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Colorectal cancer (CRC) risk is increased in childhood cancer survivors (CCS), mainly after radiation therapy (RT). In France no specific CRC screening programs have been developed for CCS. In general population, CRC screening with fecal immunochemical testing (FIT) is recommended every 2 years between 50 and 74 years.

Le montage de perfusion, assemblage de multiples dispositifs médicaux stériles, permet l’administration de médicaments par voie intraveineuse. Les risques associés aux pratiques de perfusion sont divers : erreurs médicamenteuses, incompatibilités physico-chimiques (IPC) notamment. Un groupe de travail multidisciplinaire, d’un centre de lutte contre le cancer a diffusé un schéma type en 2013. De plus, nous souhaitons améliorer la prévention des IPC.

High-dose chemotherapy (HDC) was investigated in high-risk neuroblastoma (HR-NBL) to reduce the risk of relapse. We report the results of the 30-year experience of a cohort of patients with HR-NBL treated with high-dose (HD) busulfan (Bu)-containing regimens. From 1980 to 2009, 215 patients aged >1 year with stage 4 NBL were treated with HD Bu-containing regimens at Gustave Roussy. These data were prospectively recorded in the Pediatric Transplantation Database. The median age at diagnosis was 40 months (12–218 months). All patients had a stage 4 neuroblastoma. NMYC amplification was displayed in 24% of the tumors. The hematopoietic support consisted of bone marrow or PBSCs in 46% and 49% of patients, respectively. The 5-year event-free survival and overall survival rates of the whole cohort were 35.1% and 40%, respectively. Age at diagnosis, bone marrow involvement and tumor response after induction chemotherapy were significant prognostic factors. Toxicity was manageable and decreased over time, owing to both PBSC administration and better supportive care. Based on this experience, HD Bu–melphalan (Mel) has been implemented in Europe and compared with Carboplatin–Etoposide–Mel in the European SIOP Neuroblastoma (SIOPEN)/HR-NBL randomized protocol. It has now become the standard HDC in the SIOPEN HR strategy.

High-risk neuroblastoma is characterised by poor long-term survival, especially for very high-risk (VHR) patients (poor response of metastases after induction therapy). We report the results of an intensified high-dose chemotherapy (HDC) strategy to improve the prognosis of VHR patients. This strategy was based on tandem HDC with thiotepa and busulfan–melphalan (Bu–Mel) followed by autologous stem cell transplantation (ASCT). All data were prospectively recorded in the Gustave Roussy Paediatric ASCT database. From April 2004 to August 2011, 26 patients were eligible for tandem HDC. The median age at diagnosis was 4.4 years (1–15.9). All patients had metastatic disease. MYCN was amplified in 5/26 tumours. Despite the cumulative toxicity of alkylating agents, the toxicity of the intensified HDC strategy was manageable. Thiotepa-related toxicity was mainly digestive, whereas sinusoidal obstruction syndrome was the main toxicity observed after Bu–Mel. The 3-year event-free survival of this cohort was 37.3% (21.3–56.7). This strategy will be compared with combined 131I-mIBG/Bu–Mel in the upcoming SIOPEN VHR Neuroblastoma Protocol.

