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)

Results are reported of studies to measure the extent of recovery of potentially lethal damage (PLD) in rat rhabdomyosarcoma tumor cells after irradiation both in vivo and in vitro with either high-LET or low-LET radiation. Stationary-phase cultures were found to exhibit repair of PLD following irradiation in vitro either with low-LET X rays or with high-LET neon ions in the extended-peak ionization region. Following a 9-Gy dose of 225-kVp X rays or a 3.5-Gy dose of peak neon ions, both of which reduced the initial cell survival to 6-8%, the maximum PLD recovery factors were 3.4 and 1.6, respectively. In contrast, the standard tumor excision assay procedure failed to reveal any recovery from PLD in tumors irradiated in situ with either X rays or peak neon ions. PLD repair by the in vivo tumor cells could be observed, however, when the excision assay procedure was altered by the addition of a known PLD repair inhibitor beta-arabinofuranosyladenine (beta-ara-A). When a noncytotoxic 50 microM concentration of beta-ara-A was added to the excised tumor cells immediately following a 14.5-Gy in situ dose of X rays, cell survival in the inhibitor-treated cells was lower than in the untreated cells (0.018 compared to 0.056), resulting in a PLD repair inhibition factor of 3.1. Delaying the addition of beta-ara-A for 1, 2, or 3 h following tumor excision reduced the PLD repair inhibition factor to 1.6, 1.5, and 0.9, respectively. Following tumor irradiation in situ with neon ions in the extended-peak ionization region (median LET = 145 keV/micron), less PLD repair was observed than after X irradiation. For 5.8 Gy of peak neon ions, the PLD repair inhibition factors were 2.1, 1.5, 1.3, and 1.1 at 0, 1, 2, and 3 h, respectively. We interpret the absence of measurable PLD repair using the standard tumor excision assay procedure as resulting from undetectable repair occurring during the long interval (about 2 h) required for the cell dissociation and plating procedures. We conclude that at least for our tumor system, PLD repair does occur after irradiation of tumors in situ, even though it is not detectable using the standard tumor excision assay procedure. Thus a failure to measure such repair by this assay in a given tumor system does not necessarily mean the cells are incapable of PLD repair.
Radiat Res 1986 Sep
PMID:PLD repair in rat rhabdomyosarcoma tumor cells irradiated in vivo and in vitro with high-LET and low-LET radiation. 374 69

A light and electron microscopic study of 51 cases of Ewing's sarcoma of bone (ESB) and 33 soft tissue sarcomas (carrying a variety of light microscopic diagnoses, including primitive rhabdomyosarcoma) in children and young adults was performed to clarify the similarities and differences among these tumors. Ultrastructural criteria were developed to evaluate the neoplasms. Remarkable ultrastructural uniformity was found in the cases of ESB. In contrast, the soft tissue sarcomas could be divided into two distinct groups on the basis of the ultrastructural criteria: those closely resembling primitive areas of otherwise differentiated rhabdomyosarcomas, and those indistinguishable from ESB. It is proposed that the diagnosis of soft tissue Ewing's sarcoma be reserved for lesions identical to ESB by both light and electron microscopy. The first group of sarcomas may be histogenetically related to rhabdomyosarcoma and should be distinguished from extraosseous Ewing's sarcoma, as their clinical behavior appears to be quite different.
Hum Pathol 1986 Sep
PMID:Extraosseous Ewing's sarcoma versus primitive rhabdomyosarcoma: diagnostic criteria and clinical correlation. 375 73

The dielectric properties of a rat tumour (rhabdomyosarcoma R1H), skin and muscle were measured in vivo with an open-ended coaxial line and a computer-controlled system based on a network analyser. The permittivity of the tumour R1H and of the normal tissues in anaesthetised rats was determined at frequencies between 0.2 and 2.4 GHz. No significant differences were observed either between rat tumour and muscle or between normal and 15 Gy irradiated rat tumour and skin. However, after a hyperthermia treatment at 43 degrees C for 60 min the dielectric properties, especially of the rat skin, changed due to the hyperthermic induced oedema which is related to an increase in tissue water content. The process of the oedema modifies the dielectric properties of the skin to a higher degree than those of the tumour.
Phys Med Biol 1986 Sep
PMID:Dielectric properties of Co-gamma-irradiated and microwave-heated rat tumour and skin measured in vivo between 0.2 and 2.4 GHz. 377 74

