Age (Median) at

Tumor Frequency Typical Location Radiologic Features Comments

Diagnosis

Benign

Aneurysmal bone Uncommon 10 to 30 Metaphysis of long bones; Widely expansile defect of the cortex with The lesion represents cavities filled with
cyst sometimes posterior cortical buttressing at the region of extravasated blood. Lesions may occur
arch of vertebrae expansion from the host bone and very thin secondary to trauma, or concurrent to other
cortices at outer edge of the lesion. The tumors (e.g., giant cell tumor). Pain and
lesions are osteolytic and exhibit “a soap swelling at the site of involvement
bubble” appearance

The lesion represents a hamartoma of bone. They are

painless, usually small, and of no clinical
Bone island Very common Any age Any location but skull Homogenously radiodense focus with spiculated significance except for their differentiation
“brush” border from blastic metastasis. Multiple bone islands
are termed osteopoikilosis. Osteomas are
similar lesions found in the skull

Chondroblastoma Rare 10 to 20 Proximal femur, about the Small (<5 cm) osteolytic subarticular lesion with A rare lesion composed of chondroblasts and
knee, and proximal a well-defined margin of sclerosis immature cartilage. They appear in the
humerus secondary centers of enchondral ossification
of skeletally immature patients

Chondromyxoid Rare 10 to 30 Proximal tibia and fibula Radiolucent, oval, eccentric lesion Tumor involving the cartilage-forming connective
fibroma tissue of marrow space

Discrete islands of cartilage surrounded by layered

bone. Enchondromas occur within a bone;
Enchondroma Common 15 to 35 Small tubular bones of the Small round or oval cystic defects, typically with periosteal chondromas occur next to the cortex
hands and feet; stippled matrix calcification and endosteal and under the periosteum. Long bone lesions
however, possible in scalloping are more likely to be painful. Multiple
any enchondrally enchondromas is termed Ollier disease, and if
formed bone soft-tissue hemangiomas are also presenting, it
is termed Maffucci syndrome. Solitary
enchondromas have a malignant potential of
<1%; this rate rises to 30% for Ollier disease
and higher for Maffucci syndrome

Fibrous cortical Very common 4 to 8 Distal femur and proximal Well-marginated, eccentric cortex-based Nonaggressive fibrous lesion of bone, usually
defect and distal tibia osteolytic defect smaller than 3 cm healing spontaneously

Fibrous dysplasia Common <20 Femur, tibia, craniofacial Wide variety in appearance depending on the
bones, ribs, and bone involved. Long bones demonstrate a
pelvis midly expansile medullary lesion that may
be mildly sclerotic (“smoky” or “ground
glass” appearance). “Shepherd’s crook”
deformity is a lateral bowing and coxa vara
deformity occurring in the proximal femur.
Individual lesions often exhibit a thick
“rind” of marginal sclerosis
Nonneoplastic disturbance of bone maintenance
resulting in fibrous tissue replacing normal
bone. Albright syndrome is the triad of
polyostotic fibrous dysplasia, precocious
puberty, and cutaneous hyperpigmentations
(café-au-lait spots)

Giant cell tumor

Common (18% 20 to 40 Distal radius, distal femur, Subarticular, osteolytic, mildly expansile, Tumor of the supportive tissues of bone marrow;
of benign proximal tibia eccentric, no marginal sclerosis, and composed of large mononuclear stromal cells
bone sometimes with internal septation and interspersed osteoclastic-like
tumors) multinucleated giant cells. Approximately 5%
to 10% may be malignant, marked by pain,
swelling, and aggressive radiographic
appearance
 Age (Median) at
Tumor Frequency Typical Location Radiologic Features Comments
Diagnosis

Hemangioma Common 40 to 50 Craniofacial bones and Osteolytic defect with characteristic coarsened, Represent vascular lesion of bone; typically
vertebral bodies honeycomb trabeculae; or vertical asymptomatic; rarely expanding vertebral
striations (“corduroy cloth”) lesion may relate to spinal stenosis

Nonossifying Very common 10 to 20 Metaphysis of tibia and Well-marginated, eccentric cortex-based Nonaggressive fibrous lesion of bone, usually
fibroma femur osteolytic defect >3 cm. Malignant lesions healing spontaneously
show cortical breakthrough and soft-tissue
mass

Osteoblastoma Uncommon 10 to 30 Posterior elements of the Geographic, osteolytic, eccentric, mildly Same as osteoid osteoma; highly vascular connective
vertebrae; less expansile lesion tissue nidus with interlaced osteoid tissue.
commonly in Lesions may be painful
proximal femur and
tibia

