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)

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.
 ...
PMID:KSHV-positive solid lymphomas represent an extra-cavitary variant of primary effusion lymphoma. 1548 44

Yolk sac tumors (YSTs) have a variety of morphologic patterns, some of which can resemble either endometrioid adenocarcinoma (EAC) or clear cell carcinoma (CCC). Immunohistochemical staining for alpha-fetoprotein (AFP) is usually only focal and thus is not always helpful in the diagnosis of YST, and pancytokeratin (CK) is expressed by all three tumors. We studied a battery of immunohistochemical markers with specific attention to the utility of cytokeratin 7 (CK7) in differentiating YST from EAC and CCC. A total of 46 ovarian tumors were retrieved for this study: 16 YST, 19 EAC, and 11 CCC. The three groups were analyzed for the expression of CK7, AFP, Leu-M1 (CD15), EMA, and WT1 by immunohistochemistry. In addition, CK and c-kit (CD117) were studied in the YSTs. All of the YSTs tested (100%) were positive for CK. CK7 was considered negative in all 16 YST cases (100%), although a few tumor cells (1%-2%) stained in 4 cases. In contrast, 17 of 19 EACs and all 11 CCCs had diffuse 3+ to 4+ positivity for CK7; the two other EACs showed 2+ positivity for CK7 (40% and 30% of the tumors). AFP was positive in 12 of 15 YSTs (80%), but was generally focal with 1+ staining in 10 cases (67%); only 2 cases were 3+. All of the EACs and CCCs were negative for AFP. Leu-M1 was 1+ in 9 of 15 YSTs (60%), while the remaining 6 were considered negative. Leu-M1 was positive in 10 of 15 EACs tested (67%), but the staining was variable with 1 case 3+, 3 cases 2+, and 6 cases 1+. In the CCCs, 10 cases (91%) were 3+ to 4+, and 1 case was 1+. EMA was essentially negative in 15 of 15 YSTs (100%), with 3 completely negative and 12 showing very focal (<5%) staining. Eight of 12 EACs showed 4+ staining, 3 showed 3+ staining, and 1 showed 2+ staining. All of the 11 CCCs (100%) showed 4+ staining. WT1 was negative in all cases of YST and CCC; 16 of 18 EAC tested (89%) were negative for WT1, but 2 (11%) were 4+ positive. C-kit was negative in all YSTs. In conclusion, it is important for pathologists to be aware that YSTs may mimic EACs and CCCs and that this distinction is important for the clinical management of patients with these tumors. AFP staining is focal in most YST, so an absence of staining does not exclude this diagnosis. CK7 and EMA are essentially negative in YST but are diffusely positive in CCC and EAC, making them useful markers for differentiating YSTs from both CCCs and EACs. Leu-M1 may also be helpful for distinguishing YSTs from CCCs.
 ...
PMID:The use of cytokeratin 7 and EMA in differentiating ovarian yolk sac tumors from endometrioid and clear cell carcinomas. 1548 54

Warthin tumor-like variant of papillary thyroid carcinoma is uncommon and approx 80 cases have been reported in the literature. This tumor is often associated with a favorable prognosis. In this report, a Warthin tumor-like variant of the papillary thyroid carcinoma, 5-cm in maximum dimension, underwent anaplastic changes in a 74-yr-old woman. The tumor was positive for CD15 and EMA, and a high proliferative index was noted in the anaplastic area. The patient developed distant metastases after operation and died of the disease 18 mo after the operation. The present case is the first reported case of Warthin tumor-like variant of papillary thyroid carcinoma with anaplastic changes.
 ...
PMID:Warthin tumor-like variant of papillary thyroid carcinoma: a case with dedifferentiation (anaplastic changes) and aggressive biological behavior. 1600 Aug 51

Extra-nodal Hodgkin's lymphoma (HL) represents 15% of all Hodgkin's lymphomas; the primary intestinal site accounts for 1% and with involvement of the ascending colon being rare. We present the case of a patient of 62 years of age diagnosed as having acute appendicitis. Anatomopathology on the excised appendectomy tissue indicated nodular lymphocytic predominant Hodgkin's lymphoma (NLPHL). The morphology indicated isolated L&H (lymphocytic or histiocytic) cells or in groups, surrounded by T lymphocytes, in an environment of germinal centres together with phenomena that would be interpreted as progressive transformation. Immunohistochemistry staining of the HL cells were positive for CD45, CD20, Bc16, EMA and MUM1 and negative for CD15 and CD30. No complementary treatment was administered. Following a literature search, the present case would appear to be the first of its kind.
 ...
PMID:Primary Hodgkin's lymphoma of the caecum. 1679 Mar 99

