Malignant Transformation of Monostotic Fibrous Dysplasia after Surgery: A Case Report
Case Report | Volume 7 | Issue 2 | JBST May- August 2021 | Page 1-4 | Abigail R. Tud, Cesar D. Dimayuga.
DOI:10.13107/jbst.2021.v07i02.46
Author: Abigail R. Tud[1], Cesar D. Dimayuga[1]
[1]Department of Orthopedics, The Medical City, Ortigas Avenue, Pasig City, Philippines 1605.
Address of Correspondence
Dr. Abigail R. Tud,
Musculoskeletal Tumor Unit, Philippine Orthopedic Center, Banawe corner Maria Clara Streets, Santa Mesa Heights, Quezon City, Metro Manila, Philippines 1114.
E-mail: abitud@gmail.com
Abstract
Introduction: Fibrous dysplasia (FD) is a benign lesion characterized by replacement of normal bone with abnormal connective tissue. It occurs in monostotic or polyostotic forms, with a rare but proven potential for malignant transformation. Symptoms of acute pain, rapid swelling, or an enlarging mass should increase suspicion for possible sarcomatous change. Complete surgical resection is the mainstay of treatment, and chemotherapy is recommended to improve survival.
Case Report: A 52-year-old male presented with a painful, enlarging mass on the right proximal thigh 2 years after undergoing plate fixation of the distal femur for a pathologic fracture secondary to monostotic FD. Diagnostic imaging revealed signs of recurrence, and core needle biopsy revealed aggressive features suggestive of malignant transformation. Following surgical resection and chemotherapy, the outcome has been uneventful without evidence of recurrence or metastasis at 4-year post-operation.
Conclusion: Malignant transformation in monostotic FD is rare. Symptom exacerbation should increase the suspicion for sarcomatous change and prompt the need for diagnostic imaging as well as histologic confirmation.
Keywords: Fibrosarcoma, fibrous dysplasia, malignant transformation, secondary sarcoma.
References:
1. Picci P, Sieberova G, Alberghini M, Balladelli A, Vanel D, Hogendoorn PC, et al. Late sarcoma development after curettage and bone grafting of benign bone tumors. Eur J Radiol 2011;77:19-25.
2. Muthusamy S, Conway SA, Subhaeong TY, Temple HT. Locally aggressive fibrous dysplasia mimicking malignancy: A report of four cases and review of the literature. Clin Orthop Relat Res 2015;473:742-50.
3. Qu N, Yao W, Cui X, Zhang H. Malignant transformation in monostotic fibrous dysplasia: Clinical features, imaging features, outcomes in 10 patients, and review. Medicine (Baltimore) 2015;94:e369.
4. Mardekian SK, Tuluc M. Malignant sarcomatous transformation of fibrous dysplasia. Head Neck Pathol 2015;9:100-3.
5. Riddle ND, Bui MM. Fibrous dysplasia. Arch Pathol Lab Med 2013;137:8-21.
6. Ruggieri P, Sim FH, Bond JR, Unni KK. Malignancies in fibrous dysplasia. Cancer 1994;73:1411-24.
7. Chapurlat RD, Gensburger D, Jimenez-Andrade JM, Ghilardi JR, Kelly M, Mantyh P. Pathophysiology and medical treatment of pain in fibrous dysplasia of bone. Orphanet J Rare Dis 2012;7:S3.
8. Doqanavsarqil B, Argin M, Kececi B, Sezak M, Sanli UA, Oztop F. Secondary osteosarcoma arising in fibrous dysplasia, case report. Arch Orthop Trauma Surg 2009;129:439-44.
9. Stanton RP, Ippolito E, Springfield D, Lindaman L, Wientroub S, Leet A. The surgical management of fibrous dysplasia of bone. Orphanet J Rare Dis 2012;7 Suppl 1:S1.
10. Hatano H, Morita T, Arllzumi T, Kawashima H, Ogose A. Malignant transformation of fibrous dysplasia: A case report. Oncol Lett 2014;8:384-6.
11. National Comprehensive Cancer Network (NCCN). Bone Cancer, NCCN Guidelines; 2013. Available from: http://www.nccn.org. [Last accessed on 2016 Mar 16].
| How to Cite this article: Tud AR, Dimayuga CD| Malignant Transformation of Monostotic Fibrous Dysplasia after Surgery: A Case Report | Journal of Bone and Soft Tissue Tumors | May-Aug 2021; 7(2): 1-4. |
