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Most operators perform the ileo-colic anastomosis extracorporeally because the manual intra-abdominal anastomosis technique is more difficult. Two trocars are then added for the assistant's use; these are always placed behind the robot trocars: a 12mm trocar in the left iliac fossa (between optical and R2 trocars) and a 5mm at the level of the left flank (between optical and R1 trocars). Graph 8 Performing manual intracorporeal side-to-side ileo-colic anastomosis, iso- or aniso-perista... The ileum and colon are opened along the antimesenteric border with monopolar scissors via the R1 arm. [Extracted from the article]

Summary: Primary heart tumors are uncommon in children. The majority of them are benign, with only 10% malignant. Among malignant cardiac tumors, sarcoma (rhabdomyosarcoma, angiosarcoma, synovial sarcoma) and lymphoma (Burkitt''s lymphoma, large B-cell lymphoma, lymphoblastic lymphoma) predominate. There are few published pediatric series on malignant primary cardiac tumors. We report here 3 observations of primary malignant cardiac tumors, 2 cases of sarcoma (angiosarcoma and synovial sarcoma) and 1 case of Burkitt''s lymphoma. A precise pathological diagnosis is necessary for the proper management of these patients. For sarcoma, treatment associates surgery and chemotherapy. Surgery should be as complete as possible because of the lack of chemotherapy sensitivity of some sarcomas, mainly angiosarcoma and synovial sarcoma. Therefore, the prognosis of cardiac sarcoma remains poor. For primary cardiac lymphoma, management should not be different from lymphoma in other locations. Chemotherapy is the main treatment, and surgery has to be used only when complications occur. Prognosis depends on histology and not lymphoma location, and so is better than the prognosis for sarcoma. [Copyright &y& Elsevier]

Abstract: Objective: To evaluate the impact of surgery as first-line treatment on event-free survival (EFS) of primary aggressive fibromatosis. Patients and methods: Treatments were categorized into: surgery with or without radiotherapy and nonsurgical strategies with systemic treatment alone or wait and see policy. Eighty-nine patients had initial resection of their primary tumour followed by postoperative radiotherapy in 13 cases. Twenty-three did not undergo surgery but received systemic treatment or watch and wait policy. Results: Median follow-up was 76 months. Overall 3 years EFS was 49%. In the univariate analysis, patients with microscopically complete surgery had a similar outcome to patients in the no-surgery group (3 years EFS of 65% and 68%, respectively). Gender, age, tumour size, treatment period and strategy (surgery versus no-surgery) were not statistically significant. Quality of resection according to margins and the tumour site were the only prognostic factors. There was a significant correlation between tumour site and quality of surgery (p =0.0002). Conclusions: A subset of patients with extra-abdominal fibromatosis could be managed with a nonaggressive policy, as growth arrest concerned 2/3 of nonoperated patients. When surgery is finally necessary, it should be performed with the aim of achieving negative margins. [Copyright &y& Elsevier]

Medulloblastoma is the most common primary malignant central nervous system tumor in childhood, accounting for 16–25% of cases (1). New treatment approaches have led to improved survival rates; however toxicities are still a major concern. Participants were selected from the records of patients who were treated with craniospinal irradiation for medulloblastoma. Between January 2008 and December 2012, 62 patients were diagnosed with medulloblastoma at the national institute of oncology Rabat, 27 patients were still alive at the time of the study, of which n = 16 patients were included in the study. The mean age of patients at the time of the study was 9.6 years. All children were treated with radiation therapy and chemotherapy, according to standard protocols. Median follow-up between treatment and evaluation was 4 years. All the children were assessed with the Wechsler Intelligence Scale for Children - fourth Edition (WISC-IV) three to five years after completion of radiotherapy. The test was administered by two well-trained psychologists in a distraction-free environment. The scoring was then reviewed by a psychologist from Brooklyn College. The mean standard score Full-Scale Intelligence Quotient (FSIQ) (M = 63, SD = 12.6) was found to be in the extremely low range and in the 1st percentile rank (PR), compared to the general population. All the measured primary index scales were below typical performance: verbal comprehension (M = 67.7, SD = 13.1), perceptual reasoning (M = 63.5, SD = 13.8) and processing speed (M = 62.7, SD = 15.5) were all found to be in the extremely low range, while xorking memory (M = 75.5, SD = 10.8) was found to be in the borderline range compared to the general population. To identify factors influencing the results, we performed both univariate and multivariate analyses. Age at the time of radiotherapy, initial clinical stage, total cranial radiotherapy dose, socioeconomic status, and the time of evaluation were identified as significantly impacting cognitive scores in the univariate analysis. In the multivariate analysis, only age at the time of radiotherapy and initial clinical stage remained factors significantly impacting cognitive outcomes with P = 0.001 and P < 0.001 respectively. Our study is evidence that tremendous efforts are still to be made in low-income countries to correctly measure neurocognitive dysfunction in medulloblastoma survivors and to prepare those patients to a typical life after the completion of treatment. [ABSTRACT FROM AUTHOR]

