Penel Nicolas, Coindre Jean-Michel, Bonvalot Sylvie, Italiano Antoine, Neuville Agnès, Le Cesne Axel, Terrier Philippe, Ray-Coquard Isabelle, Ranchere-Vince Dominique, Robin Yves-Marie, Isambert Nicolas, Ferron Gwennaël, Duffaud Florence, Bertucci François, Rios Maria, Stoeckle Eberhad, Le Pechoux Cécile, Guillemet Cécile, Courreges Jean-Baptiste, Blay Jean-Yves
Medical Oncology Department, Centre Oscar Lambret, 3 rue Combemale, 59020 Lille Cedex, France; SIRIC OncoLille Consortium, Clinical Research and Methodology Platform, 59037 Lille, France.
Pathology Department, Institut Bergonié, 229 Cours de l'Argonne, 33000 Bordeaux, France.
Eur J Cancer. 2016 May;58:90-6. doi: 10.1016/j.ejca.2016.02.008. Epub 2016 Mar 11.
The optimal management of rare tumours (i.e. from accurate diagnosis to management in reference centres) is a public health challenge. In 2009, the French National Cancer Institute (INCa) identified and financially supported the two expert networks for pathological and clinical diagnosis and management of soft tissue tumours.
The activities of both networks were prospectively collected using a nationwide database (rreps.org). Data describing the diagnosis management of 863 successive cases of desmoids tumours (DT) were prospectively collected from 2010 to 2013 and analysed.
The number of confirmed DT constantly improved from January 2010 to December 2013 (from 173 to 273 cases per year); the expected incidence ranged from 132 to 330 cases/year. The rate of cases diagnosed with core-needle biopsies and CTNNB1 mutational status analysis increased from 30.6 to 40.7% and from 87.8 to 94.1%, respectively. The mean delay for pathological diagnosis confirmation constantly decreased from 107 to 47 d. Among the 846 adult patients, 414 (48.9%) patients were treated by reference centres. The rate of patients managed by reference centres constantly increased with time from 36.9 to 49.5% since 2010. The median management time of the referral centres constantly decreased from 440 to 67 d.
The two expert networks worked synergistically and improved diagnosis modalities of rare desmoid tumours at a national level. The impact of management by expert networks on the outcome will be prospectively analysed in the future.
罕见肿瘤的最佳管理（即从准确诊断到在参考中心进行管理）是一项公共卫生挑战。2009年，法国国家癌症研究所（INCa）确定并资助了两个软组织肿瘤病理和临床诊断及管理专家网络。
使用全国性数据库（rreps.org）前瞻性收集两个网络的活动数据。从2010年到2013年前瞻性收集并分析了863例连续的韧带样型纤维瘤病（DT）病例的诊断管理数据。
2010年1月至2013年12月，确诊的DT病例数持续增加（从每年173例增至273例）；预期发病率为每年132至330例。经粗针穿刺活检诊断的病例率和CTNNB1突变状态分析率分别从30.6%增至40.7%和从87.8%增至94.1%。病理诊断确认的平均延迟时间从107天持续降至47天。在846例成年患者中，414例（48.9%）患者由参考中心治疗。自2010年以来，由参考中心管理的患者比例随时间持续增加，从36.9%增至49.5%。转诊中心的中位管理时间从440天持续降至67天。
这两个专家网络协同工作，在国家层面改善了罕见韧带样型纤维瘤病的诊断方式。未来将前瞻性分析专家网络管理对结果的影响。
