Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Putative human rhabdomyosarcoma (RMS) has been divided into two groups according to desmin content. Twenty-five tumors with histologic features consistent with but not necessarily sufficient to prove a diagnosis of RMS were desmin-positive. More than 95% of the tumor cells were desmin-positive, suggesting a muscle origin and supporting the diagnosis of RMS. Nine tumors for which the preferred first histologic diagnosis was also RMS were desmin-negative. Reexamination of the original histologic slides together with results from intermediate filament typing resulted in a diagnosis other than RMS for all tumors in this second group, and in several instances other tests were used to prove the correctness of the final diagnosis. The results on human material were extended to a rat model system in which RMS was induced by nickel sulfide. Again, all 24 tumors tested were desmin-positive. Vimentin was coexpressed in a varying percentage of tumor cells in RMS of human and rat origin. The results show that desmin is an excellent marker for rhabdomyosarcoma, yielding few if any false-positive or false-negative results in frozen or alcohol-fixed material.
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PMID:Desmin is a specific marker for rhabdomyosarcomas of human and rat origin. 388 Oct 39

Some human smooth muscle antibodies (SMA) react with cytoskeletal intermediate filament (IMF) antigens. The smooth muscle tissue contains two types of IMF: vimentin and desmin filaments. In this study, SMA of anti-IMF type in 52 patients' sera have been classified into anti-vimentin filament and anti-desmin filament types according to their immunofluorescence staining patterns on rat testis. This classification is based on the fact that the arterial walls of testis contains both vimentin and desmin whereas the myoid cell layer surrounding the seminiferous tubuli contains only desmin. Four out of the 52 sera gave the anti-desmin staining pattern and 40 sera showed the anti-vimentin type of staining. Thirty-two sera were further classified by using cultured human rhabdomyosarcoma (RD) cells as targets. Nine sera reacted with the intermediate filaments of the RD cells. Among these were 3 out of the 4 sera that gave the anti-desmin filament staining pattern. The anti-desmin specificity of SMA was confirmed in 1 serum by the immunoblotting technique. These results indicate that while human anti-desmin filament antibodies exist, most human SMA of anti-IMF type react with vimentin filaments.
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PMID:The detection of smooth muscle antibodies reacting with intermediate filaments of desmin type. 388 41

A case of 14-year-old girl is reported in whom an alveolar rhabdomyosarcoma occurred in the soft tissues of the left forearm 4 years prior to death. Despite extensive surgery as well as chemotherapy and radiotherapy the tumor recurred locally and produced extensive metastases including a metastasis to the brain. Cerebral metastases have not yet been reported in the literature despite extensive reports on the pathology of alveolar rhabdomyosarcoma. The morphological diagnosis of rhabdomyosarcoma was supported by the immunohistochemical demonstration of desmin, myosin, and myoglobin in the tumor cells.
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PMID:Alveolar rhabdomyosarcoma in a young female patient metastasizing to the brain. 405 Mar 50

Four embryonal rhabdomyosarcomas, one tumor diagnosed as an undifferentiated sarcoma, probably a rhabdomyosarcoma, and six different non-muscular sarcomas were investigated with antibodies specific for different intermediate filament types. The tumor cells in the rhabdomyosarcomas and the undifferentiated tumor were stained clearly by antibodies to desmin, the intermediate filament type characteristic of muscle. The staining of tumor cell by antibodies to vimentin, the intermediate filament type characteristic of certain cell types of mesenchymal origin including myoblasts, was different in these 5 cases. In one case of embryonal rhabdomyosarcoma nearly all tumor cells were stained, but in the remaining cases few or no tumor cells were positive with the vimentin antibody. In these rhabdomyosarcomas not only the large rhabdomyoblasts, but also the small undifferentiated cells were labeled by antibodies to desmin. In the latter cell type the desmin filaments were arranged typically in coils. In contrast, tumor cells in the non-muscular mesenchymal sarcomas were stained only by antibodies to vimentin but not by antibodies to desmin or prekeratin. The retention of the desmin marker characteristic of normal muscle in cases of rhabdomyosarcoma not only allowed the undifferentiated desmin-positive sarcoma to be classified as rhabdomyosarcoma but also suggests that the use of antibodies to desmin could be very helpful in the future for the diagnosis of undifferentiated rhabdomyosarcomas.
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PMID:Diagnosis of human childhood rhabdomyosarcoma of antibodies to desmin, the structural protein of muscle specific intermediate filaments. 617 91