Matériel et Méthodes: Nous avons exploré la fonction testiculaire de 171 patients âgés de plus de 17 ans guéris d’un lymphome par des dosages sériques de FSH. Résultats: Le taux de FSH est élevé chez 42,1% des patients. Trois drogues ont un effet délétère dose-dépendant sur le taux de FSH : cyclophosphamide (CPM) (p < 0,0001), CCNU (p = 0,005), et procarbazine (p = 0,0001). Après maladie de Hodgkin, le taux de FSH augmente avec le nombre de cures de MOPP reçues: 7,5 ± 6,6 UI/l (ffi 3 MOPP), 13,6 ± 8,3 (4-6 MOPP) et 24,3 ± 14 (ffl6 MOPP) (p = 0,001). Après lymphome B, le taux de FSH varie avec l’évolution des protocoles : 12,7 ± 5,7 UI/l pour les protocoles utilisés avant 1984 (CPM 12g/m2, CCNU 104mg/m2), 10,2 ± 7,6 UI/l entre 1984 et 1986 (CPM 7g/m2, CCNU 47mg/m2) et 6 ± 5,1 UI/l après 1986 (CPM 5,5g/m2, CCNU 0) (p < 0,0001). Après lymphome T, le taux de FSH est passé de 9,4 ± 5,7 UI/l pour les protocoles utilisés avant 1989 à 6,8 ± 9,6 UI/l pour les plus récents, ceci semble lié à l’absence de CCNU et de Caryolysine dans les protocoles postérieurs à 1989 (p = 0,035). Conclusion: Les alkylants ont une toxicité testiculaire dose-dépendante. Grâce à la diminution des doses d’alkylants au cours du temps, la toxicité testiculaire des protocoles récents de traitement des lymphomes a significativement diminué. [ABSTRACT FROM AUTHOR]

With the increase in survival from childhood cancer, research has increasingly focused on the educational and professional achievements of childhood cancer survivors. Yet, if large-scale studies provide an acute description of the current situation of childhood cancer survivors, little is known about their trajectories and the social processes shaping these trajectories. Using a qualitative methodology, drawing from a life course perspective, this study sought to describe the role of childhood cancer and its side effects in educational trajectories, as perceived by the participants. We investigated related processes of social adjustment to cancer, that is to say, choices or decisions that survivors related to the illness in the making of their career plans. Eighty long-term French childhood cancer survivors participating in the Euro2K longitudinal study were interviewed through in-depth, face-to-face interviews undertaken in 2011–2012. There were various types of impact described by respondents of the diagnosis of cancer on their trajectories. These varied according to gender. In women, childhood cancer tended to result in poor educational achievement, or in steering the individual towards a health care or child care occupation. This was justified by a desire to return the support that had been offered to them as patients. In men, however, childhood cancer led to a shift in career plans, because of physical sequelae, or because of concerns about their future health. Paradoxically, this limitation had a positive impact in their occupational achievement, as most of these men disregarded blue-collar jobs and chose more qualified white-collar occupations. Overall, findings suggest that childhood cancer influenced educational trajectories and, thus, socioeconomic status in adulthood, through mechanisms embedded in gender norms. These mechanisms could explain gender inequalities in educational achievement after childhood cancer reported in large-scale cohort studies. [ABSTRACT FROM AUTHOR]

