PAX3-NCOA1 alveolar rhabdomyosarcoma of the tongue: A rare entity with challenging diagnosis and management.

AbstractAlveolar rhabdomyosarcoma (ARMS) is associated with PAX3/PAX7‐FOXO1 fusion, which confers specific clinic and biologic characteristics with inferior outcomes. A minority of tumors still histologically classified as "true" ARMS lack the canonical PAX‐FOXO1 fusion but have new molecular alterations. We present the first case of PAX3‐NCOA1 ARMS with clinical data and follow‐up in a two‐year‐old girl with ARMS of the tongue and nodal extension, treated with chemotherapy, hemi glossectomy, lymph node dissection, and brachytherapy to conserve oral function and limit long‐term sequelae. Given the rarity of such variant fusion in ARMS, international collaboration is required to evaluate its prognostic value

Ability of ecological deprivation indices to measure social inequalities in a French cohort

Background: Despite the increasing interest in place effect to explain health inequalities, there is currently no consensus on which kind of area-based socioeconomic measures researchers should use to assess neighborhood socioeconomic position (SEP). The study aimed to evaluate the reliability of different area-based deprivation indices (DIs) in capturing socioeconomic residential conditions of French elderly women cohort. Methods: We assessed area-based SEP using 3 DIs: Townsend Index, French European Deprivation Index (FEDI) and French Deprivation index (FDep), among women from E3N (Etude épidémiologique auprès des femmes de la Mutuelle Générale de l’Education Nationale). DIs were derived from the 2009 French census at IRIS level (smallest geographical units in France). Educational level was used to evaluate individual-SEP. To evaluate external validity of the 3 DIs, associations between two well-established socially patterned outcomes among French elderly women (smoking and overweight) and SEP, were compared. Odd ratios were computed with generalized estimating equations to control for clustering effects from participants within the same IRIS. Results: The analysis was performed among 63,888 women (aged 64, 47% ever smokers and 30% overweight). Substantial agreement was observed between the two French DIs (Kappa coefficient = 0.61) and between Townsend and FEDI (0.74) and fair agreement between Townsend and FDep (0.21). As expected among French elderly women, those with lower educational level were significantly less prone to be ever smoker (Low vs. High; OR [95% CI] = 0.43 [0.40–0.46]) and more prone to being overweight (1.89 [1.77–2.01]) than women higher educated. FDep showed expected associations at area-level for both smoking (most deprived vs. least deprived quintile; 0.77 [0.73–0.81]) and overweight (1.52 [1.44–1.62]). For FEDI opposite associations with smoking (1.13 [1.07–1.19]) and expected association with overweight (1.20 [1.13–1.28]) were observed. Townsend showed opposite associations to those expected for both smoking and overweight (1.51 [1.43–1.59]; 0.93 [0.88–0.99], respectively). Conclusion: FDep seemed reliable to capture socioeconomic residential conditions of the E3N women, more educated in average than general French population. Results varied strongly according to the DI with unexpected results for some of them, which suggested the importance to test external validity before studying social disparities in health in specific populations

Management of the irradiated N0-neck during salvage pharyngo-laryngeal surgery

Background: Salvage surgeries are challenging procedures, with an associated poor prognosis. Management of the N0 neck in those situations remains controversial. We aim to compare oncologic outcomes regarding neck management after surgery for N0 pharyngo-laryngeal carcinoma occurring after loco-regional radiotherapy. Methods: We conducted a multicentric retrospective study, including all patients undergoing surgery for persistent, recurrent or new primary N0 carcinoma of the oropharynx, hypopharynx or larynx between 2005 and 2015, following loco-regional radiotherapy. Results: A total of 239 patients were included, concerning respectively 44%, 34% and 22% oropharyngeal, laryngeal and hypopharyngeal tumors operated. A neck dissection was performed in 143 patients (60%), with an occult nodal metastasis rate of 9%. This rate was higher for hypopharyngeal carcinomas (18%, p = 0.16) and tumors with initial nodal involvement (16%, p = 0.05). With a median follow-up of 60 months, the median overall survival (OS) and progression-free survival rates (PFS) were 34 months and 25 months. We identified negative margin excision status, age at the time of surgery (under 60) and delay between RT and surgery over 2 years as the only variables associated with better OS (p < 0.0001 and p = 0.004) and PFS (p < 0.0001 and p = 0.010) in multivariable analysis, with no difference regarding neck management. Regional progression (alone or with distant metastasis) was noted in 10 cases: 4 in the neck observation group (4%) versus 6 in the neck dissection group (4%). Conclusion: Elective lymph node dissection of irradiated neck should not be routinely performed in patients undergoing surgery for persistent, recurrent or new primary pharyngo-laryngeal carcinoma

DE LA CARTE OUTIL À LA CARTE SOURCE Représentation et gestion du fleuve Loire aux 18 e –19 e siècles

