1. Chapter 8 Nonneoplastic Diseases of Bone Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 1

2. 2 Outline  Benign Fibro-Osseous Lesions  Paget Disease of Bone  Central Giant Cell Granuloma  Osteomalacia

3. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 3 Nonneoplastic Diseases of Bone These diseases fall into various categories, and several are discussed in other chapters: › Inherited diseases affecting bone: Chapter 6 › Benign and malignant neoplasms: Chapter 7 › Central and peripheral giant cell granulomas: Chapter 2 › Aneurysmal bone cyst: Chapter 5

4. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 4 Benign Fibro-Osseous Lesions Periapical cemento-osseous dysplasia Focal cemento-osseous dysplasia Florid cemento-osseous dysplasia Fibrous dysplasia

5. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 5 Periapical Cemento-Osseous Dysplasia A relatively common disease of unknown cause that affects periapical bone Occurs most commonly in the anterior mandible of patients older than 30 years More common in women than men: Many studies indicate a predilection for black women Early lesions are well circumscribed and radiolucent; with time, they become increasingly calcified Teeth in the affected area are vital

6. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 6 Periapical Cemento- Osseous Dysplasia Diagnosis and treatment  A biopsy may be necessary when characteristic radiographic features are not evident  Histologic examination reveals a fibro- osseous lesion composed of fibrous tissue and calcifications  Early lesions consist of mainly fibrous tissue, whereas older lesions contain numerous calcifications Treatment  None

7. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 7 Florid Cemento-Osseous Dysplasia A condition of disordered cementum and bone development

8. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 8 Florid Cemento-Osseous Dysplasia Clinical and radiographic features  Usually occurs in black women older than 40 years of age  Typically affects more than one quadrant

9. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 9 Florid Cemento-Osseous Dysplasia Masses of irregular opacification are noted that are composed of dense sclerotic bone, cementum, or both

10. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 10 Focal Cemento-Osseous Dysplasia An asymptomatic fibro-osseous lesion Occurs in women between 30 and 50 years of age More common in whites than blacks Occurs in posterior mandible Isolated, well-delineated radiolucent-to- radiopaque lesion Less than 1.5 cm in size

11. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 11 Focal Cemento-Osseous Dysplasia Diagnosis and treatment  Biopsy and histologic examination are usually necessary to establish a diagnosis  Composed of numerous gritty pieces of soft and hard tissue: Fibrous connective tissue interspersed with bone trabeculae and cementum-like material

12. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 12 Fibrous Dysplasia Characterized by replacement of bone with abnormal fibrous connective tissue interspersed with varying amounts of calcification Histologically, it is a benign fibro-osseous lesion, with vascularized, cellular fibrous connective tissue interspersed with irregular trabeculae of bone The cause is unknown  One theory is that it may be due to abnormal mesenchymal cell function

13. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 13 Types of Fibrous Dysplasia Monostotic fibrous dysplasia Polyostotic fibrous dysplasia

14. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 14 Monostotic Fibrous Dysplasia Characterized by involvement of a single bone The maxilla is more frequently involved than the mandible Most commonly diagnosed in children and young adults, with no sex predilection Clinical examination reveals a painless swelling or bulging of the buccal plate

15. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 15 Polyostotic Fibrous Dysplasia  Characterized by involvement of more than one bone  Typically occurs in children, with a female predilection  When long bones are involved, they may exhibit bowing and an associated dull aching pain  Patients may have skin lesions appearing as light- brown macules called café au lait spots

16. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 16 Types of Polyostotic Fibrous Dysplasia  Craniofacial fibrous dysplasia Involves the maxilla with extension into the sinuses and adjacent zygoma, sphenoid, and occipital bones  Jaffe type Involves multiple bones along with café au lait macules on the skin  Albright syndrome Characterized by endocrine abnormalities, precocious puberty in females, stunting or deformity of skeletal growth in both sexes as a result of premature closing of the epiphyseal plates, café au lait spots

17. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 17 Polyostotic Fibrous Dysplasia Typically, a painless, progressive, unilateral enlargement of the mandible or maxilla The classic radiographic appearance is a diffuse radiopacity looking like “ground glass”

18. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 18 Polyostotic Fibrous Dysplasia Characterized by cellular fibrous connective tissue interspersed with irregularly shaped bony trabeculae Treatment  Surgical recontouring of bone for cosmetic reasons

19. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 19 Paget Disease of Bone (Osteitis Deformans)  A chronic metabolic bone disease  Characterized by resorption, osteoblastic repair, and remineralization of involved bone  Unknown cause: May be due to a virus  Most commonly occurs in men over age 50 years  The maxilla is more commonly affected than the mandible

20. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 20 Paget Disease of Bone  Enlargement of affected bone  Patient often complains of pain  Spaces may increase between teeth as jaw bone enlarges

21. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 21 Paget Disease of Bone  Radiographic A patchy radiolucency and radiopacity, “cotton wool” Hypercementosis, loss of lamina dura, and obliteration of the periodontal ligament may occur

22. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 22 Paget Disease of Bone Serum alkaline phosphatase level is significantly elevated in active disease Histologic examination reveals bony trabeculae surfaced with numerous osteoclasts and osteoblasts Treatment  Experimental; the disease is slowly progressive

23. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 23 Central Giant Cell Granuloma Central Giant Cell Lesion Composed of well- vascularized connective tissue containing many multinucleated giant cells Occurs in both peripheral (tissue) and central (bone) locations Brown tumor: Hyperparathyroidism

24. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 24 Central Giant Cell Granuloma Central Giant Cell Lesion May experience pain; not common Slow-growing lesion Destructive Unilocular or multilocular radiolucency Sclerotic or ill-defined borders Divergency of roots Treatment: Surgical excision

25. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 25 Aneurysmal Bone Cyst Pseudocyst Consists of blood-filled spaces surrounded by multinucleated giant cells and fibrous connective tissue Radiographic: “Honeycomb” or “soap bubbles” Treatment: Surgical excision and cryotherapy

26. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 26 Osteomalacia  Result of calcium deficiency and induced by certain tumors  Rickets  Hypophosphatemic vitamin D–resistant rickets  Clinical characteristics Delayed tooth eruption Periodontal disease Pathologic fractures  Treatment Based on identification of the cause

27. Copyright © 2014, 2009, 2004, 2000, 1996, 1992 by Saunders, an imprint of Elsevier Inc 27 Discussion Questions With what disease is “ground glass” appearance associated? What is Paget disease? What are the signs of osteomalacia?