DOI: 10.1148/rg.231015088
(Radiographics. 2003;23:157-177.)
© RSNA, 2003
Cortical Lesions of the Tibia: Characteristic Appearances at Conventional Radiography1
Scott M. Levine, MD,
Robert E. Lambiase, MD and
Catherine N. Petchprapa, MD
1 From the Department of Diagnostic Imaging, Brown University Medical School, Rhode Island Hospital, 593 Eddy St, Providence, RI 02903 (S.M.L., R.E.L.); and the Department of Radiology, Harvard Medical School, Massachusetts General Hospital, Boston, Mass (C.N.P.). Recipient of a Certificate of Merit award for an education exhibit at the 2000 RSNA scientific assembly. Received April 18, 2001; revision requested July 26; final revision received May 15, 2002; accepted May 17. Address correspondence to R.E.L. (e-mail: rlambiase@lifespan.org).
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Abstract
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Lesions that involve the cortex of the tibia are fairly common in radiology practice. However, the number of diseases that involve the tibial cortex is great, and it can be difficult to arrive at a limited differential diagnosis from radiographic findings. Categorization of lesions of the tibia into those that cause cortical destruction and those that cause cortical proliferation can help narrow the broad differential diagnosis. Lesions that cause cortical destruction include nonossifying fibroma, fibrous dysplasia, osteofibrous dysplasia, aneurysmal bone cyst, giant cell tumor, eosinophilic granuloma, Ewing sarcoma, neurofibromatosis, adamantinoma, osteoblastoma, chondromyxoid fibroma, hemangioendothelioma, renal cell metastatic disease, hemangioma, and hemangiopericytoma. Lesions that cause cortical proliferation include osteochondroma, stress fracture, osteoid osteoma, periosteal osteogenic sarcoma, diaphyseal dysplasia, venous stasis, cellulitis, chronic osteomyelitis, osteopathia striatum, and melorheostosis. Conventional radiography along with clinical and pathologic data can aid in diagnosis of the wide variety of disease processes that involve the tibial cortex.
© RSNA, 2003
Index Terms: Tibia, abnormalities, 45.15, 461.15, 45.20, 461.20, 45.80, 461.80 • Tibia, fractures, 45.415, 461.415 • Tibia, neoplasms, 45.30, 461.30
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LEARNING OBJECTIVES FOR TEST 5
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After reading this article and taking the test, the reader will be able to:
- Describe the broad differential diagnosis for lesions that occur in the tibial cortex.
- Recognize the entities that are highly characteristic of or specific to the tibial cortex.
- Discuss the entities that are cortically destructive and those that are proliferative and the consequent differential diagnosis.
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Introduction
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In our clinical practice, cortical lesions of the tibia are relatively common findings. However, they seem to engender disproportionate confusion, probably due to the plethora of potential diagnoses, some of which are typical of if not unique to the tibia. We have assembled a gamut of cortically based tibial lesions in an attempt to demystify the involved differential diagnoses. These are divided into lesions that cause cortical destruction and lesions that cause cortical proliferation, as we find this distinction (when it can be made) helpful in limiting the differential diagnosis. The entities are presented in an order roughly based on the frequency of occurrence; however, some entities have been grouped together on the basis of shared radiographic findings.
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Lesions That Cause Cortical Destruction
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Nonossifying Fibroma
In nonossifying fibroma, the patient age is typically less than 20 years. The male-to-female ratio is usually 2:1. The location is metaphyseal, and the epicenter is eccentric. The appearance consists of a well-defined, lytic lesion.
Commonly found in long tubular bones, particularly the tibia (43%) and femur (38%), nonossifying fibromas are seen on radiographs of nearly 50% of asymptomatic boys and 20% of girls over the age of 2 years. The lesions are lucent and occur in the metaphysis or metadiaphysis close to the growth plate (Figs 1, 2). They are more likely to involve the posterior or medial cortices. With growth and remodeling, the lesions can be seen to "migrate" into the diaphysis and usually subsequently fill in with fibro-osseous ingrowth, becoming radiopaque. Occasionally, they enlarge rather than heal. The lesions are typically referred to as fibrous cortical defects if less than 2.0 cm in diameter and confined to the cortex and as nonossifying fibromas when greater than 2.0 cm in diameter. The latter often expand into the medullary cavity. Over time, fibrous cortical defects or nonossifying fibromas may enlarge, become smaller, become sclerotic, or disappear altogether.
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Figure 1. Nonossifying fibroma in a 25-year-old man with knee pain after minor trauma. Radiograph shows an eccentric, well-defined, lytic lesion with a bubbly appearance in the metadiaphysis, an appearance consistent with nonossifying fibroma.
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Figure 2. Multiple nonossifying fibromas in a 14-year-old boy with pain in the left lower extremity after minor trauma. Radiograph shows eccentric, mildly expansile, bubbly lesions within the proximal and distal tibial metadiaphyses. In cases such as this, distinction from fibrous dysplasia can be difficult with radiography. The eccentric, cortical nature of each individual lesion should suggest the proper diagnosis. In this case, biopsy was performed both proximally and distally due to the presence of pain.
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Histologically, whorled, spindle-shaped fibroblasts within the bone cortex yield a lytic eccentric lesion bordered by sclerotic or normal bone within the metadiaphysis or metaphysis. The margins are typically well-defined with a scalloped or serpentine configuration. When these findings are noted in a child or young adult, the radiographic appearance is diagnostic. When the lesions become large, they appear expansile and multiloculated. Pathologic fracture is rare. An associated soft-tissue mass or periostitis should prompt further evaluation to exclude a more aggressive tumor (1).
Jaffe-Campanacci syndrome, first described in 1983, is a syndrome of multiple nonossifying fibromas associated with extraskeletal congenital abnormalities, which include café au lait spots, mental retardation, ocular anomalies, skin lesions, cryptorchidism or hypogonadism, and others. There have been only about a dozen reported cases (2). Most have a prominent clinical presentation; this syndrome should not routinely be included in the differential diagnosis of multiple nonossifying fibromas.