Fifty primary gastrointestinal and breast carcinomas, four embryonal rhabdomyosarcomas, six nonmuscular mesenchymal malignant tumors and one mesothelioma have been studied to determine what type of intermediate filaments they express, using affinity purified antibodies to prekeratin, vimentin and desmin and FITC or peroxidase labeled second antibodies. The tissues were alcohol fixed and paraffin embedded before use. In all carcinoma cases the tumor cells are stained by antibodies to prekeratin, while the vimentin antibody only decorates the stroma. Prekeratin positive tumor cells are not only seen in well differentiated tumors, but also in signet ring cell carcinomas. In the case of rhabdomyosarcoma the tumor cells clearly were decorated by antibodies to desmin, while the vimentin antibody only stained very few tumor cells. In cases of nonmuscular mesenchymal tumors, the tumor cells could only be labeled by antibodies to vimentin and not by antibodies to prekeratin or desmin. In biphasic tumors like mesothelioma, different parts of the tumor were separated by antibodies to prekeratin and vimentin.
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PMID:Expression of intermediate filaments in different human epithelial and mesenchymal tumors. 619 Jan 46

A 58-year-old patient presented with poorly differentiated adenocarcinoma, probably primary in the ovary and, later in the course of her illness, with pure pleomorphic rhabdomyosarcoma. There was no evidence by light or electron microscopy of a mixture of these two tumor types. Further analysis by immunoperoxidase demonstrated scattered desmin-positive (muscle) cells in the adenocarcinoma portion of the tumor, establishing the diagnosis of malignant mesodermal mixed tumor.
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PMID:Mesodermal mixed tumor. Diagnosis by analysis of intermediate filament proteins. 630 70

A primary cerebellar rhabdomyosarcoma (RMS) in a six and a half year old boy is reported. Microscopy of the surgical material revealed lobules of closely packed cells with a high mitotic rate, pleomorphic hyperchromatic nuclei and scant cytoplasm. At their periphery, the lobules merged with rounded cells with similar nuclei but more abundant cytoplasm. These areas were surrounded by interlacing fascicles of strap cells, which were occasionally multinucleated and showed cross striations. Electron microscopy (EM) revealed the primitive nature of the closely packed cells; however, occasional intermediate size filaments were present within their cytoplasm and focal basement membrane accumulation was observed. Cells with more abundant cytoplasm had large accumulations of thick and thin filaments while strap cells showed well-developed cross striations. Immunohistochemical studies (peroxidase-antiperoxidase technique) showed vimentin in the primitive cells and desmin, myoglobin and adenosine triphosphatase as the tumor cells appeared more differentiated. Immunoreaction with antibodies against glial fibrillary acidic protein, S-100 protein and neurofilament protein were negative. Electron microscopic and immunohistochemical studies in this case demonstrated that this was an exclusively mesenchymal tumor with rhabdomyoblastic differentiation and that the pattern of differentiation follows that seen in normal myogenesis.
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PMID:Primary rhabdomyosarcoma of the cerebellum--a light, electron microscopic, and immunohistochemical study. 673 10

