1/100. Fibrous dysplasia of the temporal bone. Fibrous dysplasia is characterized by a progressive replacement of normal bone elements by fibrous tissue. It is seen in the two first decades of life and is equally distributed in relation to the sex of patients. The temporal bone is involved in 18% of the cases. Fibrous dysplasia of the temporal bone is more commonly manifested in the external auditory canal (80%) and the inner and medium ear can also be involved. Sarcomatous alterations are rare. diagnosis is based on radiological images. Treatment is conservative and surgery is reserved for preserving function and preventing complications. The case of a male patient, 16 years old, who presented with progressive hearing loss over a 1-year period is reported. ( info) |
2/100. Progressive unilateral mandibular swelling in adolescence: a diagnostic dilemma. Asymmetrical swelling of the mandible in adolescence may pose a significant diagnostic dilemma. The differential diagnosis ranges from traumatic, infectious, and metabolic processes to benign and malignant tumors. Among them, fibrous dysplasia, osteomyelitis, and malignancy may present with similar clinical and radiological features, making an accurate diagnosis quite difficult. This is an illustrative case involving a 14-year-old girl who initially presented with diffuse fibrous dysplasia of the mandible and in whom a superimposed osteomyelitis of the left side subsequently developed. Multiple investigations and several biopsies were required to arrive at a diagnosis. Similarities in clinical and radiographic findings of fibrous dysplasia, osteomyelitis, and malignancy are presented, and implications for treatment are discussed. ( info) |
3/100. Cystic degeneration in fibrous dysplasia of the jaws: a case report. A case of nonspecific cystic degeneration complicating fibrous dysplasia of the mandible is presented. This condition is extremely rare in the jaw bones. The patient had a painless swelling of the right face measuring 10 cm in diameter, and there was no history of trauma. Radiographs showed a poorly defined, "ground glass" radiopaque lesion; a central well-defined cyst was confirmed by means of computed tomography. During surgery, a large cystic cavity with surrounding, soft fibrous bone that merged with the cortex was observed. Histologic examination showed a large non-epithelial-lined cystic cavity with a surrounding fibro-osseous lesion, which was consistent with a diagnosis of fibrous dysplasia. ( info) |
4/100. Examination of tumor and tumor-like conditions of bone. Surgical pathology specimens composed of bone, ranging from core biopsy to limb amputation specimens, require special attention, processing, and often unique equipment. This readily translates into additional handling steps and time, especially when one factors in clinical correlation with the surgeon and radiologic review of all images with a knowledgeable musculoskeletal radiologist. When these factors are superimposed on the rarity of these lesions in routine practice, it is not surprising that most trainees, as well as seasoned pathologists, are wary of these lesions. In this report, we use a case of osteofibrous dysplasia (Campanacci's disease) to demonstrate the dissection of such a surgical specimen and complete the report with a brief discussion of the entity. ( info) |
5/100. Occipital fibrous dysplasia tonsillar herniation and cervical syringomyelia. A small posterior fossa volume has been thought to lead to hind brain herniation. The resultant obstruction to the CSF pathways at the level of the foraman magnum has been implicated in the development and subsequent progression of syringomyelia. This report highlights the gradual development of a small posterior fossa due to fibrous dysplasia of the occipital bone with consequent tonsillar herniation resulting in syringomyelia. ( info) |
6/100. Osteofibrous dysplasia of the tibia: case report and review of the literature. A case of osteofibrous dysplasia (OFD) of the tibia with 10 years of follow-up is presented. Spontaneous healing of this lesion occurred without any surgical intervention at the age of 10 years. The diagnosis was made retrospectively on the basis of clinical and radiographic appearance and evolution. The capricious nature and indolent course of this neoplasm has led to uncertainty regarding its etiology, evolution, and treatment. A review of the literature and the ongoing discussion about this matter is presented. ( info) |
7/100. Monostotic fibrous dysplasia of the temporal bone. Fibrous dysplasia is a slowly progressive bony disorder where normal bone is replaced by abnormal fibro-osseous tissue. Its monostotic variety in the temporal bone is very rare and such a case is presented here. Computed tomography (CT) may be adequate for the diagnosis and follow-up of these patients. Limited surgery should only be considered in cases of symptomatic disease. ( info) |
8/100. Monostotic fibrous dysplasia of the lumbar spine. Monostotic fibrous dysplasia is exceedingly rare. We report a case in a 61-year-old woman with a history of recurrent low back pain and sciatica since 35 years of age. While walking, she suddenly experienced pain in her right thigh. The pain spread gradually to the buttock and calf on the same side, becoming increasingly severe. The time pattern was mechanical, with exacerbation during straining. paresthesia developed over the dorsal aspect of the right foot. Nonsteroidal antiinflammatory drugs were ineffective. Radiographs of the spine showed an expansile and heterogeneous lesion in the body of L2. Hyperactivity of L3 and L4 was seen on the bone scan. Computed tomography demonstrated heterogeneity of L2, L3, and L4, as well as hypertrophy of the neural arch of L3 and of the right posterior lamina and spinous process of L4. Alterations in L2, L3, and L4 were noted on the magnetic resonance imaging study, which showed no evidence of epidural involvement. Laboratory tests were normal. A surgical biopsy of L3 established the diagnosis of fibrous dysplasia. Since the seminal description of fibrous dysplasia in 1891, only 21 cases of monostotic spinal involvement have been published. The spinal lesions can remain clinically silent or cause spinal pain with or without neurological symptoms. Radiographic findings are variable (heterogeneity, osteolysis, expansion without cortical violation or soft tissue involvement). calcium and phosphate levels are normal. The diagnosis depends on examination of a vertebral biopsy specimen. ( info) |
9/100. Monostotic fibrous dysplasia of the sphenoid sinus: a serendipitous finding on a bone scan. A 22-year-old woman had a Tc-99m MDP whole-body scan for low back pain. A focal area of increased activity was seen in the skull base in the region of the sella turcica. A computed tomographic examination showed ground-glass opacification of the sphenoid sinus and bony sclerosis along its walls, characteristic of fibrous dysplasia. Monostotic fibrous dysplasia, the more common form compared with the polyostotic variety, occurs in 70% to 80% of all patients with fibrous dysplasia. Monostotic lesions usually involve the ribs, femur, tibia, cranium, maxilla, and mandible. The frontal and sphenoid bones are the cranial bones most commonly involved. ( info) |
10/100. Fibrous dysplasia of the orbit in an infant. Fibrous dysplasia is an idiopathic fibro-osseous lesion of the skeletal bones. These uncommon osseous lesions usually manifest within the first two decades of life. Its occurrence during infancy is extremely uncommon, and we describe here a case of fibrous dysplasia involving the orbit in a 9-month-old male child. ( info) |