You are in: eMedicine Specialties > Radiology > MUSCULOSKELETAL Fibrous Cortical Defect and Nonossifying FibromaArticle Last Updated: Nov 28, 2007AUTHOR AND EDITOR INFORMATIONSection 1 of 12
  Author: Stacy E Smith, MD, Associate Professor of Radiology, Division of Musculoskeletal Imaging, University of Maryland School of Medicine Stacy E Smith is a member of the following medical societies: American Association for Women Radiologists, American College of Radiology, American Roentgen Ray Society, Association of University Radiologists, and Radiological Society of North America Editors: Michael A Bruno, MD, Associate Professor, Departments of Radiology and Medicine, Pennsylvania State University College of Medicine; Director, Radiology Quality Management Services, Milton S Hershey Medical Center, Pennsylvania State University College of Medicine; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; Murali Sundaram, MBBS, FRCR, FACR, Consulting Staff, Department of Diagnostic Radiology, The Cleveland Clinic Foundation; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; Felix S Chew, MD, MBA, EdM, Professor, Department of Radiology, Vice Chairman for Radiology Informatics, Section Head of Musculoskeletal Radiology, University of Washington Author and Editor Disclosure Synonyms and related keywords: fibroxanthoma, nonossifying fibroma, NOF, fibrous cortical defect, FCD, benign fibrous histiocytoma, metaphyseal fibrous defect, metaphyseal supracondylar cortical defect, developmental defect, cortical avulsive irregularity, subperiosteal desmoid, periosteal desmoid, periostitis ossificans, cortical desmoid, cortical avulsive injury INTRODUCTIONSection 2 of 12
  BackgroundThe terms fibroxanthoma, nonossifying fibroma (NOF), fibrous cortical defect (FCD), and, less commonly, benign fibrous histiocytoma have all been used interchangeably in the radiology literature. NOF and FCD, however, are considered to be 2 distinct lesions with respect to size and natural history. Fibroxanthoma is the preferred term for the NOF lesion because it more accurately reflects the underlying pathologic findings. In the literature, FCD has a longer list of pseudonyms, including metaphyseal fibrous defect, metaphyseal supracondylar cortical defect, and developmental defect. FCDs have been mislabeled or described as cortical avulsive irregularities, subperiosteal or periosteal desmoid, a variant of periostitis ossificans, and cortical desmoid. The cortical desmoid—or cortical avulsive injury, as it is currently known—is believed to be separate from the cortical defect, and it has been related to repetitive stress at the attachment of the extensor tendon fibers along the linea aspera of the distal femur.1 Fibroxanthoma and FCDs are nonaggressive fibrous lesions of bone that are distinguished from one another historically by their size and natural history. Both are considered to be developmental defects and to be nonaggressive. They were first described by Lichtenstein and Jaffe in 1942,2 and they typically occurred within the metaphysis of growing long tubular bones in children, most commonly about the knee. Controversy continues regarding the correct terminology and etiology of these lesions. The distinction between FCD and fibroxanthoma is based on size and natural history. FCDs are asymptomatic, small (<3 cm), eccentrically located, metaphyseal cortical defects; most of these spontaneously disappear. However, some evolve and enlarge into fibroxanthomas. Conversely, fibroxanthomas (>3 cm) are larger, eccentric, intramedullary lesions that abut the cortex; they have a typical, superficial, scalloping pattern in the adjacent cortex. While these lesions also can heal spontaneously (with reactive bone filling in the central lucent fibrous tissue component), they can also persist, with interval growth that continues into adulthood. Typically, fibroxanthomas are asymptomatic. However, the larger lesions may become symptomatic, with a risk of pathologic fracture. Steiner suggested that these 2 lesions are secondary to cellular proliferation due to aberrations in local development.3 PathophysiologyBoth fibroxanthomas and FCDs are composed of spindle-shaped fibroblasts that are oriented in a cartwheel or storiform whorled pattern, with scattered giant cells (osteoclastlike multinucleated cells), foam (xanthoma) cells, and small amounts of collagen. Foam cells occur in 30-50% of cases and are more common in older lesions. Abundant hemosiderin in the cytoplasm of the fibroblast cells has been noted; cholesterol crystals also have been identified. At histologic analysis, no mitosis or pleomorphism is present to suggest malignancy. FrequencyUnited StatesThe exact incidence is unknown; however, 30-40% of all children may have one or more of these lesions at one time. In one study, Caffey reported a frequency of 36% in children.4 InternationalNo definite distinction has been reported between the frequency in the United States and that worldwide. Mortality/MorbidityBecause fibroxanthoma and FCD are nonaggressive fibrous lesions of bone, no increase in morbidity or mortality is noted with either one. RaceNo racial predilection exists. SexIn the literature, a slight male predominance is reported. Fibroxanthomas occur 2 times more frequently in males than in females. Also, FCDs have been reported in males twice as often as in females; however, 1 study revealed an 11:3 ratio of the incidence in males versus females. AgeBoth lesions occur in the developing skeleton. FCDs occur in younger patients, with presentation in those aged 4-8 years. Usually, FCDs are incidental findings on radiographs that are obtained for indications other than the evaluation of FCD.
