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Enchondroma
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Enchondroma,
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a chondroma
arising within bone. Solitary enchondromas, which develop in the
medullary cavity, are usually central tumours located in the metaphysis
of a long tubular bone or in the diaphysis of a short tubular bone in
the hand or foot. The presence of multiple enchondromas leads to a
condition termed enchondromatosis.
The tumours are composed of lobules of hyaline cartilage and are often
asymptomatic. If pain is present, malignant transformation should be
suspected; this complication occurs more commonly in the long tubular
bones and those of the pelvic and shoulder girdles. Most solitary
tumours involve the hands, particularly the proximal phalanges, or feet.
In addition, solitary enchondromas occur in the long tubular bones,
especially the humerus, femur, tibia and innominate bones (Fig.1).
Some enchondromas lead to bone expansion (enchondroma protuberans),
which simulates the appearance of an osteochondroma.
In adults, rib lesions may also be caused by enchondromas.
Radiographically, enchondromas of the hand or foot appear as
well-defined medullary lesions with some degree of calcification,
lobulation and endosteal erosion. Other features that may be present are
cortical expansion and pathologic fracture (Fig.2).
Enchondromas are the most common benign tumour of the hand, and the
presence of a solitary, well-marginated, lobulated intraosseous lesion
in the bones of the hands is highly suggestive of enchondroma.
Enchondromas in the long tubular bones most typically appear on
radiographs as centrally or eccentrically placed medullary osteolytic
tumours of variable size with or without calcification, with lobulated
erosion of the endosteal margin of the cortex. MR imaging of
enchondromas usually reveals a well-circumscribed lesion of low signal
intensity in T1-weighted spin-echo MR images and of high signal
intensity in T2-weighted spin-echo and many gradient-echo MR images (Fig.3).
Foci of calcification are observed as regions of low signal intensity.
Malignant transformation of a solitary enchondroma may sometimes
occur but is more likely in tumours in the long tubular or flat bones. Chondrosarcoma
is the usual result after malignant transformation of an enchondroma.
Radiographic findings suggesting malignant transformation include an
enlarging radiolucent area, pathologic fracture, soft tissue mass, and
disappearance of pre-existing calcification within the enchondroma (Fig.4).
 |
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Enchondroma, Fig. 4
AP (a) and lateral (b)
radiographs of the index finger demonstrate malignant degeneration of an
enchondroma into a chondrosarcoma. Note the ill-defined margins,
disrupted cortex, and adjacent soft tissue swelling.
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Enchondroma, Fig. 1
a. AP radiograph shows
punctate calcification in the humeral head and neck. b. CT scan
demonstrates an osteolytic lesion with well-defined sclerotic margins
and a chondroid matrix.
Enchondroma, Fig. 2
AP radiograph of the fourth
metacarpal demonstrates a pathologic fracture through an enchondroma.
Note the well circumscribed lobular borders and the chondroid
calcifications characteristic of an enchondroma.
Enchondroma, Fig. 3
a. Lateral radiograph of
the knee demonstrates chondroid calcifications in the distal femur due
to an enchondroma. b, c. A sagittal T1-weighted (b) and fat suppressed
T2-weighted (c) MR images demonstrates a well circumscribed lobulated
lesion of predominantly intermediate and high signal abnormality. The
foci of low signal intensity on both pulse sequences are secondary to
chondroid calcification. (Courtesy of Sergio Fernandez, MD, Mexico City,
Mexico)
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35 year old woman with wrist and hand pain, being evaluated with bone
scintigraphy to determine pattern of joint involvement.
