eMedicine - Chondrosarcoma : Article by Geoff Hide, = MBBS, MRCP, FRCR

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Chondrosarcoma

Last Updated: = December=20 13, 2002

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Synonyms and related = keywords:=20 bone tumors, primary bone tumor, bone malignancy, primary = osseous=20 neoplasms, sarcomas, chondroid, conventional = chondrosarcomas,=20 central chondrosarcoma, peripheral chondrosarcoma, = enchondroma,=20 osteochondroma, clear cell chondrosarcoma, myxoid = chondrosarcoma,=20 mesenchymal chondrosarcoma, dedifferentiated chondrosarcoma=20

AUTHOR INFORMATION=20 Section 1 of = 12   =20

Author=20 Information Introduction=20 Differentials=20 X-ray<= /A>=20 Cat= =20 Scan MRI = U= ltrasound=20 Nuclear=20 Medicine = Angiography=20 Intervention=20 Pic= tures=20 Bibliography

Author: Geoff=20 Hide, MBBS, MRCP, FRCR, Consultant = Musculoskeletal=20 Radiologist, Department of Radiology, Freeman Hospital; = Honorary=20 Clinical Lecturer, Faculty of Medical Sciences, University = of=20 Newcastle upon Tyne

Geoff Hide, MBBS, MRCP, FRCR, is a member of the following = medical societies: British=20 Medical Association, Royal=20 College of Physicians, and Royal=20 College of Radiologists

Editor(s): Michael A Bruno, MD, Chair,=20 Department of Radiology, Maricopa Medical Center; = Bernard D=20 Coombs, MBChB, PhD, Consulting Staff, Department of = Specialist Rehabilitation Services, Hutt Valley District = Health=20 Board, New Zealand; Murali Sundaram, MBBS, = FRCR,=20 Department of Radiology, Mayo Clinic of Rochester; = Robert M=20 Krasny, MD, Visiting Assistant Professor of = Radiology,=20 University of California at Los Angeles Medical Center; = Consulting=20 Staff, Tower Imaging, Los Angeles, California; and = Felix S=20 Chew, MD, EdM, Vice-Chair for Education, Section = Head of=20 Musculoskeletal Radiology, Professor, Department of = Radiology, Wake=20 Forest University School of Medicine =

Background:=20 Chondrosarcoma is the second most frequent primary = malignant=20 tumor of bone, representing approximately 25% of all primary = osseous=20 neoplasms. Chondrosarcomas are a group of tumors with highly = diverse=20 features and behavior patterns, ranging from slow-growing=20 non-metastasizing lesions to highly aggressive metastasizing = sarcomas.

Pathophysiology: Chondrosarcoma is a malignant = tumor=20 of cartilaginous origin, in which the tumor matrix formation is = entirely=20 chondroid in nature.=20
Chondrosarcomas are classified as central (originating within = the=20 intramedullary canal) or peripheral. Rarely, they may arise as = juxta=20 cortical lesions. Lesions are designated as primary when they = arise de=20 novo or as secondary when they occur within a preexisting lesion = such as=20 an enchondroma or osteochondroma.=20
Tumors are further categorized by grade. Grade 1 represents the = least=20 aggressive in terms of histologic features, and grade 3 represents = the=20 most aggressive. Most chondrosarcomas are pathologically = classified as=20 conventional, but other subgroups are clear cell, myxoid, = mesenchymal, and=20 dedifferentiated. This article deals exclusively with conventional = chondrosarcomas of the bone.

Frequency:

In the US: The incidence rate of = chondrosarcoma is=20 dependent on patient age, peaking at 8 cases per 1 million = population in=20 those aged 80-84 years. The incidence in children is low. Most = tumors=20 arise in patients older than 40 years. The risk of = chondrosarcoma is=20 increased in people with enchondromatosis syndromes (eg, Ollier = disease,=20 Maffucci syndrome, metachondromatosis) and in those with = hereditary=20 multiple exostosis (eg, diaphyseal aclasis). Patients with these = conditions are generally younger than other patients at = presentation.=20
Mortality/Morbidity: The overall prognosis is = related=20 to the size of the lesion, its anatomic location, and its = histologic=20 grade.=20

Axial lesions have a worse prognosis than those in the = appendicular=20 skeleton.

