Diagnosis and treatment of an odontoid fracture in a patient with polyostotic fibrous dysplasia: case report

Fibrous dysplasia of the cervical spine is rare. No prior reports have discussed odontoid fractures in the setting of fibrous dysplasia. We describe a 26-year-old man who suffered a traumatic odontoid fracture in an area of preexisting fibrous dysplasia. The patient was treated conservatively in a sterno-occipito-mandibular immobilizer brace with a good result. A review of fibrous dysplasia is presented. Alternative methods of diagnosis and treatment options in our patient are discussed.     

“Banana patella”, fibrous dysplasia of patella: A rare case report

IntroductionPatella is a very uncommon site for primary bone tumours and fibrous dysplasia lesion in patella has never been reported. Fibrous dysplasia is a benign fibrosseous lesion of the bone where the fibrous connective tissue replaces the normal bone.Case reportWe report a case of 23 years female with 7 years history of progressive swelling and anterior knee pain. Radiographs showed enlarged, deformed patella like a shape of banana. The patient underwent patellectomy with extensor mechanism repair and biopsy proved to be fibrous dysplasia.ConclusionFibrous dysplasia involving patella is very rare and never been reported earlier. Although primary patellar neoplasm are not common but should be kept in mind in chronic anterior knee pain with swelling for early diagnosis and intervention with intralesional therapy otherwise patellectomy has to be done in more advanced, deformed and enlarged lesions.     

Fibrous dysplasia

Fibrous dysplasia is a developmental disorder of bone that can present in a monostotic or polyostotic form. Primarily affecting adolescents and young adults, it accounts for 7% of benign bone tumors. Many of the asymptomatic lesions are found incidentally; the remainder present with symptoms of swelling, deformity, or pain. Fibrous dysplasia has been associated with multiple endocrine and nonendocrine disorders and with McCune-Albright and Mazabraud's syndromes. The etiology remains unclear, but molecular biology suggests a mutation in the G(s)alpha subunit and activation of c-fos and other proto-oncogenes. Fibrous dysplasia has a characteristic radiographic appearance. Most cases do not require intervention, but those that do usually are managed surgically with curettage, bone grafting, and, in some cases, internal fixation. When some intervention is necessary but surgery is not practical, treatment is with bisphosphonates. The prognosis generally is good, although poor outcomes are more frequent in younger patients and in those with polyostotic forms of the disease. The risk of malignant transformation is low.     

Polyostotic fibrous dysplasia of the cervical spine: case report and review of the literature.

BACKGROUND CONTEXT: Multiple lytic lesions of the spine usually represent metastatic or infectious disease processes. PURPOSE: To describe an extremely rare presentation of an uncommon disease process. STUDY DESIGN/SETTING: Case report/university hospital. METHODS: We describe the management of a patient who presented with a pathological fracture of C3 and multiple lytic lesions of the cervical spine. RESULTS: After reconstructive surgery, the final pathological diagnosis was fibrous dysplasia. CONCLUSION: Fibrous dysplasia is rarely seen in the cervical spine and may mimic other pathological processes. The surgical and medical management of spinal fibrous dysplasia is described.     

Fibr?se Dysplasie

Fibrous dysplasia is a benign intraosseous tumor disease which can be found in a monostotic or polyostotic form. In combination with dermatological and endocrine features it is known as McCune-Albright disease. Fibrous dysplasia originates from a genetic defect that has been identified as a postzygotic mutation of the GNAS gene leading to a malregulation of osteogenesis of the affected part of the bone. The weakening of the bone may result in isolated local pain, pathological fractures or severe deformation of the bones, which in the latter case results in loss of walking ability. Orthopedic treatment offers suitable methods to set and stabilize fractures, to strengthen weak areas of bone and to straighten out and stabilize deformed long bones. The treatment can help many patients to return to a high level of pain-free mobility or help the most affected patients lead a better life with a restricted but individual mobility.     

Cystic dysplasia of the rete testis

Cystic dysplasia of the rete testis is a rare benign testicular tumor that is found mainly in the pediatric population and that must be distinguished from malignant lesions. Ultrasonography can aid in diagnosis and in evaluation for associated renal lesions. Treatment options include orchiectomy, testicular-sparing excision of the cystic mass, and nonoperative surveillance. To increase awareness of the disorder, we present the case of a 2-month-old male infant with cystic dysplasia of the rete testis.     