Fibrous Dysplasia
In fibrous dysplasia, the patient age is typically 20–30 years. The male-to-female ratio is usually 1:1. The location is diaphyseal, and the epicenter is centric or eccentric. The appearance consists of ground-glass lucency, irregular but well-defined borders, and a mildly expansile lesion. It can involve multiple bones.
A sporadically occurring skeletal abnormality, fibrous dysplasia is the result of locally abnormal osteoblasts. Histologically, benign fibrous tissue of variable thickness and poorly formed trabeculae in varying amounts result in the wide spectrum of radiographic presentations. Lesions may be solitary (70%–80%) or multiple (20%–30%). Patients typically present in their 2nd or 3rd decade, with symptoms appearing early and being more severe in the polyostotic form.
Any bone can be affected. Long bone involvement is often intramedullary and diaphyseal in location, with rare epiphyseal involvement. The lesions may be central or eccentric (Fig 3). They are typically well-defined and of variable radiopacity, depending on the relative proportion of fibrous and osseous tissue in the lesions. Some lesions have a characteristic ground-glass appearance. This is particularly useful in the computed tomographic assessment of fibrous dysplasia, where the diagnosis is often suggested by the characteristic appearance of the matrix. The hazy appearance is secondary to randomly distributed trabeculae, without the well-organized architecture demonstrated in most trabecular bone (3). There is typically a leading edge of sclerosis or thick cortex around the periphery of the lesions (the "rind" sign). More expansile lesions will scallop the endosteal surface, thinning and weakening the cortex and making it prone to pathologic fracture. Chronic changes secondary to bone weakening may be seen, with bowing of weight-bearing structures, fracture, and remodeling. Such are the forces that produce the characteristic shepherd’s crook deformity, so well known in the femur.
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Figure 3a. Fibrous dysplasia in a 44-year-old woman with ankle discomfort. Anteroposterior (a) and lateral (b) radiographs show a typical ground-glass matrix, which is due to lack of normal cortical and trabecular organization.
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Figure 3b. Fibrous dysplasia in a 44-year-old woman with ankle discomfort. Anteroposterior (a) and lateral (b) radiographs show a typical ground-glass matrix, which is due to lack of normal cortical and trabecular organization.
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Osteofibrous Dysplasia
Osteofibrous dysplasia is sometimes referred to as ossifying fibroma. The latter term is discouraged, as it refers also to an unrelated lesion of the mandible. Osteofibrous dysplasia involves the tibia in approximately 90% of cases. The lesions are usually diaphyseal, especially involving the middle to distal third of the shaft and typically involving the anterior cortex. Patients present in the 1st or 2nd decade. Bowing and enlargement of the bone are typically seen, as is intracortical osteolysis, often with a characteristic adjacent sclerotic band (Fig 4) (4).
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Figure 4a. Osteofibrous dysplasia in a 4-year-old child. (a) Lateral radiograph of the right tibia shows a midtibial lesion with bowing. Note the intracortical osteolysis (white arrow) and adjacent sclerotic band (black arrow), which are characteristic of osteofibrous dysplasia. (b) Follow-up radiograph obtained 18 months later shows regression of the lesion without progression to pseudoarthrosis. Such healing is more common in osteofibrous dysplasia than in those lesions associated with neurofibromatosis.
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Figure 4b. Osteofibrous dysplasia in a 4-year-old child. (a) Lateral radiograph of the right tibia shows a midtibial lesion with bowing. Note the intracortical osteolysis (white arrow) and adjacent sclerotic band (black arrow), which are characteristic of osteofibrous dysplasia. (b) Follow-up radiograph obtained 18 months later shows regression of the lesion without progression to pseudoarthrosis. Such healing is more common in osteofibrous dysplasia than in those lesions associated with neurofibromatosis.
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Distinction between fibrous dysplasia, adamantinoma, and osteofibrous dysplasia can be difficult. This distinction can be made pathologically, as osteofibrous dysplasia demonstrates unique osteoblastic rimming and bone zonation, which are not seen in the other entities (5). Controversy does exist as to the possibility that osteofibrous dysplasia is in essence an early adamantinoma or is prone to transformation. Contemporary theory would consider the two as distinct entities (5,6).
It has been suggested that osteofibrous dysplasia has a more favorable prognosis than either fibrous dysplasia or adamantinoma, as it exhibits a tendency toward spontaneous regression without residual skeletal deformity (4).
Aneurysmal Bone Cyst
In aneurysmal bone cyst, the patient age is typically less than 20 years. The male-to-female ratio is 1.2:1. The location is usually metaphyseal, and the epicenter is eccentric. The appearance consists of an osteolytic, expansile, well-defined lesion with an occasional trabeculated pattern.
An aneurysmal bone cyst is an expansile lesion that contains blood-filled cystic cavities. Several hypotheses have been proposed with respect to its pathogenesis. Altered hemodynamics secondary to a coexistent lesion or a traumatic injury seems to be a common denominator, a finding substantiated by the presence of angiographically evident prominent arteriovenous shunting, as well as a histologic residual of nonrelated tumor in about 25% of cases examined pathologically (2).
The average patient is under 20 years of age, although lesions have been seen in patients as young as 3 years and as old as 70 years. Patients typically experience pain and swelling.
Long tubular bones and the spine account for 60%–70% of cases. At radiography, aneurysmal bone cysts are well-defined, cortically based, rapidly expansile lytic lesions. They can grow large enough to involve the medullary cavity, although their tendency is for eccentric expansion (a so-called blister lesion) (Figs 5, 6). Extremely rapid growth is characteristic of aneurysmal bone cysts, as expansion has more to do with vascular engorgement than with cellular proliferation, and aids in its differentiation from other eccentric, expansile lesions of bone (2). Aneurysmal bone cysts are metaphyseal in location when they involve the long bones. Purely diaphyseal lesions are seen in 8% of cases. Extension into the epiphysis is even more rare.
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Figure 5. Aneurysmal bone cyst in a 13-year-old girl with a painful, expanding mass around the knee. Anteroposterior radiograph shows an osteolytic, expansile lesion in the proximal tibial metaphysis. Although aneurysmal bone cysts are typically eccentric in location, they can appear to be medullary based when very large.
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Figure 6. Aneurysmal bone cyst in a 14-year-old boy with pain in the right midtibia. Radiograph shows an expansile lytic lesion that is more characteristically eccentric. Even with rapid expansion, a shell of cortical bone surrounding the lesion is usually discernible.
