TY - JOUR
T1 - Desmoid-type fibromatosis of the head and neck in children
T2 - A changing situation
AU - Paul, Antoine
AU - Blouin, Marie Julie
AU - Minard-Colin, Véronique
AU - Galmiche, Louise
AU - Coulomb, Aurore
AU - Corradini, Nadege
AU - Boutroux, Hélène
AU - Van den Abbeele, Thierry
AU - Leboulanger, Nicolas
AU - Denoyelle, Françoise
AU - Garabedian, Erea Nöel
AU - Couloigner, Vincent
AU - Orbach, Daniel
N1 - Publisher Copyright:
© 2019 Elsevier B.V.
PY - 2019/8/1
Y1 - 2019/8/1
N2 - 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.
AB - 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.
KW - Chemotherapy
KW - Children
KW - Desmoid fibromatosis
KW - Neck
KW - Surgery
UR - http://www.scopus.com/inward/record.url?scp=85064980615&partnerID=8YFLogxK
U2 - 10.1016/j.ijporl.2019.04.037
DO - 10.1016/j.ijporl.2019.04.037
M3 - Article
C2 - 31059930
AN - SCOPUS:85064980615
SN - 0165-5876
VL - 123
SP - 33
EP - 37
JO - International Journal of Pediatric Otorhinolaryngology
JF - International Journal of Pediatric Otorhinolaryngology
ER -