dysplasia loma linda university medical 骨纤维异常增殖症洛玛连达大学医学课件

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 a