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)

Recent studies have suggested that rhabdomyosarcoma can be divided into favourable and unfavourable histology groups. Those subtypes comprising the unfavourable histology group are alveolar, monomorphous round cell and anaplastic rhabdomyosarcoma, with a favourable histology group of embryonal rhabdomyosarcomas. An immunohistological study was undertaken on 59 childhood rhabdomyosarcomas, using antisera to keratin, S100 protein, vimentin, desmin, myoglobin, and troponin T. Our results suggest that desmin is the single most useful antibody in the diagnosis of rhabdomyosarcoma and was expressed in all our cases. The expression of troponin T in the majority of embryonal rhabdomyosarcomas but not in the other histological types has prognostic implications.
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PMID:Rhabdomyosarcoma in children: a histological and immunohistochemical study of 59 cases. 247 63

Carcinosarcomas of the prostate gland are exceedingly rare, and previous reports exist on only seven of these neoplasms. The authors studied two such tumors, which occurred in 63- and 69-year-old patients. One of them had osseous metastases develop, which were treated unsuccessfully by irradiation and diethylstilbestrol therapy. The other patient is free of disease 15 months after radical prostatectomy. Both tumors contained an intimate mixture of carcinoma and sarcoma; patient 1 displayed foci of chondrosarcoma, osteosarcoma, and leiomyosarcoma, whereas patient 2 exhibited areas of chondrosarcoma, osteosarcoma, rhabdomyosarcoma, and angiosarcoma. The phenotypic nature of these tissues was confirmed by immunohistochemical studies, showing reactivity for vimentin, S-100 protein, desmin, actin, myoglobin, or Ulex europaeus I agglutinin. Conversely, the sarcomatous components lacked prostate-specific antigen, epithelial membrane antigen, and cytokeratin, whereas carcinomatous elements expressed these three markers. The authors' data support the existence of true carcinosarcomas of the prostate, that is, malignant neoplasms with conjoint epithelial and mesenchymal differentiation. The question of whether prostatic carcinosarcoma is an entity that is totally distinct from sarcomatoid or metaplastic carcinoma remains problematic.
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PMID:Prostatic carcinosarcomas. Clinical, histologic, and immunohistochemical data on two cases, with a review of the literature. 247 43

Histopathologic and immunohistochemical study of 120 cases of soft tissue malignancy diagnosed as rhabdomyosarcoma is reported. There were 65 males and 55 females including 62 adults (greater than or equal to 15 years) and 58 children (less than 15 years). In the adult group, the tumor was common in the limbs and the trunk, whereas in the children group, the urogenital system and regions in the head and neck. Histologically, they were divided into three types: 1. embryonic type (65 cases), 2. alveolar type (28 cases) and 3. polymorphic type (27 cases). Fifty-two cases of proved rhabdomyosarcoma and 21 cases of misdiagnosed rhabdomyosarcoma were stained immunohistochemically with myoglobin antiserum. Forty seven of those 52 cases were positive to various degrees, whereas all the misdiagnosed cases were negative. It was evident that these doubted cases were misdiagnosed in previous histopathologic interpretation. Our study suggests that the immunohistochemical myoglobin stain be an important method for the differentiation of rhabdomyosarcoma. The cause of histopathologic misdiagnosis and the significance of myoglobin antiserum in the histopathologic diagnosis and differential diagnosis of this tumor are discussed.
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PMID:[Histopathologic and immunohistochemical study of rhabdomyosarcoma]. 261 28

In a review of a national series of malignant tumors in middle-aged and elderly individuals (over 40 years of age), in all 107 cases primarily diagnosed and reported to the Swedish Cancer Registry as rhabdomyosarcomas during the period 1972-1981, 4 cases were accepted as botryoid, embryonal or alveolar rhabdomyosarcoma, using light-microscopic criteria for the diagnosis. An electron-microscopic and immunohistochemical analysis was performed on the 4 cases along with 7 cases of botryoid, embryonal and alveolar rhabdomyosarcoma in patients of over 40 years of age obtained from our own files. Rhabdomyoblastic differentiation was established ultrastructurally by the presence of myofilaments and Z-like densities in 10 of these 11 cases. There were tumor cells in the formaldehyde-fixed, paraffin-embedded material which were positively stained for desmin in all cases, for myoglobin in 7/11 cases, for vimentin in 5/11 cases and for actin in all cases, using monoclonal antibodies. The demonstration of desmin by the monoclonal antibody which was used on the formaldehyde-fixed, paraffin-embedded material is of particular value in the diagnosis of rhabdomyosarcoma. Another tumor, located in the minor pelvis, lacked the light-microscopic features of botryoid, embryonal or alveolar rhabdomyosarcoma, but presented ultrastructural and immunohistochemical evidence of a rhabdomyoblastic differentiation. This tumor was epithelioid in appearance and shared features with alveolar soft part sarcoma. The label epithelioid rhabdomyosarcoma is proposed for this tumor. Nine pleomorphic sarcomas were selected from the national series as possible pleomorphic rhabdomyosarcomas because of the presence of ribbon-shaped tumor cells with an eosiniphilic cytoplasm. There was no electron-microscopic or immunohistochemical evidence of a myogenic differentiation in any of these 9 tumors. The present investigation indicates that a pleomorphic type of rhabdomyosarcoma, indistinguishable from embryonal, botryoid and alveolar rhabdomyosarcoma, is extremely rare or non-existent.
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PMID:Rhabdomyosarcoma in middle-aged and elderly individuals. 271 35

