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Fibrous Dysplasia

By; Rukundo Austin


Definition
Fibrous dysplasia is a benign condition in
which normal bone is replaced by fibrous
connective tissue due to a defect in
osteoblast differentiation and maturation.
The normal medullary space of the affected
bone is replaced by fibrous tissue. It is a
benign skeletal disorder affecting one or
multiple bones (monostotic or polyostotic
fibrous dysplasia (MFD and PFD
respectively).
Epidemiology
Incidence not known
Females > males
No race predilection
Initial symptoms manifest age 3-15
Not heritable
Questionable genetic transformation
Malignant transformation in < 1%
Variations
• Cystic (21%)
- Radiolucency surrounded by solid rim

• Sclerotic (23%)
- Dense and homogenous

• Mixed (56%)
- “Ground glass appearance”
Variations, cont’d
• Monostotic
- Most common
- 25% involve head and neck
• Polyostotic
- 15% of cases
- 50% involve head and neck
Con…………
• From one-third to one-half of patients
affected with polyostotic fibrous
dysplasia (PFD) present yellowish or
brownish non-elevated patches of
cutaneous pigmentation (cafe-au-lait
spots), situated predominantly on the
trunk
Images
• Left temporal bone
involvement
Images
• Right temporal bone
lesion
Images
Presentation
• Local pain, Swelling
• Abnormal pigmentation
• CN compression
• Spontaneous scalp hemorrhage
• Part of McCune-Albright’s syndrome
Cont………………..
Multiple endocrine disorders have been
described in association with fibrous
dysplasia. Of these, the best known is
McCune-Albright syndrome, which
consists of the triad of polyostotic
fibrous dysplasia (typically unilateral),
cutaneous cafe-au-lait spots (usually
ipsilateral to the bone lesions) , and
endocrine dysfunction(especially
precocious puberty in girls)
McCune-Albright’s Syndrome
• Polyostotic fibrous dysplasia
• Café-au-lait spots
• Endocrinopathology:
• Hyperthyroidism, hyperparathynoidism,
acromegaly, diabetes mellitus, and
Cushing syndrome
- Precocious puberty in females
Imaging
• Plain radiography is first line
• Computed tomography for complex
regions and Magnetic resonance imaging
Axial contrast-enhanced CT image obtained before thoracentesis
shows a large hemothorax and lytic expansile lesions in the left 6th and
7th ribs (arrows). The expansile 6th rib mass (*) demonstrates
heterogeneous attenuation and compresses the left lung.
Coronal contrast-enhanced CT image shows extensive
expansion of the lateral 6th rib mass (*) and compression
of the left lung.
Plain chest radiography
• Intraoral periapical
radiograph showing
diffuse mixed lesion
and ground glass
appearance of
trabeculae
• Axial computed
tomography shows
myxematous, diffuse
involvement of
multiple bones
Histology
• Fibroblasts within
woven cancellous
bone
Differential Diagnosis
• Eosinophilic granuloma
• Nonossifying fibroma
• Bone hemangioma
• Hyperparathyroidism
• Paget’s disease
• Brown’s tumor
• Aneurysmal bone cyst
Treatment
• No available cure
• Curettage
• Cranioplasty
• Calcitonin
NOTE
• Genetic, non-heritable disorder
• <1% transformation to malignancy
• Treatment is curretage or cranioplasty
References
Greenberg, M. Handbook of Neurosurgery
6th Edition. Thieme: New York 2006
Aberration in Fibrous Dysplasia. Cancer
Genet Cytegent 1994 Oct15;77(2) 114-7
Radiopaedia
http://www.jofs.in/article.asp?issn=0975-
8844;year=2013;volume=5;issue=1;spage
=67;epage=70;aulast=Diwan
•THE END
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