The paper report the results of the first European prospective nonrandomized trial dedicated to pediatric synovial sarcoma. The study included 138 patients treated from 2005 to 2012 with a multimodal therapeutic approach. The overall treatment results were higher than those previously published by pediatric groups.Background To report the results of the first European prospective nonrandomized trial dedicated to pediatric synovial sarcoma. Patients and methods From August 2005 to August 2012, 138 patients <21 years old with nonmetastatic synovial sarcoma were registered in 9 different countries (and 60 centers). Patients were treated with a multimodal therapy including ifosfamide–doxorubicin chemotherapy and radiotherapy, according to a risk stratification based on surgical stage, tumor size and site, and nodal involvement. Results With a median follow-up of 52.1 months (range 13.8–104.4 months), event-free survival (EFS) was 81.9% and 80.7%, and overall survival (OS) was 97.2% and 90.7%, at 3 and 5 years, respectively. The only significant prognostic variable at univariate analysis was the risk group: 3-year EFS was 91.7% for low-risk, 91.2% for intermediate-risk, and 74.4% for high-risk cases. In 24 low-risk patients (completely resected tumor ≤5 cm in size) treated with surgery alone, there were two local relapses and no metastatic recurrences. Among 67 high-risk patients (unresected, or axial tumor or nodal involvement), 66 underwent surgery after neoadjuvant chemotherapy. Response to chemotherapy was 55.2%, including 22.4% cases with complete or major partial remissions, and 32.8% with minor partial remissions. Conclusion This study demonstrates that collaborative prospective studies on rare pediatric sarcomas are feasible even on a European scale, with excellent treatment compliance. The overall results of treatment were satisfactory, with higher survival rates than those previously published by pediatric groups. Nonetheless, larger, international projects are needed, based on a cooperative effort of pediatric and adult oncologists. Clinical Trials number European Union Drug Regulating Authorities Clinical Trials No. 2005-001139-31. [ABSTRACT FROM PUBLISHER]

Parameningeal (PM) site is a well-known adverse prognostic factor in children with rhabdomyosarcoma (RMS). A pooled analysis of data from 1105 patients with PM RMS differentiates those with good prognosis (36% patients with 0-1 risk factor: 10-yr OS 80.9%) from high risk PM patients (28% with 3-4 factors: 10-yr OS 51.1%). Furthermore, this analysis reinforces the necessity for radiotherapy in PM RMS.Background Parameningeal (PM) site is a well-known adverse prognostic factor in children with localized rhabdomyosarcoma (RMS). To identify risk factors associated with outcome at this site, we pooled data from 1105 patients treated in 10 studies conducted by European and North American cooperative groups between 1984 and 2004. Patients and methods Clinical factors including age, histology, size, invasiveness, nodal involvement, Intergroup Rhabdomyosarcoma Study (IRS) clinical group, site, risk factors for meningeal involvement (MI), study group, and application of radiotherapy (RT) were studied for their impact on event-free and overall survival (EFS and OS). Results Ten-year EFS and OS were 62.6 and 66.1% for the whole group. Patients without initial RT showed worse survival (10-year OS 40.8% versus 68.5% for RT treated patients). Multivariate analysis focusing on 862 patients who received RT as part of their initial treatment revealed four unfavorable prognostic factors: age <3 or >10 years, signs of MI, unfavorable site, and tumor size. Utilizing these prognostic factors, patients could be classified into different risk groups with 10-year OS ranging between 51.1 and 80.9%. Conclusions While, in general, PM localization is regarded as an adverse prognostic factor, the current analysis differentiates those with good prognosis (36% patients with 0–1 risk factor: 10-year OS 80.9%) from high-risk PM patients (28% with 3–4 factors: 10-year OS 51.1%). Furthermore, this analysis reinforces the necessity for RT in PM RMS. [ABSTRACT FROM AUTHOR]

