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)

A case of extraskeletal Ewing's sarcoma arising in the chest wall of a 15-year-old male is reported in this paper. His complaint was chest and back pain. Physical examination and laboratory data disclosed no abnormality. Radiographs and computed tomography revealed chest wall tumor, and resection of the tumor with a portion of the eighth rib was performed. Histologically, there was no involvement of the rib at all. The tumor tissue was composed of solidly packed tumor cells which were uniform in size and shape. Individual tumor cells were small, round-shaped, and had scanty, ill-defined cytoplasm. The diastase-digested, PAS positive material was found in the cytoplasm. NSE (neuron specific enolase) was positive, but EMA (epithelial membrane antigen) LCA (leucocyte common antigen), and keratin were negative. With these results, we got a diagnosis of extraskeletal Ewing's sarcoma. Combination chemotherapy with vincristine, cyclophosphamide, adriamycin, actinomycin D was administered after operation, and there is no evidence of recurrence after 12 months of follow-up.
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PMID:[A case of extraskeletal Ewing's sarcoma of the chest wall]. 231 13

Follicular, papillary, anaplastic and medullary cancers of the thyroid were investigated using immunohistochemical methods. The following antibodies were used: anti-S-100, antineuron-specific enolase (NSE), antikeratin, antithyroglobulin, anticalcitonin, anticarcinoembryonic antigen (CEA), antiepithelial membrane antigen (EMA); the following hormones were also tested in the medullary carcinoma: gastrin, ACTH and serotonin. Papillary and follicular carcinoma in particular reacted with anti-S-100 and anti-NSE; the anaplastic neoplasia reacted with anti-S-100 (25%), anti-NSE (12%), antikeratin (12%), antithyroglobulin (12%), anti-CEA (37%) and anti-EMA (37%). Medullary carcinoma reacted with anticalcitonin (100%), anti-CEA (96%), anti-NSE (79%), anti-EMA (4%) and anti-S-100 (17%). We were not able to correlate the virulence of the medullary carcinoma with the anticalcitonin and anti-CEA reactivity, while the hyperplastic C cells were immunoreactive both with calcitonin or with CEA.
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PMID:An immunohistochemical study in thyroid cancer. 244 41

We report a case of cellular neurothekeoma showing unusual immunohistochemical findings and occurring on the left upper arm of a healthy 48-year-old woman. She presented with a 1 cm, red, asymptomatic, dermal nodule of 1 year duration. A biopsy showed dermal proliferation of plexiform fascicles of spindle-shaped or polygonal cells with plentiful eosinophilic cytoplasms. The immunohistochemical profile included negative stains for S-100, CD34, factor XIIIa, CD68, HMB45, cytokeratins, and EMA, strongly positive stains for neuron specific enolase (NSE), synaptophysin, and chromogranin A, and focally positive ones for NKI/C3 and alpha-smooth muscle actin. Ultrastructural analysis showed undifferentiated mesenchymal cells with cytoplasmic projections and abundant RER. Although we couldn't find any confirmative cell type in this cellular tumor, we believe that cellular neurothekeoma is predominantly composed of undifferentiated cells that can exhibit features of neuroendocrine cells in addition to fibroblastic or myofibroblastic ones, suggesting a divergent cell origin.
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PMID:Cellular neurothekeoma with possible neuroendocrine differentiation. 1040 81

Desmoplastic small round cell tumor (DSRCT) is a unique, highly aggressive neoplasm that chiefly affects male adolescents and young adults. This tumor is characterized by nests of small undifferentiated cells that show immunohistochemical evidence of epithelial, mesenchymal, and neural differentiation. We report two cases of DSRCT that lacked immunohistochemical evidence of epithelial differentiation, but were found to have the fusion transcripts characteristic of this tumor. Both patients (a 41-year-old male and a 31-year-old female) presented with large intra-abdominal masses. After diagnostic biopsy, both were treated with multi-agent chemotherapy. One patient expired 18 days after diagnosis, and the other is currently alive 28 months later. Histologically, both tumors had the characteristic features of DSRCT and were composed of small round cells with hyperchromatic nuclei and scanty cytoplasm. In one of the cases, perinuclear intracytoplasmic hyaline inclusions were seen. Immunohistochemically, neither case expressed any of the epithelial markers tested, including AE1/AE3, CAM 5.2 and EMA. Both tumors were diffusely immunoreactive for desmin with a prominent globoid "dot-like" pattern of staining in one case. Both tumors stained for vimentin, neuron specific enolase, and synaptophysin, but were negative for CD99, muscle-specific actin, and myogenin. Reverse transcriptase-polymerase chain reaction revealed EWS-WT1 fusion transcripts characteristic of this neoplasm. In conclusion, we describe two cases of DSRCT that lacked immunohistochemical evidence of epithelial differentiation but had histologic and other immunohistochemical features which suggested this diagnosis. The ability to confirm the diagnosis of this rare tumor using molecular genetic techniques is particularly useful in those cases with unusual histologic or immunophenotypic features.
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PMID:Cytokeratin-negative desmoplastic small round cell tumor: a report of two cases emphasizing the utility of reverse transcriptase-polymerase chain reaction. 1049 92

