The Intergroup Rhabdomyosarcoma Study Group (IRSG): Major Lessons From the IRS‐I Through IRS‐IV Studies as Background for the Current IRS‐V Treatment Protocols

Raney, R. Beverly; Maurer, Harold M.; Anderson, James R.; Andrassy, Richard J.; Donaldson, Sarah S.; Qualman, Stephen J.; Wharam, Moody D.; Wiener, Eugene S.; Crist, William M.

doi:10.1080/13577140120048890

Cited by 264 publications

(260 citation statements)

References 24 publications

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“…In this study, the 4-year PFS (61% AE 7.5%) and OS (72% AE 6.9%) rates were similar to those reported previously for RMS [3,9,[14][15][16][17]. Moreover, there was significant association between risk groups and 4-year PFS and OS rates: patients with LR and IR group had better outcomes than those with HR diseases, which is consistent with results reported by collaborative groups on RMS [9,15,[18][19][20][21][22][23][24].…”

Section: Discussionsupporting

confidence: 91%

“…Patients were classified by using a risk stratification system, which was adopted from that used by the Children Oncology Group (COG); it integrates post surgical grouping classification developed by the North American Intergroup Rhabdomyosarcoma Study Group IRSG (now known as the Soft Tissue Sarcoma Committee of the COG) [8], IRSG pretreatment staging classification (modified from a tumor-node-metastasis system) [9], and histology [3]. According to the risk stratification system we used, patients were stratified into a low-risk (LR) group including those with nonmetastatic embryonal tumors (i.e., embryonal histology, stage I, post surgical group I, II, or III; or embryonal histology, stage I or II, post surgical group I or II); intermediate-risk (IR) group if they had nonmetastatic alveolar histology tumors (i.e., stage I, II, or III, post surgical group I, II, or III) or an unresected…”

Section: Patientsmentioning

confidence: 99%

“…Before 2006, patients with RMS were treated according to the IRS IV protocol [3]. In June 2006, a major modification was introduced to the protocol at KHCC-the dose of cyclophosphamide was lowered to 1.2 g/m 2 because of the high toxicity associated with the VAC regimen at a cyclophosphamide dose of 2.2 g/m 2 /cycle.…”

Section: Chemotherapy Protocolmentioning

confidence: 99%

“…Overall survival (OS) rates for children with RMS have improved significantly over the last 30 years [2], primarily due to advances in multimodal therapy. All treatment strategies incorporate systematic multiagent chemotherapy and local therapy [3,4]. Chemotherapy for RMS typically comprises alkylating agents (ifosfamide or cyclophosphamide) at varying dosages, depending on regimen.…”

Section: Introductionmentioning

confidence: 99%

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Improved care of rhabdomyosarcoma in Jordan using less intensive therapy

Al-Jumaily

Ayaad

Masarweh

et al. 2012

Pediatric Blood & Cancer

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BackgroundThe care of rhabdomyosarcoma (RMS) is complex due to its multimodal nature. By following standard protocols with acceptable toxicity and building local expertise, better outcome should be achievable.ProcedureA retrospective study was conducted of records of patients (n = 45; 31 males; median age 26 months) with RMS treated at King Hussein Cancer Center in Jordan from January 2004 to December 2008. Patient demographics, tumor characteristics, risk stratification, treatment plan, and outcomes were studied. In June 2006, the cyclophosphamide dose was lowered from 2.2 g/m2 to 1.2 g/m2 per cycle because of the significant toxicity with higher dose. Survival rates, hematological toxicities, period of hospitalization due to febrile neutropenia (FN), and response rate at week 12 of treatment were compared between low‐ and high‐dose cyclophosphamide groups.ResultsFour‐year progression‐free survival (PFS) and overall survival (OS) rates were 61% ± 7.5% and 72% ± 6.9%, respectively. There was a significant difference in outcome by risk group in 4‐year PFS (low‐risk, 88% ± 12%; intermediate‐risk 63% ± 9.3%; high‐risk, 14% ± 13%; P = 0.0001) and OS (low‐risk, 88% ± 12%; intermediate‐risk 79% ± 7.5%; high‐risk, 17% ± 15%; P = 0.0011). There was significant reduction in hematological toxicities, incidence of FN, and period of hospitalization for FN in patients given low‐dose cyclophosphamide but no significant difference in PFS between low‐ and high‐dose cyclophosphamide groups.ConclusionsSurvival rates of patients with RMS in some developing countries can be improved by following or modifying evidence‐based approaches successful in developed countries and establishing multidisciplinary strategies. Therapy intensity should be increased in developing countries only when evidence supports its utility. Pediatr Blood Cancer 2013; 60: 53–58. © 2012 Wiley Periodicals, Inc.