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Giant Cell Tumor
In giant cell tumor, the patient age is typically 20–40 years (65% of cases). The male-to-female ratio is usually 1:1. The location is epiphyseal, and the epicenter is eccentric. The appearance consists of a lytic, eccentric, well-defined lesion with a nonsclerotic border.
Giant cell tumor is a relatively common bone tumor, most often occurring in the 3rd and 4th decades of life. The lesion usually manifests as pain, limitation of motion, and swelling. Seventy to 90% occur in long, tubular bones, with 25% occurring in the tibia. The lesion is by far most common in the mature skeleton following physeal closure, at which time it is a predominantly epiphyseal lesion with secondary extension into the metaphysis (Fig 7). In those rare cases of giant cell tumor that occur prior to physeal closure, the lesions tend to be metaphyseal in location. The lesions incite no periosteal or reactive bone formation. The most characteristic radiographic finding is a predilection for subchondral bone. They tend to be eccentric. Many possess a trabeculated appearance, which is the result of the destructive pattern of the host bone and not of trabeculation within the tumor matrix (7).
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Figure 7a. Giant cell tumor in a 28-year-old man with pain in the right knee. Anteroposterior (a) and lateral (b) radiographs show a lytic lesion (arrows) that extends from the subchondral epiphysis into the metaphysis. Such a location is typical of giant cell tumor, which has a predilection for subchondral bone. The age of onset, an epiphyseal epicenter, and minimal expansion often allow differentiation of this tumor from aneurysmal bone cyst with radiography.
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Figure 7b. Giant cell tumor in a 28-year-old man with pain in the right knee. Anteroposterior (a) and lateral (b) radiographs show a lytic lesion (arrows) that extends from the subchondral epiphysis into the metaphysis. Such a location is typical of giant cell tumor, which has a predilection for subchondral bone. The age of onset, an epiphyseal epicenter, and minimal expansion often allow differentiation of this tumor from aneurysmal bone cyst with radiography.
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At histologic analysis, multinucleated giant cells are interspersed in a fibrous stroma. The multinucleated giant cells are nonspecific and are seen in many processes that result in bone destruction. The mononuclear stromal cells are probably the cells of tumor origin (7). These are graded histologically as grade 1, 2, and 3 in order of increasing anaplasia. Grade 3 tumors are thought to be malignant. The true prevalence of malignant giant cell tumors is difficult to ascertain, as there is confusion in the literature due to the historic inclusion of a variety of tumors under the (mis)diagnosis of giant cell tumor based solely on their possession of giant cells. The true prevalence of primary malignant giant cell tumor is probably on the order of 5%–10% (7). Both the radiographic appearance and the histologic appearance poorly predict the clinical behavior of the tumor.
Radiographic distinction from aneurysmal bone cyst is based on an epiphyseal epicenter, involvement of subchondral bone, a central versus cortical location, significantly less bone expansion, and later age of onset.
Eosinophilic Granuloma
In eosinophilic granuloma, the patient age is typically less than 20 years. The male-to-female ratio is usually 2:1 (for single lesions). The location is diaphyseal or metaphyseal, and the epicenter is centric. The appearance consists of a lytic lesion with variable bone destruction; the lesion may appear aggressive.
Skeletal lesions in eosinophilic granuloma involve the long bones, as well as the skull, mandible, spine, and ribs. Patients are typically children or young adults and experience local pain and soft-tissue swelling. Lucent areas of variable aggressiveness within the long bones are most commonly diaphyseal and metaphyseal in location (Fig 8) (8). Although epiphyseal involvement is much less common, epiphyseal lesions are capable of crossing an open physis. When lesions become large, endosteal erosion with periostitis can occur, mimicking osteomyelitis, Ewing sarcoma, or lymphoma.
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Figure 8a. Eosinophilic granuloma in a 16-year-old boy with pain in the right calf. Oblique (a) and anteroposterior (b) radiographs show a central osseous area of increased opacity (arrow) surrounded by lysis, the so-called button sequestrum. This finding is rarely seen in the long bones, being more typical in skull lesions of eosinophilic granuloma. Also note the subtle periostitis on the anteroposterior radiograph (b).
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Figure 8b. Eosinophilic granuloma in a 16-year-old boy with pain in the right calf. Oblique (a) and anteroposterior (b) radiographs show a central osseous area of increased opacity (arrow) surrounded by lysis, the so-called button sequestrum. This finding is rarely seen in the long bones, being more typical in skull lesions of eosinophilic granuloma. Also note the subtle periostitis on the anteroposterior radiograph (b).
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Eosinophilic granuloma is usually considered an inflammatory histiocytosis. The histologic architecture is reminiscent of that of a granuloma (9). Noted on histologic sections are multinucleated phagocytic giant cells, eosinophilic leukocytes, and mononuclear histiocytes.
Ewing Sarcoma
In Ewing sarcoma, the patient age is usually under 30 years (typically early adolescence). The male-to-female ratio is 3:2. It occurs in any portion of the bone, and the epicenter is centric. The appearance consists of an aggressive, permeative lesion, often with associated periostitis or a soft-tissue component.
Radiographic diagnosis of Ewing sarcoma offers a particular challenge owing to the myriad of possible radiographic manifestations. It can involve almost any bone in the body; however, the long tubular bones are involved in slightly more than one-half of cases. The tibia is the third most common site after the femur and sacrum, being involved in 11% of cases. Approximately 20%–35% of cases involve the diaphysis alone, and epiphyseal extension is seen in about 10% (2).
Ewing sarcoma demonstrates highly aggressive biologic activity, which is reflected in its radiographic appearance (Fig 9). It is a permeative lesion that often elicits multilayered periostitis. A small number of patients (1%–2%) present with the initial radiographic finding of "saucerization," that is, secondary extrinsic erosion of the cortex as a result of periosteal extension of tumor. Soft-tissue involvement is often present. Disseminated disease is reported to occur in 15%–30% of cases at presentation, and aggressive treatment combines surgery, chemotherapy, and radiation therapy. However, tumor recurrence is still seen in nearly one-fourth of patients (10).
