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CLINICAL ORTHOPAEDICS AND RELATED RESEARCH

Number 459, pp. 40–47


© 2007 Lippincott Williams & Wilkins

Osteosarcoma, Chondrosarcoma, and Ewing’s Sarcoma


National Cancer Data Base Report
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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

Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
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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period. The total number of these three types of sarcomas re-


ported per year ranged from a low of 697 cases (2.7% of the
The National Cancer Database (NCDB) was established in 1989
as a project of the Commission on Cancer (CoC) of the Ameri- 19-year total) in 1985 (first year reported) to a maximum of 1796
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can College of Surgeons (ACoS), and has received operational cases (6.8% of the 19-year total) in 1997 (Fig 1). Within each
support funding from the American Cancer Society (ACS) since subtype, the total number of cases reported per year followed the
its inception. The NCDB serves as a comprehensive clinical same general trend, with a range of 318 in 1985 (2.7%) to 806
surveillance resource for cancer care in the United States. Its (6.7%) in 1997 for osteosarcoma, 237 in 1985 (2.5%) to 650
purpose is to improve the quality of cancer care by providing in 1998 (6.8%) for chondrosarcoma, and 142 in 1985 (2.9%) to
physicians, cancer registrars, and others with the means to com- 354 in 1998 (7.35) for Ewing’s sarcoma (Fig 1). For the most
pare their management of cancer patients with the way in which recent year reported (2003), there were 723 osteosarcomas
similar patients are managed in other cancer care centers around (6.0%), 552 chondrosarcomas (5.7%), and 240 Ewing’s sarco-
the country. Until 2001 data submission by hospital based reg- mas (4.9%).
istries was voluntary. On average approximately 1650 registries Anatomic site coding is based on ICD-O-2/3 coding schema
submitted data to the NCDB. Since 2001, participation in the not specific to individual bones. Hence, all long bones of the
NCDB has been a standing requirement for continued participa- lower extremity, including the femur, tibia, and fibula, are
tion in the CoC-Approvals program and been exclusively limited lumped together. Similarly, all long bones of the upper extrem-
to these 1400 cancer programs. Hospital registries excluded ity, including the humerus, radius, and ulna, are grouped to-
from participation starting in 2001 were typically, but not ex- gether. There is no distinction for epiphyseal, metaphyseal, and
clusively, smaller facilities reporting fewer than 300 cancer cases diaphyseal locations. No additional detailed description is avail-
annually. able for short bones of the lower extremity, short bones of the
During an 18 year period from 1985 to 2003, 35,265 upper extremity, pelvic bones, rib/sternum/clavicle (one group),
bone/joint tumors were reported from 1656 hospitals (mean 21 vertebral column (not broken down into cervical, thoracic, and
cases per facility) to the NCDB. Of those, 26,437 cases reported lumbar), or bones of the skull and face. The mandible is listed as
from 1526 hospitals (mean of 17 cases per facility) were ex- a separate site. The sacrum is included in the pelvis. Overlapping
tracted for detailed review based upon histology criteria identi- lesions are grouped separately.
fying osteosarcoma, chondrosarcoma, or Ewing’s sarcoma. We Staging data utilized the American Joint Committee on Can-
excluded 8828 cases of other entities. cer staging system for bone sarcomas. However, this staging
Data available for analysis for each of the three types of system is not universally accepted for pediatric patients. Thus,
sarcomas included date of initial diagnosis by year, patient sex, many cases with osteosarcomas and Ewing’s sarcomas did not
age, and insurance status at diagnosis; anatomic site of primary have reported staging information and for practical purposes
tumor and histologic subtypes (for osteosarcoma and chondro- these two categories could be grouped as essentially missing
sarcoma) by ICD-O-2/3 classification; NCDB analytic stage data. In addition to clinical and pathologic tumor stage group (I,
based on American Joint Committee on Cancer pathologic stage II, III, IV), the separate staging elements (G, T, N, M) are also
when reported and clinical stage otherwise; tumor grade (well reported.
differentiated, moderately differentiated, poorly differentiated, Specific surgery codes were assigned by coders based upon
undifferentiated); and first-course therapy, including reported available operative reports utilizing guidelines established by the

Fig 1. The annual number of osteosarcomas, chon-


drosarcomas, and Ewing’s sarcomas cases by year
of initial diagnosis (1985–2003).

Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
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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Beginning in 2001, cancer registries began using the third RESULTS


edition of the International Classifications of Diseases (ICD-O-
3) coding manual as a guide to tumor morphology. Accordingly,
The annual reports to the NCDB include 11,961 cases of
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clinically recognized histologic entities not previously acknowl-


