Secondary desmoplastic fibroma-like tissue changes in mandibular fibrous dysplasia: clinicopathological and molecular study of a case. Issue 1 (January 2020)

• Record Type: Journal Article
• Authors: Corsi, A.; Fadda, M.T.; Terenzi, V.; Valentini, V.; Riminucci, M.
• Is Part Of: British journal of oral and maxillofacial surgery. Volume 58:Issue 1(2020)
• Page Start: 96
• Page End: 98
• Publication Date: 2020-01
• Subjects: Fibrous Dysplasia -- Desmoplastic Fibroma -- Low-Grade Osteosarcoma -- mandible -- GNAS; Mouth -- Surgery -- Periodicals; Maxilla -- Surgery -- Periodicals; Face -- Surgery -- Periodicals; Surgery, Plastic -- Periodicals; Dentistry, Operative -- Periodicals; Face -- surgery -- Periodicals; Mouth -- surgery -- Periodicals; Maxilla -- surgery -- Periodicals; Surgery, Oral -- Periodicals; Oral Surgical Procedures -- Periodicals; Dentistry, Operative -- Periodicals; Bouche -- Chirurgie -- Périodiques; Maxillaire supérieur -- Chirurgie -- Périodiques; Face -- Chirurgie -- Périodiques; Chirurgie dentaire -- Périodiques; Dentistry, Operative; Face -- Surgery; Maxilla -- Surgery; Mouth -- Surgery; Surgery, Plastic; Electronic journals; Periodicals; 617.52059
• DOI: 10.1016/j.bjoms.2019.09.006
• Languages: English
• ISSNs: 0266-4356

Abstract

Abstract: Fibrous dysplasia may show locally aggressive behaviour reflecting secondary intralesional changes, extension to soft tissue, or malignant transformation. We report the case of a patient with polyostotic fibrous dysplasia who had a giant mandibular lesion consisting of histologically typical, genotypically-confirmed, fibrous dysplasia merged with a fibrotic and hypocellular desmoplastic fibroma-like tissue in which the same Gsα-R201H mutation was detected. The occurrence of the same mutation in both the fibrous dysplasia and areas of desmoplastic fibroma suggests that the fibroma-like tissue reflects an unusual secondary tissue change within an otherwise typical fibrous dysplasia. To the best of our knowledge, only four cases of fibrous dysplasia with desmoplastic fibroma-like tissue changes have been reported.