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)

A 2 1/2-year-old male child with rhabdomyosarcoma of the prostate was treated with radiation of the pelvis, consisting of 5,600 rads in 7 1/2 weeks, and combination chemotherapy of Vincristine and Actinomycin D. Surgery had been refused. The child is alive and disease-free more than 5 years after diagnosis, and over 2 years following the cessation of all therapy. Recent advances in radiotherapy and adjuvant chemotherapy have significantly improved the outlook in this aggressive childhood neoplasm and have made a less aggressive and less mutilating surgical approach often desirable. This, in turn, holds out the prospect of improved quality of survival in children so afflicted.
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PMID:Nonsurgical treatment of pelvic rhabdomyosarcoma: a case report. 59 58

The purpose of this study was to establish the optimal management of immature teratoma of the ovary. Pursuant to this, 20 previously untreated patients with immature teratoma were evaluated. Nine patients were at stage I of the disease, 2 had progressed to stage II, and 9 to stage III. Eight patients had grade 1 tumors, 11 had grade 2 tumors, and 1 had a grade 3 tumor. Postoperative chemotherapy was performed in 19 cases. Vincristine, actinomycin D, and cyclophosphamide (VAC) were administered in 9 cases, chemotherapy including cisplatin (P) was administered in 8 cases, and other regimens were followed in the 2 remaining cases. The median follow-up period was 62 months (range 19-108 months), and no patient was lost to follow-up. After completion of the follow-up period, 18 patients were alive and disease free, 1 was alive with liver metastasis, and 1 had died. The patient who died had suffered from a grade 3 tumor, and the recurrent tumor was a rhabdomyosarcoma. As a result of this study, it was found that immature teratoma of grades 1 and 2 can be managed successfully with VAC or P therapy. Thus, a hysterectomy should not be automatically performed in patients who still hope to give birth, yet suffer from a grade 1 or 2 immature teratoma at the time of a second operation.
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PMID:Immature teratoma of the ovary. 201 Jan 4

Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood and 20 per cent of these cancers involve the pelvis or genitourinary system. Radical pelvic surgery, such as exenteration, was considered at one time to be the standard treatment for this cancer which carried a very poor prognosis. Over the past 15 years, a combined modality approach to treating rhabdomyosarcoma, using chemotherapy, radiotherapy, and less radical surgery, has evolved and survival rates have improved. This paper presents a case of rhabdomyosarcoma involving the perineum which was treated by combination chemotherapy of Vincristine, Actinomycin-D, and Cytoxan, followed by wide local excision, interstitial and external beam radiotherapy, and postoperative chemotherapy. The literature on pelvic rhabdomyosarcoma is reviewed and the current approach to treating this cancer using multimodal therapy is discussed.
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PMID:Genital rhabdomyosarcoma: current management and review of the literature. 351 19

Forty-two children, 6 months to 17 years of age with newly diagnosed soft-tissue sarcomas (gross residual or metastatic), were treated according to either of two pilot protocols that included intensive chemotherapy before irradiation. Vincristine, actinomycin D, cyclophosphamide, and doxorubicin were used in various combinations with cisplatin alone (regimen 35) or with cisplatin plus etoposide (regimen 36) in a 20-week induction treatment; irradiation (4,000 cGy) was delayed until week 6. Fourteen (82%) of the 17 patients on regimen 35 and 15 (60%) of the 25 on regimen 36 had a complete response. Although severe leukopenia was frequent in both groups (88% and 84% of patients), there were only two fatal infections and no early deaths. Other potentially serious toxicity included a greater than 10% weight loss in 52% of the patients and hypomagnesemia in 74%. An average of 75-100% of the prescribed drug doses were administered during the induction phase of therapy. We conclude that this intensified treatment is toxic but feasible to deliver. The higher overall response rate compared to that in the preceding Intergroup Rhabdomyosarcoma Study (69% vs 53%) suggests improved therapeutic efficacy that warrants further evaluation of both regimens.
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PMID:Intensive chemotherapy including cisplatin with or without etoposide for children with soft-tissue sarcomas. 358 17

