Original Article

High-Risk Extracranial Chondrosarcoma

Long-Term Results of Surgery and Radiation Therapy

Jayant Sastri Goda, MD1,2; Peter C. Ferguson, MD3,4; Brian O’Sullivan, MD1,2; Charles N. Catton, MD1,2;
Anthony M. Griffin, MSc3; Jay S. Wunder, MD3,4; Robert S. Bell, MD3,4; Rita A. Kandel, MD5,6; and Peter W. Chung, MBChB1,2

BACKGROUND: A study was undertaken to evaluate results of surgery and radiotherapy (RT) for high-risk extracra-
nial chondrosarcomas. METHODS: Between 1986 and 2006, 60 patients underwent surgery and RT for extracranial
high-risk chondrosarcoma. Preoperative RT (median, 50 gray [Gy]) and postoperative RT (median, 60 Gy) were used
in 40% and 60% patients, respectively. Sites included pelvis/lower extremity (48%), chest wall (22%), spine/paraspi-
nal (17%), and head and neck (13%). Overall, median tumor size was 7 cm (range, 1-22 cm), and tumor grade was I, II,
and III in 22%, 64%, and 14% of cases, respectively. RESULTS: Pathologically clear surgical margins (R0) were present
in 50%, microscopic positive margins (R1) in 28%, and gross positive margins (R2) in 13%, half of whom had clinically
detectable residual disease; surgical margin was unknown in 8%. Median follow-up was 75 months (range, 5-230
months). The crude local control rate was 90%. Patients with R0, R1, and R2 resections had local control of 100%,
94%, and 42%, respectively. Of the 8 cases that had R2 resection, 3 experienced uncontrolled progression, but 5
patients had stable disease with long-term follow-up. The 10-year overall survival, progression-free survival, and
cause-specific survival were 86%, 80.5%, and 89.4%, respectively. Younger age and grade III tumors were associated
with worse progression-free survival (P ¼ .03 and .0003, respectively). CONCLUSIONS: Although surgery with com-
plete resection is paramount in management of chondrosarcoma, RT is a useful adjuvant treatment and appears to
offer excellent and durable local control where wide surgical resection is difficult to accomplish. Cancer 2011;117:2513–9.
V
C 2011 American Cancer Society.

KEYWORDS: extracranial, chondrosarcoma, radiation therapy, surgery.

Low- and intermediate-grade disease in patients with chondrosarcoma may have a relatively indolent natural history, with
a low risk of metastasis. In such patients, optimal local control assumes much significance, as it is local disease that is the cause
of morbidity. In those with high-grade conventional chondrosarcoma, and relatively uncommon mesenchymal chondrosar-
coma, a rapid growth rate is often seen, and metastatic disease also is more likely to occur.1-6 The prognosis of chondrosar-
coma patients depends not only on the tumor site, type of surgical resection, and the resulting resection margins, but also on
the grade of the tumor.7-11 Management of chondrosarcoma is challenging, considering that they have been reported to gen-
erally respond poorly to other cancer treatments such as chemotherapy and radiotherapy (RT).8,12,13 Local disease control of
chondrosarcoma is obtained by surgical excision with wide margins, which is considered to be the most effective treatment
for these tumors.7 However, the location or the size of the primary tumor may cause surgical resection with such margins to
be complicated or possible only with excessive morbidity. Moreover, certain adverse features such as grade or positive resec-
tion margins14,15 may lead to a reduction in local control, and thus further therapy may be required in such cases. In this
regard, radiation treatment can be considered as an attractive adjunctive therapy in these so-called high-risk situations. There
is no standard definition for what would be considered high-risk chondrosarcoma, but lesions located at complex sites where
complete resection is an anticipated problem and/or lesions with close or involved surgical margins and/or high-grade lesions
could be considered as such. In this regard, cranial or skull-base chondrosarcoma can be considered a high-risk lesion based
on tumor location and difficulty with radical resection. The use of RT, either in the adjuvant or primary setting, has been

