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Fibrous Dysplasia

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

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  1. Fibrous Dysplasia Jan M. Eckermann, MD Department of Neurosurgery

  2. Definition • Benign condition in which normal bone is replaced by fibrous connective tissue due to a defect in osteoblast differentiation and maturation

  3. Epidemiology • Incidence not known • Females > males • No race predilection • Initial symptoms manifest age 3-15 • Not heritable • Questionable genetic transformation • Malignant transformation in < 1%

  4. Variations • Cystic (21%) • Radiolucency surrounded by solid rim • Sclerotic (23%) • Dense and homogenous • Mixed (56%) • “Ground glass appearance”

  5. Variations, cont’d • Monostotic • Most common • 25% involve head and neck • Polyostotic • 15% of cases • 50% involve head and neck

  6. Whereyouat?

  7. Images • Left temporal bone involvement

  8. Images • Right temporal bone lesion

  9. Images

  10. Presentation • Local pain • Swelling • Abnormal pigmentation • CN compression • Spontaneous scalp hemorrhage • Part of McCune-Albright’s syndrome

  11. McCune-Albright’s Syndrome • Polyostotic fibrous dysplasia • Café-au-lait spots • Endocrinopathology: • Hyperthyroidism • Precocious puberty in females

  12. Imaging • Plain radiography is first line • Computed tomography for complex regions

  13. Histology • Fibroblasts within woven cancellous bone

  14. Whereyouat?

  15. Differential Diagnosis • Eosinophilic granuloma • Nonossifying fibroma • Bone hemangioma • Hyperparathyroidism • Paget’s disease • Brown’s tumor • Aneurysmal bone cyst

  16. Treatment • No available cure • Curettage • Cranioplasty • Calcitonin

  17. The One Slide To Remember • Genetic, non-heritable disorder • <1% transformation to malignancy • Treatment is curretage or cranioplasty

  18. Whereyouat?

  19. 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

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