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Rivista di Chirurgia Maxillo-Facciale

Official Journal of the Italian Society of Maxillofacial Surgery




Italian Journal of Maxillofacial Surgery 2010 Aprile;21(1):53-8

lingua: Inglese

Aggressive fibromatosis of the pediatric jaw: case report of surgical reconstruction

Gangemi E. N. 1, Aldiano C. 1, Carta M. 1, Verrina G. 1, Buffa P. 2, Giunta C. 2, Jasonni V. 2, Lucchese A. 3

1 Department of Maxillofacial Surgery, E.O. Ospedali Galliera, Genoa, Italy
2 Department of Pediatric Surgery, Giannina Gaslini Institute, Genoa, Italy
3 School of Dentistry, University of Ferrara, Ferrara, Italy


Aggressive fibromatosis (AF) is a benign, slow growing fibroblastic neoplasms, arising from musculoaponeurotic stromal elements. This rare neoplasms have been shown to account in 0.03% of all cancers. Twenty-five percent of all desmoid tumors occur in children under 15 years of age (Juvenile AF). It is characterized by local invasion, with a high rate of local recurrence and a tendency to destroy adjacent structures and organs. This article describes the case of juvenile AF of the head in a two-year-old female involving the left jaw. The tumor has been treated surgically by resection of the mandible and reconstruction with a bone graft from the eighth rib. Fixation was made with resorbable plating system (SonicWeld Rx®, KLS Martin). The authors discuss the clinical approach to juvenile AF and the surgical reconstruction modalities.

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