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Pancreatic neuroendocrine neoplasms (pNENs) diagnosed in childhood are very rare, with few data available. The aim was to describe the clinical presentation and behavior of children with pNENs at a national level. METHODS: National multicenter retrospective study of all patients, aged from 0 to 17 years at diagnosis, treated from 2011 to 2020 for a pNEN and registered in the French National Registry of Childhood Cancers or FRACTURE database. RESULTS: Fifteen patients, 13 well-differentiated pancreatic neuroendocrine tumors (pNETs) and two neuroendocrine carcinomas (pNECs), were selected. Median age at diagnosis was 14 years (range, 7-17). Eight patients, all with localized disease, had a cancer predisposition syndrome (CPS), including five cases diagnosed during systematic screening. Five (31%) had metastatic disease at diagnosis: three grade 2 pNETs and two pNECs. First line therapy included exclusive pancreatectomy (seven cases, all M0), active surveillance (three cases, all M0), medical therapies (somatostatin analogues, chemotherapy; four cases, all M1), and surgery with medical therapy (one M1 case). Three-year progression-free survival was 57% (confidence interval [CI] 95%: 27-78) and was significantly better for patients with low-grade well differentiated (73 vs. 0%; p < 10-4) and localized (76 vs. 20%; p = .02) tumors. The two patients with pNECs died. Three-year overall survival was 92% (CI95%: 59-99) and was significantly better in patients with low-grade tumor (100 vs. 50%; p = 10-4). CONCLUSION: Childhood pNENs occur more frequently in adolescents with CPS. Localized low-grade pNETs in children have a very good prognosis, whereas the treatment of high-grade and metastatic pNETs/pNECs should be better defined.
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INTRODUCTION: The recruitment step of all clinical trials is time consuming, harsh and generate extra costs. Artificial intelligence tools could improve recruitment in order to shorten inclusion phase. The objective was to assess the performance of an artificial intelligence driven tool (text mining, machine learning, classification ) for the screening and detection of patients, potentially eligible for recruitment in one of the clinical trials open at the "Institut de Cancérologie de Lorraine". METHODS: Computerized clinical data during the first medical consultation among patients managed in an anticancer center over the 2019-2023 period were used to study the performances of an artificial intelligence tool (SAS® Viya). Recall, precision and F1-score were used to determine the artificial intelligence algorithm effectiveness. Time saved on screening was determined by the difference between the time taken using the artificial intelligence-assisted method and that taken using the standard method in clinical trial participant screening. RESULTS: Out of 9876 patients included in the study, the artificial intelligence algorithm obtained the following scores: precision of 96 %, recall of 94 % and a 0.95 F1-score to detect patients with breast cancer (n=2039) and potentially eligible for inclusion in a clinical trial. The screening of 258 potentially eligible patient's files took 20s per file vs. 5min and 6s with standard method. DISCUSSION: This study suggests that artificial intelligence could yield sizable improvements over standard practices in several aspects of the patient screening process, as well as in approaches to feasibility, site selection, and trial selection.
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Algoritmos , Inteligencia Artificial , Ensayos Clínicos como Asunto , Selección de Paciente , Humanos , Femenino , Neoplasias de la Mama/diagnóstico , Minería de Datos/métodos , Persona de Mediana Edad , Determinación de la Elegibilidad/métodos , Aprendizaje Automático , Anciano , Masculino , Factores de Tiempo , Neoplasias/diagnósticoRESUMEN
BACKGROUND: Real-world data (RWD) related to the health status and care of cancer patients reflect the ongoing medical practice, and their analysis yields essential real-world evidence. Advanced information technologies are vital for their collection, qualification, and reuse in research projects. METHODS: UNICANCER, the French federation of comprehensive cancer centres, has innovated a unique research network: Consore. This potent federated tool enables the analysis of data from millions of cancer patients across eleven French hospitals. RESULTS: Currently operational within eleven French cancer centres, Consore employs natural language processing to structure the therapeutic management data of approximately 1.3 million cancer patients. These data originate from their electronic medical records, encompassing about 65 million medical records. Thanks to the structured data, which are harmonized within a common data model, and its federated search tool, Consore can create patient cohorts based on patient or tumor characteristics, and treatment modalities. This ability to derive larger cohorts is particularly attractive when studying rare cancers. CONCLUSIONS: Consore serves as a tremendous data mining instrument that propels French cancer centres into the big data era. With its federated technical architecture and unique shared data model, Consore facilitates compliance with regulations and acceleration of cancer research projects.
