Fibrous Dysplasia, Osteofibrous Dysplasia, and Adamantinoma
• Monostotic fibrous dysplasia is more common than polyostotic fibrous dysplasia.
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Contents
K ey P oi n t s 23.1
Introduction 411
23.2 23.2.1 23.2.2 23.2.2.1 23.2.2.2 23.2.2.3 23.2.2.4 23.2.2.5
Fibrous Dysplasia 412 Pathology 412 Manifestations 412 Monostotic Fibrous Dysplasia 412 Polyostotic Fibrous Dysplasia 413 McCune-Albright Syndrome 413 Mazabraud’s Syndrome 413 Malignancy in Fibrous Dysplasia 413 Low-Grade Central Osteosarcoma Mimicking Fibrous Dysplasia 414 Cystic Fibrous Dysplasia 416 Fibrous Dysplasia Protuberans 416 Imaging Characteristics 416 Long Bones 417 Craniofacial Bones 417 Additional Skeletal Locations 418 Other Imaging Modalities 418 Treatment and Follow-up 419
23.2.2.6 23.2.2.7 23.2.2.8 23.2.3 23.2.3.1 23.2.3.2 23.2.3.3 23.2.3.4 23.2.4
23.3 Osteofibrous Dysplasia 420 23.3.1 Pathology 420 23.3.2 Incidence and Clinical Presentation 420 23.3.3 Imaging Characteristics 420 23.3.4 Treatment and Follow-up 420 23.4 23.4.1 23.4.2 23.4.3 23.4.4 23.4.5
Adamantinoma 421 Pathology 421 Incidence and Clinical Presentation 421 Imaging Characteristics 421 Differentiated Adamantinoma 421 Treatment and Follow-up 422 References 423
S. Mannava, MD Diagnostic Radiology/Hb6, The Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland OH 44195, USA M. Sundaram, MD, FRCR Section of Musculoskeletal Radiology, Imaging Institute, Cleveland Clinic, Professor of Radiology, Cleveland Clinic Lerner School of Medicine, of Case Western Reserve University
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Monostotic fibrous dysplasia is more common than polyostotic fibrous dysplasia. Fibrous dysplasia can be encountered in any decade of life. Endocrine dysfunction and soft tissue myxoma are associated with fibrous dysplasia. A fibrous dysplasia-like lesion with cortical destruction suggests low-grade central osteosarcoma or malignant transformation. Osteofibrous dysplasia usually occurs in the first two decades of life. Osteofibrous dysplasia almost exclusively affects the tibia and fibula. Adamantinoma is a rare low-grade neoplasm that most commonly occurs in the second and third decades of life. Adamantinoma has a strong predilection for the tibia.
23.1
Introduction Fibrous dysplasia is a benign disorder of bone. This fibroosseous lesion may affect one or several bones and may be encountered in any decade of life. Monostotic fibrous dysplasia is significantly more common than polyostotic disease. Rarely, endocrine dysfunction (i.e. McCune-Albright syndrome) or soft tissue myxoma (i.e. Mazabraud’s syndrome) can be associated with fibrous dysplasia. A fibrous dysplasia-like lesion with radiographic features of cortical destruction suggests low-grade central osteosarcoma or malignant transformation. Osteofibrous dysplasia is a fibroosseous lesion with many pathologic features similar to those of fibrous dysplasia. Osteofibrous dysplasia is usually seen
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S. Mannava and M. Sundaram
in the first two decades of life and almost exclusively affects the tibia and fibula. The lesion characteristically and distinctively involves the cortex as multiple lytic lesions. Adamantinoma is a r
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