Fibro-osseous diseases Replacement of normal bone with a tissue composed of collagen fibers and fibroblasts that contain varying amounts of mineralized substance, which can be either bone or cementum-like material.
Fibrous Dysplasia Developmental hamartomatous fibro-osseous disease Unknown etiology. Four forms: Monostotic fibrous dysplasia affecting only one bone Polyostotic fibrous dysplasia affecting multiple bones Albright’s syndrome in which multiple lesions with hyperpigmentation and precocious puberty and/or hyperthyroidism Craniofacial fibrous dysplasia.
Clinical Features Painless swelling 1 st and 2 nd decades Females > Males Maxilla > Mandible Ground-Glass Radiographic appearance without clearly defined borders Teeth Displacement Active or Quiescent phases
Management “Burns itself out” – Early twenties Symptomatic treatment Biopsy treatment Surgical treatment limited in Active phase – Bleeding Regrowth following treatment in 25 – 50% if treatment is undertaken in young age.
Cemento -osseous dysplasia Asymptomatic and require no treatment Histologically the three types of cemento -osseous dysplasia are indistinguishable showing new woven bone trabeculae and/or spherules of cementum-like material. A fibrous tissue stroma is present. There is very little inflammatory component. Traumatic bone cysts have been reported in conjunction with this lesion.
Periapical Cemento -osseous Dysplasia Circumscribed lesions in periapical areas of vital teeth Anterior mandible most commonly involved African American females. [6%] Radiographically; radiolucent, of mixed density or radiopaque depending on their stage of development
Focal Cemento -osseous Dysplasia Predilection for middle-aged African American females Asymptomatic, Non- Expansile radiolucencies with associated opacities often in edentulous areas of the mandible. Previous dental extractions and may represent abnormal healing. Well circumscribed and rarely exceed 2 cm. Ossifying fibroma
Florid Cemento -osseous Dysplasia Middle-aged African American females Painless non- Expansile lesion often involving two or more jaw quadrants. Multiple confluent lobular radiopaque masses in tooth-bearing areas. Lesions may be associated with superimposed infection and osteomyelitis and idiopathic bone cysts. Un- encapsulated proliferation of cellular fibrous tissue with trabeculae or woven bone and calcification Chronic diffuse sclerosing osteomyelitis.
Ossifying Fibroma “Demarcated or rarely encapsulated neoplasm consisting of fibrous tissue containing varying amounts of mineralised material resembling bone and/or cementum” 2 nd to 4 th Decade in life Females > Males Mandible >> Maxilla Round or ovoid, expansive, painless jaw bone mass that may displace the roots of adjacent teeth and can cause root resorption. Early lesions are small and radiolucent. They become mixed radiolucent and radiopaque lesion and finally a radiopaque lesion
osteoblastoma Giant osteoid osteoma Primarily in the vertebrae and long bones Clinically it often grows rapidly Predominant clinical feature is localized pain. 2 nd decade of life and rarely in 30s Males more commonly affected than females. The mandible is the most common site. Lesions > 2 cm in diameter.
Osteoblastoma Well circumscribed with a thin radiolucency surrounding the variably calcified contents. Sunray Pattern Conservative surgical excision with curettage or local excision.
Osteoma Benign tumors of mature compact or cancellous bone. Periosteal osteomas or Endosteal osteomas Asymptomatic radiopacities. 2 nd and 5 th decades of life, Males > Females Gardner’s syndrome is an autosomal dominant condition in which patients have: Intestinal polyposis; Premalignant Multiple osteomas (usually endosteal ) of the jaws Fibromas of the skin Epidermal cysts Impacted teeth and odontomas.
osteoma Osteomas are often at the angle region of the mandible as well as the facial bones and long bones. Surgical Excision
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Langerhans Cell Histiocytosis Histiocytosis X Letterer- Siwe disease, Hand- Schüller -Christian disease, and eosinophilic granuloma. Langerhans cells are dendritic cells in the skin and mucosa that have a macrophage-like function. May have some of the properties of a tumor or have a viral etiology The acute disseminated form affects young children; Multisystem affecting the skin, bones, and internal organs (especially lungs and liver) Chemotherapy
Langerhans cell histiocytosis The chronic disseminated form of the disease is associated with a triad of punched-out bone lesions (skull and jaws), diabetes insipidus and exophthalmos (deposits in the posterior orbit). 2 nd and 3 rd decades of life. Well-defined punched-out radiolucencies, some are less defined A frequent aspect of presentation is loose teeth; radiographically they often appear as “floating teeth” Local curettage to chemotherapy.
Langerhans Cell Histiocytosis Chronic Localized Form Mandible – Bicuspid region Well-Defined Radiolucencies Aggressive Local Curettage
Schwannoma Benign tumor of the neurilemoma or nerve sheath. Usually found in the soft tissues, it can occur in bone. Well-defined radiolucency. Biopsy Treatment; surgical excision. Recurrences are rare. Histologically; well encapsulated and predominantly of spindle cells showing either an Antoni A (spindle cells arranged in palisaded whorls and waves) or Antoni B (spindle cells with a more haphazard appearance)
Neurofibroma Derived from the fibrous elements of the neural sheath Solitary lesions or as part of generalized neurofibromatosis or von Recklinghausen’s disease [AD]. Most commonly reported in soft tissues, can occur in bone and in the inferior alveolar nerve Appear as a fusiform swelling in continuity with the inferior alveolar canal with pain and paresthesia. Bony changes associated with neurofibromatosis can include cortical erosion from adjacent soft tissue lesions or medullary resorption from interosseous lesions. Localized excision. Often vascular The malignant transformation rate to neurogenic sarcoma is 5 to 15%
Paget’s Disease Osteitis deformans . Slowly progressive bone condition of unknown etiology. Males over the age of 50 years. Clinically; hyperactive bone turnover with alternate resorption of bone, a vascular phase, and finally a sclerosing phase. Most bones of the body are involved; Maxilla > Mandible Ill-fitting hats or dentures. Initial presentation is usually related to bone deformity or pain, headaches and vascular and nervous symptoms due to compression
Paget’s disease “Cotton-wool” appearance in the skull and maxilla of affected patients Hypercementosis around the roots of teeth Loss of lamina dura Obliteration of the periodontal ligament space. Systemic and Local Treatment Systemic; Calcitonin [Nasal Spray or SC] or diphosphonates [Oral or inj.] to inhibit bone resorption. Local; Cosmetic and/or functional recontouring of bone.
Paget’s disease Heart Failure and Osteosarcoma. Heart failure caused by the excessive blood supply to the remodeling bone can cause high output or left heart failure in elderly persons. Sarcomatous change has been reported in 5 to 15% of patients with Paget’s disease.
references Chapter 31: Benign Non-Odontogenic Lesions of the Jaws