Osteochondroma Common 10 to 30 Metaphyses of long bones, Sessile or pedunculated (“coat hanger” exostosis Abnormal outgrowth of lateral portion of the growth
mostly lower or “cauliflower cap”) cartilage capped plate; affects epiphyseal growth. Multiple
extremities. bony outgrowth that is continuous with osteochondromas are referred to as hereditary
Hereditary multiple underlying bone multiple exostoses (HME), and are associated
exostoses of the with a higher rate of malignancy (up to 25%)
proximal femora and compared with solitary lesions (1% to 2%)
pelvis is common

Osteoid osteoma 11% of benign 10 to 20 Cortex of proximal femur Small radiolucent nidus surrounded by Highly vascular connective tissue nidus with
bone or tibia; less radiodense reactive sclerosis interlaced osteoid tissue. Classically, pain at
tumors commonly the night that is alleviated by aspirin. Spine
posterior elements of lesions present on the concave side of a
the vertebrae painful scoliosis. Low recurrence following
excision

Simple bone cyst Common 5 to 10 Proximal humerus and Oblong, central, expansile, radiolucent Fluid-filled lesion of uncertain etiology; clinically
proximal femur subepiphyseal (less frequently diaphyseal) silent unless fractured
osteolytic lesion. At times the partial
internal septations may fracture and
inferiorly migrate (“fallen fragment” sign)
 Age (Median) at
Tumor Frequency Typical Location Radiologic Features Comments
Diagnosis

Malignant

Bone metastasis Very common >40 Spine, ribs, skull, and Individual lesions appear osteolytic, less The most common mechanism is hematogenous
pelvis; rarely distal commonly osteoblastic, and seeding from primary tumors; the breast gives
to the knees and characteristically with no or small soft- rise to 70% of female lesions, and prostate to
elbows tissue mass or periosteal reaction 60% of male lesions. Advanced osteolytic
destruction may exhibit increased serum
calcium. Osteoblastic lesions may reveal
increased levels of alkaline phosphatase

Multiple myeloma Common >40 (60) Vertebrae, rib, innominate, Osteopenia, “punch-out” lesions, “raindrop” skull Malignant plasma cell proliferation; with abnormal
femur laboratory findings of Bence Jones proteins,
anemia, thrombocytopenia, Rouleaux
formation, Mott cell

35.1*

Osteosarcoma Bimodal: first Metaphyses of long bones Osteolytic (25%), osteoblastic (50%), or mixed Aggressive lesion of mesenchymal bone-producing
mode 1 to (distal femur, (25%) lesion with poorly defined margins, cells. Lesions are painful, rapidly expanding,
30 (19); proximal tibia, and aggressive periosteal reaction (“onion with malignant potential usually requiring
second proximal humerus) skin” or Codman triangle), and soft-tissue amputation
mode in mass
elderly

25.8*

Chondrosarcoma >40 (53) Femur, innominate, Destructive lesions appearing as a central lesion
humerus in bone with stippled calcifications, and
cortical scalloping, or as a peripheral
lesion extending from the bone’s surface
appearing as an exploded osteochondroma
Malignancy of mesenchymal cartilage-producing
cells exhibiting varying degrees of
malignancy. Lesions are painful and
expanding with malignant potential. They may
be primary malignant lesions of bone, or
secondary to benign cartilage lesions (e.g.,
enchondroma, osteochondroma)

16.0*

Ewing sarcoma 1 to 30 (15) Femur, innominate, Permeative osteolytic eccentric pattern of bone Small round cell malignancy of bone. The patient
vertebrae, humerus destruction, often presenting as a scalloped exhibits a tender, warm, swollen limb
deformity of the diaphyseal cortex
(“saucerization”); in flat bones it appears
as a mildly expansile, soap-bubble,
osteolytic defect

8.4*

Chordoma 30 to 70 (59) C2 Skull, sacrum, and Appear as midline lesions, characterized by the Develops from remnant portions of the notochord;
vertebral presence of bone destruction and a large patients complain of pain and enlarging soft-
body soft-tissue mass; calcification is noted in tissue mass, and regional visceral and
up to 40% of lesions on plain films neurologic involvement, such as headaches
and diplopia for sphenooccipital lesions, and
bowel and bladder dysfunction for
sacrococcygeal lesions. Radiation is applied to
those lesions that cannot be resected

