Function and quality-of-life of survivors of pelvic and lower extremity osteosarcoma and Ewing's sarcoma: The Childhood Cancer Survivor Study

Article (PDF Available)inBritish Journal of Cancer 91(11):1858-65 · December 2004with24 Reads
DOI: 10.1038/sj.bjc.6602220 · Source: PubMed
Limb-sparing surgeries have been performed more frequently than amputation based on the belief that limb-sparing surgeries provide improved function and quality-of-life (QOL). However, this has not been extensively studied in the paediatric population, which has unique characteristics that have implications for function and QOL. Using the Childhood Cancer Survivor Study, 528 adult long-term survivors of pediatric lower extremity bone tumours, diagnosed between 1970 and 1986, were contacted and completed questionnaries assessing function and QOL. Survivors were an average of 21 years from diagnosis with an average age of 35 years. Overall they reported excellent function and QOL. Compared to those who had a limb-sparing procedure, amputees were not more likely to have lower function and QOL scores and self-perception of disability included general health status, lower educational attainment, older age and female gender. Findings from this study suggest that, over time, amputees do as well as those who underwent limb-sparing surgeries between 1970 and 1986. However, female gender, lower educational attainment and older current age appear to influence function, QOL and disability.


Function and quality-of-life of survivors of pelvic and lower
extremity osteosarcoma and Ewing’s sarcoma: the Childhood
Cancer Survivor Study
R Nagarajan
, DR Clohisy
, JP Neglia
, Y Yasui
, PA Mitby
, C Sklar
, JZ Finklestein
, M Greenberg
GH Reaman
, L Zeltzer
and LL Robison
Division of Pediatrics, Epidemiology and Clinical Research, Department of Pediatrics, University of Minnesota, Mayo Mail Code 484, 420 Delaware St.,
SE Minneapolis, MN 55455, USA;
Department of Orthopedic Surgery, University of Minnesota, Mayo Mail Code 806, 420 Delaware St., SE
Minneapolis, MN 55455, USA;
Division of Hematology/Oncology/Blood and Marrow Transplant, Department of Pediatrics, University of Minnesota,
Mayo Mail Code 484, 420 Delaware St, SE Minneapolis MN 55455, USA;
Cancer Prevention Research Program. Fred Hutchinson Cancer Research
Center, 1100 Fairview Ave. N., PO Box 19024 Seattle, WA, 98109, USA;
Department of Pediatrics, Memorial Sloan Kettering Cancer Center, 1275 York
Ave, New York, NY 10021, USA;
Department of Pediatric Hematology/Oncology, Jonathan Jacques Childrens Cancers Cancer Center, 2653 Elm Ave,
Suite 200, Long Beach, CA 90806, USA;
Department of Hematology/Oncology, Hospital for Sick Children, 555 University Ave., Toronto, Ontario
M5G1X8, USA;
Department of Hematology/Oncology, Children’s National Medical Center, 111 Michigan Ave, NW, Washington DC 20010, USA;
Department of Pediatrics, University of California at Los Angels, 10833 Le Conte Ave., #22-464 MDCC, Los Angeles, CA 90095, USA
Limb-sparing surgeries have been performed more frequently than amputation based on the belief that limb-sparing surgeries provide
improved function and quality-of-life (QOL). However, this has not been extensively studied in the paediatric population, which has
unique characteristics that have implications for function and QOL. Using the Childhood Cancer Survivor Study, 528 adult long-term
survivors of pediatric lower extremity bone tumours, diagnosed between 1970 and 1986, were contacted and completed
questionnaries assessing function and QOL. Survivors were an average of 21 years from diagnosis with an average age of 35 years.
Overall they reported excellent function and QOL. Compared to those who had a limb-sparing procedure, amputees were not
more likely to have lower function and QOL scores and self-perception of disability included general health status, lower educational
attainment, older age and female gender. Findings from this study suggest that, over time, amputees do as well as those who
underwent limb-sparing surgeries between 1970 and 1986. However, female gender, lower educational attainment and older current
age appear to influence function, QOL and disability.
