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