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)

Primary pulmonary tumors are infrequent in childhood, therefore an accurate diagnosis and treatment is often delayed. We review the English language literature and report the clinical and pathological features of eight tumors arising in the lungs of pre-adolescent children, accessioned between 1960 and 1991 in the pathology department of a children's hospital in South Africa. The ratio of pulmonary primary tumors to secondary neoplasms and to non-neoplastic lesions of the lung examined during this period was 1:5:60. Over the last 31 years we received three plasma cell granulomas, two pleuro-pulmonary blastomas, a mucoepidermoid carcinoma, an endobronchial fibrosarcoma, and a hemangioma. All patients presented with cough unresponsive to medical treatment. The incidence and spread of primary lung tumors in children was similar to that reported from other centers. Plasma cell granuloma is the most common primary tumor in the lungs of children. Aggressive behavior is most frequently encountered with pleuro-pulmonary blastoma and rhabdomyosarcoma, and because of their association with cystic lesions careful examination of lungs is required in such cases. Most other malignant neoplasms, such as muco-epidermoid carcinoma and primary fibrosarcoma, are usually of a low grade of malignancy. A decreasing incidence of bronchogenic carcinoma seems to be reported during the first two decades of life.
Pediatr Pulmonol 1992 Dec
PMID:Primary pulmonary tumors in childhood: a review of 31 years' experience and the literature. 133 97

Antibodies generated against the whole membrane preparation isolated from rhabdomyosarcoma RA-2 cells were shown by immunoblotting and immunoaffinity chromatography to recognize 58-kDa polypeptide, p58. The latter was confirmed to be a surface molecule in a test of radioiodination of RA-2 membrane by lodogen. The antibodies added to a suspension of RA-2 cells before their inoculation into rats decreased metastatic activity 50-fold without any noticeable influence on RA-2 proliferation level and viability. The data indicate that masking of p58 surface antigen by antibodies could make RA-2 cells unable to form experimental metastases in lung. We suggest that p58 may participate in the specific recognition by RA-2 of lung endothelial cells.
Int J Cancer 1992 Dec 02
PMID:Antibody against p58 surface antigen of RA-2 rat rhabdomyosarcoma cells inhibits their metastatic activity. 145 31

We report a case of rhabdomyosarcoma involving the mitral valve of a 57-year-old female. She was referred to our hospital for progressive orthopnea and edema. Chest X-ray showed marked cardiomegaly and pulmonary congestion. Echocardiogram revealed solid mass in left atrium involving the mitral valve. Emergency operation was required because of acute heart failure. At the operation, the mitral orifice was obstructed by the tumor arising from the left atrium. After partial resection of the left atrium, mitral valve replacement with a Carpentier-Edward prosthesis was performed. Subsequently microscopic examination diagnosed as rhabdomyosarcoma. She died three months after the operation of heart failure probably due to progression of the remnant of the tumor. This, to our knowledge, is the first case of mitral valve replacement for a primary rhabdomyosarcoma of the heart in Japan.
Kyobu Geka 1992 Dec
PMID:[A case report of primary rhabdomyosarcoma of the heart treated with mitral valve replacement]. 147 94

Shell vials (SV) and conventional tubes (CT) were seeded with rhabdomyosarcoma (RD) and MRC-5 cells and inoculated with clinical specimens, and the systems were evaluated for the rapid diagnosis of herpes simplex virus (HSV) infections by detection of cytopathic effects (CPE) (for CT, for 7 days) and by using fluoresceinated monoclonal antibodies (for SV, 16 h postinoculation). Of 245 genital specimens (16 from males and 229 from females) 56 (23%) seeded with MRC-5 cells (14 type 1 and 42 type 2) and 55 (22%) seeded with RD cells were detected in CT; however, CPE were recognized in only 26 (46%) of the total HSV-positive cultures 1 day postinoculation. Forty-eight (86% sensitivity, MRC-5) and 46 (84% sensitivity, RD) HSV strains were detected immunologically in SV 16 h postinoculation. Early CPE in CT or fluorescent foci in SV were easier to detect in MRC-5 than in RD cell cultures. MRC-5 and RD cells were equally sensitive to infection with HSV. CT cell cultures were more sensitive than SV but less rapid for the detection of HSV infection (P less than 0.01). We recommend using SV for the rapid diagnosis of HSV infections, but in addition, CT must be inoculated with MRC-5 or RD to ensure maximum detection of this virus.
J Clin Microbiol 1991 Dec
PMID:Comparison of shell vials and conventional tubes seeded with rhabdomyosarcoma and MRC-5 cells for the rapid detection of herpes simplex virus. 166 44

