Centre Léon Bérard, Lyon, France
Pauline Rochefort , Antoine Italiano , Valerie Laurence , Nicolas Penel , Audrey Lardy-Cleaud , Olivier Mir , Christine Chevreau , Francois Bertucci , Emmanuelle Bompas , Loic Chaigneau , Dominique Levy , Thomas Ryckewaert , Sarah Naomie Dumont , Pierre Meeus , Dominique Ranchere , Jean-Yves Blay , Philippe Alexandre Cassier
Background: Ewing’s sarcoma family of tumorsis a group of rare and aggressive tumors. Data on tumor, clinical presentation and treatment in patients (pts) older than 50 years are limited. Outcomes of older pts with EFTs have been reported to be worse than in pediatric population in some studies but with discordant results. Methods: We conducted a retrospective analysis among centers of affiliated with the French Sarcoma Group (GSF-GETO) focusing on pts diagnosed with EFTs at age ≥ 50 between 2000 and 2012. Clinical features, treatment modality and outcomes were recorded and analyzed. Results: 77 pts were identified. There were 36 females (47%) and themedian age at diagnosis was 56 years (range: 50-86). The primary tumor was located in the soft tissue in 59 pts (76.6%) and in the bone in 18 (23.4%). 56 pts (72.8%) had localized disease whereas 21 (27.2%) presented with metastases at diagnosis. Median follow-up was 71 months (m) and median overall survival (OS) was 92.8 m for the whole cohort. Among 56 pts with localized disease, 50 (87.7%) received chemotherapy (CT) in addition to local therapy (surgery in 19 pts, surgery and RT in 37 pts and RT only in 7 pts), and among these, CT was considered intensive in 36 pts (63%). For patients with localized disease, the estimated 3-year OS and relapse free survival (RFS) rates were respectively 73.3% and 62.2%. Recurrence occurred in 43 pts, with a median time to recurrence of 18.2 m. Recurrences were most often metastatic only (62.7%), followed by local only (23%). Recurrence site was lung for 16 pts (37.2%) and bone for 9 pts (20.9%). Median post-recurrence survival was 9.5 m. In univariate analysis only high LDH were marginally associated with worse overall survival in patient with localized disease. All pts with metastatic disease at presentation received CT, their estimated 3-year OS rate was 37%. Conclusions: EFTs characteristics in patients ≥ 50 years differ from the pediatric population: they had more likely soft tissue primary tumor and metastatic disease. The outcomes were worse than in younger pts but could be partially explained by less intensive therapy. The management of Ewing sarcoma in adult pts remains to be defined.
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