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)

Primary intracranial rhabdomyosarcoma (RMS) is a rare tumor in infancy and childhood that is found in various locations in the central nervous system. The clinical course worsens rapidly, and the final outcome is poor, with a median survival time of 8-10 months. Invasion of the meninges, spontaneous intratumoral bleeding, spinal leptomeningeal CSF spreading of tumor cells, and early recurrence of the mass are the distinctive features of RMS. Diagnosis of RMS may be missed: immunohistochemical staining using specific markers (myoglobin, myosin, desmin, vimentin, enolase), along with ultrastructural studies, provide the basis for making the final diagnosis. Treatment of RMS includes surgical excision, craniospinal radiation therapy, and chemotherapy. We report two cases of primary RMS in the CNS located in the posterior fossa and frontotemporal area. Both children underwent total surgical removal of the mass. Early recurrence of the tumor mass was noticed in both patients 2 months after surgery. Both children died shortly thereafter.
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PMID:Primary intracranial rhabdomyosarcoma: report of two cases. 279 Aug 36

A retrospective immunohistological analysis of 64 rhabdomyosarcomas in children was performed using antibodies against desmin and in 35 cases against myoglobin. In addition a group of 12 undifferentiated tumours in which the differential diagnosis included rhabdomyosarcomas was studied. Rhabdomyosarcomas were desmin positive in 57 cases (89%), 28 cases of which showed positivity of undifferentiated small cells (44%). Myoglobin was positive in 23 cases (66%), but only one case showed positivity of undifferentiated small cells. The results show the limited use of myoglobin in the diagnosis of rhabdomyosarcoma, especially of cases with a low degree of differentiation. Three out of 12 undifferentiated tumours were desmin positive and were reclassified as rhabdomyosarcomas. In 49 rhabdomyosarcomas the investigation was complemented by the demonstration of vimentin. Vimentin was shown to be present in 27 cases in tumour cells (55%). Undifferentiated cells were positive in 26 tumours (53%) and rhabdomyoblasts reacted in 9 cases (18%). Coexpression of vimentin and desmin in some cases reflects a situation in rhabdomyosarcomas that aberrantly mimics skeletal muscle embryogenesis. In three cases desmin and vimentin positive globular inclusions were observed. It is suggested that their formation is related to dystrophic changes of contractile and cytoskeletal filaments. From the diagnostic point of view a high percentage of desmin positive cases makes desmin a successful marker for rhabdomyoblastic tumours. It is pointed out, however, that even immunohistochemistry may not contribute to solving the problem of undifferentiated tumours and that each case must be evaluated comprehensively.
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PMID:Rhabdomyosarcoma in childhood. An immunohistological analysis with myoglobin, desmin and vimentin. 279 21

Three distinct subpopulations (A, B, C) derived from a dimethylbenzanthracene-induced rat rhabdomyosarcoma were established as permanent cell lines. Although the clonal nature of each of these subpopulations was confirmed by repeated recloning procedures, a striking intraclonal phenotypic heterogeneity was observed. By means of immunofluorescence microscopy and transmission electron microscopy, it could be shown that these subpopulations closely recapitulate stages of embryonic rhabdomyogenesis both in vitro and in vivo, but differ in their particular range of maximum differentiation. Embryonic rhabdomyogenesis is imitated most perfectly by subpopulation C, in which multinuclear myotubes are formed in vitro by fusion of mononuclear cells, and alpha-sarcomeric actin is expressed in the multinuclear cells and in a few mononuclear cells. After retransplantation in vivo, subpopulation C further proceeds in fine structural differentiation, now exhibiting myofibrils with a sarcomeric organization in the myotube-like giant cells. The cells of subpopulation B do not exceed the stage of mononuclear desmin-positive cells in vitro, but synthesize thin and thick myofilaments after retransplantation in vivo. The cells of subpopulation A recapitulate embryonic rhabdomyogenesis least successfully being confined to the stage of mononuclear desmin-positive cells. Thus, the coexistence of diverse subpopulations and the cellular maturation within these subpopulations together contribute to the phenotypic heterogeneity of rhabdomyosarcomas.
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PMID:The intraclonal and interclonal phenotypic heterogeneity in a rhabdomyosarcoma cell line with abortive imitation of embryonic myogenesis. 290 May 72

