Secondary desmoplastic fibroma-like tissue changes in mandibular fibrous dysplasia: clinicopathological and molecular study of a case

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, genoty...

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Bibliographic Details
Published in:British journal of oral & maxillofacial surgery 2020-01, Vol.58 (1), p.96-98
Main Authors: Corsi, A., Fadda, M.T., Terenzi, V., Valentini, V., Riminucci, M.
Format: Article
Language:English
Subjects:
Bone Neoplasms
Desmoplastic Fibroma
Fibroma
Fibroma, Desmoplastic
Fibrous Dysplasia
GNAS
GTP-Binding Protein alpha Subunits, Gs - genetics
Humans
Low-Grade Osteosarcoma
mandible
Mutation
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Summary: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.
ISSN:0266-4356
1532-1940
DOI:10.1016/j.bjoms.2019.09.006