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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Contribution of one case of right paratesticular rhabdomyosarcoma in a 3-year and 4-month old male patient. Following radical orchiectomy and clinical staging, grading is IRS Group I (fully resected localized disease). Subsequently, the patient received 7 polychemotherapy courses and was found to be asymptomatic 4 years after treatment.
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PMID:[Paratesticular rhabdomyosarcoma]. 988 17

Two hundred and fifty-five previously untreated patients (pts) with rhabdomyosarcoma (RMS) (age range 15 days to 17 years, median 5 years) were evaluated and treated in our institution. Head and neck primaries were seen in 125 patients (49%), abdominopelvic in 73 (29%), trunk and lung in 20 (5%) and extremity lesions in 37 (15%). The histology was: embryonal 137; alveolar 42; botryoid 18; pleomorphic 14. Forty-four patients could not be subclassified. The stage of the patients were as follows: 15 in state I, 74 in stage II, 139 in stage III and 27 in stage IV, according to the IRS grouping system. Patients were treated with a combination of surgery and radiation to doses of 35-55 Gy according to the patient's age and stage. All the patients received chemotherapy according to VAC or pulse-VAC (before 1988) and modified AVAC (after 1988) protocol. Survival curves were calculated by the Kaplan-Meier method. The statistical significance of each variable was tested by the log-rank test. Overall survival was 42 percent at 10 years. Three important predictors for survival time were clinical group (p < 0.001), age (p < 0.001) and primary site (p = 0.005). The best results involved clinical group I-II, age one to five years and orbital and genitourinary primary sites. An important predictor of survival time was also detected between those treated during the first ten years (1972-82) and last 10 years (1982-92), p < 0.005. Of the 96 deaths, 37 were from progressive disease, 24 from infection, 4 during postoperative period (first 7 days), 18 from unknown causes and 13 from other causes.
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PMID:Turkish experience with rhabdomyosarcoma: an analysis of 255 patients for 20 years. 1002 57

We report a case of paratesticular rhabdomyosarcoma in a six-year-old boy. The clinical presentation was subacute. The patient underwent a radical right inguinal orchidectomy. It was classified in IRS-III stage IA (based in the Third Intergroup Rhabdomyosarcoma Study). Subsequently, the child received 3 chemotherapy courses (9 weeks) with vincristine and actinomycin D. The patient is found to be asymptomatic 1 year after the treatment.
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PMID:[Paratesticular rhabdomyosarcoma]. 1046 68

Over last decades with modern approach to combined treatment of soft tissue sarcoma in children and adolescents, with effective systemic chemotherapy and adequate local control most frequently with conservative surgery and radiotherapy, or radiotherapy alone, results of treatment from 20% of a three-year overall survival to 75% were improved significantly. Nevertheless, combined treatment involves risk of acute radiation reactions and late side effects, so there is a need for precise radiotherapy planning with optimal schedule of fractionating, adequate radiation volume and optimal tumour dose. The purpose of our study was to evaluate the results of combined treatment of soft tissue sarcoma, role of radiotherapy in local control use of the optimal tumour dose and assessment of acute radiation reactions in an examined group of patients. A retrospective clinical study involved 47 patients treated with radiotherapy at the Institute of Oncology and Radiology of Serbia over the period from 1990 to 1997. The most frequent tumour sites were the head and neck and the extremities. According to the IRS classification most patients were in CS III (21 patients). Forty patients had histological type--Rhabdomyosarcoma (Table 1). All patients were treated with chemotherapy, and local therapy were surgery and radiotherapy or radiotherapy alone. Thirty one patients were operated on. All 47 patients were treated with radiotherapy; in 37 patients as primary treatment and in 10 patients as therapy for local relapse. Radiotherapy was planned according to tumour size, tumour site, age of the patient and type of surgery. Tumour dose from 45 Gy to 60 Gy was given in cases with a residual tumour. Lower tumour doses were used in cases of postoperative microscopic disease, in certain cases of local relapse treatment or when the size of residual tumour and patient's age allowed no delivery of higher tumour doses. Standard fractionating regimen was given to all patients, with daily fractions from 150 cGy to 214 cGy, five times per week. The majority of patients (24) were treated on Linear Accelerator machine with X photons of 10 MeV energy and with X photons of 6 MeV energy (13 patients) (Table 2). Statistical data processing was made by the following methods: Kaplan-Meier for survival rate and Long-rang and Wilcox test for assessment of the statistical significance in survival difference. In our group of patients treated over the period from 1990 to 1997 a three-year overall survival was 59.15%, and disease free survival was 46.68% (Figure 1). There were 21 patients (44.7%) without signs of the disease, 12 patients had a local disease (25.5%), 9 patients had both local and metastatic disease (19.1%) and 5 patients had only metastatic disease (10.50%). In the group of 47 patients who received radiotherapy, 24 patients received a tumour dose from 45 Gy to 60 Gy and 23 patients a tumour dose from 32 Gy to 45 Gy. The group of patients treated without tumour dose more than 45 Gy had a significantly better overall survival rate (p = 0.002) (Figure 2). Although the obtained results are in agreement with data from literature, a critical analysis is necessary. Namely, in addition to the group irradiated with a tumour dose from 32 Gy to 45 Gy, because of the postoperative microscopic disease, certain number of patients was irradiated with a "lower" dose because of an objective impossibility to administer a "higher" dose or this dose was planned for palliative reasons. The tumour dose of 45 Gy was delivered to 6 of 10 patients treated for local relapse. The tumour dose of 45 Gy was also used in four patients in CS IV, in two subjects for local control and in two as a palliative treatment. Seven patients in CS III received a tumour dose of 45 Gy, because the age of children, tumour site and tumour size permitted no higher tumour doses. That is when planning an adequate local therapy one must have in mind the initial tumour size, type of administered systematic chemo
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PMID:[Role of radiotherapy in combined therapy of soft tissue sarcoma in children and adolescents]. 1108 17

