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Desmoid-type fibromatosis of the head and neck in children: A changing situation.
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.
Afiliação
  • Paul A; Pediatric Otolaryngology Department, Necker-Enfants Malades Hospital, Public Assistance-Hospitals of Paris, Paris, France.
  • Blouin MJ; Pediatric Otolaryngology Department, Necker-Enfants Malades Hospital, Public Assistance-Hospitals of Paris, Paris, France.
  • Minard-Colin V; Pediatric Oncology Unit, Gustave-Roussy Institute, 94800, Villejuif, France.
  • Galmiche L; Pathology Department, Necker-Enfants Malades Hospital, Public Assistance-Hospitals of Paris, Université Sorbonne Paris Cité, 75015, Paris, France.
  • Coulomb A; Department of Pathology, Armand Trousseau Hospital, Public Assistance-Hospitals of Paris, Paris, France.
  • Corradini N; Institut D'Hématologie et D'Oncologie Pédiatrique, Centre Leon Berard, Lyon, France.
  • Boutroux H; Hematology and Oncology Department, Armand Trousseau Hospital, Public Assistance-Hospitals of Paris, Paris, France.
  • Van den Abbeele T; Pediatric Ear, Nose, and Throat Department, Robert Debré Hospital, Public Assistance-Hospitals of Paris, Paris, France.
  • Leboulanger N; Pediatric Otolaryngology Department, Necker-Enfants Malades Hospital, Public Assistance-Hospitals of Paris, Paris, France.
  • Denoyelle F; Pediatric Otolaryngology Department, Necker-Enfants Malades Hospital, Public Assistance-Hospitals of Paris, Paris, France.
  • Garabedian EN; Pediatric Otolaryngology Department, Necker-Enfants Malades Hospital, Public Assistance-Hospitals of Paris, Paris, France.
  • Couloigner V; Pediatric Otolaryngology Department, Necker-Enfants Malades Hospital, Public Assistance-Hospitals of Paris, Paris, France.
  • Orbach D; SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), Institut Curie, PSL University, Paris, France. Electronic address: daniel.orbach@curie.fr.
Int J Pediatr Otorhinolaryngol ; 123: 33-37, 2019 Aug.
Article em En | MEDLINE | ID: mdl-31059930
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.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Neoplasias Mandibulares / Fibromatose Agressiva / Neoplasias de Cabeça e Pescoço Tipo de estudo: Etiology_studies / Observational_studies / Risk_factors_studies Limite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male Idioma: En Revista: Int J Pediatr Otorhinolaryngol Ano de publicação: 2019 Tipo de documento: Article País de afiliação: França

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Neoplasias Mandibulares / Fibromatose Agressiva / Neoplasias de Cabeça e Pescoço Tipo de estudo: Etiology_studies / Observational_studies / Risk_factors_studies Limite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male Idioma: En Revista: Int J Pediatr Otorhinolaryngol Ano de publicação: 2019 Tipo de documento: Article País de afiliação: França