Les cardiomyopathies chroniques, induites par les anthracyclines, sont des complications tardives sévères. Certains facteurs de risque sont bien connus comme la dose cumulée d’anthracyclines, l’association à une irradiation de l’aire cardiaque ou le jeune âge au moment du traitement. Peu de moyens de prévention primaire sont actuellement validés chez l’enfant. Le dexrazoxane, un chélateur du fer, aurait un effet cardioprotecteur à court terme, mais son bénéfice à long terme, ainsi que les risques de seconde tumeur doivent être étudiés. La prise en charge actuelle repose sur une surveillance cardiologique à long terme de tout patient traité par anthracyclines de façon à détecter et traiter précocement les altérations de la fonction systolique myocardique afin de limiter leur risque de progression vers l’insuffisance cardiaque. Cette surveillance repose sur des échographies cardiaques régulières tous les 2 à 5ans. La place des examens fonctionnels plus sophistiqués (échographie cardiaque tissulaire et IRM cardiaque) dans le dépistage des cardiomyopathies reste à définir. Des polymorphismes de gènes impliqués notamment dans le métabolisme des anthracyclines et susceptibles de modifier le risque de cardiomyopathie ont été mis en évidence. La validation de l’effet de ces polymorphismes sur le risque de cardiomyopathie sur des cohortes indépendantes est une étape indispensable avant d’élaborer des modèles prédictifs clinico-génétiques du risque de cardiomyopathie. De tels modèles pourraient être utilisés dans l’avenir pour adapter et personnaliser le traitement des enfants nécessitant un traitement comportant des anthracyclines.

L’objectif de cette étude était d’évaluer l’action de la relaxation sur les différents symptômes rencontrés par les adolescents et les jeunes adultes en cours de traitement pour une tumeur.

S’il est vrai que la pédiatrie nucléaire, au même titre que la radio-pédiatrie pour la radiologie, doit être considérée comme une spécialité à part entière de la médecine nucléaire, ses applications oncologiques méritent également une considération particulière. L’enfant, en effet, n’est pas un adulte en miniature et, surtout en oncologie, il faudra tenir compte de la spécificité des différentes pathologies et de la prise en charge de la douleur, sans oublier les implications psychologiques fondamentales qu’engendre la maladie pour le jeune patient et sa famille. Dans cette présentation, nous avons voulu aborder les principales indications et les approches méthodologiques spécifiques à certains examens, en insistant sur les particularités de cette spécialité.

Recent studies have reported that the pharmacokinetics of high-dose busulfan in bone marrow transplantation (BMT) are age-dependent: with the usual dosage of 16 mg/kg over 4 days, systemic exposure is two to four times lower in children than in adults. Data suggested that the dose of busulfan should rather be calculated on the basis of the body surface area (BSA). We measured plasma pharmacokinetics of busulfan in 27 children (mean age, 5.4 years) who were administered a new dosage of 600 mg/m2 over 4 days, ie, 17.8 to 29.2 mg/kg (mean, 24.8 mg/kg), using a gas chromatography-mass spectrometry assay. Our results demonstrate that, with this new dosage, systemic exposure is significantly increased in children compared with that achieved with the usual dosage of 16 mg/kg (6,404 +/- 2,378 v 3,918 +/- 1,170 ng.h/mL; P = .003). Moreover, there is no longer a significant difference in systemic exposure between children treated with this new dosage and adults given a dose of 16 mg/kg of busulfan. However, despite the use of a dosage normalized to the BSA, there is still a wide interindividual variation in systemic exposure, ranging from 3,566 to 13,129 ng.h/mL, which may account for the high incidence of venoocclusive disease (VOD) of the liver that we have already reported. The optimal dosage and schedule of busulfan in children requires a more individual approach that could be based on dose adjustment and plasma level monitoring.

Sixty children treated for solid tumors with high-dose chemotherapy followed by bone marrow transplantation were randomly assigned to one of two antibiotic protocols. Group A received prophylaxis consisting of ceftazidime plus teicoplanin beginning before the onset of aplasia and fiver; group B received exactly the same antibiotic regimen but beginning at the onset of fever. The two groups were compared in terns of the rate of septicemia, fever of unknown origin, the time-lapse before the appearance of septicemia, the sensitivity of the causative organisms to the antibiotics, the effect of the latter on the intestinal flora, and the rate of fungal infections. The incidence of septicemia was significantly lower in group A (6.6%) than in group B (24.0%), mainly due to the prevention of episodes of early onset. Similarly, the appearance of the first episode of fever was delayed in group A, and the overall duration was reduced. Amphotericin B was prescribed empirically with the same rule in both groups, but three children in group A did not require amphotericin B. The effect on the intestinal flora was similar in the two groups; it must, however, be closely monitored so that the presence of potential pathogens can be dealt with appropriately.

