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Timothy A. Damron, MD*; William G. Ward, MD†; and Andrew Stewart, MA‡
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We summarize descriptive epidemiologic and survival data ported 854 patients with osteosarcomas, 325 patients with
from the National Cancer Data Base of the American College chondrosarcomas, and 528 patients with Ewing’s sarcoma.
of Surgeons for 26,437 cases of osteosarcoma (n = 11,961), A series of patients from Memorial Sloan-Kettering Can-
chondrosarcoma (n = 9606), and Ewing’s sarcoma (n = 4870) cer Center treated between 1921 and 1979 accrued 1,095
from 1985 to 2003. Survival data are reported on cases with
a minimum 5-year followup from 1985 to 1998 (8,104 osteo-
patients with osteogenic sarcoma, 493 with chondrosar-
sarcomas, 6,476 chondrosarcomas, and 3,225 Ewing’s sarco- coma, and 334 with Ewing’s sarcoma.2 A series including
mas). The relative 5-year survival rate was 53.9% for osteo- 1274 patients with osteosarcomas, 634 with chondrosar-
sarcoma, 75.2% for chondrosarcoma, and 50.6% for Ew- comas, and 402 with Ewing’s sarcomas from the Mayo
ing’s sarcoma. Survival rates did not change notably over the Clinic have also been reported.7 Campanacci’s1 text de-
collection period. Within osteosarcomas, the relative 5-year scribed 1,682 high-grade central osteosarcomas, 500 cen-
survival rates were 52.6% for high grade, 85.9% for paros- tral chondrosarcomas, and 788 Ewing’s sarcomas evalu-
teal, and 17.8% for Paget’s subtypes. For osteosarcoma pa- ated at the Istituto Rizzoli in Bologna.
tients, the relative 5-year survival rate was 60% for those
The care of musculoskeletal oncology patients contin-
younger than 30 years, 50% for those aged 30 to 49 years,
and 30% for those aged 50 years or older. Within chondro- ues to evolve over time, and with the ever-increasing num-
sarcomas, the relative 5-year survival rate was 76% for con- ber of facilities staffed with musculoskeletal oncology
ventional, 71% for myxoid, 87% for juxtacortical, and 52% trained surgeons, bone tumor treatment has become de-
for mesenchymal. While the National Cancer Data Base has centralized away from the major centers to some degree.
limitations, the survival data and demographics for bone While the major centers still see the largest series of pa-
sarcomas are unprecedented in numbers and duration. Our tients, reports from those individual centers are limited by
report supports continued efforts to refine data collection inherent geographic and established referral patterns. Fur-
and stimulate further data analysis. ther advances in the understanding of bone sarcoma epi-
Level of Evidence: Level II, prognostic study. See the Guide- demiology will require evaluation of tumor registry data
lines for Authors for a complete description of levels of evi- from multiple centers. The two largest tumor registries in
dence. the United States are the National Cancer Data Base
(NCDB) of the American College of Surgeons and the
National Cancer Institute’s Surveillance, Epidemiology
Much of what we know and teach about the epidemiology and End Results program.
of musculoskeletal tumors in general and bone sarcomas in The NCDB currently collects data from over 1,400
particular derives from retrospective series collected at Commission on Cancer approved cancer program regis-
large teaching centers. Many series have been reported in tries in the United States and regularly reports detailed
the peer-reviewed literature, but the largest series have survival data on the 11 most frequently diagnosed solid
been included in published textbooks. Mirra’s5 text re- tumors. However, the relatively low prevalence of bone
sarcomas leaves these tumors largely unreported. In lieu of
From the *Musculoskeletal Sciences Research Laboratories, Department of establishing a tumor-specific database, the members of the
Orthopedics, Syracuse, NY; †Wake Forest University Health Services, Win- Musculoskeletal Tumor Society have recently established
ston-Salem, NC; and the ‡Commission on Cancer, National Cancer Data
Base, American College of Surgeons, Chicago, IL. a collaborative relationship with staff from the American
This research was reviewed and approved for IRB exempt status. College of Surgeons for the specific purpose of mining the
Correspondence to: Timothy A. Damron, MD, Department of Orthopedic available data on musculoskeletal tumors.
