Cooperative Trial CWS-91 for Localized Soft Tissue Sarcoma in Children, Adolescents, and Young Adults

Dantonello, Tobias; Int-Veen, Christoph; Harms, D.; Leuschner, Ivo; Schmidt, Bernhard; Herbst, M.; Juergens, Heribert; Scheel-Walter, Hans-Gerhard; Bielack, Stefan; Klingebiel, Thomas; Dickerhoff, R.; Kirsch, Sylvia; Brecht, Ines B.; Schmelzle, R.; Greulich, M.; Gadner, Helmut; Greiner, Jeanette; Márky, Ildikó; Treuner, J.; Kościelniak, Ewa

doi:10.1200/jco.2007.15.0466

Cited by 163 publications

(150 citation statements)

References 31 publications

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“…Sharing the same classification would allow easier comparison between clinical presentation and behavior in adults and children. Finally, specific chemotherapy for A‐RMS and E‐RMS is an independent prognostic value in our study and others 2, 6, 7, 8, 9, 11, 14, 15, 38, 39, 40, 41, 43, 44, 45, 46, 47. North American and European pediatric groups have identified several risk factors (favorable location, IRSG stage, IRSG group, and age) to define the clinical group and deliver a regimen adapted to the risk group.…”

Section: Discussionsupporting

confidence: 78%

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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Section: Discussionsupporting

confidence: 78%

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

et al. 2018

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“…No decrease in survival could be shown. The result from 1990 to 2010 was similar to those in the third and fourth intergroup Rhabdomyosarcoma Studies and our results were better than those in CWS‐91 16, 20. As expected, the prognosis was worse for patients with metastatic disease than for patients with localised disease.…”

Section: Discussionsupporting

confidence: 88%

“…The prognosis regarding sex, age, pathological subtype and size was in accordance with earlier studies 9, 16, 18. Just as the report by Dantonello et al 16 showed, the primary site category with the best prognosis was the orbit and the worst was the category called other sites.…”

Section: Discussionsupporting

confidence: 87%

“…Just as the report by Dantonello et al 16 showed, the primary site category with the best prognosis was the orbit and the worst was the category called other sites. As indicated in the report from the Swedish Childhood Cancer Registry 13, the overall 5‐year survival rate was higher in 1990–1999 (78 ± 5%) than in 2000–2010 (71 ± 5%), but this difference was not significant.…”

Section: Discussionmentioning

confidence: 95%

“…During the two last periods, 1990–1999 and 2000–2010, all but one of the patients received systemic treatment with multi‐agent conventional chemotherapy according to the respective CWS protocol. The prescribed chemotherapeutical regimens were vincristine, actinomycin D, ifosfamide and doxorubicin (VAIA) for all patients in CWS‐86 9 and vincristine, actinomycin D, cyclophosphamide and doxorubicin (VACA) or etoposide, vincristine, actinomycin D, ifosfamide and doxorubicin (EVAIA) for localised RMS in CWS‐91 16. Patients with metastatic disease in CWS‐91 were recommended treatment according to the protocol of the International Society of Paediatric Oncology.…”

Section: Methodsmentioning

confidence: 99%

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Higher rates of metastatic disease may explain the declining trend in Swedish paediatric rhabdomyosarcoma survival rates

2015

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AimPositive outcomes for paediatric rhabdomyosarcoma (RMS) were high in Sweden during the 1990s, but the last decade has seen decreasing trends in overall survival rates. We investigated the incidence, patient and disease characteristics, treatment and outcome of RMS to see whether any reason could be found for this decline.MethodsThis study included 210 children under the age of 15 who were diagnosed with RMS and whose details were recorded in the population‐based Swedish Childhood Cancer Registry from 1984 to 2010.ResultsThe overall annual incidence of RMS was 4.9 per million, and the 5‐year overall survival rates were 59 ± 7% in 1984–1989, 78 ± 5% in 1990–1999 and 71 ± 5% in 2000–2010. When patients with localised disease were analysed separately, there was no difference in the 5‐year survival rates between 1990 and 1999 (82 ± 5%) and 2000–2010 (81 ± 5%), but the outcome in 1984–1989 (53 ± 8%) was significantly worse. The prevalence of metastatic disease was unexpectedly high during 2000–2010 (28%, p = 0.010), compared to an overall mean of 18% for the whole study period.ConclusionOur results suggest that a higher rate of metastatic disease may explain the declining trend in overall survival rates in paediatric RMS in Sweden over the last decade.

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Clinical characteristics and outcomes for children, adolescents and young adults with “CIC‐fused” or “BCOR‐rearranged” soft tissue sarcomas: A multi‐institutional European retrospective analysis

et al. 2023

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Cooperative Trial CWS-91 for Localized Soft Tissue Sarcoma in Children, Adolescents, and Young Adults

Abstract: Improved risk stratification enabled decreased therapy intensity for selected patients without compromising survival. Intensified chemotherapy with EVAIA did not improve outcome of localized high-risk rhabdomyosarcoma.

Cited by 163 publications

References 31 publications

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

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

Higher rates of metastatic disease may explain the declining trend in Swedish paediatric rhabdomyosarcoma survival rates

Clinical characteristics and outcomes for children, adolescents and young adults with “CIC‐fused” or “BCOR‐rearranged” soft tissue sarcomas: A multi‐institutional European retrospective analysis

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