The 5-year survival rate for grade 1 lesions is 90%, and the = rate=20 decreases to 29% for grade 3 tumors. Grade 1 lesions do not = metastasize.=20 Metastatic spread, typically pulmonary, is more frequently = associated=20 with grade 3 lesions than with others. Lymph node spread is more = common=20 than with other osseous neoplasms.

Tumor recurrence typically occurs 5-10 years after surgery, = and it=20 is often associated with more aggressive behavior and a = histologic grade=20 higher than that of the original lesion.
Race: No major difference in incidence is = observed=20 between ethnic groups.=20
Sex: A slight male predilection exists, with a = male-to-female ratio of 1.5-2:1.=20
Age: The age range is wide, but most cases = occur in=20 patients older than 40 years.=20

Secondary chondrosarcomas tend to occur in younger patients, = namely,=20 those aged 20-40 years.

Chondrosarcoma is rare in children, but it tends to be = aggressive.=20
Anatomy: Tumors are predominantly axial, and = they most=20 commonly involve the pelvic bones, femur, humerus, ribs, scapula, = sternum,=20 or spine. In tubular bones, the metaphysis is the most common site = of=20 origin. The proximal metaphysis is more frequently involved than = the=20 distal end of the bone. Involvement of the distal humerus is most = unusual.=20 Chondrosarcoma is rare in the hands and feet and usually occurs as = a=20 complication of a multiple enchondromatosis syndrome. = Chondrosarcoma=20 arising de novo in the hands and feet is also extremely unusual.
Clinical Details: The most common symptom at=20 presentation is pain, which is often present for months and = typically dull=20 in character. It may be worse at night. Local swelling may be = present, and=20 when the tumor occurs close to a joint, effusion may be present, = or=20 movement may be restricted. The average duration of symptoms prior = to=20 presentation is 1-2 years. The tumor may occasionally occur as a=20 pathologic fracture.
Preferred Examination: Radiographs are = essential for=20 the initial diagnosis, and it is sometimes supplemented with CT, = which is=20 more sensitive for detecting matrix calcification and for = confirming deep=20 endosteal cortical scalloping in intramedullary tumors.=20
MRI is the preferred modality for evaluating the extent of=20 intramedullary tumors and demonstrating extraosseous extension. = MRI is=20 useful in evaluating the thickened cartilage cap in an = osteochondroma that=20 develops a secondary chondrosarcoma. MRI is less sensitive than CT = in=20 identifying small amounts of matrix calcification within a tumor.=20
The imaging appearances of chondrosarcoma can overlap with = those of=20 other lesions, especially other cartilaginous tumors such as = enchondroma.=20 The presence of pain with any lesion (without a pathologic = fracture in=20 lesions of the hands and feet) is highly suggestive of malignancy. = Other=20 findings suggestive of malignancy in a cartilaginous tumor include = endosteal cortical scalloping of more than two thirds of the = thickness of=20 the cortex, ill-defined border and/or zone of transition, and a = large=20 soft-tissue mass. Both benign and malignant cartilaginous tumors = may show=20 a central lucency. However, lucency of an area that previously = showed=20 matrix calcification is a highly suggestive feature.
Limitations of Techniques: See Preferred = Examination=20 above.

INTRODUCTION = Section 2 of = 12

Author=20 Information Introduction=20 Differentials=20 X-ray<= /A>=20 Cat= =20 Scan MRI = U= ltrasound=20 Nuclear=20 Medicine = Angiography=20 Intervention=20 Pic= tures=20 Bibliography

Bone Infarct =
Enchondroma = and=20 Enchondromatosis
Osteochondroma and=20 Osteochondromatosis
Osteosarcoma, = Classic=20
Osteosarcoma, = Variants=20

Other Problems to be Considered:
Central =
Enchondroma
Osteosarcoma
Fibrosarcoma
Bone=20 infarct

Peripheral
Osteochondroma
Parosteal=20 osteosarcoma

DIFFERENTIALS = Section 3 of = 12

Author=20 Information Introduction=20 Differentials=20 X-ray<= /A>=20 Cat= =20 Scan MRI = U= ltrasound=20 Nuclear=20 Medicine = Angiography=20 Intervention=20 Pic= tures=20 Bibliography

