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Query: UMLS:C0268596 (EMA)
 2,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Six chordomas, ten fetal notochords and eight adult notochords were stained for keratin, vimentin, GFAP, desmin, CEA, EMA and s-100 protein. Coexpression of four kinds of intermediate filaments was common in fetal notochords and chordomas. According to the histologic and immunohistochemical characteristics, chordoma may originate from the ectopic remnants of undegenerated notochord during the embryonic stage. The chordoma consists of two cell types; nonvacuolated and physaliphorous cells, and exhibits the potentiality of multidirectional differentiation. The coexpression of different types of intermediate filaments could explain the morphological transformation of chordoma.
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PMID:[Coexpression of intermediate filaments in notochords and chordomas and its significance in the histogenesis and diagnosis of chordoma]. 128 74

Altogether 20 chordomas were reported. The site distribution included 9 cases at the sacrococcygeal region, another 9 cases at the spheno-occipital region, 1 at the cervical vertebra, and another 1 at the lumbar vertebra. Histologic examination revealed that characteristic "physaliphorous cells" were easily identified in all the 19 cases. Tissue for immunohistochemistry study was available in 18 cases. Among them, tumor cells were found strongly positive to EMA, but negative for CEA. 16/18 cases also showed positive for keratin and S-100 protein. Totally, 2 cases were studied ultrastructurally and there were abundant RER and microfilaments seen in the cytoplasm of the tumor cells but only few surface microvilli detected. The epithelial nature of chordoma is strongly supported by the ultrastructural and immunohistochemical findings of these 20 cases.
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PMID:[An immunohistochemical and ultrastructural study of 20 chordomas]. 137 90

The aim of the present investigation was to characterize the various cell types of classical and chondroid chordomas. Eight cases of classical chordoma, 1 case of sacrococcygeal chordoma with chondroid areas and 2 cases of spheno-occipital chondroid chordoma were studied. Ultrastructurally and immunohistochemically (immunoreactivity for cytokeratins, epithelial membrane antigen [EMA], tissue polypeptide antigen [TPA] and human milk fat globule protein [HMFG]) the 3 cell types (physaliferous, epithelial-like, and spindle-shaped) recognized light-microscopically presented features of epithelial differentiation and rather formed a continuous spectrum than being distinct cell types. The chondroid areas of the chondroid chordomas had similar ultrastructural and immunohistochemical properties except for the lack of immunoreactivity for EMA and HMFG. The results of the critical electrolyte concentration technique according to Scott and Dorling indicated that there was no difference in the sulfated glycosaminoglycan content between classical and chondroid chordomas: all the tumors contained chondroitin sulfate. The presence of chondroitin sulfate, immunoreactivity for vimentin and S-100 protein and areas of cartilaginous differentiation in three cases indicate a relationship both to chondromatous tumors and to normal notochord, from which chordoma is believed to originate.
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PMID:Classical and chondroid chordoma. A light-microscopic, histochemical, ultrastructural and immunohistochemical analysis of the various cell types. 175 9

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

"Chondroid chordoma" is a controversial and confusing entity that was originally described by Heffelfinger and colleagues as a biphasic malignant neoplasm possessing elements of both chordoma and cartilaginous tissue. Because the premise for this distinction was based strictly on histomorphologic criteria, the light microscopic, immunohistochemical, and electron microscopic features of the chondroid and chordoid areas of five chondroid chordomas of the skull base were evaluated separately, and compared to five typical chordomas and six low grade chondrosarcomas. Using light microscopy, chondroid chordoma revealed areas that resembled typical chordoma (chordoid areas) and areas that resembled low grade chondrosarcoma (chondroid areas). However, both the chordoid and chondroid areas had an epithelial phenotype and stained strongly for cytokeratin and EMA as well as S-100. 5'-nucleotidase, an enzyme that has been described in chordoma but not in chondrosarcoma, was found in both the chordoid and chondroid areas of one chondroid chordoma. Electron microscopic studies of both the chordoid and chondroid areas in four of the tumors demonstrated both tonofibrils and desmosomes. Chordoma demonstrated immunohistochemical and electron microscopic features that were nearly identical to chondroid chordoma. Chordoma was cytokeratin, EMA, S-100, and 5'-nucleotidase positive. Ultrastructurally, chordoma exhibited variably-sized vacuoles, abundant rough endoplasmic reticulum (RER), and desmosomes with tonofilaments. In contrast to chondroid chordoma, chondrosarcoma consistently stained for only S-100 protein and was cytokeratin, EMA and 5'-nucleotidase negative. Ultrastructurally, chondrosarcoma demonstrated a flocculogranular matrix, glycogen, abundant RER, and scalloped cellular outlines, but lacked desmosomes with tonofilaments. These findings indicate that "chondroid chordoma" is a variant of chordoma with histologic features that may mimic chondrosarcoma. Despite the resemblance of these hyalinized areas to cartilaginous tissue, these tumors retain their epithelial phenotype. Biphasic differentiation is not present. These findings undermine the original premise for distinguishing "chondroid chordoma" from typical chordoma. The authors propose that these tumors be classified as "hyalinized chordomas," rather than "chondroid chordoma," to clarify their histogenesis and avoid confusion with chondrosarcomas of the base of the skull.
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PMID:Chondroid chordoma. A hyalinized chordoma without cartilaginous differentiation. 757 5

