Prognostic Factors in Metastatic Rhabdomyosarcomas: Results of a Pooled Analysis From United States and European Cooperative Groups

Abstract

A B S T R A C T PurposeTo identify risk factors associated with outcome in children with metastatic rhabdomyosarcoma in a large cohort of patients Patients and MethodsPooled data were obtained from 788 patients treated in nine studies performed by European and American cooperative groups. Clinical factors, including age, histology, site of primary, and site(s) and number of sites of metastatic disease, were correlated with event-free survival (EFS) and overall survival (OS). ResultsSeven hundred eighty-eight patients were eligible for analysis. The 3-year OS and EFS were 34% (SE, 1.7) and 27% (SE, 1.6), respectively. By univariate analysis, 3-year EFS was significantly and adversely influenced by age, alveolar histology, location of primary tumor in unfavorable site (defined as extremity and "other" sites), presence of three or more sites of metastatic disease, and the presence of bone or bone marrow involvement. By multivariate analysis, EFS was strongly correlated to all factors except histology. Relative risks were 1.6 for age younger than 1 year or at least 10 years, 1.4 for unfavorable site of primary tumor, 1.4 for bone or bone marrow involvement, 1.4 for three or more metastatic sites. EFS was 50% for patients without any of these four adverse factors and was respectively 42%, 18%, 12%, and 5% in patients with one, two, three, or four factors (P Ͻ .0001). ConclusionThis analysis identified subsets of patients with metastatic rhabdomyosaroma with different outcomes to current therapy and offers a strategy to define patient candidates for experimental approaches to treatment.

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

“…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.…”

“…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.…”

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

“…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.…”

Clinical Significance of Transnasal Endoscopic Surgery in the Treatment of Children With Localized Parameningeal Sarcomas

“…Результаты международных исследовательских групп IRS 1 и COG 2 убедительно доказали влияние на исход лечения детей с РМС таких независимых факторов, как локализация первичной опухоли, размер, инвазия в соседние органы и ткани, а так-же метастатическое поражение региональных лим-фатических узлов и наличие отдаленных метастазов [2][3][4][5] генетического исследования [10]. Алгоритм доле-чебного стадирования РМС согласно данным COG STSC представлен на рис.…”