Bone and Soft Tissue Tumors

Fibrous Dysplasia

Fibrous Dysplasia  occurs with the development of fibrous tissue in the bone. With the development of fibrous tissue in the bone, bone tissue is replaced by fibrous tissue. That causes  bone weakening.  Bone deformity and fractures in the fragile bone develops. In mild cases, pain and other symptoms are seen. In severe cases, the pain and bone deformity  is seen. It is common under the age of 15.

In which bones is fibrous dysplasia common?

It can be seen in any bones of the body. Single bone involvement is more commonly encountered and Single bone involvement type is called Mono-static Fibrous Dysplasia. If more than one bone are involved then the type is named as polyostotic Fibrous Dysplasia. Most common involvement sites are Femur, tibia, pelvic bones, rib, skull, face bones, and humerus. Patients with polyostotic form have their first symptom before the age of 10.

What are the clinical findings in Fibrous Dysplasia?

Pain, fractures, bony deformities, and difficulty in walking are the most commonly seen complaints. Fibrous Dysplasia is rarely accompanied with endocrin diseases. Children with Fibrous Dysplasia and McCune-Albright Syndrome may present in early puberty. Hypertrophy of thyroid gland and cyst development can be seen. Cafe au lait spots can be observed in the skin of light colored kids.

How is bony deformity seen?

Femoral bone involvement results in bone bending deformity called Spherd’s Crook Deformity. Vertebral involvement may cause scoliosis. Vision and Hearing problems is seen in facial cones involvement. Joint involvement may lead arthritis.

How is diagnosis made?

Direct radiological exam, computed tomography, and MR are used in diagnosis. Bone scan and blood tastes can be done. In some cases needle or open biopsy can be necessary.

What is the treatment?

Incidentally diagnosed cases without any symptoms and deformity do not require any treatment rather than just follow-up. If any symptoms and deformity develops during follow-up, bisphosphonates are the first treatment choice. Bisphosphonates are used to prevent bone loss. They can be used either orally and parenterally.

When is surgical treatment required?

When there is a progressive deformity, excision  and bone grafting can be performed. Also, bony deformities are corrected. Pathological fractures also require surgery.

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Key Words: Fibrous Dysplasia, monostatic, polyostatic, bisphosphonate, spherds crook deformity.

About the author

Prof.Dr. Seref Aktas