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)

Ovarian small cell carcinoma of hypercalcemic type (OSCCHT) is a rare neoplasm with an aggressive behavior, broad differential diagnosis, and unknown histogenesis. To add to knowledge concerning the possible aid of immunohistochemistry in resolving problems in differential diagnosis and to further explore whether that modality points to any specific histogenesis, we undertook an immunohistochemical study of this neoplasm. Fifteen OSCCHTs (including four of the ''large cell" variant) were stained with a range of antibodies, some of which have not been investigated previously in this neoplasm. Cases were stained with AE1/3, EMA, BerEP4, CK5/6, calretinin, WT1, chromogranin, CD56, synaptophysin, CD99, NB84, desmin, S100, CD10, alpha inhibin, TTFI, and p53. Staining was classified as 0 (negative), 1+ (<5% cells positive), 2+ (5% to 25% cells positive), 3+ (26% to 50% cells positive), or 4+ (>50% cells positive). All cases were positive with p53 (two 1+, five 3+, eight 4+), 14 of 15 cases were positive with WT1 (one 1+, thirteen 4+), 14 of 15 with CD10 (three 1+, four 2+, two 3+, five 4+), 13 of 15 with EMA (three 1+, three 2+, two 3+, five 4+), 11 of 15 with calretinin (nine 1+, one 3+, one 4+), 9 of 15 with AE1/3 (eight 1+, one 2+), 4 of 15 with CD56 (one 1+, two 2+, one 4+), 3 of 15 with BerEP4 (two 2+, one 4+), 2 of 15 with synaptophysin (two 1+), and 1 of 15 with S100 (4+). All cases were negative with CK5/6, chromogranin, CD99, NB84, desmin, alpha inhibin, and TTF1. The only noticeable difference in the immunophenotype between typical OSCCHT and the large cell variant was that there was 4 +EMA positivity in three of four cases of large cell variant compared with two of 11 cases of typical OSCCHT. OSCCHT is characteristically positive with AE1/3, EMA, CD10, calretinin, WT1, and p53. Combined EMA and WT1 positivity, the latter usually intense and diffuse, may be of diagnostic value, inasmuch as only a few of the neoplasms in the differential diagnosis are positive with both antibodies. Negative staining with CD99, desmin, NB84, alpha-inhibin, and TTF1 may aid in the cases in which primitive neuroectodermal tumor, rhabdomyosarcoma, intraabdominal desmoplastic small round cell tumor, neuroblastoma, a sex cord-stromal tumor, and metastatic pulmonary small cell carcinoma are in the differential. Calretinin positivity precludes its use in the differential with granulosa cell tumors. The results of this investigation do not settle the issue of histogenesis, which remains enigmatic. The typical age distribution, follicle formation, and calretinin positivity are consistent with a sex cord origin. On the other hand, WT1 and EMA positivity and negative staining with alpha-inhibin would be unusual in a sex cord-stromal neoplasm and can be used as an argument for a surface epithelial origin. Germ cell and neuroendocrine origins seem highly unlikely.
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PMID:An immunohistochemical analysis of ovarian small cell carcinoma of hypercalcemic type. 1538 2

We describe two elderly patients with follicular lymphoma (FL) involving the skin and superficial soft tissues, with a striking proliferation of follicular dendritic cells (FDC). In addition, one patient had bone marrow involvement by FL. Histopathologically, the most remarkable feature in both cases seen at low magnification was a striking pallor of the constituent cells, which were arranged in fascicles, whorls, and round islands. The majority of the cells had the typical cytologic features of FDCs. They were intimately intermingled with centroblasts and centrocytes. A large amount of the clear cytoplasm and the pale nuclei of FDCs, which predominated in the tumors, caused the striking overall pallor of the lesions. Small reactive lymphocytes were scattered between the fascicles. A vague follicular growth pattern was seen only focally. The mantle zones were markedly reduced or absent so that the follicles were seen lying unseparated. The close intermixture of the FDCs and the germinal center cells was responsible for the FDCs appearing to be decorated with B-associated marker, and the germinal center cells seemed to be stained to some degree with FDC-markers. The tumor bulk demonstrated a diffuse and strong reaction with CD10, CD20, CD21, CD35, and stained weakly with CD79a. Fascin and CD23 showed only a weak and focal staining pattern. Bcl-2 decorated large centroblasts and small reactive T-cells. The tumor bulk was negative for actin, EMA, cytokeratins, vimentin, desmin, and factor XIIIa. The proliferative index was rather low; MIB-1 mainly decorated large centroblasts. No monoclonal rearrangement of IgH genes was detected. Epstein-Barr virus was not identified. Electron microscopy revealed typical features of FDCs intermingled with germinal center cells. Such cases may represent a diagnostic pitfall, as FDC overgrowth can mask FL and give the neoplasm the appearance of FDC sarcoma/tumor. We believe that, in both cases, the FDC proliferation had a reactive character.
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PMID:Follicular lymphoma of the skin and superficial soft tissues associated with a prominent follicular dendritic cell proliferation: an unusual pattern which may represent a diagnostic pitfall. 1546 4

