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

Twelve cases are described of a distinctive benign soft tissue lesion that may be mistaken for a sarcoma. The tumors occurred in 11 men and a woman aged 33 to 81 years (mean, 64 years), and measured from 2 to 11 cm in greatest diameter (mean, 6 cm). They were grossly described as soft, well-circumscribed, yellow-gray, with a mucoid cut surface. All cases were superficially located in the subcutis or muscular fascia of the head and neck region or the chest and back. Histologically, the tumors were characterized by a proliferation of spindle or stellate fibroblastic cells variably admixed with mature adipose tissue embedded in an abundant myxoid and collagenized stroma. The spindle and stellate fibroblastic cells were characterized by slender dendritic prolongations of their cytoplasm, which appeared to extend for short distances along connective tissue planes. Electron microscopy in two cases confirmed the dendritic nature of the fibroblastic cells, which showed elongated cytoplasmic processes lacking external lamina and displaying foci of pinocytotic activity. Immunohistochemical studies in 11 cases showed strong positivity of the spindle cells with vimentin, CD34 and bcl-2, and negative staining for smooth muscle actin, muscle-specific actin (HHF35), desmin, S-100 protein, keratin, and EMA. Because of their prominent myxoid stroma and relatively large size, some of these tumors were initially misinterpreted as low-grade sarcomas. Clinical follow-up in five cases, however, showed that the patients were alive and well without evidence of recurrence between 5 and 13 years (mean follow-up, 8 years) after simple local excision. The present cases appear to represent a distinctive form of benign soft tissue neoplasm that should be distinguished from myxoid liposarcoma and other benign and malignant myxoid tumors of superficial soft tissues.
 ...
PMID:Dendritic fibromyxolipoma: clinicopathologic study of a distinctive benign soft tissue lesion that may be mistaken for a sarcoma. 984 27

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.
 ...
PMID:[Synovial sarcoma of the pericardium]. 988 9

Authors present an additional case report of pleomorphic hyalinizing angiectatic tumor (PHAT), only recently recognized entity amongst the soft tissue tumors. 63-year-old man underwent surgery for subcutaneous tumor growing on the forearm. Grossly, ovoid well demarcated tumor 4.0 x 3.5 x 2.5 cm was removed. Histologically, hypovascular areas of spindle-cells arranged in sheets and fascicles resembling spindle-cell sarcoma and areas rich on ectatic vessels with fibrin deposits or thickened hyalinized walls were present. Some cells contained intranuclear cytoplasmatic inclusions, lobulated nuclei with or without prominent nucleoli or multiple nuclei. Mitoses were less than 1 per 30 HPF, Ki-67 nuclear positivity was observed in 2.6% of tumor cells. Immunohistochemically, cells were positive with vimentin, but negative for CD34, S100, desmin, smooth-muscle actin, EMA, VWF, CD99, bcl-2 and cytokeratins. The patient did well, without recurrent tumor after 13 months follow-up. Spectrum of tumors including solitary fibrous tumor, giant-cell angiofibroma, neurilemmoma, malignant fibrous histiocytoma and acral myxoinflammatory tumor with atypical bizarre giant cells is discussed in differential diagnosis.
 ...
PMID:Pleomorphic hyalinizing angiectatic tumor. 1181 37

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.
 ...
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

The etiology and pathogenesis of Hodgkin lymphoma (HL) are not yet known. There are implications of genes involved in programmed cell death (apoptosis), and there have been repeated suggestions of an association with Epstein-Barr virus (EBV). The aim of this study was to investigate the protein expression patterns of key cell cycle-related genes, together with evidence of apoptosis and EBV status, in relation to clinical stage in HLs. A double immunohistochemical and in situ hybridization technique was used to detect the expression of bcl-2, p53, retinoblastoma (Rb), p21, Ki67 (MIB 1), and topoisomerase IIalpha (TopoIIalpha), together with latent membrane protein-1 and EBER for EBV status and TdT-mediated dUTP-FITC nick end-labeling (TUNEL) as a measure of apoptosis, on tissue microarray sections of 62 cases of classic HL (35 NS, 17 MC, 8 LR, and 2 LD). A panel of phenotypic markers was used to facilitate recognition of Hodgkin and Reed-Sternberg (H-RS) cells: CD3, CD20, CD30, CD15, and EMA. The H-RS cells of 62 classic Hodgkin lymphomas were bcl-2-positive in 35 cases (56.45%), p53-positive in 14 (22.58%), and positive for both EBV latent membrane protein-1 and EBER in 37 (59.68%); there was complete concordance of results for EBV by both procedures. No correlation was found between expression of bcl-2, p53, or EBV markers in H-RS cells and clinical stage (P > 0.05). Expression of Rb, Ki67, p21, and TopoIIalpha did, however, show significant differences with clinical stage. Expression of Rb and p21 in CD30-positive H-RS cells decreased with more advanced stage (P < 0.001). In contrast, Ki67 and ToPoIIalpha expression increased with later stage (P < 0.01). No correlation was found between expression of any of these markers in H-RS cells and the subtypes of nodular sclerosis HL, mixed cellularity HL, and LRHL (P > 0.05). TUNEL was found in the nonneoplastic cellular background in all cases and in H-RS cells in only 10 of 62 cases (16.12%) (8 nodular sclerosis HL, 1 mixed cellularity HL, and 1 LRHL). There was a significant correlation between high expression of bcl-2 and a low score by TUNEL (P < 0.05). These data are consistent with the notion that overexpression of bcl-2 may be linked to blockage of apoptosis-mediated death of H-RS cells in classic HL. Abnormal expression of p53-related protein may not play a major role in HL, because it is present in H-RS cells in only a minority of cases. Increased expression of Ki67 and TopoIIalpha by H-RS cells is significantly associated with advanced stage and may indicate aggressive disease. Adverse clinical outcome in HL also is associated with loss of Rb and p21 protein expression, consistent with the possible roles of Rb and p21 in inhibition of the growth of H-RS cells. Within the limitations of the methods used, almost two thirds of cases of HL provide evidence of an association with EBV. The tissue microarray technique is valuable not only for examination of large numbers of cases of a disease by a complex panel of markers but also potentially as a control for staining quality in immunohistochemistry and in situ hybridization.
 ...
PMID:Apoptosis and cell cycle-related genes and proteins in classical Hodgkin lymphoma: application of tissue microarray technique. 1296 46

