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)

Thirty-six cases of synovial sarcoma (13 biphasic and 23 monophasic) were subjected to a clinicopathologic study that included electron microscopy and immunohistochemistry. The group consisted of 21 males and 15 females ranging in age from 2 to 63 years. The majority of tumors (27 cases) were found in the hip and lower extremity. Immunohistochemical study revealed that keratin, which was detected in 92% of the biphasic and 57% of the monophasic tumors, was a more sensitive marker of epithelial differentiation than EMA or CEA. The overall 5-year survival of the patients was 64%. Male sex, older age, presence of tumor necrosis, monophasic pattern, and absence of keratin positivity had an unfavourable effect on survival but lacked statistical significance. Survival was significantly lower in patients with tumors exhibiting more than 15 mitoses per 10 HPF (P less than .02) and in those with tumors showing necrosis and a mitotic rate greater than 5 mitoses per 10 HPF (P less than .005).
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PMID:Synovial sarcoma: a clinicopathological study of 36 cases. 169 34

The relationship between biphasic (BSS) and monophasic (MSS) subtypes of synovial sarcoma (SS) as well as the relationship between cells of solid/glandular areas and the spindle cells of BSS remain controversial. In order to further evaluate the immunohistochemical phenotype of SS we studied 34 primary tumours (15 BSS; 19 MSS), 7 recurrences (4 from primary BSS; 3 from primary MSS) and 8 metastases (7 BSS; one MSS), using several antibodies (EMA, CEA, keratins 1, 4, 5/6, 7, 8, 13, 18, 19, 20, vimentin, collagen IV and laminin) that work in paraffin-embedded material. Spindle cells outside solid/glandular areas of BSS and in MSS showed immunoreactivity for keratins 5/6, 7, 8, 18 and 19. The transition of solid/glandular areas to surrounding spindle cells also showed keratin staining and failed to show a distinct separation regarding the immunoreactivity for laminin and collagen IV. Peripheral cells of solid/glandular areas were immunoreactive for vimentin. No major differences were observed between immunophenotypical cell profiles of BSS and MSS, apart from the exclusive immunostaining of solid/glandular areas of BSS for keratin 13 and CEA. Downgrading of keratin and extracellular matrix antigens immunoreactivity was observed when primary tumours were compared to recurrent and/or metastatic tumours of both subtypes (MSS and BSS). We conclude that SS should be regarded as carcinomas of soft tissues with an immunohistochemical phenotype depending on the degree of epithelial differentiation: spindle cells (MSS and BSS) predominantly expressing simple keratins, and poorly differentiated (solid/glandular) as well as well-differentiated (glandular) areas (BSS) expressing, in addition, complex epithelial-type keratins.
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PMID:Immunohistochemical profile of synovial sarcoma with emphasis on the epithelial-type differentiation. A study of 49 primary tumours, recurrences and metastases. 752 Jan 65

We examined 4 cases of synovial sarcoma, 3 biphasic-one of which was located in the abdominal wall-and 1 monophasic, in patients between 16 and 54 years. Immunohistochemistry revealed the capacity of these neoplasms to express mesenchymal and epithelial markers, not only in the sarcomatous-like stroma but also in gland-like component. We also found that in the epitheliomorphous cells vimentin positivity had a characteristic basal position whereas EMA and cytocheratins were much more positive in the apical zone. This double positivity could be profitably utilized when the predominance of the gland-like component in a synovial sarcoma requires differential diagnosis from metastatic adenocarcinoma.
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PMID:[Synovial sarcoma. Immunohistochemical study of 4 cases]. 807 7

A case of malignant nerve sheath tumor with glandular differentiation in a patient with neurofibromatosis is presented. In the spindle celled areas the tumor cells reacted strongly with vimentin and S-100 protein, while the glandular epithelia reacted with EMA, cytokeratins (peptides 8, 19, and 20) and CEA, as well as a few of them also with chromogranin. Based on the results of immunohistochemical profile of the tumor cells, the differential diagnosis of this rare soft tissue tumor from biphasic synovial sarcoma with glands, as well as its distinction from any other spindle cell sarcoma with entrapped skin appendages could be greatly facilitated.
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PMID:Malignant peripheral nerve sheath tumor with glandular differentiation. Report of a case with emphasis to the usefulness of immunohistochemistry in the differential diagnosis. 933 15

