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)

Eleven cases of proliferative fasciitis and myositis in children, ages 2.5 months to 13 years, are presented. Eight lesions averaging 2.3 cm in size occurred in the extremities, two in the head and neck region and one on the chest wall. Like proliferative fasciitis and myositis in adults, these lesions consisted of admixtures of large polygonal to spindled, ganglion-cell-like fibroblasts with vesicular nuclei and prominent inclusion-like nucleoli. Seven of 11 lesions were initially diagnosed as sarcomas, most commonly rhabdomyosarcoma. Four patients were treated by wide excision (three with regional lymphadenectomy), three received chemotherapy, and one was given radiation therapy. There were some histologic differences from adult-type proliferative fasciitis and myositis. The childhood lesions were generally well circumscribed, lobulated, extremely cellular with less collagen production, and often associated with acute inflammation and microscopic foci of necrosis. Immunohistochemical comparison with adult proliferative fasciitis and myositis showed similar immuneprofiles; the ganglion-like cells stained for vimentin and actin and focally with KP1, suggesting myofibroblastic and histiocytic features. None of the lesions stained for keratin, desmin, or S-100 protein. Ultrastructural examination of two cases revealed cells with a constellation of fibroblastic, myofibroblastic, and histiocytic features. Follow-up of seven patients, averaging 58 months from diagnosis, confirmed that all are alive and well. Recognition of this cellular variant of proliferative fasciitis and myositis is important to prevent misdiagnosis as a sarcoma and unnecessary, excessive therapy.
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PMID:Proliferative fasciitis and myositis of childhood. 156 69

Using the avidin-biotin complex immunoperoxidase technique and antibodies to myoglobin, desmin, CLA, NSE, GFAP, keratin, fibronectin, alpha 1AT, lysozyme, S-100 protein, vimentin, cytokeratin, actin, the authors studied 60 cases of rhabdomyosarcoma (RMS) histopathologically diagnosed previously. Thirty-six cases showed both myoglobin and desmin positive stain, an objective evidence of the origin from skeletal muscles. The other 24 cases were identified as of non-skeletal muscle origin, including MFH, lymphoma, melanoma, neuroblastoma, malignant neurilemmoma, leiomyosarcoma etc. This study strongly suggests that histologic examination of RMS may lead to incorrect diagnosis. Histologically MFH and other types of spindle cell sarcomas invading normal skeletal muscles may be confused with pleomorphic RMS, lymphoma and neuroblastoma may be confused with embryonic RMS. Our findings indicate that myoglobin is a highly sensitive and specific tumor marker for RMS.
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PMID:[Immunohistochemical differential diagnosis of 60 cases of rhabdomyosarcoma]. 166 97

Immunocytochemistry with the use of monoclonal antibodies to vimentin, keratin, desmin, neurofilaments and leukocyte common antigen (LCA) was applied to 53 fine needle aspirates of small cell-, round-, blue-cell malignant tumors of childhood (neuroblastomas, nephroblastomas, non-Hodgkin's lymphomas, Ewing's sarcomas and rhabdomyosarcomas). The results revealed the following immunophenotypes: neuroblastomas were positive for neurofilaments. Nephroblastomas contained vimentin (in blastema cells), keratin (in epithelial and blastema cells) Non-Hodgkin's lymphomas were vimentin and LCA positive, keratin negative. Ewing's sarcomas displayed multidirectional differentiation. Rhabdomyosarcomas were desmin and vimentin positive. It is concluded that immunocytochemistry in fine needle aspirates should be used as an objective evidence to support morphologic differential diagnosis of small cell-, round-, blue cell malignant tumors of childhood.
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PMID:Immunocytochemistry in fine needle aspirates of small cell-, round-, blue-cell malignant tumors of childhood (neuroblastoma, nephroblastoma, lymphoma, Ewing's sarcoma, rhabdomyosarcoma). 166 3

Twenty-six cases of malignant soft tissue tumors with features similar to renal rhabdoid tumors were identified among approximately 3,000 childhood sarcomas entered on Intergroup Rhabdomyosarcoma Studies I-III. The tumors consisted of polygonal cells with vesicular nuclei and prominent nucleoli and cytoplasmic intermediate filament inclusions as identified by electron microscopy and immunohistochemistry. The growth pattern was predominantly solid or solid-trabecular. Immunohistochemistry showed vimentin, wide spectrum keratin, and epithelial membrane antigen to be the most consistent antigenic phenotypes. Eleven patients were infants less than 1 year of age. The tumors affected predominantly soft tissues of proximal extremities, trunk, and retroperitoneum/pelvis/abdomen. Nineteen patients died within 1 to 82 months (median, 6 months) from the start of treatment. Five patients have survived the disease for 2 to 13 years. When compared with the survival analysis of 991 Intergroup Rhabdomyosarcoma Study II patients, it was obvious that this group of tumors fares very poorly (P less than .001). The tumor belongs to the group of soft tissue neoplasms showing mesenchymal and subtle epithelial differentiation, similar to epithelioid sarcoma. Because of its identifiable histology, site and age distribution, and poor outcome, it warrants a status as an independent entity.
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PMID:Rhabdoid tumors of soft tissues: a clinicopathologic study of 26 cases enrolled on the Intergroup Rhabdomyosarcoma Study. 171 49

