Fibrous Dysplasia

Jan M. Eckermann, MD
Department of Neurosurgery
 Definition
Benign condition in which normal bone is
replaced by fibrous connective tissue due
to a defect in osteoblast differentiation and
maturation
 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, contd
Monostotic
- Most common
- 25% involve head and neck
Polyostotic
- 15% of cases
- 50% involve head and neck
Whereyouat?
 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-Albrights syndrome
 McCune-Albrights Syndrome
Polyostotic fibrous dysplasia
Caf-au-lait spots
Endocrinopathology:
- Hyperthyroidism
- Precocious puberty in females
 Imaging
Plain radiography is first line
Computed tomography for complex
regions
 Histology
Fibroblasts within
woven cancellous
bone
Whereyouat?
 Differential Diagnosis
Eosinophilic granuloma
Nonossifying fibroma
Bone hemangioma
Hyperparathyroidism
Pagets disease
Browns tumor
Aneurysmal bone cyst
 Treatment
No available cure
Curettage
Cranioplasty
Calcitonin
 The One Slide To Remember
Genetic, non-heritable disorder
<1% transformation to malignancy
Treatment is curretage or cranioplasty
Whereyouat?
 References
Greenberg, M. Handbook of Neurosurgery
6th Edition. Thieme: New York 2006
Kaye AH, Black P McL. Operative
Neurosurgery Vol 2. Harcourt Publishing:
New York 2000
Dal Cin P, Sciot R, Spelenberg F, et al.
Chromosome Aberration in Fibrous
Dysplasia. Cancer Genet Cytegent 1994
Oct15;77(2) 114-7