The activity of lactate dehydrogenase (LDH) isoenzymes was studied in clones of rhabdomyosarcoma (RMS) MX-53, being transplanted on mice of CC57W line. Clones obtained in lungs after the intravenous injection of a tumor cell suspension were transplanted into subcutaneous connective tissue (SCT) and eye anterior chamber (EAC). A study of LDH isoenzymes spectrum in transplants and native muscle tissues has been made using polyacrylamide gel electrophoresis and densitometry. We revealed a variability of LDH isoenzyme activity within one clone, and in population of clones under different proliferation conditions. Under all the proliferation conditions, we observed spectra characteristic of tumors of a given histogenesis with predominating activity of M-subunits. But during proliferation in EAC the M:H ratio decreased, and LDH spectra of tumor cells became more similar to the LDH spectrum of normal skeletal muscles. It is concluded that intra- and interclonal variations of the character "LDH isozyme spectrum" is strongly influenced by the environmental factors.
Tsitologiia 1986 Sep
PMID:[Changes in the activity of lactate dehydrogenase isoformes in mouse rhabdomyosarcoma under different proliferation conditions]. 379 63

Between 1972 and 1980, 58 children with malignant tumors underwent iridium 192 (192Ir) curietherapy at the Institut Gustave Roussy. Data from only 45 of them were reviewed (bilateral retinoblastoma and salvage procedures were excluded). The pathologic diagnosis was rhabdomyosarcoma in 75% of cases, yolk sac tumor in 13% of cases, malignant mesenchymoma in two cases, clear cell carcinoma in two cases, and undifferentiated sarcoma in one case. The two major sites were pelvis-perineum (64.5%) and head and neck (31%). The treatment was essentially based on a combination of chemotherapy, which was adapted to the histologic type of the tumor, and curietherapy. Partial surgery was carried out in certain cases, either before chemotherapy for diagnostic purposes, or after chemotherapy to remove infiltrative residual tumor. External beam radiotherapy was used in a few cases in lymph node irradiation. The radioactive material was 192Ir, using afterloading techniques, either intracavitary or interstitially or both. The tumor dose, calculated by computerized dosimetry, was 60 to 65 Gy delivered over 5 to 7 days in the majority of cases. Two patients were lost to follow-up. Thirty-five patients (78%) are alive with no evidence of disease after a mean follow-up period of 5 years. Eight patients died, one of complications related to chemotherapy and seven of the evolution of their disease. The late-complication rate, studied in 33 patients with a minimum follow-up period of 3 years, is 18% (6/33). Curietherapy seems to be a preferable conservative treatment in children with localized and accessible malignant tumors.
Cancer 1985 Sep 15
PMID:Iridium afterloading curietherapy in the treatment of pediatric malignancies. The Institut Gustave Roussy experience. 402 67

The combination of dacarbazine and doxorubicin was given to 26 children with untreated rhabdomyosarcoma to determine its efficacy as front-line chemotherapy. A treatment course consisted of 250 mg/m2 of dacarbazine given iv on Days 1-5 and 60 mg/m2 of doxorubicin given iv on Day 1. After three courses of therapy, 17 patients (65%) achieved partial response and nine failed to respond. The side effects of treatment consisted of nausea, vomiting, flu-like symptoms, neutropenia associated with fever, mucositis, and thrombocytopenia (rarely). Although the response rate is comparable to other drug combinations, the lack of complete responses to the combination indicates that it is less effective as front-line therapy.
Cancer Treat Rep 1985 Sep
PMID:Combination of dacarbazine and doxorubicin in the treatment of childhood rhabdomyosarcoma. 402 41