5.7*

Fibrosarcoma and 30 to 50 (59) Distal femur and proximal Lesions appear as osteolytic destruction in the Malignancy of deep fibrous tissue; patient
malignant tibia metaphysis, often extending to the complaints of pain and tenderness in the
fibrous diaphysis marked by cortical destruction region of involvement (usually around the
histiocytoma and soft-tissue mass knee)

*
This percentage reflects the portion of primary malignant tumors of bone, excluding multiple myeloma.
LocationCommentsLongitudinalDiaphysisRound cell lesions (Ewing sarcoma, primary lymphoma of bone, multiple myeloma),
malignant fibrous histiocytoma, adamantinomaDiametaphysisOsteoblastoma, chondromyxoid fibroma, nonossifying fibroma, Ewing
sarcomaMetaphysisAneurysmal bone cyst, chondrosarcoma, enchondroma, fibrosarcoma, giant cell tumor,
osteosarcomaEpiphysisChondroblastoma, giant cell tumorAxialCentralCentral chondrosarcoma, Ewing sarcoma, fibrous dysplasia,
primary lymphoma of bone, solitary bone cystEccentricAneurysmal bone cyst, fibrosarcoma, giant cell tumor, nonossifying
fibromaCorticalFibrous cortical defectParostealParosteal chondrosarcoma, periosteal chondroma

Bone Tumors and Related Diseases

CHAPTER 13

Bone Tumors and Related Diseases

Dennis M. Marchiori

IMAGING MODALITIES
Laboratory Tests
Bone Biopsy
Tumor Staging
Signs and Symptoms
Tumor Discriminators
Treatment Options
BONE ORIGIN
Bone Island (Enostosis) and Osteopoikilosis
Osteoid Osteoma and Osteoblastoma
Ossifying Fibroma
Osteosarcoma
BONE MARROW ORIGIN
Plasmacytoma and Multiple Myeloma
Ewing Sarcoma
Lymphoma of Bone
Leukemia
CARTILAGE ORIGIN
Chondroma, Ollier Disease, and Maffucci Syndrome
Chondroblastoma
Chondromyxoid Fibroma
Osteochondroma and Hereditary Multiple Exostoses
Chondrosarcoma
FIBROUS, HISTIOCYTIC, AND FIBROHISTIOCYTIC ORIGIN
Fibrous Dysplasia
Nonossifying Fibroma and Fibrous Cortical Defect
Desmoplastic Fibroma
Fibrosarcoma and Malignant Fibrous Histiocytoma
SYNOVIAL ORIGIN
Pigmented Villonodular Tenosynovitis
Synoviochondrometaplasia
Synoviosarcoma
MUSCLE ORIGIN
 Leiomyoma
Leiomyosarcoma
Rhabdomyosarcoma
FAT ORIGIN
Lipoma
Liposarcoma
VASCULAR ORIGIN
Hemangioma
Glomus Tumor
Lymphangioma
Angiosarcoma
NOTOCHORD ORIGIN
Chordoma
MISCELLANEOUS OR UNKNOWN ORIGIN
Aneurysmal Bone Cyst
Giant Cell Tumor
Simple Bone Cyst
Epidermoid Cyst
Interosseous Ganglion Cyst
Adamantinoma
Ameloblastoma
NONNEUROMUSCLOSKELETAL ORIGIN
Metastatic Bone Disease
Bone tumors are one of the most serious diagnostic differentials in patients with musculoskeletal complaints. Bone tumors are categorized as
either primary or secondary. Primary bone tumors arise from bone and related soft tissues directly in their site of involvement and may be either benign or malignant.
Secondary bone tumors arise “secondary” to a primary lesion and also may be benign (e.g., secondary aneurysmal bone cyst arising in an area of past trauma) or
malignant (e.g., bone metastasis from a lung carcinoma). 4

Benign tumors usually are designated as such by the suffix -oma (e.g., enchondroma) and generally are not regarded as cancers. Malignant primary tumors of bone
and other connective tissues are designated by the use of the term, or suffix, sarcoma after the tissue type involved (e.g., osteosarcoma).
Metastatic bone disease refers to a malignant tumor that secondarily seeds to bone, usually from a primary malignancy of the epithelial tissue (designated
as carcinomas) of the lung, breast, prostate, kidney, liver, and so on. For instance, if the patient has a bronchogenic carcinoma that metastasizes to the thoracic spine, the
lung lesion is the primary lesion and the thoracic spine lesion is bone metastasis of the lung lesion.
In general terms, both benign tumors of bone and bone metastasis are many times more common than primary malignancies of bone. Primary malignancy of bone is
relatively uncommon. Malignancy in general is thought to affect approximately 1 in 3 people over their lifetime. Approximately 1.4 million Americans are diagnosed
with a malignancy each year, with less than 2800 of these cases representing primary malignancies of the bone and joints and another 16,500 cases identified as
myeloma.  By comparison, there are approximately 263,000 new cases of malignancy annually of the digestive system, 215,000 cases of primarly malignancy of the
180a

