[Guidelines for management of localized inflammatory myofibroblastic tumours in children]
Aude Marie-Cardine1, Dominique Berrebi, Daniel Orbach
1CHU Charles-Nicolle, service d'immuno-hémato-oncologie pédiatrique, Rouen, France. aude.marie-cardine@chu-rouen.fr
Insights
Distinguishing paediatric inflammatory myofibroblastic tumours from sarcomas is crucial for appropriate care. Treatment involves surgery, potentially with corticotherapy, or chemotherapy for unresectable cases.
Area of Science:
- Oncology
- Paediatric Pathology
Context:
- Paediatric inflammatory myofibroblastic tumours (IMT) are rare neoplasms affecting all ages.
- IMT commonly present in the airway but can occur anywhere.
- Histological examination reveals myofibroblastic cells and inflammatory infiltrate.
Purpose:
- To provide updated guidelines for the diagnosis and treatment of localized paediatric IMT.
- To emphasize the importance of differentiating IMT from sarcomas.
Summary:
- Diagnosis requires excluding sarcoma molecular markers.
- Treatment strategies include surgery, corticotherapy, and chemotherapy for unresectable tumors.
- The Société française des cancers de l'enfant developed these guidelines based on international literature.
Impact:
- Accurate diagnosis ensures appropriate patient management.
- Guidelines aid clinicians in selecting optimal treatment for paediatric IMT.
- Distinguishing IMT from sarcoma prevents misdiagnosis and ensures correct therapeutic pathways.
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.
