Tumeurs d'Ewing extra Osseuses, quels traitements ?

Les tumeurs d'Ewing Extra Osseuses (EEO) appartiennent à la fois aux Tumeurs Mésenchymateuses Malignes (TMM), et aux tumeurs d'Ewing Osseuses (EO). Ainsi, la question se pose de les traiter selon un protocole de TMM (MMT89) ou de tumeurs d'EO (Ew93). Parmi 63 cas consécutifs d'EEO traités entre 1989 et 1999 en France, nous avons comparé ceux traités selon le MMT89 (n=32) et ceux traités selon l'Ew93 (n=31). Les caractéristiques des deux groupes sont similaires. La survie globale (SG) et la survie sans événement (SSE) sont de 70 et 58% pour le groupe Ew93, ce qui est identique à celles des 215 enfants traités pour un EO pendant cette période. Inversement, les taux de SG et de SSE sont significativement inférieurs pour le groupe MMT89 (59 et 44%) comparés à ceux des 503 enfants présentant une TMM (71 et 57%), avec un taux élevé de récidives métastatiques. En analyse multivariée, seul le protocole reste un facteur pronostique. L'Ew93 est donc mieux adapté au traitement des EEO.TOULOUSE3-BU Santé-Centrale (315552105) / SudocTOULOUSE3-BU Santé-Allées (315552109) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Etoposide and carboplatin in neuroblastoma: a French Society of Pediatric Oncology phase II study.

PURPOSE: A phase II study of etoposide (VP 16) and carboplatin (CBDCA) was performed in patients with metastatic neuroblastoma (NB). The aim of the study was to find an alternative treatment for induction with different toxicities than the VP 16/cisplatin (CDDP) combination. PATIENTS AND METHODS: Forty-seven patients who were from 6 months to 16 years of age, with either relapsed (29) or primary resistant (18) NB, were included in a cooperative multicenter phase II study of the French Society of Pediatric Oncology (SFOP). The schedule consisted of 5 consecutive days of VP 16 100 mg/m2/d and CBDCA 160 mg/m2/d. RESULTS: The response rate for the 39 assessable patients was 43%; there were four complete remissions and 13 partial remissions. Neither the status of the patients nor the total dose of CDDP that was received previously influenced response. Hematologic toxicity was marked and caused considerable delay between courses (median interval, 39 days). In these heavily pretreated patients, 16% had a more than 50% decrease in creatinine clearance and a 22% World Health Organization (WHO) grade 2 ototoxicity. CONCLUSION: This VP 16/CBDCA combination deserves further evaluation for efficacy and toxicity in newly diagnosed patients, and the combination of both drugs should be considered for high-dose therapy with bone marrow transplantation.Clinical TrialClinical Trial, Phase IIJournal ArticleMulticenter Studyinfo:eu-repo/semantics/publishe

Developmental pharmacokinetics of etoposide in 67 children: lack of dexamethasone effect

International audienc

Poor survival for infants with MYCN-amplified metastatic neuroblastoma despite intensified treatment: the International Society of Paediatric Oncology European Neuroblastoma Experience.

PURPOSE: To report the results of a prospective, nonrandomized European study on infants with neuroblastoma and MYCN gene amplification. PATIENTS AND METHODS: Infants with neuroblastoma (stage 2, 3, 4, and 4s) and MYCN gene amplification who were diagnosed between 1999 and 2004 were eligible for enrollment onto the study. After diagnosis, staging, and mandatory biologic studies, induction chemotherapy (IC) with conventional drugs was administered, followed by delayed surgery, megatherapy (busulfan-melphalan as a conditioning regimen), and local radiotherapy. RESULTS: Of the 46 infants enrolled onto the study, 35 infants were eligible; of these 35 infants, 97% had metastatic spread (24 infants had stage 4, and 10 infants had stage 4s). Two-year overall survival (OS) was 30% (SE, 0.08), with median survival time of 12 months, and 23 deaths due to disease. Two-year, event-free survival (EFS) was 29% (SE, 0.07). The treatment was well tolerated with no deaths as a result of toxicity or severe toxicity. Despite protocol adherence, 30% of the patients who were assessable for response to IC experienced disease progression or did not respond. Stage and high lactate dehydrogenase reached significance in the univariate analysis (P = .028 and .039, respectively for OS; and P = .05 and .031 respectively, for EFS). Ten of 16 patients who received megatherapy are still alive. CONCLUSION: Although treatment was well tolerated, survival was poor and our IC failed to achieve a satisfactory response in 30% of our patients. New therapeutic approaches and more intense world-wide collaboration are needed to achieve a cure in this population