Author/Authors :
Saponaro, Gianmarco Department of Oral and Maxillofacial Surgery - Catholic University of the Sacred Heart Medical School - Rome, Italy , Moro, Alessandro Department of Oral and Maxillofacial Surgery - Catholic University of the Sacred Heart Medical School - Rome, Italy , De Angelis, Paolo Department of Oral and Maxillofacial Surgery - Catholic University of the Sacred Heart Medical School - Rome, Italy , Gasparini, Giulio Department of Oral and Maxillofacial Surgery - Catholic University of the Sacred Heart Medical School - Rome, Italy , Pelo, Sandro Department of Oral and Maxillofacial Surgery - Catholic University of the Sacred Heart Medical School - Rome, Italy , Petrone, Gianluigi Department of Histopathology and Cytopathology - Catholic University of the Sacred Heart Medical School - Rome, Italy , Cordisco, Emanuela Lucci Department of Genomic Medicine - Catholic University of the Sacred Heart Medical School - Rome, Italy , Garagiola, Umberto Department of Orthodontics - University of Milan - Milan, Italy , D’Amato, Giuseppe Department of Oral and Maxillofacial Surgery - Catholic University of the Sacred Heart Medical School - Rome, Italy
Abstract :
Desmoid-type fibromatosis is a benign fibrous neoplasia originating from connective tissue, fascial planes, andmusculoaponeurotic structures of the muscles. Currently, there is no evidence-based treatment approach available for desmoidfibromatosis. In this article, a case of a patient in the pediatric age affected by desmoid fibromatosis localized in the orbit ispresented. )e aim of the article is to describe this unusual and rare location for the desmoid fibromatosis and outline the principlephases in the decision-making process and the therapeutic alternatives for a patient affected by desmoid fibromatosis.Methods.)e protocol of this review included study objectives, search strategy, and selection criteria. )e primary end point of this studywas to analyze the head and neck desmoid fibromatosis. )e secondary end point was to identify the available therapies and assesstheir specific indications.Results. )e mean age of patients was 18.9 years ranging from 0 to 66, and 52% were female. A bimodalage distribution was observed, and two age peaks were identified: 0–14 years (57%) and 28–42 years (18%). )e most commoninvolved areas were the mandible (25%) followed by the neck (21%). In 86% of the cases, the treatment was the surgical resection ofthe disease, and only in 5% of the cases, the surgical resection was followed by adjuvant radiotherapy.Conclusion. )e orbitallocation is extremely rare, especially in the pediatric population. )e management of desmoid fibromatosis is based on thefunction preservation and the maintenance of a good quality of life, but in case of symptomatic patients or aggressive course of thedisease or risk of functional damages, the surgical approach may be considered. )erapeutic alternatives to surgical resection areradiotherapy and systemic therapy.
