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