AnatomyApproximately 90% of cases of both lesions involve the tubular long bones.5, 6 Common sites include the femur (most commonly the distal femoral metaphysis [38%]), the proximal and distal tibia (43%), and the knee (55%); most lesions occur around the knee.7 The tibial diaphysis is involved in only 10% of cases. The fibula is affected in 8-10% of cases, as noted in one series at the Armed Forces Institute of Pathology (AFIP). Both fibroxanthoma and FCDs are uncommon in the upper extremity; however, of those reported in the literature, 8% were in the humerus, and both radial and ulnar lesions were rare. Less common sites include the innominate bone, clavicle, skull, scapula, mandible, and small bones of the hands and feet. Typically, both lesions are metaphyseal and arise close to the physeal plate. The FCD arises within the cortex, whereas the fibroxanthoma arises eccentrically within the medullary cavity. Both lesions usually arise from the posterior wall of the tubular bone, and involvement of the medial rather than lateral osseous surface is characteristic. With healthy growth of the osseous skeleton, the lesions extend toward the shaft, and if they do not involute and disappear, they may extend into the diaphysis. An epiphyseal location is distinctly uncommon and has been reported only in unusual cases of multifocal lesions. Both lesions are more commonly solitary, and they can occur in similar locations. Multifocal lesions in one or more bones can occur with FCDs; however, multifocal fibroxanthomas are less common and, if present, have other clinical manifestations (see Clinical Details). Moser et al described lesions in clusters in the same bone, at opposite ends of the same bone, and at opposite sides of the joints.8 Clinical DetailsTypically, FCDs are asymptomatic and are detected only incidentally on radiographs obtained for reasons other than the evaluation of FCD. Fibroxanthomas also are characteristically asymptomatic; however, in larger lesions, mild pain may occur secondary to radiographically undetected microfractures that may eventually lead to painful and radiographically evident pathologic fractures.9 Although they are most commonly solitary lesions, multiple lesions have been described. Rare reports of multiple fibroxanthomas in patients with neurofibromatosis (5%) exist in the literature. The presence of extraskeletal congenital anomalies (eg, café-au-lait spots, mental retardation, hypogonadism or cryptorchidism, ocular abnormality, cardiovascular malformations) in association with multiple nonossifying fibroxanthomas constitute the clinical and radiologic spectrum known as Jaffe-Campanacci syndrome, which was first reported in 1983.10, 11 In one series, Moser et al also noted that the coexistent osteochondromas can be present.8 Preferred ExaminationPlain radiographic findings are virtually diagnostic of both FCDs and NOFs; most of these lesions have a characteristic location and appearance. If a discrepancy in appearance or age or an atypical associated symptom is present, advanced imaging (usually computed tomography [CT] or magnetic resonance imaging [MRI]) can be performed.12, 13, 14, 15 DIFFERENTIALSSection 3 of 12
Chondromyxoid Fibroma Fibrous Dysplasia Osteoid Osteoma Other Problems to Be ConsideredAlthough the radiographic findings usually are pathognomonic, the differential diagnosis includes the following: chondromyxoid fibroma; fibrous dysplasia, particularly if lesions are multiple and polyostotic (in comparison, fibrous dysplasia has a more central and unilateral location and more of a ground-glass appearance); osteoid osteoma (typically has a central nidus); bone abscess; periosteal chondroma; chondromyxoid fibroma; avulsive cortical irregularity or periosteal desmoid (saucerlike radiolucent defect in the cortex with adjacent sclerosis and periostitis); and desmoplastic fibroma. Reports of soft-tissue desmoid or desmoplastic fibromas of bone have been noted only when the fibrous tissue in these lesions is examined at biopsy. Although much less common, consider giant cell tumor and aneurysmal bone cysts in the differential diagnosis of a large lobulated fibroxanthoma at radiographic study. However, both of these lesions are clearly distinguished at CT or MRI. A herniation pit also has been described in the differential diagnosis of FCD. The cortical avulsive injury that occurs in adolescents has been noted more frequently. It often was and still is confused with FCD. At histologic evaluation, cortical avulsive injury has a nonspecific fibrous appearance with intermixed spicules of bone and healthy osteoblasts. Later findings indicated that the histologic features of the cortical avulsive lesion (characteristically located at the insertion of the tendon at the posteromedial cortex of the distal femur) included those of healthy tendon and interspersed bone and cartilage; therefore, this lesion is separate from FCD and secondary to repetitive trauma rather than being a true developmental defect. RADIOGRAPHSection 4 of 12