This report describes two cases of alveolar rhabdomyosarcoma of the nasal cavity with unusual histological appearances mimicking clear cell carcinoma. The closely packed tumour cells were polygonal and arranged in sheets and packets. They had an appreciable amount of clear cytoplasm due to accumulation of glycogen. The diagnosis of rhabdomyosarcoma was confirmed by positive staining for desmin and myoglobin. Rhabdomyosarcoma should be included in the differential diagnosis of nasal clear cell tumours, particularly in young adults. A correct diagnosis is important, because chemotherapy is indicated even for apparently localized disease.
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PMID:Clear cell rhabdomyosarcoma of the nasal cavity and paranasal sinuses. 273 15

Tumour markers now have an established role in tumour diagnosis and patient management. However, antibodies used to detect these tumour markers have in some instances proved unreliable, with a low rate of sensitivity and specificity. In this study we wished to evaluate the role of a commercial antibody to myoglobin as a marker of rhabdomyosarcomas. The purpose of this investigation was to assess the sensitivity and specificity of myoglobin antiserum as a marker of rhabdomyosarcomas. This was performed by reacting a large number of tumours (sarcomas, carcinomas and melanomas) with a polyclonal anti human myoglobin antiserum. Staining was demonstrated in 60% of rhabdomyosarcomas. Only two tumours from a total of 226 non-skeletal muscle tumours showed a positive reaction (0.88%). One was a leiomyosarcoma and the other had been classified as an undifferentiated sarcoma but a rhabdomyosarcoma was included in its differential diagnosis. It is of interest that both had been earlier irradiated. This antiserum was therefore a specific but not a very sensitive tumor marker. Its rate of staining of rhabdomyosarcomas is compared with the results in the literature. A great disparity is found and the reasons for this are discussed.
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PMID:Myoglobin: an evaluation of its role as a marker of rhabdomyosarcomas. 275 15

Cellular heterogeneity, a regular property of malignant neoplasms, can constitute the basis for phenotypic shifting in malignant tumors. A disturbance of the balanced interactions as happens as result of all therapy measurements between tumor cell subpopulations and between tumor and host offers the possibility to profound changes on the basis of cellular heterogeneity. As tumor model we used methylcholanthrene-induced murine rhabdomyosarcomas, which were allotransplanted into nude mice. The original as well as the allotransplanted sarcomas were submitted repeated operative tumor mass reductions, comparable to an insufficient tumor surgery in man. The histology and the cellular differentiation were evaluated by light microscopy and immunohistochemical marker expression (vimentin, desmin and myoglobin). Our findings document that primary tumors, their recurrences after incomplete tumor removal, and allotransplants inclusive of their surgically induced recurrences were histologically and immunohistochemically not identical in every case when compared with each other, but despite the experimental procedures they retained basic criteria necessary for the rhabdomyosarcoma diagnosis. Changes in sarcoma histology and cellular marker expression are not only reflected by dedifferentiation but can also comprise differentiation (maturation) processes. After artificial breakdown of intratumoral cellular interactions and tumor/host relations, unknown sarcoma inherent factors directed to retaining basic properties obviously preponderate, at least for the moment, over accidental new cell clones with differing phenotypic characteristics, which could be expected by cellular heterogeneity and would result in a markedly changed tumor.
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PMID:Histological and immunohistochemical findings in experimental rhabdomyosarcomas. Comparisons between original tumors, tumor recurrences and allotransplants in nude mice. 276 8

The diagnosis of orbital rhabdomyosarcoma (RMS) in childhood gives rise to several clinical and anatomo-pathological problems. Antibodies recognizing structural proteins and cytoskeletal components have been shown to increase the diagnostic accuracy of different neoplastic lesions. In this study we examined anatomo-clinically and, where possible, by means of immunohistochemistry and electron microscopy, a series of 14 cases of orbital RMS in childhood. In the 12 cases studied by immunohistochemistry, desmin was always present, although showing variable patterns, and alpha-sarcomeric actin was found in 10 cases. alpha-Smooth muscle actin was always absent. The other markers tested (myoglobin, polyclonal actin, vimentin and enolase) proved unreliable for several reasons. We conclude that antibodies against desmin and alpha-sarcomeric actin are useful for the diagnostic definition of RMS. In addition, immunohistochemical analysis supplies data regarding the degree of tumor differentiation and may be applied to monitor radio- and chemotherapy.
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PMID:Expression of actin isoforms and intermediate filament proteins in childhood orbital rhabdomyosarcomas. 279 Jul 29

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


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