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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

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fibrous dysplasia

Fibrous Dysplasia

Jan M. Eckermann, MD

Department of Neurosurgery

definition
Definition
  • Benign condition in which normal bone is replaced by fibrous connective tissue due to a defect in osteoblast differentiation and maturation
epidemiology
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
Variations
  • Cystic (21%)
  • Radiolucency surrounded by solid rim
  • Sclerotic (23%)
  • Dense and homogenous
  • Mixed (56%)
  • “Ground glass appearance”
variations cont d
Variations, cont’d
  • Monostotic
  • Most common
  • 25% involve head and neck
  • Polyostotic
  • 15% of cases
  • 50% involve head and neck
images
Images
  • Left temporal bone involvement
images8
Images
  • Right temporal bone lesion
presentation
Presentation
  • Local pain
  • Swelling
  • Abnormal pigmentation
  • CN compression
  • Spontaneous scalp hemorrhage
  • Part of McCune-Albright’s syndrome
mccune albright s syndrome
McCune-Albright’s Syndrome
  • Polyostotic fibrous dysplasia
  • Café-au-lait spots
  • Endocrinopathology:
  • Hyperthyroidism
  • Precocious puberty in females
imaging
Imaging
  • Plain radiography is first line
  • Computed tomography for complex regions
histology
Histology
  • Fibroblasts within woven cancellous bone
differential diagnosis
Differential Diagnosis
  • Eosinophilic granuloma
  • Nonossifying fibroma
  • Bone hemangioma
  • Hyperparathyroidism
  • Paget’s disease
  • Brown’s tumor
  • Aneurysmal bone cyst
treatment
Treatment
  • No available cure
  • Curettage
  • Cranioplasty
  • Calcitonin
the one slide to remember
The One Slide To Remember
  • Genetic, non-heritable disorder
  • <1% transformation to malignancy
  • Treatment is curretage or cranioplasty
references
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