Résumé: La curiethérapie est rarement utilisée en cancérologie pédiatrique, et réservée à des institutions spécialisées. Les localisations tumorales les plus fréquemment ainsi traitées sont les rhabdomyosarcomes d’origine gynécologique ou vésicoprostatiques. Les avantages balistiques de la curiethérapie en font une technique de choix en pédiatrie, puisqu’elle permet de protéger sélectivement les tissus sains. Les techniques rapportées, les modalités de la curiethérapie (bas débit de dose, haut débit de dose, débit pulsé), les doses et les indications varient en fonction des centres. Cette revue analyse les techniques, les indications en fonction des deux localisations les plus fréquentes, gynécologique et vésicoprostatique, et les résultats de cette technique qui nécessite une indispensable approche multidisciplinaire. [ABSTRACT FROM AUTHOR]

Summary: Soft tissue tumors account for approximately 25% of neonatal tumors and are most often benign (more than 2/3 of cases). Vascular tumors are the most frequent benign tumors and infantile hemangioma accounts for 32% of these tumors, affecting 1 out of 200 children at birth. Kaposiform hemangioendothelioma (KH) is a rare vascular tumor with locally aggressive behavior. More than 50% of KH are associated with the Kasabach-Merritt phenomenon, a condition characterized by thrombocytopenia and consumptive coagulopathy. Malignant soft tissue tumors are, after neuroblastoma, the second cause of cancer in neonates. Infantile fibrosarcoma (IF) is a rare tumor that most often affects the extremities of children aged 4 years or younger. A recurrent t(12;15) (p13;q25) rearrangement fusing the ETV6 gene with the NTRK3 neurotrophin-3 receptor gene has been identified in IF. Complete conservative surgical resection is usually curative. Chemotherapy is indicated when initial surgical removal cannot be accomplished without unacceptable morbidity. Prognosis of IF is excellent, with reported overall survival rates ranging from 80 to 100%. Neonatal rhabdomyosarcoma (RMS) is a rare tumor (0.5–1% of RMS). The primary tumor predominantly involves the limbs and the genitourinary tract. Treatment is based on age-adapted chemotherapy and surgery. Prognosis of RMS in children less than 1 year old appears to be comparable with that of older children. [Copyright &y& Elsevier]

Abstract: Introduction. – Ifosfamide is an alkylating agent used in the treatment of germ-cell tumors, sarcomas and lymphomas. One of its main side effects is the encephalopathy of which the incidence may reach 30% in the literature, in adults and children just as well. Objectives. – Based on both our experience and a review of the literature, we propose some recommendations for the management of this complication. Patients and methods. – We report 15 encephalopathy cases in non-brain tumor patients, which occurred between January 1987 and March 2002 in children from 2 to 17 years old, treated for solid tumors at the Institut Gustave Roussy. Ifosfamide was administered at a posology between 5.4 and 15 g/m2/course, associated with other antimitotics such as actinomycin D, etoposide or vincristine. Results. – Six patients experienced a grade III neurological toxicity according to the NCI classification, which developed as excess drowsiness lasting up to 36 hours. Six other patients developed grade IV neurotoxicity, including two comas resolving within 4 days and four short generalized convulsions. Three other children experienced grade II drowsiness. Brain MRIs were normal and EEG showed an aspecific encephalopathy tracing. This early central neurotoxicity appeared right from the first administration, and occurred immediately after the first injection or during the second or third day of treatment. It was most often reversible, usually 3 to 5 days after the last ifosfamide administration. Five patients were administered a treatment with Methylene Blue with a demonstrable efficacy in only one case. No death or neurological sequelae have been noted. Ifosfamide has been renewed after the neurological accident in 7 of those patients. Only 1 of those 7 patients developed grade IV neurotoxicity during the next course of treatment. In 2 of those 7 children, Methylene Blue was used in a prophylactic way. No neurological disorders have been noted during the next courses of treatment. Discussion. – In the literature, the following are described as risk factors for ifosfamide encephalopathy: advanced pelvic disease, previous cisplatyl treatment and renal failure. We have not found any of these predisposing factors in our series, but three of the fifteen patients had severe neurotoxicity associated with Vincristin during previous treatments. Conclusion. – Facing a clinical diagnosis of ifosfamide encephalopathy, it is recommended to discontinue administration of ifosfamide and inject by intravenous route 50 mg Methylene Blue every 4 hours until the symptomatology recedes. The re-challenge of Ifosfamide is not contra-indicated and should be performed under prophylactic treatment with Methylene Blue by intravenous route at the dose of 50 mg every 6 hours. [Copyright &y& Elsevier]