edged as separate codes in ICD-O-2 were added that affected osteosarcoma, 9606 of chondrosarcoma, and 4870 of Ew-
only osteosarcoma coding. Before 2001, the specific osteosar- ing’s sarcoma between 1985 and 2003 (Fig 1). Osteosar-
coma histology subtype codes included high-grade conventional, coma data were available for 10,922 high-grade conven-
Paget’s, small cell, and parosteal. The newly added diagnoses in tional, 647 parosteal, 247 Paget’s, 93 small cell, 33 peri-
2001 included intraosseous (9187), intracortical (9192), perios- osteal, 11 low-grade intraosseous, and eight high-grade
teal (9193), and high-grade surface (9194) osteosarcomas. Of surface lesions. Since the osteosarcoma codes changed in
these, all except intracortical have been reported to the NCDB. 2001, the osteosarcoma histologic data for 2001 to 2003
Before 2001, these tumors had been coded as osteosarcoma not were analyzed separately to provide a relative frequency of
otherwise specified (NOS). Hence, in this review, the numbers the various subtypes in the latest classification schema.
for intraosseous, periosteal, and high-grade surface osteosarco- For this more recent time period, there were 2030 high-
mas reflect a limited period of time (2001–2003) when compared grade conventional (90.7%), 92 parosteal (4.1%), 39 Pag-
to the remainder of the osteosarcoma diagnoses. For chon- et’s (1.7%), 33 periosteal (1.5%), 24 small cell (1.1%), 11
drosarcomas, the histologic subtypes (NOS, juxtacortical,
intraosseous (0.5%), and eight high-grade surface (0.4%)
mesenchymal, and myxoid) did not change over the period of
osteosarcomas. Data were available for 8419 conventional
collection. Ewing’s sarcoma in our series did not include primi-
tive neuroectodermal tumors, as they are coded separately. Our
“NOS” (89.2%), 725 myxoid (7.7%), 210 mesenchymal
series also likely includes some soft-tissue osteosarcomas, Ew- (2.2%), and 88 juxtacortical (0.9%) chondrosarcomas.
ing’s sarcomas, and mesenchymal chondrosarcomas, as no dis- Sex and age data were available for all cases. Each of
tinction has been made between these bone and soft-tissue sar- the sarcomas was more common in males than females,
comas. with the predilection being strongest in Ewing’s sarcoma
Survival rates were calculated for each of the three sarcomas (nearly 6:4 male:female), although both parosteal and peri-
overall, for the histologic subtypes of both osteosarcoma and osteal osteosarcomas showed a reciprocal nearly 6:4 fe-
chondrosarcoma, and for three periods of data collection (1985– male:male ratio (Fig 2).
1988, 1989–1993, 1994–1998). Only data through 1998 were The age distributions were as traditionally taught for
employed to ensure 5-year followup on confirmed data and thus each tumor type, but there were exceptions (Fig 3). Almost
do not include recently recognized osteosarcoma designations 50% of reported Ewing’s sarcoma and almost 40% of the
introduced with publication of the ICD-O-3. Survival analyses osteosarcoma cases were reported in patients aged 10 to 19
were limited to categories containing at least 50 cases to assure years. Chondrosarcoma was reported among elderly pa-
sufficient sample size. Survival was calculated both as observed
tients, with over 70% of reported cases in patients aged 40
and relative (adjusted to age-matched patient population) rates.
Differences in five-year survival calculated by the Kaplan-Meier
years and older. Only 6% of Ewing’s sarcoma cases were
method were then compared using the log rank (Mantel Cox) in patients aged 40 years or older, while 30% of osteosar-
test. coma patients were in this age range.
Data reported to the NCDB are retrospective in nature. No The anatomic site was specified for 98.1% of all cases.
patient or physician identifiers were collected as part of the The largest proportion of each of the three general histo-
study. Case identification information (facility identification logic types of bone sarcomas occurred in the long bones of
number and local registry accession number) was collected for the lower extremity, although this site showed a much
administrative purposes only. Analyses were reported only at the greater proportion for osteosarcomas (57.6%) than for ei-
aggregate level to assist hospital cancer programs with quality ther chondrosarcomas (31.3%) or Ewing’s sarcomas
assurance, rather than used to make decisions about individuals (27.9%) (Table 1). For osteosarcoma, the next most com-
and their care. The American College of Surgeons has executed
mon general sites were long bones of the upper extremity
a Business Associate Agreement that includes a data-use agree-
ment, with each of its CoC approved hospitals. Results reported
(11.7%) and pelvic bones (10.1%). For chondrosarcoma,
in this study were in compliance with the privacy requirements the pelvic bones were the next most common site (18%),
of the Health Insurance Portability and Accountability Act of followed by the long bones of the upper extremity (16.3%)
1996 as reported in the Standards for Privacy of Individually and the rib/sternum/clavicle (13.6%). For Ewing’s sar-
Identifiable Health Information; Final Rule (45 CFR Parts 160 coma, we found nearly as high a percentage of pelvic bone
and 164). cases (25%) as for the number one site. Next most com-

