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)

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

A 56-year-old man was admitted to our hospital with right chest pain. Chest X-ray, CT scan and MRI revealed a chest wall tumor and enlarged mediastinal lymph nodes. Percutaneous lung biopsy was performed, and the pathological diagnosis of pleomorphic rhabdomyosarcoma was obtained. The only significant abnormal laboratory finding was elevation of serum NSE (24.5 ng/ml). Although chemotherapy (VAC-ADM) and radiation therapy were performed, the patient died about 7 months after admission. To our knowledge, only 17 cases of chest wall rhabdomyosarcoma have been reported in Japan.
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PMID:[A case of chest wall rhabdomyosarcoma]. 175 9

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

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

22 undifferentiated tumours of the Salivary Gland Register (University of Hamburg) were studied by conventional light microscopical and immunocytochemical methods to elucidate the heterogeneity of this tumour group. The following observations were made in this collective: 18 tumours displayed one or more markers for the epithelial character and were classified as carcinomas. 10 carcinomas were considered as primary ones and 8 were considered as secondary ones (metastatic or invasive "per continuitatem"). Primary carcinomas were subclassified as poorly differentiated variants of a distinctive type of salivary gland tumours, as follows: 6 cases of carcinoma in pleomorphic adenoma, and one case each of mucoepidermoid tumour, adenocarcinoma, salivary duct carcinoma and epidermoid carcinoma. Secondary carcinomas were subclassified as follows: 3 epidermoid carcinomas, 3 nasopharyngeal carcinomas and 2 bronchial carcinomas. One tumour positive for S-100 protein and NSE (Neuron-specific enolase) was classified as a metastatic melanoma. Another tumour positive for vimentin and actin was classified as a rhabdomyosarcoma of the periglandular tissue. Two tumours lacked any markers studied here and were regarded as a malignant paraganglioma and an undifferentiated lymphoma, respectively. The differential diagnosis of the undifferentiated tumours of salivary glands and the special problems of this tumour group are discussed.
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PMID:Undifferentiated tumours of salivary glands. Immunocytochemical investigations and differential diagnosis of 22 cases. 303 6

Three enolase isozymes (alpha alpha, alpha gamma, and gamma gamma) and S-100 protein in the extract of neuroendocrine tumors (neuroblastoma, ganglioneuroblastoma, ganglioneuroma, and pheochromocytoma) and nonneuroendocrine tumors (Wilms' tumor, rhabdomyosarcoma, and hepatoblastoma) were determined by means of enzyme immunoassay systems. All of the tumors examined showed a high level of alpha alpha-enolase (1.71 to 19.0 micrograms/mg protein). Levels of nervous system-specific enolases (NSE; alpha gamma and gamma gamma) in the neuroendocrine tumors were also rather high (alpha gamma, 1.64 to 7.45 micrograms/mg protein; gamma gamma, 0.052 to 5.56 micrograms/mg protein). However, the NSE concentration in the extract of nonneuroendocrine tumors was low (alpha gamma, less than 0.88 micrograms/mg protein; gamma gamma, 0 microgram/mg protein). The level of S-100 protein was relatively high in ganglioneuroma (greater than 500 ng/mg protein) and ganglioneuroblastoma (greater than 100 ng/mg protein), but low in neuroblastoma (less differentiated neuroendocrine tumor) and nonneuroendocrine tumors. Serum levels of enolase isozymes were also determined in neuroblastoma patients before and after resection of primary tumor or effective chemotherapy. The elevated level of serum NSE (alpha gamma and gamma gamma) was markedly decreased with little change in the alpha alpha level by the treatment.
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PMID:Determination of three enolase isozymes and S-100 protein in various tumors in children. 631 26