The aim of this study was to determine therapy-related risk factors for the development of melanoma after childhood cancer. Among 4401 3-year survivors of a childhood cancer in eight French and British centres and 25 120 patients younger than 20 years old at first malignant neoplasm (FMN) extracted from the Nordic Cancer Registries, 16 patients developed a melanoma as a second malignant neoplasm (SMN). A cohort study of the French and British cohorts was performed. In a nested case–control study, the 16 patients who developed a melanoma as a SMN (cases) were matched with 3–5 controls in their respective cohort according to gender, age at the first cancer, the calendar year of occurrence of the first cancer and follow-up. Radiotherapy appeared to increase the risk of melanoma for local doses >15 Gy, Odds Ratio (OR)=13 (95% Confidence Interval (CI): 0.94–174). Regarding chemotherapy, we observed an increased OR for both alkylating agents and spindle inhibitors, OR=2.7 (95% CI: 0.5–14). Children treated for a gonadal tumour as a FMN were found to be at a higher risk of melanoma, OR=8.7 (95% CI: 0.9–86). The adjusted OR for the local radiation dose was 1.07 (95% CI: 1.00–1.15). In conclusion, radiotherapy may contribute to an increased risk of melanoma as a SMN, but only at very high doses of low linear energy transfer radiation. Common genetic origins between gonadal tumours and malignant melanomas are likely. [Copyright &y& Elsevier]

From 1984 to 1997, 57 consecutive patients with non-metastatic Ewing''s sarcoma of the ribs were treated according to multimodal French Society of Pediatric Oncology (SFOP) protocols EW 84, EW 88 and EW 93. The results of treatment were reviewed and analysed. Median age was 12 years. 34 patients had large tumours (greatest tumour dimension 8 cm); pleural effusion was noted in 26. A tumour-positive margin after surgery was noted in 15 patients. Histological response after chemotherapy was assessed in 34 patients. 34 patients received radiation therapy. With a median follow-up of 5 years, the projected overall and relapse-free survival rates were 69 and 62%, respectively. The major site of relapse was local. None of the following was significant in predicting relapse: tumour size, gender, age at diagnosis, existence of pleural effusion, level of rib tumour, rib component, type of local control, surgical margin (positive or negative). Response to chemotherapy was the sole significant prognostic factor (P=0.004). Patients with pleural effusion had a higher percentage of relapse if they were treated without local radiation therapy. Our study confirms the prognostic significance of response to initial chemotherapy. Radiation therapy may be withheld in selected cases, but seems necessary in patients with pleural effusion. [Copyright &y& Elsevier]

Objectifs: En 2003 la SFCE a proposé des recommandations MDH03 de prise en charge des lymphomes de Hodgkin de l’enfant. 3 groupes: GMH1stades favorables, GMH2 intermédiaires et GMH3 avancés. Les patients étaient évalués par TDM après 4 cures. En cas de réponse < 75 %, chimiothérapie complémentaire. Pour tous, radiothérapie complémentaire 20Gy IF ± boost. Patients et méthodes: Enregistrement prospectif. 470 patients dont 407 ayant des données exploitables. Age médian 14 ans, 191 filles. Stades IA n = 19;IB n = 8;IIA n = 131;IIB n = 58;IIIA n = 41; IIIB n = 42;IVA n = 45; IVB n = 63;13 %E. Résultats: GMH1 n = 89(22 %), GMH2 n = 181(45 %), GMH3 n = 137(33 %). GMH1: 88 % de bons répondeurs (BR) après 4xVBVP. GMH2:83 % BR après 4xCOPP/ABV. GMH3:94 %BR après 3xOPPA. 4 % des pts ont eu un boost. Recul médian de 32 mois (0-67), 9/407 pts sont décédés. OS à 5 ans 96 %(+/-2 %) (GMH1 100 %, GMH2 94 %, GMH3 95 %). EFS 86 %(+/-2 %) (GMH1 97 %, GMH2 81 %, GMH3 86 %). 2 % des pts réfractaires. Conclusion: Les recommandations MDH03 ont été largement appliquées, avec des résultats encourageants. 60 % des pts ont reçu < 160mg/m2 d’anthracyclines et irradiation 20Gy. Depuis 2008, la SFCE est associée au protocole EURONET PHL-C1 qui évalue l’abstention de radiothérapie pour les BR à 2 cures et randomise la dacarbazine/procarbazine. [ABSTRACT FROM AUTHOR]