Corresponding author: Peter W. Chung, MBChB, Radiation Medicine Program, Princess Margaret Hospital, 610 University Avenue, Toronto, Ontario, M5G 2M9
Canada; Fax: (416) 946-4442; peter.chung@rmp.uhn.on.ca
1
Radiation Medicine Program, Princess Margaret Hospital, Toronto, Ontario, Canada; 2Department of Radiation Oncology, University of Toronto, Toronto, Ontario,
Canada; 3Musculoskeletal Oncology Unit at Mount Sinai Hospital, Toronto, Ontario, Canada; 4Department of Surgery, University of Toronto, Toronto, Ontario, Can-
ada; 5Department of Pathology, Mount Sinai Hospital, Toronto, Ontario, Canada; 6Department of Laboratory Medicine and Pathobiology, University of Toronto,
Toronto, Ontario, Canada
Presented at the 51st Annual Meeting of the American Society for Therapeutic Radiology and Oncology, Chicago, Illinois, November 1-5, 2009.
DOI: 10.1002/cncr.25806, Received: July 21, 2010; Revised: September 25, 2010; Accepted: October 12, 2010, Published online January 18, 2011 in Wiley Online
Library (wileyonlinelibrary.com)

Cancer June 1, 2011 2513

Original Article

established as standard care.16-18 In addition, the combina- Statistical Analysis

tion of surgery followed by adjuvant RT in extremity soft The endpoints considered were progression-free survival
tissue sarcomas has yielded dividends in optimizing cure (PFS), local control, overall survival (OS), and cause-spe-
rates and quality of life.19,20 Studies have shown this strat- cific survival (CSS). Time was calculated from the date of
egy to have improved local control with limb preservation completing combined modality therapy to the event of
compared with surgery alone.20-24 The same strategy in interest. For OS, death from any cause was considered.
extracranial chondrosarcoma using both surgery and RT For CSS, death as a result of disease or treatment compli-
in those at high risk of local recurrence when treated with cation was considered. For PFS, time to first failure or
surgery alone may yield similar dividends. death from disease was taken into consideration. The
The objective of this retrospective report was to survival plots for PFS, CSS, and OS were estimated using
assess the outcome of patients with high-risk extracranial the Kaplan-Meier method. The differences in survival
chondrosarcoma treated with combined surgery and RT. estimates were tested using log-rank test. Statistical signifi-
cance was considered at P < .05.
MATERIALS AND METHODS
After research ethics board approval, we retrospectively RESULTS
analyzed the charts of 310 consecutive patients in our Patient Characteristics
database with biopsy-proven chondrosarcoma between Of the 310 patients, 60 with extracranial chondrosarco-
1986 and 2006. Patients who had intracranial or skull mas received combined modality therapy between 1986
base disease were excluded from the analysis, except for 1 and 2006; their characteristics are shown in Table 1. The
patient who had primary disease in the maxillary sinus median age at diagnosis was 42.5 years (range, 18-
and nasopharynx with extension to the skull-base who was 84 years). Male preponderance was evident, with a
categorized under primary head and neck chondro- male:female ratio of 2:1. Of the 24 patients who had
sarcoma. Patients presenting with recurrent disease were lower extremity tumors, 11 had true lower extremity
also excluded. Staging studies included routine radio- lesions, whereas 13 had pelvic lesions. Of the 5 patients
graphy, computed tomography, magnetic resonance with upper extremity lesions, 3 had lesions in the scapula,
imaging, and bone scan as appropriate for the primary site whereas 1 each had the primary tumor located in the
and to exclude metastatic disease. High-risk chondrosar- clavicle and radius, respectively. Pure extraskeletal chon-
coma was defined as follows: lesions at complex locations drosarcoma occurred in 11 patients. Patients with primary
where complete resection was an anticipated problem head and neck chondrosarcoma had diverse subsite
and/or resection with close/involved margins and/or high- involvement (nasal septum 2, larynx with hyoid 2, maxilla
grade lesions. Combination of 1 or more of the above fac- 2, and mandible 2).
tors was considered for treating patients with combined
modality therapy. The timing of RT depended on the Treatment
clinical assessment of the treating surgical oncologist in Of 60 patients, 36 (60%) had postoperative RT
conjunction with the radiation oncologist. Patients under- with a median dose of 60 gray (Gy) (range, 39.6-70.6
went preoperative RT in situations where it was antici- Gy). The other 24 (40%) patients received preoperative
pated that there was a high probability of a positive RT; median dose was 50 Gy (range, 46-50 Gy). Clear
resection margin, particularly where more extensive (R0), microscopic positive (R1), and gross positive (R2)
surgery would result in excess morbidity. Otherwise resection margins were obtained in 30, 17, and 8 patients,
postoperative RT was delivered. Although grading is respectively. There were 5 patients for whom resection
important for prognostication, the system of grading margin status was not available.
chondrosarcomas is intrinsically subjective.14 For the
purpose of analyzing outcome, we condensed the grading Outcomes
system into a broader 2-tier system, defining low grade as Survival
grade I and II, and high grade to be grade III. The median follow-up was 75 months (range, 5-230
Patients were followed regularly at 3- to 6-month months). At last follow-up, 47 (78%) of 60 patients were
intervals with clinical examination, chest radiographs, and alive without disease, 5 (8%) of 60 were alive with disease,
appropriate imaging of the primary anatomic site, as 4 (6.7%) of 60 had died of disease, 2 (3%) patients had
needed. died of unrelated causes, and 2 (3%) patients were lost to