breast, and 186,000 cases of primary malignancy of the respiratory system diagnosed in the United States.  The incidence of primary malignant bone lesions (excluding
180a

multiple myeloma) is estimated at 8 per million persons.  The most common sarcomas include osteosarcoma (35.1%), chondrosarcoma (25.8%), Ewing sarcoma (16.0%),
61

chordoma (8.4%), and fibrosarcoma (5.7%). Because it arises from the plasma cell of bone marrow, multiple myeloma is not considered a primary malignancy of bone.
Instead, osteosarcoma is the most common primary malignancy of bone. However, multiple myeloma is by far the most common primary malignancy of bone if
considered in the group of primary bone malignancies . 135

The evaluation of a bone tumor requires careful assessment of the patient’s history and application of clinical studies in developing a concise list of differential
possibilities. Diagnostic imaging plays a major role in developing and narrowing this list. With advances in technology, biologic tissue assessments have added a
valuable tool to the arsenal of the investigator. Therefore, the diagnosis of tumors is accomplished along three dimensions of assessment: clinical (e.g., gender, age,
symptoms), imaging (e.g., location, appearance), and pathologic (e.g., microscopic cell type and molecular assessment).

Imaging Modalities
Imaging studies should define the lesion, determine its location, grade its aggressiveness, decide if the lesion is limited to one bone (monostotic) or if multiple bones are
involved (polyostotic), assess soft-tissue involvement, and identify the lesion’s matrix. Plain film radiography remains the chief imaging modality for the initial
assessment of bone tumors. Sometimes the radiographic presentation reveals a lesion that is nonaggressive with classic characteristics, thus requiring no further
examination and leading to an immediate diagnosis. However, further assessment often is necessary when lesions are poorly defined or accompanied by significant
clinical signs and symptoms.
Computed tomography (CT) provides direct thin axial slices of anatomy, allowing for a more detailed assessment of complex anatomy (e.g., spine) than can be
accomplished with plain film. CT demonstrates calcification well and therefore is capable of demonstrating calcification within the lesion’s matrix and the cortical
response of the lesion’s host bone. Plain film radiographs and CT scans of the chest help to assess the possibility of pulmonary metastasis, the presence of which may
alter the treatment plan.
Although radionuclide bone scanning lack specificity, it is sensitive to the presence of early disease and is widely used to assess the possibility of multiple lesions, a
finding that substantially narrows down the diagnostic list of possibilities. There is one notable exception to the sensitivity of bone scans. Bone scans have elevated rates
of false negative findings in the presence of multiple myeloma and purely lytic lesions. In this and other cases it may be best to apply radiographic surveys or
multiregional magnetic resonance imaging (MRI) studies.
MRI has the ability to demonstrate abnormality of the bone marrow and delineate extraosseous involvement. Replacement of normal marrow by pathologic
processes (e.g., metastasis, multiple myeloma, osteomyelitis) is readily demonstrated and provides an early sign of disease. However, MRI remains inferior to both plain
film and CT for detailing calcification, ossification, cortical destruction, and periosteal reaction. MRI is especially valuable for assessing the neurologic impact of the
lesion. Plain film radiographs efficiently provide information about the rate of growth and aggressiveness of a lesion, but do not establish a histologic diagnosis with the
same accuracy as a biopsy. On MRI studies, most bone tumors are dark on T1-weighted images and bright on T2-weighted images. Fibrous tissue, cortical bone,
desmoids, and scar tissue are dark on both T1- and T2-weighted images. Hemangiomas, lipomas, and liposarcomas are bright on both T1- and T2-weighted images
because of the blood components of these lesions.
The information offered through imaging is coupled with clinical data, laboratory studies, and possibly biopsy to identify the specific lesion present.

