Learning objectives
To present a spectrum of varied,
typical and atypical,
imaging features of both monostotic and polyostotic form of Fibrous Dysplasia (FD),
with special interest in the differential diagnosis.
Background
Introduction
FD is a rare,
congenital,
non-heritable entity,
regarding benign intramedullary abnormalities either presenting as an isolated skeletal finding (monostotic type,
M-FD),
accounting for 70-80% of cases,
or affecting multiple bones (polyostotic type,
P-FD).
P-FD can occasionally exhibit a monomelic pattern.
The ribs are one of the most common sites of M-FD,
while the femur and tibia are most commonly involved in P-FD.
Craniofacial FD affects only the skull and facial bones,
with the frontal,
sphenoid,
ethmoid,
orbit,
zygoma,
maxilla,
mandible,
and temporal bones being...
Imaging findings OR Procedure Details
A retrospective review of 15 patients with FD and 1 patient with OFD was conducted.
Radiographs were available in 11 patients,
MRI in 5 patients and CT in 3 patients.
M-FD was found in 11 patients,
involving the pelvic bones (3 patients),
the humerus (2 patients),
the femur (2 patients),
the fascial bones (2 patients),
the tibia (1 patient),
and the ribs (1 patient) [Fig.
1,
2,
3,
4].
P-FD was found in 4 patients.
In one of them,
the ipsilateral femur and tibia were...
Conclusion
FD is usually asymptomatic and can appear as an incidental finding with typical radiographic features.
Though benign as a rule,
it can rarely display locally aggressive behavior,
mimicking malignancy.
Comorbidities are common in P-FD and should be excluded by the work-up process.
References
1.
Helena Florez,
Pilar Peris,
Núria Guañabens.
Fibrous Dysplasia: Clinical review and therapeutic management.
Medical Clinics (Barcelona),
2017 (article in press).
2.
Hideo Moriokaa,
Yasuhiro Kamataa,
Kazumasa Nishimotoa,
Michiro Susab,
Kazutaka Kikutaa,
Keisuke Horiuchia,
Aya Sasakic,
Kaori Kameyamac,
Masaya Nakamuraa,
Morio Matsumotoa.
Fibrous Dysplasia with Massive Cartilaginous Differentiation (Fibrocartilaginous Dysplasia) in the Proximal Femur: A Case Report and Review of the Literature.
Case Reports in Oncology,
2016; 9:126–133 (DOI: 10.1159/000443476).
3.
Mehmet Haydar Atalar,
Ismail Salk,
Recep Savas,
Ismail Onder Uysal,
Hulusi Egilmez.
CT and...