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)

In view of the personal observation that malignant peripheral neuroectodermal tumours (MPNT) can present different histological growth patterns, 41 cases of MPNT were histologically and immunohistochemically studied. The median age of the 41 patients was 15 years (range: 9 months - 23 years). There were 27 males and 14 females. Most tumours (23/41) were located in the thoracopulmonary region. In 31/41 cases there was bone as well as soft tissue involvement. The following histopathological patterns were found: Ewing's sarcoma-like (n = 7), atypical Ewing's sarcoma-like (n = 4), neuroblastoma-like (n = 8), rhabdomyosarcoma-like (n = 8), and hemangiopericytoma-like (n = 1). In 2 cases combined patterns were noted, one tumour being characterized by neuroblastoma-like and Burkitt's lymphoma-like features. Most cases of MPNT differed from the cytological features of typical Ewing's sarcoma in that they contained hyperchromatic nuclei with distinct nucleoli. Some reticulin fibrils were found in between the cells of some cases. Immunohistochemically, 19/23 cases reacted positively to vimentin, 29/32 to neuron specific enolase (NSE), 16/28 to protein S-100, and 1/9 to glial fibrillary acidic protein. 12/24 cases reacted positively to NSE and protein S-100. Neurofilaments and desmin were not found in the formalin fixed material of the present study. The results show that most cases of MPNT can be distinguished from typical Ewing's sarcoma by cytological and histological findings. Differential diagnosis from atypical Ewing's sarcoma, neuroblastoma, and rhabdomyosarcoma is possible by immunohistochemistry.
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PMID:[Malignant peripheral neuroectodermal tumors. Histological and immunohistological conditions in 41 cases]. 267 76

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

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

158 cases of the Cooperative Ewing's Sarcoma Trials (CESS 81/86), which have been documented at the Pediatric Tumor Registry, Kiel, were studied by conventional light microscopy and immunohistochemistry. There were 77 cases of typical Ewing's sarcoma with 70 cases being located in the skeleton and 7 in soft tissues. Of the 14 cases of atypical Ewing's sarcoma 7 cases each were localized in bone and in soft tissue, respectively. In contrast to typical Ewing's sarcoma, cells of atypical Ewing's sarcoma were larger and displayed more heterochromatin. Both, typical and atypical Ewing's sarcoma reacted positively for vimentin. Other stains were negative, notably the neuron specific enolase (NSE). In 55 cases a diagnosis of malignant peripheral neuroectodermal tumor (MPNT) was made. Histologically most of these tumors resembled atypical Ewing's sarcoma. By immunohistochemistry positive reactions were found for NSE, vimentin, protein S-100, neurofilaments and glial fibrillary acidic protein. In 3 cases a diagnosis of small cell osteosarcoma was made. There were 2 cases of undifferentiated sarcoma of bone, 2 cases of soft tissue sarcoma of undetermined histogenesis and 2 cases of rhabdomyosarcoma. Of the 4 tumors which could be investigated for response to polychemotherapy, 1 each corresponded to grade II and III, respectively, and 2 to grade IV according to the classification of histologic grade of regression established by Salzer-Kuntschik et al. (1983).
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PMID:[Cooperative Ewing's Sarcoma Studies 81/86: pathologico-anatomic and immunohistochemical findings and differential diagnosis of Ewing sarcoma]. 306 61

