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)

Rhabdomyosarcoma (RMS) of the hepatobiliary system is extremely rare in adults. To our knowledge only three cases have been reported in the literature, all involving the gallbladder. The present case concerns a 40-year-old woman who presented with epigastric pain and obstructive jaundice and was found to have a fusiform, submucosal neoplasm in the common bile duct. Histologically, the tumor presented a diagnostic problem due to a predominant sclerotic growth pattern suggesting an epithelial tumor. Extensive sampling revealed a focal alveolar growth pattern with rhabdomyoblasts, although cross striations were not seen. Electron microscopy failed to demonstrate the characteristic thick myofilaments and/or Z-band material. The diagnosis was supported by strongly positive immunohistochemical staining for myoglobin and desmin; the keratin stain was negative. A subsequent supraclavicular metastasis showed the typical histology of an alveolar RMS. The histologic features of the primary tumor suggest that RMS in this location may be underrecognized due to regional similarities to either primary or metastatic infiltrating carcinomas.
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PMID:Rhabdomyosarcoma of the common bile duct in an adult. 351 54

544 malignant soft tissue tumors have been collected at the Pediatric Tumor Registry in Kiel including 300 cases of rhabdomyosarcoma (55%). In 237 of the 300 cases the diagnosis is certain. Liposarcoma and malignant fibrous histiocytoma which are typical tumors of adult age are rare in our material. Among rhabdomyosarcomas embryonal rhabdomyosarcoma (eRMS) clearly predominates accounting for almost 72% of all rhabdomyosarcomas. Differentiation of tumor cells in eRMS may vary considerably. Therefore, three groups of eRMS were distinguished and analyzed for clinico-pathologic features: 1. Primitive eRMS with less than 10 rhabdomyoblasts. 2. Intermediate eRMS with 10-50% rhabdomyoblasts. 3. Well differentiated eRMS with greater than 50% rhabdomyoblasts. By immunohistochemistry, vimentin positive cells were found in all three groups. The number of desmin positive cells depended upon the grade of differentiation. Thus, there were more desmin positive cells in well differentiated eRMS. Primitive and well differentiated eRMS were predominantly located in the head and neck area, intermediate eRMS in the abdomen. Primitive eRMS were noted in higher stages than tumors of the other two groups. Response to chemotherapy as evaluated in the 7th week of treatment was better in well differentiated eRMS. Moreover, patients of this group achieved more often complete remission. It is concluded from the present study that differentiation in eRMS may have an influence on the clinical presentation and clinical course of the disease. Therefore, this question should be investigated in more detail in a larger prospective study.
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PMID:[Rhabdomyosarcoma: morphology and cellular differentiation]. 352 25

Rhabdomyosarcoma of the conjunctiva is a rare lesion, with few previously reported cases. We have observed such a tumor occurring in a 16-year-old girl. The clinical and pathologic entities to be considered in differential diagnosis in such cases are several; in this circumstance, immunohistologic reactivity for desmin and myoglobin (two muscle-related proteins) allows a definitive interpretation of rhabdomyosarcoma to be made. This article documents the clinicopathologic features of the epibulbar rhabdomyosarcoma seen in our patient and discusses the immunocytochemical characteristics of myogenic tumors in general, in the context of ocular neoplasia.
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PMID:Embryonal rhabdomyosarcoma of the conjunctiva. A clinicopathologic and immunohistochemical study. 352 22

Fetal heavy chain skeletal myosin is normally present in fetal skeletal muscle. The study of 21 cases of rhabdomyosarcoma using specific antisera for fetal myosin, as well as for slow myosin, myoglobin, and desmin, led to positive findings in all cases with at least one antiserum. Desmin was localized in all cases and fetal myosin in 17 cases (81%), while myoglobin and slow myosin were present in 11 and eight cases, respectively. The localization of fetal myosin in rhabdomyosarcoma indicates that it is a type of oncofetal antigen. Because fetal myosin is found in small rhabdomyoblasts, it can be a useful marker in cases that usually constitute diagnostic problems.
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PMID:Fetal heavy chain skeletal myosin. An oncofetal antigen expressed by rhabdomyosarcoma. 353 37

A transplantable tumor strain designated YNnu and a cultured cell line designated YN were established from the human paratesticular rhabdomyosarcoma of a 15-year-old boy. In vitro the cells showed ultrastructural features of immature mesenchymal cells, and a few cells were labeled by antibodies to myoglobin and/or desmin. In nude mice, the cells became to contain numerous myofibrils with Z-bands, and considerable number of cells reacted with anti-myoglobin and/or anti-desmin antibodies. We conclude that spindle-shaped or small round cells are the most immature rhabdomyoblast with multiplicity for cellular differentiation.
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PMID:Embryonal rhabdomyosarcoma in nude mice and in vitro. 354 91