British Journal of Cancer (2004) 91, 1858 1865. doi:10.1038/sj.bjc.6602220
Published online 9 November 2004
& 2004 Cancer Research UK
Keywords: osteosarcoma; Ewing’s sarcoma; quality of life; function; lower extremity; bone tumour
Malignant bone tumours, predominantly osteosarcoma and
Ewing’s sarcoma, account for approximately 6% of all cancer
diagnosed under the age of 20 years (Ries et al, 1999) in the United
States. Nearly two-thirds of these tumours have a primary site in
the lower extremity of pelvis. Historically, treatment of extremity
bone tumours with amputation resulted in poor survival rates
(Wang and Schulz, 1953; Dahlin et al, 1961; Friedman and Carter,
1972). Beginning in the 1970s, treatment strategies changed to
include the use of multiagent chemotherapy (Rosen et al, 1979;
Link et al, 1986; Nesbit et al, 1990). This led to significant
improvements in the prognosis of children and young adults with
osteosarcoma and Ewing’s sarcoma and increased the overall
survival rate for patients with nonmetastatic disease from 10 to
20% (Wang and Schulz, 1953; Dahlin et al, 1961; Friedman and
Carter, 1972) to approximately 60% (Burgert et al, 1990; Bacci et al,
1998; Nesbit et al, 1990; Provisor et al, 1997). Enabled by improved
radiographic and surgical techniques, the surgical management of
patients with lower extremity lesions was increasingly character-
ized by an expanded use of limb-sparing surgeries (Springfield,
While there is general agreement that limb-sparing techniques
are the preferred approach for patients with upper extremity
tumours (Cheng and Gebhardt, 1991; Aboulafia and Malawer,
1993), debate continues regarding the long term outcomes of
amputation compared to limb-sparing surgery for lower extremity
primaries (Nagarajan et al, 2002). The current investigation
assessed self-reported function and quality of life (QOL) among
a large cohort of long-term survivors of childhood lower extremity
bone tumours.
Subject selection and eligibility
The childhood Cancer Survivor Study (CCSS) is a cohort of indivi-
duals with a confirmed diagonosis of cancer who participated
Received 8 March 2004; revised 23 June 2004; accepted 16 September
2004; published online 9 November 2004
*Correspondence: Dr R Nagarajan; E-mail:
British Journal of Cancer (2004) 91, 1858 1865
2004 Cancer Research UK All rights reserved 0007 – 0920/04
in the Long-Term Follow-Up Study, a multi-institutional
study of individuals who survived for 5 or more years after
treatment for cancer, leukaemia, tumours or similar illnesses
diagnosed during childhood or adolescence. The methods and
cohort characteristics of the CCSS have been previously presented
in detail (Robison et al, 2002). Briefly, as of 1 November 2000,
20 276 participants met the cohort inclusion criteria of: (1)
diagnosis of one of the following cancers: brain tumour,
leukaemia, Hodgkin’s disease, non-Hodgkin’s lymphoma, kidney
cancer, neuroblastoma, soft-tissue sarcoma, or cancer of the bone;
(2) initial treatment of one of the 25 collaborating CCSS institution
see Appendix; (3) diagnosis date between 1 January 1970 and 31
December 1986; (4) age less than 21 years at diagnosis; and (5)
survival 5 years from diagnosis. A total of 1042 of the 1596 5-year
survivors of a bone tumour directly participated by completing the
CCSS baseline data collection questionnaire. Excluded were 328
survivors who declined participation, 221 considered lost to
follow-up after extensive tracing, and five who were pending.
The present report is restricted to the subset of 629 participants
who fulfilled the following additional criteria: (1) diagnosis of
osteosarcoma or Ewing’s sarcoma: (2) tumour located in the lower
extremity or the pelvis: (3) 18 years of age or older at the time of
the present evaluation: (4) availability of complete medical
records: (5) alive and able to complete a self-reported (not by
proxy) function and QOL assessment.
Overall, 84% (528/629) of the subjects contacted for the current
study participated by completing the function and QOL ques-
tionnaire. Of the 101 nonparticipants, two were lost to follow-up,
75 declined participation and 24 were pending participation at the
time of analysis. Nonparticipants were slightly more likely than
participants to be younger at diagnosis (12.7 years vs 13.5 years)
and to have an extremity lesion (97 vs 91%), but were less likely to
have graduated from college (27 vs 48%).