FindingsThe imaging features of fibroxanthomas are pathognomonic. Typically, a metadiaphyseal, eccentric, ovoid or round, intramedullary geographic radiolucency, with a sharply marginated sclerotic rim of reactive bone and a scalloped contour, is present. The medullary rim typically is thicker than the cortical rim. While they initially appear near the physeal plate, they eventually appear further down within the diaphysis, following a normal growth pattern. The epiphysis is never affected. Larger lesions can appear multiloculated, with dimensions as large as 4-7 cm, with erosion of the endosteal surface of the cortex.14, 15, 16, 17 Since fibroxanthomas are thought to evolve from the FCD, the typical FCD appears similar but smaller, with the following defining features:
While they typically are eccentric lesions within tubular bones, fibular lesions are an exception in that the entire diameter of the bone can be involved; this makes diagnosis slightly more difficult. Usually, fibroxanthomas are slow-growing lesions. Variations in size and density are secondary to interval growth and the natural resolution of the lesions; these processes include reactive bone formation, which can fill in and replace the central lucent defect over time. This increase in sclerosis can be confusing to the observer and often is mistaken for fibrous dysplasia when it is present in the midst of the progressive changes. Initially, some of the lesions may be confused with neoplasms if one is not aware of the typical location and spectrum of their appearances over time. Knowledge of these characteristics is particularly important if periosteal reaction is present, since periosteal reaction occurs only with a pathologic fracture through the lesion. Degree of ConfidenceThese nonaggressive fibrous lesions have a characteristic location and appearance and are rarely confused with other lesions if the observer is aware of the spectrum of appearances over time. The degree of confidence is virtually certain in most cases; further imaging is required only if atypical symptoms or associated injuries are present. False Positives/NegativesCortical avulsive injury to the distal posteromedial femur often has been confused with FCD. Usually, the former lesion has more cortical irregularity and is located at the direct insertion of the gastrocnemius muscle. They are more common in young athletes. These 2 may be part of a spectrum of the same type of lesion but, for now, are considered separately. CT SCANSection 5 of 12
FindingsAdvanced imaging usually is not required unless an atypical appearance or symptom is present. The characteristic lesion is eccentrically located, intramedullary, well marginated, and sclerotic, with a central lucency. Heterogeneous lucency and reactive sclerosis is identified at later stages in the case of the involuting fibroxanthoma. The central fibrous component has a nonspecific soft-tissue attenuation value; older lesions have a high attenuation value secondary to the reactive bone component. CT images are useful because they can clearly depict the cross-sectional extent of bone involvement and the degree of cortical thinning, features that can help in determining the risk of pathologic fracture. The literature states that a tubular bone with a fibroxanthoma has a high risk for fracture if the lesion involves more than 50% of the width or if it extends more than 33 mm. If a fracture has occurred, a mild periosteal reaction may be present with subtle soft-tissue swelling; otherwise, no soft-tissue component is seen with fibroxanthomas, and the margins are very well demarcated. With FCDs, the subtle apparent thickening of the cortical margin is noted with only subtle central cortical lucency because these lesions are very small in early disease. CT may help elucidate the irregular avulsive injury that is often confused with this lesion. Early differentiation between stress injuries caused by the adductor magnus muscle and the medial head of the gastrocnemius muscle, as well as clarification of the characteristic cortical defect, is possible with CT. When radiographic findings are not diagnostic, CT findings may obviate biopsy. Degree of ConfidenceBoth lesions are fairly characteristic, and advanced imaging rarely is performed. The degree of confidence is 99%. False Positives/NegativesFalse-positive findings may include very small cortical avulsive irregularities (see Findings). A small cortical osteoid osteoma may be confused as an FCD if the imaging sections are too thin and the nidus is not elucidated. However, clinical pain occurs with osteoid osteomas, which should not be confused with FCDs. MRISection 6 of 12
