Fibrous bone dysplasia: A case report

Published

2024-08-30

How to Cite

Bautista Litardo, N., & Peralta Rodríguez, R. (2024). Fibrous bone dysplasia: A case report. Oncology Journal (Ecuador), 34(2), 62–67. https://doi.org/10.33821/730

Issue

Section

Case Reports

Authors

DOI:

https://doi.org/10.33821/730

Keywords:

Fibrous dysplasia of bone, monostotic, maxillectomy, zoledronic acid

Abstract

Introduction: Fibrous bone dysplasia is a bone disease that affects the normal composition of the bone, in any part of the skeletal system, whether it is monostotic or polyostic, causing tumor masses of fibrous connective tissue. At the same time, it encompasses a wide phenotypic spectrum that can vary depending on the age of onset and the affected apparatus, such as endocrinopathies. Regarding its diagnosis, if it only affects a single bone without any other finding, it would only need histopathological confirmation. For its treatment could be surgery, antiresorptive drugs such as zolendronic acid, immunological medications and pain management if required. Clinical Case: A 14-year-old boy presents with facial asymmetry in the Internal Medicine department. Diagnostic workshop: In computed axial tomography of the skull, an expansive sclerotic lesion with ground glass density is evident at the level of the upper jaw and right zygomatic region. The pathology report of a biopsy taken confirmed the diagnosis of fibrous bone dysplasia. Conclusion: This type of dysplasia is rare, and may have a non-progressive asymptomatic course, or cosmetic changes and exacerbating pain in the progressive stage, which poses a challenge for the patient’s diagnosis. Several factors must be taken into consideration to choose the best treatment for the patient. For this reason, it should be studied early for an early therapeutic decision.

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Author Biographies

Noemi Bautista Litardo, Sociedad de Lucha Contra el Cáncer del Ecuador

Jefa del servicio de Endocrinología, SOLCA–Guayaquil, Ecuador

Raúl Peralta Rodríguez, Sociedad de Lucha Contra el Cáncer del Ecuador

Servicio de Medicina Interna, SOLCA. Guayaquil, Ecuador.

References

Javaid MK, Boyce A, Appelman-Dijkstra N, Defabianis P, Offiah A, Arundel P, et al. Best practice management guidelines for fibrous dysplasia/McCune-Albright syndrome: A consensus statement from the FD/MAS international consortium. Orphanet J Rare Dis. 2019;14(1): 139. https://doi.org/10.1186/s13023-019-1102-9

Samieirad S, Momtaz MM, Mohtasham N, Mohammadzadeh F, Ebrahimzadeh N, Tohidi E. Surgical treatment of fibrous dysplasia in the maxillary bone of a 12 year-old girl: A case report. World J Plast Surg. 2021;10(3): 126-33. https://doi.org/10.52547/wjps.10.3.126

Saber A, Patel B. Osteofibrous Dysplasia [Internet]. NCBI Bookshelf; 2023. Disponible en: https://www.ncbi.nlm.nih.gov/books/NBK563281/?report=printable

Diyora B, Dey S, Dubey A, Lakdawala L. Cranial fibrous dysplasia: An institutional experience and review of the literature. Surg Neurol Int. 2022;13: 66. https://doi.org/10.25259/SNI_1218_2021

Kim DY. Current concepts of craniofacial fibrous dysplasia: Pathophysiology and treatment. Arch Craniofacial Surg. 2023;24(2): 41-51. https://doi.org/10.7181/acfs.2023.00101

Spencer T, Pan KS, Collins MT, Boyce AM. The clinical spectrum of McCune-Albright syndrome and its management. Horm Res Paediatr. 2019;92(6): 347-56. https://doi.org/10.1159/000504802

Jiménez C, Schneider P, Baudrand R, García H, Martínez A, Mendoza C, et al. Clinical features of Chilean patients with Fibrous Dysplasia/McCune-Albright Syndrome. Rev Médica Chile. 2022;150: 1275-82. https://doi.org/10.4067/S0034-98872022001001275

Zambrano A, Zambrano W, Orellana D, Fernández J. Fibrous Dysplasia, 6-year review. Rev Cienc Av. 2022;1(2): 1-6.

Hartley I, Zhadina M, Collins MT, Boyce AM. Fibrous Dysplasia of Bone and McCune-Albright Syndrome: A Bench to Bedside Review. Calcif Tissue Int. 2019;104(5): 517-29. https://doi.org/10.1007/s00223-019-00550-z

Anitha N, Leena S, Malathi L. Fibrous dysplasia recent concepts. J Pharm Bioallied Sci. 2015;7(Supplement 1): 171-2. https://doi.org/10.4103/0975-7406.155892

Zhadina M, Roszko KL, Geels RES, De Castro LF, Collins MT, Boyce AM. Genotype-Phenotype Correlation in Fibrous Dysplasia/McCune-Albright Syndrome. J Clin Endocrinol Metab. 2021;106(5): 1482-90. https://doi.org/10.1210/clinem/dgab053

Boyce A, Collins MT. Fibrous dysplasia/McCune-Albright syndrome: A rare, mosaic disease of G?s activation. Oxford University Press on behalf of the Endocrine Society 2019; 2019. https://doi.org/10.1210/endrev/bnz011

Lopez-Garibay LA, Guevara-Valmaña O, Telich-Tarriba JE, Navarro-Barquín DF, Haro-Alvarez N, Andrade-Delgado L, et al. Craniofacial Fibrous Dysplasia: Surgical Management and Long-Term Outcomes at a Referral Center in Mexico City. Indian J Plast Surg. 2023;56(02):124-9. https://doi.org/10.1055/s-0042-1760251

Valadares LP, Ferreira BSDA, Cunha BMD, Moreira LA, Batista FGA, Hottz CDF, et al. Effects of zoledronic acid therapy in fibrous dysplasia of bone: A single-center experience. Arch Endocrinol Metab. https://doi.org/10.20945/2359-3997000000459

Rotman M, Hamdy NAT, Appelman?Dijkstra NM. Clinical and translational pharmacological aspects of the management of fibrous dysplasia of bone. Br J Clin Pharmacol. 2019;85(6): 1169-79. https://doi.org/10.1111/bcp.13820

Chapurlat R, Legrand MA. Bisphosphonates for the treatment of fibrous dysplasia of bone. Bone. 2021;143: 115784. https://doi.org/10.1016/j.bone.2020.115784

Chattopadhyay A, Jain S, Sharma A. Craniofacial Fibrous Dysplasia. JCR J Clin Rheumatol. 2019;1(1):1-2.

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