A precordial tumor of the pericardium was radiologically diagnosed as the cause of an untypical clinical picture of heart disease in a 41-year-old soldier. As the patient had an increased asbestos exposure due to his profession, he was admitted to operation under the tentative diagnosis of a pericardial mesothelioma and the question of an occupational disease (BK 4105). Microscopic and immunohistochemical findings are compatible with the diagnosis of a synovial sarcoma of the pericardium. The present immunohistochemical marker spectrum allowed a reliable differentiation between synovial sarcoma and pericardial mesothelioma, which is more frequent than synovial sarcoma. The epithelioid component was determined using the following antibodies: MNF 116, CK 19, CK 7, EMA and Ber EP-4 were positive while Factor VIII, Calretinin, S100, Vimentin, CEA, CD 31, bcl-2 and HBA-71 were negative. The sarcomatous component was determined with antibodies to Vimentin, bcl-2 and HBA-71 which were positive, and to MNF 116, CK 19, CK 7, Factor VIII, Calretinin, S100, EMA, CEA, Ber EP-4 and CD 31 which were negative. Synovial sarcomas of the pericardium in the lower anterior mediastinum or the myocardium are exceedingly rare. A causal relationship between tumor formation and an increased asbestos exposure--similar to the epidemiologically based experiences with pericardial mesothelioma--is not likely. Primary extrapericardial synovial sarcoma could be excluded.
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PMID:[Synovial sarcoma of the pericardium]. 988 9

We report 15 primary renal neoplasms with morphologic, immunohistochemical, and molecular features identical to those of synovial sarcoma. These tumors form a distinct subset of the entity previously designated as embryonal sarcoma of the kidney. Most were diagnosed between the ages of 20 and 50 years. On gross examination, tumors are large, partially necrotic, and usually contain smooth-walled cysts. Microscopically, tumors are characterized by mitotically active, monomorphic plump spindle cells with indistinct cell borders growing in short, intersecting fascicles. Grossly identified cysts are lined by mitotically inactive polygonal eosinophilic cells with apically oriented nuclei ("hobnailed epithelium"). The spindle cells are immunoreactive for vimentin, often immunoreactive for EMA, but typically non-immunoreactive for desmin, actin, S100, or cytokeratins, whereas the cyst epithelium is cytokeratin-positive. These findings are consistent with monophasic, spindled synovial sarcoma encircling dilated native renal collecting ducts. The presence of an SYT-SSX gene fusion resulting from the t(X;18) characteristic of synovial sarcoma was demonstrated by reverse transcriptase polymerase chain reaction in three of three tumors in which adequate RNA could be obtained from paraffin blocks. An additional case demonstrated the characteristic t(X; 18) translocation on cytogenetic analysis, but adequate material to perform molecular studies was not available in this case or the remaining 11 cases. Primary renal synovial sarcoma is a distinctive clinicopathologic entity confirmed by molecular detection of SYT-SSX fusion transcripts.
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PMID:Primary renal synovial sarcoma: molecular and morphologic delineation of an entity previously included among embryonal sarcomas of the kidney. 1093 49

A rare case of a monophasic pulmonary synovial sarcoma is reported. A 44-year-old Japanese man underwent lower lobectomy for a nodular mass in his right lung. Immunohistochemical study of the excised primitive spindle cell sarcoma revealed occasional positive stains by hitherto reported antigens of S-100, cytokeratin 7, high molecular weight cytokeratin (34 beta E12), pankeratin (AE1/AE3), and EMA, which were helpful for the differential diagnosis of other spindle cell sarcomas. Furthermore, positive immunostains for MEF2, VS38c (plasma cell antigen), and bcl-2 were rather significant findings in the present case. The definitive evidence that molecular genetic analysis showed a clonal single electrophoretic band of SYT-SSX mutated chimera gene was conclusive for the pathological diagnosis. The implications of the frequently seen ultrastructure of oligocilia and concentric membranous bodies with positive stains for VS38c and MEF2 are discussed. In the difficult pathological diagnosis of a rare and undifferentiated type of sarcoma with unusual clinicopathological features generated at an unusual site, comprehensive ultrastructural, immunohistochemical, and cytogenetic studies will lead to the correct pathological diagnosis and elucidate the detailed characteristics of the tumor.
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PMID:An ultrastructural, immunohistochemical, and cytogenetical study of a monophasic pulmonary synovial sarcoma: implications of the frequent ultrastructure of oligocilia and concentric membranous bodies with positive immunostaining for VS38c and MEF2. 1290 68

