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)

218 patients examined for localized cancer of the extremities (50% benign, 50% malignant) are reported. The incidence is considered by sex and age. Demolition surgery was carried out in 35 patients and 27 locoregional treatment with antiblastics and radioactive isotopes was employed. Conservative surgery was used in 158. Operative mortality was less than 1% (2 cases out of 218) and did not reach 6% if amputations and disarticulations alone are considered. There were no deaths in cases submitted to radiochemotherapy and inguinocrural hymphadenectomy. The series includes a 15-years survival for giant-cell tumour of the femur treated with radioactive microsphores, an 11-years survival for a popliteal melanoma submitted to radiocolloid infiltration, a 9-year survival for femoral osteosarcoma for which hip disarticulation was carried out and a 4-year survival for a recurrent rhabdomyosarcoma of the hip operated by hemipelvectomy.
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PMID:[Methods of treatment of tumors of the soft parts and bones of the limbs and extremities. Analysis of case records]. 46 96

Rhabdomyosarcoma usually presents as a soft tissue mass, which may invade adjacent bone. However, the patient presents occasionally with bony metastases. Recognition of these is important for staging and management. Fifty-eight cases have been reviewed; 14 of these had local bone invasion by the soft tissue tumour. All bones involvel were flat bones; 12 showed permeated bone destruction and two showed geographic destruction. Bone expansion was seen in half the involved bones. Twelve of the 58 cases showed secondary bone deposits, which were the presenting feature in five. Although 10 cases had permeated bone destruction, two were very well defined with a wide range of radiological appearances. The radiological differential diagnosis includes neuroblastoma, leukaemic infiltration, lymphoma, histiocytosis X, solitary and multifocal osteosarcoma and other deposits.
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PMID:The radiology of bone changes in rhabdomyosarcoma. 62 1

Combination chemotherapy with adriamycin and DTIC was used in 102 evaluable patients under 15 years of age who had previously treated metastatic solid tumors. Responses, defined as 50% or more reduction in all tumor masses, occurred in 10 out of 27 patients with neuroblastoma, 3 out of 8 patients with Wilms tumor, 7 out 15 patients with Ewing sarcoma, 2 out of 6 patients with osteosarcoma, 5 out of 13 patients with rhabdomyosarcoma, and 15 out of 33 patients with miscellaneous tumors which included a patient who had a complete regression of an extensive juvenile angiofibroma. Response rate to combination chemotherapy with adriamycin and DTIC in patients with Ewing sarcoma was significantly superior to the response rate obtained with adriamycin alone in another Southwest Oncology Group Study. Major toxicity included nausea, vomiting, myelosuppression, high incidence of pneumocystis carinii pneumonia (5 patients) and congestive heart failure (4 patients). There was 7 drug-associated deaths due to sepsis (1), pneumocystis carinii pneumonia (4), and congestive heart failure (2).
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PMID:Combination chemotherapy with adramycin (NSC-123127) and dimethyl triazeno imidazole carboxamide (DTIC) (NSC-45388) in children with metastatic solid tumors. 95 60

The ability of dynamic, gadolinium-enhanced magnetic resonance (MR) imaging to allow prediction of histologic responses to initial chemotherapy was evaluated in 20 patients with osteosarcoma (n = 12), Ewing sarcoma (n = 4), rhabdomyosarcoma (n = 3), or synovial sarcoma (n = 1). Tumor signal intensity was measured on fast low-angle shot (FLASH) gradient-echo images obtained at 15-second intervals before and 3 or more minutes after manual intravenous injection of gadopentetate dimeglumine. Signal intensity was plotted against time, and slopes were calculated for the percentage increase in signal intensity per minute. Slopes and changes in maximum tumor size during and after chemotherapy were correlated with histologic evaluations of tumor response. Eleven of the 20 tumors met histologic criteria for response. Histologic response was moderately correlated with slopes obtained during chemotherapy (rs [Spearman rank correlation] = .53, P = .02) but not with changes in tumor size (rs = .02, P = .94). Tumor slopes obtained after chemotherapy were highly correlated with histologic findings (rs = .65, P = .007); the correlation with changes in tumor size increased but remained nonsignificant (rs = .41, P = .11).
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PMID:Pediatric musculoskeletal tumors: use of dynamic, contrast-enhanced MR imaging to monitor response to chemotherapy. 843 Feb 8