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Figure 9a. Ewing sarcoma in a 17-year-old girl with discomfort in the right calf. Anteroposterior (a) and lateral (b) radiographs show subtle infiltrative osteolysis, cortical "tunneling," and linear periostitis, all of which indicate a biologically aggressive process.
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Figure 9b. Ewing sarcoma in a 17-year-old girl with discomfort in the right calf. Anteroposterior (a) and lateral (b) radiographs show subtle infiltrative osteolysis, cortical "tunneling," and linear periostitis, all of which indicate a biologically aggressive process.
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Histologically, the cell of origin is still controversial, although most evidence seems to point to a reticuloendothelial cell lineage or the undifferentiated bone marrow mesenchymal cell (10). Most patients are below the age of 30 years, with most between 10 and 15 years of age. Ewing sarcoma is slightly more common in males than females and is extremely rare in the black community.
Neurofibromatosis
Neurofibromatosis occurs at all ages. The male-to-female ratio is 1:1. The appearance is variable and includes cystic lesions; pseudoarthrosis of the tibia is characteristic.
Along with a wealth of other radiographic skeletal manifestations, neurofibromatosis sometimes involves the tibia in two distinct ways: pseudoarthrosis and multiple cortical lucencies around the knee.
Pseudoarthrosis.—
A basic defect in the mesoderm results in abnormal bone that is prone to bowing deformities, pathologic fracture, and poor healing, leading to pseudoarthrosis (Fig 10). This is most common in the tibia. Such defects rarely are the result of intraosseous neurofibromas. They occasionally result from reactive bone due to prior subperiosteal hemorrhage or to a contiguous extraosseous soft-tissue lesion such as a neurofibroma. This poorly organized bone frequently becomes incorporated into the growing host bone (3). The lesions tend to involve the distal half of the diaphysis and be refractory to treatment. Knowledge of the clinical history aids significantly in making the radiographic diagnosis. However, solitary pseudoarthrosis involving the tibia in a child may be the first clinical manifestation of neurofibromatosis.
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Figure 10. Pseudoarthrosis of the tibia in a 7-year-old boy with neurofibromatosis who presented with leg swelling and deformity. Radiograph shows tibial pseudoarthrosis and a nonhealed fracture of the distal fibula.
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Cystic Lesions around the Bone.—
Rarely, neurofibromatosis manifests as multiple cortically based lucencies that are mildly expansile and are radiographically similar to nonossifying fibroma (Fig 11). This manifestation is most common about the knees. The origin of these lesions is controversial. They may represent incorporated cortical neurofibromas, be true nonossifying fibromas, or represent foci of mesenchymal dysplasia (11). The greater size and large number of lesions is characteristic for this presentation of neurofibromatosis, in distinction to single or multiple nonossifying fibromas or fibrous cortical defects in the nonaffected patient.
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Figure 11. Cystic lesions in a patient with neurofibromatosis. Radiograph shows multiple well-defined, slightly expansile areas of lucency around the knee. Their origin is controversial, although the radiographic appearance is consistent with multiple nonossifying fibromas.
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Adamantinoma
In adamantinoma, the patient age is usually 20–50 years. The male-to-female ratio is 1.3:1. The location is most commonly the tibia in the anterior cortex and the middle third of the diaphysis. The epicenter is typically eccentric. The appearance consists of a multilocular or slightly expansile osteolytic lesion, which may be locally aggressive.
Adamantinoma is a rare, locally aggressive lesion that is usually 3.0–15.0 cm in diameter when discovered. It has a strong predilection for the tibia, which is the site of involvement in 80% of cases. It has a particular propensity for the anterior tibial diaphysis.
There are several hypotheses postulated for its pathogenesis (5). Displacement of basal epithelium during embryonic development is currently favored and is supported by the anterior tibial predominance, where enchondrally formed bone is closest to the skin surface. Adamantinoma is an epithelial lesion, at times containing fibro-osseous or Ewing sarcoma–like cell components. It is most often diaphyseal in location, although occasionally the lesion can extend to involve the metaphysis. It rarely involves the metaphysis or epiphysis exclusively.
The typical presentation is gradual onset of dull pain. A low-grade malignancy, adamantinoma has the ability to metastasize. Fifteen percent of patients die with metastases, commonly to the lung, bone, lymph nodes, pericardium, and liver. Treatment involves wide en bloc resection; the disease appears to be more aggressive when recurrent after therapy.
The most characteristic radiographic feature is the location: the middle anterior cortex of the tibial diaphysis (Figs 12–14) (2). Adamantinoma can be central or eccentric; it can also be multilocular or expansile. Lesions can be sharply or poorly marginated lytic lesions. Periostitis is rarely seen without an associated pathologic fracture. Satellite lesions are not uncommon and aid in differential diagnosis.
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Figure 12. Adamantinoma in a 19-year-old woman with calf pain. Lateral radiograph of the right tibia shows an appearance that is not readily distinguishable from that of fibrous dysplasia. Satellite lesions are not uncommon with adamantinoma.
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Figure 13a. Adamantinoma in a 29-year-old man with pain. Anteroposterior (a) and lateral (b) radiographs of the left tibia show a well-defined, slightly expansile intracortical area of lucency in the anterior middiaphysis. The location is typical of adamantinoma.
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Figure 13b. Adamantinoma in a 29-year-old man with pain. Anteroposterior (a) and lateral (b) radiographs of the left tibia show a well-defined, slightly expansile intracortical area of lucency in the anterior middiaphysis. The location is typical of adamantinoma.
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Figure 14. Adamantinoma in a 21-year-old woman with pain. Radiograph of the left tibia shows mixed sclerosis and lysis in the middiaphysis. Radiographic distinction between fibrous dysplasia and adamantinoma can be difficult. Note the minimal expansion and the endosteal scalloping.
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Fibrous dysplasia can appear very similar to adamantinoma, both radiographically and pathologically in selected histologic fields. These similarities led to an early misconception that adamantinoma represents malignant transformation of fibrous dysplasia. Modern histologic and cytologic techniques have proved that the two are distinct and nonrelated entities (5).