The CCSS protocol and contact documents were reviewed and
approved by the Human Subjects Committee at each participating
institution. All contact documents including the baseline ques-
tionnaire and the function and QOL assessments can be viewed at
Assessment of function and QOL
Subjects completed a self-administered questionnaire that in-
cluded the Toronto Extremity Salvage Score (TESS) (Davis et al,
1996, 1999) and the Quality of Life for Cancer Survivors (QOL-CS)
instrument (Ferrel et al, 1995a, b) The TESS (30 questions)
measures physical disability based on the patient’s report of their
function and was developed and validated specifically for
individuals who have undergone surgery for lower extremity
musculoskeletal tumours. The QOL-CS questionnaire (41 ques-
tions), developed and validated specifically for cancer survivors,
measures four domains of QOL (physical, psychological, social,
and spiritual well-being).
Cancer treatment information
Information on the characteristics of the original cancer diagnosis
was obtained on all consented eligible cases from the treating
institution. Surgical local control procedures were grouped into
amputation and nonamputation by using the abstracted ICD-9
codes and participants’ questionnaire responses. Subjects (n ¼ 24)
with an amputation 3 or more years after diagnosis were classified
by their initial surgical treatment.
Site codes (long bones of the lower extremity or pelvis) from the
CCSS medical records abstraction forms were the primary source
for classifying tumour site. More specific site information (e.g.
distal femur or proximal tibia) was obtained using a skeletal
diagram given to the bone tumour survivors, who were asked to
indicate the site of their tumour.
Data analysis
A priori participants were categorized to one of four groups
defined by age at diagnosis of the bone tumour (p12 years vs X12
years) and by the type of surgery (amputation vs limb-sparing
surgery) resulting in four age/surgery groups (p12/Amp and
X12/Amp: under and over the age of 12 years with an amputation;
p12/LS and X12/LS: under and over the age of 12 years with a
limb-sparing surgery). For comparison purposes, the X12/LS age-
surgery group was used as the reference group. The age cutoff of 12
years old was chosen because it represents an approximate marker
for emotional maturity (preadolescence and early adolescence)
(Brindis et al, 1992) and pubertal development, and is the
approximate age at which significant bone growth starts (Kreipe,
1992). Amputees were further subdivided into amputation only
and amputation with radiation therapy. Nonamputees (limb-
salvage) were categorised into: (1) radiation treatment only, (2)
arthrodesis, (3) arthrodesis with radiation treatment, (4) endo-
prosthetic reconstruction, (5) endoprosthetic reconstruction with
radiation treatment, (6) surgery not otherwise specified and (7)
surgery not otherwise specified with radiation treatment. There
were no rotationplasties noted. General health status was assessed
from questions from the initial baseline survey of CCSS
participants that asked about self-perceived general health.
Responses were dichotomised into good/very good/excellent
health vs fair/poor health.
TESS and QOL-CS questionnaires were scored according to
instructions of the authors of the respective scales. TESS and QOL-
CS scores were dichotomised for analyses by using the 25th
percentile of the scores. Comparisons of TESS and QOL-CS scores
across the age/surgery groups were performed using general linear
models, adjusting for perceived general health, gender, educational
attainment and age at the completion of the CCSS baseline
questionnaire. The TESS scale incorporated a self-rating scale of
disability, which was collapsed into a dichotomous variable: not
disabled (not at all disabled and mildly disabled) and disabled
(moderately and completely/severely disabled). The associations of
the age/surgery variable with the dichotomous disability variable
and with scoring below the 25th percentile on TESS or QOL-CS
were assessed using logistic regression models, adjusting for
general health, gender, educational attainment, and a categorical
age at questionnaire completion variable. In order to assure that
adjustment for general health status did not influence the
relationship between the age/surgery groups and the outcomes,
multivariate analyses were conducted with and without inclusion
of the general health status variable, which provided comparable
results. Given that the primary comparisons are based upon testing
of a priori hypotheses, no correction was made for multiple
Characteristics of study participants
In total, 80% of the participants had a previous diagnosis of
osteosarcoma. The most frequent site of tumour was the distal
femur (42%), followed by the proximal tibia (18%) (see Table 1).
The majority of participants had undergone amputation (63) and
was over the age of 12 years at diagnosis (65%). Figure 1
graphically represents the number of amputations performed by
year that appears to decrease with time. The median time between
diagnosis and evaluation was 21 years (range 1231 years). The
majority of survivors reported an educational level beyond high
school and considered themselves to be in good health.
Function/QOL of survivors of paediatric bone tumours
R Nagarajan et al
British Journal of Cancer (2004) 91(11), 1858 1865& 2004 Cancer Research UK
QOL, function and self-perception of disability (Tables 24)
Subjects in all four age/surgery groups scored relatively high with
mean function scores (scale of 1 100) between 83 and 88 and
mean quality-of-life scores (scale of 1 10) between 6.8. and 7.0
(Table 2) with no statistical difference in scores across groups.