Antibodies against the M and B subunits of creatine kinase were assessed for their usefulness in the diagnosis of poorly differentiated rhabdomyosarcoma. Routinely processed formaldehyde-fixed tissue and the avidin-biotin-peroxidase complex technique were used. The majority of the poorly differentiated and all of the moderately and well-differentiated rhabdomyosarcomas studied showed immunostaining for the M subunit. The rhabdomyoblastic component of malignant "triton" tumors was also positive. Staining, although weak compared with that of the rhabdomyosarcomas, was also observed in a few leiomyosarcomas, hemangioendotheliosarcomas, malignant fibrous histiocytomas, and ganglioneuroblastomas. On the other hand, staining for the B subunit was seen in many types of soft tissue tumors, including rhabdomyosarcoma, Ewing's sarcoma, and (ganglio)neuroblastoma. The results indicate that creatine kinase subunit M is a useful marker for distinguishing poorly differentiated rhabdomyosarcoma from other types of small round cell tumors in children, such as neuroblastoma, Ewing's sarcoma, and malignant lymphoma.
Hum Pathol 1985 Sep
PMID:Creatine kinase subunits M and B as markers in the diagnosis of poorly differentiated rhabdomyosarcomas in children. 402 46

This clinicopathologic study concerns 8 cases of extraskeletal Ewing's sarcoma, including electron-microscopic examination of one case. In three patients, autopsy was done. The age of the patients ranged from 12 to 31 years with a median of 16 years. The tumors mainly arose in the soft tissues of the trunk (4 cases) and the lower extremity (3 cases). Histologically, they were made up of closely packed uniform, small cells, arranged in sheets separated by strands of fibrovascular stroma. The tumor cells had round to oval nuclei with finely dispersed chromatin and scanty ill-defined cytoplasm almost invariably containing a fair amount of diastase-digested PAS-positive material. Ultrastructurally, the tumor cells were composed principally of undifferentiated mesenchymal cells, and contained prominent pools of glycogen in the cytoplasm. Aggregates of intermediate filaments were seen in a perinuclear location. These light- and electron-microscopic findings are indistinguishable from those of Ewing's sarcoma of the bone. Differential points from other soft-tissue small round cell sarcomas such as malignant neuroepithelioma (peripheral neuroblastoma), embryonal or alveolar rhabdomyosarcoma were briefly discussed.
Acta Pathol Jpn 1985 Sep
PMID:Extraskeletal Ewing's sarcoma. A clinicopathologic and electron microscopic. Analysis of 8 cases. 408 99

This paper reports 4 cases of rhabdomyosarcoma arising from the middle ear, maxillary sinuses, soft palate and larynx. In 3 patients, tumor resection was performed and the other was only biopsied. 3 patients died 6 months, 40 days and 19 months after diagnosis. One had recurrence 4 months after operation and was then treated with hematoporphyrin derivative (HPD)-laser photoradiation. The result was satisfactory and the patient has been living well for 2 years. Histopathology, treatment and prognosis of this tumor are discussed.
Zhonghua Zhong Liu Za Zhi 1985 Sep
PMID:[Rhabdomyosarcoma in E. N. T--report of 4 cases]. 409 83

Rhabdomyosarcoma commonly involves the orbit resulting in unilateral proptosis, swelling, and restricted ocular motility. We saw two unusual cases of children with nasopharyngeal rhabdomyosarcoma which caused rapid bilateral visual loss due to simultaneous spread of the tumor to the orbital apices. Initial CT scans revealed the tumor, but were misinterpreted as "normal," causing a significant delay in diagnosis. We recommend high resolution CT scans of the nasopharynx, orbits, and suprasellar region in any child with rapid bilateral visual loss to rule out this common childhood tumor.
J Clin Neuroophthalmol 1984 Sep
PMID:Rhabdomyosarcoma causing rapid bilateral visual loss in children. 623 56


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