Monostotic fibrous dysplasia of the cranial vault: a case report

Fibrous dysplasia, when it occurs in the craniofacial region, mostly involves the skull base and is rarely localized in the cranial vault. Although there have been several reports on magnetic resonance imaging (MRI) findings of fibrous dysplasia involving the skull base, cases occurring in the cranial vault have seldom been reported. We describe here a rare case of monostotic fibrous dysplasia that occurred in the parietal bone and discuss the characteristics of the MRI findings. A 47-year-old female was admitted to our hospital with a complaint of vertigo. A computed tomography (CT) scan did not reveal any intraparenchymal lesions in either the infra- or supratentorium, and her vertigo improved immediately without any treatments. However, a solitary osteolytic lesion was found incidentally in the left parietal bone. MRI showed that the lesion demonstrated hypointensity on T1-weighted images and hyperintensity on T2-weighted images, and was enhanced heterogeneously following injection of Gadolinium-DTPA. Removal of the parietal bone containing the lesion was performed according to the patient's wishes. The histopathological findings of the removed tissues corresponded to fibrous dysplasia. Although it is well known that craniofacial fibrous dysplasia demonstrates iso- or hypointensity on T1- as well as T2-weighted images, in the present case, the lesion showed apparent hyperintensity on T2-weighted images. These findings suggest that fibrous dysplasia can display various MR intensities depending on its origin.     

Thoracic outlet syndrome caused by fibrous dysplasia of the first rib

Fibrous dysplasia causing thoracic outlet syndrome is rare. A 41-year-old woman presented with neurogenic thoracic outlet syndrome with imaging that demonstrated a large tumor of her proximal left first rib. Transaxillary excision was unsuccessful due to involvement of the subclavian vasculature and brachial plexus. Subsequent posterolateral thoracotomy and resection of her first rib revealed fibrous dysplasia. Thoracotomy should be considered in these cases for optimal vascular control and identification of thoracic outlet anatomy.     

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.     

Mazabraud's syndrome: intramuscular myxoma associated with fibrous dysplasia of bone

A 53 year old male with a large swelling on the medial aspect of his right thigh was referred with a presumptive diagnosis of soft tissue sarcoma. However, biopsy revealed intramuscular myxoma and X-rays and CT scans suggested fibrous dysplasia of adjacent bone. Angiography had shown an expanded, hypervascular, intramedullary lesion in the femur, and a large avascular soft tissue mass lying medially in the distal thigh. Fibrous dysplasia of the femur was confirmed on bone biopsy. Subsequently one large and two smaller intramuscular myxomata were excised, with an uneventful postoperative course. This case illustrates Mazabraud's syndrome: the rare association between benign intramuscular myxoma and fibrous dysplasia of bone.     

Treatment of femoral fractures associated with fibrous dysplasia: a case report

Fibrous dysplasia is a rare condition in which bone tissue is replaced by fibro-osseous lesions. A sixteen-year-old male patient with fibrous dysplasia underwent plate fixation for subtrochanteric fracture of the proximal femur. Eighteen months postoperatively, progressive femoral deformity and refracture occurred. Upon failure in three-month conservative treatment, the plate was removed and intramedullary fixation was performed. Control radiographs at the end of 18 months after intramedullary fixation showed complete fracture healing. This case emphasizes that intramedullary fixation should be the first choice to treat femoral fractures and to prevent refractures in patients with fibrous dysplasia.     

Fibrous dysplasia of a third toe

Fibrous dysplasia is a benign disorder of bone, consisting of one or more foci of fibro osseous tissue within the matrix of the affected bone. Fibrous dysplasia usually effects the femur, tibia, ribs, and facial bones and is rarely seen in feet. An unusual case of fibrous dysplasia involving the third toe is presented.     

Fibrous dysplasia in combination with aneurysmal bone cyst of the occipital bone and the clivus: case report and review of the literature

OBJECTIVE AND IMPORTANCE: Fibrous dysplasia of the cranium is a relatively uncommon disorder that affects primarily the anterior cranial region; its occurrence in the cranial base in combination with aneurysmal bone cyst (ABC) constitutes an extremely rare condition, only two cases of which have been reported previously in the literature. It is important to recognize and treat these cases properly because of the special location in the cranial base and the possibility of neural structure impingement. CLINICAL PRESENTATION: We report the case of a 19-year-old man with a slowly enlarging mass of the occiput, with computed tomographic and magnetic resonance imaging revealing involvement of petrous and basisphenoid bone and growing ABC. INTERVENTION: Open biopsy confirmed the diagnosis of fibrous dysplasia. Partial excision of the lesion and removal of the ABC were performed in a second stage after embolization. CONCLUSION: ABC associated with fibrous dysplasia of the cranial base may enlarge rapidly after puberty and require excision. This is facilitated by preoperative embolization.     

