Prise en charge des tumeurs des plexus choroïdes chez l enfant (étude de la cohorte française)

Introduction : Les tumeurs des plexus choroïdes (TPC) sont rares et surviennent chez le jeune enfant. On distingue trois sous type histologiques : les papillomes des plexus choroïdes (PPC), les papillomes atypiques (APP) et les carcinomes (CPC). L histologie est un facteur pronostic majeur et conditionne la prise en charge thérapeutique. L objectif de cette étude est de décrire la cohorte des enfants porteurs d une TPC et d identifier des stratégies thérapeutiques. Matériel et Méthodes : Nous avons étudié la prise en charge de 119 enfants porteurs d une TPC traité entre janvier 2000 et décembre 2012 dans les centres français de la SFCE.Résultats: 68 patients ont été pris en charge pour un PPC, 24 pour un APP (23 localisés et 1 métastatique) et 27 pour un CPC (20 localisés et 7 métastatiques). L âge médian au diagnostic était de 1.83ans et le sex ratio de 1.64.Tous les patients ont eu une chirurgie d exérèse. 1 CPC a eu un traitement néoadjuvant par chimiothérapie et 32 patients (1 PPC, 7 APP et 24 CPC) ont eu une chimiothérapie adjuvante. 2 APP et 10 CPC ont été traité par radiothérapie. La survie globale à 5 ans était de 100% pour les PPC, 95.7%pour les APP, 44.4% pour les CPC localisé et 32.1% pour les CPC métastatiques. Conclusion : Le sous type histologique prédit le comportement clinique des TPC. Il est indispensable d avoir un diagnostic de certitude. La résection chirurgicale complète est le traitement de choix pour les trois sous types. Un traitement adjuvant par chimiothérapie et/ou radiothérapie reste à définir pour les APP avec résidu et les CPC.Background: Choroid Plexus Tumor (CPT) are rare pediatric tumor which can be divided into three distinct histologic subtypes: choroid plexus papilloma (CPP), atypical (APP) and carcinoma (CPC). Histology is an important prognostic factor and determine therapeutic modalities. The aim of this study is to review and analyse the treatment of these tumors. Methods: We reviewed the therapeutic managment of 119 cases of pediatric CPT treated in French Center Cancer between 2000 and 2012. Results: 68 patients had CPP, 24 APP (23 localized and 1 metastatic) and 27 CPC (20 localized and 7 metastatics). The median age at the diagnosis was 1.83 years and the sex ratio was 1.64. All patients were treated with surgery. 1 CPC had neoadjuvant chemotherapy and 32 patients ( 1 CPP, 7APP et 24 CPC) had chemotherapy. 2 APP and 10 CPC were treated with radiotherapy. The 5 years overall survival rate was 100% for CPP, 95.7% for APP, 44.4% for localized CPC and 32.1% for metastatic CPC. Conclusion: The histologic grade predict the clinical behavior of pediatric CPT. It is essential to have a definitive diagnosis. Complete resection is the treatment of choice regardless of tumor subtype. Adjuvant therapy with chemotherapy and/or radiotherapy remains to be defined for APP with incomplete resection and CPCABYMES-CHRUPPA-BU (971202102) / SudocSudocFranceF

Gliomes de bas grade de la fosse postérieure chez l'enfant (expérience toulousaine à propos de 46 cas)

TOULOUSE3-BU Santé-Centrale (315552105) / SudocSudocFranceF

Phospholipase A2 et neutropénie fébrile en hémato-oncologie pédiatrique (à propos d'une étude rétrospective unicentrique portant sur 158 cas)

TOULOUSE3-BU Santé-Allées (315552109) / SudocTOULOUSE3-BU Santé-Centrale (315552105) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Maternal and perinatal characteristics, congenital malformations and the risk of wilms tumor: the ESTELLE study