Les enfants suivis en oncologie ont un risque infectieux augmenté en particulier de bactériémies et fongémies, cause majeure de morbidité qui peut mettre en jeu le pronostic vital. Nous avons étudié rétrospectivement toutes les hémocultures positives entre 2000 et 2007 du département de pédiatrie de l’Institut Gustave Roussy, afin de décrire les caractéristiques épidémiologiques des patients à risque et de déterminer l’évolution de ces épisodes. Nous avons recensé 1091 hémocultures positives, soit 345 épisodes bactériémiques chez 290 patients ; 47 % ont eu lieu chez des patients neutropéniques et 13 % après une autogreffe. Les germes mis en évidence étaient des cocci gram positif dans 42,3 % des cas puis des bacilles gram négatifs (BGN) dans 35,7 % des cas. Les infections étaient polymicrobiennes dans 15,6 % des cas et il y a eu 2,3 % de fongémies. Dans 50 % des cas, la voie veineuse centrale a été retirée. En outre, l’infection a été compliquée d’un choc septique dans 9 % des cas et d’un transfert en USI dans 5,2 %, surtout en cas de BGN ; seulement deux décès ont été constatés. L’amélioration des connaissances sur les bactériémies en oncologie pédiatrique va permettre d’optimiser leur prise en charge en terme de gestion des voies d’abord et de l’antibiothérapie. [ABSTRACT FROM AUTHOR]

Les séquelles endocriniennes après allogreffe de moelle osseuse et irradiation corporelle totale (TBI) pour hémopathie sont bien connues, contrairement à celles des autogreffes de moelle sans TBI. 88 enfants ayant reçu une chimiothérapie à haute dose et une autogreffe de moelle sans TBI pour une tumeur solide (52 % de neuroblastomes) à un âge moyen de 6 ± 4.4 ans, ont été étudiés à un âge moyen de 16 ± 5.7 ans et un recul de 10 ± 3.6 ans post-greffe. 57 % ont reçu un conditionnement contenant du Busulfan. L’étude de la croissance montre une perte moyenne de presque 1 DS entre le diagnostic de tumeur et le bilan actuel, corrélée à l’utilisation de la radiothérapie du squelette. La taille finale est inférieure à –2DS dans 22 % des cas. 11 enfants avaient un déficit en hormone de croissance, secondaire à une irradiation craniale chez seulement 5 d’entre eux. La puberté s’est déroulée normalement chez la plupart des garçons (87 %), mais 89 % ont une atteinte tubulaire testiculaire (FSH > 14 UI/l pour 62 %, volume testiculaire < 10 ml pour 86 %). Seulement 2 garçons ont une atteinte Leydigienne après irradiation testiculaire, mais 22 % ont des taux de LH > 8 UI/l. 66 % des filles ont une atteinte ovarienne dont l’incidence est liée à l’utilisation du Busulfan, l’âge et le stade pubertaire au moment de la greffe. 86 % ont un examen et une fonction thyroïdienne normale. Des anomalies thyroïdiennes ne sont observées qu’après irradiation cervicale ou traitement d’un neuroblastome : élévation de la TSH > 6 mUI/l (8 %), nodules thyroïdiens (6.8 %). La fonction surrénalienne est normale chez tous les patients. Conclusions: 1/L’insuffisance ovarienne et l’atteinte tubulaire testiculaire sont les principales séquelles endocriniennes de la chimiothérapie à haute dose avec autogreffe de moelle avec une fréquence équivalente à celle des conditionnements avec TBI. 2/La croissance est moins altérée après conditionnement par chimiothérapie seule qu’avec TBI. 3/L’irradiation cervicale et le neuroblastome (ou sa prise en charge) sont les principaux facteurs de risque d’atteinte thyroïdienne dans cette population. [Copyright &y& Elsevier]

