Huge desmoplastic fibroma of the paediatric mandible: Surgical considerations and follow-up in three cases.

The desmoplastic fibroma is a rare locally aggressive spindle-cell proliferation made up of fibroblasts/myofibroblasts immersed in a dense collagenic stroma. Head and neck localisation of desmoplastic fibromas is even more rare and the literature is limited to small case series and reviews. A comprehensive review of the English literature from 1968 to 2011 revealed only 79 cases of mandibular desmoplastic fibromas and only 18 (22%) in patients under 4 years of age. Complete resection with clear margins is the surgical first option, but it can be difficult to achieve in the paediatric mandible, especially for giant tumours as the three reported here. Mandibular reconstruction in these cases is mandatory to ensure correct craniofacial development. The authors present three cases of huge mandibular desmoplastic fibromas in paediatric patients treated with mandibulectomy and immediate reconstruction using rib grafts. Indications, reconstructive options and results are discussed.