Seventy-nine patients with rhabdomyosarcoma (RMS) received treatment at the National Cancer Center Hospital between 1962 and 1985. The patients ranged in age from 4 months to 74 years with a median age of 6 years. Forty-six patients were male and 33 were female. The primary tumor site of RMS was the same as in the previous report. The head and neck region was the most frequent site (40.5%), followed by the extremities (34.1%), genitourinary region (15.2%), trunk (5.1%) and retroperitoneum (5.1%). Histologic types were embryonal RMS in 45 patients, alveolar RMS in 23 patients, pleomorphic RMS in 8 patients and unclassified RMS in 3 patients. As of October 1985, 14 of the 79 patients were still alive. Between 1962 and 1971, 38 patients were not treated by any protocol. After 1972, 41 patients received treatment using a 3 stage-related, multiple-modality program. In the first protocol, chemotherapy consisted of Vincristine, Cyclophosphamide, and Actinomycin-D, and 1 of 18 patients have survived more than 5 years. The cumulative 5-year survival rate of the first protocol was 11.1%. In the second treatment program, which involved Adriamycin in addition to the 3 drugs cited above, 4 of 23 patients have survived more than 5 years. The cumulative 5-year survival rate, 33.2%, was very improved.
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PMID:[Treatment of rhabdomyosarcoma at the National Cancer Center Hospital]. 359 4

Vincristine (VCR) is an effective agent in the treatment of childhood rhabdomyosarcoma. Clinically, schedules differ in frequency of administration. To determine the influence of administration frequency, accumulation of VCR in xenografts of human rhabdomyosarcoma has been evaluated following administration of drug at 7- or 21-day intervals. Accumulation was estimated from initial uptake, retention of unchanged drug in tumor tissues, tumor sensitivity, and growth rate. Data suggested that scheduling VCR every 7 days would be more effective than every 21 days, due to more rapid accumulation to cytotoxic levels. The effect of scheduling was examined in two rhabdomyosarcoma xenografts, where in vivo responses were similar to those predicted based upon drug uptake, retention, and growth characteristics for the tumors. Scheduling VCR at 7-day intervals was clearly superior to administration at 21-day intervals in these models.
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PMID:Scheduling of vincristine: drug accumulation and response of xenografts of childhood rhabdomyosarcoma determined by frequency of administration. 360 83

Adolescent and young adult patients with pelvic sarcomas continue to have a poor prognosis with standard combination chemotherapy and local irradiation. In addition to a significant risk of local failure, these patients are at high risk for systemic relapse. Twenty-three consecutive patients with Ewing's sarcoma, alveolar rhabdomyosarcoma, undifferentiated sarcoma, or malignant peripheral neuroepithelioma originating in the pelvis were treated with short, intensive combined modality therapy. This approach integrates 5 cycles of VADRIAC chemotherapy (Vincristine, Adriamycin, Cyclophosphamide) with high dose irradiation to the primary lesion (55-60 Gy) and sites of gross metastatic disease (45-50 Gy). Following achievement of a complete response, intensification therapy consisting of total body irradiation (TBI) (8.0 Gy), high dose VADRIAC chemotherapy, and autologous bone marow transplantation is given. All therapy is completed within 6-7 months. No maintenance chemotherapy is given; no surgery is intended. Of the twenty-three patients with pelvic sarcomas treated on this combined modality protocol, 22 achieved a complete remission. Local control was achieved and maintained in all twenty-three patients. With a median follow-up of 21 months since initiation of treatment, there have been nine relapses (all systemic). Seven relapses occurred among the thirteen patients who presented with overt metastatic disease and the other two relapses were among the ten patients with localized disease at presentation. All seven metastatic patients who relapsed have died, whereas both of the relapsed localized patients remain alive. Acute and late toxicities have been acceptable using this aggressive combined modality approach. Induction chemotherapy had a significant impact on reduction of the typically large (greater than 10 cm diameter) soft tissue mass associated with these pelvic tumors, thus facilitating achievement of local control by high dose irradiation. Of 18 patients with measureable soft tissue tumor, all experienced a partial response (greater than 50% reduction in size) following the initial two cycles of chemotherapy given prior to local irradiation. In conclusion, this short, intensive chemoradiotherapeutic regimen is highly effective in controlling the primary lesion (100% local control) and inducing a complete response in a high proportion (96%) of these high risk pediatric and young adult patients with pelvic sarcomas. The role of TBI as "systemic" adjuvant therapy to control micrometastatic disease is discussed as still under investigation.
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PMID:Treatment of pelvic sarcomas in adolescents and young adults with intensive combined modality therapy. 367 16