2514 Cancer June 1, 2011

 RT in Extracranial Chondrosarcomas/Goda et al

Table 1. Baseline Patient Characteristics and Treatment

Details of Extracranial High-Risk Chondrosarcoma

Characteristics Patients,
N 5 60
No. %

Age at diagnosis, y
Median 42.5
Range 18-84

Sex
Men 40 67%
Women 20 33%

Tumor size (maximum dimension), cm

Median 7
Range 1-22

Anatomical sites
Lower extremity/pelvis 24 40%
Upper extremity 5 8%
Head and neck sites 8 13%
Chest wall 13 20%
Spine/paraspinal 10 17% Figure 1. Overall survival of chondrosarcoma patients treated
Histology with surgery and radiation therapy is shown according to
tumor grade.
Chondrosarcoma NOS 43 72%
Myxoid chondrosarcoma 14 23%
Mesenchymal chondrosarcoma 2 3%
Chondroid chondrosarcoma 1 2%

Grade of tumor
I 13 22%
II 39 65%
III 8 13%

Margin status after surgery

Resection with clear margins (R0) 30 50%
Resection with microscopic margins (R1) 17 28.3%
Resection with gross residual disease (R2) 8 13.3%
Resection status not known 5 8.4%

Neoadjuvant radiation 24 40%

Mean dose, 49.9 Gy
Median dose, 50 Gy
Range, 46-50

Adjuvant radiation 36 60%

Mean dose, 58.8 Gy
Median dose, 60 Gy
Range, 39.6-70.6

NOS indicates not otherwise specified; Gy, gray.

Figure 2. Local control rates for chondrosarcoma patients
treated with surgery and radiation therapy are shown.
follow-up with their final disease status unknown. The 5-
and 10-year PFS, CSS, and OS were 85% and 81%, 95%
and 89%, and 91% and 86%, respectively. On univariate
analysis, patients with low-grade (grade I and II) tumors Local control and patterns of relapse
and older patients (age
45 years) fared better in terms of The crude local control rate was 90% at 10 years
PFS (P ¼ .005 and P ¼ .03, respectively). Tumor size (Fig. 2). As expected, local control was suboptimal (42%)
(8 cm vs >8 cm), primary tumor site, soft tissue involve- in the presence of R2 resection (Fig. 3). Of 8 patients who
ment, and timing of RT were not statistically significant had R2 resection on pathological examination, 3 experi-
factors for PFS. Tumor grade was a statistically significant enced uncontrolled progression (2 pelvis and 1 thoracic
prognostic factor for OS (P ¼ .009) (Fig. 1). spine), whereas 5 patients had stable local disease (3

Cancer June 1, 2011 2515

Original Article

OS,

cGy indicates centigray; RT, radiotherapy; OS, overall survival; M, male; S, surgery; ART, adjuvant radiation therapy; Ds, disease; F, female; NED, no evidence of disease; CT, chemotherapy; NRT, neoadjuvant
mo