FindingsUsually, both fibroxanthoma and FCDs are noted incidentally on images obtained for reasons other than evaluation of these lesions. Kransdorf et al described their characteristic low signal intensity on both T1- and T2-weighted spin-echo MRIs.13 This finding is similar to that of other nonossified lesions that they reviewed. Low signal intensity on T2-weighted images was thought to represent hemosiderin deposits or large amounts of collagen, which are consistent with the fibrous tissue content. A peripheral hypointense rim represents the marginal sclerosis depicted on radiographs and the peripheral reactive sclerosis present at histologic evaluation. Bright areas can appear with sufficient cellularity. In a study by Jee et al, internal septa were noted in 95% of cases; these were correlated with subtle trabeculae depicted on the radiographs.12 Usually, MRI is not indicated in FCDs. However, study findings have shown that this intracortical lesion typically has low signal intensity on T1-weighted images and has increasing signal intensity on proton density– and T2-weighted images, which are consistent with their heterogeneous fibrous histologic characteristics. A rim of lower signal intensity represents the sclerotic border that abuts the medullary and cortical borders. Degree of ConfidenceBecause MRI is not commonly used in the evaluation of these lesions, data are limited. The degree of confidence with MRI alone is approximately 95%. False Positives/NegativesOther fibrous lesions can have the same signal intensity changes as those of fibroxanthoma and FCDs. However, the location and lack of pain should be helpful. ULTRASOUNDSection 7 of 12
FindingsTypically, ultrasonography is not used to evaluate these lesions. NUCLEAR MEDICINESection 8 of 12
FindingsMinimal-to-mild accumulation of bone-seeking pharmaceutical agents (eg, technetium-99m [99mTc]) occurs and is indicative of a benign process. The intensity of the uptake is much less than that of active bone lesions. In a child or adolescent, a typical eccentric region of agent uptake located a short distance from the physis of a tubular bone is diagnostic for fibroxanthoma in the appropriate setting. FCDs do not have uptake on bone scintigrams obtained with 99mTc. In children, this finding can be masked by the proximity of the lesion to the distal femoral epiphyseal plate, which itself has marked tracer uptake.1, 18, 19 Degree of ConfidenceData are limited. False Positives/NegativesCortical avulsive irregularities have increased tracer uptake in a location similar to that expected for a FCD. However, the former lesion has obvious uptake, unlike the usual pattern of no uptake in FCDs. Early osteosarcoma, particularly in the periosteal variant, can have 99mTc uptake within the distal posterior femur. Radiographic correlation helps in distinguishing between the aggressive lesion and benign FCD. ANGIOGRAPHYSection 9 of 12
FindingsAngiography is not performed in these lesions. At histologic evaluation, they are not vascular. INTERVENTIONSection 10 of 12
No specific treatment or intervention is required for FCDs, and they are usually left alone. They may persist into adulthood without complications and eventually become fibroxanthomas. However, if they are removed at biopsy, they do not recur. With respect to fibroxanthomas, small asymptomatic lesions do not require biopsy or treatment. With larger lesions, careful radiographic observation and decreased vigorous activity of the patient are recommended. Curettage and bone graft procedures are performed to prevent a pathologic fracture if the lesion becomes larger than 33 mm in diameter or involves more than 50% of the transverse diameter of a critical weight-bearing bone. Medical/Legal Pitfalls
Special Concerns
MULTIMEDIASection 11 of 12
REFERENCESSection 12 of 12
Fibrous Cortical Defect and Nonossifying Fibroma excerpt Article Last Updated: Nov 28, 2007 | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