In the paper the authors present the evaluation of head and neck rhabdomyosarcoma (RMS) treatment in 14 children admitted to Pediatric Institute in the past 25 years. The treatment was related to the stage of the neoplasm at the time of diagnosis and consisted in the surgical intervention combined with chemo- and radiotherapy. Embryonal stage was diagnosed in 10 children (71.4%) and alveolar stage in 4 children (28.6%). In the analysed material primary neoplastic changes were found: in 6 cases in the orbit, in 2 cases in the maxillary sinus and nasal cavity, in 2 cases in the mandible. We noted also 1 case in each of the following structures: the nasopharynx, the palate, the pterygoideo-palatal fossa, the neck. In the years 1973-1991--7 children were treated according to the IRS I and II projects. Three of the children presented with the third stage of neoplastic disease and the rest with the second. Between the years 1991-1997--7 other children were hospitalized and underwent treatment based on CWS-91 project. Six of them showed the third stage of neoplasm, and only one--the first. The performed analysis revealed the insignificant improvement in the effectiveness of the treatment after the introduction of the CWS-91 project.
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PMID:[Evaluation of treating head and neck rhabdomyosarcoma in the Pediatric Institute in Poznan in 1973-1997]. 1135 71

Treatment outcomes of bladder/prostate rhabdomyosarcoma (RMS) in multi- and single-institutional trials are reviewed. Remarkable strides have occurred in the treatment of this disease. Decreasing duration of chemotherapy, less cumulative doses of radiation therapy, and improving survival have been documented. A focus on bladder preservation has not adversely affected survival in most studies. Even if organ preservation is not possible, improvements in urinary diversion surgical technique still offer improved quality of life. The IRS III was a pivotal study in improving survival and quality of life. We recommend protocol enrollment whenever available. We also emphasize the use of magnetic resonance imaging and second look surgery.
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PMID:Treatment modalities of bladder/prostate rhabdomyosarcoma: a review. 1280 68

We report a case of intrascrotal rhabdomyosarcoma in a 59-year-old-male. The patient with a mass in the right scrotum was at first diagnosed with epididymitis and treated with antibiotics. The mass however, grew gradually and right orchiectomy revealed intrascrotal alveolar rhabdomyosarcoma. Para-aortic lymph node metastasis was pointed out by abdominal computed tomography. He received multidrug chemotherapy consisting of vincristine, actinomycin-D, cyclophosphamide, adriamycin, and cisplatin (IRS-III regimen 35). A complete response was obtained 3 months after the start of the chemotherapy. The patient is alive without recurrence 2 years after the surgery.
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PMID:[A case of intrascrotal rhabdomyosarcoma with para-aortic lymph node metastasis--complete response after multidrug chemotherapy]. 1289 35