Laboratory Tests
Laboratory tests are less helpful than imaging studies for diagnosing bone tumors. Benign bone tumors demonstrate normal laboratory values, and malignant tumors
often demonstrate normal laboratory values. However, a few characteristic laboratory findings may be seen in malignancy. For example, increased serum calcium levels
and increased hydroxyproline in the urine are associated with massive bone osteolysis, as seen in generalized lytic metastasis or multiple myeloma. Some osteosarcoma,
osteoblastic metastasis, and other bone-proliferating malignancies often are accompanied by increased serum alkaline phosphatase levels. Multiple myeloma is
associated with monoclonal immunoglobulins “M-spike” on serum electrophoresis, Bence Jones proteins in the urine, hyperglobulinemia (reversed albumin-to-globulin
[A : G] serum ratio), elevated creatine, and decreased hematocrit levels.

Bone Biopsy
Bone biopsy is the removal of suspect tissue from the body for examination by a pathologist. In most circumstances a biopsy provides the most accurate diagnosis
possible, typically more accurate than can be obtained by imaging. However, biopsy is not especially helpful for determining the lesion’s rate of growth and
aggressiveness. The latter qualities are best assessed by conventional and specialized imaging modalities; therefore, a biopsy and imaging studies are complementary,
leading to an end diagnosis.
The accuracy of the biopsy results depends on which region of the lesion is collected, the skill of the person performing the biopsy, and the clinical circumstances in
which the biopsy takes place. It is generally most accurate if the biopsy is taken from the most aggressive and viable portion of the lesion. This often requires an open
biopsy approach. However, an open biopsy is associated with higher complication rates than a closed (percutaneous) biopsy approach.  Open biopsy procedures may
171

contaminate malignant cells in normal tissue, interfering with otherwise successful limb-sparing procedures. Closed biopsy procedures generally are safer, but may not
produce enough tissue for accurate diagnosis. Biopsy of highly vascular lesions is cautioned generally, given the risk of massive hemorrhage.

Tumor Staging
Tumor staging is integral to patient management. Once a tumor is found, the extent of disease is defined along three parameters. The first parameter defines the size of
the tumor (T) and whether it has invaded surrounding tissues. The second parameter examines the extent of lymph node involvement (N). Lastly, one must know
whether the tumor has metastasized to other regions of the body (M). These three parameters define the “TNM” system of staging lesions. Although some variation in
the stages exists depending on the source and type of tumor being staged, a general presentation of the TNM method is presented in Box 13-1.
Box 13-1   Tumor Staging
Tumor staging is the process of characterizing the extent of the patient’s disease along three parameters: tumor (T), lymph nodes (N), and metastasis (M).
T: Indicates the size and regional involvement of the tumor
TX: Primary tumor cannot be assessed
T0: No evidence of a primary tumor
Tis: Carcinoma in situ (the tumor cells are restricted to the epithelial layer of the involved tissue)
T1: Localized tumor; diameter is 2 cm or less
T2: Localized tumor; diameter is 5 cm or less and mild involvement of same organ tissue
T3: Advanced tumor; diameter is greater than 5 cm and there is extensive involvement of same organ tissue
T4: Massive tumor; diameter is greater than 5 cm and there is involvement of nerves, blood vessels, bone, or other organs
N: Indicates the extent of lymph node involvement
NX: Regional lymph nodes cannot be assessed
N0: No evidence of metastasis to regional lymph nodes
N1: Small tumor in one lymph node
N2: Medium tumor in one or more lymph nodes
N3: Large tumor in one or more lymph nodes
M: Indicates the presence or absence of distant metastasis
MX: Distal metastasis cannot be assessed
M0: No evidence of distal metastasis
M1: Evidence of distal metastasis present
A tumor stage (I to IV) can be calculated once the T, N, and M parameters are determined. In general, the higher the tumor stage, the lower the patient’s chance of
survival.
Stage I: T1 N0 M0
Stage II: T2 N1 M0
Stage III: T3 N0 M0, T1–3 N1 M0
Stage IV: T4 N0–1 M0, T0–4 N2–3 M0, T0–4 N0–4 M1

Signs and Symptoms

Clinically important lesions are usually detected secondary to the patient’s complaint of pain or attention to a palpable mass. Aggressive bone lesions generate signs and
symptoms directly, secondary to bone and tissue destruction. Classically the bone pain experienced is more dramatic at night and unrelated to physical activity. The
patient may exhibit fever, impaired mobility, and cachexia with aggressive lesions. In contrast, most benign lesions are clinically asymptomatic, but commonly are
recognized after pathologic fracture. This concept has been termed traumatic determinism; that is, the presence of the benign lesion is only determined as a result of
trauma and subsequent fracture. Less frequently, asymptomatic bone lesions are detected serendipitously on radiographs obtained for unrelated reasons.
Tumor Discriminators
Many tumors and tumor-like conditions produce similar imaging findings. The following list of radiologic and clinical parameters assists in narrowing the usually broad
number of pathologic possibilities for a given radiographic appearance. In addition, the following parameters assist in evaluating the aggressiveness and clinical
importance of a lesion.