Primary effusion lymphoma (PEL) is a unique form of non-Hodgkin lymphoma (NHL) associated with Kaposi sarcoma-associated herpesvirus (KSHV; HHV-8) that displays a distinct constellation of clinical, morphologic, immunologic, and molecular characteristics. Rare KSHV-containing immunoblastic lymphomas occurring in solid tissues have been described. Whether they represent part of the spectrum of PEL has not been determined. The morphologic, immunophenotypic, and molecular features of KSHV-positive solid lymphomas occurring in 8 HIV+/AIDS patients were systematically investigated and compared with those of 29 similarly analyzed PELs. The 8 KSHV-positive solid lymphomas were virtually indistinguishable from the 29 PELs based on morphology (immunoblastic/anaplastic), immunophenotype (CD45 positive; T cell antigen negative; CD30, EMA, CD138 positive; CD10, CD15, BCL6 negative) and genotype (100% immunoglobulin genes rearranged; no identifiable abnormalities in C-MYC, BCL6, BCL1, BCL2; and uniformly EBV positive). The only identifiable phenotypic difference was that the KSHV-positive solid lymphomas appeared to express B cell-associated antigens (25%) and immunoglobulin (25%) slightly more often than the PELs (<5% and 15%, respectively; P = 0.11 and P = 0.08, respectively). The clinical presentation and course of the patients who develop KSHV-positive solid lymphomas were also similar, except for the lack of an effusion and somewhat better survival (median 11 months vs. 3 months). However, the 3 KSHV-positive solid lymphoma patients alive without disease 11, 25, and 44 months following initial presentation were recently diagnosed patients and, unlike the other patients with KSHV-positive solid lymphomas, received anti-retroviral therapy. These findings strongly suggest that these decidedly rare KSHV-positive solid lymphomas belong to the spectrum of PEL. Therefore, we propose that the KSHV-positive solid lymphomas be designated extra-cavitary PELs.
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PMID:KSHV-positive solid lymphomas represent an extra-cavitary variant of primary effusion lymphoma. 1548 44

Histological, clinical and immunohistochemical analysis of 6 cases of primary liver lymphomas (PLL) are presented. PLL represents 4.3% of primary malignant liver tumors diagnosed in our department. The patients were relatively young people, who despite the presence of a large tumor, were in good general health status. There were no signs of scirrhosis, and cancer markers were normal. All lymphomas were CD20, CD79a, BAX positive, CD3, CD30, EMA, CD10, CD5, CD59, c-myc, Bcl2, EBV(LMP), CK negative. The proliferation index (Ki67) was high, ranging from 50-100%. In two cases positive staining for Bcl6 and in another one for cyclin D1 was obtained. The major histological type of the tumor was diffuse large B-cell lymphoma. Positive immunohistochemical results with BAX and the lack of Bcl2, c-myc and CD59 are associated with better prognosis. We have not confirmed the value of Bcl6 and CD10 stains as a predictor of poor outcome. Despite clinically advanced stage at the time of diagnosis, if treated appropriately, the primary lymphoma of the liver has relatively good prognosis (five of our patients are alive).
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PMID:Primary lymphoma of the liver -- morphological and clinical analysis of 6 cases. Success of aggressive treatment. 1587 91