Osteoblastoma
In osteoblastoma, the patient age is typically 10–30 years. The male-to-female ratio is usually 2:1. The spine and sacrum are the most common locations, but it can occur in any bone. The epicenter is centric. The appearance is very variable and may be lytic or blastic.
Osteoblastoma is a rare, benign, osteoid-elaborating tumor. It is a distinct entity from osteoid osteoma and not simply a large version of the latter (12), although occasionally osteoblastoma will have the radiographic appearance of a large osteoid osteoma (Fig 15). Typically, osteoblastomas are greater than 2.0 cm in diameter. At radiography, they are highly variable and may be blastic, lytic, or mixed lytic-blastic. The lesions may be well-defined, exophytic, or very aggressive in radiographic appearance.
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Figure 15. Osteoblastoma in an 11-year-old boy with pain after mild trauma. Anteroposterior radiograph of the left tibia shows a well-defined cortical area of lucency with surrounding sclerosis in the proximal diaphysis. The appearance is not unlike that of a large osteoid osteoma. The radiographic appearances of osteoblastomas can be quite variable, and the diagnosis is usually based on biopsy results.
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Distinction from osteogenic sarcoma may be difficult at both histologic analysis and radiography. At histologic analysis, osteoblastoma is characterized by pure osteoid and woven bone production, occasionally with focal osteoblastic rimming, something that is essentially never seen with osteogenic sarcoma (12).
A small percentage of osteoblastomas fall into the category of crossover lesions. These lesions are locally aggressive clinically as well as radiographically aggressive but do not metastasize. Controversy exists as to whether these represent low-grade osteogenic sarcomas with low metastatic potential, malignant transformation of a previous osteoblastoma, or a distinct subpopulation of true osteoblastomas (12).
Chondromyxoid Fibroma
In chondromyxoid fibroma, the patient age is typically 20–30 years. The male-to-female ratio is usually 1:1. The location is metaphyseal, and the epicenter is eccentric. The appearance consists of a lucent, well-defined, slightly expansile lesion with endosteal sclerosis; it is oriented along the long axis of the host bone.
Chondromyxoid fibroma is a rare, benign, cartilaginous neoplasm. It typically manifests as an eccentric, lucent, metaphyseal lesion oriented along the long axis of a bone (Fig 16). When the lesion grows large, a "cortical bite" (2) without periostitis is characteristic. Chondromyxoid fibroma affects long bones in 80% of cases, 55% of which involve the tibia and femur. In the tibia, proximal metaphyseal involvement is most common.
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Figure 16a. Chondromyxoid fibroma in a 16-year-old boy with pain and swelling. Anteroposterior (a) and lateral (b) radiographs of the left tibia show a mildly expansile eccentric focus of osteolysis in the proximal metaphysis. The lesion is oriented along the long axis of the bone.
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Figure 16b. Chondromyxoid fibroma in a 16-year-old boy with pain and swelling. Anteroposterior (a) and lateral (b) radiographs of the left tibia show a mildly expansile eccentric focus of osteolysis in the proximal metaphysis. The lesion is oriented along the long axis of the bone.
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At histologic analysis, variable proportions of fibrous, chondroid, and myxoid tissue are seen. Chondroid is thought to be the cell of origin, and it has been suggested that the name be changed to fibromyxoid chondroma to clarify the cell of origin (13).
Chondromyxoid fibroma is a benign lesion, and surgical resection is performed for cure (14). The 10%–15% recurrence rate is postulated to be the result of inadequate resection (13). Metastases do not occur, as this is a benign process. However, benign implants or seeding has been described (14). These differ from metastatic deposits in that they possess a limited growth potential. On occasion, it may be difficult to distinguish chondromyxoid fibroma from chondrosarcoma at histologic analysis; previously reported cases of chondromyxoid fibroma with metastases probably represent incorrectly classified low-grade chondrosarcomas.
Hemangioendothelioma
In hemangioendothelioma, the patient age is typically 30–50 years. The male-to-female ratio is usually 2:1. The location is diaphyseal or metaphyseal, and the epicenter is centric or eccentric. The appearance consists of lytic, often multiple lesions, characteristically in a regional distribution.
Hemangioendothelioma (angiosarcoma) is a malignant bone tumor most likely to affect men in the 3rd to 5th decades. It most commonly affects the tibial diaphysis or metaphysis. It is distinctly multicentric in 20%–50% of cases, involving multiple bones or multiple sites within the same bone (Fig 17). Lesions are lytic; reactive sclerosis is uncommon. Margins may be well-defined or ill-defined.
At pathologic analysis, the lesion is composed of irregular, anastomosing vascular channels. The cell of origin is the endothelial cell, unlike in hemangiopericytoma, where the cell of origin is the pericyte (15). Hemangioendothelioma often arises in abnormal bone, such as is seen in the setting of chronic osteomyelitis, prior radiation treatment, or sites of prior metallic fixation.
A regional pattern of involvement is characteristic, which also may be seen in acquired immunodeficiency syndrome–related Kaposi sarcoma. This is thought to result from the venous drainage patterns of long bones, which favor metaphyseal-to-metaphyseal venous flow. These entities have a similar pattern of metastatic spread, that is, metachronous implantation rather than multicentric (synchronous) development (16).
The distinction from the rare cystic angiomatosis arises from the congenital nature of the latter, which is secondary to abnormal development of early vascular channels. The skeletal lesions of cystic angiomatosis tend to be well-defined lucencies, often with a sclerotic rim, with a widespread skeletal distribution. Organ involvement in cystic angiomatosis is a much greater clinical concern than is the skeletal involvement (2).
Renal Cell Metastatic Disease
Skeletal metastases are common in renal cell carcinoma. The metastatic focus may manifest many years following nephrectomy. Most commonly involved are the thoracolumbar spine, pelvis, ribs, and femur. Solitary lesions are frequent.
At radiography, poorly defined lytic lesions are the most common manifestation. Occasionally, the lesions are expansile and demonstrate some septation or trabeculation (17). A purely cortical location is quite rare (Fig 18).
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Figure 18. Metastatic renal cell carcinoma in a 70-year-old man with calf pain 1 year after nephrectomy for renal cell carcinoma. Anteroposterior radiograph of the right tibia shows an expansile cortical lesion. A single osteolytic skeletal metastatic focus is common in renal cell carcinoma; however, an expansile cortical lesion is unusual.