A case of sacrococcygeal chordoma in a 9-year-old boy is presented. The symptoms at presentation were pain in both legs and sacrococcygeal region for the last two years that increased in the last four weeks irradiating mainly to the left leg. X-ray and CT scan examinations of the lumbar region revealed an expansive process in the coccygeal region with multiple calcifications and a partially eroded coccyx. There was no invasion of the retroperitoneum and regional lymph nodes. A biopsy was performed and showed cords and nests of cells with large cytoplasm, sometimes vacuolated, nuclei with moderate pleomorphism and clumped chromatin. Immunohistochemistry with avidin-biotin peroxidase technique showed positivity for CK, S-100 protein, CEA, vimentin and to EMA. Chordomas are a distinctly uncommon neoplasm in the first two decades of life, specially in the sacrococcygeal region. They have an aggressive behavior. Treatment of choice is complete resection.
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PMID:Sacrococcygeal chordoma in a 9-year-old boy. 858 26

The parachordoma is a seldom, benign tumor of uncertain histogenesis. A case in a 25-year-old man is presented. The parachordoma in the present study reacted with antibodies to GFA, S-100, NSE and vimentin, but not with antibodies to EMA, keratin and NF. Differential diagnoses are the chordoma, the extraskeletal myxoid chondrosarcoma and the subcutaneous sacrococcygeal myxopapillary ependymoma. The immunohistochemical reactions of these tumors were compared and we found that the parachordoma had an immunologically different staining pattern. The parachordoma is thus immunologically different from the chordoma, the extraskeletal myxoid chondrosarcoma and the subcutaneous sacrococcygeal myxopapillary ependymoma. We conclude that the parachordoma is an entity of its own. The immunohistochemical reactions indicate that the parachordoma is a neuroepithelial tumor with glial differentiation.
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PMID:Parachordoma of the sacrococcygeal region--a neuroepithelial tumor. 860 41

Seven cases of chordoma were studied immunohisto-chemically with antibodies directed to epithelium, nerve, mesenchyme, muscle and histiocyte. Six cases of chondrosarcoma and three cases of mucinous carcinoma of colon were studied as control. The results showed that the chordomas were positive for cyto-K 6/7, EMA 7/7, CEA 6/7, Vim 7/7, S-100 7/7, alpha-AT 7/7, Lyso 4/7, while negative for GFAP and Des in all 7 cases indicating that chordoma, just like notochord, had the potentiality of multidirectional differentiation. In contrast, none of the chondrosarcomas was positive for cyto-K, EMA or CEA, but positive for Vim and S-100 in 6 cases. Three cases of colon mucinous carcinoma were positive for epithelial antibodies, but none reacted with other antibodies. Thus, immunohistochemical studies are of help in the diagnosis and differential diagnosis of chordoma.
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PMID:[Significance of immunohistochemical study in the diagnosis and differential diagnosis of chordoma]. 869 96

Cranial and cervical chordomas can spread by para- or retropharyngeal extension up to the region of the salivary glands or the jaw and may simulate a tumor of the salivary glands or jaw. In occasional cases, because the tumors often expand slowly, months or years may pass between the first clinical symptoms and diagnosis. Diagnostic problems exist in differentiating these chordomas from pleomorphic adenoma, mucinous carcinoma, or chondrosarcoma. Ten relevant observations of typical cranial and cervical chordomas (Salivary Gland Register Hamburg 1965-1996) were analyzed more closely by pathohistological and immunohistochemical means. The exact diagnosis is based upon the evidence of blown-up, bubble-like ("physaliform") cells which contain mucus drops in a vacuolized cytoplasm and are surrounded by extensive areas of mucoid mucus. The pattern of immunohistochemistry is characterized by the multifold expression of cytokeratin, vimentin, and EMA. The differential diagnosis is discussed with reference to further types of chordoma (chondroid chordoma, dedifferentiated chordoma with spindle cell sarcomatous transformation), chondrosarcoma, pleomorphic adenoma, and mucus-producing carcinoma.
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PMID:[Cranial and cervical chordomas. A differential diagnostic problem]. 965 6


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