1、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 symptom
2、s manifest age 3-15 Not heritable Questionable genetic transformation Malignant transformation in 1%,Variations,Cystic (21%) Radiolucency surrounded by solid rimSclerotic (23%) Dense and homogenousMixed (56%) “Ground glass appearance”,Variations, contd,Monostotic Most common 25% involve head and nec
3、k 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 Syndro
4、me,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 gran
5、uloma 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 cranioplas
6、ty,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,
