Appearance Of Fibrous Dysplasia Research Articles

9. A case report of mazabraud's syndrome and literature review

Background Mazabraud's syndrome is rare and has characteristic radiological and histopathological findings. Within radiological circles the appearance is such a common radiology registrar trap that it is considered a ‘spotter’ amongst practicing radiologists. The pathological appearance of fibrous dysplasia and intramuscular myxomas are individually characteristic however the differing natural history and management means that the connection between both entities may not be apparent on histopathology alone. Clinicoradiological findings A 43 year old man presented with a 2 month history of a tender mass below the inguinal ligament. An ultrasound of the right thigh found a 4.4 cm solid intramuscular tumour which was subsequently imaged with X-ray and MRI and showed typical features of polyostotic fibrous dysplasia involving the pelvis and proximal femur with a Shepherd's crook deformity and multiple soft tissue masses with features of intramuscular myxomas and the diagnosis of Mazabraud's syndrome was given. Six years after the initial diagnosis the patient presented after a fall with a pathological subtrochanteric femoral fracture. A total hip replacement was performed and one intramuscular lesion was sampled. Pathological findings The femoral neck was deformed and the medulla was cystically expanded by blood and fragments of pale grey fibrous tissue which had ‘Chinese character’ shaped metaplastic trabeculae on morphology. The myxoid lesion was surrounded by a thin rim of incomplete skeletal muscle and had a gelatinous grey surface which was hypocellular and hypovascular microscopically confirming the presence of fibrous dysplasia and intramuscular myxoma and thus the diagnosis of Mazabraud's syndrome. Conclusion The combination of fibrous dysplasia and intramuscular myxoma is characteristic of Mazabraud's syndrome.

Fibrous dysplasia of the thoracic spine

Fibrous dysplasia occurs rarely in the spine, especially in the thoracic spine. According to the literature, less than 20 cases have been reported. We report in this paper a 48-year-old female patient with fibrous dysplasia of the first thoracic spine and left side of the first rib with left thoracic first root compression. She complained of severe pain of the left arm, numbness on the ulnar side of the left forearm, and weakness of the middle to little fingers. Computed tomography and magnetic resonance imaging showed the characteristic appearance of fibrous dysplasia, which includes ground-glass opacity, an expansile nature, and lytic lesions with sclerotic rims. She underwent surgical intervention with radical tumor resection combined with stabilization and fusion with a mesh graft. The pain was relieved. The thoracic first root compression signs improved after the operation. Nineteen months after the operation, there was no recurrence of the tumor. We suggest that radical tumor resection combined with stabilization and fusion with an anterior or posterior approach using a mesh graft for thoracic fibrous dysplasia can achieve definite decompression and prevent tumor recurrence.

English

Fibrous dysplasia is a benign fibro-osseous lesion that can occur as an isolated skeletal lesion (monostotic form) or affect multiple skeletal sites (polyostotic form). The monostotic type is 30 times more common than the polyostotic type. (4) However, this is a very rare lesion, making a correct diagnosis difficult, especially since osteofibrous dysplasia and adamantinoma of the tibia have to be taken into account in the differential diagnosis of the disease. Here is an unusual case of aggressive monostotic fibrous dysplasia of tibia. We say this case is a rare presentation as the appearance was atypical and did not correspond to classical appearance of fibrous dysplasia and was subsequently proved histopathologically.

Craniofacial fibrous dysplasia (CFD) of the maxilla in an 11-year old boy: A case report

We present the case of a surgically treated 11-year old boy with a diagnosis of craniomaxillofacial fibrous dysplasia (CFD) in the maxillary sinus. When first seen in the outpatient clinic of our department he had minimal symptoms. After initial radiological diagnostics by computed tomography scans (CT-scans) the patient was treated operatively by radical excision of the tumor. The radiographs showed the typical intramedullary located and well-defined lesions, which eroded the cortical bone with the typical appearance of fibrous dysplasia. The histopathology showed the typical curved extending fibrous trabeculae in C, O and Y-shape which were embedded in a moderately cellular morphologically inconspicuous stroma, confirming the initial suspicion of fibrous dysplasia of the maxillary bone. Cone beam tomography was a valuable tool in determining the re-ossification of bone at the affected side. Local resection can be curative in limited disease.

Bone invasion by an esthesioneuroblastoma mimicking fibrous dysplasia

We report the unusual findings of a superior nasal esthesioneuroblastoma extending intracranially with a pattern of invasion of the frontal bone and skull base mimicking the radiographic appearance of fibrous dysplasia. We correlate the CT and MR appearance of the bony changes with the surgical and pathologic findings and review the radiographic appearance of esthesioneuroblastoma and fibrous dysplasia.

