Maxillo-Facial Surgery Division, Head and Neck Department, University Hospital of Parma, via Gramsci 14, 43100 Parma, Italy.
J Craniomaxillofac Surg. 2013 Jul;41(5):367-70. doi: 10.1016/j.jcms.2012.10.019. Epub 2012 Dec 5.
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.
促纤维增生性纤维瘤是一种罕见的局部侵袭性梭形细胞增生，由浸润在致密胶原基质中的成纤维细胞/肌成纤维细胞组成。头颈部促纤维增生性纤维瘤更为罕见，文献仅限于小病例系列和综述。对 1968 年至 2011 年的英文文献进行全面回顾，仅发现 79 例下颌促纤维增生性纤维瘤，其中仅 18 例（22%）患者年龄在 4 岁以下。彻底切除并切缘清晰是首选的手术方法，但在儿童下颌骨中可能难以实现，尤其是对于这三个报告中的巨大肿瘤。在这些情况下，下颌骨重建是确保正确颅面发育所必需的。作者报告了三例儿童下颌巨大促纤维增生性纤维瘤的病例，采用下颌骨切除术和肋骨移植即刻重建。讨论了适应证、重建选择和结果。
