TY - JOUR
T1 - Parameningeal rhabdomyosarcoma in pediatric age
T2 - Results of a pooled analysis from North American and European cooperative groups
AU - Merks, J. H.M.
AU - de Salvo, G. L.
AU - Bergeron, C.
AU - Bisogno, G.
AU - de Paoli, A.
AU - Ferrari, A.
AU - Rey, A.
AU - Oberlin, O.
AU - Stevens, M. C.G.
AU - Kelsey, A.
AU - Michalski, J.
AU - Hawkins, D. S.
AU - Anderson, J. R.
PY - 2014/1
Y1 - 2014/1
N2 - Background: Parameningeal (PM) site is a well-known adverse prognostic factor in children with localized rhabdomyosarcoma (RMS). To identify risk factors associated with outcome at this site, we pooled data from 1105 patients treated in 10 studies conducted by European and North American cooperative groups between 1984 and 2004. Patients and methods: Clinical factors including age, histology, size, invasiveness, nodal involvement, Intergroup Rhabdomyosarcoma Study (IRS) clinical group, site, risk factors for meningeal involvement (MI), study group, and application of radiotherapy (RT) were studied for their impact on event-free and overall survival (EFS and OS). Results: Ten-year EFS and OS were 62.6 and 66.1% for the whole group. Patients without initial RT showed worse survival (10-year OS 40.8% versus 68.5% for RT treated patients). Multivariate analysis focusing on 862 patients who received RT as part of their initial treatment revealed four unfavorable prognostic factors: age <3 or >10 years, signs of MI, unfavorable site, and tumor size. Utilizing these prognostic factors, patients could be classified into different risk groups with 10-year OS ranging between 51.1 and 80.9%. Conclusions: While, in general, PM localization is regarded as an adverse prognostic factor, the current analysis differentiates those with good prognosis (36% patients with 0-1 risk factor: 10-year OS 80.9%) from high-risk PM patients (28% with 3-4 factors: 10-year OS 51.1%). Furthermore, this analysis reinforces the necessity for RT in PM RMS.
AB - Background: Parameningeal (PM) site is a well-known adverse prognostic factor in children with localized rhabdomyosarcoma (RMS). To identify risk factors associated with outcome at this site, we pooled data from 1105 patients treated in 10 studies conducted by European and North American cooperative groups between 1984 and 2004. Patients and methods: Clinical factors including age, histology, size, invasiveness, nodal involvement, Intergroup Rhabdomyosarcoma Study (IRS) clinical group, site, risk factors for meningeal involvement (MI), study group, and application of radiotherapy (RT) were studied for their impact on event-free and overall survival (EFS and OS). Results: Ten-year EFS and OS were 62.6 and 66.1% for the whole group. Patients without initial RT showed worse survival (10-year OS 40.8% versus 68.5% for RT treated patients). Multivariate analysis focusing on 862 patients who received RT as part of their initial treatment revealed four unfavorable prognostic factors: age <3 or >10 years, signs of MI, unfavorable site, and tumor size. Utilizing these prognostic factors, patients could be classified into different risk groups with 10-year OS ranging between 51.1 and 80.9%. Conclusions: While, in general, PM localization is regarded as an adverse prognostic factor, the current analysis differentiates those with good prognosis (36% patients with 0-1 risk factor: 10-year OS 80.9%) from high-risk PM patients (28% with 3-4 factors: 10-year OS 51.1%). Furthermore, this analysis reinforces the necessity for RT in PM RMS.
KW - Meningeal involvement
KW - Parameningeal
KW - Radiotherapy
KW - Rhabdomyosarcoma
UR - http://www.scopus.com/inward/record.url?scp=84905391526&partnerID=8YFLogxK
U2 - 10.1093/annonc/mdt426
DO - 10.1093/annonc/mdt426
M3 - Article
C2 - 24356633
AN - SCOPUS:84905391526
SN - 0923-7534
VL - 25
SP - 231
EP - 236
JO - Annals of Oncology
JF - Annals of Oncology
IS - 1
ER -