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)

The treatment of soft tissue sarcomas in children at the Joint Center for Radiation Therapy, Children's Hospital Medical Center, and the Sidney Farber Cancer Institute from 1970 to 1976 has been reviewed. Twenty-seven patients were diagnosed with rhabdomyosarcoma, and twenty patients were diagnosed with soft tissue sarcomas of other histologies. An aggressive, combined modality therapeutic approach was applied in the treatment of all patients with emphasis placed on conservation of function. Of irradiated patients, local control was achieved in 96% of those with rhabdomyosarcoma and 85% in other sarcomas. Cumulative relapse-free survival (actuarial) at 5 years is projected at 65% for the rhabdomyosarcoma patients and at 63% for the other sarcoma patients. Although there were differences in chemotherapy regimens (vincristine, actinomycin-D and cyclophosphamide for rhabdomyosarcoma and adriamycin and DTIC for other soft tissue sarcomas), the surgical and radiation therapeutic approaches are similar for both groups. The high probability of local control using function-conserving surgery and high dose radiation therapy supports this emerging approach. Improvements in survival will require better control of metastatic disease.
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PMID:The role of radiation therapy in the treatment of soft tissue sarcomas of childhood. 10 Feb 8

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

As the adjuvant chemotherapy of osteosarcoma has been proved highly effective, that of soft tissue sarcoma has been expecting. However, there is still some question as to its effectiveness. The indication and methods were studied on literatures and our own cases. A survey of the literature regarding to the chemotherapeutic effect upon advanced soft tissue sarcoma shows response rate ranging 20 to 50 per cent. On the other hand, soft tissue sarcoma has a variety of histological type and malignancy and the sensitivity to chemotherapy varies considerably according to each tissue type. The literatures and our results indicate that most effective sarcoma is rhabdomyosarcoma, which is absolutely advisable to apply adjuvant chemotherapy. The same is the sarcoma with similar histological pattern. As to other type sarcomas, the therapy has to be applied according to their grade, stage, age and the effect of chemotherapy evaluated by advanced tumor. Most prevalent agents used for soft tissue sarcoma are adriamycin, cyclophosphamide, actinomycin-D, vincristine and DTIC. These agents usually used as combination called VAC, CYVADACT, CYVADIC, BCD.
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PMID:[Adjuvant chemotherapy of soft tissue sarcoma]. 230 50

As part of two sequential protocols using intensive combined modality treatment in pediatric and adolescent sarcomas, 31 consecutive patients with primary chest wall tumors were treated between November 1977 and March 1986. This group included 13 patients with peripheral neuroepithelioma (Askin's tumor), 11 patients with Ewing's sarcoma, 3 patients with rhabdomyosarcoma, and 4 patients with undifferentiated sarcomas. Following complete work-up, 17 patients presented with localized disease and 14 patients presented with metastases. Patients received intensive combined modality treatment with combination chemotherapy (vincristine, cyclophosphamide, Adriamycin, +/- actinomycin-D and DTIC) and high-dose conventionally fractionated radiation therapy to the primary (55-60 Gy) and non-pulmonary metastases (45-50 Gy). Radiation techniques used for the primary chest wall tumor varied with the clinical presentation. Patients achieving a complete response received either low-dose fractionated TBI (1.5 Gy/0.15 Gy fx/5 weeks) or high-dose TBI (8 Gy/4 Gy fx/2 days) and an intensive cycle of chemotherapy followed by autologous bone marrow transplantation. Twenty-five of 31 patients were judged to have a complete response (including 1 patient with complete resection). With minimum follow-up of 6 months and median follow-up of 36 months from completion of treatment, 14 patients remain disease-free with 2 additional patients alive in second remission after relapse. Patients with localized disease at presentation have improved disease-free survival and overall survival compared to patients with metastases at presentation. All 17 localized patients achieved a CR and 11 are NED compared to 8 of 14 metastatic patients achieving a CR and only 3 are NED. There have been 5 loco-regional recurrences with 3 "in-field" failures and 2 failures in the regional pleura. There were no treatment-related deaths and no clinically significant cases of pneumonitis. To date, 2 patients have significant treatment related morbidity, including 1 patient with scoliosis requiring surgery and 1 patient with acute leukemia developing 42 months after the start of therapy (presently in remission). We conclude that this intensive combined modality therapy results in a high CR rate and good local control with acceptable morbidity. Patients with metastatic disease at presentation remain a therapeutic challenge.
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PMID:Treatment of sarcomas of the chest wall using intensive combined modality therapy. 264 97