TESS and QOL-CS scores correlated with self-report assessments
of disability, having Pearson’s, correlation coefficients of 0.62 and
0.45, respectively. Mean TESS scores for self-ratings of completely/
severely disabled, moderately disabled, mildly disabled, and not at
all disabled were 61.2, 74.4, 86.9, and 95.4, respectively. Mean QOL-
CS scores for these same groups were 5.0, 6.2, 7.0, and 7.6. When
the self-rating scores were collapsed into those who considered
themselves disabled or not disabled, significant differences were
found between the two disability groups in mean TESS (72.1 vs
90.1, Pp0.001) and QOL-CS scores (6.0 vs 7.2, Pp0.001).
Characteristics of the survivors classified as disabled or scoring
below the 25th percentile of TESS and QOL-CS are provided in
Table 3. In pairwise comparisons of the age/surgery groups, no
significant differences in mean TESS or QOL-CS scores, self-
perception of disability, or scoring below the 25th percentile on
QOL-CS or TESS scores were found after adjusting for general
health, gender, educational status, and age at questionnaire
completion (data not shown).
Poor general health status, older current age, and failure to
graduate from high school predicted self-perception of disability
and lower TESS and QOL-CS scores and were all statistically
significant in univariate analyses (Table 4). Those in the p12/Amp
group were significantly less likely to score below the 25th
percentile of TESS scores compared to subjects in other age/
surgery groups, while females were more likely to score below the
25th percentile of TESS scores compared to the males. Those with a
pelvic lesion and those receiving pelvic irradiation were more
likely to score below the 25th percentile of QOL-CS scores
(Table 4).
In multivariable analysis (Table 4) of self-perceived disability,
age/surgery group was not predictive; however, poor health status,
older current age (440 years), and not graduating high school
predicted an increased likelihood of perceived disability. Those in
the p12/Amp group were significantly less likely to score below
the 25th percentile in TESS scores. Poor health status, female
gender, and not graduating from high school were predictive of
TESS scores below the 25th percentile. When examining QOL-CS
scores, only female gender and poor health status were predictive
of scoring below the 25th percentile.
Tumour location/surgical procedure
Primary tumour site was not predictive of QOL-CS or TESS scores
below the 25th percentile; however, those with a proximal tibia
lesion appeared less likely to rate themselves as disabled (see
Table 5). There was a slightly lower than expected proportion of
cases with pelvic tumours (46/528) which typically would account
for 25% of Ewing’s sarcoma and 10% of osteosarcoma presenta-
tions (Arndt and Crist, 1999). This is likely due to the poorer
prognosis of those with pelvic tumours at the time this cohort was
diagnosed. Analysis of the two surgical groups (amputation vs
nonamputation) revealed no differences in self-perception of
disability or in scoring below the 25th percentile on TESS or
QOL-CS after adjusting for general health status, gender, educa-
tion, and age at questionnaire completion. In the site and surgery
group analyses, poor general health and older age at questionnaire
Table 1 Characteristics of study population
Lower extremity
survivors (n ¼ 528)
Female 270 (51.1%)
Male 258 (48.9%)
General health
Poor 49 (9.7%)
Good 457 (90.3%)
No high school 21 (4.2%)
High school graduate 235 (47.5%)
College graduate 239 (48.3%)
Specific site
Distal femur 226 (42.8%)
Proximal tibia 98 (18.6%)
Pelvis 46 (8.7%)
Other femoral sites 63 (11.9%)
Other tibial sites 46 (8.7%)
Fibular sites 39 (7.4%)
Nonspecific, nonpelvic site 10 (1.9%)
Tumor type
Osteosarcoma 422 (79.9%)
Ewing’s sarcoma 106 (20.1%)
Surgery type
Amputation only 317 (60.0%)
Amputation/XRT 19 (3.6%)
XRT only 32 (6.1%)
Arthrodesis/XRT 1 (0.2%)
Endoprosthesis/XRT 7 (1.3%)
NOS/XRT 40 (7.6%)
Arthrodesis 10 (1.9%)
Endoprosthesis 49 (9.3%)
Nonamputation, nonspecific 53 (10.0%)
Age at diagnosis
Mean (range) 13.5 (1 20)
Median 14.0
Years from diagnosis to questionnaire completion
Mean (range) 20.8 (13 31)
Median 21.0
Age at questionnaire completion (years)
Mean (range) 34.8 (19.49)
Median 35.0
p30 132 (25.0%)
31 35 145 (27.5%)
36 39 128 (24.2%)
X40 123 (23.3%)
21 23 28 26 24 27 32 17 22 18 18 21 15
Year of diagnosis
Figure 1 Frequency of amputation over time. Shaded bars represent the
number of amputations and open bars represent the number of limb
sparing procedures.
