Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1261473 (sarcoma)
25,952 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clear cell sarcoma (CCS) is an uncommon, clinicopathologically distinct neoplasm that typically arises in association with tendons and aponeuroses. It shares several histologic and ultrastructural features with malignant melanoma. Clear cell sarcoma occasionally may be confused with other tumors of soft tissue that have a predominantly epithelioid appearance, including epithelioid leiomyosarcoma, epithelioid neurofibrosarcoma, synovial sarcoma, and epithelioid sarcoma. To assess the potential contribution of immunohistochemistry to this differential diagnosis, a panel of immunostains was applied to examples of each of these neoplasms. All six CCSs contained vimentin, and five were reactive with the melanoma-specific monoclonal antibody HMB-45. In addition, five CCSs expressed neuron-specific enolase, four cases displayed S100 protein, and four examples contained LN3 antigen. Synaptophysin and Leu-7 antigen were present in one case each. Cytokeratin, epithelial membrane antigen, carcinoembryonic antigen, desmin, muscle-specific actin, and leukocyte common antigen were invariably absent. No other primary epithelioid neoplasm of soft tissue reacted with HMB-45. Clear cell sarcoma could be separated from epithelioid leiomyosarcoma by the presence of desmin and muscle-specific actin in the latter neoplasm. Similarly, both synovial sarcoma and epithelioid sarcoma differed from CCS by their expression of cytokeratin and epithelial membrane antigen. Clear cell sarcoma and malignant melanoma were immunohistochemically indistinguishable, supporting the concept that they share a common pattern of differentiation.
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PMID:Clear cell sarcoma. An immunohistochemical analysis of six cases and comparison with other epithelioid neoplasms of soft tissue. 252 Dec 88

The expression of intermediate filaments (IFs) in 34 malignant fibrous histiocytomas (MFHs) was studied immunohistochemically and ultrastructurally. Using the avidin-biotin-peroxidase method, positive reactions were detected as follows: for desmin in 12 tumors, for neurofilament in two tumors, for cytokeratin in one tumor, and for vimentin in 30 tumors. Desmin immunoreactivity was found in tumors of all four histologic subtypes and cytokeratin immunoreactivity was found in one tumor of the myxoid type. Because of the cross-reactivity of anti-neurofilament antibody with reactive histiocytes, the immunoreactivity for neurofilament seemed to be non-specific. Ultrastructurally, five of 13 tumors studied contained some tumor cells showing myofibroblastic or smooth muscle cell differentiation. A few tumor cells in one cytokeratin-positive tumor had tonofilaments in their cytoplasm. Desmin expression in some MFHs seemed to be due to myofibroblastic or smooth muscle cell differentiation of some tumor cells. Cytokeratin expression seemed to indicate epithelial differentiation in some MFHs. This varied expression of IFs in MFHs may reflect the heterogeneous nature of MFHs, and suggests that MFHs represent the final stages of dedifferentiation of several different types of sarcomas or, alternatively, represent forms of poorly differentiated sarcoma with the potential of developing into more differentiated sarcomas of heterogeneous origin.
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PMID:Expression of intermediate filaments in malignant fibrous histiocytomas. 255 Mar 49

The histogenesis of clear cell sarcoma was investigated by immunohistochemical examination of five tumors (two melanotic and three amelanotic) and electron microscopic examination of two of these tumors (one melanotic and one amelanotic). Melanin production was observed histologically in two of the tumors. The cytoplasm of cells in both types of tumor contained various numbers of melanosomes. Melanoma-specific antibody (HMB-45), anti-S-100 protein, and anti-vimentin antibodies gave positive reactions in four tumors, while all tumors showed Leu-7 immunoreactivity. No cytokeratin or epithelial membrane antigen (EMA) was detected immunohistochemically in any tumor. The immunoreactivity of this type of tumor with HMB-45 antibody strongly suggests melanocytic differentiation rather than schwannian or synovial differentiation. The reaction of the cells of one tumor with only Leu-7 indicates the existence of undifferentiated clear cell sarcoma of neuroectodermal origin that does not show definite melanocytic differentiation.
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PMID:Clear cell sarcoma. An immunohistochemical and ultrastructural study. 260 58