Sarcomas of childhood rank fifth in incidence of malignant tumors in children younger than 15 years. Among the soft tissue sarcomas, approximately 50% are rhabdomyosarcomas. The remainder represent a heterogeneous group of diverse sarcomas which are not unique to children and include fibrosarcoma, synoviosarcoma, malignant fibrous histiocytoma, malignant schwannoma, angiosarcoma, leiomyosarcoma, and others. The most common bone cancers in childhood are osteosarcoma and Ewing's sarcoma. Although a multidisciplinary approach utilizing surgery, irradiation, and combination chemotherapy is routinely used in management of virtually all children with solid tumors, the value of adjuvant chemotherapy in select bone and rare soft tissue sarcomas is currently being tested. Multiagent chemotherapy including vincristine, dactinomycin, cyclophosphamide, and Adriamycin (doxorubicin) contribute to cure rates in 65% to 75% of children with localized rhabdomyosarcoma, Stages I to III, when combined with surgery and/or irradiation. Other drugs which hold promise include platinum, DTIC, methotrexate, and VP-16. The efficacy of similar drugs in the rarer pediatric soft tissue sarcomas other than rhabdomyosarcoma and its variants requires prospective randomized trials evaluating histologic grade, tumor size, and nodal status. It has been suggested that the high-grade sarcomas presenting with minimal tumor bulk are most sensitive to combined radiotherapy-chemotherapy, whereas the low-grade sarcomas are more resistant to such therapy. Tumor cell heterogeneity contributes to biologic diversity and response to treatment. Chemotherapy as adjuvant therapy to irradiation is currently recommended and utilized for Ewing's sarcoma with survival rates approaching 80%, and disease-free survival of approximately 75% for those with localized disease. Children with widespread and metastatic disease at presentation fare less well. Although multiple single agents exhibit response rates ranging from 40% to 60%, including cyclophosphamide, Adriamycin, dactinomycin, BCNU, mithramycin, and 5-fluorouracil, new and more effective agents are needed. Controversy regarding the value of multiagent chemotherapy in osteosarcoma has stimulated prospective randomized trials. Evaluation of local control rates as well as sites and occurrence of metastases are essential in assessing the contribution of aggressive combined modality therapy in the pediatric sarcomas. Emphasis on refinement of therapy in determining the risk/benefit ratio from adjuvant chemotherapy in pediatric sarcomas is mandatory. Enhancement of early local reactions is apparent when adjuvant chemotherapy is used with local radiotherapy.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:The value of adjuvant chemotherapy in the management of sarcomas in children. 388 37

A case report of a Stage III botryoid rhabdomyosarcoma of the nasopharynx associated with a six-and-a-half-year survival is presented. Treatment consisted of surgery, radiotherapy (6,000 rads TCT) to the nasopharynx and maxillary sinuses bilaterally, and six cycles of polychemotherapy with Vincristine, Adriamycin, Cyclophosphamide and DTIC, without major loss of function or cosmetic deformity. The histology of the lesion is discussed with reference to recent classification and prognosis. The authors suggest that the histological type and prognosis of rhabdomyosarcoma of the nasopharynx in children may be better correlated in future studies.
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PMID:Botryoid rhabdomyosarcoma of the nasopharynx. 686 97

We have reviewed our experience using single-dose dactinomycin (Act D). Twenty-nine patients with Ewing's sarcoma or rhabdomyosarcoma received 114 courses of Act D (2 mg/m2) in combination with vincristine, cyclophosphamide, or DTIC with or without radiotherapy. Side effects included nausea and vomiting (100% of the courses), severe thrombocytopenia (16%), granulocytopenia (18%), and mucositis (25%). In addition, skin reactions were observed in 74% of the courses following concomitant radiotherapy. These results were compared with those following 152 courses of combination chemotherapy which contained doxorubicin in place of Act D and which were administered to these same patients. It is concluded that single-bolus Act D is no more toxic, is at least as effective, and is more conveniently administered than the traditional divided daily dose of Act D.
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PMID:Tolerance to single-dose dactinomycin in combination chemotherapy for solid tumors. 722 64

One hundred and forty adult patients with advanced sarcomas (125 soft tissue and 15 bone) were treated with a combination chemotherapy regimen consisting of cyclophosphamide, vincristine, Adriamycin, and DTIC (CYVADIC). There were 21 (15%) complete and 45 (32%) partial responders, with an overall response rate of 47%. The response rate was 50% (17% complete) for patients with soft tissue sarcomas compared with 20% (none complete) for patients with bone sarcomas. The median duration of response was 9.5 months (range, 1-40+ months) for complete responders and 7 months (range, 1-31 months) for partial responders. The median time to achieve response was 9 weeks and the median number of courses of therapy to response was three. The median survival time was 16 months for responders compared with 7 months for nonresponders (P = 0.001). The most responsive tumor types were neurofibrosarcoma, rhabdomyosarcoma, leiomyosarcoma, fibrosarcoma, and angiosarcoma. Pulmonary and soft tissue metastases were more responsive than bone and liver metastases. CYVADIC toxicity was predominantly limited to myelosuppression, vomiting, fever of unknown origin, and neuropathy. CYVADIC is an effective combination chemotherapy regimen in the treatment of advanced sarcomas.
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PMID:Cyclophosphamide, vincristine, adriamycin, and DTIC (CYVADIC) combination chemotherapy for the treatment of advanced sarcomas. 737 60

The role of chemotherapy for soft tissue sarcoma with the exception of rhabdomyosarcoma remains controversial. Several randomized trials have suggested only doxorubicin (ADR) and ifosfamide produced a single-agent response rate above 20% in advanced sarcoma. As a combination chemotherapy, the doxorubicin-based combination, ADR + DTIC (ADIC) and CYVADIC, showed a higher response rate. Ifosfamide in addition to doxorubicin (Ifos + ADR or ADIC) appeared to have major activity with a higher complication rate. The role and value of adjuvant chemotherapy have not yet been established. Most randomized studies have suggested that no survival benefit was observed in the chemotherapy group relative to the control group. Further basic and clinical investigation is necessary to obtain a better prognosis in high-grade malignant soft tissue sarcoma.
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PMID:[Chemotherapy for soft tissue sarcoma--current concepts and review]. 821 66