Whole Body Bone Scintigraphy
Enchondroma and Enchondromatosis
Last Updated: May 14, 2002 |
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| Synonyms and related keywords:
chondroma, Ollier's disease, Ollier disease, benign cartilaginous
neoplasms, benign bone neoplasms, osseous neoplasms, pathologic
bone fracture, hyaline cartilage rests |
| |
AUTHOR
INFORMATION |
Section
1 of 12 
 |
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| Author: Felix
S Chew, MD, EdM, Vice-Chair for Education, Section
Head of Musculoskeletal Radiology, Professor, Department of
Radiology, Wake Forest
University School of Medicine
Coauthor(s): Catherine
Maldjian, MD, Consulting Staff, Clifton Medical
Imaging
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| Felix S Chew, MD, EdM, is a member of the following medical
societies: American Roentgen Ray
Society, Association of University Radiologists, and Radiological
Society of North America
|
| Editor(s): Michael A Bruno, MD, Chair,
Department of Radiology, Maricopa Medical Center; Bernard
D Coombs, MBChB, PhD, Assistant Professor, Department of
Radiology, University of Colorado Health Sciences Center; Murali
Sundaram, MBBS, FRCR, Department of Radiology, Mayo
Clinic of Rochester; Robert M Krasny, MD,
Visiting Assistant Professor of Radiology, University of
California at Los Angeles Medical Center; Consulting Staff, Tower
Imaging, Los Angeles, California; and Lawrence M Davis, MD,
Assistant Professor of Diagnostic Imaging (Clinical), Department
of Diagnostic Imaging, Brown Medical School |
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INTRODUCTION |
Section
2 of 12 
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Background: Enchondromas are benign cartilaginous
neoplasms in bone. The primary significance of enchondroma is related to
its complications, most notably pathologic fracture, and a small incidence
of malignant transformation, which may be associated with pathologic
fracture.
Enchondromas are usually solitary benign lesions in intramedullary
bone. When multiple enchondromas coexist, the diagnosis of
enchondromatosis should be considered.
Multiple enchondromas may occur in 3 distinct disorders: (1) Ollier
disease is a nonhereditary disorder characterized by multiple enchondromas
with a predilection for unilateral distribution. The enchondromas can grow
large and be disfiguring. (2) Maffucci syndrome is nonhereditary, is less
common than Ollier disease. This syndrome results in multiple hemangiomas
in addition to enchondromas. (3) Metachondromatosis consists of multiple
enchondromas and osteochondromas, and it is the only 1 of the 3 disorders
that is hereditary. Metachondromatosis is inherited by autosomal dominant
transmission.
Pathophysiology: Enchondromas are ectopic hyaline
cartilage rests in intramedullary bone. The lesions replace normal bone
with mineralized or unmineralized hyaline cartilage, thereby generating a
lytic pattern on radiographs or, more commonly, a lytic area containing
rings and arcs of chondroid calcifications. The lesions probably arise
from cartilaginous rests that are displaced from the growth plate.
Endosteal growth may occur and does not imply malignant transformation
in the hands and feet, in which the lesions appear to be more cellular.
Although the extent of cellularity is not correlated with malignant
transformation, mitotic figures are seldom seen in the lesions, and their
presence may be correlated with malignancy. Pathologic fracture
predisposed by thinning of the cortex is not typically associated with
malignancy in the hands and feet; however, in other areas such as the long
bones and flat bones, pathologic fracture is suggestive of malignant
transformation.
Complete transgression through the cortex with a soft tissue component
is highly suggestive of malignancy. Although malignant transformation to
several types of tumors is reported, chondrosarcoma is the most common by
far.
Frequency:
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In the US: Enchondromas account for 12-14% of
benign bone neoplasms and 3-10% of osseous neoplasms in general. |
Mortality/Morbidity: Most often, enchondromas are of
no consequence, and patients are asymptomatic. Enchondromas are not life
threatening; however, painful malignant transformation should be the
primary concern and cannot be excluded, even in the presence of a benign
appearance on images. Malignant transformation is virtually nonexistent in
the hands and feet but may be seen in the long bones and flat bones.