Surgery, Suite 130, 550 Harrison Street, Syracuse, NY 13202. Phone: 315-
464-4472; Fax: 315-464-6446; E-mail: damront@upstate.edu. We summarize the currently available data for the three
DOI: 10.1097/BLO.0b013e318059b8c9 most common bone sarcomas reported to the NCDB. We
40
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Number 459
June 2007 National Cancer Data Base Sarcoma Data 41
presumed these data would confirm previously established surgery of the primary site and use of chemotherapy and/or
demographic characteristics and published survival rates radiation therapy.
for patients with these three bone sarcomas. Descriptive epidemiologic and survival data for 26,437 cases
of osteosarcoma (n ⳱ 11,961), chondrosarcoma (n ⳱ 9606), and
Ewing’s sarcoma (n ⳱ 4870) were identified for the 19-year
MATERIALS AND METHODS
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Clinical Orthopaedics
42 Damron et al and Related Research
Commission on Cancer’s Facility Oncology Registry Data Stan- All descriptive analysis and survival calculations were per-
dards manual. Limited explanation of the surgery treatment formed using SPSS v. 12 (SPSS Inc, Chicago, IL), a standard
codes is provided. The general categories include local tumor statistical package (p < 0.05).
destruction, local excision, partial resection, radical excision or
resection, amputation of the limb, and major amputation.
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Number 459
June 2007 National Cancer Data Base Sarcoma Data 43
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Clinical Orthopaedics
44 Damron et al and Related Research
615/13,632). However, fully 19.2% (2613/13,632) of and 17.8% for Paget’s subtypes (Fig 5). For osteosarcoma
cases did not distinguish between even these two broad patients, relative 5-year survival rates were 60% for pa-
categories (Table 3). tients older than 30 years, 50% for patients aged between
Survival data are reported on cases with a minimum 30 and 49 years, and 30% for patients 50 years and older.
5-year followup from 1985 to 1998 (8104 osteosarcomas, Within chondrosarcomas, observed and relative 5-year
6476 chondrosarcomas, and 3225 Ewing’s sarcomas). The survival rates were 68.4% and 75.9%, respectively, for
observed and corresponding relative 5-year survival rates conventional, 64.1% and 71.0% for myxoid, 80.3% and
were 51.2% and 53.9%, respectively, for osteosarcoma; 86.5% for juxtacortical, and 49.2% and 51.7% for mesen-
67.8% and 75.2% for chondrosarcoma, and 50.2% and chymal subtypes.
50.6% for Ewing’s sarcoma (Fig 4). Survival rates did not
change notably over the three periods analyzed (1985– DISCUSSION
1988, 1989–1993, 1994–1998). Over these three time pe- Our report represents the single largest series of cases for
riods, the relative 5-year survival rates were 52.1%, each of the three most common bone sarcomas, with here-
55.2%, and 53.5%, respectively, for osteosarcoma; 74.7%, tofore unreported survival curves according to histologic
76.5%, and 74% for chondrosarcoma; and 50.7%, 49.1%, subtype, year of diagnosis, and multiple demographic vari-
and 52.2% for Ewing’s sarcoma. ables. While the database has limitations in terms of its
Among osteosarcomas, the observed and relative 5-year reporting of local recurrences, the survival data and de-
survival rates were 50.1% and 52.6%, respectively, for mographics are unprecedented in numbers and duration.
conventional high-grade intramedullary, 83.8% and 85.9% Some of these limitations are those common to most
for parosteal, 48.2% and 49.5% for small cell, and 13.0% large registries. First, data has been collected over a long
period of time. By necessity, the documentation for and
the coding of the data have evolved over time and may
TABLE 2. Stage of Reported Chondrosarcomas, potentially have introduced some variation into data docu-
Ewing’s Sarcoma, and Osteosarcoma Reported to mentation and coding. However, the standardization inher-
the NCDB: 1985–2003 ent to such a large and well-managed database is also the
Ewing’s strength of such a study. No single institution or even
Stage Chondrosarcomas Sarcoma Osteosarcomas consortium of major institutions could rival the numbers of
I 53.6 4.1 11.3
patients generated by this database. Second, the collection
II 11.3 15.1 22.8 of the data is biased by the mechanism triggering collec-
III 1.1 1.6 1.7 tion (the generation of a pathology report), the type of
IV 5.5 13.5 10.1 institutions reporting data (American College of Surgeons
NA 1.5 44.3 27.0 accredited cancer centers), and because data collection for
Unknown 27.0 21.4 27.0
Total cases 9606 4870 11,961
sarcomas is not limited to members of the Musculoskeletal
Tumor Society or even to musculoskeletal oncologists.