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Investigación Biomédica , Neoplasias , Humanos , Minería de Datos , Registros Electrónicos de Salud , Neoplasias/terapia , LenguajeRESUMEN
BACKGROUND: Adolescents (15-19 years) with sarcoma are known to have significantly worse survival than children (0-14 years). One possible reason may be that the adolescent sarcomas exhibit specific biological characteristics resulting in differences in clinical presentation and treatment resistance behaviors. The BIOSCA project aims to further explore these age-related differences in survival accounting for molecular tumor characteristic in children and adolescents with sarcoma. METHODS: A retrospective national population-based observational study with documented somatic genetic analyses was conducted between 2011 and 2016 of all patients aged from 0 to 17 years with a diagnosis of sarcoma using the National Registry of Childhood Cancers Database. RESULTS: A total of 1637 children (0-9years: 40%), preadolescents (10-14years: 35%) and adolescents (15-17 years: 25%) with a diagnosis of bone (N = 845) or soft-tissue (N = 792) sarcoma were included. Adolescents had significantly worse outcome for undifferentiated small round cell sarcoma (USRCS), alveolar rhabdomyosarcoma (ARMS), and epithelioid sarcoma. Five-year overall survivals were worse among CIC-rearranged USRCS cases (47% [95%CI:21-69]) as compared to other USRCS, and PAX3::FOXO1 ARMS patients (44% [95%CI:32-55]) as compared to other ARMS. Adjusting for stage and genomic-profiling status, adolescents with USRCS were 1.6-fold more likely to die than children (P = 0.05), while the difference in survival between age of ARMS patients was weaken. Indeed, the prevalence of PAX3::FOXO1 increased significantly with age. CONCLUSION: Age was an independent prognostic factor of outcome only in patients with USRCS, while the association between age and survival of patients with ARMS could be partly explained by differences in prevalence of PAX3::FOXO1.
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BACKGROUND: Alveolar rhabdomyosarcoma (ARMS) is an aggressive pediatric cancer and cases with fusion PAX3-FOXO1 and PAX7-FOXO1 seem to have a poor prognosis. The aim is to evaluate whether PAX-FOXO1 alterations influence clinical outcome in childhood and adolescence population with ARMS. PROCEDURE: A population-based study was conducted between 2011 and 2016 in patients less than 17 years with a diagnosis of ARMS. Overall survival (OS) depending on fusion status with clinical factors was analyzed. RESULTS: Out of 111 ARMS patients recorded in the French National Childhood Cancer Registry during the 2011-2016 period, 61% expressed PAX3-FOXO1, 15% expressed PAX7-FOXO1, 13% were FOXO1 fusion-positive without PAX specification, and 7% were PAX-FOXO1 negative (n = 4 missing data). Compared to patients with PAX7-FOXO1 positive ARMS, those with PAX3-FOXO1 positive tumor were significantly older (10-17 years: 57.4% vs. 29.4%), and had more often a metastatic disease (54.4% vs. 23.5%). Poorer 5-year OS for patients with PAX3-FOXO1 and PAX not specified FOXO1-positive tumor were observed (44.0% [32.0-55.4] and 35.7% [13.1-59.4], respectively). After adjustment for stage at diagnosis, patients with positive tumor for PAX3-FOXO1 were 3.6-fold more likely to die than those with positive tumor for PAX7-FOXO1. CONCLUSION: At the population level, PAX3-FOXO1 was associated with a significant higher risk of death compared to PAX7-FOXO1-positive and PAX-FOXO1-negative tumors, and could explain poorer 5-year OS observed in adolescence population diagnosed with ARMS. A continuous risk score derived from the combination of clinical parameters with PAX3-FOXO1 fusion status represents a robust approach to improving current risk-adapted therapy for ARMS.
