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Fibrous dysplasia

MedGen UID:
120444
•Concept ID:
C0259779
•
Neoplastic Process
Synonym: Fibrous dysplasia of the bones
SNOMED CT: Fibrous dysplasia of bone (10623005); Osteitis fibrosa (10623005); Fibrous dysplasia (789439000); Jaffe-Lichtenstein syndrome (10623005)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
HPO: HP:0010734
Monarch Initiative: MONDO:0000845
Orphanet: ORPHA249

Definition

Tumor-like growths that consist of replacement of the medullary bone with fibrous tissue, causing the expansion and weakening of the areas of bone involved. Especially when involving the skull or facial bones, the lesions can cause externally visible deformities. The skull is often, but not necessarily, affected, and any other bone or bones may be involved. Fibrous dysplasia can either effect isolated bones (Monostotic fibrous dysplasia) or also generalized all bones of the body (Polyostotic fibrous dysplasia). [from HPO]

Professional guidelines

PubMed

The Clinical Spectrum of McCune-Albright Syndrome and Its Management.
Spencer T, Pan KS, Collins MT, Boyce AM
Horm Res Paediatr 2019;92(6):347-356. Epub 2019 Dec 19 doi: 10.1159/000504802. PMID: 31865341Free PMC Article
Best practice management guidelines for fibrous dysplasia/McCune-Albright syndrome: a consensus statement from the FD/MAS international consortium.
Javaid MK, Boyce A, Appelman-Dijkstra N, Ong J, Defabianis P, Offiah A, Arundel P, Shaw N, Pos VD, Underhil A, Portero D, Heral L, Heegaard AM, Masi L, Monsell F, Stanton R, Dijkstra PDS, Brandi ML, Chapurlat R, Hamdy NAT, Collins MT
Orphanet J Rare Dis 2019 Jun 13;14(1):139. doi: 10.1186/s13023-019-1102-9. PMID: 31196103Free PMC Article
Fibrous dysplasia. Pathophysiology, evaluation, and treatment.
DiCaprio MR, Enneking WF
J Bone Joint Surg Am 2005 Aug;87(8):1848-64. doi: 10.2106/JBJS.D.02942. PMID: 16085630

Recent clinical studies

Etiology

Coexistence of craniopharyngioma and cranial fibrous dysplasia: a case series of clinicopathological study.
Fan YH, Li Z
Orphanet J Rare Dis 2022 Mar 18;17(1):126. doi: 10.1186/s13023-022-02281-1. PMID: 35303908Free PMC Article
Best practice management guidelines for fibrous dysplasia/McCune-Albright syndrome: a consensus statement from the FD/MAS international consortium.
Javaid MK, Boyce A, Appelman-Dijkstra N, Ong J, Defabianis P, Offiah A, Arundel P, Shaw N, Pos VD, Underhil A, Portero D, Heral L, Heegaard AM, Masi L, Monsell F, Stanton R, Dijkstra PDS, Brandi ML, Chapurlat R, Hamdy NAT, Collins MT
Orphanet J Rare Dis 2019 Jun 13;14(1):139. doi: 10.1186/s13023-019-1102-9. PMID: 31196103Free PMC Article
Craniofacial fibrous dysplasia.
Hanifi B, Samil KS, Yasar C, Cengiz C, Ercan A, Ramazan D
Clin Imaging 2013 Nov-Dec;37(6):1109-15. Epub 2013 Aug 29 doi: 10.1016/j.clinimag.2013.01.010. PMID: 23993753
Fibrous dysplasia. Pathophysiology, evaluation, and treatment.
DiCaprio MR, Enneking WF
J Bone Joint Surg Am 2005 Aug;87(8):1848-64. doi: 10.2106/JBJS.D.02942. PMID: 16085630
Fibrous dysplasia.
Parekh SG, Donthineni-Rao R, Ricchetti E, Lackman RD
J Am Acad Orthop Surg 2004 Sep-Oct;12(5):305-13. doi: 10.5435/00124635-200409000-00005. PMID: 15469225

