Paul Antoine, Blouin Marie-Julie, Minard-Colin Véronique, Galmiche Louise, Coulomb Aurore, Corradini Nadege, Boutroux Hélène, Van den Abbeele Thierry, Leboulanger Nicolas, Denoyelle Françoise, Garabedian Erea-Nöel, Couloigner Vincent, Orbach Daniel
Pediatric Otolaryngology Department, Necker-Enfants Malades Hospital, Public Assistance-Hospitals of Paris, Paris, France.
Pediatric Oncology Unit, Gustave-Roussy Institute, 94800, Villejuif, France.
Int J Pediatr Otorhinolaryngol. 2019 Aug;123:33-37. doi: 10.1016/j.ijporl.2019.04.037. Epub 2019 Apr 26.
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.
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.
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 cranial nerve sacrifice or sensory impairment.
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.
韧带样型纤维瘤病（DF）是一种罕见的良性病变，以其局部侵袭性而闻名。肿瘤的治疗仍存在争议。原发性头颈部（HN）是DF患儿中第二大最常受累的部位。本研究旨在分析儿童HN-DF的特异性，重点关注肿瘤及治疗的长期影响。
这项回顾性多中心研究分析了1993年至2013年间接受HN-DF治疗的儿童。审查了所有医疗档案，并根据所提供的初始治疗方法分析其结果。
选取了16名儿童。下颌和下颌下区域是主要发病部位（11例）。8名儿童接受化疗作为一线治疗，3例肿瘤得到控制，5例需要额外治疗。6名儿童接受了初次手术：3例为单纯手术，3例在肿瘤进展后需要额外治疗。对2名儿童采取了观察等待的态度，1例未进行任何额外治疗，另1例随后接受了额外化疗。控制疾病的总治疗负担包括活检（1例）、手术（3例单次手术、1例多次手术）、手术联合化疗（6例）和单纯药物治疗（5例）。手术后遗症包括面神经麻痹（腮腺受累病例）、牺牲第XI颅神经或感觉障碍。
HN-DF是一种局部且广泛的疾病，仅靠手术难以控制。由于初始肿瘤位置或治疗方法的原因，后遗症很常见。需要以多学科方式讨论初始保守策略，以尽量以最低的发病率控制疾病。