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In a patient with enchondromatosis, the incidence of chondrosarcoma
is much higher than in other patients, and the rate may be as high as
50%. |
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In Maffucci syndrome, hemangiomas also may undergo sarcomatous
transformation; however, osseous lesions do so more frequently and
most commonly result in chondrosarcomas. |
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In the event of malignant transformation to chondrosarcoma, patients
with low-grade chondrosarcoma have a 5-year survival rate of 65-85%,
whereas patients with highest grade chondrosarcomas have a 5-year
survival rate of 15%. |
Race: No racial predilection is known.
Sex: Enchondromas occur equally in males and females.
Age: Solitary enchondromas most often are discovered
in those aged 20-40 years. Ollier disease is usually detected in those
aged 0-10 years.
Anatomy: Solitary enchondromas are intramedullary
lesions, although they may expand enough to cause endosteal scalloping of
the cortex. They have a predilection for small bones of the hands and
feet, where most occur. Of these, half are in the proximal phalanx,
followed in frequency by the metacarpal and middle phalanx and, lastly, by
the distal phalanges and carpus. Other locations are the shoulder, pelvis,
and long bones. Enchondromas tend to occupy the diaphyseal region in the
short tubular bones and metaphyseal region in the longer bones. Ollier
disease occurs with highest frequency in the long bones.
Approximately 50% of solitary enchondromas are found in the hands,
typically in the middle and distal portions of the metacarpals and the
proximal portions of the phalanges. The next most common sites are the
proximal and distal parts of the femur and the proximal part of the
humerus. Enchondromas at the mid shaft of the tibia are rare.
Clinical Details: When patients have pain, malignant
transformation should be suspected, even in the absence of suggestive
radiographic findings. Rapid growth of the lesion may also be suggestive
of malignancy. Enchondromas are metabolically active and may continue to
grow and evolve throughout the patient's lifetime. Progressive
calcification over a period of years is not unusual. Calcification in
enchondromas is progressive. Loss of calcification in a focal region
suggests malignant degeneration with destruction of the underlying
enchondroma by sarcomatous tissue.
Primary clinical complications include pathologic fracture and
malignant transformation, which may be concomitant. In particular,
pathologic fracture does not imply malignant transformation in the hands
and feet, in which endosteal scalloping and cortical thinning may be
extensive without malignant transformation.
When the lesion has calcifications, the primary differential diagnoses
are bone infarct and chondrosarcoma.
When the lesion is purely lytic, as shown on radiographs, the
differential diagnosis consists of benign lytic lesions such as
nonossifying fibroma, simple bone cyst, fibrous dysplasia, eosinophilic
granuloma, and clear cell chondrosarcoma (which tends to involve the end
of the bone, in particular, the proximal humerus).
Preferred Examination: Initially, radiography is the
imaging modality of choice. If further characterization is necessary, MRI
or CT is performed. If uncertainty remains, bone scanning may be helpful,
but some lesions require biopsy.
Limitations of Techniques: X-ray findings may not
demonstrate or adequately characterize subtle calcifications to the extent
that CT scans do. When calcifications are not present on either
examination, MRI may be performed. MRI should reveal the classic
appearance of noncalcified hyaline cartilage. Dystrophic calcifications in
bone infarcts may be difficult to differentiate from chondroid matrix with
ionizing radiation, and MRI may be helpful.
Occasionally, dystrophic calcifications in bone infarcts may be
difficult to differentiate from enchondromas by using plain radiographs.
When a clearly serpiginous rind of sclerosis encapsulates the lesion, the
diagnosis of bone infarct is straightforward; however, when this finding
is not present, MRI may be useful in differentiating the 2 entities.
In lesions in which calcifications are not seen radiographically, the
diagnosis may be made with the help of MRIs. CT scans may demonstrate the
presence of calcification not depicted on radiographs.