We report five urothelial carcinomas (one primary and four metastatic) with pseudosarcomatous stromal reaction. The exuberant stromal reaction led to a histologic misdiagnosis in three of the original small biopsy specimens. The differential diagnoses of primary spindle cell lesions of urinary tract include spindle cell carcinoma, carcinosarcoma, sarcoma, and benign pseudosarcomatous lesions. The distinction between those conditions and urothelial carcinomas with pseudosarcomatous stromal reaction is obviously of great clinical significance. In an initial small biopsy specimen, it may be difficult to make such a distinction. Immunostaining for cytokeratin and examination of more material should be performed. In our study of metastatic urothelial carcinomas with pseudosarcomatous stromal reaction, clinicopathologic correlations along with immunostaining for keratin proved to be useful. The stromal inductive capability of transplanted urothelium, which has been established in animal experiments, has occasionally also been reported in humans. The malignant urothelium in humans similarly appears to be capable of inducing stromal reaction.
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PMID:Pseudosarcomatous stromal reaction in primary and metastatic urothelial carcinoma. A source of diagnostic difficulty. 276 24

A biphasic synovial sarcoma occurring in the wall of a pathologically thickened femoral vein was diagnosed in a 34-year-old woman. This sarcoma was resected together with removal of a thrombus. A local recurrence was seen 5 years later, but the patient is alive and well 11 years after the first operation. Immunohistochemical evaluation revealed cytokeratin and epithelial membrane antigen in a portion of the tumor cells, as are typically seen in synovial sarcoma. This case shows that so-called synovial sarcoma may arise in places remote from any joints and that its origin may not be a synovial or perisynovial tissue in all cases. More likely, synovial sarcomas develop from transformed mesenchymal cells, some of which have acquired the epithelial phenotype.
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PMID:Intravascular synovial sarcoma. 282 Aug 61

Nude mice xenotransplants have been performed on human primary sarcomas of bone and soft tissues in order to delve into the cell heterogeneity of these neoplasms. Particular emphasis has been given to the group of small round blue cell sarcomas (Ewing's sarcomas and peripheral neuroectodermal tumors). Out of 31 xenotransplanted sarcomas, 16 cases have grown positively, and many of them continue to be transferred into nude mice on a regular time basis, being presently considered as fully established nude lines. Here we report the results of such a system, which has been followed with optical, electron microscopical, immunohistochemical and cytogenetic techniques. Osteosarcomas make up the group with the highest number of positivities taken (6 out of 9 transplanted cases). Diversity in growth rate, positive tumors and morphology are taken into consideration. Epithelial foci were seen in one of the transplanted neoplasms. Malignant fibrous histiocytoma and other mesenchymal sarcomas of bone and soft tissues are reviewed. Changes in immunohistochemical reactivity (alpha-1AT and alpha-1AQT) were observed in the transplanted neoplasms. Cytogenetic analysis performed on an undifferentiated (primitive) soft-tissue sarcoma provided clues to its synovial origin. Analysis of Ewing's sarcoma performed on nude mice transplanted tumors shows heterogeneous immunohistochemical response, with enhancement of cytokeratin positive cells, not previously seen in the primary neoplasms.
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PMID:Value of nude mice xenografts in the expression of cell heterogeneity of human sarcomas of bone and soft tissue. 285 74

The clinical and pathologic findings of 16 examples of a distinctive stromal tumor of the breast designated as "myofibroblastoma" are reported. Eleven of the 16 patients were men, and the average age at presentation was 63 years. Fourteen were treated by local excision and two by simple mastectomy. None of the lesions recurred or metastasized. The tumors were grossly nodular and well-demarcated from the surrounding mammary tissue. Ducts and lobules were not engulfed by the neoplasm. Microscopically, the lesions were formed by uniform, slender, bipolar spindle cells haphazardly arranged in fascicular clusters separated by broad bands of hyalinized collagen. Ultrastructural examination of four lesions identified a predominance of myofibroblasts. Immunoreactivity for S-100 protein and cytokeratin was absent in the 10 tumors examined, but desmin immunoreactivity was focally present in three lesions. The differential diagnosis of myofibroblastoma includes reactive processes and benign neoplasms such as nodular and proliferative fascititis, fibromatosis, spindle-cell lipoma, neurofibroma, neurilemmoma, and leiomyoma. Malignant neoplasms such as stromal sarcoma, malignant fibrous histiocytoma, and spindle-cell or metaplastic carcinoma should not be confused with a myofibroblastoma. The clinical significance of this entity lies primarily in its recognition as a distinctive benign neoplasm.
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PMID:Myofibroblastoma of the breast. Sixteen cases of a distinctive benign mesenchymal tumor. 1788 May 43