Atypical teratoid/rhabdoid tumors (AT/RT) are highly malignant lesions of childhood that carry a very poor prognosis. AT/RT can occur in the central nervous system (CNS AT/RT) and disease in this location carries an even worse prognosis with a median survival of 7 months. In spite of multiple treatment regimens consisting of maximal surgical resection (including second look surgery), radiation therapy (focal and craniospinal), and multi-agent intravenous, oral and intrathecal chemotherapy, with or without high-dose therapy and stem cell rescue, only seven long-term survivors of CNS AT/RT have been reported, all in patients with newly diagnosed disease. For this reason, many centers now direct such patients, particularly those under 5 years of age, or those with recurrent disease, towards comfort care rather than attempt curative therapy. We now report on four children, two with newly diagnosed CNS AT/RT and two with progressive disease after multi-agent chemotherapy who are long term survivors (median follow-up of 37 months) using a combination of surgery, radiation therapy, and intensive chemotherapy. The chemotherapy component was modified from the Intergroup Rhabdomyosarcoma Study Group (IRS III) parameningeal protocol as three of the seven reported survivors in the literature were treated using this type of therapy. Our four patients, when added to the three reported survivors in the literature using this approach, suggest that patients provided this aggressive therapy can significantly alter the course of their disease. More importantly, we report on the first two survivors after relapse with multi-agent intravenous and intrathecal chemotherapy treated with this modified regimen.
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PMID:Continuous remission of newly diagnosed and relapsed central nervous system atypical teratoid/rhabdoid tumor. 1580 79

Differently from adult oncologists that considered synovial sarcoma (SS) a tumor with uncertain chemosensitiveness, since two decades pediatric oncologists in Europe assumed that chemotherapy played an important role in SS treatment, so most pediatric patients were included in rhabdomyosarcoma protocols, receiving adjuvant chemotherapy regardless of risk factors. The German and Italian groups reviewed the data of grossly resected SS patients in order to define a risk-adapted treatment program for the next European protocol. A total of 150 patients < 21 years with localized SS who underwent initial gross resection between 1975 and 2002 were the object of this study. All but four cases received adjuvant chemotherapy. Post-operative radiotherapy was administered to 50% Group I and to 92% Group II patients. Five-year event-free survival (EFS) and overall survival (OS) were 77% and 89%, respectively. Survival rates were influenced by tumor size (EFS 92% and 56% for size < or = and > 5 cm, respectively) and local invasiveness, not by surgical margins. No metastatic relapses occurred in Group I < or = 5 cm patients, while the outcome was poor for T2B patients (EFS 41%) due to a high rate of metastatic relapse. Our study was unable to assess the role of adjuvant treatments in grossly-resected SS, but identified a subset of low-risk patients (IRS Group I, size < or = 5 cm), for which the omission of adjuvant chemotherapy could be suggested, and a subset of high-risk patients (T2B), who need treatment intensification.
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PMID:Grossly-resected synovial sarcoma treated by the German and Italian Pediatric Soft Tissue Sarcoma Cooperative Groups: discussion on the role of adjuvant therapies. 1629 43

A 38-year-old man, a chronic smoker, presented to us with a large left upper lobe mass lesion and left-sided pleural effusion. After resection, a residual mass remained. On histopathologic examination, a diagnosis of pleiomorphic rhabdomyosarcoma stage III of the lung was made. Chemotherapy using IRS (Intergroup Rhabdomyosarcoma Study) IV protocol with radiation therapy (RT) at week 9 was planned. The tumor progressed within 6 weeks of chemotherapy to involve the diaphragm and the pericardium (inoperable disease). Chemotherapy was abandoned, and we referred the patient to receive RT, which the patient refused; and he died of progressive disease 1 month later. The poor response to chemotherapy suggests that alternative treatment modalities including RT/second-look surgery or novel chemotherapeutic strategies should be tried in such a case.
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PMID:Primary pulmonary rhabdomyosarcoma in adults: case report and review of literature. 1756 41


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