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)

Forty-five consecutive cases of pediatric rhabdomyosarcoma were studied as to age at onset, histological type, primary site, stage of disease when first seen, method of treatment, and survival. It appears that rhabdomyosarcoma is a relatively radiosensitive tumor which can be controlled locally with radiation alone or in combination with surgery to excise the bulk of the tumor mass. On the other hand, chemotherapy must necessarily play a major role in treatment since most cases are first seen in the late stages of the disease. Close cooperation among a wide range of specialists, such as the pediatrician, radiation therapist, pediatric oncologist, pathologist, and surgeon is essential to accomplish a maximum therapeutic effect in the unfortunate children who develop this relatively rare but aggressive tumor.
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PMID:Rhabdomyosarcoma in the pediatric age group. 17 1

Intracellular collagen was detected by electron microscopy in 14 sarcomas including six osteogenic sarcomas, three liposarcomas, three malignant fibrous histiocytomas, one pleomorphic rhabdomyosarcoma, and one childhood rhabdomyosarcoma. It was contained in not only the fibroblastic cells, but also in the osteoblastic, lipoblastic, myofibroblastic, and primitive cells of the various tumors. The banded intracellular collagen fibrils were observed in large phagocytic vesicles and in smaller membrane-bound vesicles which also appeared to fuse with lysosomes. Residual banding could be seen as well in many such phagolysosomes. Banded collagen was also noted in a primary explant in tissue culture. These findings suggest that the configurations of intracellular collagen seen, are parts of a continuum of a secondary pathway of collagen degradation in mesenchymal tissue and that pathway is one factor indicating a close interrelationship between these sarcomas.
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PMID:Intracellular collagen fibrils in human sarcomas. 21 51

Twenty-six cases of childhood rhabdomyosarcoma involving the pelvis were treated at Childrens Hospital of Los Angeles between 1950 and 1972. The median survival of 17 patients treated up to 1970 was 6 months. Of a group of 9 patients treated with intensive chemotherapy (ICG) prior to surgery and/or x-ray therapy, 5 are surviving from 12 to 60 months and have a relative survival rate of 4.5 times that of the mixed therapy group (MTG) (p = 0.01). Deaths have occurred only in patients with Stage IV disease. The use of preoperative chemotherapy with or without x-ray therapy followed by local surgical excision when necessary can be as effective in prolonging the survival of children with pelvic rhabdomyosarcoma as those obtained with more traditional approaches; this limitation in use of surgery is likely to preserve the quality of life, should the child live to adulthood.
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PMID:Intensive chemotherapy as primary treatment for rhabdomyosarcoma of the pelvis. 119 51

A study of 77 consecutive cases of childhood rhabdomyosarcoma treated initially at the Children's Hospital of Los Angeles from 1950 to 1972 demonstrated an impressive improvement in survival of patients when intensive chemotherapy was occurred in Stage II and Stage III disease. Eight out of 9 patients with a primary lesion of the lower extremities developed intra-abdominal disease. A general approach for treatment of Stage II, III, and IV rhabdomyosarcoma is proposed which involves intensive chemotherapy, following biopsy, as the principle treatment, with radiation and surgery used as a adjuvants.
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PMID:The influence of chemotherapy on the prognosis of rhabdomyosarcoma. 123 31

Human tumor cell lines that do not express O6-methylguanine-DNA methyltransferase (MGMT) in detectable quantities (Mer-) are hypersensitive to the effects of O6-guanine-alkylating agents. Because the Mer- phenotype enhances tumor response to such agents, we investigated possible mechanisms involved in regulation of MGMT expression in a panel of Mer+ and Mer- pediatric rhabdomyosarcoma xenograft and cell lines. All Mer- cell and xenograft lines lacked not only MGMT activity but also the protein and mRNA as well, suggesting that its expression is transcriptionally regulated. Transfection of Mer+ and Mer- rhabdomyosarcoma cell lines with MGMT gene promoter-CAT constructs yielded similar levels of CAT expression, indicating that Mer- cells possessed the necessary factors to support transcription. Methylation in the 5'-untranslated region of the MGMT gene was assayed by Southern analysis using methylation-sensitive restriction enzymes. Digestion with HpaII and its methylation-insensitive isoschizomer, MspI, revealed little overall correlation between methylation and MGMT expression. However, methylation in a single SmaI site at position-69 was observed in all MGMT deficient lines but not in any MGMT expressing lines. These results suggest that methylation of specific cytosines in the MGMT promoter may play a role in suppressing its expression, as well as being a potentially useful marker for the Mer- phenotype.
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PMID:Cytosine methylation and suppression of O6-methylguanine-DNA methyltransferase expression in human rhabdomyosarcoma cell lines and xenografts. 150 76