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Rabdomiosarcoma Alveolar , Rabdomiosarcoma , Niño , Humanos , Adolescente , Rabdomiosarcoma Alveolar/patología , Factores de Transcripción Paired Box , Factor de Transcripción PAX7 , Factor de Transcripción PAX3 , Factores de Transcripción Forkhead , Proteína Forkhead Box O1 , Proteínas de Fusión OncogénicaRESUMEN
Few studies have investigated the seasonal patterns of embryonal tumours. Based on data from the French National Registry of Childhood Cancers, the present study aimed to investigate seasonal variations in embryonal tumour incidence rates by month of birth and by month of diagnosis. The study included 6635 primary embryonal tumour cases diagnosed before the age of 15 years over the period 2000-2015 in mainland France. Assuming monthly variations in incidence rates were homogeneous over 2000-2015, we used a Poisson regression model to test for overall heterogeneity in standardised incidence ratios (SIRs) by month of birth or diagnosis. The seasonal scan statistic method was used to detect monthly excesses or deficits of embryonal tumour cases over the whole study period. The annual reproducibility of the observed monthly variations was formally tested. An overall heterogeneity in incidence rates by month of birth was observed for rhabdomyosarcoma in boys only. Based on the month of diagnosis, a seasonality was evidenced for unilateral retinoblastoma, with a lower incidence rate in the summer (SIRJul-Aug = 0.68, 95% CI = 0.52-0.87), whilst the incidence rate of rhabdomyosarcoma tended to be lower in August (SIRAug = 0.68, 95% CI = 0.52-0.89). No seasonality was detected for the other embryonal tumour groups by month of birth or month of diagnosis. This study is one of the largest to have investigated the seasonality of childhood embryonal tumours. The study showed a seasonal variation in the incidence rates by month of diagnosis for unilateral retinoblastoma and rhabdomyosarcoma. Our findings are likely to reflect a delay in consultation during the summer months. However, the role of seasonally varying environmental exposures cannot be ruled out.
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Neoplasias de la Retina , Retinoblastoma , Rabdomiosarcoma , Masculino , Humanos , Adolescente , Reproducibilidad de los Resultados , Incidencia , Francia/epidemiologíaRESUMEN
BACKGROUND: The initial management of patients with sarcoma is a critical issue. We used the nationwide French National Cancer Institute-funded prospective sarcoma database NETSARC to report the management and oncologic outcomes in adolescents and young adults (AYAs) patients with sarcoma at the national level. PATIENTS AND METHODS: NETSARC database gathers regularly monitored and updated data from patients with sarcoma. NETSARC was queried for patients (15-30 years) with sarcoma diagnosed from 2010 to 2017 for whom tumor resection had been performed. We reported management, locoregional recurrence-free survival (LRFS), progression-free survival (PFS), and overall survival (OS) in AYA treated in French reference sarcoma centers (RSC) and outside RSC (non-RSC) and conducted multivariable survival analyses adjusted for classical prognostic factors. RESULTS: Among 3,227 patients aged 15-30 years with sarcoma diagnosed between 2010 and 2017, the study included 2,227 patients with surgery data available, among whom 1,290 AYAs had been operated in RSC, and 937 AYAs in non-RSC. Significant differences in compliance to guidelines were observed including pre-treatment biopsy (RSC: 85.9%; non-RSC 48.1%), pre-treatment imaging (RSC: 86.8%; non-RSC: 56.5%) and R0 margins (RSC 57.6%; non-RSC: 20.2%) (p < 0.001). 3y-OS rates were 81.1% (95%CI 78.3-83.6) in AYA in RSC and 82.7% (95%CI 79.4-85.5) in AYA in non-RSC, respectively. Whereas no significant differences in OS was observed in AYAs treated in RSC and in non-RSC, LRFS and PFS were improved in AYAs treated in RSC compared to AYAs treated in non-RSC (Hazard Ratios (HR): 0.58 and 0.83, respectively). CONCLUSIONS: 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.
