Rhabdomyosarcoma: the experience of the pediatric unit of Kasr El-Aini Center of Radiation Oncology and Nuclear Medicine (NEMROCK) (from January 1992 to January 2001) (original) (raw)
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Radiotherapy and Oncology, 1985
This therapeutic trial deals with 81 patients with stage III rhabdomyosarcoma (RMS), from different centers of the International Society of Pediatric Oncology. These patients were included between October 1975 and March 1983. The aim of this work is to minimize the sequelae of treatment without jeopardizing the survival rate. After a pretrial course of VAC, two groups of patients are compared: the first treated with systematic extensive surgery or radiotherapy on the initial tumor volume, and the second treated with combined chemotherapy using vincristine, dactinomycin, adriamycin, cyclophosphamide until maximum tumor reduction, followed by radiotherapy or surgery on the residual mass only. Eighty one patients were included in the trial; 15 patients were not randomized due to the failure of the pretrial course of chemotherapy, 3 were excluded after randomization (2 were found not to have RMS, and in one case the protocol was not observed). The preliminary results for the 63 patients show a survival rate of 40% at 3 years. The sequential scheme for patients at 3 years follow-up does not show a superiority in either arm.
Rhabdomyosarcoma: The Experience of Single Institution
2006
This study is a retrospective analysis ofthe treatment results of pediatric rhabdomyosarcoma patients who attended the pediatric unit of Kasr-EI-Aini Center of Radiation Oncology and Nuclear Medicine (NEMROCK) from January 1992 to January 2001. Fifty-five new cases of pediatric rhabdomyosarcoma attending the pediatric unit outpatient clinic of (NEMROCK) were evaluated. Males constituted 63.6% of the cases (35 cases) and females 36.4% (20 cases). The median age was 6 years and the ages of the patients ranged from 1 to 9 years. (For Stages, this disease was diagnosed in most of the cases in early stages (40/55,72.7%) versus late stages (15/55,27.3%). This is because the most common site in this study was the head and neck, which causes early parental notification). Pathologically, embryonal type was the commonest statistically (48/55, 87.3%) compared to the alveolar type (7/55, 12.7%), Concerning site of the primary tumor it was found to be highest in the head and neck (20/55, 36.4%) ...
Risk Stratification Treatment of Pediatric Rhabdomyosarcoma: South Egypt Cancer Institute Experience
Journal of Cancer Therapy, 2012
Risk stratification allows tailoring of treatment protocol using, for selected patients, reduced total chemotherapy exposure, including decreases in alkylator therapy and avoidance of agents with recognized risk of late complications (anthracyclines), elimination of irradiation and reduction of radiotherapy dose. Patients and Methods: Twenty-nine newly diagnosed pediatric rhabdomyosarcoma patients attended the pediatric oncology department between January 2008 and May 2011. Patients were divided into 3 groups according to age, stage, group, pathology and site of the tumor. Treatment protocol tailored according to risk group. Results: Twenty-nine newly diagnosed pediatric rhabdomyosarcoma patients were evaluated. Seven patients had low risk, Intermediate risk included 12 patients, and 10 patients had high risk. After three years median follow up, event free survival was 51.7% for all patients however it was 86%, 67% and 10% for low, intermediate and high risk respectively (P = 0.0002). There was statistical difference for survival among different sites, histology, clinical group and stage as risk factors within each risk group, no statistically survival significance of any of these factors within the same risk group. Conclusion: Risk stratification is the best single predictor factor for pediatric rhabdomyosarcoma and allows tailoring of the treatment protocol. For selected patients, reductions in total chemotherapy exposure, elimination of irradiation in selected low risk patients and reduction of radiotherapy dose according to postoperative margin and nodal status is safe.