Epithelioid sarcoma (ES) and epithelioid hemangioendothelioma (EH) both occur preferentially in the soft tissues, and may be confused with one another microscopically. We compared 8 examples of each tumor immunohistochemically, using formalin-fixed tissue, the ABC method, unconjugated Ulex europaeus I agglutinin (UEA), rabbit antibody to UEA, and monoclonal antibodies to epithelial membrane antigen (EMA), cytokeratin (CK), factor VIII-related antigen (FVIIIRAG), and blood group isoantigens A, B, and H (BGI). Six of 8 cases of ES, and 7 of 8 epithelioid hemangioendotheliomas bound UEA; similarly, 6 of 8 ES cases were reactive for BGI, as were 4 of 8 examples of EH. All epithelioid sarcomas were positive for CK, and 7 displayed EMA, whereas these antigens were lacking in EH. Conversely, 5 of 8 cases of EH contained FVIIIRAG, which was absent in all examples of ES. These findings underscore the nonspecificity of UEA-binding and BGI-expression as markers of endothelial differentiation. Moreover, they suggest that sole reliance upon these immunohistologic reactants for the identification of vascular tumors may result in diagnostic error. Inasmuch as ES and EH differ in biological behavior, such a mistake would be significant. Thus, we advocate the inclusion of immunostains for EMA, CK, and FVIIIRAG in the evaluation of histologically-similar cases of epithelioid sarcoma and epithelioid hemangioendothelioma.
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PMID:Epithelioid sarcoma and epithelioid hemangioendothelioma: an immunocytochemical and lectin-histochemical comparison. 310 Dec 80

Six cases of epithelioid sarcoma were studied by conventional light microscopy and immunohistochemistry. The six cases account for 1.4% of the 417 cases of soft tissue sarcoma collected at the Paediatric Tumor Registry, Kiel. The average age of the five male and one female patient was 10.8 years (median: 13 years). Particular clinical findings included the location of the tumours; three were found in the pelvis, two in the head and neck, and one in the hand. Four patients are living without disease, and one patient died of disease three years after diagnosis. Histologically, four of the six tumours revealed multinucleated giant cells. Immunohistochemically using a panel of mono- and polyclonal antibodies all cases stained positively for vimentin, cytokeratin, epithelial membrane antigen (EMA), and human milk fat globulin (HMFG-2). Five cases were positive for neuron specific enolase (NSE), and three stained positively for protein S-100. A positive reaction for alpha-1-antichymotrypsin was noted in two cases. These immunohistochemical findings attest to the multidirectional differentiating capabilities of epithelioid sarcoma and support the concept of derivation from a multipotent mesenchymal stem cell.
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PMID:Epithelioid sarcoma in children and adolescents. An immunohistochemical study. 310 23

In order to define the immunohistochemical features of spindle cell proliferations of the urinary tract, we studied 18 lesions of this type with a panel of antibodies to epithelial and mesenchymal determinants. Histologically, 13 cases showed features that were not conclusively those of either spindle cell carcinoma or sarcoma, three contained overtly epithelial areas, and two were classified as "postoperative spindle cell nodules" (PSCN). Nine tumors in the indeterminate group and three with focally overt carcinomatous growth stained for cytokeratin and epithelial membrane antigen (EMA) and were classified as carcinomas. Eleven of these coexpressed vimentin, but desmin and muscle-specific actin were undetectable. The four remaining indeterminate neoplasms expressed desmin and muscle-specific actin as well as vimentin, but they lacked epithelial markers; these were categorized as leiomyosarcomas. Both PSCN were reactive for cytokeratin with each of three separate monoclonal antibody preparations. Desmin, actin, and vimentin were also observed in such lesions, but EMA was not. Because spindle cell proliferations may be difficult to interpret in routinely stained tissue sections, the additional information provided by immunohistochemical studies is helpful in establishing a diagnosis and separating biologically disparate lesions.
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PMID:Spindle-cell proliferations of the urinary tract. An immunohistochemical study. 328 97


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