Rhabdomyosarcoma (RMS), a common soft tissue tumor in children, may often be difficult to distinguish from Ewing's sarcoma, neuroblastoma, and malignant lymphomas. Confirmation of the skeletal muscle origin of RMS depends partly on the demonstration of striations in tumor cells that are usually undetectable in poorly differentiated tumors. A number of tissue markers (e.g., myoglobin and desmin) are currently being used to establish the origin of RMS. However, most of these markers lack specificity and have relatively low sensitivity. We have investigated the specificity and sensitivity of anti-skeletal muscle antibody (ASMA) from patients with myasthenia gravis in the diagnosis of childhood RMS. Out of eight cases of childhood RMS (four embryonal and four alveolar) examined, two showed striations with hematoxylin and eosin and four with phosphotungstic acid hematoxylin. Myoglobin was detected in five tumors; only well-differentiated tumor cells contained myoglobin. Anti-desmin antibody and ASMA reacted with cells in all the eight tumors whether or not the tumor cells were well differentiated. Anti-skeletal muscle antibody did not react with nine lymphomas, four Ewing's sarcomas, four neuroblastomas, four osteogenic sarcomas, four lipomas, eight duct carcinomas of the breast, and eight squamous cell carcinomas of the lung. Eight leiomyomas and four leiomyosarcomas of the uterus were compared for their reactivity with anti-desmin antibody and ASMA. All the tumors stained with anti-desmin antibody and none with ASMA. The results show that ASMA is useful in the diagnosis of childhood RMS and is a more sensitive reagent than anti-myoglobin antibody. Unlike anti-desmin antibody, it can distinguish skeletal muscle tumors from smooth muscle tumors.
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PMID:Use of anti-skeletal muscle antibody from myasthenic patients in the diagnosis of childhood rhabdomyosarcomas. 355 41

Three human rhabdomyosarcoma cell lines (Rh10, Rh18, and Rh28) have been established from three independently derived xenografts. These lines have been characterized as mesenchymal in origin (reactivity to desmin and vimentin antibodies) and as expressing a human fetal muscle surface antigen recognized by monoclonal antibody 5.1 H11. Measurable levels of creatine phosphokinase have been detected in the cell lines. Rh10 and Rh28 exhibit the same chromosomal translocation and express an atypical lactate dehydrogenase isoenzyme which may be homologous to those previously reported in other tumor types. The karyotype analysis has confirmed that each cell line was derived from its respective tumor and thus provides a unique model for future investigations.
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PMID:Characterization of cell lines derived from xenografts of childhood rhabdomyosarcoma. 360 78

Titin is a major constituent protein of sarcomeric muscles and is thought to give rise to an elastic filament component underlying the myofibrillar organization. Monoclonal antibodies to titin have been characterized on normal and pathological human material and on human cell lines in culture. A positive immunocytochemical reaction was restricted to sarcomeric muscles and did not occur on visceral or vascular smooth muscles or on various nonmuscle tissues. When different tumor types were examined titin antibodies reacted solely with rhabdomyosarcomas and did not react with leiomyosarcoma or leiomyoma, or with the nonmuscle tumor types tested. In rhabdomyosarcomas a noticeably smaller population of cells were positive with antibodies to titin than with antibodies to desmin showing that individual cells within a rhabdomyosarcoma achieve different degrees of myogenic differentiation. The results reinforce the use of desmin as a marker for muscle sarcomas and show that a positive identification of rhabdomyosarcoma can be achieved by immunocytochemistry with the parallel use of desmin and titin antibodies.
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PMID:Monoclonal antibodies to titin in conjunction with antibodies to desmin separate rhabdomyosarcomas from other tumor types. 372 60

A new human embryonal rhabdomyosarcoma cell line, designated JR-1, is described that closely resembles the tumour from which it was derived. Comparative studies, by light and electron microscopy reveal morphological features such as myofibre formation, that are concordant with embryonal rhabdomyosarcoma. Immunohistological investigations using a panel of monoclonal antibodies indicate that the cell surface antigen profile of the JR-1 cell line is similar to other embryonal rhabdomyosarcomas. In addition the cell line expresses the cytoplasmic intermediate filament protein desmin, only found in cells of rhabdoid origin. Karyotyping JR-1 shows the cells to contain variable numbers of chromosomes (range 44-100). DNA flow cytometry indicates that cells have an DNA content which is approximately twice normal. The JR-1 cell line has a doubling time of 29 h in culture and, in common with several other human cell lines, produces xenografts in nude mice within 6 weeks of inoculation. With detailed studies on the original tumour and the JR-1 cell line, the latter should prove an excellent model system for investigating the biology of rhabdomyosarcoma.
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PMID:Comparative studies between a new human rhabdomyosarcoma cell line, JR-1 and its tumour of origin. 373 Feb 58

The RMZ cell line was established from a bone marrow metastasis of a human alveolar rhabdomyosarcoma. Since the beginning of the in vitro culture, RMZ cells showed differentiation-related morphological heterogeneity: actively proliferating polygonal or spindle-shaped cells were observed along with a few multinucleated myotube-like structures and giant cells, frequently multinucleated. All these cell types were still present after over 40 passages. A set of clonal derivatives has been obtained from the second in vitro subculture. All the clones showed the same morphological heterogeneity of the parental cells, but differed from one another in the degree of differentiation. Multinucleated myotube-like structures were strongly stained by anti-desmin antibody; most mononuclear cells were weakly stained. About 80% of RMZ and cloned cells were scored as desmin-positive in cytocentrifuged preparations. The expression of embryonic myosin heavy chain, specifically recognized by the monoclonal antibody BF-G6, was found in RMZ cell line and was localised in the myotube-like structures. Only a few giant cells and rare mononucleated polygonal cells were stained. The average proportion of BF-G6 positive cells in cytocentrifuged preparations was of about 6% of the total RMZ cells. In the two RMZ clones studied, the expression of embryonic myosin was correlated to the proportion of myotube-like structures: a BF-G6 positivity of 35% was found in the most differentiated one.
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PMID:RMZ: a new cell line from a human alveolar rhabdomyosarcoma. In vitro expression of embryonic myosin. 380 Dec 82


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