Pdf osteofibrous dysplasia of the tibia in children. 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. Imaging in osteofibrous dysplasia, osteofibrous dysplasialike adamantinoma, and classic adamantinoma. The best imaging modality to detect a nidus is thinsection ct 11. Rare form of fibrous dysplasia that primary affects the tibia and is confined to the cortices. Dinkova department of oral surgery, faculty of dental. Adamantinoma of the tibia and fibula with cytogenetic analysis. This study reported a case of osteofibrous dysplasia. The purposes of this retrospective study were to assess specific mri features of adamantinoma. The etiology of the tumor is still a matter of debate. First, ct image reconstruction was performed to mirror the patients original anatomy. Software is now available that allows the performance of reconstructions. Long term followup study on natural history, results of treatment and relationship with adamantinoma time frame.
Lesions are eccentric, intracortical, and osteolytic. When questions arise in the diagnosis of the tumor. Osteofibrous dysplasia exhibited diverse imaging features ranging from lesions confined to the cortex to more aggressive lesions with complete. Weve noticed that youre using an ad blocker our content is brought to you free of charge because of the support of our advertisers. This article discusses the epidemiology, clinicopathological and imaging features, diagnosis, treatment, and prognosis of adamantinoma of the long bones. Osteofibrous dysplasia in a cockatiel nymphicus hollandicus. It is most commonly seen in the middiaphysis of the tibia. Findings including age and anterior tibail diaphysis location possibly suggest adamantinoma of tibial diaphysis versus other differential of osteofibrous dysplasia. The webs most comprehensive source for bone tumor information. Osteofibrous dysplasia, osteofibrous dysplasialike. This condition should be differentiated from nonossifying fibroma and fibrous dysplasia.
Mri of adamantinoma of long bones in correlation with. 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. Osteofibrous dysplasia is a benign fibroosseous cortical lesion that occurs almost exclusively in the tibia and fibula. Correlation of radiological imaging features with surgical histology and assessment of the use of radiology in. To continue enjoying our content, please turn off your ad. The purpose of the present study was to describe mr imaging findings of osteofibrous dysplasia.
Some consider it synonymous with ossifying fibroma because of histological similarities, but it is generally considered a separate entity due. 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. 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 is a noncancerous tumor that typically. P agets disease of bone is a frequently encountered incidental finding on radiographs and scintigrams. Osteofibrous dysplasia, osteofibrous dysplasia like adamantinoma and adamantinoma. A classic adamantinoma arising from osteofibrous dysplasia like adamantinoma in the lower leg. 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. Key imaging features of tibial osteofibrous dysplasia ofd, osteofibrous dysplasialike adamantinoma ofdad, and classic adamantinoma ad. The predominance of an osteofibrous dysplasialike pattern with the. Mri may exhibit an exaggerated image due to the intensive edema around the lesion, resembling a malignant. The proposed association between osteofibrous dysplasia and adamantinoma has led some to advocate resection of the entire lesion.
Fibrous dysplasia fd is part of a rare group of bone dysplasia. Radiography, computed tomography, and magnetic resonance imaging. Diagnostic radiologymusculoskeletal imagingtumors basic. Fibrous dysplasia is a bone disease characterized by abnormal differentiation of. Osteofibrous dysplasia is a rare, clinicopathologically. Osteofibrous dysplasia ofd is a rare, benign, selflimiting, fibroosseous lesion occurring in long bones especially of lower limbs. 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. Fibrous dysplasia in the maxillomandibular region case report cholakova r. 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. The differentiation of adamantinoma from fibrous dysplasia and osteofibrous dysplasia may be difficult by using plain radiographs alone.
Fibrous dysplasia treated with virtual planning report of two cases. Osteofibrous dysplasia is a rare, noncancerous benign tumor that affects the long bones. Imaging in osteofibrous dysplasia, osteofibrous dysplasia. To date,the diagnostic characteristics of osteofibrous dysplasia on computed tomography ct or magnetic resonance imaging mri. Nord gratefully acknowledges frederick singer, md, director, endocrinebone disease program, john wayne cancer institute. 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. Fibrous dysplasia nord national organization for rare disorders.
John hunter is a professor in the department of radiology musculoskeletal section at uc davis school of medicine. 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. Mri of adamantinoma of long bones in correlation with histopathology. Adamantinoma, fibrous dysplasia and osteofibrous dysplasia ossifying fibroma share many radiographic and pathological features, but their interrelationships are unclear. Radiographically, the intermediate phase of pagets disease. An adult female cockatiel nymphicus hollandicus was presented with the following problems. To continue enjoying our content, please turn off your ad blocker. Although osteofibrous dysplasia has been considered in the past to be a variant of fibrous dysplasia, it has a quite different presentation. Osteofibrous dysplasia, osteofibrous dysplasialike adamantinoma and adamantinoma. 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 radiology reference article. Osteofibrous dysplasia exhibited diverse imaging features ranging from lesions confined to the cortex to more aggressive lesions with complete intramedullary involvement or perilesional. Ministry program for orphan diseases has triggered the development of.
Campanacci described this condition in two leg bones, the tibia and fibula, and coined the term. Osteofibrous dysplasia is a rare and benign nonneoplastic condition of unknown etiology in humans and mammals. Variable expansion of the external cortical surface is present, with sclerosis of the internal cortical surface. Developed by renowned radiologists in each specialty, statdx provides comprehensive decision support you can rely on osteofibrous dysplasia. Developed by renowned radiologists in each specialty, statdx provides comprehensive decision support you can rely on adamantinoma. Imaging in osteofibrous dysplasia, osteofibrous dysplasialike. Osteofibrous dysplasia ofd and adamantinoma are rare bone tumors that are most often found in the tibia shinbone.
Clinicopathological features, diagnosis, and treatment of. Osteofibrous dysplasia radiology reference article radiopaedia. This research was supported by the intramural research program of the. In this series of radiographs comprising imaging of osteofibrous dysplasia like and classic adamantinomas.
Congenital osteofibrous dysplasia, involving the tibia of. 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 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. 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.
Paediatric osteofibrous dysplasialike adamantinoma with. In mccunealbright syndrome mas, fibrous dysplasia is associated with. 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 rare, benign nonneoplastic condition with no known cause. 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. Adamantinoma is a primary lowgrade, malignant bone tumor that is predominantly located in the midportion of the tibia. A comparative study of fibrous dysplasia and osteofibrous dysplasia. 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.
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