Osteofibrous dysplasia is a rare, clinicopathologically. Osteofibrous dysplasia, described by companacci, in 1976, occurs almost exclusively within the diaphyseal cortex of the tibia or fibula of infants and children under the age of 10 years. To date,the diagnostic characteristics of osteofibrous dysplasia on computed tomography ct or magnetic resonance imaging mri. Fibrous dysplasia nord national organization for rare disorders.
Osteofibrous dysplasia, osteofibrous dysplasialike adamantinoma and adamantinoma. In this series of radiographs comprising imaging of osteofibrous dysplasia like and classic adamantinomas. Developed by renowned radiologists in each specialty, statdx provides comprehensive decision support you can rely on osteofibrous dysplasia. The aim of this study was to correlate the imaging features with surgical histology for tibial osteofibrous dysplasia ofd, osteofibrous dysplasia like adamantinoma ofdla and classical adamantinoma and to determine the additional role of imaging. Osteofibrous dysplasia radiology reference article. This article discusses the epidemiology, clinicopathological and imaging features, diagnosis, treatment, and prognosis of adamantinoma of the long bones. Adamantinoma, fibrous dysplasia and osteofibrous dysplasia ossifying fibroma share many radiographic and pathological features, but their interrelationships are unclear. To continue enjoying our content, please turn off your ad blocker. Nord gratefully acknowledges frederick singer, md, director, endocrinebone disease program, john wayne cancer institute.
Osteofibrous dysplasia is almost exclusive of the tibia and fibula, adamantinoma is strikingly prevalent in the tibia, but can also occur in other long bones and even in the soft tissues. Osteofibrous dysplasia exhibited diverse imaging features ranging from lesions confined to the cortex to more aggressive lesions with complete intramedullary involvement or perilesional. Osteofibrous dysplasia is a rare, benign nonneoplastic condition with no known cause. This study aims to describe our experience managing pediatric tibial osteofibrous dysplasia with such an approach and to report functional outcomes in children treated thus. Osteofibrous dysplasia is a rare and benign nonneoplastic condition of unknown etiology in humans and mammals. The best imaging modality to detect a nidus is thinsection ct 11. A classic adamantinoma arising from osteofibrous dysplasia like adamantinoma in the lower leg.
A comparative study of fibrous dysplasia and osteofibrous dysplasia. Fibrous dysplasia is a bone disease characterized by abnormal differentiation of. Clinicopathological features, diagnosis, and treatment of. Long term followup study on natural history, results of treatment and relationship with adamantinoma time frame. While radiographic findings of osteofibrous dysplasia are wellestablished, magnetic resonance mr imaging findings of osteofibrous dysplasia have not been fully described in the literature and have been reported anecdotally only 79. Adamantinoma is a primary lowgrade, malignant bone tumor that is predominantly located in the midportion of the tibia. Osteofibrous dysplasia and adamantinoma orthoinfo aaos.
This research was supported by the intramural research program of the. Congenital osteofibrous dysplasia, involving the tibia of. John hunter is a professor in the department of radiology musculoskeletal section at uc davis school of medicine. The differentiation of adamantinoma from fibrous dysplasia and osteofibrous dysplasia may be difficult by using plain radiographs alone. It is most commonly seen in the middiaphysis of the tibia. Rare form of fibrous dysplasia that primary affects the tibia and is confined to the cortices.
Osteofibrous dysplasia is a benign fibroosseous cortical lesion that occurs almost exclusively in the tibia and fibula. Osteofibrous dysplasia ofd and adamantinoma are rare bone tumors that are most often found in the tibia shinbone. Congenital osteofibrous dysplasia, involving the tibia of a neonate sang yoon kim, md, 1, 2 and sang hoon lee, md 1 1 department of radiology and research institute of radiology, university of ulsan. The purposes of this retrospective study were to assess specific mri features of adamantinoma. This condition should be differentiated from nonossifying fibroma and fibrous dysplasia. Software is now available that allows the performance of reconstructions. In mccunealbright syndrome mas, fibrous dysplasia is associated with. Imaging in osteofibrous dysplasia, osteofibrous dysplasia. The proposed association between osteofibrous dysplasia and adamantinoma has led some to advocate resection of the entire lesion. The webs most comprehensive source for bone tumor information. Key imaging features of tibial osteofibrous dysplasia ofd, osteofibrous dysplasialike adamantinoma ofdad, and classic adamantinoma ad. Variable expansion of the external cortical surface is present, with sclerosis of the internal cortical surface. Adamantinoma of the tibia and fibula with cytogenetic analysis.