We examined pulmonary carcinomas with prominent sarcoma-like lesions both clinicopathologically and immunohistochemically. Grossly, two tumors had predominantly endobronchial growths, four bulky parenchymal growths, and two endobronchial, parenchymally mixed growths. In these eight patients, six tumors were completely resected, one patient was given irradiation only, and one patient died in the early postoperative period. On the basis of specific differentiation of the sarcoma-like lesions, the tumors were separated into three groups: two with "true" sarcoma differentiated into soft tissues such as striated muscle or osteoid tissue; three with a fibromatous sarcoma resembling atypical pseudosarcomatous stroma; and three with spindle cell carcinoma with evidence of epithelial differentiation. The prognosis was poor, and tumors with specific differentiation into rhabdomyosarcoma, chondrosarcoma, or spindle cell carcinoma progressed more rapidly than did those with a fibromatous sarcoma. Because the fibromatous sarcoma-like lesions were found to relate to a longer survival time for the patients, we wish to emphasize that a distinction of sarcomatous components should be made with regard to assessing the prognosis of pulmonary carcinoma with sarcoma-like lesions.
J Thorac Cardiovasc Surg 1990 Dec
PMID:Carcinosarcoma and spindle cell carcinoma of the lung. Clinicopathologic and immunohistochemical studies. 170 Oct 11

Northern analysis of tumour RNA has been used to examine the expression of members of the myf family of muscle determining genes (myf3, myf4, myf5 and myf6) in a series of 20 rhabdomyosarcomas. A 2.0 kb myf3 transcript was observed in 85% of tumours, a 1.8 kb myf4 transcript was detected in 70% of tumours and a 1.7 kb myf5 transcript was observed in 55% of tumours. Transcription of myf6 occurred in 28% of tumours, but there were several transcript sizes (1.2, 1.5, 2.0 and 3.5 kb) and in some individual tumours two or more transcripts were observed. Only two rhabdomyosarcomas, one classified as embryonal and one as pleomorphic, failed to exhibit transcription of members of the myf gene family. We were unable to detect transcription of myf genes in neuroblastomas, Wilms' tumours, hepatoblastomas, paediatric non-Hodgkin's lymphoma and leiomyosarcomas. When considered together these observations suggest that expression of myf genes could provide an extremely useful marker in the diagnosis of rhabdomyosarcoma.
Br J Cancer 1991 Dec
PMID:Expression of members of the myf gene family in human rhabdomyosarcomas. 176 65

We have previously reported the existence of 2 forms of mRNA for the human muscle acetylcholine receptor (AChR) alpha-subunit, thought to be generated by alternate splicing of a primary transcript and to encode 2 alpha-subunit protein isoforms. The 2 predicted alpha-subunit isoforms, differing by the insertion of 25 amino acids at position 58/59, have been synthesized from cRNA transcripts using rabbit reticulocyte lysates; these protein isoforms could be differentiated by immunoprecipitation using antibodies raised against synthetic peptides. The antibodies were used to demonstrate translation of both AChR alpha-subunit isoforms in the rhabdomyosarcoma (muscle) cell line TE671, in an approximate 1:1 ratio.
FEBS Lett 1991 Dec 16
PMID:Two isoforms of the muscle acetylcholine receptor alpha-subunit are translated in the human cell line TE671. 176 41