Diagnosis

Fibrous Dysplasia/McCune-Albright Syndrome: A Rare, Mosaic Disease of Gα s Activation.
Boyce AM, Collins MT
Endocr Rev 2020 Apr 1;41(2):345-70. doi: 10.1210/endrev/bnz011. PMID: 31673695Free PMC Article
Best practice management guidelines for fibrous dysplasia/McCune-Albright syndrome: a consensus statement from the FD/MAS international consortium.
Javaid MK, Boyce A, Appelman-Dijkstra N, Ong J, Defabianis P, Offiah A, Arundel P, Shaw N, Pos VD, Underhil A, Portero D, Heral L, Heegaard AM, Masi L, Monsell F, Stanton R, Dijkstra PDS, Brandi ML, Chapurlat R, Hamdy NAT, Collins MT
Orphanet J Rare Dis 2019 Jun 13;14(1):139. doi: 10.1186/s13023-019-1102-9. PMID: 31196103Free PMC Article
Fibrous dysplasia of bone: craniofacial and dental implications.
Burke AB, Collins MT, Boyce AM
Oral Dis 2017 Sep;23(6):697-708. Epub 2016 Sep 1 doi: 10.1111/odi.12563. PMID: 27493082Free PMC Article
Fibrous dysplasia.
Riddle ND, Bui MM
Arch Pathol Lab Med 2013 Jan;137(1):134-8. doi: 10.5858/arpa.2012.0013-RS. PMID: 23276185
Fibrous dysplasia. Pathophysiology, evaluation, and treatment.
DiCaprio MR, Enneking WF
J Bone Joint Surg Am 2005 Aug;87(8):1848-64. doi: 10.2106/JBJS.D.02942. PMID: 16085630

Therapy

Efficacy of antiresorptive agents in fibrous dysplasia and McCune Albright syndrome, a systematic review and meta-analysis.
Bertin H, Moussa MS, Komarova S
Rev Endocr Metab Disord 2023 Dec;24(6):1103-1119. Epub 2023 Aug 26 doi: 10.1007/s11154-023-09832-2. PMID: 37632645
Phosphatonins: From Discovery to Therapeutics.
Kritmetapak K, Kumar R
Endocr Pract 2023 Jan;29(1):69-79. Epub 2022 Oct 7 doi: 10.1016/j.eprac.2022.09.007. PMID: 36210014
History of etidronate.
Watts NB, Chesnut CH 3rd, Genant HK, Harris ST, Jackson RD, Licata AA, Miller PD, Mysiw WJ, Richmond B, Valent D
Bone 2020 May;134:115222. Epub 2020 Jan 3 doi: 10.1016/j.bone.2020.115222. PMID: 31911206
Consensus guidelines on the use of bisphosphonate therapy in children and adolescents.
Simm PJ, Biggin A, Zacharin MR, Rodda CP, Tham E, Siafarikas A, Jefferies C, Hofman PL, Jensen DE, Woodhead H, Brown J, Wheeler BJ, Brookes D, Lafferty A, Munns CF; APEG Bone Mineral Working Group
J Paediatr Child Health 2018 Mar;54(3):223-233. doi: 10.1111/jpc.13768. PMID: 29504223
Fibrous dysplasia. Pathophysiology, evaluation, and treatment.
DiCaprio MR, Enneking WF
J Bone Joint Surg Am 2005 Aug;87(8):1848-64. doi: 10.2106/JBJS.D.02942. PMID: 16085630