Une santé dégradée pendant l’enfance ou l’adolescence, parce qu’elle peut avoir un impact sur la réussite scolaire ou les stratégies éducatives, peut réduire les chances de mobilité sociale et peut, par conséquent, contribuer à la formation des inégalités sociales de santé. Cependant, les études tendent à confondre les pathologies, rendant difficile l’exploration des mécanismes à l’œuvre. Nous proposons d’étudier ici le cas des cancers de l’enfant et de l’adolescent au travers d’une recherche à méthodes mixtes.

From July 1989 to December 1995, 16 children with metastatic Ewing's sarcoma or PNET were treated with high-dose chemotherapy (HDC) consisting of Busulfan (600mg/m2) and Melphalan (140 mg/m2) followed by autologous SCT. Eight/16 were metastatic at diagnosis: 6/8 were consolidated in first intention and 2 after treatment of a metastatic relapse, 8/16 were localized at diagnosis and received HDC as a consolidation after treatment of a metastatic relapse. Prior to HDC, conventional treatments consisted of chemotherapy for all the patients (pts), surgery of the primary tumor (12/16), surgery of the metastases (4/16), radiotherapy on the primary tumor (10/16). At the time of HDC, CR or GPR of the primary tumor was obtained in 13 and 3 pts, respectively. CR, GPR and PR of the metastases were observed in 10, 5 and 1 pt, respectively. Toxicity was severe but acceptable: 1 toxic death occurred (cerebral hemorrhage). The mean durations of neutropenia and thrombopenia were 10 days (d) (SD=5 d) and 39 d (SD=20 d), respectively. Overall survival at 5 years post SCT was 70% and DFS was 55%. All the 6 metastatic pts grafted in first intention are alive with NED with a median follow up (FU) of 21m (range 13–33m), the 2 metastatic pts grafted after a metastatic relapse died 12 and 22m post SCT. Among the pts with initially localized tumors, 5 are alive with NED with a median FU of 36m post SCT (range 15–59m), 1 is alive with disease, 1 is dead of PD and 1of toxic death. In conclusion, the HDC with Busulfan and Melphalan was toxic but feasible. Prognosis of these children with metastatic disease compares very favorably with historic controls.

Cranio-spinal RxT is the standard prophylaxy in MB but, because of its late effects, children under 3 years of age are currently treated with conventional chemotherapy in order to delete or even avoid RxT. Among these patients treated without RxT in the SFOP study, 20 relapsed under conventional chemotherapy and entered a study of HDC followed by ABMT. Their median age at diagnosis was 23m (R5-71) and the relapse occurred at a median time of 6.3m (±5m) after initiation of chemotherapy. A complete surgery of local relapse was performed in 4/20 and these patients were not evaluable for response. Sixteen out of twenty had measurable disease at primary site (9 pts) at metastatic sites (3 pts) or both (4 pts). Conditioning regimen consisted of combination Busulfan 600mg/m2over 4 days and Thiotepa 900mg/m2over 3 days. After recovery of aplasia, pts with local relapse received local RxT limited to posteriorfossa. Among the 16 pts with measurable disease, following HDC, 6 CR, 6 PR, 3 NR, 1 NE were observed (Response rate 75%). For the 20 pts, EFS is 60% with a median follow up of 9 m post BMT (R3-65). Nine pts with localized relapse are alive NED without craniospinal RxT. Toxicity was high but manageable. One complication related death occurred 1 m post BMT. In conclusion: with a 75% response rate, this HDC proved to be very efficient in relapsed MB. A longer follow up is necessary to demonstrate whether, after local relapse, HDC could replace craniospinal RxT as prophylaxis of CNS metastases.