227
119

103

193

144
182
70
44

15

10
9

2
Follow-up

(NED)
(NED)

Alive (NED)

(NED)
(NED)
at Last

Dead (Ds)
(Ds)
(Ds)

(Ds)

Alive (Ds)

(Ds)
(Ds)
Treatment Status

Alive
Alive
Alive
Alive
Alive

Alive
Alive
Alive
Alive
SþRTþCT
SþRT

SþRT
None
NA

NA
S
S
S
S

S
Relapses
No. of

—
—
3
3
1
1
3

1
1
Figure 3. Local control rates in chondrosarcoma patients
treated with surgery and radiation therapy are shown accord-

relapse (lung)
ing to resection margins. R0 indicates complete resection; R1,

Relapse,
Time to
positive microscopic resection margin; R2, gross positive

PD (local),
PD (local)

PD (local)
PD (local)
resection margin.

mo

126
12
12
28
31
72

48
8
soft tissue
Lung, brain,
Relapse

Pelvis, lung
cervical spine, 1 lumbar spine, and 1 upper extremity) Dose, Site(s)

spine,
with long-term follow-up (68-140 months).
Pelvis

Pelvis

Pelvis
Spine
Spine
Lung
Lung
Lung

Lung

Lung
The pattern of relapse and outcome of relapsed
patients is summarized in Table 2. A total of 12 (20%)
Table 2. Treatment Details and Outcome of Patients Who Had Progression and/or Relapse

cGy
6000
7000
6600
3960
6600

6000
5600

5000
4800
4600
6600
patients had either relapse or progression of disease.
Surgical Treatment RT

—
Median time to relapse was 29.5 months (range, 8-126
months). Five of 8 patients who had high-grade tumors
had distant metastasis, whereas this occurred in only 3 of

NRTþ S
S þART

S þART
NRTþS

NRTþS
SþART
SþART
SþART
SþART
SþART

SþART

SþART
52 patients with low-grade tumors. All 7 distant relapses
(with 2 of these patients relapsing beyond 5 years) were
pulmonary metastases, of which 1 patient subsequently
Margins

relapsed in spine, soft tissue, and brain. NA

R1
R1
R1
R0
R1

R0
R0
R2

R0

R2
R2
Salvage treatment after relapse

radiation therapy; PD, progressive disease; NA, details not available.

3/mesenchymal
1/conventional
3/conventional

2/conventional
1/conventional

Grade 2/conventional

3/conventional
2/conventional

Of the 9 patients who received salvage surgery, 3

also received further RT. In total, 5 patients who had
Grade 3/myxoid

Grade 3/myxoid

2/myxoid

2/myxoid
Histology

salvage treatment for their relapse had their disease con-

Grade/

trolled at last follow-up. The median OS in the subset

Grade
Grade
Grade
Grade
Grade

Grade
Grade
Grade
Grade

of patients who received salvage treatment after local/

systemic relapse was 86.5 months (range, 2-227 months).
Thoracic spine
Thoracic spine
Larynxþhyoid
Patient Age/ Primary

Chest wall
Paraspinal
Scapula

Scapula

DISCUSSION
Pelvis

Pelvis
Pelvis

Pelvis
Number Sex Site

Tibia

The current report describes the efficacy of combined

modality therapy with surgery and RT in a cohort of
59/M
38/M

37/M
39/M

32/M

40/M
27/M
39/M
18/M

patients with localized extracranial chondrosarcoma

34/F

40/F
44/F

treated over a period of 20 years. Patients had excellent

local control rates that compare well with surgical series
reported in the literature.14,15 Although the role of RT in
10
11
12

chondrosarcoma is controversial, given the risk of local

1
2
3
4
5

6
7
8

2516 Cancer June 1, 2011

 RT in Extracranial Chondrosarcomas/Goda et al

10-Year OS

FU indicates follow-up; DFS, disease-free survival; DSS, disease-specific survival; PFS, progression-free survival; MFS, metastasis-free survival; OS, overall survival; NR, not reported; NCDB, National cancer
recurrence if surgery was used as the sole method of

77.6%/64.3%
management in this patient population, the addition of

5-Year/

77%/67%
59%/46%

77%/66%

72%/67%
82%/80%
RT might improve local control rates. As might be

—/52%

—/86%
expected, in our cohort of patients, local control was a

NR
direct function of resection margins and OS a function of

10-year DFS >1950 70%

the grade of the disease.