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Sarcoma , Neoplasias de los Tejidos Blandos , Humanos , Adolescente , Adulto Joven , Niño , Estudios Prospectivos , Sarcoma/diagnóstico , Sarcoma/cirugía , Neoplasias de los Tejidos Blandos/cirugía , Bases de Datos Factuales , Supervivencia sin ProgresiónRESUMEN
BACKGROUND: Large-scale trials have shown that hypofractionated adjuvant breast radiotherapy was as effective in terms of survival and local control as conventional fractionated radiotherapy, and acute toxicity was reduced with hypofractionated radiotherapy. However, there is a lack of data about the toxicity of breast with regional nodal irradiation (RNI). The aim of this study was to assess the effect of fractionation on radiation-related acute skin toxicity in patients receiving RNI in addition to whole-breast or chest wall irradiation, using real-life data. METHODS: We conducted a prospective, multicenter cohort study with systematic computerized data collection integrated into Mosaiq®. Three comprehensive cancer centers used a standardized form to prospectively collect patient characteristics, treatment characteristics and toxicity. RESULTS: Between November 2016 and January 2022, 1727 patients were assessed; 1419 (82.2%) and 308 (17.8%) patients respectively received conventional fractionated and hypofractionated radiation therapy. Overall, the incidence of acute grade 2 or higher dermatitis was 28.4% (490 patients). Incidence was lower with hypofractionated than with conventional fractioned radiation therapy (odds ratio (OR) 0.34 [0.29;0.41]). Two prognostic factors were found to increase the risk of acute dermatitis, namely 3D (vs IMRT) and breast irradiation (vs chest wall). CONCLUSION: Using real-life data from unselected patients with regional nodal irradiation, our findings confirm the decreased risk of dermatitis previously reported with hypofractionated radiation therapy in clinical trials. Expansion of systematic data collection systems to include additional centers as well as dosimetric data is warranted to further evaluate the short- and long-term effects of fractionation in real life.
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Neoplasias de la Mama , Dermatitis , Traumatismos por Radiación , Radioterapia de Intensidad Modulada , Humanos , Femenino , Neoplasias de la Mama/complicaciones , Estudios Prospectivos , Estudios de Cohortes , Hipofraccionamiento de la Dosis de Radiación , Traumatismos por Radiación/epidemiología , Traumatismos por Radiación/etiología , Dermatitis/complicaciones , Radioterapia Adyuvante/efectos adversos , Radioterapia de Intensidad Modulada/efectos adversosRESUMEN
INTRODUCTION: Thyroid cancer is the first cause of endocrine malignancy among children. Over the past decades, an increase in the incidence rates (IR) has been observed around the world. Our study aimed to describe epidemiology, therapeutic management and survival rates of children and adolescents with thyroid cancer in France. METHODS: A population-based study was conducted between 2000 and 2018 in children and adolescents less than 17 years with a diagnostic of thyroid cancer. RESULTS: A total of 774 thyroid cancers were included: 579 papillary (PTC), 83 follicular (FTC), and 111 medullary carcinomas (MTC). PTC are more frequent in females and in adolescents whereas MTC mainly concerned children, mostly with a familial predisposition. Almost all patients underwent thyroidectomy, completed for most patients with PTC and FTC by radioiodine therapy. Cervical dissection was performed more frequently in patients having PTC and MTC compared to those with FTC. Between 2000 and 2018, thyroid cancers IR in children fluctuated between 1.3 and 3.2 per million, without any significant trend. The median follow-up time was 11.3 years in children, and 5.7 years in adolescents. The 5year-OS was greater than 98.5%. CONCLUSIONS: Population-based studies are crucial for better understanding and delineation of best management of rare diseases as thyroid cancers in pediatric and adolescent population. Considering the very favorable survival, a stratification should be proposed between cases at low risk and cases at high risk of relapse, in order to consider a strategy of therapeutic de-escalation in the most favorable cases.
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Adenocarcinoma Folicular , Neoplasias de la Tiroides , Adenocarcinoma Folicular/epidemiología , Adenocarcinoma Folicular/terapia , Adolescente , Niño , Femenino , Humanos , Incidencia , Radioisótopos de Yodo , Recurrencia Local de Neoplasia , Neoplasias de la Tiroides/diagnóstico , Neoplasias de la Tiroides/epidemiología , Neoplasias de la Tiroides/terapiaRESUMEN
BACKGROUND: Sarcomas are rare, heterogeneous, ubiquitously localized malignancies with many histologic subtypes and genomic patterns. The survival of patients with sarcoma has rarely been described based on this heterogeneity; therefore, the authors' objective was to estimate survival outcomes in patients who had sarcomas using the 2020 version of the World Health Organization classification of soft tissue and bone tumors. METHODS: Patients older than 15 years who had incident sarcoma diagnosed between 2005 and 2010 were extracted from 14 French population-based cancer registries covering 18% of the French metropolitan population. Vital status for each patient was actively followed up to June 30, 2013. Net survival (NS) was estimated using the unbiased Pohar-Perme method. RESULTS: Overall, 4202 patients were included. NS declined with increasing age at diagnosis. According to topographic groups, large 5-year NS disparities were observed, ranging from 47% among women with gynecologic sarcomas to 89% among patients with skin sarcomas. Patients with soft tissue, bone, and gastrointestinal sarcomas had 5-year NS rates of 53%, 61%, and 70%, respectively. Similar heterogeneity was observed according to histologic subtypes, with 5-year NS ranging from 19% for patients with angiosarcomas to 96% for patients with dermatofibrosarcomas. Patients with sarcoma who displayed missense mutations had a better 5-year NS (74%); those with MDM2-amplified sarcomas had the worst NS (45%). CONCLUSIONS: NS rates in patients with sarcoma are presented here for the first time based on the 2020 World Health Organization classification applied to population-based registry data. Large prognostic heterogeneity was observed based on age, topographic and histologic groups, and genomic alteration profiles, constituting a benchmark for future studies and clinical trials.