Monostotic fibrous dysplasia of a cervical vertebra

Summary Fibrous dysplasia has been frequently reported to involve the spine in the polyostotic form, but only rarely in the monostotic form of the disease. The present case demonstrates monostotic fibrous dysplasia of the fourth cervical vertebra. We present the plain radiographic, CT-scan, scintigraphy and histologic examinations. C3–C4 posterior and anterior arthrodesis was performed. The post-operative follow-up was satisfactory.     

Fibrous dysplasia of the temporal bone: the use of computerized tomography

Fibrous dysplasia of the temporal bone is a rare condition characterized histologically by proliferation of fibrous tissue with scattered trabeculae of immature bone. Eighteen cases of monostotic fibrous dysplasia of the temporal bone have been reported in the literature. The clinical course of temporal bone fibrous dysplasia is unpredictable. Potential complications include cholesteatoma, recurrence, and malignant transformation. Surgery has been the recommended treatment, but the indications, approach, and extent have not been clearly established. The introduction of computerized tomography with high-resolution bone reconstruction is a significant advance in the therapeutic approach to temporal bone fibrous dysplasia. It accurately defines the extent of the disease within the temporal bone, and periodic scanning will reveal any progression. This information can be used to resolve many surgical dilemmas and to minimize secondary complications. This article includes a comprehensive review of the literature on temporal bone fibrous dysplasia and summarizes a case in which computerized tomography was used.     

Monostotic fibrous dysplasia of the foot

Fibrous dysplasia is a benign, nonfamilial disorder of the skeleton, characterized by expanding fibroosseous lesions occupying single or multiple bones with possible extraskeletal anomalies. Fibrous dysplasia begins in childhood and is not usually recognized until adolescence or adulthood. When fibrous dysplasia affects the foot, it is often the expression of the polystotic form of the disease. This paper will review the pathology of the disease and monostotic involvement of the first metatarsal.     

Fibrous Dysplasia of the Maxilla: Diagnostic Reliability of the Study Image. Literature Review

Objective Fibrous dysplasia (FD) is a benign bone disorder in facial bones. This study evaluates the possibility of diagnosing fibrous dysplasia on imaging alone, without biopsy of the lesion, which is often burdensome for the patient. Materials and Methods The authors bring their experience of four cases of bone lesions of the maxillofacial region and present a review of published studies. The imaging techniques evaluated are computed tomography (CT) and magnetic resonance imaging (MRI) with and without contrast. Results The literature review demonstrates that it is impossible to make diagnosis of fibrous dysplasia exclusively by imaging. Radiographic images often show a ground-glass appearance, which is characteristic but not pathognomonic of fibrous dysplasia. Conclusion Although CT and MRI images may in many cases suggest a diagnosis of fibrous dysplasia, histological examination or follow-up imaging should follow.     

Chondrosarcoma arising in monostotic fibrous dysplasia treated with total femur resection and megaprothesis: A case report

IntroductionFibrous dysplasia is tumor like lesions of bone which develop as substitution of bone by an expansion of fibrous connective tissue mixed with hard trabeculae. Chondrosarcomas is one of common malignant primary bone tumor derived from heterogenous group of neoplasm producing chondroid matrix. Chondrosarcoma arising in fibrous dysplasia, especially in monostotic fibrous dysplasia is a very rare case.Case reportA 54-year-old male presented with chief complaint of pain on left thigh. Patient with history of pathological fracture on left femoral diaphysis 3 years ago due to fibrous dysplasia and had underwent curettage, open reduction, and internal fixation at other hospital. Plain radiography revealed expansive lytic lesion, interrupted periosteal reaction with plate and screw attached to the lesion, and soft tissue mass. MRI T2FS sequence showed hyperintense mass extending from subtrochanteric to distal of left femoral diaphysis. Histopathological result from biopsy suggested chondrosarcoma.ConclusionsMalignant transformation of monostotic type was less frequently compared to polyostotic type. Among all malignant transformation cases, alteration to chondrosarcoma was more scarce than other malignancy such as osteosarcoma and fibrosarcoma. Wide surgical margin and reconstruction in chondrosarcoma provide good local control and functional outcome.     

Fibrous dysplasia masqurading as plural mass

Fibrous dysplasia is a benign fibro osseous developmental anomaly. Ribs are most frequent site in mono ostotic form. Though majority of these tumours are benign, they may very rarely transform in to malignancy. The clinical features are usually presented as asymptomatic swellings in 2^nd to 3^rd decade, pain indicates rapid onset of growth or malignant transformation. Pathological fractures are reported. In X-ray chest, these rib tumors appear as plural masses creates a diagnostic dilemma. Computerised Axial Tomographics (CAT) scan usually diagnostic. Fine needle aspiration cytology is not much informative. Surgical Excision of the tumour and chest wall reconstruction is the treatment of choice of these rare benign neoplasms.