当前位置： SCI文献检索 > INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY期刊下所有文献 > Desmoid-type fibromatosis of the head and neck in children: A changing situation.

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

Abstract：

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), XIth 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.

journal_name

Int J Pediatr Otorhinolaryngol

journal_title

International journal of pediatric otorhinolaryngology

authors

Paul A,Blouin MJ,Minard-Colin V,Galmiche L,Coulomb A,Corradini N,Boutroux H,Van den Abbeele T,Leboulanger N,Denoyelle F,Garabedian EN,Couloigner V,Orbach D

doi

10.1016/j.ijporl.2019.04.037

subject

Has Abstract

pub_date

2019-08-01 00:00:00

pages

33-37

eissn

0165-5876

issn

1872-8464

pii

S0165-5876(19)30199-5

journal_volume

123

pub_type

杂志文章,多中心研究

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