Prognosis

The Clinical Spectrum of McCune-Albright Syndrome and Its Management.
Spencer T, Pan KS, Collins MT, Boyce AM
Horm Res Paediatr 2019;92(6):347-356. Epub 2019 Dec 19 doi: 10.1159/000504802. PMID: 31865341Free PMC Article
Fibrous dysplasia.
Riddle ND, Bui MM
Arch Pathol Lab Med 2013 Jan;137(1):134-8. doi: 10.5858/arpa.2012.0013-RS. PMID: 23276185
Craniofacial fibrous dysplasia.
Ricalde P, Magliocca KR, Lee JS
Oral Maxillofac Surg Clin North Am 2012 Aug;24(3):427-41. Epub 2012 Jul 6 doi: 10.1016/j.coms.2012.05.004. PMID: 22771278
Fibrous dysplasia.
Parekh SG, Donthineni-Rao R, Ricchetti E, Lackman RD
J Am Acad Orthop Surg 2004 Sep-Oct;12(5):305-13. doi: 10.5435/00124635-200409000-00005. PMID: 15469225
Fibrous dysplasia.
Stanton RP, Montgomery BE
Orthopedics 1996 Aug;19(8):679-85. doi: 10.3928/0147-7447-19960801-14. PMID: 8856779

Clinical prediction guides

Different clinical presentation and management of temporal bone fibrous dysplasia in children.
Mierzwiński J, Kosowska J, Tyra J, Haber K, Drela M, Paczkowski D, Burduk P
World J Surg Oncol 2018 Jan 15;16(1):5. doi: 10.1186/s12957-017-1302-5. PMID: 29335001Free PMC Article
Craniofacial fibrous dysplasia.
Hanifi B, Samil KS, Yasar C, Cengiz C, Ercan A, Ramazan D
Clin Imaging 2013 Nov-Dec;37(6):1109-15. Epub 2013 Aug 29 doi: 10.1016/j.clinimag.2013.01.010. PMID: 23993753
Fibrous dysplasia.
Lietman SA, Levine MA
Pediatr Endocrinol Rev 2013 Jun;10 Suppl 2:389-96. PMID: 23858622
Craniofacial fibrous dysplasia.
Ricalde P, Magliocca KR, Lee JS
Oral Maxillofac Surg Clin North Am 2012 Aug;24(3):427-41. Epub 2012 Jul 6 doi: 10.1016/j.coms.2012.05.004. PMID: 22771278
Fibrous dysplasia. Pathophysiology, evaluation, and treatment.
DiCaprio MR, Enneking WF
J Bone Joint Surg Am 2005 Aug;87(8):1848-64. doi: 10.2106/JBJS.D.02942. PMID: 16085630

Recent systematic reviews

Craniofacial fibrous dysplasia: Systematic review of facial management.
Bouet B, Schlund M, De Massary M, Nicot R
J Stomatol Oral Maxillofac Surg 2023 Dec;124(6S2):101660. Epub 2023 Oct 20 doi: 10.1016/j.jormas.2023.101660. PMID: 37866506
Efficacy of antiresorptive agents in fibrous dysplasia and McCune Albright syndrome, a systematic review and meta-analysis.
Bertin H, Moussa MS, Komarova S
Rev Endocr Metab Disord 2023 Dec;24(6):1103-1119. Epub 2023 Aug 26 doi: 10.1007/s11154-023-09832-2. PMID: 37632645
Surgical management of syndromic versus non-syndromic craniofacial fibrous dysplasia: a systematic review and meta-analysis.
Dalle Carbonare M, Manisali M
Br J Oral Maxillofac Surg 2022 Nov;60(9):1166-1175. Epub 2022 Jun 16 doi: 10.1016/j.bjoms.2022.06.002. PMID: 35817637
Prevalence of Different Forms and Involved Bones of Craniofacial Fibrous Dysplasia.
Yang L, Wu H, Lu J, Teng L
J Craniofac Surg 2017 Jan;28(1):21-25. doi: 10.1097/SCS.0000000000002830. PMID: 27922965
Fibrous dysplasia of the temporal bone: systematic review of management and hearing outcomes.
Fandiño M, Bhimrao SK, Saxby AJ, Dar Santos RC, Westerberg BD
Otol Neurotol 2014 Dec;35(10):1698-706. doi: 10.1097/MAO.0000000000000602. PMID: 25280053

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