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Section: Discussionsupporting

confidence: 91%

Section: Patientsmentioning

confidence: 99%

Section: Chemotherapy Protocolmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Improved care of rhabdomyosarcoma in Jordan using less intensive therapy

Al-Jumaily

Ayaad

Masarweh

et al. 2012

Pediatric Blood & Cancer

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“…Rhabdomyosarcoma (RMS) accounts for <3% of adult soft tissue sarcoma but is the most frequent soft tissue sarcoma histological subtype before age 10 and the 4th most prevalent cancer during childhood 1, 2, 3, 4. Five‐year overall survival (OS) of children has dramatically improved in the last 30 years based on the results of successive studies of large multinational collaborative trials dedicated to children.…”

Section: Introductionmentioning

confidence: 99%

Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study

et al. 2018

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Five‐year overall survival (OS) of localized RMS exceeds 70% in children (<18) but is very poor in adult patients. We analyzed the outcome and prognostic factors (PF) of a national series of adult patients with RMS in a large study. The study population consisted of two different cohorts: a retrospective cohort (157 adult patients treated in 13 reference centers between 05/1981 and 02/2010) and the prospective cohort (292 patients with RMS diagnosed and treated between 01/2010 and 12/2014 in France) included in the NetSarc database. A descriptive analysis of patients’ characteristics and prognostic factors was conducted on both series which were compared. In the retrospective series, histological subtypes were embryonal (E‐RMS) for 21% of patients, alveolar (A‐RMS) for 35% of patients, and “adult‐type” P‐RMS (pleomorphic, spindle cell RMS, not otherwise specified) (P) for 44% patients. This distribution significantly differed in the prospective cohort: A‐RMS: 18%; E‐RMS: 17%; and P‐RMS 65%. With a median follow‐up of 8.5 years, 5‐year OS for localized RMS and advanced RMS (with nodes and/or metastases) was 43% and 5%, respectively, (P < 0.0001), and median OS was 51, 33, and 16 months for E‐RMS, A‐RMS, and P‐RMS, respectively, in the retrospective cohort. The median OS was less than 40 months for the prospective nationwide cohort for the entire population. In a multivariate analysis of the retrospective study, independent prognostic factors for OS were A‐RMS, R0 resection, and adjuvant radiotherapy (RT). For localized RMS, age and use of pediatric chemotherapy (CT) regimen are independent prognostic factors. Adult patients with RMS have a poorer overall survival than pediatric patients, and survival varies considerably across histological subtypes.

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Fluorine-18-fluorodeoxyglucose (FDG) positron emission tomography (PET) computed tomography (CT) for the detection of bone, lung and lymph node metastases in rhabdomyosarcoma

Breunis

Haveman

Vaarwerk

et al. 2016

Cochrane Database of Systematic Reviews

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Rhabdomyosarcoma (RMS) is the most common paediatric soft-tissue sarcoma and can emerge throughout the whole body. For patients with newly diagnosed RMS, prognosis for survival depends on multiple factors such as histology, tumour site, and extent of the disease. Patients with metastatic disease at diagnosis have impaired prognosis compared to those with localised disease. Appropriate staging at diagnosis therefore plays an important role in choosing the right treatment regimen for an individual patient. Fluorine-18-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) is a functional molecular imaging technique that uses the increased glycolysis of cancer cells to visualise both structural information and metabolic activity. 18F-FDG-PET combined with computed tomography (CT) could help to accurately stage the extent of disease in patients with newly diagnosed RMS. In this review we aimed to evaluate whether 18F-FDG-PET could replace other imaging modalities for the staging of distant metastases in RMS. Objectives To determine the diagnostic accuracy of 18F-FDG-PET/CT imaging for the detection of bone, lung, and lymph node metastases in RMS patients at first diagnosis. Search methods We searched MEDLINE in PubMed (from 1966 to 23 December 2020) and Embase in Ovid (from 1980 to 23 December 2020) for potentially relevant studies. We also checked the reference lists of relevant studies and review articles; scanned conference proceedings; and contacted the authors of included studies and other experts in the field of RMS for information about any ongoing or unpublished studies. We did not impose any language restrictions. Selection criteria We included cross-sectional studies involving patients with newly diagnosed proven RMS, either prospective or retrospective, if they reported the diagnostic accuracy of 18F-FDG-PET/CT in diagnosing lymph node involvement or bone metastases or lung metastases or a combination of these metastases. We included studies that compared the results of the 18F-FDG-PET/CT imaging with those of histology or with evaluation by a multidisciplinary tumour board as reference standard. Data collection and analysis Two review authors independently performed study selection, data extraction, and methodological quality assessement according to Quality Assessment of Diagnostic Accuracy Studies 2 (QUADAS-2). We analysed data for the three outcomes (nodal involvement and lung and bone metastases) separately. We used data from the 2 × 2 tables (consisting of true positives, false positives, true negatives, and false negatives) to calculate sensitivity and specificity in each study and corresponding 95% confidence intervals. We did not consider a formal meta-analysis to be relevant because of the small number of studies and substantial heterogeneity between studies. Main results Two studies met our inclusion criteria. The diagnostic accuracy of 18F-FDG-PET/CT was reported in both studies, which included a total of 36 participants. We considered both studies to be at high risk of bias for the do...

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The Intergroup Rhabdomyosarcoma Study Group (IRSG): Major Lessons From the IRS‐I Through IRS‐IV Studies as Background for the Current IRS‐V Treatment Protocols

Cited by 264 publications

References 24 publications

Improved care of rhabdomyosarcoma in Jordan using less intensive therapy

Improved care of rhabdomyosarcoma in Jordan using less intensive therapy

Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study

Fluorine-18-fluorodeoxyglucose (FDG) positron emission tomography (PET) computed tomography (CT) for the detection of bone, lung and lymph node metastases in rhabdomyosarcoma

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