DFS/DSS/PFS/MFS

10-year DFS <1950 28%,

Traditionally RT has had a limited role in the radical

10-year DSS 70.6%

5-Year/10-Year

10-year DSS 80%

10-year DFS 67%

10-year PFS 80.5%

management of chondrosarcomas, as they are considered

5-year DSS 87.2%,

10-year MFS 88%

5-year DSS 84%,

5-year DFS 69%,

to be radioresistant tumors.8,12,13 Thus, most series have
reported on control rates with surgery alone, with few
reporting the role of RT as a part of multimodality
management.15,25 The more recent use of proton therapy

NR

NR

NR
or mixed proton/photon therapy for skull-base and spinal

surgery þ RT (21%)
sarcomas, in both radical and adjuvant settings, has
allowed higher doses to be delivered, thereby overcoming

Surgery (59.5%),

Surgery aloneb
Surgery alonea
Treatment
resistance and improving local control rates.16,26 The

Surgery alone
Surgery alone

Surgery alone
Surgery alone

Surgery alone

Surgery þ RT
emerging evidence of efficacy of these radiation techni-
ques in skull-base chondrosarcoma coupled with success
achieved using combined modality approach (RT and
surgery) for soft tissue sarcomas, which have also been
considered relatively radio-resistant tumors,20,23,27 makes Patients
No. of

a case for the use of RT as an adjuvant modality, for

280

344

400
71

67

69
64

24
60
selected cases, in chondrosarcoma.
Reported series of chondrosarcoma patients from Limb, limb girdle, and mobile spine

Seven patients had surgery þ RT (2 patients had preoperative RT, and 5 patients had postoperative RT).
Pelvis, sacrum, and mobile spine
the past 3 decades have focused on outcome in patients
treated with surgery alone and are presented in Table 3.
Entire skeleton except skull

Long bones and limb girdle

OS at 10 years was reported to be in the range of 50% to
80% and disease-free survival at 10 years between 28%
Involvement

and 88% with surgery alone for various primary sites. Of

Table 3. Selected Literature of Treatment Outcome in Chondrosarcoma

Head and neck

Site(s) of

note is that in these studies, the majority of the patients

Extracranial
had pelvic chondrosarcomas, which portend a poorer Scapula
Pelvis

Pelvis

prognosis. This has been attributed to the anatomic

complexity of the pelvis and often close proximity of
the tumor to the surrounding neurovascular and visceral
Mean FU, mo

structures, resulting in suboptimal surgical resection

Median 115
Median 150

Median 140
Median/

margins.
Median 75
Mean 138

Mean 156

The majority of our patient cohort had primary

NR

NR

NR

skeletal tumors with or without soft tissue involvement,

and pure extraskeletal chondrosarcomas were observed in
1948-1974
1909-1975

1970-1992
1911-1990

1985-1995

1967-1999
1975-1996

1989-2004
1986-2006

11 of 60 patients. Of these 11 patients who had pure

Period
Study

extraskeletal soft tissue disease, 7 (64%) patients had myx-

Two patients had Surgery þ RT.

oid histology, whereas the rest had conventional histology.

data base; RT, radiotherapy.

Although they have been included in the analysis along

Koch 2000 (NCDB, USA)

with skeletal chondrosarcomas, it is not clear if extraskele-

tal myxoid chondrosarcomas (EMCs) behave in a similar
Bjornsson 199814
Pritchard 1980

manner to true skeletal myxoid chondrosarcomas (SMCs)