Seventy-eight cases of adjuvant chemotherapy for primary soft tissue sarcoma including 51 cases of intra-arterial infusion chemotherapy were studied. The patients ranged in age from 1 to 92 years with a median age of 34 years. Thirty-nine patients were male and 39 were female. The seventy-eight cases were comprized of 17 rhabdomyosarcoma, 12 liposarcoma, 12 neurogenic sarcoma, 10 malignant fibrous histiocytoma, 8 leiomyosarcoma, 7 angiosarcoma, 8 others and 4 unclassified sarcomas. Fifty-one patients with soft tissue sarcoma of the extremities were treated by intra-arterial infusion chemotherapy with either VCQ (Vincristine and Carbazilquinone) or VCQ, A (Vincristine, Carbazilquinone and Adriamycin). Out of 42 patients with measurable lesions, 2 CR, 4 PR, 33 NC and 3 PD were obtained. Histological examinations demonstrated histological effect of GI 19, G IIa 11 and G IIb 7 by Ohboshi and Shimosato's criteria. Remarkable effects of treatment were noted in most rhabdomyosarcoma patients. After intra-arterial infusion chemotherapy, a variety of surgical procedures ranging from marginal resection and wide resection to radical amputation were employed in 44 patients. Local recurrence was 27% and distant metastasis developed in 47% of cases.
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PMID:[Adjuvant chemotherapy in the treatment of primary soft tissue sarcomas, with special reference to intra-arterial infusion chemotherapy]. 647 41

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

Topotecan and vincristine were evaluated alone or in combination against 13 independent xenografts and 1 vincristine-resistant derivative, representing childhood neuroblastoma (n = 6), rhabdomyosarcoma (n = 5), or brain tumors (n = 3). Topotecan was given by i.v. bolus on a schedule found previously to be optimal. Drug was administered daily for 5 days on 2 consecutive weeks with cycles repeated every 21 days over a period of 8 weeks. Doses of topotecan ranged from 0.16 to 1.5 mg/kg to simulate clinically achievable topotecan lactone plasma systemic exposures. Vincristine was administered i.v. every 7 days at a fixed dose of 1 mg/kg. Given as a single agent, vincristine induced complete responses (CRs) in all mice bearing two rhabdomyosarcomas (Rh28 and Rh30) and some CRs in Rh12-bearing mice (57%) but relatively few CRs (<29%) in other tumors. As a single agent, topotecan induced CR in a low proportion of tumor lines. A dose-response model with a logit link function was used to investigate whether the combination of topotecan and vincristine resulted in greater than expected responses compared with the activity of the agents when administered alone. Only CR was used to evaluate tumor responses. The combination resulted in significantly greater than expected CRs than individual agents in nine tumor lines (four neuroblastoma, three brain tumors, and two rhabdomyosarcomas). Similar event-free (failure) distributions were shown in SJ-GBM2 glioblastoma xenografts, whether vincristine was administered on day 1 or day 5 of each topotecan course. To determine whether the increased antitumor activity with the combination was attributable to a change in drug disposition, extensive pharmacokinetic studies were performed. However, little or no interaction between these two agents was determined. Toxicity of the combination was marked by prolonged thrombocytopenia and decreased hemoglobin. However, approximately 75 and 80% of the maximum tolerated dose of each single agent, topotecan (1.5 mg/kg) or vincristine (1 mg/kg), could be given in combination, resulting in a combination toxicity index of approximately 1.5. These results show that the therapeutic effect of combining topotecan with vincristine was greater than additive in most tumor models of childhood solid tumors, and toxicity data suggest that this can be administered to mice with only moderate reduction in the dose levels for each agent.
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PMID:Synergy of topotecan in combination with vincristine for treatment of pediatric solid tumor xenografts. 1058 79


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