“The many faces of fibrous dysplasia: a single-center experience”

Natali Sideri, Vasiliki Siachou, Dimitrios Fillipiadis, Olga Savvidou, Olympia Papakonstantinou

Abstract


Purpose: To present a single-center experience on imaging features of fibrous dysplasia (FD). 

Materials and methods: A 10-year database overview in our department identified 20 patients with FD, aged 13-74 years. X-rays were available in 11, Computed Tomography (CT) in 12 and Magnetic Resonance Imaging (MRI) in 8 patients. FD lesions were evaluated for their number, distribution and morphology, by two experienced musculoskeletal radiologists.

Results: Eleven (11) patients exhibited monostotic type of FD (M-FD), four (4) patients polyostotic type (P-FD) and five (5) patients craniofacial FD (CF-FD), accounting for totally 70 lesions, mostly asymptomatic. Two P-FD patients had a history of McCune-Albright Syndrome (MAS). Radiographic appearance of lesions varied from ground glass (n=61) to mixed lucent/sclerotic (n=4) or completely lucent (n=5) with or without septations, whereas most lesions (n=58) appeared expansile. The classic “rind sign” was present in seven (7) M-FD lesions and in only three (3) lesions of the P-FD type, while bone deformity was not uncommon, particularly in P-FD lesions. MR imaging findings were not specific for FD. Malignant change was seen in one P-FD patient whereas co-existent exostoses were present in another P-FD case. Fractures complicated three P-FD lesions.

Conclusions: FD is usually an incidental finding, demonstrating typical radiographic characteristics, meeting the “Do-not-touch-lesion” criteria. However, atypical findings, mimicking malignancy, are not uncommon. Detailed knowledge of the varied radiological features allows narrowing the differential diagnosis. Comorbidities are common in the P-FD type and should be excluded by workup.


Keywords


Fibrous Dysplasia; Imaging; Do-not-touch lesion; Mc-Cune Albright syndrome.

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DOI: http://dx.doi.org/10.36162/hjr.v7i3.508

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