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Hemangioma
In hemangioma, the patient age is typically 40–50 years. The male-to-female ratio is usually 1:2. The location is epiphyseal and metaphyseal, and the epicenter is eccentric or centric. The appearance consists of trabeculated, slightly expansile, lytic lesions.
Intraosseous hemangiomas are most commonly seen in women between the 4th and 5th decades. Although intraosseous hemangiomas tend to involve the bones of the skull, face, and spine, long bone involvement is occasionally seen and is most likely to be found in the femur, tibia, and humerus (15). The classic findings on plain radiographs include a lucent, slightly expansile lesion in the epiphysis or metaphysis with latticelike trabeculation; however, findings are variable (Fig 19a).
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Figure 19a. (a) Hemangioma in a 40-year-old woman who experienced minor trauma. Radiograph shows an eccentric focus of osteolysis in the middiaphysis with a coarse trabecular appearance. Latticelike trabeculae, which are commonly seen in the skull, can suggest the diagnosis of hemangioma. (b) Hemangioma in a 55-year-old woman with calf pain. Anteroposterior radiograph of the left tibia shows a small focus of osteolysis (arrow) in the medial diaphyseal cortex. Intracortical hemangiomas are quite rare and mimic osteoid osteoma, stress fracture, and intracortical abscess, as well as the very rare intracortical osteogenic sarcoma.
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Figure 19b. (a) Hemangioma in a 40-year-old woman who experienced minor trauma. Radiograph shows an eccentric focus of osteolysis in the middiaphysis with a coarse trabecular appearance. Latticelike trabeculae, which are commonly seen in the skull, can suggest the diagnosis of hemangioma. (b) Hemangioma in a 55-year-old woman with calf pain. Anteroposterior radiograph of the left tibia shows a small focus of osteolysis (arrow) in the medial diaphyseal cortex. Intracortical hemangiomas are quite rare and mimic osteoid osteoma, stress fracture, and intracortical abscess, as well as the very rare intracortical osteogenic sarcoma.
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A much rarer lesion is the intracortical hemangioma (Fig 19b). At radiography, these are small, well-defined osteolytic lesions that may be associated with cortical thickening or periostitis (18). The differential diagnosis includes stress fracture, osteoid osteoma, or cortical abscess.
Hemangiopericytoma
In hemangiopericytoma, the patient age is typically 50–60 years. The male-to-female ratio is 1.8:1. The location is typically the soft tissues; if it is intraosseous, the spine, sacrum, and long tubular bones are affected. Extrinsic cortical destruction can occur if there is a soft-tissue epicenter. The appearance consists of aggressive bone destruction.
Hemangiopericytoma is typically a soft-tissue tumor. Rarely, it has an intraosseous origin. The tumor may be benign or malignant. It is very difficult to predict subsequent behavior on the basis of its radiographic appearance or histologic fea-tures. A soft-tissue or parosteal site of origin can result in extrinsic cortical bone destruction (Fig 20).
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Figure 20. Hemangiopericytoma in a 40-year-old man with calf pain and swelling. Anteroposterior radiograph of the left proximal calf shows a mass centered in the soft tissues with scattered calcifications within the matrix. Hemangiopericytomas typically arise from the soft tissues and result in extrinsic bone destruction.
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The cell of origin is thought to be the vascular pericyte. No distinct cytologic features of the pericyte exist, and thus pathologic diagnosis is based on the overall architecture of the tumor. Many thin-walled blood vessels are seen, surrounded by closely packed stromal cells. The pericyte may be identified by means of specific characteristics at electron microscopy (15).
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Lesions That Cause Cortical Proliferation
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Osteochondroma
Osteochondroma occurs in children or adults, and the male-to-female ratio is 1.6:1. The location is the metaphysis, pointing away from the neighboring joint, and the epicenter is exophytic. The appearance consists of cortical and medullary spaces contiguous with host bone.
An osteochondroma may occur in any bone that is formed by enchondral bone growth. It is intimately associated with the growth plate. One theory of pathogenesis describes a focus of ectopically oriented physis growing at right angles to the longitudinal axis of the bone (2). Symptoms relate to local pressure and mass effect. There may be nerve impingement or compromise of local blood flow. Most commonly, osteochondroma manifests as a slowly expanding, hard, painless mass.
At pathologic analysis, there is normal enchondral bone formation, which is at right angles to the physis. The cortical and medullary spaces are continuous with those of the parent bone (Fig 21). The bone itself is of normal architecture. The lesion points away from the affected joint due to pull from the attached muscles or tendons. A cartilaginous cap is seen, which is thickest in children. The average diameter of an osteochondroma in a tubular bone at diagnosis is 4.0 cm (2).
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Figure 21. Osteochondroma in a 24-year-old man with pain and a hard mass. Anteroposterior radiograph of the distal left tibia shows an eccentric lesion contiguous to the underlying cortical and trabecular bone, an appearance characteristic of osteochondroma.
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Multiple osteochondromas are seen in an autosomal dominant syndrome. In the hereditary form, there are often associated defects of normal bone modeling and associated osseous deformities.
Stress Fracture
Stress fracture occurs at all ages, and there is no sex predilection. The location is diaphyseal in the tibia, and the epicenter is eccentric. The appearance consists of smooth cortical thickening, sometimes associated with lucent foci.
There are two types of stress fractures: those that occur when abnormal stress is placed on normal bone (fatigue fracture) (Fig 22) and those that occur when normal stress is placed on abnormal bone (insufficiency fracture) (Fig 23). In both cases, a distinct fracture line is only occasionally appreciated; instead, smooth reactive periostitis is incited by micromotion at the injury and is often radiographically evident. The bones of the lower extremities are commonly affected.
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Figure 22. Stress fracture in a 25-year-old female long-distance runner with midtibial pain. Radiograph shows cortical sclerosis with an incomplete fracture (arrow) of the anterolateral cortex of the tibial middiaphysis.
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Figure 23. Insufficiency fracture in a 55-year-old woman with renal failure and secondary hyperparathyroidism who presented with discomfort in the left lower calf. Radiograph of the distal diaphysis of the tibia shows a sclerotic line (arrow) and a mature callus at the site of an insufficiency fracture. Note the radiopaque cement from fixation of a previous fracture in the more proximal tibial diaphysis.
