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Master Thesis

Tumeurs Malignes des Gaines Nerveuses Périphériques (MPNST) des enfants et adolescents en France de 1995 à 2017 : étude multicentrique rétrospective des données cliniques et biologiques issues des protocoles MMT95, NRSTS05 et du registre national des cancers de l’enfant et de l’adolescent

Abstract : Introduction: MPNST are very rare and aggressive tumors, which affect children, adolescents, and young adults. These tumors appear frequently in NF1 context. These tumors are problematic for diagnosis, treatment, and survival. We decided to make this study to help to understand better MPNST. Methods: we made a multicentric retrospective study of pediatric patients treated in French Pediatric Oncology units, between 1995 and 2017. A pathologic review was compulsory for inclusion, at diagnosis or realized in our study if applicable. Results: we included 66 patients, with age from 0,1 to 18 years. 32 (48,5%) patients suffered from NF1. Overall survival was 39% and Event Free Survival was 39%. Poor prognosis factors were NF1 status, FNCLCC grade 3, size of tumor ≥ 10cm and metastases. The only effective treatment, in association with surgery, were radiotherapy. Conclusion: MPNST are very rare tumors, with a bad prognosis, even for localized forms. We need to make a better stratification of patients, with prognostic factors found in our study, and to develop new therapies to improve overall survival of these patients.
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https://dumas.ccsd.cnrs.fr/dumas-03411711
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Submitted on : Tuesday, November 2, 2021 - 2:44:30 PM
Last modification on : Wednesday, December 8, 2021 - 3:55:31 AM

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  • HAL Id : dumas-03411711, version 1

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Jordane Thierry Benoît Chaix. Tumeurs Malignes des Gaines Nerveuses Périphériques (MPNST) des enfants et adolescents en France de 1995 à 2017 : étude multicentrique rétrospective des données cliniques et biologiques issues des protocoles MMT95, NRSTS05 et du registre national des cancers de l’enfant et de l’adolescent. Sciences du Vivant [q-bio]. 2021. ⟨dumas-03411711⟩

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