Patient Age
The age of the patient is an important clue helping to differentiate between lesions that look the same but are unique to certain age groups. Conversely, the patient’s age
may suggest a diagnostic possibility that would not otherwise be considered from the radiographic appearance because of an atypical presentation. Age is more helpful
when the age range associated with the tumor is narrow. Given only the patient’s age, an examiner can determine which tumor is present with a high degree of
accuracy.  (Tables 13-1 and 13-2 present the ages at which common benign and malignant tumors develop, respectively.) Generally with malignant tumors, Ewing
69

sarcoma and osteosarcoma are present in children, and metastatic bone disease and multiple myeloma are found in patients older than 40 years of age. Benign tumors
are most common in patients 10 to 30 years of age, slightly younger for simple bone cysts, and slightly older for lipomas and hemangiomas.
TABLE 13-1
TYPICAL AGES FOR THE DEVELOPMENT OF SELECTED BENIGN TUMORS

Age (in Years) Tumor

5 to 10 Simple bone cyst

10 to 20 Chondroblastoma, nonossifying fibroma, osteoid osteoma

10 to 30 Aneurysmal bone cyst, chondromyxoid fibroma, osteoblastoma, osteochondroma

15 to 35 Enchondroma, osteoma

20 to 40 Giant cell tumor

30 to 50 Lipoma

40 to 50 Hemangioma

TABLE 13-2
TYPICAL AGES FOR THE DEVELOPMENT OF SELECTED MALIGNANT TUMORS

Age (in Years) Tumor

Less than 1 Metastatic neuroblastoma

1 to 30 Ewing sarcoma, osteosarcoma

Age (in Years) Tumor

20 to 40 Giant cell tumor, parosteal osteosarcoma

30 to 50 Fibrosarcoma, malignant fibrous histiocytoma, primary lymphoma of bone

More than 40 Chordoma, chondrosarcoma, metastatic bone disease, multiple myeloma

Location
Individual tumors are often predisposed to occur in certain bones (Table 13-3). Even more suggestive is the longitudinal (diaphysis, metadiaphysis, metaphysis, and
epiphysis) (Fig. 13-1) and transverse (central or eccentric medullary, cortical, periosteal, and parosteal) (Fig. 13-2) location within bone (Table 13-4 and Fig. 13-3).
TABLE 13-3
SUMMARY OF COMMON BENIGN AND MALIGNANT LESIONS

Age (Median) at
Tumor Frequency Typical Location Radiologic Features Comments
Diagnosis

Benign

Aneurysmal bone Uncommon 10 to 30 Metaphysis of long bones; Widely expansile defect of the cortex with The lesion represents cavities filled with extravasated
cyst sometimes cortical buttressing at the region of blood. Lesions may occur secondary to trauma, or
posterior arch of expansion from the host bone and very concurrent to other tumors (e.g., giant cell tumor).
vertebrae thin cortices at outer edge of the lesion. Pain and swelling at the site of involvement
The lesions are osteolytic and exhibit “a
soap bubble” appearance

The lesion represents a hamartoma of bone. They are

painless, usually small, and of no clinical significance
Bone island Very common Any age Any location but skull Homogenously radiodense focus with except for their differentiation from blastic
spiculated “brush” border metastasis. Multiple bone islands are
termed osteopoikilosis. Osteomas are similar lesions
found in the skull

Chondroblastoma Rare 10 to 20 Proximal femur, about the Small (<5 cm) osteolytic subarticular lesion A rare lesion composed of chondroblasts and immature
knee, and proximal with a well-defined margin of sclerosis cartilage. They appear in the secondary centers of
humerus enchondral ossification of skeletally immature
patients

Chondromyxoid Rare 10 to 30 Proximal tibia and fibula Radiolucent, oval, eccentric lesion Tumor involving the cartilage-forming connective tissue of
fibroma marrow space

Discrete islands of cartilage surrounded by layered bone.