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Neoplasias Óseas , Sarcoma , Neoplasias de los Tejidos Blandos , Neoplasias Óseas/epidemiología , Femenino , Humanos , Sistema de Registros , Sarcoma/diagnóstico , Sarcoma/epidemiología , Sarcoma/genética , Neoplasias de los Tejidos Blandos/patología , Tasa de SupervivenciaRESUMEN
INTRODUCTION: Childhood head and neck cancers (HNC) are rare and represent a complex group of anatomical topographies. The aim of this study is to describe the distribution, the incidence and survival rates of children with malignant HNC in France. METHODS: A population-based study was conducted between 2000 and 2015 in children less than 15 years with a diagnosis of HNC using the French National Registry of Childhood Cancers database (RNCE). Age-standardized incidence rates (ASR) and survival analysis were performed. RESULTS: The 1623 included HNC represented 5.6% of all cancers included in the RNCE. The thyroid was the leading tumor site category (26.6%), followed by head and neck soft tissue location (15.4%) and the nasopharynx (10.8%). The most common cancers were thyroid gland carcinomas (26.1%), rhabdomyosarcomas (23.9%) and Burkitt Lymphomas (8.6%). Nasopharynx cancers and soft-tissue sarcomas were statistically more frequent in boys, while thyroid carcinomas were significantly more frequent in girls. The annual ASR was 8.6 new cancer cases per million children. For all HNC combined, the 5-year overall survival (OS) was 87.7% [95%CI: 85.9-89.2]. There was no statistically significant variation in 5-year OS between 2000-2007 and 2008-2015. CONCLUSIONS: Epidemiological data on HNC distribution, incidence and survival contributes to better understand these tumors by quantifying their impact on the French population and assessing their burden. Regarding the exclusion of topographies and some histological origins performed by some authors, this report proposes new recommendations to study HNC in a pediatric population.
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Neoplasias de Cabeza y Cuello , Niño , Femenino , Francia/epidemiología , Neoplasias de Cabeza y Cuello/epidemiología , Humanos , Incidencia , Masculino , Sistema de Registros , Tasa de SupervivenciaRESUMEN
BACKGROUND: Childhood cancer survival currently exceeds 80 % five years after diagnosis in high-income countries. In this study, we aimed to describe long-term trends and to investigate socioeconomic and spatial disparities in childhood cancer survival. METHODS: The study included 28,073 cases recorded in the French National Registry of Childhood Cancers from 2000 to 2015. Contextual census data (deprivation indices, population density, spatial accessibility to general practitioners) were allocated to each case based on the residence at diagnosis. Overall survival (OS) and conditional 10-year OS for 5-year survivors were estimated for all cancers combined and by diagnostic group and subgroup. Comparisons were conducted by sex, age at diagnosis, period of diagnosis, and contextual indicators. Hazard ratios for death were estimated using Cox models. RESULTS: All cancers combined, the OS reached 82.8 % [95 % CI: 82.4-83.3] at 5 years and 80.8 % [95 % CI: 80.3-81.3] at 10 years. Conditional 10-year OS of 5-year survivors reached 97.5 % [95 % CI: 97.3-97.7] and was higher than 95 % for all subgroups except osteosarcomas and most subgroups of the central nervous system. In addition to disparities by sex, age at diagnosis, and period of diagnosis, we observed a slight decrease in survival for cases living in the most deprived areas at diagnosis, not consistent across diagnostic groups. CONCLUSION: Our results confirm the high 5-year survival for childhood cancer and show an excellent 10-year conditional survival of 5-year survivors. Additional individual data are needed to clarify the factors underlying the slight decrease in childhood cancer survival observed in the most deprived areas.
