fibrous dysplasia jan m. eckermann, md department of neurosurgery
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Fibrous DysplasiaFibrous Dysplasia
Jan M. Eckermann, MDJan M. Eckermann, MD
Department of NeurosurgeryDepartment of Neurosurgery
DefinitionDefinition
• Benign condition in which normal bone is replaced by fibrous connective tissue due to a defect in osteoblast differentiation and maturation
EpidemiologyEpidemiology
• Incidence not known
• Females > males
• No race predilection
• Initial symptoms manifest age 3-15
• Not heritable
• Questionable genetic transformation
• Malignant transformation in < 1%
VariationsVariations
• Cystic (21%)- Radiolucency surrounded by solid rim
• Sclerotic (23%)- Dense and homogenous
• Mixed (56%)- “Ground glass appearance”
Variations, cont’dVariations, cont’d
• Monostotic- Most common- 25% involve head and neck
• Polyostotic- 15% of cases- 50% involve head and neck
Whereyouat?Whereyouat?
ImagesImages
• Left temporal bone involvement
ImagesImages
• Right temporal bone lesion
ImagesImages
PresentationPresentation
• Local pain
• Swelling
• Abnormal pigmentation
• CN compression
• Spontaneous scalp hemorrhage
• Part of McCune-Albright’s syndrome
McCune-Albright’s SyndromeMcCune-Albright’s Syndrome
• Polyostotic fibrous dysplasia
• Café-au-lait spots
• Endocrinopathology:
- Hyperthyroidism
- Precocious puberty in females
ImagingImaging
• Plain radiography is first line
• Computed tomography for complex regions
HistologyHistology
• Fibroblasts within woven cancellous bone
Whereyouat?Whereyouat?
Differential DiagnosisDifferential Diagnosis
• Eosinophilic granuloma
• Nonossifying fibroma
• Bone hemangioma
• Hyperparathyroidism
• Paget’s disease
• Brown’s tumor
• Aneurysmal bone cyst
TreatmentTreatment
• No available cure
• Curettage
• Cranioplasty
• Calcitonin
The One Slide To RememberThe One Slide To Remember
• Genetic, non-heritable disorder
• <1% transformation to malignancy
• Treatment is curretage or cranioplasty
Whereyouat?Whereyouat?
ReferencesReferences
• 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
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