Avid uptake of [18F]-FDG in fibrous dysplasia can mimic skeletal involvement in Hodgkin’s disease

Fibrous dysplasia is a common benign skeletal disorder that is assumed to result from a development failure during the bone maturization process. It can be either mono- or polyostotic. Most patients present before the age of 30 years [1]. To date, only a few descriptions of the appearance of fibrous dysplasia on [ 18 F]-fluorodeoxyglucose–positron emission tomography (FDG PET) images have been reported in the literature [2, 3]. We present here the case of monostotic fibrous dysplasia which was detected through the avid uptake of [ 18 F]-FDG. A 12-year-old boy was diagnosed with Hodgkin’s disease. The initial [ 18 F]-FDG PET images showed cervical lymphadenopathy and an avid tracer uptake in the right proximal femur. This finding was interpreted as skeletal involvement of lymphoma, thus defining clinical stage IV disease. After four courses of chemotherapy, a second [ 18 F]-FDG PET (a) was performed for response evaluation. At this time, pathologic tracer uptake was only seen in the right proximal femur (a, arrow), and we therefore considered residual lymphoma in the differential diagnosis. The corresponding radiograph (b) revealed an ill-defined area of ground-glass opacity and a cortical thickening (b, arrowhead). Magnetic resonance imaging showed the lesion to be hypointense in T1weighted sequences and hyperintense in T2-weighted sequences, and demonstrated a strong uptake of contrast medium (c). Due to the significant implications of possible residual lymphoma, open biopsy was performed. The hematoxylin and eosin-stained section (d) showed typical features of fibrous dysplasia with randomly organized trabeculae of woven bone surrounded by immature fibrous tissue.

The “Pirate Sign” in Fibrous Dysplasia

Fibrous dysplasia commonly involves the skull in both its monostotic and polyostotic variants. We present two cases of fibrous dysplasia involving the sphenoid wing, which were strikingly similar in their bone scan appearance. Both patients demonstrated intense increased uptake of Tc-99m MDP in a pattern reminding us of a "pirate wearing an eyepatch." We propose that this characteristic appearance of fibrous dysplasia of the sphenoid wing be called the "pirate sign." A review of the literature revealed several other pathologic conditions that have been reported to involve the sphenoid bone and should be considered in the differential diagnosis of abnormal bone tracer uptake in this region.

Ophthalmic manifestations of fibrous dysplasia: A disease of children and adults

ObjectiveTo determine whether adults constitute a significant proportion of patients presenting with ophthalmic complications of fibrous dysplasia. DesignA retrospective case series. ParticipantsTwenty patients with biopsy-proven fibrous dysplasia participated. Main outcome measuresPatient demographics, major signs and symptoms, occurrence of visual loss, pattern of bone involvement, and radiographic appearance of fibrous dysplasia were studied. ResultsIn contrast to observations made in the past, the authors found that adults constitute a significant proportion of patients suffering from the ophthalmic complications of fibrous dysplasia. Of 20 patients, 9 were younger than 18 years of age (children) and 11 were 18 years of age or older (adults) at the time of presentation. Changes in facial contour and symmetry were the most common presenting signs and symptoms. Five patients, four of whom were adults, presented because of acute visual loss. Most patients had monostotic lesions that crossed suture lines to involve multiple craniofacial bones. On computed tomographic scan, most fibrous dysplasia lesions had a characteristic, pagetoid appearance, with alternating areas of radiolucency and radiodensity. ConclusionsBecause fibrous dysplasia of the orbital bones can be a cause of significant dysfunction and disfigurement, as well as a treatable cause of blindness in both children and adults, the diagnosis of fibrous dysplasia should not be ruled out based solely on the age of the patient. The characteristic radiologic appearance of this disease allows one to differentiate fibrous dysplasia from other tumors associated with bony expansion or density changes, specifically meningioma.

Radiographic appearance of fibrous dysplasia associated with the maxillary sinus

Fibrous dysplasia is a relatively rare tumorous lesion in the maxillofacial region. The radiographic appearance of this lesion varies widely in the jaw. Generally, the occurrence rate is higher in the maxilla than in the mandible.

Appearance of Fibrous Dysplasia on ln-111 Labeled Leukocyte Scintigraphy

Appearance of Fibrous Dysplasia on ln-111 Labeled Leukocyte Scintigraphy

Albright's syndrome with hypophosphatemic rickets and hyperthyroidism: a case report

In this abstract we report a case of Albright's syndrome associated with hypophosphatemic rickets and hyperthyroidism in a six-year-old girl. She had suffered from repeated fractures of her long bones owing to multiple locations of radiolucent areas and generalized skeletal demineralization. The biopsy in the lucent area revealed histologic appearance of fibrous dysplasia.

MR appearance of fibrous dysplasia.

Magnetic resonance (MR) imaging is utilized in diverse clinical circumstances for evaluation of the musculoskeletal system. Consequently, common benign skeletal lesions may be detected incidentally following MR scan obtained for unrelated reasons. The spectrum of MR appearances of fibrous dysplasia has not been previously reported. We retrospectively reviewed all radiologic images (including the MR scans) of 11 sites of fibrous dysplasia encountered in seven patients. On MR, the fibrous dysplastic lesion causes an "expanded" bony contour and is characterized by decreased signal on T1-weighted image. The signal on T2-weighted MR scan is variable, however. Since fibrous dysplasia is a (relatively) common skeletal lesion that can be encountered incidentally, all radiologists should be familiar with its MR appearance. Furthermore, in the unlikely possibility of surgical intervention, MR is useful in determining the extent of disease within the affected bone and planning the preoperative strategy.

Fibrous dysplasia of the jaw bones. Analysis of five cases.

Five clinically typical cases of fibrous dysplasia are analysed. Histopathologic and histochemical study revealed great variability from one lesion to another, and in one and the same specimen. One case showed the histologic features of fibrous dysplasia, ossifying fibroma and giant cementoma. The microscopic appearance of fibrous dysplasia is affected by the age of the process and of the patient. Definitive pathologic differentiation between the benign fibro-osseus lesions of the jaw bones can be made on the basis of microscopic characteristics together with clinical findings. The best way of treating fibrous dysplasia is by conservative surgery. In one case postoperative regrowth was successfully controlled with ACTH and oxyphenbutazone.