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Background. The initial management of patients with sarcoma is critical. We used the French National Cancer Institute-funded nationwide sarcoma database NETSARC to report the management and oncologic outcomes in children, adolescents and young adults (AYAs) patients with sarcoma at the national level. Patients and methods. Data from patients with sarcoma are collected, regularly monitored and updated using NETSARC. NETSARC was searched for pediatric (Results. We collected data from 3,972 pediatric and AYA patients with sarcoma diagnosed between 2010 and 2017, including 714 children, 1,290 AYAs treated in RSC, and 937 AYAs in non-RSC. Significant differences in compliance to guidelines were observed regarding pre-treatment biopsy (Children: 83.5%; AYA in RSC: 85.9%; AYA in non-RSC 48.1%), pre-treatment imaging (Children: 81.2%; AYA in RSC: 86.8%; AYA in non-RSC: 56.5%) and R0 margins (Children: 52%; AYA in RSC 57.6%; AYA in non-RSC: 20.2%) (pConclusions. This study highlights the importance for AYA patients with sarcoma to be managed in national sarcoma reference centers involving multidisciplinary medical teams with paediatric and adult oncologists.

Complete surgical resection constitutes the mainstay of treatment for locally aggressive, rarely metastazing tumor and low-grade soft tissue sarcomas (LAS). Local relapse is the most common tumor event, especially in the presence of positive margins (R1 margins). The aims of this study are to assess the impact of the national network on patient care and to evaluate the role of immediate re-excision in children, adolescents and young adults with incompletely resected LAS.National retrospective multicenter study of all young patients (≤25 years) included in the Sarcoma "ConticaBase" treated for LAS between 2005 and 2017 for whom pathology/biology review was available via the national NETSARC + network.A total of 96 patients were identified (median age: 16 years). Tumors were localized in 99% of cases (1 N+ tumor). With a median follow-up of 4.7 years (range: 0.1-11.9), eight local relapses and two distant metastases were observed. No patient died. Overall 5-year event-free survival (EFS) was 90.4% [95%CI, 84.3-97]. Five year EFS for R1 patients (n = 51) with (n = 24) and without (n = 27) immediate re-excision was 90.5% [95%CI, 78.8-100.0] and 80.3% [95%CI, 64.7-99.9], respectively (p = 0.34). The 37 patients directly treated in a reference center more commonly had a diagnostic biopsy (78% vs. 21%; p 0.001), more complete surgery (R0: 65% vs. 14%; p 0.001) and less commonly underwent re-excision (16% vs. 54%; p 0.001).This large series indicates that LAS are rare in young patients and have a favorable prognosis. Immediate management in reference centers is associated with better standard of care. The main tumor events are local relapses.

Background Since 2010, nationwide networks of reference centers for sarcomas (RREPS/NETSARC/RESOS) collected and prospectively reviewed all cases of sarcomas and connective tumors of intermediate malignancy (TIM) in France. Methods The nationwide incidence of sarcoma or TIM (2013–2016) was measured using the 2013 WHO classification and confirmed by a second independent review by expert pathologists. Simple clinical characteristics, yearly variations and correlation of incidence with published clinical trials are presented and analyzed. Results Over 150 different histological subtypes are reported from the 25172 patients with sarcomas (n = 18712, 74,3%) or TIM (n = 6460, 25.7%), with n = 5838, n = 6153, n = 6654, and n = 6527 yearly cases from 2013 to 2016. Over these 4 years, the yearly incidence of sarcomas and TIM was therefore 70.7 and 24.4 respectively, with a combined incidence of 95.1/106/year, higher than previously reported. GIST, liposarcoma, leiomyosarcomas, undifferentiated sarcomas represented 13%, 13%, 11% and 11% of tumors. Only GIST, as a single entity had a yearly incidence above 10/106/year. There were respectively 30, 64 and 66 different histological subtypes of sarcomas or TIM with an incidence ranging from 10 to 1/106, 1–0.1/106, or < 0.1/106/year respectively. The 2 latter incidence groups represented 21% of the patients with 130 histotypes. Published phase III and phase II clinical trials (p−6) are significantly higher with sarcomas subtypes with an incidence above 1/106 per. Conclusions This nationwide registry of sarcoma patients, with exhaustive histology review by sarcoma experts, shows that the incidence of sarcoma and TIM is higher than reported, and that tumors with a very low incidence (16/year) are less likely to be included in clinical trials.