The recent WHO classification of non-Hodgkin's lymphoma is based on the morphology and immunohistochemical expression of the lymphoma cells and to a lesser extent, on the molecular and cytogenetic findings. Fifty-three cases of non-Hodgkin's lymphoma were included in the study. Of these, seven cases were primary extra nodal lymphomas. Twenty two patients had peripheral blood and/or bone marrow involvement. A detailed morphological assessment was done and classified using the International working formulation. The two most common types encountered were diffuse large cell lymphoma and small lymphocytic lymphoma. Immunohistochemistry was done using labeled streptavidin-biotin peroxidase complex with CD3, CD20, CD15, CD30, CD 45 (leukocyte common antigen), Cyclin D1, EMA (epithelial membrane antigen). 38 cases (72%) showed B cell expression and 12 cases (22.5%) showed T cell expression. Three cases did not express either marker. B-cell diffuse large cell lymphoma (26%) was found to be the predominant B cell non-Hodgkin's lymphoma. The commonest T-cell lymphoma was T lymphoblastic lymphoma (67%) followed by peripheral T cell angioimmunoblastic lymphoma (25%). Immunohistochemistry is a useful and necessary diagnostic modality and helps subdivide prognostically different types of non-Hodgkin's lymphoma.
 ...
PMID:Immunohistochemical typing of non-Hodgkin's lymphoma-comparing working formulation and WHO classification. 1693 15

Classic Hodgkin's lymphoma (cHL) most often involves lymph nodes, and gastric involvement is rare. Hodgkin's and Reed-Sternberg (H-RS) cells in cHL are known to often lack expression of several B-lineage markers, such as CD20, CD79a, Oct-2, and Bob-1. We present an extremely rare case of mixed-cellularity cHL in the stomach in which expression of these B-cells was detected immunohistochemically. The patient was an 83-year-old Japanese woman who developed a sensation of abdominal fullness and appetite loss. Endoscopic and abdominal computed tomography examinations revealed a gastric ulcer lesion and swelling of para-aortic lymph nodes, respectively. A subtotal gastrectomy was performed, and the histopathologic diagnosis was established as a typical cHL compatible with stomach origin. The patient underwent postoperative chemotherapy of 3 cycles of ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) and has since been in complete remission. Immunohistochemically, the H-RS cells in the cHL were positive not only for CD30 but also for CD20, CD79a, Oct-2, and Bob-1, whereas they were negative for CD3, CD15, CD45, EMA, and ALK1. Our patient may have had an intermediate cHL disease overlapping that of non-Hodgkin's peripheral B-cell lymphoma, possibly reflecting derivation from germinal-center B-cells.
 ...
PMID:Primary gastric Hodgkin's lymphoma expressing a B-Cell profile including Oct-2 and Bob-1 proteins. 1756 19

Acral myxoinflammatory fibroblastic sarcoma (AMIFS) is a low-grade sarcoma that presents mostly in distal extremities of middle-aged patients. The clinicopathologic features, immunohistochemical profile and follow-up data of five cases (three men and two women; age 39-65 years) are presented. The tumors presented as a slow-growing, poorly circumscribed, subcutaneous masses in the hands (three), foot (one) and calf (one), with dermal involvement in two cases. They had myxoid and hyaline stroma with dense acute and chronic inflammation. Spindle cells, large bizarre ganglion-like cells and multivacuolated cells were seen. Variable reactivity in lesional cells were noted for vimentin, Alpha-1-antitrypsin (A1AT), factor XIIIa, CD68, CD95, CD117, Alpha-1-antichymotrypsin (A1ACT), CD34, AE1/3, S-100 protein, EBER, CD63 and CD15. MIB-1 showed 5-30% nuclear labeling. They were negative for cytokeratin AE1/3, smooth muscle actin, CD30, ALK-1, EMA, desmin, CMV, HMB-45 and Melan-A. Follow up ranged from 2 weeks to 95 months (mean 54). One patient was lost to follow up; three underwent excision and one patient had below the knee amputation. Two patients developed metastases (one died of disease), and two patients are alive without evidence of disease. AMIFS are rare tumors that may involve joints and tendons leading to clinical diagnosis of ganglion cyst or tenosynovitis.
 ...
PMID:Acral myxoinflammatory fibroblastic sarcomas: are they all low-grade neoplasms? 1819 Apr 43

