BACKGROUND AND METHODOLOGY: The paediatric rare tumours group from the Société française des cancers de l'enfant makes syntheses and guidelines for diagnosis and treatment for localized paediatric inflammatory myofibroblastic tumours according to international articles. MAIN UPDATING: All ages are concerning. Localizations are ubiquitous, more frequently in the superior and inferior airway. Histology showed a majority of fusiform cells, corresponding to myofibroblastic cells and an inflammatory infiltrate. Inflammatory myofibroblastic tumour diagnosis should only be confirmed in the absence of sarcoma molecular markers. CONCLUSIONS: Distinction between inflammatory myofibroblastic tumour and sarcoma is essential due to the different care. The curative treatment of inflammatory myofibroblastic tumour consists on surgery with before or after corticotherapy. In case of unresectability, chemotherapy may be helpful to avoid mutilating surgery.
BACKGROUND AND METHODOLOGY: The paediatric rare tumours group from the Société française des cancers de l'enfant makes syntheses and guidelines for diagnosis and treatment for localized paediatric inflammatory myofibroblastic tumours according to international articles. MAIN UPDATING: All ages are concerning. Localizations are ubiquitous, more frequently in the superior and inferior airway. Histology showed a majority of fusiform cells, corresponding to myofibroblastic cells and an inflammatory infiltrate. Inflammatory myofibroblastic tumour diagnosis should only be confirmed in the absence of sarcoma molecular markers. CONCLUSIONS: Distinction between inflammatory myofibroblastic tumour and sarcoma is essential due to the different care. The curative treatment of inflammatory myofibroblastic tumour consists on surgery with before or after corticotherapy. In case of unresectability, chemotherapy may be helpful to avoid mutilating surgery.