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Supervivientes de Cáncer , Neoplasias , Supervivientes de Cáncer/estadística & datos numéricos , Niño , Estudios de Cohortes , Femenino , Francia/epidemiología , Humanos , Masculino , Neoplasias/mortalidad , Sistema de Registros , Factores Socioeconómicos , Tasa de SupervivenciaRESUMEN
Purpose: This study was undertaken to determine cancer survival and describe the spectrum of cancers diagnosed among French adolescent and young adult (AYA) population. Methods: All cases of cancer diagnosed in 15-24 years, recorded by all French population-based registries (18% of the French population), over the 2000-2016 period, were included. Age-standardized incidence rates, conventional annual percentage change (cAPC) of incidence over time, and 5-year overall survival (5yOS) were calculated. Results: We analyzed 2734 cancer diagnoses in adolescents and 4199 in young adults. Overall incidence rates were 231.9/106 in 15-19 year olds and 354.0/106 in 20-24 year olds. The most frequently diagnosed cancers in male AYA were malignant gonadal germ-cell tumors (GCT), Hodgkin lymphoma (HL), and malignant melanoma and were HL, thyroid carcinoma, and malignant melanoma in females. Cancer incidence was stable over time with a cAPC of 0.8% (p = 0.72). For all cancers combined, 5yOS was 86.6% (95% CI: 85.8-87.4), >85% for HL, non-Hodgkin lymphomas (NHL), GCT, thyroid carcinomas, and malignant melanomas, and around 60% and lower for osteosarcomas, Ewing tumors, hepatic carcinomas, and rhabdomyosarcomas. The 5yOS has significantly improved from 2000-2007 to 2008-2015 for all cancers pooled, with a substantial gain of 4% for 15-19 year olds and 3% for 20-24 year olds. Conclusion: Notwithstanding the encouraging results for some cancers, and overall, persistent poorer survivals in AYA were shown compared to children for acute lymphoblastic leukemia, osteosarcoma, Ewing tumor, rhabdomyosarcoma, and malignant hepatic tumors. These disparities require further investigation to identify and address the causes of these inferior outcomes.
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Neoplasias , Adolescente , Femenino , Francia/epidemiología , Humanos , Incidencia , Linfoma/epidemiología , Masculino , Melanoma , Neoplasias/epidemiología , Neoplasias de Células Germinales y Embrionarias/epidemiología , Sistema de Registros , Tasa de Supervivencia , Adulto JovenAsunto(s)
Neoplasias , Niño , Humanos , National Cancer Institute (U.S.) , Neoplasias/epidemiología , Estados UnidosRESUMEN
BACKGROUND: The exhaustive collection of new sarcoma cases and their second histologic review offer a unique opportunity to study their incidence and time trends in France according to the major subtypes. METHODS: Data were collected from population-based cancer registries covering 22% of the French population. Crude and world age-standardized incidence rates (ASR) were estimated according to anatomic, histological and genetic groups, age and sex over the 2010-2013 period. RESULTS: Time trends in incidence were calculated by the annual percent change over the 2000-2013 period. During the most recent period (2010-2013), 3942 patients with sarcoma were included. The ASR of soft-tissue and bone sarcomas, and gastro-intestinal stromal tumors (GIST) were 2.1, 1.0 and 0.6, respectively. For the four most frequent histological subtypes (unclassified, leiomyosarcoma, GIST and liposarcoma), the ASR ranged from 0.4 to 0.7. ASRs were 1.9 for complex genomic and 1.3 for recurrent translocation sarcomas. The time-trend analysis showed a significant increase of sarcoma incidence rate between 2000 and 2005, which stabilized thereafter. Incidence rates increased for four histological subtypes (GIST, chondrosarcoma, myxofibrosarcoma, solitary fibrous tumors) and decreased for three (leiomyosarcomas, Kaposi sarcoma and fibrosarcoma). CONCLUSION: To our knowledge, this study is the first to investigate sarcoma incidence based on a systematic pathological review of these cancers and on the updated sarcoma classifications. Due to the paucity of literature on sarcomas, future studies using data from population-based cancer registries should consider a standardized inclusion criterion presented in our study to better describe and compare data between countries.
