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)

A 7-month-old boy had a giant pigmented lesion involving the trunk and thighs that exhibited many hyperpigmented hairy and verrucous nevi. One of the nevi ulcerated and on histological examination consisted of pleomorphic rhabdomyosarcoma cells that stained for muscle-specific actin (HHF-35), desmin, and myoglobin. Around the tumor, in the dermis, benign pigmented nevus cells were observed. The occurrence of malignant tumors, other than malignant melanoma, in pigmented nevi is rarely described.
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PMID:Rhabdomyosarcoma in a congenital pigmented nevus. 137 40

Twenty-one cases of embryonal rhabdomyosarcoma, composed mainly of elongated spindle cells arranged in a fasciculated or storiform pattern, were retrieved from the files of the German-Italian Cooperative Soft Tissue Sarcoma Study. The term spindle cell rhabdomyosarcoma is proposed to designate this histotype. Spindle cell rhabdomyosarcoma predilected male patients (18 males, three females) and involved mostly the paratesticular area (12 cases) and the head and neck region (six cases). Histologically, all cases were characterized by a uniform proliferation of elongated spindle cells with eosinophilic and fibrillar cytoplasm mimicking smooth muscle fibers; immunocytochemical studies disclosed high expression of the muscle markers titin, desmin, and myoglobin. Clinical information was available in 17 cases; according to the Intergroup Rhabdomyosarcoma Study (IRS) grouping system, 13 were classified in group I, two in group II, and two in group III. Sixteen patients were well and alive 24 to 100 months after diagnosis; one patient died from disease progression 24 months after diagnosis. Analysis of our results determined that spindle cell rhabdomyosarcoma constitutes a rare variant of the embryonal form, showing a high degree of skeletal muscle differentiation and a low malignant potential; it should therefore be distinguished from classical forms of embryonal rhabdomyosarcoma.
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PMID:Spindle cell rhabdomyosarcoma. A prognostically favorable variant of rhabdomyosarcoma. 159 14

A sarcoma arising from the inferior vena cava occupied the entire lumen of the inferior vena cava, right atrium, hepatic veins and common iliac veins. Its histological appearance was non-specific sarcoma, except for the presence of a few rhabdomyoblasts and some immature cartilaginous tissue. Immunohistochemically, some tumor cells were positive for myoglobin, desmin, HHF-35, and vimentin. Electron microscopy revealed that some tumor cells contained myofilaments and Z bands in the cytoplasm, which are characteristics of rhabdomyosarcoma. The tumor was diagnosed as rhabdomyosarcoma with focal cartilaginous differentiation (malignant mesenchymoma) of the inferior vena cava.
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PMID:Rhabdomyosarcoma with focal cartilaginous differentiation (malignant mesenchymoma) of the inferior vena cava. 163 40

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

The authors assessed a panel of immunohistochemical stains against 109 pediatric solid tumors, primarily rhabdomyosarcomas, under the auspices of the Intergroup Rhabdomyosarcoma Study. Fresh tumor tissue received from participating organizations was divided into portions that were either frozen or fixed in formalin, alcohol, or B5. Immunostaining was performed by the avidin-biotin complex method using monoclonal antibodies to desmin, neurofilaments, vimentin, cytokeratin, and leukocyte common antigen on cryostat sections. Tissue was also embedded in paraffin and stained with antimuscle-specific actin (MSA) and polyclonal antibodies to desmin, creatine kinase M subunit (CKM), myoglobin, and neuron-specific enolase (NSE). Antidesmin staining of cryostat sections was the most sensitive indicator of rhabdomyosarcoma (58 of 62 specimens positive). Results with this reagent in alcohol-fixed and formalin-fixed tissue were similar (46 of 56 positive versus 43 of 56 positive, respectively) and comparable with results with anti-MSA in formalin-fixed tissue (43 of 55 positive). However, the proportion of cells stained by antidesmin was higher in alcohol-fixed tissue than in formalin-fixed tissue. Staining with antimyoglobin and anti-CKM was much less satisfactory, with positivity rates of 17 of 37 and 11 of 57, respectively, in formalin-fixed rhabdomyosarcomas. Immunostaining of muscle markers revealed evidence of myogenesis in six undifferentiated sarcomas and in two sarcomas with inadequate histologic study on hematoxylin-eosin-stained sections. However, positivity was also noticed in samples of fibromatosis, Wilms' tumor, ectomesenchyoma, peripheral primitive neuroectodermal tumor, renal rhabdoid tumor, myositis ossificans, malignant fibrous histiocytoma, and embryonal sarcoma of the liver. The authors conclude that combined use of antidesmin and anti-MSA enhances the diagnosis of childhood sarcomas, especially when employed with other techniques such as electron microscopic study.
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PMID:Immunohistochemical study of childhood rhabdomyosarcomas and related neoplasms. Results of an Intergroup Rhabdomyosarcoma study project. 171 May 39