Le traitement des tumeurs malignes du petit bassin de l’enfant nécessite souvent un traitement lourd et multimodal dont le recours à la radiothérapie dans le contrôle tumoral local. L’irradiation peut générer elle même des complications sur les organes en développement de l’enfant. L’exclusion pelvienne chirurgicale permet de déplacer les organes intra pelviens hors du champs d’irradiation. Entre 1987 et 2008, 20 enfants suivis à l’IGR ont bénéficié d’une exclusion des viscères intrapelviens par mise en place d’une prothèse d’expansion associée ou non à une compartimentation abdominale complémentaire. La durée moyenne d’exclusion était de 97 jours. La tolérance était bonne dans 85 % des cas. 2 patients ont présenté une complication lors de l’intervention de mise en place. La radiothérapie externe a pu être réalisée dans 90 % des cas avec une dose moyenne de 50,6 Gy. 2 Patients ont eu une entérite aiguë rapidement résolutive et un seul patient a présenté des complications digestives sévères à long terme. La mise en place d’une prothèse d’expansion en vue de protéger les viscères intrapelviens est une technique sûre et reproductible chez l’enfant. Elle permet d’éloigner les organes à risque du champ d’irradiation sans surmortalité et au prix d’une morbidité faible et acceptable. [ABSTRACT FROM AUTHOR]

Objectifs: colliger des observations de MM pédiatriques afin d’améliorer notre connaissance de cette pathologie. Méthode: étude rétrospective des MM pris en charge par la SFCE. Résultats: 12 patients (6 garçons) d’âge médian de 12 ans ont été identifiés. La localisation était péritonéale (7 cas), vaginale testiculaire (1 cas), péricardique (1 cas), multi sérique (3 cas). 6 cas étaient métastatiques dans au diagnostic. Le délai moyen diagnostique était de 200 jours. Le traitement initial était multimodal chez 9 patients. Une chirurgie a été réalisée chez 7 patients au diagnostic et chez 3 patients après chimiothérapie. 9 patients ont reçu de la chimiothérapie durant leur traitement de première ligne. Les agents utilisés étaient variés. Une CHIP a été réalisée chez 5 patients. À l’issue de la première ligne de traitement ; 5 RC, 2 RP, 0 MS et 4 MP ont été observées. 1 patient a été perdu de vue. 66 % des patients ont nécessité plusieurs lignes de traitement (jusqu’à 5). La SSE à 2 et 5 ans est de 38 and 30 %. La SG à 2 et 5 ans est respectivement de 90 and 80 %. Conclusion: MM est une tumeur extrêmement rare de l’enfant qui semble différente de la maladie observée chez l’adulte (moins de localisation pleurale et un pronostic supérieur malgré de fréquentes rechutes). [ABSTRACT FROM AUTHOR]

Objectifs: L’esthésioneuroblastome (ENB) est une tumeur rare développée aux dépend de l’épithélium de la cavité nasale supérieure et de l’ethmoïde. Nous avons revu l’expérience de l’Institut Gustave Roussy sur les ENB des enfants et adolescents. Matériels et Méthodes: Onze enfants et adolescents ont pris en charge sur une période de 20 ans. 9 patients ont reçu un traitement par chimiothérapie initiale suivie de chirurgie et de radiothérapie. Un patient a été traité par chirurgie et radiothérapie sans chimiothérapie ; un autre patient en rémission complète après chimiothérapie n’a pas eu de chirurgie avant la radiothérapie. Résultats: 10 patients étaient évaluables pour la réponse au traitement, parmi lesquels 7 ont été mis en rémission complète ou partiale par la chimiothérapie. Seul un patient a rechuté localement et sous forme métastatique 9 mois après le diagnostic. Il est décédé en quelques semaines. Les 10 autres sont vivants en première rémission. Avec un suivi médian de 8,8 ans (extrêmes : 9,9-16,4), la survie sans évènement et la survie globale à 5 ans sont de 91 % (95 % IC : 62 % – 98 %). Conclusions: Ces résultats montrent la chimiosensibilité des ENB et l’efficacité d’un traitement combinant chimiothérapie, chirurgie et radiothérapie. [Copyright &y& Elsevier]