Desmoid tumours of the head and neck in children: Review of management.
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.


MATERIAL AND 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.
['Risoud|M|M|', 'Mortuaire|G|G|', 'Leroy|X|X|', 'Leblond|P|P|', 'Fayoux|P|P|']
D000293:Adolescent D000971:Antineoplastic Combined Chemotherapy Protocols D002648:Child D002675:Child, Preschool D005260:Female D018222:Fibromatosis, Aggressive D005500:Follow-Up Studies D005602:France D006258:Head and Neck Neoplasms D006801:Humans D007223:Infant D007231:Infant, Newborn D008297:Male D009361:Neoplasm Invasiveness D009364:Neoplasm Recurrence, Local D012189:Retrospective Studies D016896:Treatment Outcome
Desmoid tumour Head and neck Paediatric