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[Fibrous dysplasia in the head and neck region: a report of three cases].

Fibrous dysplasia is a benign process in which bone expands due to abnormal development of fibrous tissue. Involvement of single or multiple bones may occur. Common sites of involvement in the head and neck region are the maxilla and the mandible. Temporal bone involvement is rare. We report three patients with fibrous dysplasia, in whom the temporal bone (female, age 35 years) and the maxilla (male, age 17 years; female, age 20 years) were affected. The former underwent mastoidectomy, while the latter two patients underwent mass excision with lateral rhinotomy. No recurrences were detected within long-term follow-ups.

Adolescent↗

[Pathological bone structure (author's transl)].

The pathological and anatomical basis of various radiographic changes in a number of diseases of the skeleton is discussed. The conditions include abnormalities of bone formation and bone removal, including osteoporosis, fibrous dysplasia, osteomyelosclerosis, osteitis deformans, posttraumatic changes and ossifying periostitis. The form and structure of the pathological and anatomical substrate determine the radiological appearances and are essential for arriving at a diagnosis.

Adult↗

Clinical, computed tomographic, and histopathologic characteristics of juvenile ossifying fibroma with orbital involvement.

A 9-year-old boy who had had painless progressive proptosis of the right eye for 18 months, initially diagnosed as fibrous dysplasia, was found to have a circumscribed mass involving the frontal bone in the roof of the orbit. Biopsy of the mass led to the diagnosis of juvenile ossifying fibroma and the tumor was removed by a craniotomy approach. One year after surgery, the child appeared to be healthy. Ossifying fibroma can sometimes be differentiated from fibrous dysplasia both clinically and by computed tomography. Ossifying fibroma usually appears between the ages of 7 and 28 years and produces slowly progressive proptosis and displacement of the globe in a direction that depends on the original site of the tumor. Fibrous dysplasia usually develops during the first decade of life and produces facial asymmetry, proptosis, and displacement of the globe. An ossifying fibroma characteristically has well-defined margins, is round or ovoid, is usually monostotic, and produces expansion of the involved bone. Fibrous dysplasia is usually sclerotic and expands the bone throughout its length rather than in a localized fashion. Its borders are poorly defined and the lesion is often polyostotic.

Child↗

Technetium bone imaging as an adjunct in the management of fibrous dysplasia.

The diagnosis of fibrous dysplasia is one that is often made on the basis of biopsy findings and routine radiographs of involved areas. Until the advent of total body scanning, early cases of polyostotic fibrous dysplasia went undiagnosed until swelling, asymmetry, or deformity was noticed in other body parts. More sensitive radiographic techniques, (this is, bone imaging) are now available which offer a means for early detection of polyostotic involvement and more accurate delineation of lesions of the monostotic variety.

Adolescent↗

Magnetic resonance imaging and [11C]methyl-L-methionine positron emission tomography of fibrous dysplasia--two case reports.

Two cases of fibrous dysplasia in the skull base bone appeared hypointense on T1- and T2-weighted magnetic resonance imaging, with accumulation of [11C]methyl-L-methionine ([11C]Met) on positron emission tomography (PET). Fibrous dysplasia is a benign bone disorder which is identified by its distinctive radiography, computed tomography, and bone scintigraphy findings. [11C]Met PET may indicate the presence of viable tumor-like cells in fibrous dysplasia.

Biopsy↗

Fibrocartilaginous dysplasia of bone.

Fibrocartilaginous dysplasia is a benign, unusual complication of fibrous dysplasia occurring in the lower extremities, especially the femoral neck. Rapid growth and chondroid features may cause fibrocartilaginous dysplasia to be misdiagnosed as malignant. True chondrosarcomatous transformation of fibrous dysplasia is probably very rare. A recent case of fibrocartilaginous dysplasia is presented.

Adolescent↗

Osteosarcoma, fibrous dysplasia, and a chromosomal abnormality in a 3-year-old child.

Osteosarcoma is a type of malignant bone tumor that is rare among children less than five years old. This report describes a 3-year-old boy with osteosarcoma of the right proximal femur and polyostotic fibrous dysplasia of the right femur and tibia. After hemipelvectomy, histologic examination of the amputated limb disclosed that the osteosarcoma had developed in a focus of fibrous dysplasia. Cytogenetic analysis of the patient's blood lymphocytes revealed a 4q-/7p + translocation in all cells. The patient's mother had an identical translocation but did not have a history of osteosarcoma or evidence of fibrous dysplasia. The chromosomal abnormality and the developmental osseous disorder may have predisposed this patient to developing osteosarcoma at an exceptionally young age.

Child, Preschool↗

Sclerosing lesions of the temporal bone.

Sclerosis of the mastoid portion of the temporal bone is often seen on radiographs of patients who have or have had chronic otitis media. Occasionally lesions are seen that cause sclerosis of the entire temporal bone. The otolaryngologist must be alert to the fact that these findings may signify important pathology beyond that of chronic otitis media and indeed may be indicative of life threatening problems in adjacent or distant areas. In this paper the differential diagnoses of sclerotic lesions of the temporal bone will be discussed. These include fibrous dysplasia, osteopetrosis, meningioma, ossifying fibroma and metastatic lesions to the temporal bone. Three representative cases with sclerotic temporal bones are presented. The first is a patient with a conductive hearing loss secondary to stenosis of the external auditory canal. He was found to have a fibrous dysplasia of the temporal bone. The second case is an elderly male with a draining ear and a facial nerve paresis. He was found to have adenocarcinoma of the prostate gland metastatic to the temporal bone. The third case is that of a woman with a meningioma who demonstrated a sclerotic temporal bone on routine radiographs of the skull.

Adenocarcinoma↗