Chondrosarcoma, in particular low-grade chondrosarcoma, may be
indistinguishable from enchondroma. Certain imaging features may be
helpful in distinguishing enchondroma from chondrosarcoma (see X-Ray
section below). However, even a lesion with no imaging features suggestive
of malignancy and an imaging appearance compatible with an enchondroma
should be resected when it is associated with pain. Low-grade
chondrosarcoma may be indistinguishable from enchondroma on all images,
and the diagnosis may be difficult, even with histologic evaluation.
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DIFFERENTIALS |
Section
3 of 12
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Chondrosarcoma
Other Problems to be Considered:
Bone infarct
Benign lytic lesions - Nonossifying fibroma, simple bone cyst, fibrous
dysplasia, eosinophilic granuloma, clear cell chondrosarcoma
Findings: A classic pattern of calcifications,
described as rings and arcs, is the most characteristic finding; this
finding is pathognomonic when it is seen in the hands. In the long bones,
calcifications may be difficult to distinguish from the dystrophic
calcifications seen in bone infarction. Radiographs may not depict the
rings and arcs of calcifications, particularly in the hands and feet.
Low-grade chondrosarcoma may be indistinguishable from enchondroma;
however, in most cases, chondrosarcoma has certain imaging features that
are indicative of its aggressive behavior.
Cortical breakthrough, soft-tissue mass, and deep endosteal scalloping
of the cortex are 3 features that are described more frequently in
chondrosarcoma. However, with the caveat that in the small bones of the
hands and feet, deep endosteal scalloping with consequent pathologic
fracture does not imply malignancy, because enchondromas are more cellular
and expansile in these locations.
In Ollier disease, enchondromas often appear to be larger than they do
in other conditions.
Because enchondromas occur in young patients and can be large, growth
of the affected limbs may be adversely affected, and pathologic fractures
may occur. Enchondromatosis can occasionally have the appearance of linear
lucencies, in which the chondrocytes appear to line up in a vertical
orientation along the length of the bone.
In Maffucci syndrome, associated soft-tissue hemangiomas are seen. Soft
tissue hemangiomas typically have numerous rounded calcifications with
central lucencies, which are consistent with phleboliths on plain
radiograph. T2-weighted MRIs of these lesions show high-signal-intensity
tubular structures, which are consistent with slow flow in vascular
channels.
Metachondromatosis has associated osteochondromas, which differ from
conventional osteochondromas in that they point toward rather than away
from the joint.
Degree of Confidence: On radiographs, the degree of
confidence in the diagnosis of an enchondroma depends on the clinical
situation and the appearance of the lesion. In the presence of pain,
almost regardless of appearance, further investigation is warranted. A
lesion that is predominantly lucent often requires further investigation,
as does a lesion that is nonuniformly mineralized.
False Positives/Negatives: Enchondroma and a low-grade
chondrosarcoma cannot be reliably distinguished on the basis of
radiographic findings alone in most cases. A calcified enchondroma can
also mimic the appearance of a calcified marrow infarct.
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Findings: The features of enchondroma on CT scan are
the same as those depicted on radiographs.
Enchondromas are endosteal lesions with a lobular morphology and
variable mineralization. Often, the mineralization is in the form of rings
and arcs, which correspond to calcification around lobules of cartilage. A
pathologic fracture may be present. Sometimes, endosteal scalloping is
present, but this feature may be suggestive of degeneration of the
enchondroma to a chondrosarcoma. Enchondromas should not penetrate the
cortex or extend into the soft tissues.
If a densely mineralized or uniformly mineralized lesion has a region
that is lucent, degeneration of the enchondroma to a chondrosarcoma is
suggested, and biopsy is probably necessary.
Degree of Confidence: With CT, degree of confidence in
the diagnosis of an enchondroma depends on the clinical situation and the
appearance of the lesion. The absence of pain, uniform or dense
mineralization, and endosteal scalloping or cortical penetration are all
indications of an enchondroma. However, if any of these features is
absent, further investigation may be necessary.
