Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The outlook for children with rhabdomyosarcoma has change significantly in these last years. With an adeguate combined modality therapy more than 50% of these children may be cured. The results of the IRS-I indicate that the 3 year relapse-free survival rates are 85% for patients in group I 70% for those in group II, 45% for those in group III and 15% for those in group IV. In addition to the clinical group other significant prognostic factors are histologic cell type (alveolar, unfavorable) and primary site (disease in extremities and in retroperitoneal area, unfavorable). The chemiotherapy must be used in all patients for 12-24 months. The effective drugs are VCR, ACT-D, CTX, ADR combined in different schedules. It has been demonstrated that the effective doses of radiotherapy range from 4000 to 5000 rad and that radiotherapy may be omitted in patients in group I. Now a less aggressive surgical procedures may be employed, and patients with primary tumor in the orbit or in the pelvic organs may be cured saving the eye, or the bladder, the vagina and the uterus.
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PMID:[Rhabdomyosarcoma: therapeutic results and prospectives]. 654 8

Rhabdomyosarcoma is a highly malignant, small blue cell tumor characterized by muscle differentiation. With modern treatment, more than 70% of children and adolescents with this disease are cured. Adequate biopsy to obtain sufficient tissue for accurate diagnosis and molecular characterization is critical. Patients must be assessed for tumor extent; the Intergroup Rhabdomyosarcoma Study (IRS) clinical group and Staging system is universally applied in North America. Multidisciplinary therapy is necessary to maximize cure rates. Local control relies on complete surgical excision when possible; those whose tumors are not completely excised and those with alveolar histology tumors require local irradiation to maximize local control. In North America, vincristine (Oncovin); Eli Lilly and Company, Indianapolis, http://www.lilly.com), dactinomycin (Cosmegen); Merck & Co., Inc., Whitehouse Station, NJ, http://www.merck.com), and cyclophosphamide are the standard chemotherapy agents. The IRS has used therapeutic window studies to confirm the predictive nature of preclinical xenograft models and to identify several new single agents and combinations of agents with activity in high-risk patient groups. Despite these efforts, the outcome for these high-risk patients remains poor. The next generation of Children's Oncology Group studies will evaluate the efficacy of topoisomerase-I inhibitors and dose-compression therapy approaches. New advances in molecular characterization of tumors, including gene-expression analysis, may identify new therapeutic targets that can be exploited by expanded preclinical drug discovery efforts, and hold the promise of revolutionizing risk-based therapies.
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PMID:Rhabdomyosarcoma: new windows of opportunity. 1607 19