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Running is the most common cause of fatigue fractures in the tibia. These fractures tend to occur in the middle to distal tibial diaphysis in long-distance runners, although more proximal involvement is seen in children and short-distance runners. Smooth periosteal and endosteal cortical thickening may be seen. Radionuclide bone scans often show characteristic findings and may show unsuspected bilateral involvement. When insufficiency fractures occur in the tibia, they often involve the distal metaphysis and are transverse in nature.
Osteoid Osteoma
In osteoid osteoma, the patient age is typically less than 20 years. The male-to-female ratio is usually 2:1. The location is diaphyseal or metaphyseal; epiphyseal lesions are very rare. The epicenter is eccentric. The appearance consists of a lucent nidus that is sometimes calcified, associated with surrounding sclerosis.
The typical triad of pain, worse at night and relieved by salicylates, is seen in 30%–50% of patients with osteoid osteoma. Lesions are common in the femur and tibia. Pathologically, a nidus of highly vascularized stroma within cortical or cancellous bone or in a subperiosteal location incites exuberant bone sclerosis (12). A juxtaarticular osteoid osteoma may result in proliferative synovitis with little bone formation, making radiographic diagnosis difficult (2). Many believe that prostaglandins produced by the nidus cause pain by acting on unmyelinated sensory nerves in the region (2).
Often, clinical symptoms and radiographic findings are diagnostic. A lucent lesion, usually less than 1.0 cm in diameter, surrounded by a rim of sclerosis is seen in few other entities (Fig 24). The radiographic differential diagnosis includes intracortical hemangioma, stress fracture, and chronic osteomyelitis. Possibly the most difficult distinction to make radiographically is that between osteoid osteoma and intracortical osteogenic sarcoma. The latter is extremely rare, representing 0.1% of osteogenic sarcomas. Intracortical osteosarcoma is seen in the tibia 50% of the time and in the femur 50% of the time, usually in a similar age group as is osteoid osteoma. The radiographic appearances of the two lesions can be very similar, although intracortical osteogenic sarcoma has a tendency to be less well-defined. Histologically, the two can be confused as well, although more anaplasia is typically seen in osteogenic sarcoma. The clinical presentation of intracortical osteogenic sarcoma usually involves less pain of more gradual onset (12).
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Figure 24. Osteoid osteoma in a 19-year-old man with severe midtibial pain. Lateral radiograph of the right tibia shows an osteolytic focus (arrow) with surrounding cortical thickening that involves the anterior cortex of the middiaphysis. Note the similarity in radiographic appearance to that of intracortical hemangioma (Fig 19b).
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The natural history of osteoid osteoma may be slow resolution over several years; however, pain usually prompts more expedient intervention. The goal of treatment is removal or destruction of the nidus. In the past, this was accomplished by surgical means. Image-guided percutaneous thermal coagulation is now used to destroy the nidus and treat this entity on an outpatient basis (19,20).
Periosteal Osteogenic Sarcoma
In periosteal osteogenic sarcoma, the patient age is from the teenage years to the 20s. There is no gender predilection. It occurs in the femur or tibia in 85% of cases; a diaphyseal location is more common. The epicenter is in the periosteum. The appearance consists of perpendicular periosteal spiculation.
Periosteal osteogenic sarcoma is a rare subpopulation of osteogenic sarcoma, accounting for 1% of osteogenic sarcomas. It is seen in a slightly older group of patients than the classic medullary osteogenic sarcoma. The prognosis is somewhat better. Eighty-five percent of cases involve the femur or tibia (2).
The radiographic appearance is fairly characteristic. There is localized cortical saucerization, with the lesion "longer than it is wide." Characteristic perpendicular periosteal spiculation is seen (Fig 25).
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Figure 25. Periosteal osteogenic sarcoma in a 28-year-old woman with lower calf pain. Radiograph shows cortical destruction (arrows) in the posterior cortex of the tibial diaphysis with surrounding new bone formation. The appearance is typical of a periosteal osteogenic sarcoma.
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At histologic analysis, the dominant tissue is chondrosarcomatous, unlike the classic medullary osteogenic sarcoma (12). Often, very scant amounts of osteoid are seen; however, this is sufficient to classify the tumor as osteogenic.
Diaphyseal Dysplasia
Diaphyseal dysplasia often manifests in childhood. There is a slight male predominance, and the epicenter is cortical. The appearance consists of smooth cortical thickening, characteristically sparing the epiphysis and metaphysis, with near obliteration of the medullary canal.
Diaphyseal dysplasia is a rare entity that results from lack of diaphyseal osteoclastic activity (21). It follows an autosomal dominant inheritance pattern. There are two distinct forms: Camurati-Engelmann disease, which is more severe, and Ribbing variant, which is milder. The more severe form usually manifests as myopathy and abnormal gait in childhood (22). Cases may be discovered incidentally in adulthood. Long bones are most conspicuously involved. Radiographs reveal striking diffuse cortical thickening of the diaphyses of the long bones that is characteristically bilateral and symmetric (Fig 26). The metaphyses and epiphyses remain normal. The latter finding alone should suggest the proper diagnosis.
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Figure 26a. Diaphyseal dysplasia in a 55-year-old woman with bilateral knee pain. Anteroposterior (a) and lateral (b) radiographs of the left tibia and fibula show extensive sclerosis with near obliteration of the medullary spaces of both the tibia and fibula but with sparing of the metaphyses and epiphyses. Such a circumferential pattern of sclerosis in a symmetric distribution is not seen in melorheostosis. The findings are characteristic of diaphyseal dysplasia. There was similar symmetric involvement of all the long bones.
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Figure 26b. Diaphyseal dysplasia in a 55-year-old woman with bilateral knee pain. Anteroposterior (a) and lateral (b) radiographs of the left tibia and fibula show extensive sclerosis with near obliteration of the medullary spaces of both the tibia and fibula but with sparing of the metaphyses and epiphyses. Such a circumferential pattern of sclerosis in a symmetric distribution is not seen in melorheostosis. The findings are characteristic of diaphyseal dysplasia. There was similar symmetric involvement of all the long bones.