False Positives/Negatives: Distinguishing an
enchondroma from a low-grade chondrosarcoma is often problematic;
sometimes, the diagnosis cannot be resolved without a biopsy. Because
cartilage lesions often are not uniform at histologic examination, the
entire lesion may need to be removed at biopsy.
Findings: MRI findings may be useful in distinguishing
enchondromas in the long bones from bone infarcts.
Typically, bone infarcts are encapsulated by a serpiginous rind of
decreased signal intensity on T1- and T2-weighted images. Frequently, this
rind is subtended by a high signal intensity line. This finding has been
called the double-line sign, and it consists of a high-signal-intensity
rind circumscribed by and immediately adjacent to a low-signal-intensity
rind on T2-weighted images.
Conversely, enchondromas tend to have lobulated borders with a cluster
of numerous tiny locules of high-signal-intensity foci on T2-weighted
images that appear to coalesce with one another and reflect the high fluid
content of hyaline cartilage. On T1-weighted images, enchondromas
demonstrate low-to-intermediate signal intensity.
When a calcific chondroid matrix is observed on radiographs, decreased
signal intensity is expected to occur in those areas on images obtained
with all MRI pulse sequences. These areas may become particularly
pronounced on gradient-echo images.
Degree of Confidence: When combined with a typical
appearance on radiographs and an absence of pain, the diagnosis of
enchondroma with MRI finding can be made with a high degree of confidence.
Enchondromas may also be incidental findings at routine examination, such
as MRI of the knee. If the lesions have a typical appearance, a confident
diagnosis may be made; however, the acquisition of correlative
radiographic findings is often prudent.
False Positives/Negatives: Occasionally, enchondromas
can be difficult to distinguish from other intraosseous benign and
malignant lesions on the basis of MRI findings alone. Correlation with
other results, particularly radiographic results, may help. The presence
of bone pain always warrants further investigation.
| |
ULTRASOUND |
Section
7 of 12
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Findings: Ultrasonography is not used in the diagnosis
of enchondroma.
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NUCLEAR
MEDICINE |
Section
8 of 12
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Findings: With technetium-99m radionuclide bone
scanning, findings are typically negative in uncomplicated enchondromas,
but the presence of pathologic fracture results in intense activity at the
fracture site. Radionuclide bone scans in patients with multiple
enchondromas or patients in whom an enchondroma is actively calcifying may
show increased activity, but the activity typically is less intense than
that of intramedullary chondrosarcoma. Intramedullary chondrosarcomas
demonstrate increased activity on radionuclide bone scans.
Degree of Confidence: Radionuclide bone scanning is
one method used to assess lesions depicted on radiographs or MRIs that are
presumed to be enchondromas. If the bone scan results are negative, the
possibility of a malignancy (eg, chondrosarcoma) is extremely remote. If
the scan results are positive, biopsy is typically necessary. Radionuclide
bone scan findings are not reliable in differentiating enchondroma from
calcified marrow infarct.
False Positives/Negatives: Radionuclide bone scan
findings are nonspecific; therefore, one cannot reliably diagnose a focal
bone lesion on the basis of these findings alone. However, the physiologic
information provided by a bone scan, when combined with information from
other imaging modalities, can be helpful.
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ANGIOGRAPHY |
Section
9 of 12
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Findings: Angiography is not used in the diagnosis or
evaluation of solitary enchondroma. In Maffucci syndrome, angiography may
play a secondary role in identifying the extent and origin of the vascular
malformation.
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INTERVENTION |
Section
10 of 12
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Intervention: CT-guided percutaneous needle biopsy
occasionally is indicated in the management of enchondroma. However,
because benign and low-grade malignant cartilage lesions may be difficult
to distinguish at histologic examination, the risk of sampling error
usually precludes a confident diagnosis. Therefore, needle biopsy is not
generally useful.