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Neoplasias Óseas/epidemiología , Tumores del Estroma Gastrointestinal/epidemiología , Recurrencia Local de Neoplasia/epidemiología , Sistema de Registros , Sarcoma/epidemiología , Neoplasias de los Tejidos Blandos/epidemiología , Tumores Fibrosos Solitarios/epidemiología , Adolescente , Adulto , Anciano , Niño , Preescolar , Femenino , Francia/epidemiología , Humanos , Incidencia , Lactante , Recién Nacido , Masculino , Persona de Mediana Edad , Adulto JovenRESUMEN
BACKGROUND: On average 185 children are diagnosed each year in France with a cancer in their first year of life, representing 11 % of cancers diagnosed in children less than 15 years. METHODS: A retrospective population-based observational study was conducted between 2000 and 2014 of all infants with a diagnosis of cancer using the National Registry of Childhood Cancers Database. RESULTS: Out of 2760 cases of primary cancers in infancy, there were mainly neuroblastomas 30.1 %), central nervous system (CNS) tumors (16.1 %), leukemias (15.3 %), retinoblastomas (11.6 %), and Wilms tumors (6.9 %). Embryonal malignancies accounted for 55.2 % of cases. Most diagnoses showed a male excess, particularly for malignant gonadal germ-cell tumors (GCT) with a 17.5 sex-ratio. The annual incidence rate, 242.9 per million infants overall, was stable over the study period for all types of cancer. Most deaths occurred within the first month of life (70.8 % of deaths). The 5-year overall survival (5-y OS) was 81.0 % (95 %CI, 79.4-82.4) with large contrasts between diagnoses. The best 5-y OS (>85 %) were observed for retinoblastomas, carcinomas, nephroblastomas, GCT, neuroblastomas, and hepatoblastomas. Conversely, the lowest 5-y OS (<65 %) were observed for acute myeloid leukemias, CNS tumors, and lymphoid leukemias. We observed no substantial change over time (80.5 % [95 %CI, 77.7-82.9] in 2000-2004 and 82.6 % [95 %CI, 80.0-84.9] in 2010-2014) for all cancers combined. The same result has been found whatever the diagnostic group. CONCLUSION: Our results contribute to better understand these tumors by quantifying their impact on the French population and assessing the burden of some devastating infant cancers.
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Neoplasias/epidemiología , Femenino , Francia/epidemiología , Humanos , Incidencia , Lactante , Recién Nacido , Masculino , Neoplasias/mortalidad , Estudios Retrospectivos , Análisis de SupervivenciaRESUMEN
Importance: Many women dread undergoing mammography, and some may not attend or reattend breast cancer screening because of the discomfort or pain induced by breast compression. Objective: To evaluate the noninferiority of the self-compression mammography technique for reducing breast thickness compared with standard compression. Design, Setting, and Participants: This prospective, parallel-group, noninferiority randomized clinical trial was conducted from May 7, 2013, to October 26, 2015, at 6 cancer care centers in France. Participants were women aged 50 to 75 years, without a history of recent breast surgical procedure or treatment, and who could perform self-compression. Analyses were performed on intention-to-treat basis from January 27, 2017, to March 30, 2018. Interventions: Patients were randomized 1:1 to the self-compression group or the standard compression group. Main Outcomes and Measures: Primary end point was breast thickness expressed as the mean of 4 views: right and left craniocaudal and right and left mediolateral oblique. The predefined noninferiority margin was a difference of 3 mm, with a 1-sided 95% CI. Secondary end points included compression force, image quality, requirement for additional views, pain, and patient satisfaction and radiographer assessment questionnaires. Results: Among the 549 women randomized, 548 (97.3%) completed the trial. Of these, 275 (48.8%) (mean [SD] age, 61.35 [6.34] years) were randomized to the self-compression arm and 273 (48.5%) (mean [SD] age, 60.84 [6.41] years) to the standard compression arm. The difference in the mean thickness between the 2 arms was lower than the noninferiority margin, with an upper 1-sided 95% CI less than 3 mm (-0.17; 95% CI,-∞ to 1.89 mm; P < .05). Compression force was higher in the self-compression group compared with the standard compression arm for the 4 mammographic views. Pain was statistically significantly lower in the self-compression group (n = 274) compared with the standard compression group (n = 269) (median [interquartile range (IQR)] score, 2 [1-5] vs 3 [1-5]; P = .009). No difference was reported in the image quality scores of the 2 groups or in the number of additional views performed (median [IQR] extra views, 2 [2-2] vs 2 [2-3] extra views; P = .64), whatever the indication, including insufficient image quality (29 [16.8%] vs 27 [15.0%] insufficient quality views; P = .65). No adverse effects or pain were reported by the participants after the self-compression mammography. Conclusions and Relevance: Self-compression does not appear to be inferior to standard compression mammography in achieving minimal breast thickness without increasing pain or compromising image quality; this technique may be an effective option for women who want to be involved in their breast examination. Trial Registration: ClinicalTrials.gov identifier: NCT02866591.