Present study
Bergh 200134

Griffin 200837
Sheth 199636

Pring 200115
Evans 19779

or to soft tissue sarcomas. It is generally debated whether

Study

true myxoid chondrosarcoma of bone exists. The 2002

World Health Organization classification classified this
b
a

Cancer June 1, 2011 2517

Original Article

entity in the ‘‘tumors of uncertain differentiation’’ cate- may, in these situations, be low enough to allow successful
gory, although molecular studies suggest that EMC is a residual tumor cell kill by moderately high doses of RT.
distinct and separate entity from SMC.29 These tumors Grade of tumor is considered to be an important
may have a distinct natural history as compared with determinant of distant failure and ultimately sur-
skeletal conventional and skeletal myxoid chondro- vival.14,15,30 The influence of grade on probability of local
sarcomas. Data on EMC have been limited to case reports control is not clear, and this is confounded by many stud-
and small series. However, a retrospective study from 2 ies documenting only PFS without distinguishing
centers studying the role of surgery and chemotherapy in between local and distant relapse, which is true for our
87 patients with EMC suggests that this histology is asso- study as well. As expected, PFS and OS for patients with
ciated with inferior outcomes (local recurrence rate of high-grade tumors were worse and consistent with other
37%, distant metastasis rate of 26%, and 10-year OS of studies.9,15,34
65%);28 another small comparative study of SMC and In a study by Pritchard et al, larger primary tumor
EMC has also shown poorer prognosis in patients with size was shown to be associated with a statistically signifi-
EMC, with a higher rate of distant metastasis.29 cant difference in the 10-year OS, with a cutoff at
A report on head and neck chondrosarcoma from 10 cm.33 In our current series, we were not able to demon-
the United States derived data from the National Cancer strate a difference based on tumor size with 8 cm used as a
Database, where the majority of patients had received cutoff (10-year PFS of 87% for tumors 8 cm vs 78% for
surgery alone (59.5%), whereas a subset of patients (21%) tumors >8 cm, P ¼ .77). Somewhat similar results were
had received combined surgery and adjuvant RT, and observed by Wirbel et al in his series of pelvic chondrosar-
reported a 10-year PFS of 70.6% and OS of 64.3%.30 comas, where tumor volume was not a significant factor
Although this study did not specifically comment on the affecting the rate of local recurrences. In this study, the
efficacy of adjuvant RT in its cohort of patients, the local recurrence rates were 22% for large-volume tumors
majority of patients who had RT were those with positive (>500 mL) and 20% for small-volume tumors (<500
margins after surgery and those with myxoid or mesen- mL).35
chymal subtypes. As with all retrospective series, patient selection sig-
The definition of surgical adequacy varies in differ- nificantly influenced outcome in our patient cohort. The
ent series, although most are guided by the criteria small number of patients may have influenced the finding
described by Enneking, which considers adequate margins that tumor size was not a predictor for outcome. Consid-
as ‘‘wide excision’’ (removal of tumor together with ering that all these tumors were felt to have a high risk of
margin of normal tissue completely encircling the tumor) local recurrence if treated with surgery alone, combined
or ‘‘radical excision’’ (complete removal of tumor, includ- surgery and RT seems appropriate; this is reflected in the
ing all the involved muscles from their origin to insertion local control rates, which compare favorably to series
and all the involved bone from joint to joint).7,31,32 This where surgery was the sole modality of treatment. We
is considered to be the most important prognostic factor recognize that although these data are compelling, only a
associated with local control of disease.7,11,15,33 The issue randomized trial will truly define the role of (neo)adju-
of need for RT in patients with R0 resections in our series vant RT in this setting.
could be debated; however, many patients were treated
preoperatively because of concerns with regard to the
ability to obtain a wide R0 resection akin to a policy of Conclusions
preoperative RT in soft tissue sarcoma.14,15,34 Currently, we offer RT preoperatively (50 Gy) to patients
Overall, patients who had gross residual disease (R2) with tumors where positive margins are anticipated or in
fared poorly, supporting the need for adequate gross surgi- the postoperative setting (66-70 Gy) to those with unex-
cal resection in this disease; however, the local control rate pected positive margins and where further surgery would
for such patients without RT might have been anticipated result in significant morbidity. There are few series evalu-
to be worse than that achieved in our study. Thus, RT as ating the role of RT in chondrosarcoma in the extracranial
an adjunct to surgery might be most beneficial to that setting. Our data suggest that although complete resection
group of patients where there is microscopic contamina- remains the primary treatment in the management of
tion, that is, in R1 resections or possibly in R0 resection chondrosarcoma, the addition of RT in this disease
with very close resection margins. The burden of disease appears to provide excellent and durable local control in