An elderly woman having a nasal tumor diagnosed as alveolar rhabdomyosarcoma was referred to our hospital. Histological reexamination of the patient revealed that the tumor was composed of lobules of compactly arranged small round atypical cells. The atypical cells contained large oval to round vesicular nuclei, and scanty cytoplasm showing PAS positive glycogen material. The tumor cells were immunohistochemically positive for NSE, S-100 protein and vimentin, but showed negative reaction for myoglobin, desmin, EMA, keratin, LCA, chromogranin and MIC2. Ultrastructurally, the tumor cells contained a few cluster of glycogen particles and less organellae and filaments, and there were no cytoplasmic processes, neurosecretory granules or neurofilaments. Pathological findings suggested primitive neuroectodermal tumor resembling extraskeletal Ewing's sarcoma, but negative reaction for MIC2 immunohistochemically could not ascertain the diagnosis. Location of the tumor and age of the patient of the present case were unusual.
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PMID:Primitive neuroectodermal tumor in sinonasal region. 1007 61

Rhabdomyosarcoma is a common childhood malignancy that may occasionally occur as a component of a mixed mesenchymal tumor, e.g., a triton tumor or malignant ectomesenchymoma. A case is reported of a 13-year-old boy who had resection of a paratesticular embryonal rhabdomyosarcoma with subsequent radiation and chemotherapy. Two years later, a retroperitoneal metastasis was resected. Histology showed a mixture of rhabdomyoblasts, ganglion cells, and a third population of cells with combined features of these two distinct cell types. Electron microscopy confirmed the presence of rhabdomyoblasts with characteristic bundles of myofilaments and Z-band material, and ganglion cells with prominent nuclei and nucleoli, rough endoplasmic reticulum, dense core granules, filaments, and tubules. Notably, the third cell population showed features of both rhabdomyoblasts and ganglion cells. Immunohistochemistry confirmed the mixed population of rhabdomyoblasts (positive for vimentin, desmin, negative for S-100, NSE), ganglion cells (positive for S-100 and NSE, negative for vimentin and desmin), and the third population expressing all test antigens. The features of this metastatic lesion are those of a malignant ectomesenchymoma with combined rhabdomyosarcoma and ganglioneuroma components. It is postulated that this lesion results from biphenotypic expression of tumor cells that previously expressed only rhabdomyoblastic differentiation. The role of prior chemotherapy and radiotherapy in this particular case is unclear.
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PMID:Rhabdomyosarcoma metastasizing as a malignant ectomesenchymoma. 1050 46

Malignant soft tissue tumors other than rhabdomyosarcoma (RMS) are uncommon in infancy, representing approximately 5% of pediatric sarcomas. The pathological categorization of non-RMS soft tissue malignancies from these young patients is complicated by variation in both morphologic and immunohistochemical features. A search covering an 11-year period identified 19 patients presenting at birth or in infancy with a clinical or referral diagnosis of soft tissue sarcoma. After histologic and immunohistochemical review, nine of these tumors were classified as primitive neuroectodermal tumor (PNET), three as infantile hemangiopericytoma (HPC), two as infantile fibrosarcoma (FS), and five as undifferentiated sarcoma. Those identified as undifferentiated sarcomas showed an atypical spindle and ovoid cell morphology, with cellular pleomorphism and high mitotic rate, but lacking the fascicular growth pattern of classic infantile fibrosarcoma. Immunohistochemical staining in this group showed variable weak positivity for a range of markers (desmin, smooth muscle actin, Myo-D1, PGP, NSE, S100, CO56, cytokeratin, and CD99), and did not fit readily into any distinct diagnostic category. In this series, tumors classified as soft tissue PNETs had a poor prognosis despite aggressive treatment. However, once RMS, PNET, and other rare specific lesions are excluded, the remaining undifferentiated sarcomas, despite their unusual morphology and immunohistochemistry, appear to behave in a similar favorable manner to infantile fibrosarcoma.
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PMID:Aberrant immunohistochemical expression in nonrhabdomyosarcoma soft tissue sarcomas of infancy: retrospective review of clinical material. 1237 Jul 73


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