Introduction: This study evaluates the management of patients aged 0 to 30 years treated for sarcoma with data registered in the NETSARC French national database and compared the survival of adolescents and young adults (AYA) patients managed in reference sarcoma national centers (AYA in NC) and in non-NC (AYA outside NC). Patients and Methods: NETSARC (netsarc.org) is a network of 26 reference sarcoma centers funded by the French National Cancer Institute. Patients’ characteristics and follow-up are prospectively collected in a database regularly monitored and updated. Patients’ survival was compared between groups. The primary endpoint was overall survival (OS). Secondary endpoints were local progression-free survival (LPFS), progression-free survival (PFS), and the quality of initial surgery. Results: Among 3,972 patients aged 0 to 30 years diagnosed between 2010 and 2017, 714 children, 1,290 AYAs in NC and 937 AYAs outside NC were included. Compliance to guidelines between AYAs treated in versus outside reference centers was significantly different (p

Background: Since 2010, presentation to a designated sarcoma tumor board and pathological review by an expert network are mandatory for sarcoma patients in France. NETSARC+ (merging the 3 initial RREPS, RESOS & NETSARC) collected prospectively all cases of reviewed sarcomas and tumors of intermediate malignancy (TIM) nationwide. We report on the incidence of subtypes according to WHO classification from 2013 to 2016. Methods: Sarcoma or TIM confirmed by review of expert sarcoma pathologists were all prospectively integrated in the database; the results using the latest WHO classification are presented for the years 2013 to 2016, including yearly variations. Correlation of the incidence of each histotype with dedicated published clinical trials was conducted. Results: 139 different histological subtypes are reported among the 25172 patients with sarcomas (n = 18710, 64%) or TIM (n = 6460, 36%), respectively n = 5838, n = 6153, n = 6654, and n = 6527 yearly from 2013 to 2016. Over these 4 years, the observed yearly incidence of sarcomas, TIM, and both was therefore 79.7, 24.9 and 95.1/106/year, above that previously reported. GIST, liposarcoma, leiomyosarcomas, undifferentiated sarcomas represented 13%, 13%, 11% and 11% of all sarcomas. Only GIST, as a single entity exceeded a yearly incidence above 10/million per year. There were respectively 30, 63 and 66 different histological subtypes of sarcomas or TIM (single entities or lumped together, e.g. MPNST, or vascular sarcomas...) with an incidence ranging from 10 to 1/106/year, 1-0.1/106 per year, or < 0.1/106/year respectively. The 2 later “incidence groups” included 21% of the patients. The incidence of 8 histotypes varied significantly over this 4 years. Patients with tumors with an incidence above 1/106 per year have significantly higher numbers of dedicated published phase III and phase II clinical trials (p < 10-6). Conclusions: This nationwide registry of sarcoma patients with an histology reviewed by sarcoma experts shows that the incidence of sarcoma and TIM is higher than previously reported, may vary over years for some histotypes, and that tumors with an incidence < 10e6 have a much lower access to clinical trials. Funding Statement: NetSARC (INCA & DGOS) and RREPS (INCA & DGOS), RESOS (INCA & DGOS) and LYRICAN (INCA-DGOSINSERM 12563), Institut Convergence PLASCAN (17-CONV-0002), Association DAM’s, Ensemble contre Le GIST, Eurosarc (FP7-278742), la Fondation ARC, Infosarcome, InterSARC (INCA), LabEx DEvweCAN (ANR-10-LABX-0061), Ligue de L’Ain contre le Cancer, La Ligue contre le Cancer, EURACAN (EC 739521) funded this study. Declaration of Interests: The funders had no role in the study design, data collection, data analysis, data interpretation, or writing of the report.

Les sarcomes sont des cancers rares et heterogenes, dont la prise en charge est complexe et non optimale pour la majorite des patients. Les trois elements cles de la prise en charge sont l’obtention d’un diagnostic avere, la discussion pluridisciplinaire du dossier en reunion de concertation pluridisciplinaire specialisee et la chirurgie effectuee dans une equipe entrainee. Afin d’ameliorer significativement la prise en charge des patients, trois reseaux nationaux anatomopathologiques et cliniques dedies aux sarcomes/GIST/tumeurs desmoides et constitues de centres experts regionaux ont ete structures en 2010 avec le soutien de l’Institut national du cancer et de la direction generale de l’Offre de soins: les reseaux RRePS, NetSarc et ResOs. Une base de donnees partagee a ete mise en oeuvre avec pour objectif de collecter differents indicateurs de qualite de prise en charge et de suivre leur evolution a l’echelle nationale. Apres cinq annees d’activite, les trois reseaux couvrent entre 73 et 91 % des 4 000 nouveaux patients annuels attendus. Cette organisation en reseaux de soins apporte de nombreux benefices pour les patients: elle permet de faciliter l’acces aux expertises anatomopathologiques et cliniques ainsi qu’une meilleure prise en charge initiale (imagerie, biopsie), une meilleure qualite de chirurgie et un meilleur acces aux traitements innovants. Cette organisation permet egalement de structurer la recherche, d’optimiser l’utilisation de techniques diagnostiques et la prescription de traitements couteux et de standardiser la collecte de donnees de prise en charge et de suivi a l’echelle nationale.