Germinal centers within the lymph node follicles are T-cell-dependent, antigen-driven B-cell proliferations that develop from the rapid clonal expansion of a few founder cells. The end results of this B-cell expansion are memory B cells or plasma cells. Two morphologic forms of plasma cell can be recognized in the germinal center: classic plasma cells, characterized morphologically by peripherally clumped arrangement of nuclear chromatin, and cells with a nuclear morphology more resembling that of the centrocytes, which the authors have termed "centrocytoid plasma cells." In this study the authors examined the distribution and immunohistochemical characteristics of these two populations of germinal center plasma cells. The centrocytoid plasma cells were arranged in a band stretching from the junction of the dark and light zone to the periphery of the germinal centers, while the classic plasma cells were mainly present at the germinal center periphery. Both marked with CD38, CD138, and VS38c, recognized markers for plasma cells; however, EMA and CD31 were present only in the classic form of plasma cell. The proliferation marker Ki67 was present in less than 1% of the cells labeling with CD138. Others have demonstrated Ki67 in 50% of the cells labeled with Blimp-1, which is consistent with Blimp-1 appearing earlier than CD138 in ontogeny. CD10 is co-expressed with CD138 in about 10% of cells and CD45 with CD138 in about 5% of cells. Their topographic features, together with the progressive acquisition of plasma cell markers, suggest that the centrocytoid plasma cells may be the precursors of the classic plasma cells. Of note, both the forms of plasma cell were absent in follicles of follicular lymphoma, which supports the concept that in this disease, lymphocytes fail to differentiate and mature beyond the centrocyte stage.
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PMID:Centrocytoid plasma cells of the germinal center. 1589 23

Plasmablastic lymphoma is an HIV-associated non-Hodgkin's lymphoma that primarily affects the oral cavity and jaws. The purpose of this report is to describe the first case of plasmablastic lymphoma occurring in an HIV-negative, nonimmunocompromised individual, and to review the histopathologic and immunohistochemical phenotype of this lymphoma. Histopathologically, our case exhibited a dense, diffuse lymphocytic infiltrate of noncohesive large lymphocytes with plasmacytoid features. Immunohistochemical analysis revealed positivity for the B-cell marker CD79a, VS38c, Epstein-Barr virus latent membrane protein (LMP), immunoglobulin G (IgG), and lambda light chain restriction. Neoplastic cells were negative for leukocyte common antigen, CD20, CD3, CD10, CD138, Bcl-2, Bcl-6, desmin, actin, EMA, S-100, HMB45, Alk-1, HHV8, IgA, IgM, and cytokeratin. The features of this rare disease are summarized based on a comprehensive review of the epidemiologic, clinical and immunohistochemical findings of previously reported cases.
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PMID:Oral plasmablastic lymphoma in an HIV-negative patient: a case report and review of the literature. 1603 78

Mucinous tubular and spindle cell carcinomas are low-grade renal epithelial neoplasms, which were first recognized as a specific entity in the World Health Organization 2004 classification. Forty-five documented cases have been reported. We present 15 additional cases that were incidentally discovered in ten women and five men, with a mean age of 53 years. The tumor is characteristically made up of large eosinophilic regular spindle cells separated by a myxoid stroma with intercellular alcian-blue-positive clear droplets. In peripheral areas, elongated tubules and papillae covered by cubic cells are found. Until this entity had been defined, pathologists used to classify these tumors as variants of solid papillary carcinomas with compressed and elongated papillae, metanephric adenomas, and sarcomatoid carcinomas. In the literature, cytogenetic data indicate various chromosomal losses and gains, but no loss of 3p or trisomy 7 and/or trisomy 17. In two cases, we demonstrate chromosomal loss involving chromosomes 1, 4, 6, 11, 8, 13, 14, 15, 18, and 22. In our 15 cases, immunohistochemistry favored a distal tubule origin (EMA(+), AE1/AE3(+), CK7(+), CK19(+), E-cadherin(+), AMACR(+), and CD10(-)). Prognosis was favorable in our cases, while in the literature, two metastatic cases were reported. Further investigations are required to determine the frequency and true prognosis of these tumors, which are easily identifiable morphologically.
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PMID:Mucinous tubular and spindle cell carcinoma: a report of 15 cases and a review of the literature. 1623 Nov 79