Findings: Radiographs typically show a lucent = lesion,=20 which frequently contains matrix calcification, particularly in=20 well-differentiated tumors. The degree of organization of the = matrix=20 calcification can be correlated with the grade of the tumor. = Aggressive=20 tumors contain irregular calcifications, and they often have large = areas=20 showing no calcification at all. Well-differentiated lesions tend = to have=20 more developed matrix with the typical appearance of rings and = arcs.=20
The margin of intramedullary lesions is determined by the = degree of=20 aggression of the tumor, and it is frequently ill defined. = Endosteal=20 scalloping may be present, and when its depth is more than two = thirds the=20 normal thickness of the cortex, this scalloping is useful in=20 distinguishing chondrosarcoma from enchondroma, except in lesions = of the=20 hands and feet. Benign enchondromas in these areas can cause = considerable=20 cortical thinning, and they may occur as a pathologic fracture.=20
Cortical destruction and/or a soft-tissue mass are indicators = of the=20 malignant nature of the tumor. Destruction of matrix calcification = that=20 was previously visible in an enchondroma is also an indicator of = malignant=20 transformation.
Degree of Confidence: The size of the tumor is = often=20 poorly assessed on radiographs alone, and MRI is advised to = demonstrate=20 both the intramedullary and the soft-tissue extent of the lesion. = CT can=20 be helpful in identifying matrix calcification in some lesions = that appear=20 entirely lucent on radiographs.

X-RAY = Section 4 of = 12

Author=20 Information Introduction=20 Differentials=20 X-ray<= /A>=20 Cat= =20 Scan MRI = U= ltrasound=20 Nuclear=20 Medicine = Angiography=20 Intervention=20 Pic= tures=20 Bibliography

Quick Find

Author=20 Information
Introduction
Differentials
X-ray<= /A>
Cat= =20 Scan
MRI<= BR>U= ltrasound
Nuclear=20 Medicine
= Angiography
Intervention
Pic= tures
Bibliography

Click=20 for related images.

Related Articles

Bone Infarct=20

Enchondroma=20 and Enchondromatosis

Osteochondroma=20 and Osteochondromatosis

Osteosarcoma,=20 Classic

Osteosarcoma,=20 Variants

Continuing Education =

CME available for this topic. = Click here=20 to take this=20 = CME.

Patient Education

Click here=20 for patient education. =

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Findings: In as many as 90% of cases, tumors = appear as=20 lucent areas containing chondroid matrix calcification. Endosteal=20 scalloping and cortical destruction are frequently easier to = appreciate on=20 CT scans than on radiographs.=20
CT can be used to guide percutaneous biopsy, and it is the = modality of=20 choice for investigating possible pulmonary metastatic disease.
Degree of Confidence: CT can often be used to=20 successfully categorize the lesion as being of cartilaginous = origin. Its=20 medullary extent can be assessed more accurately with CT than with = radiography. However, MRI is superior, and it is also the most = useful=20 modality for determining soft tissue extension.

CAT SCAN = Section 5 of = 12

Author=20 Information Introduction=20 Differentials=20 X-ray<= /A>=20 Cat= =20 Scan MRI = U= ltrasound=20 Nuclear=20 Medicine = Angiography=20 Intervention=20 Pic= tures=20 Bibliography

Findings: MRI typically demonstrates lobulated = lesions=20 of high signal intensity on T2-weighted images. Lobules are = commonly=20 separated by low-signal-intensity septa. On T1-weighted images, = the lesion=20 generally has low signal intensity.=20
Areas of matrix calcification are shown as signal voids on = images=20 obtained with all sequences, but small amounts may not be = identifiable.=20 MRI may demonstrate large aggregates of calcium, but tiny = scattered=20 calcifications may be completely missed because of partial-volume=20 averaging. MRI can be used to assess soft tissue extension and the = intramedullary extent of the tumor. MRIs can demonstrate endosteal = cortical scalloping, but this feature is more easily assessed by = using CT.=20
MRI is useful in assessing the thickness of the cartilage cap = of=20 osteochondromas to identify chondrosarcoma transformation. = Chondrosarcomas=20 show variable patterns of enhancement after the administration of = contrast=20 material.
Degree of Confidence: MRI is the method of = choice for=20 clarifying the intramedullary and extraosseous extent of a = chondrosarcoma,=20 but features related to cortical bone and matrix calcification are = more=20 accurately assessed by using CT.