Functionally equivalent genetic maternal can be labelled by an epigenetic marking process and used differentially depending on whether its origin is maternal or paternal. This phenomenon is known as genomic imprinting and is manifested at either the chromosomal or gene level. Genomic imprinting seems to play an important role in cancer predisposition syndromes, and phenotypic consequences are evident in constitutional deletion syndromes and uniparental disomies. Moreover, there seems to be a preferential retention of paternal alleles in sporadic tumours such as Wilms' tumour, rhabdomyosarcoma, osteosarcoma and retinoblastoma. To investigate whether chromosomes involved in acquired abnormalities of haematologic neoplasms show a similar 'parent of origin' bias, we studied the inheritance of the translocated chromosomes 9 and 22 in cases of Philadelphia-chromosome-positive leukaemia, using unique specific chromosome band polymorphisms. Here we show that the translocated chromosome 9 was of paternal origin, whereas the translocated chromosomes 22 were derived exclusively from the maternal copy, in 11 cases with reliable polymorphisms. Our data therefore provide evidence that imprinting phenomena may play an important role in acquired tumour-specific chromosome rearrangements.
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PMID:Parental origin of chromosomes involved in the translocation t(9;22). 140 53

This study investigates the potential of in vivo 31P magnetic resonance spectroscopy (MRS) to characterize musculoskeletal tumors and to determine preoperative levels of histological necrosis, which is an important clinical indicator of patient response. Pretherapy MRS was performed on 28 patients with large musculoskeletal tumors: 13 with osteosarcoma, 3 with chondrosarcoma, 5 with malignant fibrous histiocytoma, 1 with desmoid tumor, 1 with Ewing's, 2 with hemangioendothelioma, 1 with myxoid liposarcoma, 1 with synovial cell sarcoma, and 1 with rhabdomyosarcoma. Fifteen patients had follow-up MRS examinations after commencement of chemotherapy (mean of five/patient), eight of whom have now had surgery. Elevated levels of PMEs (P < 0.01), P(i) (P < 0.01), and PDEs (P < 0.02) as well as elevated tumor pH (P < 0.05) were observed in all patients. The synovial cell sarcoma was characterized by high levels of PMEs (> 20%) and low pH (pH 6.76). This contrasted with the spectra obtained from the malignant fibrous histiocytomas which had high levels of PDEs (17 +/- 5%). Reductions in PDE levels postchemotherapy were associated with a high degree of necrosis (> 90%) at surgery, while an increase in PDE levels was associated with a low level of histological necrosis. Likewise, reductions in the ratios PDE/NTP and PDE/PCr and an increase in P(i)/PDE were also associated with a high level of necrosis.
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PMID:Tissue characterization and assessment of preoperative chemotherapeutic response in musculoskeletal tumors by in vivo 31P magnetic resonance spectroscopy. 146 Nov 10

The topoisomerase I inhibitor 9-dimethylaminomethyl-10-hydroxycamptothecin (topotecan) was evaluated against a panel of xenografts comprising four lines of adult colon adenocarcinoma, three colon tumors derived from adolescents, six childhood rhabdomyosarcomas from previously untreated patients as well as sublines selected in vivo for resistance to vincristine and melphalan, and three lines of childhood osteogenic sarcoma. Efficacy was determined at maximal tolerated dose levels using intermittent i.p. administration [every 4 days for 4 doses (q4dx4)] or daily p.o. or i.p. administration 5 days per week for up to 20 courses. On a q4dx4 schedule, the maximum tolerated dose (MTD) was 12.5 mg/kg per administration, which caused marked weight loss and lethality in approximately 5% of the tumor-bearing mice. This schedule caused significant growth inhibition (but no tumor regression) in advanced adult colon adenocarcinomas. The minimal treated/control (T/C) ratios were 0.49, 0.54, and 0.3 for three of the tumor lines and were achieved at 18-21 days after the initiation of treatment. In contrast, rhabdomyosarcomas were considerably more sensitive, with T/C ratios being < 0.1 for three lines, whereas topotecan was less active against two other rhabdomyosarcoma xenografts (minimal T/C ratios, 0.17 and 0.14). As inhibitors of topoisomerase I have been demonstrated to have activity in the replication phase of the cell cycle (S-phase-specific), prolonged administration schedules were examined. Mice received topotecan 5 days per week for 3 weeks either by i.p. injection or by oral gavage (p.o.). In selected experiments, p.o. administration was continued for up to 20 weeks. Oral administration for 3 weeks (2 mg/kg per dose) resulted in complete regression of all six lines of rhabdomyosarcoma, with two lines demonstrating no regrowth during the period of observation (> or = 84 days). Similar results were obtained after i.p. administration, suggesting significant schedule dependency for these tumors. For colon tumors, the daily administration schedule (i.p. or p.o.) demonstrated some advantage over the intermittent schedule, resulting in partial regressions and significant inhibition of the growth of several colon adenocarcinoma lines. In rhabdomyosarcoma Rh12 and VRC5 colon adenocarcinoma, both of which demonstrated intermediate sensitivity to topotecan, and in osteosarcoma OS33, protracted p.o. administration for 13-20 weeks (1.0-1.5 mg/kg per dose given daily x 5 days) caused complete regression without regrowth in Rh12 and OS33 tumors and partial regression of all VRC5 tumors. No toxicity was observed using this schedule of administration. Topotecan demonstrated significant activity against all three osteosarcoma xenografts examined, with optimal schedules causing complete regression in two lines.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Evaluation of 9-dimethylaminomethyl-10-hydroxycamptothecin against xenografts derived from adult and childhood solid tumors. 146 61