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Neoplasias de la Mama/diagnóstico por imagen , Mamografía/métodos , Autocuidado , Anciano , Femenino , Francia , Humanos , Persona de Mediana Edad , PresiónRESUMEN
INTRODUCTION: Breast cancer of young women has medico-psychological issues, particularly in the perspective of "after cancer" period. The objective of the study was to analyze the changes in management, in a decade including the publication of Cancer Plans I and II. METHODS: This retrospective unicentric study involved two populations of women aged 40 and treated at the "Institut de cancérologie de Lorraine" in 2002-2004 (P1) and 2012-2014 (P2). Epidemiological-demographic, histological, diagnostic and therapeutic sequences were analyzed. RESULTS: 216 patients were included. The mean age was 35.7 years (range 22-40), with no significant epidemiological difference, except for an increased rate of obesity in P2. Preoperative biopsies became almost systematic and the pre-therapeutic care plan rate increased over the decade from 3.8% to 8% (p < 0.001). The rate of breast-conserving treatment remained stable. The sentinel lymph node rate increased from 4.4% in P1 to 31.9% in P2 (p < 0.001), while the rate of lymph node dissection decreased (p < 0.001) between the two periods. Resort to breast nurses, psycho-oncologists, fertility experts and oncogenetic consultations has also increased. CONCLUSION: There is an improvement in the care and management of young women under 40 because of the first Cancer Plans. A study about the impact on the prognosis and a reflection to improve the experience of illness for these women are to lead.
Asunto(s)
Neoplasias de la Mama/terapia , Adulto , Factores de Edad , Biopsia/tendencias , Mama/patología , Neoplasias de la Mama/diagnóstico , Neoplasias de la Mama/epidemiología , Neoplasias de la Mama/patología , Instituciones Oncológicas , Estudios de Cohortes , Femenino , Preservación de la Fertilidad/estadística & datos numéricos , Francia/epidemiología , Pruebas Genéticas/estadística & datos numéricos , Pruebas Genéticas/tendencias , Humanos , Mastectomía Segmentaria/estadística & datos numéricos , Mastectomía Segmentaria/tendencias , Obesidad/epidemiología , Embarazo , Pronóstico , Estudios Retrospectivos , Biopsia del Ganglio Linfático Centinela/estadística & datos numéricos , Biopsia del Ganglio Linfático Centinela/tendencias , Factores de Tiempo , Adulto JovenRESUMEN
BACKGROUND: In order to evaluate at the population level the impact of the actions developed in France since 2004 to organize the care of adolescents and young adults (AYAs) with cancer, we conducted the present study to provide an unbiased view of the pathway of care of these patients. METHODS: Using a population-based registry, we conducted a review of all cases of cancer diagnosed during 2012 and 2013 in 15- to 24-year-old patients living in nineteen French administrative areas. RESULTS: The median times for diagnosis and treatment of the 993 included AYAs were 9 weeks (3-22) and 1 day (0-20), respectively. Delays in diagnosis were significantly longer in young adults than in adolescents, especially for soft-tissue sarcomas (48.7 weeks vs. 15.4 weeks, P = 0.04) and bone tumors (21.4 weeks vs. 10.1 weeks, P = 0.04). The first physicians seen by patients were mostly general practitioners (67.4%). Most patients (77.5%) were treated in adult units. Management decisions were taken within the context of a multidisciplinary team (MDT) in 85.3% of cases. MDT meetings that involved both pediatric and adult oncologists were uncommon (15.7% of patients). Twenty-six percent of patients were included in randomized or nonrandomized clinical studies. The proportion of inclusion was significantly higher in adolescents (39.5%) than in young adults (16.8%). CONCLUSION: In France, pathways of care for AYAs are heterogeneous. It is necessary to organize a national network of expert centers with adequate medical skills and specific psychosocial support and facilities to provide the best possible care for these patients.