MRI Section 6 of = 12    <= IMG=20 height=3D10 alt=3D"Click here to go to the next section in = this topic"=20 src=3D"http://www.emedicine.com/images/next3.gif" width=3D31 = align=3Dmiddle border=3D0>

Author=20 Information Introduction=20 Differentials=20 X-ray<= /A>=20 Cat= =20 Scan MRI = U= ltrasound=20 Nuclear=20 Medicine = Angiography=20 Intervention=20 Pic= tures=20 Bibliography

Findings: Ultrasonography has no role in = evaluating=20 intramedullary lesions confined to the bone. It can demonstrate = soft=20 tissue extension; therefore, it may be useful in guiding = percutaneous=20 biopsy.=20
Ultrasonography is useful as a means of assessing the thickness = of the=20 cartilage cap overlying an osteochondroma, although access to the = lesion=20 can be difficult in certain areas. If the cap measures more than = 1.5 cm in=20 a skeletally mature patient, transformation to chondrosarcoma may = have=20 occurred.

ULTRASOUND = Section 7 of = 12

Author=20 Information Introduction=20 Differentials=20 X-ray<= /A>=20 Cat= =20 Scan MRI = U= ltrasound=20 Nuclear=20 Medicine = Angiography=20 Intervention=20 Pic= tures=20 Bibliography

Findings: Central chondrosarcomas typically = show=20 significantly increased uptake of the radioisotope on isotopic = bone scans,=20 but differentiation between chondrosarcoma and enchondroma is = unreliable.=20 Uptake on isotopic bone scanning indicates metabolic activity in = an=20 osteochondroma, but it cannot be used to distinguish this type of = uptake=20 from uptake due to malignant transformation. The absence of = increased=20 uptake makes malignancy highly unlikely.
Degree of Confidence: See Findings above.

NUCLEAR MEDICINE = Section 8 of = 12   <= IMG=20 height=3D10=20 alt=3D"Click here to go to the previous section in this = topic"=20 src=3D"http://www.emedicine.com/images/back3.gif" width=3D31 = align=3Dmiddle border=3D0> =

Author=20 Information Introduction=20 Differentials=20 X-ray<= /A>=20 Cat= =20 Scan MRI = U= ltrasound=20 Nuclear=20 Medicine = Angiography=20 Intervention=20 Pic= tures=20 Bibliography

Findings: Angiography is generally not = required in the=20 staging of chondrosarcoma.

ANGIOGRAPHY = Section 9 of = 12

Author=20 Information Introduction=20 Differentials=20 X-ray<= /A>=20 Cat= =20 Scan MRI = U= ltrasound=20 Nuclear=20 Medicine = Angiography=20 Intervention=20 Pic= tures=20 Bibliography

Intervention: Histologic confirmation of the = nature of=20 a lesion is required for optimal management. Although biopsy is = often=20 performed as an open surgical procedure, percutaneous biopsy with = imaging=20 guidance can be helpful in certain scenarios. Ultrasonography is = useful=20 for guiding needle biopsy of the soft tissue component of a tumor. = CT-guided percutaneous biopsy of bone lesions may be helpful, but = the=20 pathologic differentiation between benign and low-grade malignant=20 chondrosarcomas is difficult. Needle biopsy samples may be = inadequate to=20 allow precise pathologic diagnosis, and open surgical biopsy is = often=20 performed.=20
The mainstay of treatment is surgical resection because = chondrosarcomas=20 respond poorly to chemotherapy or radiation therapy.
Medical/Legal Pitfalls:

Although they are malignant tumors, chondrosarcomas often = show=20 features suggestive of a benign lesion.

Readers should be cautious not to mistake them for benign = lesions=20 because of their well-circumscribed appearance and lack of = periosteal=20 reaction.

