Nonneoplastic Diseases of Bone Chapter 8 Nonneoplastic Diseases of Bone

Outline Benign Fibro-osseous Lesions Paget Disease of Bone Central Giant Cell Granuloma Osteomalacia

Nonneoplastic Diseases of Bone (pg. 277) (Box 8-1) These diseases fall into several categories. Several are discussed in other chapters. Inherited diseases affecting bone – Ch 6 Benign and malignant neoplasms – Ch 7 Central and peripheral giant cell granulomas – Ch 2 Aneurysmal bone cyst – Ch 5 This chapter covers several other nonneoplastic diseases not included elsewhere.

Benign Fibro-osseous Lesions Periapical Cemento-osseous Dysplasia Focal Cemento-osseous Dysplasia Florid Cemento-osseous Dysplasia Fibrous Dysplasia

Periapical Cemento-osseous Dysplasia (pgs. 277-278) A relatively common disease of unknown cause that affects periapical bone Clinical and radiographic features Discovered on radiographs Occurs most commonly in the anterior mandible of patients older than 30 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. Older lesions may be radiolucent with central opacifications.

Periapical Cemento-osseous Dysplasia (cont.)

Periapical Cemento-osseous Dysplasia (cont.) (pgs. 277-278) Diagnosis and treatment A biopsy may be necessary in cases where 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, where older lesions contain numerous calcifications. Treatment None

Periapical Cemento-osseous Dysplasia (cont.)

Focal Cemento-osseous Dysplasia (cont.) (pgs. 277-278) An asymptomatic fibro-osseous lesion The histologic features are similar to periapical cemento-osseous dysplasia and florid cemento-osseous dysplasia, but it has unique clinical and radiographic features.

Focal Cemento-osseous Dysplasia (cont.) Clinical and radiographic features Usually occurs in women between 30 and 50 years of age More common in white than in black individuals Typically arises in the posterior mandible Appears as an isolated, well-delineated radiolucent to radiopaque lesion less than 1.5 cm

Focal Cemento-osseous Dysplasia (cont.) Diagnosis and Treatment Biopsy and histologic examination are usually necessary to establish a diagnosis, A surgical feature is that it is composed of numerous gritty pieces of soft and hard tissue.

Florid Cemento-osseous Dysplasia (pgs. 278-279) A condition of disordered cementum and bone development Characteristically involves multiple quadrants in the maxilla and mandible.

Florid Cemento-osseous Dysplasia (cont.) (pgs. 278-279) Clinical and Radiographic Features Occurs most often in middle-aged black women who are older than 49 It typically affects more than one quadrant of the maxilla and mandible, often in posterior areas. Masses of irregular opacification are noted that are composed of dense, sclerotic bone, cementum, or both.

Florid Cemento-osseous Dysplasia (cont.)

Florid Cemento-osseous Dysplasia (cont.) Diagnosis and Treatment Often diagnosed based on characteristic clinical presentation and radiographic appearance In an edentulous patient, the sclerotic masses may perforate the mucosa. This may lead to osteomyelitis, requiring surgery and antibiotics.

Fibrous Dysplasia (pg. 279) A disease characterized by replacement of bone with abnormal fibrous connective tissue interspersed with varying amounts of calcification The cause is unknown. One theory is it may be due to abnormal mesenchymal cell function. Histologically, it is a benign fibro-osseous lesion, with vascularized, cellular fibrous connective tissue interspersed with irregular trabeculae of bone.

Types of Fibrous Dysplasia Monostotic fibrous dysplasia Polyostotic fibrous dysplasia

Monostotic Fibrous Dysplasia (pg. 279) 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.

Polyostotic Fibrous Dysplasia (pgs. 279-280) 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.

Polyostotic Fibrous Dysplasia (cont.) There are several types 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

Polyostotic Fibrous Dysplasia (cont.) (pgs. 279-280) Clinical and radiographic features A painless enlargement of affected bone or bones Typically, a painless, progressive, unilateral enlargement of the mandible or maxilla. The classic radiographic appearance is a diffuse radiopacity looking like “ground glass.”

Polyostotic Fibrous Dysplasia (cont.)

Polyostotic Fibrous Dysplasia (cont.) (pgs. 279-280) Diagnosis and treatment Characterized by cellular fibrous connective tissue interspersed with irregularly shaped bony trabeculae In fibrous dysplasia, radiographic changes blend into the surrounding normal bone. Treatment Surgical recontouring of bone for cosmetic reasons

Polyostotic Fibrous Dysplasia (cont.)

Paget Disease of Bone (Osteitis deformans) (pgs. 280-281) 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 The maxilla is more commonly affected than the mandible.

Paget Disease of Bone Clinical and radiographic features Radiographic (pgs. 280-281) Clinical and radiographic features Enlargement is common; the patient often complains of pain. Spaces may increase between teeth as bone enlarges. Radiographic A patchy radiolucency and radiopacity, “cotton wool” Hypercementosis, loss of lamina dura, and obliteration of the periodontal ligament may occur.

Paget Disease of Bone (cont.)

Paget Disease of Bone (cont.) (pgs. 280-281) Diagnosis and Treatment The serum alkaline phosphatase level is significantly elevated in active disease. Histologic examination reveals bony trabeculae surfaced with numerous osteoclasts and osteoblasts. Prominent reversal lines may create a pattern known as mosaic bone. Treatment Experimental; the disease is slowly progressive

Paget Disease of Bone (cont.)

Central Giant Cell Granuloma(Central Giant Cell Lesion) (pgs. 281-282) Composed of well-vascularized connective tissue containing many multinucleated giant cells Occurs within bone A disease of bone that develops over a long period of time The result of calcium deficiency In young children, it is usually caused by a deficiency of vitamin D. In adults, it may be related to various health problems.

Central Giant Cell Granuloma(Central Giant Cell Lesion) (cont.)

Osteomalacia Clinical and radiographic features Treatment May be associated with delayed tooth eruption and periodontal disease Treatment Based on identification of the cause

Discussion Questions With what disease are “ground glass” and “fingerprint pattern” associated? What is Paget disease? What are the signs of osteomalacia?