La recherche pluridisciplinaire entreprise sur la Loire pour comprendre son fonctionnement et son aménagement nous amène à utiliser des sources aussi diverses que variées 1. Sur un pas de temps large, du 18 e siècle à nos jours, le croisement de sources actuelles issues de relevés de terrain et de données archivistiques (manuscrits, plans, cartes et schémas) permet de réaliser une diachronie de la Loire. Notre objectif est à la fois de comprendre l'évolution morpho-dynamique du fleuve (formation d'îles, déplacements du lit) et d'observer les héritages laissés par les sociétés, du point de vue notamment des aménagements (digues, levées), des politiques de gestion, des techniques et des vulnérabilités. Nous nous proposons de montrer la méthodologie mise en œuvre dans ce travail sur le fleuve Loire en décrivant chacune des sources potentiellement utilisables pour l'étude diachronique. Cette approche géo-historique, initiée par le travail de Roger Dion (Dion 1934,1961) sur le val de la Loire, permet de comprendre comment les cartes anciennes, confrontées à des sources modernes comme les photographies aériennes, peuvent constituer de véritables outils d'analyse. La zone étudiée concerne la Loire bourbonnaise, nivernaise et moyenne (de Decize à Orléans) (fig. 1). Grâce à une représentation cartographique de synthèse (SIG), nous essaierons de comprendre si les «anciens» ouvrages fluviaux ont influé sur la dynamique de la Loire. Ne seraient-ils pas la raison aggravante des inondations du fleuve car leur fonction première n'était pas celle de protéger les sites contre les inondations ? Un système d'information géographique (SIG) historique, dont nous énumérerons les étapes de développement, permet de retracer l'évolution morpho-dynamique du fleuve et des aménagements fluviaux. Nous nous demanderons si les ouvrages fluviaux ont eu un effet amplifiant sur les crues en renforçant le resserrement du lit, ce qui favorise la montée des eaux et accentue la vulnérabilité des sites. Afin de comprendre le fonctionnement et l'aménagement actuels de la Loire, nous avons été amenés à utiliser une grande diversité de sources. Notre étude se faisant sur un pas de temps long (du 18 e siècle à nos jours) croise des sources actuelles (relevés de terrain) avec des données archivistiques. Notre démarche a permis de réaliser une diachronie représentative de la Loire et de reconstituer ainsi son évolution (formation d'îles), les déplacements de son lit et les effets des héritages (aménagement, politiques de gestion, technique). Les cartes anciennes des 18 e et 19 e siècles ont servi de base à un système d'information géohistorique tendant à démontrer graphiquement cette évolution, ses manifestations et ses effets

HERITAGE ET ENSEIGNEMENT DES STRATEGIES DE LUTTE CONTRE LE RISQUE D'INONDATION DE LA LOIRE DEPUIS LE 18 EME SIECLE

International audienceHeritage and knowledge strategies of risk management of the Loire river floods since the 18 ème century The geo-historic approach of the management strategies against the risk of flood in the Loire Nivernaise makes it possible to carry out a typology of the fluvial engineering works and to identify their evolution since the 18th century. The "levees" (longitudinal dykes) were initially built to improve the navigation. Later, the engineer adapts these installations against the floods of the river. The evolutiont of engineering works required the multiplication of the observations on the river works directed after the large floods of 1846, 1856 and 1866. The role of the engineers appears essential as well for installation as the of strategies against the floods of the river. Because of their transformations, the engineering works are nowadays major works of protection against floods.Héritage et enseignement des stratégies de gestion du risque d'inondation de la Loire depuis le 18 ème siècle L'approche géo-historique des stratégies de lutte contre le risque d'inondation en Loire Nivernaise permet de réaliser une typologie des aménagements de protection et d'identifier leur évolution depuis le 18è siècle. Les levées ont été construites, initialement, pour répondre aux impératifs de navigation. Par la suite, l'ingénieur les a adaptées pour en faire des ouvrages de lutte contre les crues du fleuve. L'ajustement des aménagements a exigé la multiplication des observations sur les ouvrages fluviaux dans le cadre d'un programme de lutte contre les crues né à la suite des inondations historiques de 1846, 1856 et 1866 (phrase pas claire). Le rôle des ingénieurs apparaît comme essentiel tant pour l'aménagement que l'établissement de stratégies de lutte contre les inondations du fleuve. Ils ont modifié et adapté les aménagements tant et si bien que les levées de navigation ont traversé les siècles pour devenir aujourd'hui des ouvrages majeurs de protection contre les inondations

Quand navigation rime avec inondation !

International audienc

De la ville vulnérable à la ville durable face au risque d'inondation : le rôle des aménagements fluviaux en Loire Nivernaise (Nevers et Charité-sur-Loire)

International audienc

Héritages et influences contemporaines des anciens ouvrages de navigation de la Loire moyenne (France)

International audienc

Factors associated with under-reporting of head and neck squamous cell carcinoma in cause-of-death records: A comparative study of two national databases in France from 2008 to 2012

International audienceObjective: To date, no study has evaluated the detection rate of head and neck squamous cell carcinoma (HNSCC) in cause-of-death records in Europe. Our objectives were to compare the number of deaths attributable to HNSCC from two national databases in France and to identify factors associated with under-reporting of HNSCC in cause-of-death records.Methods: The national hospital discharge database and the national underlying cause-of-death records were compared for all HNSCC-attributable deaths in adult patients from 2008 to 2012 in France. Factors associated with under-reporting of HNSCC in cause-of-death records were assessed using multivariate Poisson regression.Results: A total of 41,503 in-hospital deaths were attributable to HNSCC as compared to 25,647 deaths reported in national UCoD records (a detection rate of 62%). Demographics at death were similar in both databases with respect to gender (83% men), age (54% premature deaths at 25–64 years), and geographic distribution. In multivariate Poisson regression, under-reporting of HNSCC in cause-of-death records significantly increased in 2012 compared to 2010 (+7%) and was independently associated with a primary HNSCC site other than the larynx, a former primary or second synchronous cancer other than HNSCC, distant metastasis, palliative care, and death in hospitals other than comprehensive cancer care centers. The main study results were robust in a sensitivity analysis which also took into account deaths outside hospital (overall, 51,129 HNSCC-attributable deaths; a detection rate of 50%). For the year 2012, the age-standardized mortality rate for HNSCC derived from underlying cause-of-death records was less than half that derived from hospital discharge summaries (14.7 compared to 34.1 per 100,000 for men and 2.7 compared to 6.2 per 100,000 for women).Conclusion: HNSCC is largely under-reported in cause-of-death records. This study documents the value of national hospital discharge databases as a complement to death certificates for ascertaining cancer deaths