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)

The existence of chondroid chordoma (CC), initially described in 1973, has remained controversial. Since the antigenic profiles of both chordoma (CD) and cartilaginous (chondroid) lesions have been well characterized, we decided to study chondroid chordoma immunohistochemically. Our hypothesis was that chondroid chordoma should display a hybrid or mixed pattern of staining: chordomatous areas with an epithelial phenotype and cartilaginous areas with a mesenchymal (non-epithelial) phenotype. An analysis of CC (seven cases) was performed and compared with results obtained on notochord, cartilage, classic CD (18 cases), peripheral chondromas (two cases), and peripheral chondrosarcomas (CS, eight cases). Four epithelial markers were employed: MKER and AE-1 (both monoclonal antibodies to cytokeratin); PKER (a polyclonal antibody to cytokeratin); and, EMA (epithelial membrane antigen). In addition, selected cases were tested for the presence of neurofilament (NF) and glial fibrillary acidic protein (GFAP). All 18 CD's exhibited the expected epithelial immunophenotype - MKER+, AE-1+, PKER+, and EMA+ - a reaction pattern nearly identical to that found in fetal notochord. This reinforced the importance of the growth pattern in assessing the presence of chordomatous elements. All chondromas and CS's failed to express any of the epithelial markers studied and contained only S-100 immunoreactivity, like cartilage. Chondroid chordoma resembled cartilaginous tumors immunohistochemically; no mixed pattern with even focal epithelial marker reactivity was identified. All CC tested were also NF and GFAP negative. We conclude that CC either does not exist or is extremely rare and that these tumors are cartilaginous in nature.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Does chondroid chordoma exist? 243 34

Chordomas are slowly growing malignant tumors arising from notochordal rests. They are occurring in adults (50 to 60 year old) and are mainly (85%) located in sacrococcygeal or spheno-occipital regions; other main localization is cervical spine. Chordomas are usually discovered in patients with pain or symptoms due to compression of surrounding viscera. Radiologically it is characterized by association of osteolysis and soft tissues opacity. On macroscopic examination tumoral tissue has mucoid appearance; under microscope it is made up with lobules of epithelial-appearing cells surrounded by acid mucosubstances. Tumorous cells contain glycogen and neutral mucosubstances. They are surrounded by argyrophilic rim due to pericellular condensation of intercellular matrix, well viewed on electron microscope examination. When their cytoplasm is filled with vacuoles, cells take up typical physaliphorous appearance. Chordomas cells express epithelial differentiation antigens (low molecular weight cytokeratins, EMA, CAM 52, HFM 62, even CEA), Vimentin and S-100 Protein: this triple positivity allow differentiation between chordomas and numerous others tumors. Correct treatment of chordoma is achieved with an initially complete excision. Local recurrences are frequent and sometimes inoperable: in this cases radiotherapy alone may be performed (70 grays). Sarcomas (fibroblastic or Malignant fibrous histiocytoma) may occur after radiotherapy or without it. Hematogenous metastasis occur in 10% to 15% of patients. Survival rate at five years is included between 50% and 75%. Chondroid chordoma is a special entity occurring in younger patients (35 year old) and located in spheno-occipital region. In addition to chordomas it contain chondroid (benign or malignant) islands. Mean survival rate (16 years) is far better than for chordoma or chondrosarcoma.
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PMID:[Chordomas]. 329 77