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Venous Stasis
Venous stasis occurs at any age, and there is no sex predilection. The tibia and fibula are the most common locations. The epicenter is cortical. The appearance consists of diffuse, mature cortical thickening.
Severe venous insufficiency or venous ectasia can result in tibial or fibular cortical thickening secondary to mature periosteal bone formation (Fig 27). Venous stasis is probably the most common cause of diffuse cortical thickening in the tibia. The pathogenesis is uncertain; it may be due to tissue hypoxia, venous hypertension, or other local environmental change that ultimately leads to periosteal stimulation (diffuse, often asymmetric cortical thickening results). Physical examination results typically suggest the appropriate diagnosis.
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Figure 27a. Venous stasis in a 22-year-old woman with swelling of the left lower leg. (a) Radiograph shows mature periostitis involving the medial cortex of the tibial diaphysis (arrowhead). Also noted is a phlebolith (arrow). (b) Oblique venogram shows a large venous varix close to the affected area of tibial cortex.
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Figure 27b. Venous stasis in a 22-year-old woman with swelling of the left lower leg. (a) Radiograph shows mature periostitis involving the medial cortex of the tibial diaphysis (arrowhead). Also noted is a phlebolith (arrow). (b) Oblique venogram shows a large venous varix close to the affected area of tibial cortex.
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Cellulitis
Cellulitis occurs at any age, and there is no sex predilection. It can occur at any location. The epicenter is cortical. The appearance consists of focal cortical thickening.
Overlying cellulitis can cause diffuse cortical thickening as a result of chronic inflammation with resultant periosteal stimulation. By definition, the inflammatory process does not involve the underlying bone, although it may abut the periosteal membrane (Fig 28). Therefore, distinction between osteomyelitis and cellulitis may be difficult on the basis of plain radiographs alone, as both can result focally in thickened cortical bone. Triple-phase radionuclide bone scanning or magnetic resonance (MR) imaging may be useful in distinguishing the two entities.
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Figure 28. Cellulitis of the right lower extremity in a 45-year-old man. Anteroposterior radiograph shows laminar periostitis (arrows). Radionuclide studies failed to demonstrate underlying osteomyelitis.
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Chronic Osteomyelitis
Chronic osteomyelitis occurs at all ages, and there is no sex predilection. It occurs at all locations. The appearance consists of diffuse cortical thickening, sometimes in conjunction with a lucent central lesion.
The tibia is one of the more common locations for chronic osteomyelitis, perhaps secondary to its immediately subcutaneous location, which predisposes it to traumatic bacterial implantation. The rather poor blood supply, particularly of the anterior cortex, facilitates chronic infection. Often, radiographs demonstrate thickened cortical bone without obvious bone destruction due to chronic periosteal stimulation and endosteal or periosteal bone deposition (Fig 29). The differential diagnosis includes stress fracture, osteoid osteoma, and venous stasis changes. Diagnosis often relies on tissue culture; however, other imaging modalities, including MR imaging and nuclear medicine studies, are helpful.
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Figure 29. Chronic osteomyelitis in a 62-year-old man with a draining sinus. Radiograph shows sclerosis and lysis involving the middle of the right tibia. According to the patient’s history, the infection had been present for at least 15 years.
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Osteopathia Striatum
Osteopathia striatum (Voorhoeve disease) occurs at any age. There is no gender predilection. The location is tubular bones, and the epicenter is centric. It has a benign, striated appearance.
Osteopathia striatum is a rare, benign dysplasia of bone, involving the epiphysis and metaphysis of tubular bones. It is typically bilateral, although occasionally it can be unilateral. There is an association with Goltz syndrome (23).
Osteopathia striatum is typically asymptomatic, although there can be associated joint discomfort. However, the discomfort may be incidental, leading to radiographic diagnosis. Radiographically prominent vertical striations predominate in the metaphyses and epiphyses of the long bones (Fig 30). The differential diagnosis in-cludes normal variation in the prominence of periarticular vertical trabeculation, adult osteopetrosis, and enchondromatosis.
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Figure 30a. Osteopathia striatum in a 28-year-old man after minor trauma. Anteroposterior (a) and lateral (b) radiographs show linear bands that extend to the joint space, findings consistent with osteopathia striatum. The femur was also affected.
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Figure 30b. Osteopathia striatum in a 28-year-old man after minor trauma. Anteroposterior (a) and lateral (b) radiographs show linear bands that extend to the joint space, findings consistent with osteopathia striatum. The femur was also affected.
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Melorheostosis
Melorheostosis is usually discovered in childhood, occasionally in adulthood. The male-to-female ratio is usually 1:1. It can occur in a single limb, and the lower extremity is more commonly affected than the upper extremity. The epicenter is periosteal or endosteal. The appearance consists of cortical hyperostosis in one or multiple bones, often with intervening soft-tissue calcification or ossification.
The limb involved with melorheostosis often demonstrates joint pain, swelling, and limitation of motion in childhood. There is often associated growth disturbance, muscular contraction, and limb length discrepancy. There may be overlying skin changes.
At radiography, contiguous bones of an extremity are often involved, although there may be involvement of a single bone (Fig 31). Bilaterality is extremely rare. There is cortical hyperostosis with intervening soft-tissue calcification or ossification. There may be endosteal hyperostosis with obliteration of the medullary space (23).
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Figure 31. Melorheostosis in an 11-year-old boy with discomfort in and swelling of the right lower extremity. Radiograph obtained with a long leg cassette shows cortical hyperostosis that involves the femur and tibia, a finding typical of melorheostosis.
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At pathologic analysis, thickened and enlarged osseous trabeculae are noted, associated with fibrous tissue replacement of the marrow space. There is controversy involving the distribution of melorheostosis. It has been suggested that the distribution mimics that of the sclerotomes (zones supplied by individual spinal sensory nerves), implying a neurogenic origin (24).
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Conclusion
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We have reviewed a variety of tumors and disease processes that involve the tibial cortex. The distinction between cortical proliferation versus destruction can be an aid to limiting the differential diagnosis in these entities.
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References
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Abstract
LEARNING OBJECTIVES FOR TEST...