A novel human mammary epithelial cell line, HME348, was established from benign breast tissue from a 44-year-old germ-line BRCA2 mutation carrier with a history of stage 1 breast cancer. Mutation analysis showed that the patient had a known 6872del4 BRCA2 heterozygous mutation. The human mammary epithelial cells passaged in culture exhibited cellular replicative aging as evidenced by telomere shortening, lack of telomerase activity, and senescence. Ectopic expression of telomerase (hTERT) reconstituted telomerase activity in these cells and led to the immortalization of the cells. When grown on glass, the majority of immortalized HME348 cells expressed ESA and p63 with a small population also expressing EMA. In three-dimensional Matrigel culture, HME348 cells formed complex branching acini structures that expressed luminal (EMA, CK18) and myoepithelial (p63, CALLA, CK14) markers. Three clones derived from this culture were also p63(+)/ESA(+)/EMA(+/-) on glass but formed similar acinar structures with both luminal and myoepithelial cell differentiation in Matrigel confirming the mammary progenitor nature of these cells. Additionally, the experimentally immortalized HME348 cells formed acini in cleared mammary fat pads in vivo. As this is the first report establishing and characterizing a benign human mammary epithelial cell line derived from a BRCA2 patient without the use of viral oncogenes, these cells may be useful for the study of BRCA2 function in breast morphogenesis and carcinogenesis.
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PMID:Telomerase immortalization of human mammary epithelial cells derived from a BRCA2 mutation carrier. 1654 10

Papillary renal cell carcinoma (RCC) is subclassified in type 1 displaying cells with scanty pale cytoplasm arranged in a single layer and in type 2 showing pseudostratified cells with eosinophilic cytoplasm. However, the existence of more variants of papillary RCC may be inferred by the recognition of few cases with different morphological features. We report the clinicopathologic, immunohistochemical, ultrastructural, and interphase cytogenetic features of 12 papillary RCC composed by oncocytes. Ten patients were males and their median age was 67 years. The tumors were well demarcated and their median diameter was 7.1 cm. Solid oncocytoma-like areas occurred in 11 cases. The cytoplasm of the neoplastic cells was filled by mitochondria with lamellar cristae. All cases were positive for the antimitochondrial antigen and racemase and showed variable immunoreactivity for cytokeratins (AE1/AE3, CK8-18, CK7, CK19), EMA, CD10, vimentin, and parvalbumin. MIB1 was detected in 0 to 6 cells per 1 high-power field. Fluorescent in situ hybridization analysis on formalin-fixed paraffin-embedded tissue showed three or more signals for chromosome 7 and 17 (for both > or =30% of nuclei in 7 of 12 neoplasms). In males, signals of chromosome Y were absent in more than 80% of the neoplastic nuclei. One patient died of metastases. Interphase cytogenetic analysis by fluorescent in situ hybridization can be a diagnostic tool in cases mimicking an oncocytoma.
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PMID:Oncocytic papillary renal cell carcinoma: a clinicopathologic, immunohistochemical, ultrastructural, and interphase cytogenetic study of 12 cases. 1673 Mar 6

4 cases (2 males and 2 females) of mucinous tubular and spindle-cell carcinoma of the kidney were studied. The patients' age was 43 to 87 years. Microscopically, this tumor comprised two major cell populations. One of them involved spindle cells with a sparse cytoplasm, which were arranged in the fascicular pattern, often resembling low-grade smooth muscle tumors. The other population was small cuboidal cells with round nuclei and an eosinophilic cytoplasm. The mitotic activity was low. Only one tumor had both cell components. The other three tumors were represented by the second type cells. Immunohistochemical staining with avidin-biotin was performed by using cytokeratin, vimentin, SMA, p53, Bcl 2, EMA, and CD10. All cases were stained positively with antibodies to the cytokeratins AE1-AE2 and vimentin. The expression of EMA, Bcl 2, and CK7 was varying in different tumors. Responses to smooth muscle actin, p53, CK20, and CD10 were negative in all cases. Mucinous tubular and spindle-cell carcinoma of the kidney should be differentiated from angiomyolipoma with the preponderance of a leiomyomatous component, renal smooth muscle neoplasms, metanephrotic adenoma, and renal cell carcinoma with sarcomatoid differentiation.
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PMID:[Mucinous tubular and spindle-cell carcinoma of the kidney]. 1698 88


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