INTERVENTION = Section 10 of=20 12   =

Author=20 Information Introduction=20 Differentials=20 X-ray<= /A>=20 Cat= =20 Scan MRI = U= ltrasound=20 Nuclear=20 Medicine = Angiography=20 Intervention=20 Pic= tures=20 Bibliography

PICTURES = Section 11 of=20 12

Author=20 Information Introduction=20 Differentials=20 X-ray<= /A>=20 Cat= =20 Scan MRI = U= ltrasound=20 Nuclear=20 Medicine = Angiography=20 Intervention=20 Pic= tures=20 Bibliography

Caption: = Picture 1.=20 Chondrosarcoma. Frontal radiograph of the left fibula head=20 demonstrates a lucent lesion that contains the typical = chondroid=20 matrix calcification. Low-grade tumor.

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Picture = Type:=20 X-RAY

Caption: = Picture 2.=20 Chondrosarcoma. Frontal radiograph of the left acetabulum=20 demonstrates an expansile lucent lesion with no internal = matrix=20 calcification (same patient as in Images 2-5). Low-grade = central=20 tumor.

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Picture = Type:=20 X-RAY

Caption: = Picture 3.=20 Chondrosarcoma. Bone-window CT scan of left acetabulum = demonstrates=20 matrix calcification in the expansile lucent lesion in the = anterior=20 column (same patient as in Images 2-5). Low-grade central = tumor.=20

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Picture = Type:=20 CT

Caption: = Picture 4.=20 Chondrosarcoma. T2-weighted axial MRI of the pelvis = demonstrates the=20 high signal intensity of the acetabular lesion (same patient = as in=20 Images 2-5). Low-grade central tumor.

3D"Click <= /TD> View Full=20 Size Image

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Picture = Type:=20 MRI

Caption: = Picture 5.=20 Chondrosarcoma. T1-weighted axial MRI of the pelvis = demonstrates the=20 low signal intensity of the acetabular lesion (same patient = as in=20 Images 2-5). Low-grade central tumor.

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Picture = Type:=20 MRI

Caption: = Picture 6.=20 Chondrosarcoma. Frontal radiograph of right side of upper = abdomen=20 demonstrates a destructive, expansile lesion of the 12th = rib. The=20 lesion contains irregular calcification (same patient as in = Image=20 7). High-grade central tumor.

3D"Click <= /TD> View Full=20 Size Image

eMedicine=20 Zoom View (Interactive!)

Picture = Type:=20 X-RAY

Caption: = Picture 7.=20 Chondrosarcoma. CT of the right side of the upper abdomen=20 demonstrates the expansile tumor with a large associated = soft-tissue=20 mass containing foci of calcification (same patient as in = Image 6).=20 High-grade central tumor.

3D"Click <= /TD> View Full=20 Size Image

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Picture = Type:=20 CT

Caption: = Picture 8.=20 Chondrosarcoma. Frontal radiograph of the pelvis = demonstrates=20 extensive calcification overlying the left ilium and in the = lateral=20 soft tissues. No bone destruction is shown (same patient as = in=20 Images 9-10). High-grade secondary peripheral tumor. =

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Picture = Type:=20 X-RAY

Caption: = Picture 9.=20 Chondrosarcoma. CT scan of the pelvis demonstrates a large=20 soft-tissue mass that contains calcification arising from a=20 broad-based sessile osteochondroma on the posterior aspect = of the=20 ilium (same patient as in Images 8 and 10). High-grade = secondary=20 peripheral tumor.

3D"Click <= /TD> View Full=20 Size Image

eMedicine=20 Zoom View (Interactive!)

Picture = Type:=20 CT

Caption: = Picture 10.=20 Chondrosarcoma. T2-weighted axial MRI of the pelvis = demonstrates a=20 lobulated high-signal-intensity soft tissue with=20 local-signal-intensity septa arising from the osteochondroma = on the=20 posterior aspect of the ilium (same patient as in Images = 9-10).=20 Several areas of low signal intensity are shown; these = correspond to=20 focal areas of dense calcification. This appearance is = typical of=20 cartilaginous material. High-grade secondary peripheral = tumor.=20

3D"Click = View Full=20 Size Image

eMedicine=20 Zoom View (Interactive!)

Picture = Type:=20 MRI

Caption: = Picture 11.=20 Chondrosarcoma. Lateral radiograph of the distal femur in a = patient=20 with hereditary multiple exostoses (same patient as in = Images=20 12-13). Several osteochondromas of varying appearances arise = from=20 the metaphyseal region; these typically grow away from the = joint.=20 Soft tissue calcification is shown overlying the most = posterior=20 osteochondroma. High-grade secondary peripheral tumor. =

3D"Click = View Full=20 Size Image

eMedicine=20 Zoom View (Interactive!)

