Desmoid-type fibromatosis of the head and neck in children: A changing situation

Antoine Paul, Marie Julie Blouin, Véronique Minard-Colin, Louise Galmiche, Aurore Coulomb, Nadege Corradini, Hélène Boutroux, Thierry Van den Abbeele, Nicolas Leboulanger, Françoise Denoyelle, Erea Nöel Garabedian, Vincent Couloigner, Daniel Orbach

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    Résumé

    Introduction: Desmoid-type fibromatosis (DF) is a rare benign lesion known for its local aggressiveness. The tumor management still remains under debate. Primary head and neck (HN), represents the second most prevalently affected sitein children with DF. This study aims to analyze the specificity of HN-DF in children, focusing on long-term effects of the tumor and therapies. Methods: This retrospective multicenter study analyzed children treated for a HN-DF between 1993 and 2013. All medical files were reviewed and their outcomes analyzed according to the initial therapies provided. Results: Sixteen children were selected. Mandibular and submandibular areas were the main locations (11 cases). Eight children underwent chemotherapy as first-line therapy with tumor control in 3 cases and 5 cases needing additional treatment. Six children underwent primary surgery: isolated in 3 cases and with additional treatment after tumor progression in 3 cases. A wait-and-see attitude was adopted for 2 children without any additional treatment in 1 case, and followed by additional chemotherapy in the other case. Total burden of treatment to control the disease was a biopsy (1 case), surgery (3 unique cases, 1 multiple case), surgery with chemotherapy (6 cases), and exclusive medical therapies (5 cases). Surgical postoperative sequelae were facial palsy (cases of parotid gland affection), XI th cranial nerve sacrifice or sensory impairment. Conclusion: HN-DF is a local and extensive disease that is difficult to control with surgery alone. Sequelae are frequent due to the initial tumor location or therapies. Initial conservative strategies need to be discussed in a multidisciplinary way in order to try to control the disease with the minimal morbidity.

    langue originaleAnglais
    Pages (de - à)33-37
    Nombre de pages5
    journalInternational Journal of Pediatric Otorhinolaryngology
    Volume123
    Les DOIs
    étatPublié - 1 août 2019

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