Adjuvant therapy is currently established in the treatment of osteosarcoma, Ewing's sarcoma and rhabdomyosarcoma. Of the 12 reported randomized studies of adjuvant chemotherapy for soft tissue sarcoma, only 2 show a significant overall survival advantage for chemotherapy (the most important endpoint). In three randomized trials, the survival of the observation arm exceeds that of the chemotherapy arm. In two additional studies, subset analyses currently indicate a significant DFS advantage for adjuvant chemotherapy in extremity lesions, but no significant improvement in survival. Although initial NCI reports showed significantly prolonged survival for the subset of chemotherapy-treated extremity primaries, survival on longer follow-up is no longer significantly different. In the subset analysis of retroperitoneal sarcomas in the same NCI study, the survival of the control group is superior to the treatment group. Doxorubicin associated cardiotoxicity has occurred in about 10% of treated patients, occasionally contributing to treatment-related deaths. Based on these data, adjuvant chemotherapy should be considered investigational for adult soft-tissue sarcomas of any primary site. Future randomized trials should include patients at high risk for metastases (large, high-grade lesions) with a reasonable likelihood of local control by radical resection, or resection with uninvolved margins and subsequent radiotherapy. Low-grade sarcomas are currently cured by surgical resection in 80% of cases, and thus should not be included in adjuvant trials.
Semin Oncol 1991 Dec
PMID:Adjuvant therapy for sarcomas. 177 77

Previous investigations of the pediatric soft tissue tumor alveolar rhabdomyosarcoma have identified a characteristic translocation t(2;13)(q35;q14). We have employed a physical mapping strategy to localize the site of this translocation breakpoint on chromosome 13. Using a panel of somatic cell hybrid and lymphoblast cell lines with deletions and unbalanced translocations involving chromosome 13, we have mapped numerous probes from the 13q12-q14 region and demonstrate that this region is divisible into five physical intervals. These probes were then mapped with respect to the t(2;13) rhabdomyosarcoma breakpoint by quantitative Southern blot analysis of an alveolar rhabdomyosarcoma cell line with two copies of the derivative chromosome 13 and one copy of the derivative chromosome 2. Our findings demonstrate that the t(2;13) breakpoint is localized within a map interval delimited by the proximal deletion breakpoints in lymphoblast lines GM01484 and GM07312. Furthermore, the breakpoint is most closely flanked by loci D13S29 and TUBBP2 within this map interval. These findings will facilitate chromosomal walking strategies for cloning the regions disrupted by the alveolar rhabdomyosarcoma translocation. In addition, this physical map will permit rapid determination of the proximity of new cloned sequences to the translocation breakpoint.
Genomics 1991 Dec
PMID:Localization of the rhabdomyosarcoma t(2;13) breakpoint on a physical map of chromosome 13. 178 2

Among a cohort of 981 children who were followed up 4.3-26.5 years after cessation of antileukemic therapy, eight patients in remission of acute lymphoblastic leukemia (ALL) developed a distinctively new malignant disease. The second malignant neoplasms (SMN) included brain tumors, basal cell carcinomas, thyroid cancer, leiomyosarcoma and finally rhabdomyosarcoma in a patient who also had suffered from Hodgkin's disease while still on antileukemic treatment. Cranial radiation had been given to 58.4% of the patients in the study group, which consisted of 895 ALL patients who had completed various chemotherapy protocols. With one exception, the SMN appeared after 7.5-16.5 years at a location previously exposed to radiotherapy (RT). The estimated cumulative risk of SMN appearing within 20 years after diagnosis was 2.9%, and the corresponding risk for cases with RT was 8.1% compared to 0.3% for those without (p = 0.05). In a Cox regression analysis, the incidence rate ratio of SMN between patients with and without RT was 6.7 (95% CI = 0.8, 57.7). Based on age-, year- and sex-specific cancer incidence figures for Norway, the overall standardized incidence rate ratio (SIR) of SMN after treatment for ALL was 5.9 (95% CI = 2.2, 12.9). The number of brain tumors among patients who had received cranial radiation was nearly 27 times greater than expected, whereas no such tumors were seen after chemotherapy. Individuals treated for childhood ALL are at increased risk of a new malignancy, and this seems mainly to be associated with previous irradiation.
Acta Paediatr Scand 1991 Dec
PMID:Second malignant neoplasms in patients treated for childhood leukemia. A population-based cohort study from the Nordic countries. The Nordic Society of Pediatric Oncology and Hematology (NOPHO). 178 95


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