title: Desmoid tumours of the head and neck in children: Review of management.
abstract: OBJECTIVE
  Desmoid tumours of the head and neck in children are rare, local invasive and potentially fatal tumours. The purpose of this review is to discuss the management of these tumours in the light of a case series and a review of the literature.
  
  
  METHODS
  This retrospective study summarised the medical data of children treated for desmoid tumours of the head and neck between 1976 and 2014.
  
  
  RESULTS
  Five of the 6 children were treated by radical surgical resection, with positive surgical margins (R1) in 2 cases, followed by recurrence requiring further resection. One child with a very advanced lesion was treated by weekly methotrexate and vinorelbine chemotherapy for 18months, allowing 93% reduction of tumour volume without recurrence.
  
  
  CONCLUSIONS
  Desmoid tumours of the head and neck in children are more aggressive than their adult counterparts and are associated with high morbidity and mortality and a high recurrence rate. CT and MRI imaging assessment should preferably be performed before biopsy. External beam radiotherapy must be avoided in children as it is less effective than in adults, and is responsible for long-term cosmetic and functional sequelae and even a risk of second tumours. Treatment is surgical whenever radical resection is possible. In patients presenting an excessive risk of morbidity and mortality, chemotherapy devoid of long-term adverse effects (such as methotrexate in combination with a Vinca alkaloid) can be proposed. Long-term follow-up must be ensured due to the risk of recurrence.
authors.affiliation: ORL et Chirurgie Cervico-Faciale Pédiatrique, Hôpital Jeanne de Flandre, CHRU Lille, avenue Eugène-Avinée, 59037 Lille cedex, France.
authors.forename: M
authors.initials: M
authors.lastname: Risoud
authors.affiliation: ORL et Chirurgie Cervico-Faciale, Hôpital Huriez, CHRU Lille, 59037 Lille cedex, France.
authors.forename: G
authors.initials: G
authors.lastname: Mortuaire
authors.affiliation: Service d'Anatomo-Pathologie, Centre de Biologie-Pathologie, CHRU Lille, 59037 Lille cedex, France.
authors.forename: X
authors.initials: X
authors.lastname: Leroy
authors.affiliation: Service d'Oncologie Pédiatrique, Centre Oscar Lambret, 59037 Lille cedex, France.
authors.forename: P
authors.initials: P
authors.lastname: Leblond
authors.affiliation: ORL et Chirurgie Cervico-Faciale Pédiatrique, Hôpital Jeanne de Flandre, CHRU Lille, avenue Eugène-Avinée, 59037 Lille cedex, France. Electronic address: pierre.fayoux@chru-lille.fr.
authors.forename: P
authors.initials: P
authors.lastname: Fayoux
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D005260:Female; D018222:Desmoid Tumors / Q000401:mortality / Q000473:pathology* / Q000601:surgery / Q000628:therapy*; D005500:Follow-Up Studies; D005602:France; D006258:Head and Neck Neoplasms / Q000401:mortality / Q000473:pathology* / Q000601:surgery / Q000628:therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009361:Neoplasm Invasiveness; D009364:Neoplasm Recurrence, Local / Q000473:pathology* / Q000601:surgery / Q000628:therapy*; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: Desmoid tumour; Head and neck; Paediatric