A new human rhabdomyosarcoma cell line (HX170c) has been established from a paratesticular embryonal tumour in a 5-year-old male. The cells grew as an adherent monolayer with a doubling time of 32 h and showed pleomorphic features. Intermediate filament analysis revealed the line to be mesenchymal in origin (reactivity to vimentin and desmin antibodies). The line was tumorigenic in nude mice, possessed elevated levels of creatine phosphokinase (mainly of the MM isoenzyme form) and had a near diploid mean chromosome number of 50. In vitro cell cloning determinations gave colony forming efficiencies of 0.01% in soft agar and 24% in a monolayer anchorage-dependent assay. Radiosensitivity determinations using a monolayer clonogenic assay with feeder layer support showed the cells to be among the more radiosensitive human tumour cell types (surviving fraction at 2 Gy of 0.26) that have been investigated. Furthermore, experiments utilising continuous low dose rate radiation at 3.2 cGy min-1, showed that, under these experimental conditions, the cells possessed only a very low capacity to recover from radiation-induced damage (dose reduction factor at 1% cell survival of 1.07 for 150 versus 3.2 cGy min-1). As other human tumour cells of an embryonal cell origin (e.g. neuroblastoma and germ cell tumours of the testis) have also been shown to be radiosensitive it appears that sensitivity to radiation may be a common property of this group of tumours.
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PMID:High intrinsic radiosensitivity of a newly established and characterised human embryonal rhabdomyosarcoma cell line. 293 Jun 81

We recently identified three cases of primitive rhabdomyosarcoma (PRMS) presenting with diffuse bone marrow infiltration but inconspicuous soft tissue primaries, referred to The Johns Hopkins Hospital (JHH) as acute leukemia. In each case, the diagnosis of rhabdomyosarcoma was established using immunohistochemical staining and electron microscopy. Ultrastructural examination of tumor cells showed a feltwork of thin filaments, discontinuous basal lamina, glycogen, and primitive cell junctions without cell processes or neurosecretory granules. This presentation of PRMS may be more common than recognized, since it can be readily misdiagnosed as a hematopoietic tumor. While positive staining for muscle-specific actin, desmin, myoglobin, or other markers of skeletal muscle differentiation may be diagnostic, negative staining is inconclusive, requiring recognition of the "minimal" ultrastructural findings of primitive rhabdomyosarcomas.
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PMID:Primitive rhabdomyosarcoma presenting with diffuse bone marrow involvement: an immunohistochemical and ultrastructural study. 297 40

We have investigated 56 histologic and 13 cytologic specimens of malignant round cell tumors of childhood. The immunohistological detection of intermediate filament polypeptides, neuron specific enolase (NSE) as well as leukocyte common antigen (LCA) allows the histological classification of such tumors. Neuroblastomas demonstrate a positive reaction with antibodies to neurofilaments and NSE independent of differentiation. Rhabdomyosarcomas could be labeled by the desmin antibody, while Ewing sarcomas, malignant lymphomas as well as nonmuscular sarcomas only express vimentin. In nephroblastomas the intermediate filament specific antibodies reveal expression of keratin and vimentin in blastema cells, while tubules are only labeled by the keratin antibody. In undifferentiated nephroblastomas, which lack formation of tubules blastema cells are keratin negative and vimentin positive. Thus antibodies to intermediate filaments, LCA and NSE seem to be useful tools to distinguish the so called "round cell tumors" of childhood.
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PMID:[Significance of immunohistologic methods in the differential diagnosis of solid tumors in childhood]. 301 2

Malignant fibrous histiocytomas (MFH) belong to the most frequent soft tissue tumours in adults and have to be discriminated from other tumours with similar morphology. Various tumour markers aid the differential diagnosis. Twenty cases of MFH were studied immunohistochemically using antibodies to vimentin, TPA, desmin, lysozyme, alpha 1-antitrypsin, alpha 1-antichymotrypsin, S-100 protein, neurone-specific enolase (NSE), laminin, fibronectin and ferritin. Vimentin and lysozyme were found in the tumour cells of all, alpha 1-antitrypsin of 18, alpha 1-antichymotrypsin of 19, fibronectin of 16 and ferritin of 12 cases. Antibodies of TPA, desmin, S-100 protein, NSE and laminin did not reveal positive immunoreactivity. Exclusion of spindle-cell carcinoma can be made by positive vimentin and negative TPA reactivity, of melanoma by negative S-100 reactivity, and of leio- and rhabdomyosarcoma by lack of desmin immunoreactivity. Schwannomas contain S-100 protein, but lack lysozyme, alpha 1-antitrypsin, alpha 1-antichymotrypsin and fibronectin. Pleomorphic liposarcomas cannot be distinguished from MFH on the basis of immunohistochemical staining. Vimentin, alpha 1-antitrypsin, alpha 1-antichymotrypsin and fibronectin can, therefore, be regarded as useful markers in the differential diagnosis of MFH.
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PMID:[Immunohistochemical studies in the differential diagnosis of malignant fibrous histiocytoma]. 302 16

