Mr findings of the osteofibrous dysplasia korean j radiol 151, janfeb 2014 maturation is completed 14. Adamantinoma ad is a rare, lowgrade malignant primary bone tumor that occurs most often in the tibia andor fibula of adolescent persons and young adults. Although ofd and differentiated adamantinoma showed similar radiologic findings, differentiated adamantinoma was more likely to be a recurrent lesion than osteofibrous dysplasia and seemed to. Fibrous and osteofibrous dysplasia of bone clinical features. Dinkova department of oral surgery, faculty of dental medicine department of allergology, physiotherapy and. It is also called as kempsoncampanacci lesion or cortical fibrous dysplasia. Oct 20, 2016 osteofibrous dysplasia is a rare, noncancerous benign tumor that affects the long bones. Ossifying fibroma and fibrous dysplasia of the jaw are maxillofacial fibroosseous lesions that should be distinguished each other by a pathologist because they show distinct patterns of disease. Ossifying fibromas are benign bone lesions that should be differentiated from nonossifying fibromas and fibrous dysplasia. For just over 100 years, adamantinoma has been recognized as a primary bone tumor with epithelial characteristics and predominantly involving the tibia. Benassi ms, campanacci l, gamberi g, ferrari c, picci p, sangiorgi l, campanacci m. This form occurs, in decreasing order of frequency, in the craniofacial bones, ribs, femurs, tibias, and humeri. Osteofibrous dysplasia ofd is a rare, benign, fibroosseous lesion that typically is seen within the cortex of the tibia in children.
Although osteofibrous dysplasia has been considered in the past to be a variant of fibrous dysplasia, it has a quite different presentation. In most cases it occurs in the tibia of young adults. Histopathology of fibrous dysplasia basem attum ob team. Osteofibrous dysplasia radiology reference article. Osteofibrous dysplasia is a benign fibroosseous intracortical lesion that occurs almost exclusively in the tibia and fibula of children. Osteofibrous dysplasia, osteofibrous dysplasia like adamantinoma and adamantinoma. The tumor is characterized by slow clinical progression, with the potential to metastasize, mainly to the lungs and usually long after presentation of the primary tumor, with metastasis occurring with increasing frequency after local recurrence 15. Osteofibrous dysplasia differs clinically and microscopically from both ossifying fibrome of the jaws and fibrous dysplasia, despite the fact that such terms as ossifying fibroma of long bones and intracortical fibrous dysplasia have been applied te these lesions. There are many treatment option available, but still management of fd. This study reported a case of osteofibrous dysplasia arising in the humerus of adult, resulting in good prognosis after a. It is most commonly seen in the middiaphysis of the tibia. Paediatric osteofibrous dysplasialike adamantinoma with.
No history of trauma was noted and he was systemically well with no clinical signs of acute inflammation. Histopathology showed a benign fibroosseous lesion consisting of bland spindle cells with irregular trabeculae of woven bone 4. May 07, 2018 of three newborns with osteofibrous dysplasia of the tibia, two had swelling and one had pathologic fracture. Adamantinoma of bone is a rare malignant tumor that is almost exclusively seen in the diaphysis of the tibia. Osteofibrous dysplasia and adamantinoma orthoinfo aaos.
Mri of adamantinoma of long bones in correlation with. Monostotic fibrous dysplasia is more common than polyostotic fibrous dysplasia. Fracture callus should have a history of trauma, and osteofibrous dysplasia occurs almost exclusively in the tibia and fibula. Histological evaluation is necessary to distinguish between osteofibrous dysplasia ofd ofd, ofdlike adamantinoma and classic adamantinoma as these lesions are indistinguishable radiologically.
Jun 30, 2007 to report long term treatment outcomes of osteofibrous dysplasia and association with adamantinoma. This lesion has been shown by immunohistochemical studies to frequently contain cytokeratin. The histopathology of fibrous dysplasia of bone in patients with. Mr images of 24 pathologically proven osteofibrous dysplasia cases. He developed periodic relapses of pain in the clavicle. Read comparative study of fibrous dysplasia and osteofibrous dysplasia. Osteofibrous dysplasia almost exclusively affects the tibia and fibula. A comparative study of fibrous dysplasia and osteofibrous dysplasia with regard to g s mutation at the arg201 codon polymerase chain reactionrestriction fragment length polymorphism analysis of paraffinembedded tissues akio sakamoto, yoshinao oda, yukihide iwamoto, and masazumi tsuneyoshi from the department of anatomic pathology. Osteofibrous dysplasia and adamantinoma request pdf. Osteofibrous definition of osteofibrous by merriamwebster. Fibrous dysplasia mccune albright syndrome fdmas represents a wide spectrum of. A classic adamantinoma arising from osteofibrous dysplasia like adamantinoma in the lower leg.
