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)

A case of malignant nerve sheath tumor with glandular differentiation in a patient with neurofibromatosis is presented. In the spindle celled areas the tumor cells reacted strongly with vimentin and S-100 protein, while the glandular epithelia reacted with EMA, cytokeratins (peptides 8, 19, and 20) and CEA, as well as a few of them also with chromogranin. Based on the results of immunohistochemical profile of the tumor cells, the differential diagnosis of this rare soft tissue tumor from biphasic synovial sarcoma with glands, as well as its distinction from any other spindle cell sarcoma with entrapped skin appendages could be greatly facilitated.
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PMID:Malignant peripheral nerve sheath tumor with glandular differentiation. Report of a case with emphasis to the usefulness of immunohistochemistry in the differential diagnosis. 933 15

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.
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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

BACKGROUND: Primary pure breast sarcoma is a rare disease and constitutes 0.2-1.0% of all mammary malignancies. The establishment of a diagnosis of soft tissue sarcoma is difficult in adults. Immunohistochemical analysis usually proves to be helpful in indistinguishable cases. The simplistic step is to classify sarcomas on a simple descriptive basis as spindle cell sarcomas, myxoid sarcomas, pleomorphic sarcomas, and small round cell sarcomas. CASE REPORT: Here, we present a rare case of primary spindle cell sarcoma of the breast. A 43-year-old woman was admitted to our clinic with a 2-month history of a left breast lump. Histopathological examination showed a tumor of 2.5 cm in diameter and of nuclear and histological grade 2. In the immunohistochemical examination, vimentin positivity, high nuclear overexpression of P53, high Ki-67 and S-100, desmin, leukocyte common antigen, keratin, and smooth muscle antigen, CD34, HMB45 and EMA negativity were detected. CONCLUSION: Most invasive breast neoplasms are epithelial tumors, and mesenchymal breast tumors are rarely seen. In primary breast sarcoma, adequate surgical tumor excision, tumor grade, and tumor diameter seem to be the most important prognostic factors.
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PMID:Primary Spindle Cell Sarcoma of the Breast. 2082 39

Sarcomatoid variant of anaplastic large cell lymphoma (ALCL) is one of the rarest histologic variants of ALCL that consists of large, bizarre, often spindle-shaped, neoplastic cells resembling a soft tissue sarcoma. We report here such a case of ALCL with both pulmonary and multiple nodal involvement in a 47-year-old woman who initially presented with fever, cough, sputum, itching skin, and weight loss. The initial transbronchial lung biopsy showed discohesive pleomorphic malignant cells in a strong inflammatory milieu reminiscent of inflammatory malignant fibrous histiocytoma (MFH). Subsequent cervical lymph node biopsy revealed a spindle cell sarcoma predominantly composed of plump spindle and oval neoplastic cells in interweaving fascicles, with sparse inflammatory infiltrates, resembling pleomorphic-storiform type of MFH. However, these tumor cells in the lung and node lesions revealed essentially similar immunohistochemical features that were positive for CD30, EMA, TIA-1, granzyme B, and fascin, but negative for anaplastic lymphoma kinase (ALK), and T- or B-lineage-specific marker. The spindled cells stains diffuse strong positive for smooth muscle actin (SMA), along with vimentin. Further studies showed that the tumor produced large quantities of the proinflammatory cytokines interleukin-2 (IL-2), IL-6, and IL-8, which we believe may contribute to the pathogenesis of sarcomatoid transformation of this tumor, and was associated with the patient's inflammatory symptoms. To the best of our knowledge, this is the first reported case of sarcomatoid variant of ALK-negative ALCL with null cell phenotype and in situ production of proinflammatory cytokines presenting as multiple nodes and pulmonary involvement.
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PMID:Sarcomatoid variant of ALK- anaplastic large cell lymphoma involving multiple lymph nodes and both lungs with production of proinflammatory cytokines: report of a case and review of literature. 2519 51