoplasm. A single mitosis, but no necrosis, was identified. By IMHC, the cells were positive for inhibin, melan-A, and synaptophysin, and negative for GFAP, EMA, cytokeratins, S-100, HMB-45, and chromogranin. Electron microscopy study performed from paraffin-embedded tissues demonstrated abundant mitochondria, and lipid vacuoles. This case confirms the occurrence of adrenal cortical neoplasms in the CNS and is the first report of an intradural, intramedullary adrenal cortical adenoma of the spinal cord, and the first to occur in a male. This tumor should be considered in the differential diagnosis of tumors of the CNS.
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PMID:Spinal adrenal cortical adenoma with oncocytic features: report of the first intramedullary case and review of the literature. 1530 40

Hemangiopericytoma (HPC) is a rare tumor of the central nervous system, most commonly found in the cranial cavity. HPCs in the spine are rare, and very few of them are primary osseous HPC. The aims of this study were to describe a rare case of primary osseous HPC in the thoracic spine and review the literature. A 54-year-old man presented with a 3-month history of back pain. Aneuro logical examination revealed no motor or sensory deficits. Magnetic resonance imaging (MRI) and computed tomography (CT) scan showed a tumor originating from the bone structure of the T10 vertebra with paravertebral extension, and chest CT revealed pulmonary metastases. A laminectomy, face-totomy,and subtotal resection of the tumor was performed with posterior pedicle screw system fixation followed by radiotherapy. The post-operative course was uneventful. His back pain was resolved completely after surgery. The patient survived with tumor during the 18-month follow-up period. Histopathology and immunohistologic findings were consistent with HPC. On immunohistochemistry, the tumor was positive for vimentin and CD34, partially positive for S-100, but negative for EMA, desmin, CD117, and CD1a. A literature review identified eight such cases reported between 1942 and 2013. As a conclusion, clinical manifestations of primary osseous spinal HPCs are different from intraspinal meningeal HPCs. Although showing certain variability, histopathology and immunohistochemical examinations are essential to establish the diagnosis. Surgical resection and radiotherapy are the treatment of choice. *These authors contributed equally to this work.
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PMID:Primary osseous hemangiopericytoma in the thoracic spine. 2488