Purpose: Wilms tumor (WT), or nephroblastoma, is an embryonic tumor that constitutes the most common renal tumor in children. Little is known about the etiology of WT. The aim of this study was to investigate whether maternal or perinatal characteristics were associated with the risk of WT. Methods: The ESTELLE study is a national-based case–control study that included 117 cases of WT and 1,100 controls younger than 11 years old. The cases were children diagnosed in France in 2010–2011 and the controls were frequency matched with cases by age and gender. The mothers of case and control children responded to a telephone questionnaire addressing sociodemographic and perinatal characteristics, childhood environment, and lifestyle. Unconditional logistic regression models adjusted on potential cofounders were used to estimate the odds ratios (OR) and their confidence intervals (95% CI). Results: High birth weight and the presence of congenital malformation were associated with WT (OR 1.9 [95% CI 1.0–3.7] and OR 2.5 [95% CI 1.1–5.8], respectively). No association with breastfeeding or folic acid supplementation was observed. Conclusions: Although potential recall bias cannot be excluded, our findings reinforce the hypothesis that high birth weight and the presence of congenital malformation may be associated with an increased risk of WT. Further investigations are needed to further elucidate the possible role of maternal characteristics in the etiology of WT

Pharmacokinetics of oral vinorelbine in French children with recurrent or progressive primary low‐grade glioma

International audienc

ETMR-like infantile cerebellar embryonal tumors in the extended morphologic spectrum of DICER1-related tumors

International audienc

Birth characteristics and childhood malignant central nervous sytem tumors: the ESCALE study (French Society for Childhood Cancer).

International audienceBACKGROUND: Determining the role of pre- and perinatal factors in the aetiology of childhood malignant central nervous (CNS) tumors, using data from the French national case-control study, ESCALE. METHODS: ESCALE included all children in France less than 15 years old with a diagnosis of acute leukaemia, lymphoma, malignant CNS tumor, or neuroblastoma (2003-2004). In all, 209 malignant CNS tumor cases (80% of the eligible cases) and 1681 population-based controls (71%) were included using quotas ensuring frequency matching with the cases by age and gender. Case and control mothers were interviewed using a standardised telephone interview, which elicited birth characteristics, congenital malformation, maternal reproductive history, and use of assisted reproductive technologies for the index child. RESULTS: The cases and controls did not differ in terms of gestational age at birth, birth weight, birth order, breastfeeding, or parental age at birth. There was no association between assisted reproduction for the index child and malignant CNS tumor (OR=1.1 [0.6-2.2]). A positive association between a maternal history of one miscarriage and malignant CNS tumor was observed (OR=1.4 [1.0-2.0], p<0.05), especially for glial cell tumors (other glioma: OR=2.0 [1.1-3.6]). CONCLUSION: The results suggest a possible association between a maternal history of one miscarriage and the risk of malignant CNS tumor

Mortality in Children with Optic Pathway Glioma Treated with Up-Front BB-SFOP Chemotherapy.

In terms of overall survival (OS), limited data are available for the very long-term outcomes of children treated for optic pathway glioma (OPG) with up-front chemotherapy. Therefore, we undertook this study with the aim of clarifying long-term OS and causes of death in these patients.We initiated and analyzed a historical cohort study of 180 children with OPG treated in France with BB-SFOP chemotherapy between 1990 and 2004. The survival distributions were estimated using Kaplan-Meier method. The effect of potential risk factors on the risk of death was described using Cox regression analysis.The OS was 95% [95% CI: 90.6-97.3] 5 years after diagnosis and significantly decreased over time without ever stabilizing: 91.6% at 10 years [95% CI: 86.5-94.8], 80.7% at 15 years [95% CI: 72.7-86.8] and 75.5% [95% CI: 65.6-83] at 18 years. Tumor progression was the most common cause of death (65%). Age and intracranial hypertension at diagnosis were significantly associated with a worse prognosis. Risk of death was increased by 3.1[95% CI: 1.5-6.2] (p=0.002) for patients less than 1 year old at diagnosis and by 5.2[95% CI: 1.5-17.6] (p=0.007) for patients with initial intracranial hypertension. Boys without diencephalic syndrome had a better prognosis (HR: 0.3 [95% CI: 0.1-0.8], p=0.007).This study shows that i) in children with OPG, OS is not as favorable as previously described and ii) patients can be classified into 2 groups depending on risk factors (age, intracranial hypertension, sex and diencephalic syndrome) with an OS rate of 50.4% at 18 years [95% CI: 31.4-66.6] in children with the worst prognosis. These findings could justify, depending on the initial risk, a different therapeutic approach to this tumor with more aggressive treatment (especially chemotherapy) in patients with high risk factors