Survival rate of children with rhabdomyosarcoma and prognostic factors
2007
Survival rate of children with rhabdomyosarcoma and prognostic factors Background: Rhabdomyosarcoma is the most frequent tumor of soft tissue in children. The survival rate of the patients is significantly increased since the 1970s. This study was undertaken to evaluate the 5-and 10-year survival rates of patients with rhabdomyosarcoma in a single center in Iran. Methods: A total of 77 children with rhabdomyosarcoma up to 15 years old who had been treated at the Hematology and Oncology Department in Ali Asghar Children Hospital from 1993 to 2003 were evaluated for their age and gender, as well as histology, stage and primary site of the tumor at diagnosis. Results: The mean age of the patients was 6.58 years (SD=4.02, median 6 years). In this series, 46 patients (59.7%) were male and 31 (40.3%) female, and the mean survival time of patients was 8 years (95% CI: 8-9). The 5-year survival rate was the highest in patients with localized tumor, stage I and II (82.25% and 86.88% respectively). The survival rate of patients with embryonal tumor was 86.8%, in those with primary orbital tumor was 94%, and in those with genitourinary tumor was 85.71%. The 5-and 10-year overall survival rates were 79.54% and 77.92%, respectively. Conclusions: Children with rhabdomyosarcoma of lower stage, embryonal histology, and orbital and genitourinary primary sites had a better survival rate. Poor prognosis was associated with metastasis of the tumor at the time of presentation, alveolar histology (48%), and tumor of the extremities (58%). In our study, only the stage of the tumor was significantly different in the variables (P=0.0077) because of the small number of patients. Children who survived the first 5 years after diagnosis were found to have an excellent survival rate.
Rhabdomyosarcoma in adolescents: A CCHE experience
Journal of Solid Tumors, 2015
Objective and aim: A lower outcome of rhabdomyosarcoma in elder children and young adolescents is increasingly recognized. Our aim was to focus on the outcome of elder children and adolescents with rhabdomyosarcoma treated at our Center (CCHE). Patients and methods: Patients between the age 10 and 18 years diagnosed as rhabdomyosarcoma and treated according to IRS-IV, or IRS V protocols determined according to site, stage, and age at diagnosis. Results: Thirty-two children were recruited at Children's Cancer Hospital-Egypt. They were 12 females (37.5%) and 20 males (62.5%), with a mean age of 12.8y. The mean follow up period was 29.4 months (July 2007 till December 2012). Seventeen patient, were allocated to IRS-IV, and 15 patient to IRS-V. The overall survival and failure free survival were 59.1% and 40.4% respectively. Forty six percent of patients had alveolar pathology, 68.8% presented with tumors at unfavorable sites; 37.5% had metastases at presentation; and 21.9% had intracranial extension at presentation. Risk status, clinical grouping and tumor size affected FFS significantly. Local control was done by radiation therapy in 62.5%, surgery alone in 9.4%, and both radiation and surgery in 25%. Conclusion: The outcome of adolescent group showed inferior results compared to younger children. More cooperation and knowledge about RMS, in this age category, can help develop more intensive unified management approach for them.
International Journal of Oncology, 1995
To avoid the delayed consequences of treatment with radiotherapy, an effort was made to determine if patients with rhabdomyosarcoma could be cured with chemotherapy as the sole form of treatment. Alternatively, if radiotherapy and/or surgery were required to reduce the severity and incidence of delayed sequelae, an effort was made to determine if there was an optimum safe period for delaying implementation of these definitive forms of treatment. In patients where primary (immediate) definitive non-mutilating surgical extirpation of tumor was not feasible, exclusive treatment with chemotherapy was implemented. If considered necessary or appropriate, delayed surgery and/or radiation therapy were employed in 3 circumstances: (i) to consolidate a partial response; (ii) failure to respond; (iii) recurrent disease. The outcome of the delays prior to the implementation of definitive therapy was analyzed as a function of local and systemic recurrence and cure. Fifty-two patients were evaluated. Seven underwent primary nonmutilating surgical extirpation of localized tumor followed by adjuvant chemotherapy. The remaining 45 were treated with primary chemotherapy and 44 responded. Actuarial survival curves of the delay in initiating definitive therapy in the 52 patients revealed that the optimum delay to attain the best survival was 5 months. In circumstances where definitive therapy was not electively introduced, recurrent disease during remission appeared between 7 and 14 months in 7 patients on continued treatment, and in one patient at 30 months, 8 months after discontinuation of chemotherapy. Based upon the 5-month delay an analysis of survival was
Cancers, 2022
We report here the results of the prospective, non-randomized, historically controlled CWS-2002P study in patients ≤ 21 years with localized RMS developed with the aim to improve the long-term outcome by adapting the burden of therapy to risk profile and to investigate the feasibility and relation to the outcome of maintenance therapy (MT) in the high-risk groups. Patients were allocated into low-risk (LR), standard-risk (SR), high-risk (HR), and very high-risk (VHR) groups. Chemotherapy consisted of vincristine (VCR) and dactinomycin (ACTO-D) for all patients with the addition of ifosfamide (IFO) in the SR, HR, and VHR and doxorubicin (DOX) in the HR and VHR groups. Low-dose cyclophosphamide and vinblastine maintenance therapy (MT) over 6 months was recommended in the HR and VHR groups. A total of 444 patients have been included in this analysis. With a median follow-up of 9·6 years (IQR 7·6–10·9) for patients alive, the 5-year EFS and OS for the whole group was 73% (95% CI 69–77) ...