Amplification and rearrangement of cellular proto-oncogenes are two of the several possible genetic alterations implicated in carcinogenesis and tumor progression. Although morphologically similar tumors may be heterogeneous at the level of the genome, some tumor types have shown relatively frequent and consistent abnormalities of specific oncogenes. In order to determine the frequency of oncogene amplification and rearrangement in several types of human sarcomas and to determine if histologically similar tumors have common genetic alterations, we analyzed 26 primary sarcomas by Southern hybridization. The oncogene probes utilized were N- and H-ras, sis, EGF-R (erb-B-1), neu (erb-B-2), fos, N- and c-myc, mos, and yes. The tumors studied included: five rhabdomyosarcomas (one alveolar, four embryonal), six malignant fibrous histiocytomas, six leiomyosarcomas, four liposarcomas, two Ewing's sarcomas, one osteosarcoma, and two fibrosarcomas. Oncogene abnormalities were identified in three tumors. One rhabdomyosarcoma showed 12-fold amplification and concurrent rearrangement of sis. This particular tumor also revealed rearrangement of H-ras and 15-fold amplification of c-myc. A second rhabdomyosarcoma revealed rearrangement of neu. A liposarcoma had a sis rearrangement. These findings suggest that many sarcomas show no common structural oncogene abnormalities. The presence of differing oncogene alterations within the rhabdomyosarcoma group indicates genetic heterogeneity among histologically similar sarcomas. The finding of a sis rearrangement in both a liposarcoma and a rhabdomyosarcoma, however, may suggest common oncogenesis among different tumor types.
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PMID:Genomic alterations in sarcomas: a histologic correlative study with use of oncogene panels. 149 46

Nine patients who had an osteosarcoma that had developed as a second malignant neoplasm in a previously irradiated site were managed at a major center for the treatment of tumors in children. The doses of radiation had averaged 4144 centigray (range, 2300 to 8000 centigray) and chemotherapy had been administered, when appropriate, for the primary malignant lesion (Ewing sarcoma, malignant fibrous histiocytoma, Hodgkin lymphoma, neuroblastoma, neurofibrosarcoma, rhabdomyosarcoma, and Wilms tumor). The interval between the initial treatment and the diagnosis of the secondary sarcoma averaged ten years and one month (range, five years and ten months to twenty-one years and nine months). Three patients were alive, two of them with active disease, at the time of writing. The other six had died within three years (average, fifteen months) after the second diagnosis. The prevalence of secondary osteosarcoma is increasing as the survival of children who have a malignant lesion increases. Plans for tumor therapy should take into account the risk of this complication, which is usually fatal.
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PMID:Osteosarcoma as a second malignant neoplasm in children. 152 94

Tumours of mixed glial and sarcomatous elements occurring in intracranial neoplasms are well recognised and have been termed gliosarcomas. These tumours account for up to 8% of all glioblastomas. The sarcomatous elements are thought to derive from the neoplastic transformation of mesenchymal cells in or adjacent to the tumour. This transformation usually has the appearance of a fibrosarcoma or angiosarcoma. Alternative mesenchymal neoplastic differentiation may occur, however, giving rise to the appearances of chondrosarcoma and osteosarcoma. In 1969 Goldman described a case in which the sarcomatous elements of a mixed gliosarcoma appeared, on the basis of light microscopy alone, to differentiate towards skeletal muscle having the features of a rhabdomyosarcoma. He coined the term gliomyosarcoma. In 1986 Barnard et al reported a second case and demonstrated the features of rhabdomyosarcoma using the electron microscope. A further case characterised with both light microscopic and immunohistochemical techniques is reported.
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PMID:Gliomyosarcoma: an immunohistochemical analysis. 152 49


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