We report 2 primary renal synovial sarcoma. These tumors were formerly designated as embryonal cystic sarcoma of the kidney. Most cases are diagnosed between the ages of 20 and 50 years. Some cases show local recurrence after nephrectomy. On gross examination, tumors are large, partially necrotic, and usually contain cysts. Microscopically, tumors are characterized by monomorphic plump spindle cells. The cysts are lined by mitotically inactive epithelial cells without striking cellular atypia. The spindle cells were immunoreactive for EMA, CD56, and sometimes for CD99. They were non-reactive for desmin, actin, S 100, and cytokeratins. The cyst epithelium is cytokeratin positive. The presence of a SYT-SSX gene fusion resulting from the t(X;18) characteristic for synovial sarcoma was demonstrated by reverse transcriptase polymerase chain reaction in both tumors. Primary renal synovial sarcoma is a distinctive tumor entity, which should be considered in renal tumors consisting of spindle cells.
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PMID:[Primary renal synovial sarcoma. A new entity in the morphological spectrum of spindle cell renal tumors]. 1460 53

We describe here the establishment of a new synovial sarcoma cell line, SYO-1, derived from a biphasic synovial sarcoma that developed in the groin of a 19-year-old female. The cell line was maintained for more than 70 passages (more than 24 months) in vitro. The SYO-1 cells in monolayer culture exhibited a spindle shape without conspicuous pleomorphism. Immunohistochemically, the cells were positive for vimentin, type IV collagen, S-100, mdm2, bcl-2, c-Met and c-Kit. Tumors developed by their implantation in nude mice histologically showed biphasic features that were composed of areas of fascicles of spindle cells and areas of compact proliferation of polygonal to ovoid cells, which occasionally formed epithelial plaque and expressed cytokeratin and EMA. SYO-1 cells harbored the characteristic t(X;18)(p11.2;q11.2) translocation by chromosome analysis and SYT-SSX2 chimeric transcript by RT-PCR. The SYO-1 cells, the first characterized cell line derived from biphasic synovial sarcoma retaining the characteristic genetic and phenotypic features of the tumor, will be useful for various investigations on synovial sarcoma, especially for its epithelial differentiation.
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PMID:Establishment and characterization of a biphasic synovial sarcoma cell line, SYO-1. 1474 40

We describe a case of a poorly differentiated monophasic synovial sarcoma arising in the lung of a 50-year-old man. The tumor, which was located in the right upper lobe, was lobulated, relatively well-circumscribed, and whitish to yellowish in color. Microscopically, it was composed exclusively of ovoid to polygonal or short spindle cells, with a high nuclear to cytoplasmic ratio and relatively scant cytoplasm, arranged in solid sheets or in a hemangiopericytomatous pattern with intervening wiry collagen fibers. At the periphery of the tumor, entrapped benign alveolar epithelium produced a pseudo-biphasic appearance. In some areas, an abundance of keloidal collagen imparted a close resemblance to a solitary fibrous tumor, making it difficult to establish the diagnosis on the initial needle biopsy, although the malignant nature of the tumor was suggested because of nuclear anaplasia. Immunohistochemically, the tumor was positive for cytokeratin AE1/AE3, CAM5.2, EMA, vimentin, bcl-2 protein, calretinin, and CD34. The reverse transcriptase-polymerase chain reaction (RT-PCR), using RNA extracted from fresh-frozen tissue, demonstrated SYT/SSX-1 fusion transcripts, confirming the diagnosis of synovial sarcoma. Microscopic examination demonstrated metastatic deposits in hilar lymph nodes. This case indicates that a primary pulmonary synovial sarcoma, particularly in its poorly differentiated form, is a diagnostically challenging and highly aggressive neoplasm typically found at an advanced stage.
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PMID:Primary poorly differentiated monophasic synovial sarcoma of the lung. A case report with immunohistochemical and genetic studies. 1864 80


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