Enchondromas occur within a bone; periosteal
Enchondroma Common 15 to 35 Small tubular bones of the Small round or oval cystic defects, typically chondromas occur next to the cortex and under the
hands and feet; with stippled matrix calcification and periosteum. Long bone lesions are more likely to be
however, possible painful. Multiple enchondromas is termed Ollier
 Age (Median) at
Tumor Frequency Typical Location Radiologic Features Comments
Diagnosis

disease, and if soft-tissue hemangiomas are also

presenting, it is termed Maffucci syndrome. Solitary
in any enchondrally endosteal scalloping enchondromas have a malignant potential of <1%;
formed bone this rate rises to 30% for Ollier disease and higher for
Maffucci syndrome

Fibrous cortical Very common 4 to 8 Distal femur and proximal Well-marginated, eccentric cortex-based Nonaggressive fibrous lesion of bone, usually healing
defect and distal tibia osteolytic defect smaller than 3 cm spontaneously

Fibrous dysplasia Common <20 Femur, tibia, craniofacial Wide variety in appearance depending on the Nonneoplastic disturbance of bone maintenance resulting
bones, ribs, and bone involved. Long bones demonstrate in fibrous tissue replacing normal bone. Albright
pelvis a midly expansile medullary lesion that syndrome is the triad of polyostotic fibrous dysplasia,
may be mildly sclerotic (“smoky” or precocious puberty, and cutaneous
“ground glass” appearance). hyperpigmentations (café-au-lait spots)
“Shepherd’s crook” deformity is a
lateral bowing and coxa vara deformity
occurring in the proximal femur.
Individual lesions often exhibit a thick
“rind” of marginal sclerosis

Giant cell tumor

Common (18% 20 to 40 Distal radius, distal femur, Subarticular, osteolytic, mildly expansile, Tumor of the supportive tissues of bone marrow;
of benign proximal tibia eccentric, no marginal sclerosis, and composed of large mononuclear stromal cells and
bone sometimes with internal septation interspersed osteoclastic-like multinucleated giant
tumors) cells. Approximately 5% to 10% may be malignant,
marked by pain, swelling, and aggressive
radiographic appearance

Hemangioma Common 40 to 50 Craniofacial bones and Osteolytic defect with characteristic Represent vascular lesion of bone; typically asymptomatic;
vertebral bodies coarsened, honeycomb trabeculae; or rarely expanding vertebral lesion may relate to spinal
vertical striations (“corduroy cloth”) stenosis

Nonossifying fibroma Very common 10 to 20 Metaphysis of tibia and Well-marginated, eccentric cortex-based Nonaggressive fibrous lesion of bone, usually healing
femur osteolytic defect >3 cm. Malignant spontaneously
lesions show cortical breakthrough and
soft-tissue mass

Osteoblastoma Uncommon 10 to 30 Posterior elements of the Geographic, osteolytic, eccentric, mildly Same as osteoid osteoma; highly vascular connective tissue
vertebrae; less expansile lesion nidus with interlaced osteoid tissue. Lesions may be
commonly in painful
proximal femur and
tibia
 Age (Median) at
Tumor Frequency Typical Location Radiologic Features Comments
Diagnosis

Osteochondroma Common 10 to 30 Metaphyses of long Sessile or pedunculated (“coat hanger” Abnormal outgrowth of lateral portion of the growth plate;
bones, mostly lower exostosis or “cauliflower cap”) cartilage affects epiphyseal growth. Multiple
extremities. capped bony outgrowth that is osteochondromas are referred to as hereditary
Hereditary multiple continuous with underlying bone multiple exostoses (HME), and are associated with a
exostoses of the higher rate of malignancy (up to 25%) compared with
proximal femora solitary lesions (1% to 2%)
and pelvis is
common

Osteoid osteoma 11% of benign 10 to 20 Cortex of proximal femur Small radiolucent nidus surrounded by Highly vascular connective tissue nidus with interlaced
bone or tibia; less radiodense reactive sclerosis osteoid tissue. Classically, pain at night that is
tumors commonly the alleviated by aspirin. Spine lesions present on the
posterior elements concave side of a painful scoliosis. Low recurrence
of the vertebrae following excision

Simple bone cyst Common 5 to 10 Proximal humerus and Oblong, central, expansile, radiolucent Fluid-filled lesion of uncertain etiology; clinically silent
proximal femur subepiphyseal (less frequently unless fractured
diaphyseal) osteolytic lesion. At times
the partial internal septations may
fracture and inferiorly migrate (“fallen
fragment” sign)

