Fibrous dysplasia is an uncommon benign disorder of unknown etiology. The disease was first described by McCune and Albright in separate publications in 1937. The term, fibrous dysplasia was suggested by Lichtenstein in 1938. The disease has since been found to have 3 different variants: monostotic, polyostotic, and McCune-Albright syndrome. It is a slowly progressive bony disorder where normal bone is replaced by abnormal fibrosseous tissue. Involvement of fibrous dysplasia of the temporal bone is usually unilateral. The squama becomes thickened and the pneumatic system is obliterated. Because fibrous dysplasia shows a predilection for the facial and cranial bone, where it causes deformity and dysfunction. In this paper, we report a case of fibrous dysplasia of the temporal bone. We discuss the characterisitic features of this specific location of the disease, the differential diagnosis, and the treatment policy. We also address the issue of vertigo.
Congenital Abnormalities ; Diagnosis, Differential ; Dizziness ; Fibrous Dysplasia, Monostotic* ; Fibrous Dysplasia, Polyostotic ; Hearing Loss* ; Hearing* ; Temporal Bone* ; Vertigo

The X-ray radiograph, CT scan and MRI appearance of 5 patients with pathologically proven fibrous dysplasia in thoracic and lumbar spine vertebrae were retrospectively analyzed. Plain radiographs, CT scans and MR images showed the presentation of eccentric lesion with intact cortex bone and marginal sclerosis in vertebral bodies without involvement of vertebral appendix and extraosseous soft tissue. The lesion masses were round (one being oval-shaped) and radiolucent in plain radiographs and CT scans. Homogeneous long signal was observed on T1 weighted image and strongly enhanced when gadolinium was administered. On T2 weighted MRI, short signal was found in the anterior part of the mass, long signal in the posterior part, and short and slight long signal in the middle part, without partitioning and laminating change. There was a good correlation between radiological features and surgical findings. These findings may be useful to diagnose fibrous dysplasia in spine.
Fibrous Dysplasia, Monostotic/diagnosis ; Fibrous Dysplasia, Monostotic/*radiography ; Lumbar Vertebrae ; Magnetic Resonance Imaging ; Retrospective Studies ; Thoracic Vertebrae ; Tomography, X-Ray Computed ; Young Adult

Nine typical cases of ossifying fibroma in the tibia or fibula were retrospectively reviewed for clinical and histopathological aspects of this disease. Along with the case analysis, light and polarized microscopic examinations were performed in each case. Differential points with monostotic fibrous dysplasia were as follows; 1) ossifying fibroma begins in the significantly younger age, which is in most cases below 10 years of age, 2) it is confined to diaphysis of tibia or fibula, 3) radiologically, multilocular osteolytic destructive change in eccentric pattern is usually combined with anterior or anterolateral bowing deformity, 4) microscopically, characteristic findings are presence of osteoblastic rimming, peripheral maturation, and zonal phenomenon. In the treatment of ossifying fibroma, definitive surgical treatment should be delayed until skeletal maturity, when segmental resection in wide margin including periosteum might be preferred.
Congenital Abnormalities ; Diagnosis ; Diaphyses ; Fibroma, Ossifying ; Fibrous Dysplasia, Monostotic ; Fibula ; Fluconazole ; Osteoblasts ; Periosteum ; Retrospective Studies ; Tibia

The association of intramuscular myxoma and fibrous dysplasia is a rare disease known as Mazabraud's syndrome. We present a case of Mazabraud's syndrome coexisting with a uterine tumor and resembling an ovarian sex cord tumor (UTROSCT). This uterine tumor showed a high mitotic index and cytological atypia. To the best of our knowledge, the coexistence of the two different entities has not been reported in the literature.
Aged ; Biopsy ; Buttocks/pathology/surgery/ultrasonography ; Diagnosis, Differential ; Female ; Fibrous Dysplasia, Monostotic/complications/*diagnosis/surgery ; Humans ; Magnetic Resonance Imaging ; Myxoma/complications/*diagnosis/surgery ; Ovarian Neoplasms/*diagnosis ; Rare Diseases ; Sex Cord-Gonadal Stromal Tumors/*diagnosis ; Syndrome ; Uterine Neoplasms/complications/*diagnosis/surgery