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)

Epithelioid sarcoma (ES) is a distinctive soft tissue neoplasm with a predilection for the distal extremities of young adults. This tumor typically contains nodular aggregates of epithelioid and spindle cells with zonal necrosis. The neoplastic cells are generally reported to coexpress keratin and vimentin and are often stated to be positive for CD34. However, there is no large series with extensive immunohistochemical data, there are few data with regard to expression of different keratin subtypes, and there are no large series discussing the epithelioid sarcoma subtypes. In the current study, we immunohistochemically evaluated 88 typical and 24 variant (8 angiomatoid, 9 large cell/rhabdoid, and 7 "fibroma-like") ESs. Nearly all ESs with typical histology (94%) were positive for keratin 8 (K8), whereas 72% were positive for K19, 48% for intermediate- and high-molecular-weight keratins (34betaEH12), and 22% for K7; reactivity with the latter two antibodies was usually seen in only a minority of tumor cells. Vimentin reactivity was present in all cases, EMA in 96% of cases and muscle-specific actin and CD34 were noted in 41% and 52% of the cases, respectively. A few ESs (7%) showed focal cytoplasmic CD31 reactivity, but none exhibited a distinctive membrane staining pattern, and examples tested for FVIIIRAg were negative. The angiomatoid, fibroma-like, and large cell-rhabdoid ES variants had immunohistochemical profiles similar to the classic cases, supporting a common pathogenesis. Although not consistently expressed in ES, the presence of CD34 is helpful in distinguishing this entity from primary and metastatic carcinomas and other sarcomas such as malignant rhabdoid tumor.
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PMID:Epithelioid sarcoma: an immunohistochemical analysis of 112 classical and variant cases and a discussion of the differential diagnosis. 1082 2

Thyroid anaplastic (undifferentiated) carcinomas (TACs) comprise a morphologically heterogeneous group of tumors, which can arise in the background of differentiated papillary or follicular carcinoma. The thyroid epithelial differentiation varies in these tumors and has not been completely characterized. In this study, we immunohistochemically analyzed different variants TACs from 35 patients by using antibodies specific to 9 different keratin polypeptides, epithelial membrane antigen, thyroid transcription factor I (TTF-1), and thyroglobulin. These tumors were histologically divided into 3 categories: squamoid-cohesive (SC, 13 tumors), spindle cell sarcomatous (SS, 8 cases) and intermediate group, including tumors with giant cells and solid epithelioid components (GC, 18 tumors); 4 tumors had 2 components. The patients ages ranged from 40 to 89 years, with a mean age in all groups of 70 years. TTF-1 was present in only 2 of 9 of the SC tumors, and absent in all other TACs, but was present in entrapped differentiated components. Thyroglobulin was absent in all but 1 case. A complex keratin (K) pattern of stratified epithelia was typically seen in the SC tumors with extensive K7, K8, K17, K18, and K19, and variable K13 and K14 expression; EMA was also present. K16 was limited to squamous pearls in 1 tumor, and K10 was absent. The GC carcinomas typically had K8 and K18, whereas the expression of K7 was variable and that of K14, K17, and K19 sporadic; EMA was variably present in half of the cases. The keratins in spindle cell sarcomatous tumors were usually limited to K7, K8, and K18, often in limited numbers of cells. EMA was present in 1 case only. These results indicate a complex pattern of keratins in squamoid and giant cell TACs, similar to papillary carcinoma and suggesting the possibility of relationship. There was a progressive loss of epithelial differentiation and keratins in sarcomatoid TACs. Loss of TTF-1 is a nearly uniform feature of TAC and disallows the use of this marker to pinpoint a thyroid origin of these tumors.
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PMID:Variable expression of keratins and nearly uniform lack of thyroid transcription factor 1 in thyroid anaplastic carcinoma. 1101 83