A case of dedifferentiated leiomyosarcoma of the uterus was examined using immunohistochemistry. The tumor arose in the myometrium, and was a whitish large nodule with hemorrhage and necrosis. Histologically it was a well differentiated leiomyosarcoma with foci showing epithelioid pattern, and in part resembling malignant fibrous histiocytoma (MFH) and giant cell tumor (GCT). Additionally, small round neoplastic cells arranged in an alveolar manner, simulating alveolar rhabdomyosarcoma, were seen in some areas. Neoplastic cells in well differentiated areas expressed desmin, muscle-specific actin and LeuM1, whereas those in epithelioid and poorly differentiated areas lacked these antigens. Instead, tumor cells in epithelioid and small round cell areas were positive for keratin. Interestingly, most tumor cells in well differentiated, epithelioid and small round cell areas were also positive for MB1. However, tumor cells in GCT- and MFH-like areas reacted with none of the antibodies used. Ultrastructurally, some tumor cells possessed various amounts of microfilaments with or without dense patches, whereas others lacked them. These findings suggest that the divergent antigen expression was attributable to different levels of differentiation, and that poorly differentiated components had lost their native features.
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PMID:Histological and immunohistochemical observations of dedifferentiated leiomyosarcoma of the uterus. 171 42

An immunohistochemical analysis using a series of mono- and polyclonal antibodies against myoglobin, desmin, vimentin, actin, NSE, S-100 protein and keratin was done on alveolar rhabdomyosarcoma (3 cases), embryonal (3) and pleomorphic rhabdomyosarcoma of the orbit (1). All tumors were stained by monoclonal antibodies to desmin and vimentin, whereas 6 of the 7 cases were stained by myoglobin. The results indicate that desmin and vimentin are highly sensitive immunohistochemical markers for the diagnosis of rhabdomyosarcoma, especially in poorly differentiated ones.
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PMID:Orbital rhabdomyosarcoma. Immunohistochemical studies of seven cases. 211 61

We report an unusual case of primary cutaneous embryonal rhabdomyosarcoma presenting as a solitary skin lesion on the anterior chest of a 20-month-old child. The tumor was characterized by small, round to oval, poorly differentiated cells. Immunohistochemically, the tumor was negative for NSE, S-100 protein, LCA, and keratin but positive for muscle-specific actin, myoglobin, desmin, and vimentin, thus indicating the presence of myogenous differentiation. Ultrastructural analysis demonstrated thick and thin filaments. Special studies showed no evidence of a primary rhabdomyosarcoma in the patient at a more typical location, nor was there any evidence of metastases.
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PMID:Primary cutaneous rhabdomyosarcoma. 220 84

Eight primary carcinomas of the lung with a prominent spindle-cell sarcomatoid component were studied by immunocytochemical staining and electron microscopy. The eight tumors were indistinguishable by conventional light microscopy, with the exception of one unusual neoplasm that followed multiple pathways of differentiation with elements of squamous cell carcinoma, rhabdomyosarcoma, chondrosarcoma, and an undifferentiated spindle-cell population. Reticulin fiber production by individual spindle cells and a sharp demarcation of the carcinomatous and sarcomatoid domains by light microscopy were not useful differentiating features. Three of the eight tumors exhibited keratin expression in both the carcinomatous and spindle-cell components. Both immunocytochemical and electron microscopic analyses were required to detect epithelial differentiation, as in one case keratin was identified only by immunocytochemical staining and in another only by ultrastructural examination. Epithelial differentiation was undetectable in the sarcomatoid component of five tumors, and in one case immunoreactive myoglobin was identified in spindle cells; skeletal muscle differentiation was confirmed ultrastructurally. We propose that pulmonary carcinomas exhibiting evidence of epithelial differentiation in a sarcomatoid component be termed spindle-cell carcinomas and that those biphasic tumors exhibiting mesenchymal differentiation into specific tissues, such as neoplastic bone, cartilage, or striated muscle, or lacking epithelial differentiation by light microscopy, immunocytochemistry, and electron microscopy be classified as carcinosarcomas. This distinction may ultimately be unnecessary, because these two tumors may represent different points along a morphologic and biologic continuum.
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PMID:Pulmonary carcinomas with a sarcomatoid element: an immunocytochemical and ultrastructural analysis. 244 86

Five cases of epithelioid sarcoma are reported, of which four were studied by immunohistochemistry and one by electron microscopy. Immunohistochemically, the results of cytokeratin showed that these four cases were positive to different degrees by polyclonal cytokeratin and three out of four revealed positive results with low molecular weight cytokeratin monoclonal antibody. Two cases were positive with carcinoembryonic antigen and the other two were negative. The immunohistochemical stain was helpful for differential diagnosis from other sarcomas, such as malignant fibrous histiocytoma and rhabdomyosarcoma. By electron microscopy, tumor cells could be divided into light and dark cells which possessed more or less cell organelles, intermediate microfilaments in the cytoplasm and numerous pinocytic vesicles lying along the cell membrane. The occasional presence of poorly developed desmosomes or the absence of tonofibril bundles and glandular structures can rule out the diagnosis of carcinoma. One of these cases was originally diagnosed as metastatic carcinoma to the skin by light microscopy, which was refuted by electron microscopy, and the keratin positive reaction by immunohistochemistry was helpful for the diagnosis of epithelioid sarcoma. Therefore, the authors suggest that immunohistochemical and electron microscopic studies be used for diagnosing difficult cases of epithelioid sarcoma. From our observation, we consider that the cells of epithelioid sarcoma may derive from undifferentiated mesenchymal cells possessing the potentials of differentiating into synovioblasts, histiocytes or fibroblasts.
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PMID:[Epithelioid sarcoma--immunohistochemical and ultrastructural study]. 246 40

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


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