Rare cases of nonhepatocytic mixed stromal and epithelial tumors of the liver with associated calcification and ossification have been described previously. We report 6 similar cases in children, including 2 cases associated with ectopic ACTH production. The patients were between 2 and 14 years of age at diagnosis. All tumors presented as a solitary liver mass with no extrahepatic involvement. Two adolescent females with palpable abdominal tumors presented with Cushing syndrome that abated after excision of the tumors. The other children had tumors identified incidentally on imaging studies or at laparotomy. All tumors were well circumscribed, ranging in size from 4.0 to 30.0 cm in greatest diameter. Histologically, they shared an organoid arrangement of cellular nests that were comprised of an admixture of both spindled and epithelioid cells. These cellular nests were surrounded by a band of delicate myofibroblasts and set in a dense fibrous stroma that contained slit-like to dilated blood vessels. A variable proliferation of bile ducts extended from the fibrous stroma and focally surrounded the cellular nests. One case showed a sheet-like overgrowth of the nested cells with associated necrosis. The cellular nest cells were immunoreactive for EMA, CD56, neuron specific enolase, pan-cytokeratin (4 of 6 cases), vimentin (5 of 6 cases), and WT-1 amino terminus (4 of 6 cases). Cytokeratin and EMA stained mostly epithelioid nest cells, with vimentin and WT-1 staining predominantly the spindled nest cells. The 3 cases from adolescent females showed immunoreactivity for ACTH in the nested cell population but not in the surrounding stromal cells. Immunohistochemical stains for synaptophysin and chromogranin were negative in all cases. Psammomatous calcifications were present focally in 2 cases and were extensive in 3 cases. Ossification or osteoid formation was present in 4 cases. The 1 patient whose tumor had sheet-like overgrowth of the nested cell population had a local recurrence with multiple hepatic nodules 1 year following the original resection. A 2-year-old patient has been subsequently diagnosed with nephroblastomatosis and Wilms tumor of the kidney. Follow-up information was available in an additional 3 patients with no tumor recurrence or metastatic disease at 2, 3, and 14 years.
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PMID:Nested stromal epithelial tumor of the liver: six cases of a distinctive pediatric neoplasm with frequent calcifications and association with cushing syndrome. 1561 52

Extra-adrenal paragangliomas constitute 10 % or less of phaeochromocytomas/paragangliomas. Even rarer is the occurrence of paragangliomas outside the usual distribution of paraganglionic tissue. We report a case of extra-adrenal paraganglioma occurring in the small intestine mesentery in a 65-year-old man. To our knowledge, there are only seven case reports of paraganglioma occurring in this non-typical site. Computed tomography showed a solid expansive non-homogenously enhancing mesenteric mass, measuring 10 x 8 cm with peripheral cystic component. Histologically, the tumour had a typical organoid "zellballen" pattern, showed immunohistochemical positivity for synaptophysin, neuron specific enolase, CD-56, chromogranin, and focally vimentin, and was cytokeratin and EMA negative. S-100 protein stained few sustentacular cells. The patient was free from recurrence or metastasis three months after tumour resection. Although rare, paraganglioma should be included in the preoperative differential diagnosis of solid mesenteric tumours, to prevent any potential life-threatening event peroperatively in the case of a catecholamines-producing tumour.
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PMID:Paraganglioma of the mesenterium: a case report. 1818 23

Extrarenal rhabdoid tumor (ERRT) is a rare, aggressive tumor with extremely poor prognosis. We report a case of ERRT with intraspinal extension in a 1.5-year-old child diagnosed by fine needle aspiration cytology (FNAC) and immunohistochemistry. The child presented with a right lumbar region lump of two months duration. Ultrasound guided FNAC was performed and cell block was prepared. Smears were highly cellular and showed a dispersed population of large round cells having abundant pale eosinophilic cytoplasm, centrally to eccentrically placed nucleus with large prominent nucleoli. Immunohistochemistry was carried out on cell block which was positive for epithelial membrane antigen EMA and Vimentin. It was negative for leucocyte common antigen [LCA], wilms tumor 1, WT1, desmin and neuron specific enolase NSE, thus ruling out other tumors like lymphoma, Wilms tumor, rhabdomyosarcoma, and neuroblastoma. A final diagnosis of ERRT was given. ERRT is an extremely rare tumor of retroperitoneal area; it should be included in the differential diagnosis of malignant round cell tumor in children. Cell block in this case is mandatory for putting up the panel of immunohistochemistry which can clinch the diagnosis of rhabdoid tumor and treatment can be started as early as possible.
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PMID:Cytomorphology and immunohistochemistry of extrarenal rhabdoid tumor: a case report with review of literature. 2223 21