Osteofibrous dysplasia and adamantinoma in children and. Osteofibrous dysplasia is a fibroosseous lesion also predominantly confined to the tibia with radiologic features similar to those of adamantinoma. Fibrous dysplasia in the maxillomandibular region case report cholakova r. Further investigations are needed for effective treatment of fibrous dysplasia. This study reported a case of osteofibrous dysplasia arising in the humerus of adult, resulting in good prognosis after a surgical treatment. It does not spread to other parts of the body and many cases are treated conservatively with careful observation over time.
Introduction osteofibrous dysplasia ofd is a rare, benign, selflimiting, fibroosseous lesion occurring in long bones especially of lower limbs. From the histopathology files at our institute, formalinfixed, paraffinembedded tissue blocks of seven cases of fibrous dysplasia and seven cases of osteofibrous dysplasia, which had been decalcified in hydrochloric acid for a maximum of 3 days, were used for this study. Most reports of osteofibrous dysplasia describe isolated tibial lesions, although a significant subgroup describe isolated and ipsilateral fibular. Osteofibrous dysplasia and fibrous dysplasia exhibit similar radiologic and histopathologic features. Adamantinoma from the greek word adamantinos, meaning very hard is a rare bone cancer, making up less than 1% of all bone cancers. The etiology of fibrous dysplasia has been linked to an activating mutation in the gene that encodes the. Ishida et al reported a duration of symptoms in 11 of 12 patients ranging from 2 months to 5 years, with an average of 14 months. Osteofibrous dysplasia and adamantinoma in children and adol.
Osteofibrous dysplasia is considered as a separate pathological entity in view of its different presentation and treatment, although histopathologically similar to ossifying fibroma. Given figure the radiological appearance and histological findings, a diagnosis of osteofibrous dysplasia like. Thyroid pathology 33, 34, 61 see additional file 4. The lesions occur principally in adults and are more common in males. Osteofibrous dysplasia ofd and adamantinoma are rare and most commonly arise in the tibia of young individuals. Campanacci described this condition in two leg bones, the tibia and fibula, and coined the term. To report long term treatment outcomes of osteofibrous dysplasia and association with adamantinoma. Surgery must be delayed for as long as possible in osteofibrous dysplasia of the ulna, as well as that of the tibia and fibula. Most recently, it has been surmised that ossifying fibroma and adamantinoma are on a continuum with osteofibrous dysplasia like adamantinoma representing an intermediate step between the two lesions. Extensive, deforming and unstable lesions require en bloc tumour resection and limb reconstruction. Osteofibrous dysplasia is a tumorlike bone lesion that usually presents as a painless swelling or anterior bowing of the tibia park et al. A clinicopathological and immunohistochemical study of 12 cases of osteofibrous dysplasia ofd, two cases of differentiated adamantinoma, and five cases of adamantinoma of long bones is presented. Frangenheim first described the lesion in 1921 and reported it as a congenital osteitis fibrosa.
This is the first report of a q227l mutation in fd, although it has been previously documented in pituitary adenoma. This condition should be differentiated from nonossifying fibroma and fibrous dysplasia of. Immufigurenohistochemical staining for keratin highlighted small clusters of epithelial cells 5. Key words bone tumor osteofibrous dysplasia ulna references 1. Osteofibrous dysplasia kempsoncampanaccis disease full. A comparative study of fibrous dysplasia and osteofibrous. Comparative study of fibrous dysplasia and osteofibrous. Best practice management guidelines for fibrous dysplasiamccune. It almost always occurs in the bones of the lower leg and involves both epithelial and osteofibrous tissue the condition was first described by fischer in 19. Some consider it synonymous with ossifying fibroma because of histological similarities, but it is generally considered a separate entity due to different presentation and treatment. Analysis of stromal cells in osteofibrous dysplasia and. Osteofibrous dysplasia is a rare, noncancerous benign tumor that affects the long bones.