Three cases of soft-tissue sarcomas with the characteristic histologic features of alveolar rhabdomyosarcoma, but lacking cytoplasmic cross-striations, were studied ultrastructurally and immunohistochemically to confirm the diagnosis and evaluate the histogenesis. The results showed that it was not possible to judge the skeletal muscle derivation of the cells at the ultrastructural level. However, immunohistochemically, the results of every case were positive for desmin-the muscle type of the intermediate filament protein. The results suggest that demonstration of desmin may be a helpful adjunct tool in the diagnosis of poorly differentiated alveolar rhabdomyosarcomas.
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PMID:Alveolar rhabdomyosarcoma. Demonstration of the muscle type of intermediate filament protein, desmin, as a diagnostic aid. 676 34

Ewing's sarcoma (ES) and peripheral neuroepithelioma (PN) are closely related tumors, and it can be difficult to distinguish them from other small-round-cell tumors (SRCTs). The glycoprotein p30/32MIC2 is highly, but not exclusively, expressed in both ES and PN. Although the monoclonal antibody (Mab) HBA71, which reacts with P30/32MIC2, has been reported to be relatively specific and highly sensitive for both neoplasms, it is not readily available. Yet, Mab O13 is commercially available, and it purportedly displays the same immunostaining characteristics as HBA71. Because O13 has not been studied extensively, we immunostained 21 ES/PNs and 147 other tumors or lesions that might show SRCT-like features with O13. The results were similar to those reported for HBA71. We found O13 to be 100% sensitive for ES/PN; and, no immunostaining was noted on the SRCTs often included in the differential diagnosis of ES/PN (i.e., conventional neuroblastoma, rhabdomyosarcoma, and non-lymphoblastic lymphomas). But, O13 immunoreacted with lymphoblastic lymphomas and some other tumors and normal tissues. Nonetheless, this nonspecific reactivity should not cause diagnostic problems, if an antibody panel containing anti-desmin and anti-leukocyte common antigen is used in conjunction with O13. We conclude that, within the proper diagnostic context, strong immunoreactivity of a SRCT tumor for O13 should be considered good evidence that the tumor is ES/PN.
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PMID:Immunohistochemical profile of monoclonal antibody O13: antibody that recognizes glycoprotein p30/32MIC2 and is useful in diagnosing Ewing's sarcoma and peripheral neuroepithelioma. 779 84

Human skeletal muscle differentiation and maturation follows a precise sequence of events. To investigate whether and to what extent rhabdomyosarcoma (RMS) cells follow a comparable sequence, 29 fresh frozen specimens of RMS (14 primary and 15 relapses) were immunostained with antibodies directed against developmentally regulated myosin heavy chains (MHC), ie, fetal, fast, and slow MHC, in addition to desmin and vimentin. Four distinct patterns of expression were observed: I) RMS cells expressing exclusively vimentin and desmin (n = 7), II) in addition to expression of vimentin and desmin, a minority of neoplastic cells were immunoreactive with fetal MHC (n = 6), III) in addition to pattern II, fast MHC was expressed (n = 7), and IV) RMS cells simultaneously expressing vimentin, desmin, fetal, fast, and slow MHC (n = 9). Accordingly, the proportion of the MHC immunoreactive RMS cells increased gradually along with the four patterns of expression evolving from less than 25% up to 75% for fetal MHC, from less than 25% up to 50% for fast MHC, and up to 25% for slow MHC in the last category. Vimentin and desmin were coexpressed by almost all RMS cells. Double immunostaining revealed that comparable with the myogenic cells in the developing fetal skeletal muscle, expression of fetal MHC could be demonstrated in the same neoplastic cells either in conjunction with fast or slow MHC. In contrast, only in RMS, slow MHC expression in conjunction with fast MHC could be observed in the neoplastic cells. Neither the shape or size of neoplastic RMS cells, nor the histopathological types, nor tumor localization were related to the expression pattern of developmentally regulated MHC (fetal, fast, and slow MHC). These results confirm the commitment of the RMS cells to the myogenic pathway and demonstrate a restricted and aberrant differentiation pattern of the neoplastic cells in RMS compared with normal myogenesis, independent of histopathological types of RMS.
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PMID:Expression of developmentally regulated muscle proteins in rhabdomyosarcomas. 752 32


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