Orbital Desmoid-Type Fibromatosis: A Case Report and Literature Review
Purpose. Desmoid-type fibromatosis is a benign fibrous neoplasia originating from connective tissue, fascial planes, and musculoaponeurotic structures of the muscles. Currently, there is no evidence-based treatment approach available for desmoid fibromatosis. In this article, a case of a patient in...
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| Format: | Article |
| Language: | English |
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Wiley
2018-01-01
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| Series: | Case Reports in Oncological Medicine |
| Online Access: | http://dx.doi.org/10.1155/2018/1684763 |
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| author | Alessandro Moro Paolo De Angelis Giulio Gasparini Sandro Pelo Gianluigi Petrone Emanuela Lucci Cordisco Umberto Garagiola Giuseppe D’Amato Gianmarco Saponaro |
| author_facet | Alessandro Moro Paolo De Angelis Giulio Gasparini Sandro Pelo Gianluigi Petrone Emanuela Lucci Cordisco Umberto Garagiola Giuseppe D’Amato Gianmarco Saponaro |
| author_sort | Alessandro Moro |
| collection | DOAJ |
| description | Purpose. Desmoid-type fibromatosis is a benign fibrous neoplasia originating from connective tissue, fascial planes, and musculoaponeurotic structures of the muscles. Currently, there is no evidence-based treatment approach available for desmoid fibromatosis. In this article, a case of a patient in the pediatric age affected by desmoid fibromatosis localized in the orbit is presented. The aim of the article is to describe this unusual and rare location for the desmoid fibromatosis and outline the principle phases in the decision-making process and the therapeutic alternatives for a patient affected by desmoid fibromatosis. Methods. The protocol of this review included study objectives, search strategy, and selection criteria. The primary end point of this study was to analyze the head and neck desmoid fibromatosis. The secondary end point was to identify the available therapies and assess their specific indications. Results. The mean age of patients was 18.9 years ranging from 0 to 66, and 52% were female. A bimodal age distribution was observed, and two age peaks were identified: 0–14 years (57%) and 28–42 years (18%). The most common involved areas were the mandible (25%) followed by the neck (21%). In 86% of the cases, the treatment was the surgical resection of the disease, and only in 5% of the cases, the surgical resection was followed by adjuvant radiotherapy. Conclusion. The orbital location is extremely rare, especially in the pediatric population. The management of desmoid fibromatosis is based on the function preservation and the maintenance of a good quality of life, but in case of symptomatic patients or aggressive course of the disease or risk of functional damages, the surgical approach may be considered. Therapeutic alternatives to surgical resection are radiotherapy and systemic therapy. |
| format | Article |
| id | doaj-art-4c259ff4394c493f981c6b03825b6837 |
| institution | Kabale University |
| issn | 2090-6706 2090-6714 |
| language | English |
| publishDate | 2018-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Oncological Medicine |
| spelling | doaj-art-4c259ff4394c493f981c6b03825b68372025-02-03T06:10:49ZengWileyCase Reports in Oncological Medicine2090-67062090-67142018-01-01201810.1155/2018/16847631684763Orbital Desmoid-Type Fibromatosis: A Case Report and Literature ReviewAlessandro Moro0Paolo De Angelis1Giulio Gasparini2Sandro Pelo3Gianluigi Petrone4Emanuela Lucci Cordisco5Umberto Garagiola6Giuseppe D’Amato7Gianmarco Saponaro8Department of Oral and Maxillofacial Surgery, Catholic University of the Sacred Heart Medical School, Rome, ItalyDepartment of Oral and Maxillofacial Surgery, Catholic University of the Sacred Heart Medical School, Rome, ItalyDepartment of Oral and Maxillofacial Surgery, Catholic University of the Sacred Heart Medical School, Rome, ItalyDepartment of Oral and Maxillofacial Surgery, Catholic University of the Sacred Heart Medical School, Rome, ItalyDepartment of Histopathology and Cytopathology, Catholic University of the Sacred Heart Medical School, Rome, ItalyDepartment of Genomic Medicine, Catholic University of the Sacred Heart Medical School, Rome, ItalyDepartment of Orthodontics, University of Milan, Milan, ItalyDepartment of Oral and Maxillofacial Surgery, Catholic University of the Sacred Heart Medical School, Rome, ItalyDepartment of Oral and Maxillofacial Surgery, Catholic University of the Sacred Heart Medical School, Rome, ItalyPurpose. Desmoid-type fibromatosis is a benign fibrous neoplasia originating from connective tissue, fascial planes, and musculoaponeurotic structures of the muscles. Currently, there is no evidence-based treatment approach available for desmoid fibromatosis. In this article, a case of a patient in the pediatric age affected by desmoid fibromatosis localized in the orbit is presented. The aim of the article is to describe this unusual and rare location for the desmoid fibromatosis and outline the principle phases in the decision-making process and the therapeutic alternatives for a patient affected by desmoid fibromatosis. Methods. The protocol of this review included study objectives, search strategy, and selection criteria. The primary end point of this study was to analyze the head and neck desmoid fibromatosis. The secondary end point was to identify the available therapies and assess their specific indications. Results. The mean age of patients was 18.9 years ranging from 0 to 66, and 52% were female. A bimodal age distribution was observed, and two age peaks were identified: 0–14 years (57%) and 28–42 years (18%). The most common involved areas were the mandible (25%) followed by the neck (21%). In 86% of the cases, the treatment was the surgical resection of the disease, and only in 5% of the cases, the surgical resection was followed by adjuvant radiotherapy. Conclusion. The orbital location is extremely rare, especially in the pediatric population. The management of desmoid fibromatosis is based on the function preservation and the maintenance of a good quality of life, but in case of symptomatic patients or aggressive course of the disease or risk of functional damages, the surgical approach may be considered. Therapeutic alternatives to surgical resection are radiotherapy and systemic therapy.http://dx.doi.org/10.1155/2018/1684763 |
| spellingShingle | Alessandro Moro Paolo De Angelis Giulio Gasparini Sandro Pelo Gianluigi Petrone Emanuela Lucci Cordisco Umberto Garagiola Giuseppe D’Amato Gianmarco Saponaro Orbital Desmoid-Type Fibromatosis: A Case Report and Literature Review Case Reports in Oncological Medicine |
| title | Orbital Desmoid-Type Fibromatosis: A Case Report and Literature Review |
| title_full | Orbital Desmoid-Type Fibromatosis: A Case Report and Literature Review |
| title_fullStr | Orbital Desmoid-Type Fibromatosis: A Case Report and Literature Review |
| title_full_unstemmed | Orbital Desmoid-Type Fibromatosis: A Case Report and Literature Review |
| title_short | Orbital Desmoid-Type Fibromatosis: A Case Report and Literature Review |
| title_sort | orbital desmoid type fibromatosis a case report and literature review |
| url | http://dx.doi.org/10.1155/2018/1684763 |
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