Rhabdomyosarcoma is an often primitive tumor capable of diverse morphologic manifestations. The article describes three cases of childhood rhabdomyosarcoma in which a significant population of lipid-rich tumor cells was present. The lack of specificity and potentially confusing nature of this feature are discussed, and the ultrastructural diagnosis of rhabdomyosarcoma is briefly reviewed.
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PMID:Lipid-rich rhabdomyosarcoma--a potential source of diagnostic confusion. 175 99

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

Issues in diagnosis and complex treatment of pediatric rhabdomyosarcoma were studied in 198 patients with morphologically verified disease. Tumors most often developed in the genitourinary organs (36.6%), head and neck (37%) and--less frequently--on the trunk and extremities (26.4%). The diagnostic workup included instrumental methods, ultrasonography and computed tomography. All modern modalities of cancer treatment, viz. surgery, drug and radiotherapy were used in those patients. As a rule, treatment was either combined or complex. Two-year-survival rate ranged from 83% for rhabdomyosarcoma of the orbit to 50% for those of the trunk and extremities and 47% for head and neck neoplasms. Two-year survival for patients with rhabdomyosarcoma of non-genitourinary sites was 54%.
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PMID:[Rhabdomyosarcoma in children]. 228 35

In an attempt to evaluate the radiocurability of microscopic disease in childhood rhabdomyosarcoma (RMS) with total tumor doses of less than 4,000 cGy, we performed a retrospective analysis of all patients with microscopic residual RMS who were treated at the Memorial Sloan-Kettering Cancer Center (MSKCC) during the years 1970 to 1987. There were 32 patients ranging in age from 3 months to 22 years (median, 6 years) with microscopic residual of either (1) a localized primary tumor (MSKCC, stage IB; Intergroup Rhabdomyosarcoma Study [IRS] group IIA), 19 patients; or (2) an involved lymph node region with the primary tumor completely resected (MSKCC stage III; IRS group IIC), 13 patients. Twenty-nine of the 32 patients presented with embryonal histology. All patients were treated with combination chemotherapy (CT) and megavoltage external beam radiotherapy (RT). The RT was delivered in either conventional fractionation of 180 to 200 cGy daily (30 patients) or hyperfractionation of 150 cGy twice daily (two patients). Fifteen patients received RT doses of less than 4,000 cGy with a range of 3,000 to 3,600 cGy and a median value of 3,100 cGy; 17 patients received 4,000 cGy or more with a range of 4,000 to 6,000 cGy and a median value of 4,600 cGy. With a median follow-up of 11 years, the relapse-free survival was 25 of 32 patients (less than 4,000 cGy, 12 of 15; greater than or equal to 4,000 cGy, 13 of 17). The RT local control rate was 30 of 32 (less than 4,000 cGy, 14 of 15; greater than or equal to 4,000 cGy, 16 of 17 [P = .94]). Our results suggest that radiation doses of below 4,000 cGy, when combined with effective multiagent CT, may be sufficient for local control of microscopic disease in childhood embryonal RMS.
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PMID:Radiocurability of microscopic disease in childhood rhabdomyosarcoma with radiation doses less than 4,000 cGy. 239 58

Five hundred thirteen soft tissue tumours of childhood referred to the International Society of Pediatric Oncology (SIOP) Rhabdomyosarcoma study have been reviewed. The period covered was from January 1975 to December 1983. Three hundred thirty-nine neoplasms were regarded as embryonal rhabdomyosarcoma. The histological diagnosis of all referred tumours is given, and a classification of rhabdomyosarcomas developed during the course of the study is described. The relationship between histological subtypes and aspects of clinical behaviour is presented. The classification is thought to be helpful in diagnosis and also in assessment of the likelihood of recurrence and/or metastasis of childhood rhabdomyosarcoma.
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PMID:Histopathological classification of childhood rhabdomyosarcoma: a report from the International Society of Pediatric Oncology pathology panel. 247 74


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