2518 Cancer June 1, 2011

 RT in Extracranial Chondrosarcomas/Goda et al

those at high risk of local failure when treated with surgery 19. Pisters PW, Harrison LB, Leung DH, Woodruff JM, Casper
alone. ES, Brennan MF. Long-term results of a prospective random-
ized trial of adjuvant brachytherapy in soft tissue sarcoma.
J Clin Oncol. 1996;14:859-868.
CONFLICT OF INTEREST DISCLOSURES 20. Yang JC, Chang AE, Baker AR, et al. Randomized prospec-
tive study of the benefit of adjuvant radiation therapy in the
The authors made no disclosures. treatment of soft tissue sarcomas of the extremity. J Clin
Oncol. 1998;16:197-203.
21. Suit HD, Mankin HJ, Wood WC, Proppe KH. Preoperative,
REFERENCES intraoperative, and postoperative radiation in the treatment of
primary soft tissue sarcoma. Cancer. 1985;55:2659-2667.
1. Dahlin DC, Beabout JW. Dedifferentiation of low-grade
22. Lindberg RD, Martin RG, Romsdahl MM, Barkley HT Jr.
chondrosarcomas. Cancer. 1971;28:461-466.
Conservative surgery and postoperative radiotherapy in 300
2. Johnson S, Tetu B, Ayala AG, Chawla SP. Chondrosarcoma
adults with soft-tissue sarcomas. Cancer. 1981;47:2391-2397.
with additional mesenchymal component (dedifferentiated
23. McNeer GP, Cantin J, Chu F, Nickson JJ. Effectiveness of
chondrosarcoma). I. A clinicopathologic study of 26 cases.
radiation therapy in the management of sarcoma of the soft
Cancer. 1986;58:278-286.
somatic tissues. Cancer. 1968;22:391-397.
3. McFarland GB Jr, McKinley LM, Reed RJ. Dedifferentia-
24. Suit HD, Russell WO. Radiation therapy of soft tissue
tion of low grade chondrosarcomas. Clin Orthop Relat Res.
sarcomas. Cancer. 1975;36:759-764.
1977;(122):157-164.
25. Donati D, El Ghoneimy A, Bertoni F, Di Bella C, Mercuri
4. Tetu B, Ordonez NG, Ayala AG, Mackay B. Chondrosar-
M. Surgical treatment and outcome of conventional pelvic
coma with additional mesenchymal component (dedifferen-
chondrosarcoma. J Bone Joint Surg Br. 2005;87:1527-1530.
tiated chondrosarcoma). II. An immunohistochemical and
26. DeLaney TF, Liebsch NJ, Pedlow FX, et al. Phase II study
electron microscopic study. Cancer. 1986;58:287-298.
of high-dose photon/proton radiotherapy in the manage-
5. Bertoni F, Picci P, Bacchini P, et al. Mesenchymal chondro-
ment of spine sarcomas. Int J Radiat Oncol Biol Phys. 2009;
sarcoma of bone and soft tissues. Cancer. 1983;52:533-541.
74:732-739.
6. Unni KK. Mesenchymal chondrosarcomas. In: Dahlin’s
27. Wunder JS, Nielsen TO, Maki RG, O’Sullivan B, Alman
Bone Tumours. General Aspects and Data on 11087 cases.
BA. Opportunities for improving the therapeutic ratio for
5th ed. Philadelphia, PA: Lippincott-Raven; 1996:109-115.
patients with sarcoma. Lancet Oncol. 2007;8:513-524.
7. Gitelis S, Bertoni F, Picci P, Campanacci M. Chondrosar-
28. Drilon AD, Popat S, Bhuchar G, et al. Extraskeletal myxoid
coma of bone. The experience at the Istituto Ortopedico
chondrosarcoma: a retrospective review from 2 referral cen-
Rizzoli. J Bone Joint Surg Am. 1981;63:1248-1257.
ters emphasizing long-term outcomes with surgery and chem-
8. Sanerkin NG, Gallagher P. A review of the behaviour of chon-
otherapy. Cancer. 2008;113:3364-3371.