A case of CD30-positive microvillous lymphoma (MVL) in an 87-year-old man who was encountered generalised lymphadenopathy is presented. Histopathologically, the tumour showed a morphological mimic of anaplastic large cell lymphoma (ALCL) with sinusoidal growth pattern. Immunohistochemically (IHC), the tumour cells were CD30(+), CD20(+), CD45(+), BCL-2(+), BCL-6(+), MUM1(+), Ki-67(+), CD45RO(-), CD3(-), CD10(-), CD15(-), CD56(-), EMA(-), TIA-1(-) and ALK(-). Flow cytometry confirmed the IHC. In situ hybridisation for Epstein-Barr virus RNA was negative. Electron microscopically, the tumour cells were similar to large transformed lymphocytes and had circumferentially profuse microvillous projections resembling those of epithelial mesothelioma cells. In conclusion, CD30-positive MVLs are indistinguishable from ALCLs that have ultrastructural microvillous projections by morphology alone. However, the lack of EMA, TIA-1 and ALK expression in this MVL case facilitated a definite distinction from ALCLs. The results of a panel of three markers (CD10(-), Bcl-6(+) and MUM1(+)) suggested that the present case of CD30-positive MVLs has an activated non-germinal centre B-cell origin.
 ...
PMID:CD30-positive diffuse large B-cell lymphoma with microvillous features: so-called microvillous lymphoma. 1912 65

Human herpesvirus type 8 (HHV8), also known as Kaposi's sarcoma-associated herpesvirus, is a human gamma herpesvirus that underlies the pathogenesis of Kaposi's sarcoma, primary effusion lymphoma and multicentric Castleman's disease. We recently encountered two cases of HHV8-positive large B-cell lymphoma with features not previously described. The first patient was a 61-year-old immunocompetent man with an enlarged cervical lymph node containing scattered large, bizarre cells in a reactive background of lymphocytes, plasma cells and scattered regressed follicles resembling those of hyaline-vascular Castleman's disease. The appearance suggested classical Hodgkin's lymphoma, but the large cells were negative for CD15, CD30, CD20 and CD3, and positive for MUM1/IRF4, EMA, HHV8, EBER and dim IgM lambda. The second patient was a 59-year-old HIV-positive man who died after several weeks of fever, night sweats, anemia, thrombocytopenia, hepatosplenomegaly and multiorgan failure. At autopsy an intravascular large B-cell lymphoma that was positive for MUM1/IRF4, HHV8 and IgM lambda, and negative for CD20 and EBER involved multiple organs, including lung, heart, kidney, liver and spleen. On the basis of the histologic features in these two cases, the presence of HHV8 was unexpected. These cases expand the spectrum of lymphoproliferative disorders that can be associated with HHV8.
 ...
PMID:HHV8-positive, EBV-positive Hodgkin lymphoma-like large B-cell lymphoma and HHV8-positive intravascular large B-cell lymphoma. 1928 57

Lymphocyte depleted classical Hodgkin lymphoma (LDHL) is a vanishing category of classical Hodgkin lymphoma (CHL); many cases previously placed in this category are now recognised as diffuse large B-cell lymphoma (DLBCL), anaplastic large-cell lymphoma (ALCL), or nodular sclerosis CHL with lymphocyte depletion. In addition, the recent recognition of high grade B-cell lymphomas intermediate between DLBCL and CHL (grey-zone lymphomas) raises the question of whether LDHL exists at all as a category of CHL. We studied eight cases that fulfilled diagnostic criteria of LDHL according to the 2008 WHO Classification. The cases involved lymph nodes (7 cases) and pleura (1 case) from four males and four females (age 30-71 years; median 62 years). All tumors contained numerous Hodgkin-Reed-Sternberg (HRS) cells, fibroblasts and histiocytes and scattered lymphocytes. In three cases the tumors had a more diffuse fibrotic appearance, while in five cases they appeared reticular and anaplastic. Neoplastic cells in all cases expressed CD30, CD15, fascin, weak PAX5 and MUM-1 and lacked CD45, Alk-1, EMA, CD3, CD68, Mart-1 and cytokeratin. Oct.2 and/or Bob-1 were expressed in all cases. Two cases variably expressed CD20 but were CD79a negative. Four cases were positive for EBV. All the four cases with adequate DNA had clonally rearranged IGH genes. The combined morphologic, immunophenotypic and molecular genetic features of this group of cases distinguish LDHL from other disease entities, including grey-zone lymphomas.
 ...
PMID:Lymphocyte depleted Hodgkin lymphoma: an evaluation with immunophenotyping and genetic analysis. 1945 61


<< Previous 1 2 3 4 5 6 Next >>