Pleomorphic rhabdomyosarcoma in adults over 30 years of age was a diagnosis frequently made in the 1960s and 1970s. Since the general acceptance of malignant fibrous histiocytoma (MFH) as a tumor entity at the end of the 1970s, however, it has become a very rare tumor in adults. Therefore, 21 cases originally diagnosed on the basis of histology and clinical data as pleomorphic rhabdomyosarcoma in the 1960s and 1970s were reexamined immunohistochemically. Other types of pleomorphic sarcomas involved in the differential diagnosis were also studied. Specific antibodies against vimentin, desmin, creatine kinase subunit M, skeletal muscle actin and myosin, and myoglobin, and the avidin-biotin-peroxidase complex technique were used. The immunohistochemical findings indicate that rhabdomyosarcoma occurs only rarely in adults over 30 years of age and that the majority of the tumors have to be reclassified as MFH or leiomyosarcoma. On the other hand, several pleomorphic sarcomas were found to be diagnosed incorrectly as MFH or liposarcoma by routine histologic stains and electron microscopy. The revised diagnosis was pleomorphic rhabdomyosarcoma for one case and pleomorphic leiomyosarcoma for the other cases. Thus, this study clearly shows the usefulness of immunohistochemistry as a technique in the diagnosis of pleomorphic sarcomas in adults.
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PMID:Pleomorphic rhabdomyosarcoma in adults: immunohistochemistry as a tool for its diagnosis. 302 31

Esthesioneuroblastoma (EN), a malignant neuroblastic tumor arising in the superior portion of the nasal cavity, shares histologic similarities with a number of primary malignant tumors that arise in this region, including rhabdomyosarcoma, lymphoepithelioma, and lymphoma. To establish an antigenic profile of EN as an aid in the differential diagnosis of these histologically similar nasal tumors, immunostaining was performed for the following intermediate filaments: keratin, neurofilament, glial fibrillary acidic protein, and desmin; neuron-specific enolase (NSE), S-100 protein, chromogranin, human common leukocyte antigen (HLE), epithelial membrane antigen (EMA), myoglobin, and carcinoembryonic antigen (CEA) on 21 primary nasal tumors: eight EN, five lymphoepitheliomas, two small cell carcinomas, three lymphomas, and three rhabdomyosarcomas. Keratin and CEA stained only the carcinomas (6/7+, 4/7+), respectively; desmin and myoglobin only rhabdomyosarcoma (3/3+, 1/3+); and HLE only lymphomas (3/3+). Chromogranin and neurofilament staining occurred exclusively in one case each of EN. S-100 and NSE commonly stained EN (5/8+, 6/8+), but carcinomas (1/7+, 2/7+) and rhabdomyosarcomas (1/3+, 3/3+) were also positive. Despite the apparent nonspecificity of NSE and S-100, an antigenic profile of positive NSE of S-100 staining with negative epithelial, muscle, and lymphoid antigens uniquely identified six of eight EN. Chromogranin and neurofilament positivity was further evidence for EN in two cases. This antigenic profile is a helpful adjunct in the diagnosis of EN and other primary malignant nasal tumors.
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PMID:Esthesioneuroblastoma. Intermediate filaments, neuroendocrine, and tissue-specific antigens. 303 34

A correlated cytologic and histologic study of seven cases of embryonal rhabdomyosarcoma is presented. The diagnosis of rhabdomyosarcoma was established by light and electron microscopy and immunohistochemistry of the operative specimens. The cytologic appearance of the smears corresponded well with the histopathologic findings. Cytologically, two main cell types were distinguished: a predominant primitive, small round cell with scant cytoplasm and a large cell with an abundant cytoplasm, sometimes tadpole- or ribbon-shaped. The tumor cells were often enclosed in a background of mucosubstances. The lack of cytologic features proving rhabdomyoblastic differentiation, such as cross-striation, necessitates the use of additional methods in the cytologic diagnosis of embryonal rhabdomyosarcoma. The value of the embedding technique for ultrastructural analysis and immunohistochemistry in the demonstration of desmin in aspirates is emphasized in the diagnosis of embryonal rhabdomyosarcoma.
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PMID:Cytology of embryonal rhabdomyosarcoma: a cytologic, light microscopic, electron microscopic, and immunohistochemical study of seven cases. 307 10


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