Primary rhabdomyosarcoma of the cerebellum. Histopathological and immunohistochemical study: a necropsy case. A necropsy case of primary cerebellar rhabdomyosarcoma occurred in a 38-year-old man has been investigated by histological and immunohistochemical techniques. In the most differentiated rhabdomyoblasts microscopic analysis showed obvious cross-striations and immunohistochemical reactivity for myoglobin (PAP method). Many tumor cells were positive for vimentin and muscle-specific intermediate filament protein desmin, but neither for glial fibrillary acidic protein nor neuron-specific enolase. The diagnostic role of the immunohistochemistry in this tumor is pointed out. The clinicopathological features of 30 cases of primary rhabdomyosarcoma of the central nervous system previously reported in the literature are briefly reviewed, and the histogenesis is discussed.
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PMID:[Primary rhabdomyosarcoma of the cerebellum. Histopathological and immunohistochemical study of an autopsy case]. 174 82

At necropsy, a 7-year-old Holstein cow, clinically diagnosed via rectal palpation as having malignant abdominal neoplasia was found to have many metastatic tumors of various sizes in the abdominal and thoracic cavities and in the liver, lung, kidney, adrenal, uterus, and lymph nodes. These tumors were examined by histologic and immunohistochemical methods using anti-human myoglobin, anti-bovine myoglobin, and anti-desmin sera. Tumors were diagnosed as undifferentiated alveolar rhabdomyosarcomas. The tumors in the abdominal and thoracic cavities differed histologically from the metastases in organs. The former tumors consisted of only undifferentiated cells, most of which did not stain positively for desmin or myoglobin. The metastatic tumors contained a few rhabdomyoblastic cells that stained positively for desmin and myoglobin. Tumors tissues from the cow were transplanted and propagated through six passages in athymic nude mice. After one passage, the transplanted tumor histologically resembled those from the abdominal serosa and consisted of cells with scanty cytoplasm with an alveolar arrangement. Cells from this tumor stained positively for desmin and negatively for myoglobin. After two passages through nude mice, tumor cells were of two distinct histologic types: those cells with scanty cytoplasm and elongated cells with plentiful eosinophilic cytoplasm. Both of these cells stained positively for both desmin and myoglobin. After six passages, cross striations were detected in neoplastic cells by electron microscopy. These findings illustrate that a highly undifferentiated bovine rhabdomyosarcoma, in which most of the cells in the original tumor lacked desmin and myoglobin, became better differentiated and stained positively for desmin and myoglobin after serial transplantation in nude mice.
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PMID:Bovine undifferentiated alveolar rhabdomyosarcoma and its differentiation in xenotransplanted tumors. 175 Jan 69

A human tumour cell line, designated RMS-YM, was established from a childhood rhabdomyosarcoma. The monolayer cells were polygonal, round or spindle-shaped. The cells became multilayered and formed many focal piles when confluent. RMS-YM became stable with a doubling time of about 30 h and has been maintained for 104 passages to date. Tumourigenicity of the cells was confirmed by heterotransplantation into nude mice. Morphological features were similar to those of the primary tumour, and myofibrils were found by electron microscopy. The expression of desmin and human myoglobin, and high levels of striated muscle system specific enzymes were recognised. Chromosomal analysis revealed possible gene amplification in the form of homogeneously staining regions. Oncogene analysis was performed on the primary tumour and the cell line, but neither N-myc nor N-ras genes were amplified, nor were Ki-ras, Ha-ras or N-ras genes mutated at the 12th, 13th and 61st codons. The RMS-YM cell line may provide a system to identify novel genes which are amplified in rhabdomyosarcoma.
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PMID:Cytogenetic and cellular characteristics of a human embryonal rhabdomyosarcoma cell line, RMS-YM. 190 91

We report a case of primary rhabdomyosarcoma of the ileum occurring in a 45-year-old man. The rhabdomyoblastic nature was immunohistochemically confirmed by positivity with anti-myoglobin and anti-striated actin antisera. We discuss the differential diagnosis with similar tumors. To our knowledge, ileal rhabdomyosarcomas have not been previously reported in adults.
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PMID:Primary rhabdomyosarcoma of the ileum in an adult. 200 Nov 60

The cytologic and histologic findings in two cases of the extremely rare alveolar rhabdomyosarcoma of the vulva are reported. The tumor cells in fine needle aspiration smears from one case and tumor imprints from both cases were isolated or were in sheets or reticular patterns. The nuclei were round to oval; only a few cells were multinucleated. The chromatinic material was increased in amount and finely granular. Many mitotic figures were observed. The cytoplasm was scanty in general, but some cells had abundant cytoplasm; cross striations were not recognized. The tumor cells were positive with immunocytochemical stains for desmin, vimentin and myoglobin. Similar findings were observed in biopsy and surgical specimens.
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PMID:Alveolar rhabdomyosarcoma of the vulva. Report of two cases. 204 36


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