The type, differentiation and histogenesis of the tumor cells of alveolar soft part sarcoma (ASPS) have been analyzed in a series of ten cases by a light-microscopic, ultrastructural, immunohistochemical and cytologic investigation and quantitative DNA analysis. Four tumors deviated from ordinary ASPS: three were wholly or partly of the so-called pleomorphic variant of ASPS and a fourth tumor showed calcifications of the psammoma body type. The ultrastructural findings and immunohistochemical demonstration of desmin supported the hypothesis of a rhabdomyomatous differentiation and gave no support to epithelial (negative immunoreactions for cytokeratins, epithelial membrane antigen, HMFG-1 and -2, tissue polypeptide antigen (TPA] or neuroectodermal (negative for S-100 protein, glial fibrillary acidic protein, neurofilaments) differentiation. The negative immunoreactions for vimentin and myoglobin and the positive reaction for neuron specific enolase (NSE) do not exclude a rhabdomyomatous differentiation since in rhabdomyosarcomas the undifferentiated rhabdomyoblasts generally contain vimentin and the differentiated tumor cells contain myoglobin and rhabdomyosarcoma has previously been reported as being positive for NSE. The production of external lamina material peripherally in the tumor cell nests and around vessels in the vascular septa was demonstrated both ultrastructurally and by immunohistochemistry using antibodies against collagen IV and laminin. The cytologic appearance in smears obtained by fine-needle aspiration from a case of the pleomorphic variant showed some resemblance to that of a carcinoma. The seven tumors with an ordinary cell appearance were found to show a diploid DNA-distribution at a quantitative analysis performed on paraffin sections, while the three tumors wholly or partly of the pleomorphic type showed an additional tetraploid peak.
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PMID:Alveolar soft part sarcoma. An immunohistochemical, cytologic and electron-microscopic study and a quantitative DNA analysis. 312 65

A case of embryonal rhabdomyosarcoma of the left kidney is reported. A 16-year-old boy was admitted with the complaint of left abdominal pain and fever on January 6, 1983. Radiological examination showed a tumor of the left kidney; and, nephrectomy was performed. Histopathologically the entire tumor was composed of undifferentiated round cells. Diagnosis of embryonal rhabdomyosarcoma was made on the basis of special stains including immunohistochemical study with nervous tissue specific enolase. Although radiation and chemotherapy were performed postoperatively, the tumor recurred and the patient died on October 22, 1983. The problems of differential diagnosis of embryonal rhabdomyosarcoma from sarcomatous types of nephroblastoma, particularly rhabdoid tumor and other undifferentiated renal tumors were discussed. Fifteen rhabdomyosarcoma of the kidney including our case have been reported in the Japanese literature.
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PMID:[A case of embryonal rhabdomyosarcoma of the kidney]. 406 Dec 1

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

Enolase isozymes (alpha, beta and gamma enolases) in the extracts of pediatric tumors (neuroblastoma, ganglioneuroblastoma, rhabdomyosarcoma and Wilms' tumor) were determined by means of enzyme immunoassay systems. All tumor tissues examined contained alpha enolase at high levels (2070-19100 ng/mg protein). The beta and gamma enolases were present at high levels particularly in rhabdomyosarcoma (886 +/- 750 ng/mg protein) and (ganglio)neuroblastoma (2060 +/- 890 ng/mg protein), respectively. Immunohistochemical studies confirmed these results. Serum levels of these enolase isozymes were also determined in pediatric tumor patients. Before treatment, a serum sample from a patient with rhabdomyosarcoma contained a high level of beta enolase and serum samples from patients with (ganglio)neuroblastoma contained high levels of gamma enolase. However, the levels of serum beta and gamma enolases were low in patients with Wilms' tumor. The elevated level of beta or gamma enolase in serum from rhabdomyosarcoma or (ganglio)neuroblastoma patients was markedly decreased after adequate treatment (operation, chemotherapy or radiation). The results indicated that the enolase isozymes are useful marker antigens for differential diagnosis and therapeutic monitoring of neuroblastoma and rhabdomyosarcoma.
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PMID:Enolase isozymes as markers for differential diagnosis of neuroblastoma, rhabdomyosarcoma, and Wilms' tumor. 632 51

A case of rhabdomyosarcoma of the embryonal alveolar type arising from the prostate in a 6-year-old boy was reported with a immunohistochemical survey. Tumor cells in the biopsied inguinal lymph node were weakly positive for myoglobin and CPK-mm and showed positive staining for neuron specific enolase (NSE). At autopsy, an immunohistochemical examination demonstrated that the tumor cells, especially differentiated elongated cells, were strongly positive for myoglobin, CPK-mm and NSE. An enzyme immunoassay for three distinct subunits of NSE revealed a 320 fold increase in the beta subunit of enolase which is specific for heart and skeletal muscle. The diagnostic significance of immunohistochemical methods for rhabdomyoblasts was discussed.
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PMID:Rhabdomyosarcoma of the prostate--report of a case with an immunohistochemical study of the neoplastic rhabdomyoblast. 639 21


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