Rare cancer patients face lower survival and experience delays in diagnosis and therapeutic mismanagement. Considering the specificities of rare cancers, referral networks have been implemented in France to improve the management and survival of patients. The IG&eacute, AS research program aims to assess the networks&rsquo, ability to reduce inequalities. Data analysis of the IG&eacute, AS cohort (n = 20,590, sarcoma diagnosed between 2011 and 2014) by gathering medical data and geographical index will identify risk factors associated with the belated access to expertise or with no access to expertise. Intermediate results show that referral networks give sarcoma patients access to sarcoma expertise despite the remoteness of some of them. Regional expert centers mostly receive requests from within their area while national referral centers receive requests from the whole country. Delays in the access to expertise may be reduced by making outside practitioners more sensitive to the issues of rare cancers. The perception and involvement of outside practitioners in this device will be assessed using a qualitative survey. All the results are discussed and will contribute to design guidelines to improve early access to expertise and reduce inequalities. Results of the IG&eacute, AS research program may contribute to the assessment of referral sarcoma networks and provide some useful lessons to improve cancer care management.

Hydroformylation of alkenes can be carried out in short time and with low syngas pressure under microwave (MW) dielectric heating. Alkenes, carrying O-, N-, or C-nucleophilic fragments, can be designed for domino reactions, mainly cyclocondensations. Allyl and homoallyl alcohols are excellent substrates for cyclizative hydroformylation to lactols under MW heating. In the presence of NaOAc as an additional nucleophile, a domino reaction occurs giving 2-acetoxytetrahydrofurans, suitable to introduce a C-nucleophile on tetrahydrofuran rings through an oxocarbenium ion. The synthesis of the furanopiperidine substructure of cyclopamine is described as an application. With alkene amides, the domino process collapsed to a transient acyliminium ion that further cyclized with an additional C-nucleophile. To perform domino hydroformylation Pictet-Spengler or aza-Sakurai reactions, an autoclave under conventional heating is essential. Syntheses of (±)-epilupinine and of a homoberberine alkaloid are reported to illustrate each sequence. Although apparently more versatile, hydroformylation of alkenes using MW heating is sensitive to the nature of the nucleophiles present in the substrates, evidencing that the conventional heating process cannot be completely replaced by MW irradiation.

11564Background: Soft tissue sarcomas (STS) are rare tumors accounting for less than 1% of human cancers. While the highest incidence of sarcomas is observed in elderly, this population is often ex...

The French National Cancer Institute suggested the improvement of sarcoma management when performed in a multidisplinary expert team. Since 2009, a network of 26 reference multidisciplinary boards has been created to improve the quality of care of sarcoma patients in France. All patient cases presented are uploaded in the NetSarc network collecting patient characteristics, clinical situation at the time of the discussion, medical decision, and patient outcome (survival and relapse). Patients and methods From January 2010 to April 2012, 8934 patients were included on this database. Patients were presented from centers within (N = 5911, 66%) or outside (N = 2343, 26%) the Netsarc Network (parameter unknown in N = 679, 8% patient). Patient and disease characteristics and treatment results are described. Results There were 4662 women (52%) and 4272 men (48%). Median size of the sarcoma was 95mm (range2- > 400). Soft tissue, visceral, and bone represented 6381 (71%) 1285 (14%) and 1264 (14%) of patients respectively. In soft tissue tumours, grade 1, 2, 3 were reported in 44%, 33%, 23% of patients. The most frequent histological subtypes were leiomyosarcomas (all sites) (n = 769, 9%), GIST (558, 6%), dedifferentiated liposarcomas (n = 439, 5%). Most frequent primary sites were thigh (1268, 14%), retroperitoneal (796, 9%), thoracic wall (460, 5%), uterus (405, 4.5%), legs (325, 4%). 1052 (11%) patients were reported to have metastases. Clinical and disease status at the time of discussion was prior to: biopsy (N = 824, 9%), first surgery (N = 1751, 20%), adjuvant treatment (N = 2806, 31%). 6161 (67%) patients had a surgery, n = 1472 (24%) in the Netsarc network and outside n = 1843 (30%); in n = 2846 (46%) this information is not still documented. Patients whose primary surgery was performed in Netsarc centers had R0, R1, R2, and fragmented primary surgery in 61%, 31%, 5%, 2% vs 37%, 40%, 16%, 7% in primary care centers non-Netsarc centers (p Conclusions This first analysis of the NetSarc database shows that more patients had optimal surgery in reference centers than in primary care centers. This tool will ensure the monitoring of dedicated action to improve the management of sarcoma patients in the future. Disclosure All authors have declared no conflicts of interest.