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Number 459
June 2007 National Cancer Data Base Sarcoma Data 45
TABLE 3. First Course of Therapy for Chondrosarcomas, Ewing’s Sarcoma, and Osteosarcoma
Reported to the NCDB: 1985–2003
Therapy Chondrosarcomas Ewing’s Sarcoma Osteosarcomas
Surgery only 68.6 3.3 15.8
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Other concerns deserve attention as they are amenable females) from bone and joint sarcomas during 2006.3 In
to improvement. Anatomic site coding should be more 2006, the American Cancer Society provided its annual
specific than is currently the case. Specific bones should report on epidemiologic data derived from the National
be included as subcodes within the broader categories cur- Cancer Institute’s Surveillance, Epidemiology and End
rently utilized. There is also no distinction between epiph- Results and survival data from the National Center for
yseal, metaphyseal, and diaphyseal portions of the long Health Statistics for all cancers. In this report, tumors of
bones. In addition, staging data have not been available for bone and joints are not among the top 10 cancers in esti-
most of the pediatric cases as the American Joint Com- mated new cancers, and limited data are reported. Only for
mittee on Cancer has emphasized staging schemes for children under age 15 are relative survival rates for bone
adults (Table 5). This void has resulted in a large percent- and joint sarcomas reported based on data from 1974 to
age of cases reported with stage not applicable for osteo- 2001. Based on these data for pediatric patients, 5-year
sarcomas and Ewing’s sarcomas. All Ewing’s sarcomas relative survival rates for all bone and joint sarcomas have
should have been coded as high-grade, but yet 2.8% were improved (p < 0.05) from 1974 to 1976 (55%) through
still incorrectly coded as low-grade. From the standpoint 1995 to 2001 (71%). In fact, these reported 5-year survival
of surgery coding, more meaningful definitions are needed rates improved most notably between the 1983 to 1985
that better correspond to clinically utilized procedures. In collection period (53–57%) and the 1986 to 1988 and 1989
addition, internal hemipelvectomy should be recoded as a to 1991 collection periods (62–63%) and then again during
limb-sparing procedure rather than as a “major amputa- the latest collection period (71%).
tion.” Other end points, such as local control and outcomes While most of our findings reflect long-held beliefs,
of limb-sparing surgical reconstructions, would add con- some of our findings question common clinical under-
siderably to the usefulness of the registry. standing of bone sarcomas. For osteosarcoma, a bimodal
Reporting of national database statistics for bone sar- age distribution is often quoted, with an early and largest
comas is sparse. The American Cancer Society reports peak (approximately 60% of all osteosarcoma patients)
there will be an estimated 2760 new cases (1500 in males, between age 10 and 19 and a later peak (approximately
1260 in females) and 1260 deaths (730 in males, 530 in 10%) after age 60.2 In our data, the age distribution for
TABLE 4. First Course Surgery for Chondrosarcomas, Ewing’s Sarcoma, and Osteosarcoma Reported to
the NCDB: 1985–2003
Surgical Procedure Chondrosarcomas Ewing’s Sarcoma Osteosarcomas
Local tumor destruction 26.1 19.1 11.9
Local excision 1.2 1.0 0.4
Partial resection 0.8 0.7 0.5
Radical excision or resection with limb salvage 29.1 35.3 38.9
Amputation of limb 10.0 10.5 19.6
Major amputation 4.9 2.6 4.2
Surgery, NOS 27.8 30.7 24.6
Total cases 7959 2312 8454
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Clinical Orthopaedics
46 Damron et al and Related Research
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Fig 4. Five-year observed survival rates with minimum 5-year Fig 5. Five-year observed survival rates for histologic sub-
survival data for osteosarcoma, chondrosarcoma, and Ewing’s types of osteosarcoma; cases diagnosed between 1985 and
sarcoma; cases diagnosed between 1985 and 1998. 1998.
osteosarcoma of all types showed an early peak from age ally used in the pediatric population for whom better out-
10 to 19, but it represented only 38.5% of patients and comes in the range of 60% to 70% have been reported.