Malignant

Bone metastasis Very common >40 Spine, ribs, skull, and Individual lesions appear osteolytic, less The most common mechanism is hematogenous seeding
pelvis; rarely distal commonly osteoblastic, and from primary tumors; the breast gives rise to 70% of
to the knees and characteristically with no or small soft- female lesions, and prostate to 60% of male lesions.
elbows tissue mass or periosteal reaction Advanced osteolytic destruction may exhibit
increased serum calcium. Osteoblastic lesions may
reveal increased levels of alkaline phosphatase

Multiple myeloma Common >40 (60) Vertebrae, rib, Osteopenia, “punch-out” lesions, “raindrop” Malignant plasma cell proliferation; with abnormal
innominate, femur skull laboratory findings of Bence Jones proteins, anemia,
thrombocytopenia, Rouleaux formation, Mott cell

35.1*

Osteosarcoma Bimodal: first Metaphyses of long bones Osteolytic (25%), osteoblastic (50%), or mixed Aggressive lesion of mesenchymal bone-producing cells.
mode 1 to (distal femur, (25%) lesion with poorly defined Lesions are painful, rapidly expanding, with
30 (19); proximal tibia, and margins, aggressive periosteal reaction malignant potential usually requiring amputation
second proximal humerus) (“onion skin” or Codman triangle), and
mode in soft-tissue mass
elderly
 Age (Median) at
Tumor Frequency Typical Location Radiologic Features Comments
Diagnosis

25.8*

Chondrosarcoma >40 (53) Femur, innominate, Destructive lesions appearing as a central Malignancy of mesenchymal cartilage-producing cells
humerus lesion in bone with stippled exhibiting varying degrees of malignancy. Lesions are
calcifications, and cortical scalloping, or painful and expanding with malignant potential. They
as a peripheral lesion extending from may be primary malignant lesions of bone, or
the bone’s surface appearing as an secondary to benign cartilage lesions (e.g.,
exploded osteochondroma enchondroma, osteochondroma)

16.0*

Ewing sarcoma 1 to 30 (15) Femur, innominate, Permeative osteolytic eccentric pattern of Small round cell malignancy of bone. The patient exhibits a
vertebrae, humerus bone destruction, often presenting as a tender, warm, swollen limb
scalloped deformity of the diaphyseal
cortex (“saucerization”); in flat bones it
appears as a mildly expansile, soap-
bubble, osteolytic defect

8.4*

Chordoma 30 to 70 (59) C2 Skull, sacrum, and Appear as midline lesions, characterized by Develops from remnant portions of the notochord; patients
vertebral the presence of bone destruction and a complain of pain and enlarging soft-tissue mass, and
body large soft-tissue mass; calcification is regional visceral and neurologic involvement, such as
noted in up to 40% of lesions on plain headaches and diplopia for sphenooccipital lesions,
films and bowel and bladder dysfunction for
sacrococcygeal lesions. Radiation is applied to those
lesions that cannot be resected

5.7*

Fibrosarcoma and 30 to 50 (59) Distal femur and proximal Lesions appear as osteolytic destruction in the Malignancy of deep fibrous tissue; patient complaints of
malignant tibia metaphysis, often extending to the pain and tenderness in the region of involvement
fibrous diaphysis marked by cortical (usually around the knee)
histiocytoma destruction and soft-tissue mass

*
This percentage reflects the portion of primary malignant tumors of bone, excluding multiple myeloma.

From Dorfman HD, Czerniak B: Bone tumors, St. Louis, 1998, Mosby; Dorfman HD, Czerniak B: Bone cancer, Cancer 75:2003, 1995.
LOCATION OF SELECTED TUMORS WITHIN A BONE

Location Comments

Longitudinal
Diaphysis Round cell lesions
Ewing sarcoma
primary lymphoma of bone
multiple myeloma
Malignant fibrous histiocytoma
Adamantinoma
Diametaphysis Osteoblastoma
Chondromyxoid fibroma
Nonossifying fibroma
Ewing sarcoma
Metaphysis Aneurysmal bone cyst,
Chondrosarcoma
Enchondroma,
Fibrosarcoma,
Giant cell tumor,
Osteosarcoma
Epiphysis Chondroblastoma
Giant cell tumor

Axial

Central Central chondrosarcoma,

Ewing sarcoma,
Fibrous dysplasia,
Primary lymphoma of bone,
Solitary bone cyst
Eccentric Aneurysmal bone cyst,
Fibrosarcoma,
Giant cell tumor,
Nonossifying fibroma
Cortical Fibrous cortical defect
Parosteal Parosteal chondrosarcoma,
Periosteal chondroma