What is Osteofibrous dysplasia?

What is Osteofibrous dysplasia?

Osteofibrous dysplasia is a noncancerous tumor that typically develops during childhood. It does not spread to other parts of the body and many cases are treated conservatively with careful observation over time. An adamantinoma is a cancerous tumor that is capable of spreading and requires surgery to remove.

What is fibrous dysplasia monostotic?

Fibrous Dysplasia (FD) is a developmental disorder of bone that can affect one bone (monostotic type) or multiple bones (polyostotic type). The disease can be associated with hyperpigmentation and endocrinological disorders. It is usually observed in adolescents and young adults and comprises 7% of benign bone tumors.

What gene causes fibrous dysplasia?

Symptoms and Causes Fibrous dysplasia results from a genetic mutation (change) on chromosome 20. This mutation happens on one gene that directs bone formation and growth, but what causes this mutation to occur remains unknown.

What causes Osteofibrosis?

The cause of osteofibrous dysplasia is unknown. Treatment is usually conservative, involving observation until the bone stops growing (skeletal maturity). Bracing may help prevent bowing of the limb and fractures. Surgery may be recommended once bone growth is complete.

What is florid osseous dysplasia?

Florid cemento-osseous dysplasia (FCOD) is a condition that occurs in the jaw bone, especially close to where the teeth are formed. People with FCOD develop lesions in the jaw, were spots of normal bone are replaced with a mix of connective tissue and abnormal bone.

What is craniofacial fibrous dysplasia?

Craniofacial fibrous dysplasia is a bone disease of the face and skull that replaces normal bone with fibrous-type tissue. This tissue is not as hard as normal bone, and because it is soft and stringy, it makes the bone more fragile and prone to break.

How do you get rid of fibrous dysplasia?

Although fibrous dysplasia is a genetic disorder, it’s caused by a gene mutation that’s not passed from parent to child. There’s no cure for the disorder. Treatment, which may include surgery, focuses on relieving pain and repairing or stabilizing bones.

Where does periapical cemento-osseous dysplasia occur in the posterior jaw?

Periapical cemento-osseous dysplasia typically arises in the anterior mandible and involves one or only a few teeth. Another limited form that occurs in the posterior jaw and typically involves molar teeth is called focal cemento-osseous dysplasia.

What kind of disease is mandibuloacral dysplasia type a?

Mandibuloacral dysplasia type A may also be classified as a laminopathy, a general term for the group of disorders associated with a mutation of the LMNA gene. The ZMPSTE24 mutation that causes mandibuloacral dysplasia type B can also cause restrictive dermopathy syndrome.

How is florid cemento osseous dysplasia complicated by osteomyelitis?

Florid cemento-osseous dysplasia may be complicated by osteomyelitis and drainage of necrotic bone debris into the oral cavity or to the skin surface through osteocutaneous sinus tracts. At imaging, lesions are initially lytic, with a mixed lytic and sclerotic appearance seen later]

Where does idiopathic osteosclerosis occur in the mandible?

In 90% of patients it occurs in the mandible, usually near the first molar or second molar or premolar. At imaging, idiopathic osteosclerosis has sharp margins and is small and round or oval, sclerotic, periapical, and tooth related, occasionally with peripheral spiculation. In 20% of patients, it is not related to a tooth.