Current Opinion in Pediatrics, 2009
Rhabdomyosarcoma is a rare childhood cancer that affects only approximately 300 children per year in the United States. The purpose of this review is to provide the reader a greater understanding of the complex diagnosis, assessment and treatment of rhabdomyosarcoma in children. This review focuses on the new risk classification that is the foundation of all present rhabdomyosarcoma protocols developed by the Children's Oncology Group of the United States and Canada. The new risk classification of low, intermediate and high encompasses the staging and grouping categories that were previously utilized. This review also provides a complete list of diagnostic tests and imaging required to identify rhabdomyosarcoma in any body site. Rapid diagnosis and recognition of this rare disorder will facilitate long-term survival. Rhabdomyosarcoma today has an overall survival of 70%, depending on the site, and in orbital and other sites survival is as high as 90%. The treatment approaches that have led to this doubling in survival over the last 25 years are reviewed. For a practitioner, this review can be used as a reference when a child with a suspicious mass is encountered.
European Journal of Cancer, 1998
The second International Society of Paediatric Oncology (SIOP) study for rhabdomyosarcoma (MMT84) had several goals. The two principal aims were: (1) to improve the survival of children with rhabdomyosarcoma; and (2) to reduce the late eVects from therapy by restricting the indications for surgery and/or radiotherapy after good response to initial chemotherapy. A further aim was to investigate the role of high-dose chemotherapy in young patients with parameningeal primary tumours. 186 previously untreated eligible patients entered the study. Patients with completely resected primary tumour received three courses of IVA (ifosfamide, vincristine and actinomycin D). Patients with incompletely resected tumour received six to 10 courses of IVA according to stage. Patients achieving complete remission with chemotherapy alone did not usually receive radiotherapy or undergo extensive surgery, but patients remaining in partial remission received local therapy with surgery and/or radiotherapy. Only patients over 5 years of age with parameningeal disease and patients over 12 years with tumours at any site were given systematic irradiation. Complete remission was achieved in 91% (170/186) of all patients. With a median follow-up of 8 years, the 5-year overall survival was 68% (3% standard error of the mean (SEM)) and the 5-year event-free survival 53% (4% SEM). These results show an improvement over previous SIOP study (RMS75) in which survival was 52% and event-free survival was 47%. Among the 54 patients who exhibited isolated local relapse, 35% (19/54) survived in further remission longer than 2 years after retreatment, including local therapy (surgery radiotherapy). Analysis of the overall burden of therapy received by all surviving children (including primary treatment and treatment for relapse if required) showed that 24% (28/116) were treated by limited surgery followed by three courses of IVA, 29% (34/116) were treated by chemotherapy alone (after initial biopsy) and 13% (15/116) received chemotherapy plus conservative local treatment (limited surgery or radiotherapy for residual disease). Only 34% (39/116) received intensive local therapy de®ned as radical wide ®eld radiotherapy or radical surgery or both. Compared with the results obtained in the previous SIOP study, treatment in MMT84 was based on response to initial chemotherapy and, despite an overall reduction of the use of local therapy, signi®cantly improved survival for patients with non-metastatic disease. This trial, also for the ®rst time, provides evidence that retreatment after local relapse can achieve long-term second remissions.
Outcome of paediatric rhabdomyosarcoma attended in a tertiary care hospital, Dhaka, Bangladesh
Bangladesh Medical Research Council Bulletin
Background: Rhabdomyosarcoma (RMS) is a malignant tumor of mesenchymal origin, is the most common form of paediatric soft tissue sarcoma. It is the third most common solid tumor comprising 4.5% of all childhood cancer with an annual incidence of 4.5 cases per million children. There is so no statistical data on RMS in Bangladesh because no study has done in Bangladesh. Objective: To evaluate the clinical characteristics and treatment outcome of children with rhabdomyosarcoma among tertiary care. Methods: A retrospective study carried out analysing the medical records of 39 patients with rhabdomyosarcoma from the BSMMU tumor registry. Medical records of children with rhabdomyosarcoma were reviewed over a period of 6 years from June 2011 to May 2017. Most patients received multimodality therapy (chemotherapy, surgery, radiotherapy). Results: The male female was ratio 1.2:1. More than half of cases presented with a mass at varying sites The predominant complaint 12.0% was pain. The pr...