Prognostic Factors and Clinical Outcomes in Children and Adolescents With Metastatic Rhabdomyosarcoma—A Report From the Intergroup Rhabdomyosarcoma Study IV

Breneman, John C.; Lyden, Elizabeth; Pappo, Alberto S.; Link, Michael P.; Anderson, James R.; Parham, David M.; Qualman, Stephen J.; Wharam, Moody D.; Donaldson, Sarah S.; Maurer, Harold M.; Meyer, William H.; Baker, K. Scott; Paidas, Charles N.; Crist, William M.

doi:10.1200/jco.2003.06.129

Cited by 429 publications

(353 citation statements)

References 26 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%

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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%

“…Clinical presentation is also less favorable. All retrospective studies note more aggressive disease with more unfavorable sites 1, 36, 37, 38, 39, more LN, more metastasis 9, 36, 40, 41, and more A‐RMS. Nevertheless, all parameters being equal, the outcome of adult patients with RMS is less favorable.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2018

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“…As expected, the prognosis was worse for patients with metastatic disease than for patients with localised disease. The 5‐year overall survival rate for metastatic disease (48 ± 8%) was better than corresponding previously published results 7, 21, 22.…”

Section: Discussioncontrasting

confidence: 69%

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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“…All tumors were obtained from patients enrolled on the Intergroup Rhabdomyosarcoma Study Group IV protocol [25][26][27] or later studies. Cases were selected on the basis of submission by the treating institution of a paraffin block or formalinfixed tissue for banking at The Biopathology Center.…”

Section: Paraffin-embedded Rhabdomyosarcoma Tumors and Tissue Microarmentioning

confidence: 99%

Expression and genomic status of EGFR and ErbB-2 in alveolar and embryonal rhabdomyosarcoma

et al. 2006

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Both epidermal growth factor receptor (EGFR) and ErbB-2 play an important role in cancer biology and constitute promising molecular targets of therapy. EGFR and ErbB-2 expression has been observed in rhabdomyosarcoma cell lines but not analyzed systematically in rhabdomyosarcoma tumors. Tissue microarray sections representing 66 rhabdomyosarcoma tumors (34 embryonal rhabdomyosarcoma, 32 alveolar rhabdomyosarcoma) were surveyed by immunohistochemistry using antibodies specific for EGFR and ErbB-2. Immunostains were assessed for intensity (0: no immunostaining; 1: weak; 2: moderate; 3: strong) and percentage of at least 500 neoplastic cells exhibiting membranous or membranous and cytoplasmic immunostaining. EGFR and ErbB-2 expression was considered positive if the product of intensity and percentage was greater than 10. Patients had a median age of 5.7 years (range 8 months-19.1 years), and of 65/ 66 patients, 38 were males and 27 were females. Expression of ErbB-2 was identified in 22/66 (33%) cases and tended to be more frequent in the alveolar subtype (13/32, 41%, vs 9/34, 26%, P ¼ 0.30). Expression of EGFR was identified in 31/66 (47%) cases and correlated with the embryonal subtype (26/34, 76%, vs 5/32, 16%, Po0.0001) independent of stage, age, and gender. Coexpression of EGFR and ErbB-2 was identified in eight tumors, of which six were embryonal rhabdomyosarcoma. None of the cases exhibited EGFR or ErbB-2 gene amplification, as assessed using fluorescence in situ hybridization. Furthermore, analysis of 11 additional rhabdomyosarcoma tumors (six alveolar; five embryonal) revealed no evidence of mutations in EGFR exons 18, 19, 20, and 21. In summary, expression of EGFR and/or ErbB-2 is detected in a sizeable subset of rhabdomyosarcoma tumors without evidence of EGFR or ErbB-2 amplification or mutations in the EGFR tyrosine kinase domain. Notably, expression of EGFR correlates with the embryonal subtype, which is also more likely to coexpress EGFR and ErbB-2.

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Prognostic Factors and Clinical Outcomes in Children and Adolescents With Metastatic Rhabdomyosarcoma—A Report From the Intergroup Rhabdomyosarcoma Study IV

Cited by 429 publications

References 26 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

Expression and genomic status of EGFR and ErbB-2 in alveolar and embryonal rhabdomyosarcoma

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