We present a case of a classical Hodgkin lymphoma occurring in clusters of marginal zone B-lymphocytes (MZBLs). Most lymphoid follicles possessed hyperplastic germinal centers, while a portion of the follicles exhibited a progressive transformation of the germinal center (PTGC). Clusters of MZBLs showed a perifollicular distribution. The classic Reed-Sternberg cells were found in clusters of MZBLs. A portion of the Reed-Sternberg cells were CD15+, CD20+, CD30+, CD79a+, fascin+, vimentin+, EMA-, and bcl-2-. Some Reed-Sternberg cells were surrounded by CD3+ CD45RO+ CD57-rosettes. In situ hybridization studies demonstrated strong expression of EBER in classic Reed-Sternberg cells and their variants. The overall morphological, immunohistological, and EBV findings confirmed that the present case is a classical Hodgkin lymphoma. The MZBLs were CD20+, CD79a+, sIgM+/-, sIgD-, CD5-, CD21-, CD43-, CD45RO-, and Bcl-2-. Some MZBLs had polytypic intracytoplasmic immunoglobulin. Problems arising in the differential diagnosis between lymphocyte-predominant Hodgkin lymphoma and PTGC have been described. An occasional association between MZBLs clusters and PTGC has been reported previously. This case suggests that classical Hodgkin lymphoma should be added to the differential diagnosis of PTGC.
 ...
PMID:Classical Hodgkin lymphoma occurring in clusters of nodal marginal zone B-lymphocytes in association with progressive transformation of germinal center. A case report. 1453 39

The correlation between the histological features and clinical outcome remains poor in pediatric intracranial ependymomas. We performed a retrospective study of a group of 31 patients (diagnosed from 1985 to 1995) to assess prognostic implications of the current grading system, of histological and immunohistochemical features, and of ploidy status estimated by flow cytometry. Immunoexpression of a broad spectrum of antigens was evaluated, including MIB-1, topoisomerase-IIalpha, cyclin D1, glial and epithelial proteins (GFAP, EMA, cytokeratins), molecules involved in controlling apoptosis (bcl-2, caspase-3/CPP32), and p53 oncoprotein. Univariate and multivariate statistical analyses were performed to evaluate the influence of each variable on both the progression free survival (PFS) and the overall survival (OS) with at least 7-year follow up. Although we showed a significant correlation between histological grade and prognosis, the current grading system failed in predicting outcome in nearly one third of individual cases. Problems with interpathologist reproducibility were also demonstrated. The extent of surgical resection was the only clinical factor that was associated with survival. Both the PFS and the OS were significantly decreased for the following pathological variables: increased cellularity (>300 nuclei per HPF), mitotic activity of >7 per 10 HPF, increased MIB-1 labeling index (LI), topoisomerase-IIalpha LI, S-phase fraction, and p53 and bcl-2 positivity. Increased cyclin D1 LI was demonstrated to have only a marginally significant impact on PFS. A flow chart modeling was further performed to formulate a scheme for discriminating of prognostic subgroups. Based on that, p53 immunopositivity and/or MIB-1 LI of >5% (after subtotal resection) or MIB-1 LI of >15% (after complete resection) were the strongest indicators of the tumor's aggressive behavior and of a poor prognosis of the disease. Foci of hypercellularity should be specifically looked for in ependymomas for assessing the immunohistochemical studies.
 ...
PMID:Pediatric intracranial ependymomas: prognostic relevance of histological, immunohistochemical, and flow cytometric factors. 1455 80

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.
 ...
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.
 ...
PMID:Primary poorly differentiated monophasic synovial sarcoma of the lung. A case report with immunohistochemical and genetic studies. 1864 80

Plasmablastic lymphoma (PBL) is a rare and relatively new entity originally described in HIV-infected individuals. This subset of Epstein-Barr-virus (EBV)-related non-Hodgkin lymphomas is now regarded as a distinct clinicopathological category of AIDS-associated lymphomas occurring preferentially in the oral cavity and showing a poor prognosis. We describe for the first time an EBV-associated PBL with an isolated cutaneous distribution on the lower extremities in an HIV-infected heterosexual male and point to the unique clinical, morphological and immunophenotypic characteristics of this lymphoma. The patient presented with fast growing solid and livid nodules on both legs. The large, blastic tumor cells showed the following immunophenotype: CD138+, CD45+, CD20-, CD10-, CD3-, CD30-, bcl-2-, bcl-6-, LMP-1- and EMA-. The proliferation fraction (Mib-1) was >90%. EBV association was demonstrated by in situ hybridization (EBV-encoded RNAs 1/2). Polymerase-chain-reaction-based DNA analysis demonstrated a clonal IgH rearrangement in the absence of a bcl-2/IgH translocation. PBL in HIV patients may occur not only in the oral cavity, but can probably involve any other organs including the skin.
 ...
PMID:Cutaneous plasmablastic lymphoma in an HIV-positive male: an unrecognized cutaneous manifestation. 1511 93


1 2 3 4 Next >>