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 78-year-old man was admitted to our hospital with cough and left anterior chest pain. Chest X-ray examination on admission revealed a tumor shadow in the left upper lobe. Malignant tumor cells were observed on histopathological examination of a specimen obtained by bronchoscopic biopsy. Radiotherapy was performed but was not effective, and the patient died of respiratory failure 4 months after admission. Autopsy revealed a 15 cm diameter tumor with marked local invasion tendency without distant metastasis. Microscopically, the tumor consisted partly of squamous cell carcinomas, and partly of fibrosarcomas, composed of spindle cells and osteo-chondrosarcoma. The tumor was therefore diagnosed as carcinosarcoma. Immunohistochemical examination showed positive keratin and EMA staining only in the squamous cell carcinoma component of the tumor.
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PMID:[A case of carcinosarcoma of the lung]. 162 91

True malignant mixed tumor (TMMT) of salivary glands, with both carcinomatous and sarcomatous components, is exceedingly rare. We offer a case of TMMT in a 79-yr-old man, which may represent the first report example of this unusual neoplasm arising in the tongue. The carcinomatous component was mainly of solid basaloid carcinoma with focal glandular differentiation, while the sarcomatous component was composed of pleomorphic elements such as chondrosarcoma, myxosarcoma and fibrosarcoma. Carcinoma cells at the periphery of solid nests occasionally merged into these sarcomatous elements. Immunohistochemically, basaloid carcinoma cells showed positive reaction for both low molecular weight cytokeratin and S-100 protein, whereas carcinoma cells lining ductal spaces were positive for a wide spectrum of keratin and EMA. The sarcomatous elements revealed the presence of vimentin and S-100 protein. Ultrastructurally, basal lamina-like material and/or mucoid precipitates often accumulated separating the tumor cells from each other singly or into a few cell group. Some sarcomatous cells assumed the myoepithelial features, such as the presence of microfilament bundles with dense bodies and pinocytotic vesicles along the cell periphery. These findings may indicate that TMMT shares a common histogenesis with pleomorphic adenoma.
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PMID:Ultrastructural and immunohistochemical observations of a true malignant mixed tumor (carcinosarcoma) of the tongue. 169 22

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

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

We report two cases of a previously unrecognized neoplasm, each characterized by prominent lobular configuration in the subcutaneous tissue. Within the neoplasms were distinctive garland-shaped structures composed of glial fibrillary acidic protein (GFAP) positive cells with indistinct borders, encased in concentric loops of fine collage fibers. In some areas, the neoplastic cells were distributed in small lacunae. The extracellular space between the collagenous tissue and the cells was filled with copious myxoid matrix. One of the neoplasms also demonstrated areas with spindle cells which resembled leiomyoma. Immunohistochemistry was negative for smooth muscle actin (1A4), muscle actin (HHF35), S-100 protein, desmin, cytokeratin, KP1, and epithelial membrane antigen (EMA.) Currently, both patients are free of recurrence or metastasis 2 and 4 years after primary surgical excision. The neoplasms, which we term benign polymorphous mesenchymal tumor of soft parts (BPMT), should be distinguished from ossifying fibromyxoid tumor of soft parts, extraskeletal mesenchymal chondrosarcoma, neoplasms arising in ectopic breast tissue and mixed tumor of the skin.
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PMID:Benign polymorphous mesenchymal tumor (mesenchymal hamartoma) of soft parts. Report of two cases. 918 15

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

Chondrosarcomas are malignant cartilage-forming tumors that represent the second most common malignant solid tumor of bone. These biologically poorly understood neoplasms vary considerably in clinical presentation and biologic behavior. Chemotherapy and radiation therapy are generally ineffective. Here we describe the establishment and characterization of a new human chondrosarcoma cell line named ch-2879, and we compare the cell line with its tumor of origin. The cell line was established from a recurrent grade 3 chondrosarcoma of the chest wall and characterized by growth kinetics and morphologic studies. Immunocytochemistry and RT-PCR were performed to examine the expression of cartilage-specific phenotypes. Genetic characterization was performed using cytogenetics, fluorescence in situ hybridization, flow cytometry, and molecular techniques for analysis of the genes implicated in cell cycle control, amplification of MDM2, CDK4, and Cyclin D1, and mutations in the p53 gene. ch-2879 cells were subcultured for more than 80 passages. They expressed vimentin, HNK-1, HBA-71, Ki-67, cyclin D1, Fli-1, S-100, p21, p27, and p53 and were negative for cytokeratin, EMA, p14, p16, MDM2, Rb, and c-erb-b2 antigens. Cytogenetically the recurrent tumor showed a hyperhaploid karyotype with clonal numerical and structural abnormalities. The sole structural abnormality was a chromosome derivative of a t(1;21) translocation. The cell line at passage 3 showed two populations: the hyperhaploid and an exactly duplicated, hypotriploid population. After the 18th passage, only the hypotriploid population was present. The cells expressed collagen 2. Molecular comparison of the primary and recurrent tumor evidenced an in vivo molecular change consisting of a deletion of 9p21 genes in the recurrence, probably caused by a selection process. Because of its gene expression profile, including expression of genes implicated in chondrogenesis in uncoated plastic dishes, this cell line may prove useful for cellular and molecular studies as well as studies of chondrosarcoma characterization and treatment.
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PMID:Establishment and characterization of a continuous human chondrosarcoma cell line, ch-2879: comparative histologic and genetic studies with its tumor of origin. 1280 23

Chordomas are rare malignant bone tumours considered to arise from notochordal remnants that persist in the axial skeleton. Although their morphology can resemble that of a carcinoma, chondrosarcoma or malignant melanoma, the axial location and their well-defined immunophenotype, including expression of cytokeratins (CK7/20/8/18/19) and S100, generally allow the diagnosis to be made with confidence once the possibility is considered. In contrast, making a robust diagnosis of an extra-axial chordoma has been difficult in the absence of specific markers for chordomas. We have recently shown in gene expression microarray and immunohistochemistry studies that brachyury, a transcription factor crucial for notochordal development, is a specific and sensitive maker for chordomas. We now present a case of an intracortical tibial tumour, with detailed report of the imaging, and morphological features consistent with a chordoma, where notochordal differentiation was demonstrated with an antibody to brachyury. The tumour cells were also positive for cytokeratins, including CK19, and S100, CEA, EMA and HMBE1, findings which support the diagnosis of chordoma. Brachyury can be employed as a marker of notochordal differentiation and help identify confidently, for the first time, extra-axial bone and soft tissue chordomas, and tumours which may show focal notochordal differentiation.
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PMID:Diagnosing an extra-axial chordoma of the proximal tibia with the help of brachyury, a molecule required for notochordal differentiation. 1681 May 40


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