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Soft tissue sarcoma in children, adolescents and young adults: Outcomes according to compliance with international initial care guidelines

Authors :
Gaëlle Pierron
S. Pannier
Alexia Savignoni
Daniel Orbach
Hervé Brisse
Arnaud Gauthier
C. Collignon
Sabine Sarnacki
Liesbeth Cardoen
B. Tiléa
Sylvie Helfre
Olivia Boccara
Pascale Philippe-Chomette
Matthieu Carton
CHU Necker - Enfants Malades [AP-HP]
Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
Université de Paris (UP)
AP-HP Hôpital universitaire Robert-Debré [Paris]
Département de radiothérapie oncologique [Paris]
Institut Curie [Paris]
Imagine - Institut des maladies génétiques (IHU) (Imagine - U1163)
Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP)
Source :
EJSO-European Journal of Surgical Oncology, EJSO-European Journal of Surgical Oncology, WB Saunders, 2020, 46, pp.1277-1286. ⟨10.1016/j.ejso.2019.11.518⟩
Publication Year :
2020
Publisher :
Elsevier BV, 2020.

Abstract

Outcomes for adults with soft tissue sarcoma are better when managed at referral centers. Care guidelines advise for 5 main criteria: 1-Imaging before biopsy; 2-Tumor biopsy before surgery; 3-Multidiscipinary team discussion (MTD) before biopsy; 4-Biopsy in "expert centers"; 5-Somatic molecular biology feasible. The aim is to describe and assess the prognostic impact of initial management of STS according to the type of referring centers and the number of optimal criteria.Monocentric retrospective analysis of the management of 127 youths (0-25 years) with localized STS treated from 2006 to 2015.Median age at diagnosis was 9.6 years (range: 025). Overall, only 41% patients had 5/5, 28% 3-4, 31% ≤2. No adequate imaging was performed before surgery/biopsy for 18% patients, no biopsy before treatment for 29%. Patients referred by "expert centers" had higher compliance to guidelines (P = 0.025). Upfront surgery was performed in 59/127 patients. Immediate re-operation was inversely related to the number of criteria (0% when 5 criteria vs. 14% for 3-4, 46% if ≤ 2; P 0.001). For malignant tumors, outcome was better when 5 criteria were reached: 5 year EFS 90.8% (81.4-100.0%) vs. 71.6 for (60.4-84.9%; ≤4 criteria; p = 0.033), OS 93.6% (85.5-100%) vs. 79.5% (68.9-91.8%; p = 0.11), and LRFFS 90.6% (81.0-100.0) vs. 73.1% (62.0-86.3%; p = 0.047).Less than half of the youths with STS are initially managed according to international guidelines, highlighting the need for better information about optimal management. These results plead for immediate management in reference centers to reduce initial burden of therapy.

Details

ISSN :
07487983
Volume :
46
Database :
OpenAIRE
Journal :
European Journal of Surgical Oncology
Accession number :
edsair.doi.dedup.....fb729a22298c2980e85de8661bf5cf22
Full Text :
https://doi.org/10.1016/j.ejso.2019.11.518