A small percentage of the patients develop metastases, sometimes quite late in the course. From january 1984 to july 2001, 14 patients with osteofibrous dysplasia were followed for an average of 108 months 78 to 260 months. Osteofibrous dysplasia is a benign fibroosseous cortical lesion that occurs almost exclusively in the tibia and fibula. The etiology of the tumor is still a matter of debate. A comparative study of fibrous dysplasia and osteofibrous dysplasia with regard to expressions of cfos and cjun products and bone matrix proteins. The question has been raised if ossifying fibroma is a relative of fibrous dysplasia or a true neoplasm. Clinical, radiographical and histopathological findings will help in confirming the lesion. Osteofibrous dysplasia is a rare benign condition that afflicits the long bones it is also known as ossifying fibroma of long bones and congenital osteitis fibrosa. Treatment of osteofibrous dysplasia and associated lesions.
Congenital pseudoarthrosis of the tibia is thought to be a related lesion. Fibrous dysplasia osteofibrous dysplasia langerhans cell histiocytosis erdheim chester disease chest wall hamartoma joint lesions synovial chondromatosis aneurysmal bone cyst osteoid osteoma zbenign bone forming tumor. Dinkova department of oral surgery, faculty of dental medicine department of allergology, physiotherapy and clinical radiology medical university, plovdiv, bulgaria. Although ofd has typical histopathologic features, areas resembling ofd have often been noted at the periphery of otherwise classic adamantinomas, and some have suggested that ofd may be either a precursor to or a regressive phase of adamantinoma. Pdf the aim of this study was to describe mr findings of osteofibrous dysplasia. There is an association with neurofibromatosis type 1. Osteofibrous dysplasia is a rare, clinicopathologically distinct lesion whose variable nomenclature has led to unnecessary confusion. She denies fatigue, weight loss, and any other masses.
Osteofibrous dysplasia is a rare, benign nonneoplastic condition with no known cause. Osteofibrous dysplasia ofd and adamantinoma are rare bone tumors that are most often found in the tibia shinbone. 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. Typical radiographic findings of osteofibrous dysplasia reveal eccentric, fairly wellmarginated osteolytic lesions with a sclerotic border in the anterior cortex of the tibial diaphysis. The previously suggested relationship between adamantinoma and osteofibrous dysplasia is substantiated by this case. Ossifying fibromas of the craniofacial skeleton 123 4. Arrest in development of cortical bone, leading to lesions composed of irregular woven bone and immature fibroblastlike spindle cells gain of function mutations in gnas guanine nucleotidebinding protein. Fibrous dysplasia is a common benign skeletal lesion that may involve one bone monostotic or multiple bones polyostotic and occurs throughout the skeleton with a predilection for the long bones, ribs, and craniofacial bones.
Pdf fibrous dysplasia of the bones is an uncommon congenital skeletal. Our institution has treated 42 patients with adamantinomas since 1972 and has evaluated. Detection of gnas1 mutations by msred is a valuable adjunct to the histopathological diagnosis of fd. Osteofibrous dysplasia and adamantinoma have similar clinical presentations, as well as similar radiologic and pathologic findings. 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. Adamantinoma is a rare tumour, which most often affects the tibia and produces lytic and sometimes destructive lesions, which can cause fractures. Adamantinoma of long bones and osteofibrous dysplasia are rare, osteolytic primary bone tumours of uncertain origin containing areas of fibrous and fibroosseous proliferation. No mutations were detected in osteofibrous dysplasia. Adamantinoma is a primary lowgrade, malignant bone tumor that is predominantly located in the midportion of the tibia. The former lesion has a predilection for the cortex of the tibia.
The initial symptoms of adamantinoma are often indolent and nonspecific and depend on location and extent of the disease. Hatori m, watanabe m, hosaka m, sasano h, narita m, kokubun s. Apr 27, 2012 osteofibrous dysplasia and fracture callus can be difficult to differentiate, but the history and location should help, and they typically have prominent osteoblastic rimming around the bone trabeculae. An immunohistochemical study correlated to histogenesis. Mri of adamantinoma of long bones in correlation with histopathology fibrous dysplasia vs adamantinoma of the tibia. Fibrous dysplasia is a benign fibroosseous lesion, which may present in either monostotic or polyostotic forms. A total of 28 cases of osteofibrous dysplasia in 25 patients were managed at a referral center for pediatric bone tumors with observation in the first instance, then limited surgical intervention.
