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)

Paraffin-embedded specimens from 21 patients (mean age 49 years) with malignant melanocytic tumors of the central nervous system were studied. Extraneuronal primary tumors were situated at the trunk (38%), the lower (14%) or upper extremity (10%), and the head/neck region (5%). In 33% no extraneural primary tumor could be detected. The tumor location was frontal (19%), occipital (19%), parietal, spinal, multifocally (14%, respectively), or temporal (5%). Four subtypes were distinguished according to the predominant histological cell type: pleomorphic, epithelioid, spindle- and mixed-cell tumors. 29% contained no melanin, most of them belonging to the epithelioid subtype. The morphology and immunohistochemical reactivity for different antibodies (KL-1, EMA, VIM, HMB-45, NKI-C3, S-100, and MIB-1/Ki-67) were assessed. Positive staining was demonstrated for HMB-45 (in 86% of cases), NKI-C3 (100%), S-100 (95%), vimentin (75%), and KL-1 (33%). No expression of the cytokeratin EMA could be detected. The mean proliferation index measured by MIB-1 immunoreactivity was 21%. The 4 histological subtypes were found to express different antigen patterns. In the analysis of CNS tumors of unknown origin, the panel of antibodies used for diagnosis should include HMB-45 as the most specific marker for malignant melanoma.
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PMID:Malignant melanoma in the CNS, subtyping and immunocytochemistry. 940 2

Extramammary Paget's disease (EMPD) is a rare cutaneous neoplasm. The aim of this study was to elaborate the clinical and pathological features of Chinese EMPD male patients. The study comprised 246 patients with EMPD at our institute from January 1993 to December 2012. Scrotum was the most common initial site. The average age of onset was 63.9 years but the mean delay in diagnosis was 3.6 years. EPMD spread exclusively to the inguinal lymph nodes and the right inguinal lymph nodes are more likely to suffered Paget cells infiltration. Accompanying malignancies were found in 20 patients. Pathological examination revealed 63 patients defined as invasive EMPD. Immunohistochemical detection showed various expression levels of EMA, CEA, CK7, HER2/neu, Ki67, P53, CK20 and S100 in tumor tissues, but negative expression of VIM, LCA and HMB45. HER2/neu protein exhibited a significant association with invasive EMPD. A novel histological type of EMPD with CK7-/S100+ was identified. Elevated serum PSA level was observed in only 16% patients. Invasive EMPD often had advanced age of onset. Metastatic EMPD showed significantly shorter in the delay in diagnosis and the greater length of skin lesion in contrast to others. This study demonstrates the clinical and pathological features of Chinese male EMPD patients, and may provide implications for the management of Chinese EMPD patients.
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PMID:Clinical and pathological characteristics of extramammary Paget's disease: report of 246 Chinese male patients. 2672 23

Sarcomatoid carcinoma of the gallbladder, composed of epithelial and sarcomatous components, is a rare malignant tumor with a poor prognosis. A 61-year-old man was admitted because of abdominal distention for 2 weeks. Enhanced CT showed a large gallbladder tumor with central necrosis. The tumor measuring 18 cm in maximum diameter showed intense peripheral FDG uptake with SUVmax of 15.4. The specimen of the resected tumor revealed tubular adenocarcinoma and sarcomatoid tissue with chondroid differentiation. Immunohistochemical staining showed the sarcomatoid tissue was positive for EMA, CD10, and VIM, with proliferation index of 80%. These pathologic findings were consistent with sarcomatoid carcinoma.
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PMID:FDG PET/CT in Sarcomatoid Carcinoma of the Gallbladder With Chondroid Differentiation. 2685 17

Synovial sarcoma (SS) is a mesenchymal tumor of uncertain histogenesis which is defined by the translocation t(X;18). Transducer-like enhancer of split 1 (TLE1) as a new immunomarker for SS has offered an alternative to pathologists in distinguishing synovial sarcoma from other mesenchymal neoplasms, especially in limited molecular facilities. The main aim was to study the expression and diagnostic specificity and sensitivity of TLE1 in SS. We performed this immunohistochemical study on 155 SS (107 monophasic, 35 biphasic, and 10 poorly differentiated), 10 fibrosarcomas, 10 angiosarcomas, 10 epithelioid sarcomas, 10 Ewing sarcoma/PNETs and 8 malignant peripheral nerve sheath tumors (MPNST) using TLE1 immunomarker. Furthermore, in problematic cases (n=43), molecular confirmation was performed by fluorescent in situ hybridization (FISH) to detect the t(X;18) translocation. We correlated the TLE1 overexpression with the t(X;18) and other established biomarkers (CD117, CD56, cytokeratin AE1/AE3, EMA, CD99, BCL2, VIM, CD34, S100, Ki67, SMA). TLE1 expression was observed in 76.19% (112/147) of the SS, including 75.96% (79/104) of monophasic, 78.79% (26/33) of biphasic, and 70% (7/10) of poorly differentiated type. 65.99% (97/147) of SS cases showed a strong to moderate staining of TLE1. Other mesenchymal tumors showed very low or absent staining of TLE1 (P < 0.001). The overall sensitivity and specificity of TLE1 expression for the diagnosis of SS were 86.21% and 78.57%, respectively. Further molecular analysis showed that t(X;18) was clearly correlated with TLE1 expression (P=0.000). TLE1 is a specific and sensitive diagnostic immunomarker for SS and can be helpful to distinguish SS from other mesenchymal neoplasms.
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PMID:Transducer-like enhancer of split 1 (TLE1) as a novel biomarker for diagnosis of synovial sarcoma correlates with translocation t(X;18): a study of 155 cases in China. 3193 40