gradually diminished in older age categories. In fact, the Clearly, age made a difference, as shown by the difference
major difference in age in our population of osteosarcoma in survival curves for patients with osteosarcomas of dif-
patients appeared to be in the young adult category, as our
series included only 30.6% of all osteosarcomas in the 40
and older age group, nearly identical to that described by TABLE 5. American Joint Committee on
others.6 Fully 25.8% of the osteosarcomas occurred in the Cancer (AJCC)/UICC Staging System for
20 to 39 age group, where the normally lower occurrence, Bone Sarcomas
particularly in the 30 to 39 age group, typically creates the
Tumor Grade Description
“valley” in the bimodal distribution. Even for conventional
osteosarcoma, for which it has been said 85% of cases T1 < or = 8 cm
present before the age of 30 and 60% in the second decade, T2 > 8 cm
T3 Multiple/discontinuous tumors
in our series, the group younger than 30 represented only N1 Regional lymph node involvement
62% and those in the second decade only 40%.4,5 G1 Well-differentiated
The second commonly held view of osteosarcoma that G2 Moderately differentiated
appears untrue is the overall prognosis is clearly better G3 Poorly differentiated
than Ewing’s sarcoma.6,7 In our series, the relative 5-year G4 Undifferentiated
Stage IA G1 or 2 T1 N0 M0
survival rate for all osteosarcomas was only 53.9%, well Stage IB G1 or 2 T2 N0 M0
within the reported error limits for Ewing’s sarcoma at Stage IIA G 3 or 4 T1 N0 M0
50.6%. This is likely due to a number of factors. First, the Stage IIB G 3 or 4 T2 N0 M0
aforementioned difference in age distribution even for Stage III Any G T3 N0 M0
conventional osteosarcoma, with a shift into early adult- Stage IVA Any G Any T N0 M (lung only)
Stage IVB Any G Any T Any N Any M
hood, introduces an age group that may be less tolerant of
the high-intensity, multiagent chemotherapy regimen usu- M = metastasis
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Number 459
June 2007 National Cancer Data Base Sarcoma Data 47
ferent age categories. The relative 5-year survival rate in tions that record sarcomas are represented by members of
our series was 60% for osteosarcoma patients younger a single organization, the Musculoskeletal Tumor Society,
than 30 years. Second, since our series represents all- coordinated efforts between the American College of Sur-
comers, including those in advanced stages and those with geons and the Musculoskeletal Tumor Society may prove
the worst histologic diagnoses (eg, Paget’s osteosarcoma), fruitful in improving both the data collection process and
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the poorer prognosis of the latter lowers the overall group the quality of the data collected. Prior to the current col-
survival. Third, treatment was not standardized, and only laborative effort, the only formal coordination between the
wCX1AWnYQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC4/OAVpDDa8KKGKV0Ymy+78= on 04/20/2023
46% of osteosarcoma patients received combined surgery two societies was by way of a designated member from the
and chemotherapy as part of their initial treatment. Further Musculoskeletal Tumor Society being a recognized repre-
detailed analysis of specific age, stage, and histologic sub- sentative to the American College of Surgeons. With the
type categories is clearly needed to explore osteosarcoma current effort and continued similar efforts in this vein, the
survival in greater depth. Musculoskeletal Tumor Society hopes to continue to ex-
For chondrosarcoma, the anatomic distribution may pand this collaboration and to improve the data collection
have differed somewhat from conventional description. process.
According to some, the pelvis is the most common site of Acknowledgments
involvement for chondrosarcoma.2,5 In our series, the long
The authors wish to thank each of the current and former mem-
bones of the lower extremity were nearly twice as com-
bers of the Musculoskeletal Tumor Society who have partici-
mon. However, our data set did not allow differentiation of pated in data collection for the National Cancer Database at
individual long bones of the upper or lower extremity. Cancer Centers accredited by the American College of Surgeons.
Sites usually thought rare for chondrosarcomas were seen In addition, the authors thank Jerri Linn Phillips, MA, CTR,
to host these tumors on occasion, including the spine Manager, Information Technology and Data Standards Section,
(5.1%), small bones of the lower extremity (2.2%), and National Cancer Data Base, Division of Research and Optimal
small bones of the upper extremity (2.1%). Patient Care, American College of Surgeons.
For Ewing’s sarcoma, as earlier described for osteosar-
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