Mri of adamantinoma of long bones in correlation with histopathology. Pdf mri of adamantinoma of long bones in correlation with. 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 is a cortical not medullary lesion with osteoblastic rimming of bone and lamellar bone that does mature. We discuss tumifactive amyloid deposits, the brown tumours of hyperparathyroidism, the various types of cysts that can be seen in bone, nora lesion, subungual exostosis, haemophilic pseudotumors, nonossifying fibroma, fibrous dysplasia, osteofibrous dysplasia, paget disease, tophaceous gout. 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. Clinical and imagiological features the commonest fibrousosseous lesions of the orbit and sinonasal tract are of and fd. Osteofibrous definition is composed of bone and fibrous connective tissue. Adamantinoma, osteofibrous dysplasia and differentiated.
Intramuscular myxoma associated with fibrous dysplasia, orphanet encyclopedia. The diagnosis between these two entities may be challenging, because they share similar features. Osteofibrous dysplasia is a rare, nonneoplastic condition of unknown etiology that affects the long bones. Some consider it synonymous with ossifying fibroma because of his. Of three newborns with osteofibrous dysplasia of the tibia, two had swelling and one had pathologic fracture. Of the six cases of osteofibrous dysplasia immunostained with a cytokeratin antibody, two were cytokeratin positive. On histopathology the lesion was confirmed to be a osteofibrous lesion with fibrous tissue embedding osseous trabeculae bordered by osteoblast and zonal architecture continuous with the cortex.
Ossifying fibroma and fibrous dysplasia of the jaw are maxillofacial fibroosseous lesions that should be distinguished each other by a pathologist because they show distinct patterns of. Histopathological, immunohistochemical, argyrophilic nucleolar organizer region and dna ploidy analysis, pathology international on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. It is tender when bumped, but painless at rest and with everyday activities. Longterm outcome following treatment of adamantinoma and. A fibrous dysplasialike lesion with cortical destruction suggests lowgrade central osteosarcoma or malignant transformation. John hunter is a professor in the department of radiology musculoskeletal section at uc davis school of medicine. Adamantinoma ad is an ultimately rare, lowgrade malignant bone tumor. Nagasaki, japan and 7department of pathology, osaka university graduate school of medicine, suita. Abstract osteofibrous dysplasia ofd is a rare, benign, fibroosseous lesion that typically is seen within the cortex of the tibia in children. A case showing clinical, radiologic, and pathologic features of osteofibrous dysplasia of the tibia and fibula ossifying fibroma of the long bones associated with scattered epithelioid islands typical of adamantinoma is reported.
Komiya and inoue reported similar presenting complaints in a series of 10 cases. Fibrous dysplasia, osteofibrous dysplasia, and adamantinoma. Osteofibrous dysplasia ofd is a rare, benign, lesion that is typically seen in children. May 07, 2018 does osteofibrous dysplasia progress to adamantinoma and how should they be treated bone joint j. Pdf mri of adamantinoma of long bones in correlation. Fibrous dysplasia is a slowly progressing bone lesion resulting from displacement of the normal medullary bone with abnormal. An 11yearold child presented to the emergency department in 2005 with episodes of pain in his right clavicle. Pseudotumors of bone and bone lesions mimicking tumours. Osteofibrous dysplasia usually occurs in the first two decades of life. The diagnosis is usually not difficult, given the symptoms, radiology, and histology.
Adamantinoma ad is a rare, lowgrade malignant primary. Osteofibrous dysplasia ossifying fibroma of the tibia. Osfd to ensure longterm funding for the omim project, we have diversified our revenue stream. Osteofibrous dysplasia is a noncancerous tumor that typically develops during childhood. Subsequently, kempson reported two cases affecting the tibia of young children and named the lesion ossifying fibroma. A clinicopathological and immunohistochemical study of. He was extensively investigated and a diagnosis of fibrous dysplasia of the right clavicle was reached on histology. Cytokeratin expression and distribution in adamantinoma of the long bones and osteofibrous dysplasia of tibia and fibula.