drosarcoma of bone. J Bone Joint Surg Br. 1979;61-B:395-400.
29. Antonescu CR, Argani P, Erlandson RA, Healey JH, Lada-
9. Evans HL, Ayala AG, Romsdahl MM. Prognostic factors in
nyi M, Huvos AG. Skeletal and extraskeletal myxoid chon-
chondrosarcoma of bone: a clinicopathologic analysis with
drosarcoma: a comparative clinicopathologic, ultrastructural,
emphasis on histologic grading. Cancer. 1977;40:818-831.
and molecular study. Cancer. 1998;83:1504-1521.
10. Lee FY, Mankin HJ, Fondren G, et al. Chondrosarcoma of bone:
30. Koch BB, Karnell LH, Hoffman HT, et al. National cancer
an assessment of outcome. J Bone Joint Surg Am. 1999;81:326-338.
database report on chondrosarcoma of the head and neck.
11. Marcove RC, Mike V, Hutter RV, et al. Chondrosarcoma
Head Neck. 2000;22:408-425.
of the pelvis and upper end of the femur. An analysis of fac-
31. Enneking WF, Spanier SS, Goodman MA. A system for the
tors influencing survival time in 1 hundred and thirteen
surgical staging of musculoskeletal sarcoma. Clin Orthop Relat
cases. J Bone Joint Surg Am. 1972;54:561-572.
Res. 1980;153:106-120.
12. Dahlin DC, Henderson ED. Chondrosarcoma, a surgical
32. Fiorenza F, Abudu A, Grimer RJ, et al. Risk factors for survival
and pathological problem: review of 212 cases. J Bone Joint
and local control in chondrosarcoma of bone. J Bone Joint Surg
Surg Am. 1956;38-A:1025-1038.
Br. 2002;84:93-99.
13. Ozaki T, Hillmann A, Lindner N, Blasius S, Winkelmann
33. Pritchard DJ, Lunke RJ, Taylor WF, Dahlin DC, Medley
W. Chondrosarcoma of the pelvis. Clin Orthop Relat Res.
BE. Chondrosarcoma: a clinicopathologic and statistical analy-
1997;(337):226-239.
sis. Cancer. 1980;45:149-157.
14. Bjornsson J, McLeod RA, Unni KK, Ilstrup DM, Pritchard
34. Bergh P, Gunterberg B, Meis-Kindblom JM, Kindblom LG.
DJ. Primary chondrosarcoma of long bones and limb girdles.
Prognostic factors and outcome of pelvic, sacral, and spinal
Cancer. 1998;83:2105-2119.
chondrosarcomas: a center-based study of 69 cases. Cancer.
15. Pring ME, Weber KL, Unni KK, Sim FH. Chondrosarcoma
2001;91:1201-1212.
of the pelvis. A review of sixty-four cases. J Bone Joint Surg
35. Wirbel RJ, Schulte M, Maier B, Koschnik M, Mutschler
Am. 2001;83-A:1630-1642.
WE. Chondrosarcoma of the pelvis: oncologic and func-
16. Amichetti M, Amelio D, Cianchetti M, Enrici RM, Minniti G.
tional outcome. Sarcoma. 2000;4:161-168.
A systematic review of proton therapy in the treatment of chon-
36. Sheth DS, Yasko AW, Johnson ME, Ayala AG, Murray JA,
drosarcoma of the skull base. Neurosurg Rev. 2010;33:155-165.
17. Wanebo JE, Bristol RE, Porter RR, Coons SW, Spetzler Romsdahl MM. Chondrosarcoma of the pelvis. Prognostic
RF. Management of cranial base chondrosarcomas. Neuro- factors for 67 patients treated with definitive surgery. Cancer.
surgery. 2006;58:249-255. 1996;78:745-750.
18. Bloch OG, Jian BJ, Yang I, et al. A systematic review of in- 37. Griffin AM, Shaheen M, Bell RS, Wunder JS, Ferguson
tracranial chondrosarcoma and survival. J Clin Neurosci. PC. Oncologic and functional outcome of scapular chon-
2009;16:1547-1551. drosarcoma. Ann Surg Oncol. 2008;15:2250-2256.

Cancer June 1, 2011 2519