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Number 459
June 2007 National Cancer Data Base Sarcoma Data 43

sarcoma and osteosarcoma Stage II (Table 2). Stage IV


disease was present at diagnosis in 5.5% of chondrosar-
comas, 13.5% of Ewing’s sarcomas, and 10.1% of osteo-
sarcomas. The unavailability of staging data for pediatric
patients due to the lack of a consensus staging system is
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underscored by the high percentage of “NA” coding for


both osteosarcoma (27%, 3230/11,961 cases) and Ewing’s
sarcoma (44.3%, 2158/4870 cases), both tumors with a
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higher proportion of young patients when compared to


chondrosarcoma (1.5%, 144/9606 cases), predominately a
disease of adulthood. Although grade was missing in over
36% of cases, 84% (6723/8022) of chondrosarcomas were
low grade and 80% (5457/6851) of osteosarcomas were
high grade. For Ewing’s sarcoma, which is generally con-
sidered a high-grade sarcoma by definition, 1956 of the
total 4870 cases were still assigned grades, and 2.8% (55
cases) of those graded Ewing’s cases were inaccurately
assigned low grades.
Although the first course of therapy varied consider-
ably within each type of sarcoma, the most common
type of treatment for chondrosarcoma was surgery only
(69%) (Table 3). For Ewing’s sarcoma, the most fre-
quent treatments were nearly equally divided between
surgery/chemotherapy (24%) and radiation/chemotherapy
Fig 2A–B. (A) The percent distribution of osteosarcoma,
(23%), while 18% received chemotherapy alone and 15%
chondrosarcoma, and Ewing’s sarcoma by sex. (B) The per- received surgery/radiation/chemotherapy. For osteosar-
cent distribution of osteosarcoma histologic subtypes. coma, combined surgery and chemotherapy were em-
ployed initially only for 46% of patients.
The first course of therapy involved surgery in 18,725
mon for Ewing’s sarcoma was the upper-extremity long of the total 26,437 cases (71%). For osteosarcoma, 71%
bones (13.1%) followed by the rib/sternum/clavicle (8454/11,961 cases) had initial therapy that included sur-
(10.7%) and vertebral column (9.3%). All other sites of gery. For chondrosarcoma, 83% (7959/9606 cases) had
sarcoma occurrence represented less than 10% of each surgery as part of initial therapy. For Ewing’s sarcoma,
type of sarcoma’s cases. only 47% (2312/4870 cases) involved initial surgery. The
For those 53.1% with available American Joint Com- most frequently employed surgical intervention for each of
mission on Cancer staging data, the most prevalent stage the diseases was a limb-sparing radical excision/resection
of chondrosarcomas was Stage I, and for both Ewing’s (Table 4). Excluding those cases for which the specific
type of surgery was not specified (26.7%, 5001/18,725
cases), nonablative surgery was performed for 69%
(4371/6376) of osteosarcomas, 79% (4560/5745) of chon-
drosarcomas, and 81% (1299/1603) of Ewing’s sarcomas.
Radiation was employed for 10% (1198/11,961) of os-
teosarcoma, 14% (1339/9606) of chondrosarcoma, and
46% (2238/4870) of Ewing’s sarcoma cases. External
beam radiotherapy was the most commonly employed
technique, used in 91.6% of irradiated osteosarcomas,
94.5% of irradiated chondrosarcomas, and 93.3% of irra-
diated Ewing’s sarcomas (Table 3).
Chemotherapy was employed for 71% (8547/11,961) of
osteosarcoma, 8% (771/9606) of chondrosarcoma, and
89% (4314/4870) of Ewing’s sarcoma cases. When speci-
Fig 3. Percent distribution of osteosarcoma, chondrosar- fied, chemotherapy was usually multiagent (76.3%,
coma, and Ewing’s sarcoma by patient age. 10,404/13,632) as opposed to single agent (4.5%,

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Clinical Orthopaedics
44 Damron et al and Related Research

TABLE 1. Anatomic Site Distribution of Chondrosarcomas, Ewing’s Sarcoma, and Osteosarcoma


Reported to the NCDB: 1985–2003
ICD-O-2/3 Primary Site Chondrosarcomas Ewing’s Sarcoma Osteosarcomas
Long Bones – Upper limb 16.3 13.1 11.7
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Short Bones – Upper limb 2.1 1.4 0.7


Long Bones – Lower limb 31.3 27.9 57.6
Short Bones – Lower limb 2.2 4.0 1.9
Overlapping lesions 0.2 0.1 0.1
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Bone of limb, NOS 0.9 0.6 0.6


Bones – Skull and face 7.3 2.7 5.9
Mandible 0.9 1.1 4.1
Vertebral column 5.1 9.3 2.5
Rib, sternum, clavicle 13.6 10.7 3.2
Pelvic bones 18.0 25.1 10.1
Overlapping lesions 0.4 0.5 0.2
Bone, NOS 1.7 3.4 1.4
Total cases 9606 4870 11,961

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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Radiation only 3.2 1.6 1.9


Surgery and chemotherapy 3.5 24.4 45.5
Surgery and radiation 8.3 1.0 2.4
Radiation and chemotherapy 1.0 22.8 2.0
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Surgery, radiation and chemotherapy 1.1 15.1 3.2


Chemotherapy only 1.9 17.6 15.9
Other specified Rx 1.7 8.9 5.2
No 1st course Rx 10.7 5.3 8.0
Total cases 9606 4870 11,961

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
two societies was by way of a designated member from the
wCX1AWnYQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC1y0abggQZXdtwnfKZBYtws= on 09/20/2022

46% of osteosarcoma patients received combined surgery


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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