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)

We report on a case of a sarcoma arising in the hyaline-vascular variant of Castleman disease (HVCD) of the skin and subcutis. The patient was a 38-year-old man who clinically presented with a subcutaneous non-fixed cyst-like mass on his right shoulder with an unremarkable prior medical history. Histologic sections showed a biphasic tumor with numerous atretic lymphoid follicles located in the deep dermis and subcutis and a spindle-cell neoplasm mainly situated in the deep subcutis and adjacent soft tissue. The atretic lymphoid component fulfilled the criteria for HVCD, whereas the spindle-cell lesion showed all the criteria for sarcoma including nuclear atypia and frequent mitotic figures. The sarcomatous component was diffusely positive for fascin, nerve growth factor receptor, and CD34 with focal weak reactivity for CD21 and CNA.42. Stains for CD23, CD31, CD35, CD99, ALK-1, SMA, ASMA, desmin, factor XIIIa, AE1-AE3, EMA, bcl-2, S-100, Melan-A, HMB-45, Cam 5.2, and factor VIII were negative in the neoplastic spindle cells. No monoclonal population of lymphocytes was detected and we could not identify EBV or HHV-8 virus by PCR. Electron microscopy of the sarcomatous component showed spindle cells with labyrinth-like invaginations of the nucleus and numerous long, slender, interwoven cytoplasmic processes. The sarcomatous component in this case is most consistent with a poorly differentiated follicular dendritic cell sarcoma based upon the morphologic and ultrastructural findings.
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PMID:Sarcoma arising in hyaline-vascular castleman disease of skin and subcutis. 1612 Oct 55

As a result of overlapping morphologic and immunohistochemical features, it can be difficult to distinguish synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma/primitive neuroectodermal tumor in core biopsies. To analyze and compare immunohistochemical profiles, we stained tissue microarrays of 23 synovial sarcomas, 23 malignant peripheral nerve sheath tumors, and 27 Ewing sarcomas with 22 antibodies potentially useful in the differential diagnosis, and analyzed the data with cluster analysis. Stain intensity was scored as none, weak, or strong. For CD99, tumors with membranous accentuation were independently categorized. Cluster analysis sorted five groups, with like tumors clustering together. Synovial sarcoma clustered into two groups: one cytokeratin and EMA positive (n = 11), the other mostly cytokeratin negative, EMA positive, bcl-2 positive and mostly CD56 positive (n = 9). Malignant peripheral nerve sheath tumor clustered into two groups: one S100 positive, with nestin and NGFR positivity in most (n = 10), the other mostly S100 negative, and variably but mostly weakly positive for nestin and NGFR (n = 11). Ewing sarcomas clustered into a single group driven by membranous CD99 staining. Thirteen cases failed to cluster (outliers), while three Ewing sarcomas clustered into groups of other tumor types. Paired antibodies for each tumor type determined by visual assessment of cluster analysis data and statistical calculations of specificity, sensitivity, and predictive values showed that EMA/CK7 for synovial sarcoma, nestin/S100 for malignant peripheral nerve sheath tumor, and membranous CD99/Fli-1 for Ewing sarcoma yielded high specificity and positive predictive values. Cluster analysis also highlighted aberrant staining reactions and diagnostic pitfalls in these tumors. Hierarchical cluster analysis is an effective method for analyzing high-volume immunohistochemical data.
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PMID:Cluster analysis of immunohistochemical profiles in synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma. 1652 78