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.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to British Journal of Oral and Maxillofacial SurgeryAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- WHO classification of tumours of soft tissue and bone.4th ed. IARC Press, Lyon2013
- Diagnostic value of investigating GNAS mutations in fibro-osseous lesions: a retrospective study of 91 cases of fibrous dysplasia and 40 other fibro-osseous lesions.Mod Pathol. 2013; 26: 911-921
- Gsalpha gene mutations in monostotic fibrous dysplasia of bone and fibrous dysplasia-like low-grade central osteosarcoma.Virchows Arch. 2001; 439: 170-175
- Activating GNAS mutations in parosteal osteosarcoma.Am J Surg Pathol. 2014; 38: 402-409
- GNAS mutations in fibrous dysplasia: a comparative study of standard sequencing and locked nucleic acid PCR sequencing on decalcified and nondecalcified formalin-fixed paraffin-embedded tissues.Appl Immunohistochem Mol Morphol. 2016; 24: 660-667
- Clinical guidelines for the management of craniofacial fibrous dysplasia.Orphanet J Rare Dis. 2012; 7: S2
- Our experience in the surgical management of craniofacial fibrous dysplasia: what has changed in the last 10 years?.Acta Otorhinolaryngol Ital. 2017; 37: 436-443
- Desmoplastic fibroma of bone arising in fibrous dysplasia.Am J Clin Pathol. 1983; 79: 630-633
- Desmoplastic fibroma arising in fibrous dysplasia. Chromosomal analysis and review of the literature.Clin Orthop Relat Res. 1989; 247: 272-278
- Monostotic fibrous dysplasia of the proximal femur and liposclerosing myxofibrous tumor: which one is which?.J Bone Miner Res. 2006; 21: 1955-1958
Article info
Publication history
Published online: September 26, 2019
Accepted:
September 9,
2019
Identification
Copyright
© 2019 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