If CT scans show a densely mineralized or uniformly mineralized lesion
with a lucent region, degeneration of the enchondroma to a chondrosarcoma
is suggested, and biopsy is probably necessary.
| |
PICTURES |
Section
11 of 12
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| Caption: Picture
1. Enchondroma and enchondromatosis. Frontal radiograph of the
right hand demonstrates a lytic expansile lesion in the fifth
metacarpal bone, with thinning of the cortex with a somewhat
scalloped appearance (in the same patient as in Image 2). A
pathologic fracture is noted. No appreciable calcifications are
seen in the lesion. |
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| Caption: Picture
2. Enchondroma and enchondromatosis. Detail of a lytic expansile
lesion in the fifth metacarpal bone in the right hand (in the same
patient as in Image 1). There is thinning of the cortex with a
somewhat scalloped appearance. A pathologic fracture is noted. No
appreciable calcifications are seen in the lesion. |
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| Picture Type:
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| Caption: Picture
3. Enchondroma and enchondromatosis. Frontal radiograph of the
left hand demonstrates an expansile lytic lesion in the proximal
phalanx of the fifth digit with a distinct zone of transition,
thinning of the cortex, and a pathologic fracture (in the same
patient as in Image 4). The lesion involves the diaphysis and
approaches the end of the bone near the metacarpophalangeal joint.
This finding is not uncommon in enchondromas in the small bones.
Note the fuzzy calcifications in the matrix of the lesion. |
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| Caption: Picture
4. Enchondroma and enchondromatosis. Detail of an expansile lytic
lesion in the proximal phalanx of the fifth digit with a distinct
zone of transition, thinning of the cortex, and a pathologic
fracture (in the same patient as in Image 3). |
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7. Enchondroma and enchondromatosis. Axial CT image demonstrates a
fluffy calcific matrix within the medullary canal of the distal
femur (in the same patient as in Images 5-6). The surrounding
cortex is intact. |
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| Caption: Picture
9. Enchondroma and enchondromatosis. Axial T1-weighted MRI shows
an intramedullary lesion with low signal intensity and lobular
morphology in the distal femur (in the same patient as in Images
8-12). The endosteal aspect of the cortex is not affected. |
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| Caption: Picture
10. Enchondroma and enchondromatosis. Coronal T1-weighted MRI
image demonstrates predominantly decreased signal intensity within
a lesion in the distal femur (in the same patient as in Images
8-12). The lesion has a lobular morphology. No endosteal
scalloping is noted. |
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11. Enchondroma and enchondromatosis. Axial T2-weighted MRI shows
regions of high signal intensity in the lesion (in the same
patient as in Images 8-12). No surrounding edema is noted. |
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12. Enchondroma and enchondromatosis. Coronal T2-weighted MRI
image demonstrates small lobulated foci of increased signal
intensity separated by a background mesh of decreased signal
intensity (in the same patient as in Images 8-11). The adjacent
cortex is intact. |
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| Picture Type: MRI |
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BIBLIOGRAPHY |
Section
12 of 12
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| Bauer HC, Brosjo O, Kreicbergs A: Low risk of recurrence of
enchondroma and low-grade chondrosarcoma in extremities. 80 patients
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| Flemming DJ, Murphey MD: Enchondroma and chondrosarcoma. Semin
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| Healey JH, Lane JM: Chondrosarcoma. Clin Orthop 1986 Mar; (204):
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| Hudson TM, Chew FS, Manaster BJ: Radionuclide bone scanning of
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| Jaffe HL, Lichtenstein L: Solitary benign enchondroma of bone. Arch
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| Murphey MD, Flemming DJ, Boyea SR, et al: Enchondroma versus
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| Peiper M, Zornig C: Chondrosarcoma of the thumb arising from a
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| Robbin MR, Murphey MD: Benign chondroid neoplasms of bone. Semin
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| Unni KK: Chondroma. In: Dahlin's Bone Tumors: General Aspects and
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1311-6[Medline]. |
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