Osteofibrous dysplasia ofd is a rare, benign, selflimiting, fibroosseous lesion occurring in long bones especially of lower limbs. Fibrous dysplasia in the maxillomandibular region case report cholakova r. Imaging in osteofibrous dysplasia, osteofibrous dysplasialike adamantinoma, and classic adamantinoma. Although osteofibrous dysplasia has been considered in the past to be a variant of fibrous dysplasia, it has a quite different presentation. The predominance of an osteofibrous dysplasialike pattern with the. Findings including age and anterior tibail diaphysis location possibly suggest adamantinoma of tibial diaphysis versus other differential of osteofibrous dysplasia. Osteofibrous dysplasia in a cockatiel nymphicus hollandicus. Weve noticed that youre using an ad blocker our content is brought to you free of charge because of the support of our advertisers. Osteofibrous dysplasia is a noncancerous tumor that typically. Sometimes mainly in younger patients, osteolyses are multifocal and extended in the diaphysis and associated with anterior bowing of the tibia, so that the imaging is, at least initially, identical to that of osteofibrous dysplasia. An adult female cockatiel nymphicus hollandicus was presented with the following problems. The most common location is the middle part of the tibia shin, although the fibula a smaller bone in the calf and the long bones in the arm humerus, radius, or ulna may also be affected. Radiography, computed tomography, and magnetic resonance imaging.
Dinkova department of oral surgery, faculty of dental. Correlation of radiological imaging features with surgical histology and assessment of the use of radiology in. The purpose of the present study was to describe mr imaging findings of osteofibrous dysplasia. The prominence of the osteoblasts led kempson in 1966 to describe the entity as ossifying fibroma of the long bones 2. Osteofibrous dysplasia is a benign fibroosseous lesion of bone which is most commonly occurred in cortical bone of anterior midshaft of the tibia of infancy and childhood. Some consider it synonymous with ossifying fibroma because of histological similarities, but it is generally considered a separate entity due.
Osteofibrous dysplasia exhibited diverse imaging features ranging from lesions confined to the cortex to more aggressive lesions with complete. Also called fibroosseous dysplasia, ossifying fibroma of long bones, campanaccis lesion mean age years, range 039 years, no gender preference closely related to fibrous dysplasia but osteofibrous dysplasia. Osteofibrous dysplasia ofd, osteofibrous dysplasialike adamantinoma ofdla, and classic adamantinoma ad are widely regarded as belonging to the same family of bone tumours with ofd at the benign end of the spectrum and ad at the malignant end. Osteofibrous dysplasia ofd, ofdlike adamantinoma and classic adamantinoma are postulated to represent a spectrum of morphologically similar disease and cannot be reliably distinguished by imaging. Osteofibrous dysplasia, osteofibrous dysplasia like adamantinoma and adamantinoma. It is also called as kempsoncampanacci lesion or cortical fibrous dysplasia.
Twentytwo comparable cases have been reported in the literature with such diagnoses as ossifying fibroma, congenital fibrous dysplasia, and congenital fibrous. The characteristic radiographic appearance of osteofibrous dysplasia see the images below has been well reported. First, ct image reconstruction was performed to mirror the patients original anatomy. Radiographically, the intermediate phase of pagets disease. Lesions are eccentric, intracortical, and osteolytic. Diagnostic radiologymusculoskeletal imagingtumors basic. Imaging in osteofibrous dysplasia, osteofibrous dysplasialike.
Osteofibrous dysplasia radiology reference article radiopaedia. For example, rare lesions such as adamantinoma, osteofibrous dysplasia, and chondromyxoid fibroma are seen predominantly in the tibia with infrequent involvement of other bones in the appendicular skeleton. Pdf osteofibrous dysplasia of the tibia in children. Paediatric osteofibrous dysplasialike adamantinoma with. When questions arise in the diagnosis of the tumor. Ministry program for orphan diseases has triggered the development of. The etiology of the tumor is still a matter of debate. To continue enjoying our content, please turn off your ad. Developed by renowned radiologists in each specialty, statdx provides comprehensive decision support you can rely on adamantinoma. Fibrous dysplasia treated with virtual planning report of two cases. This study reported a case of osteofibrous dysplasia. Fibrous dysplasia fd is part of a rare group of bone dysplasia. Osteofibrous dysplasia is a rare, noncancerous benign tumor that affects the long bones.
Osteofibrous dysplasia, osteofibrous dysplasialike. Mri of adamantinoma of long bones in correlation with histopathology. P agets disease of bone is a frequently encountered incidental finding on radiographs and scintigrams. Campanacci described this condition in two leg bones, the tibia and fibula, and coined the term. The aim of this study was to correlate the imaging features with surgical histology for tibial osteofibrous dysplasia ofd, osteofibrous dysplasialike adamantinoma ofdla and classical. Mri may exhibit an exaggerated image due to the intensive edema around the lesion, resembling a malignant.
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