Parental smoking, maternal alcohol, coffee and tea consumption and the risk of childhood brain tumours: the ESTELLE and ESCALE studies (SFCE, France)

International audiencePURPOSE: To investigate whether parental smoking around the time of pregnancy or maternal consumption of beverages (alcohol, coffee, or tea) during pregnancy were associated with the risk of CBT.METHODS: We pooled data from two French national population-based case-control studies with similar designs conducted in 2003-2004 and 2010-2011. The mothers of 510 CBT cases (directly recruited from the national childhood cancer register) and 3,102 controls aged under 15 years, frequency matched by age and gender, were interviewed through telephone, which included questions about prenatal parental smoking and maternal consumption of alcohol, coffee and tea. Odds ratios (OR) and 95% confidence intervals (CI) were estimated using unconditional logistic regression, adjusted for age, sex and study of origin.RESULTS: No association was seen between CBT and the mother smoking or drinking alcohol, coffee, or tea during the index pregnancy. The OR between CBT and paternal smoking in the year before birth (as reported by the mother) was 1.25 (95% CI 1.03, 1.52) with an OR of 1.09 (0.99, 1.19) for every 10 cigarettes per day (CPD) smoked. The association between paternal smoking and CBT appeared to be stronger in children diagnosed before the age of five years (OR 1.52, 95% CI 1.14, 2.02) and for astrocytoma (OR 1.86, 95% CI 1.26, 2.74).CONCLUSION: We found some evidence of a weak association between paternal smoking in the year before the child's birth and CBT, especially astrocytomas. These findings need to be replicated in other samples, using similar classifications of tumour subtypes

Factors related to pregnancy and birth and the risk of childhood brain tumours: The ESTELLE and ESCALE studies (SFCE, France)

International audienceLittle is known of the causes of childhood brain tumors (CBT). The aims of this study were to investigate whether extremes of birth weight were associated with increased risk of CBT and whether maternal preconceptional folic acid supplementation or breastfeeding reduced the risk. In addition, other maternal characteristics and birth related factors were also investigated. We pooled data from two French national population-based case-control studies with similar designs conducted in 2003–2004 and 2010–2011. The mothers of 510 CBT cases (directly recruited from the national childhood cancer register) and 3,102 controls aged under 15 years, frequency matched by age and gender did a telephone interview, which focussed on demographic and perinatal characteristics, and maternal life style habits and reproductive history. Odds ratios (OR) and 95% confidence intervals (CI) were estimated using unconditional logistic regression, adjusted for age, sex, study of origin and relevant confounders. No association was found between CBT and birth weight or fetal growth. The use of preconceptional folic acid supplementation was rare (5.3% in cases and 7.8% in controls) and the OR was 0.8 (95% CI 0.5, 1.4). There was no association with breastfeeding, even prolonged (six months or more; OR 1.0, 95% CI 0.8, 1.4). Neither was there any association between CBT and other investigated factors (maternal body mass index, gestational weight gain, congenital abnormality, maternal reproductive history or use of fertility treatments. Although large, this study was underpowered for subtype analyses. Pooling data with other population-based studies may provide further insight into findings by CBT subtypes