Rare bone sarcomas: a retrospective analysis of 145 adult patients from the French Sarcoma Group.

Fiche publication

Date publication

octobre 2021

Journal

International journal of cancer

Auteurs

Membres identifiés du Cancéropôle Est :
Dr CHAIGNEAU Loïc , Pr KURTZ Jean-Emmanuel

Tous les auteurs :
Boudou-Rouquette P, Martin E, Kempf E, Penel N, Toulmonde M, Bompas E, Duffaud F, Firmin N, Bertucci F, Kurtz JE, Chaigneau L, Isambert N, Saada-Bouzid E, Dubray-Longeras P, Larousserie F, Anract P, Chevreau C, Blay JY, Piperno-Neumann S,

Lien Pubmed

https://www.ncbi.nlm.nih.gov/pubmed/34611903

Résumé

The benefit of chemotherapy (CT) in rare bone sarcomas is not documented in prospective studies. This retrospective study from the French sarcoma network for bone tumors ResOs was performed in adult patients (pts) from 1976 to 2014, with histologically verified diagnosis of leiomyosarcomas (LMS), undifferentiated pleomorphic (UPS), or radiation-associated sarcomas of bone. The median follow-up was 4.7 years (95% CI 3.7-6.5). Clinical features, treatment modalities and outcomes were recorded and analyzed from 145 pts (median age 53 years [range 20-87]). Site of disease was extremities (66%) or axial skeleton (34%), 111 (77%) presented with localized and potentially resectable disease. The most common histological subtypes were UPS (58%) and LMS (33%); 58% were high-grade tumors. Surgery was performed in 127 pts. In the 111 localized pts, 28 pts (25%) underwent upfront surgery or exclusive radiotherapy (RT; > 50 Grays) without CT, whereas 83 pts (75%) received either neo-adjuvant (n = 26) or adjuvant CT (n = 13) or both (n = 44). Neo-adjuvant and adjuvant CT was mostly doxorubicin- (95%/86%) and cisplatin- (67%/63%) based. R0 resection was achieved in 59 pts, and a good histological response in 15 patients (25%). Adjuvant RT was performed in 24 (22%) pts. For the whole cohort (n=145), the 5-year overall survival (OS) rate was 53% [42; 62]. In univariate analysis, age ≤60 was associated with a longer disease-free survival (DFS)(p = 0.0436). Neo-adjuvant and adjuvant CT tended to be associated with better DFS (p=0.056) with no significant impact on OS in this retrospective series. This article is protected by copyright. All rights reserved.