Picture = Type:=20 X-RAY

Caption: = Picture 12.=20 Chondrosarcoma. CT scan of the distal femur demonstrates a=20 broad-based osteochondroma with a thick overlying = soft-tissue cap=20 that contains focal calcification. The metaphyseal contour = is=20 irregular because of the presence of several other = osteochondromas=20 in this patient with hereditary multiple exostoses (same = patient as=20 in Images 11 and 13). High-grade secondary peripheral tumor. =

3D"Click = View Full=20 Size Image

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Picture = Type:=20 CT

Caption: = Picture 13.=20 Chondrosarcoma. Fast spin-echo T2-weighted axial MRI of the = distal=20 femur in a patient with hereditary multiple exostoses (same = patient=20 as in Images 11-12). Image demonstrates the thick cartilage = cap=20 overlying a broad-based osteochondroma. Areas of focal = reduced=20 signal intensity in the cartilage cap correspond to foci of = dense=20 calcification. High-grade secondary peripheral tumor. =

3D"Click View Full=20 Size Image

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Picture = Type:=20 MRI

Bauer HC, Brosjo O, Kreicbergs A, Lindholm J: Low risk of = recurrence=20 of enchondroma and low-grade chondrosarcoma in extremities. 80 = patients=20 followed for 2-25 years. Acta Orthop Scand 1995 Jun; 66(3): = 283-8[Medline].=20
Flemming DJ, Murphey MD: Enchondroma and chondrosarcoma. = Semin=20 Musculoskelet Radiol 2000; 4(1): 59-71[Medline].=20
Healey JH, Lane JM: Chondrosarcoma. Clin Orthop 1986 Mar; = (204):=20 119-29[Medline].=20
Hudson TM, Chew FS, Manaster BJ: Radionuclide bone scanning = of=20 medullary chondrosarcoma. AJR Am J Roentgenol 1982 Dec; 139(6): = 1071-6[Medline].=20
Marco RA, Gitelis S, Brebach GT, Healey JH: Cartilage = tumors:=20 evaluation and treatment. J Am Acad Orthop Surg 2000 Sep-Oct; = 8(5):=20 292-304[Medline].=20
Murphey MD, Flemming DJ, Boyea SR, et al: Enchondroma versus = chondrosarcoma in the appendicular skeleton: differentiating = features.=20 Radiographics 1998 Sep-Oct; 18(5): 1213-37; quiz 1244-5[Medline].=20
Resnik D, Kyriakos M, Greenaway GD: Tumors and tumor-like = lesions of=20 bone: imaging and pathology of specific lesions. In: Diagnosis = of Bone=20 and Joint Disorders. 4th ed. Philadelphia, Pa: WB Saunders Co; = 2002:=20 3897-919.=20
Wang XL, De Beuckeleer LH, De Schepper AM, Van Marck E: = Low-grade=20 chondrosarcoma vs enchondroma: challenges in diagnosis and = management.=20 Eur Radiol 2001; 11(6): 1054-7[Medline].

BIBLIOGRAPHY = Section 12 of=20 12

Author=20 Information Introduction=20 Differentials=20 X-ray<= /A>=20 Cat= =20 Scan MRI = U= ltrasound=20 Nuclear=20 Medicine = Angiography=20 Intervention=20 Pic= tures=20 Bibliography

NOTE: =
Medicine is a = constantly=20 changing science and not all therapies are clearly = established. New=20 research changes drug and treatment therapies daily. The = authors,=20 editors, and publisher of this journal have used their best = efforts=20 to provide information that is up-to-date and accurate and = is=20 generally accepted within medical standards at the time of=20 publication. However, as medical science is constantly = changing and=20 human error is always possible, the authors, editors, = and=20 publisher or any other party involved with the publication = of this=20 article do not warrant the information in this article is = accurate=20 or complete, nor are they responsible for omissions or = errors in the=20 article or for the results of using this information. The = reader=20 should confirm the information in this article from other = sources=20 prior to use. In particular, all drug doses, indications, = and=20 contraindications should be confirmed in the package = insert. FULL = DISCLAIMER=20

Chondro= sarcoma=20 excerpt
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