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The Desmoid Tumor Research Foundation

December 2016

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

Author Information

G Mortuaire,M Risoud,P Fayoux,P Leblond,X Leroy

ORL et Chirurgie Cervico-Faciale Pédiatrique, Hôpital Jeanne de Flandre, CHRU Lille, avenue Eugène-Avinée, 59037 Lille cedex, France.,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.,ORL et Chirurgie Cervico-Faciale, Hôpital Huriez, CHRU Lille, 59037 Lille cedex, France.,Service d'Anatomo-Pathologie, Centre de Biologie-Pathologie, CHRU Lille, 59037 Lille cedex, France.,Service d'Oncologie Pédiatrique, Centre Oscar Lambret, 59037 Lille cedex, France.

Publication

Summary

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.

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