Function/QOL of survivors of paediatric bone tumours
R Nagarajan et al
British Journal of Cancer (2004) 91(11), 1858 1865 & 2004 Cancer Research UK
completion predicted worse outcomes (data not shown). Female
gender and less than a high school education were less consistent
in predicting poorer outcomes in the surgical group analysis.
As cure rates for childhood cancers continue to rise, increasing
numbers of childhood cancer survivors will be entering adulthood
and will require close follow-up for late effects of their therapy.
One particular group warranting close evaluation are survivors of
bone tumours because of the nature of treatment, which often
includes intensive chemotherapy, surgery and, at times, radiation
therapy. As reviewed by Langeveld, several studies have already
highlighted problems encountered by adult survivors of paediatric
bone tumours (Langeveld et al, 2002). Late effects relating to
chemotherapy and irradiation (e.g. infertility, cardiomyopathy) are
varied and have been the subject of extensive research (Bhatia et al,
2003). Late effects relating to surgical local control of paediatric
lower extremity bone tumours have been less thoroughly explored
(Nagarajan et al, 2002). Local control procedures include
amputation and limb-sparing procedures (e.g. the use of radiation,
arthrodesis, endoprosthesis, allograft bone, etc.). The decision to
perform a limb-sparing surgery is based on the ability to achieve
an equivalent oncologic result and a comparable or better
functional result compared to amputation (Yaw, 1999).
Amputation was standard treatment prior to the development of
radiological and surgical techniques that have made limb-sparing
surgery the more frequent treatment choice (Provisor et al, 1997;
Bacci et al, 1998; Bielack et al, 2002). The preference for limb-
sparing surgery to treat paediatric lower extremity bone tumours
had been the subject of debate and warranted careful evaluation in
the past. The issues surrounding the debate have been succinctly
described in four questions (Simon, 1991): (1) will survival be the
same? (2) how do the immediate and delayed morbidities
(complications) compare? (3) how does function compare? and
Table 2 Raw scores of function (TESS), quality of life (QOL-CS), quality of life subscales and percent self-reported as disabled
Age/surgery subgroups
Outcome All participants (n ¼ 528) p12/Amp (n ¼ 121) p12/LS (n ¼ 63) 412/Amp (n ¼ 215) 412/LS (n ¼ 129)
Mean (SD) 85.5 (14.3) 87.6 (12.4) 88.6 (15.7) 83.8 (13.1) 84.5 (16.8)
Range 17.2 100 64.2 100 23.3 100 33.7 100 17.2 100
Mean (SD) 6.9 (1.4) 7.0 (1.4) 7.0 (1.2) 6.8 (1.3) 6.8 (1.4)
Range 1.4 9.7 6.3 9.4 3.4 9.0 3.2 9.7 2.4 8.9
Mean (SD) 6.0 (2.0) 5.6 (19) 6.3 (1.8) 6.0 (2.0) 6.1 (1.9)
Range 0.1 10 0.1 10 2.3 10 0.7 10 1.3 9.7
Mean (SD) 7.4 (1.9) 7.5 (1.9) 7.6 (2.0) 7.3 (1.9) 7.4 (2.0)
Range 0.9 10 0.9 10 1.0 10 1.4 10 1.0 10.0
Mean (SD) 6.5 (1.6) 6.8 (1.6) 6.5 (1.5) 6.4 (1.6) 6.4 (1.6)
Range 0.9 9.9 0.9 9.9 2.7 9.2 2.2 9.8 2.0 9.3
Mean (SD) 7.9 (1.6) 8.2 (1.6) 8.2 (1.5) 7.9 (1.7) 7.7 (1.6)
Range 1.0 10 1.9 10 4.0 10 1.0 10 1.6 10.0
Self-rating disability (expressed as subjects (%))
Severely/completely 24 (4.6%) 2 (1.7%) 3 (4.8%) 11 (5.1%) 8 (6.3%)
Moderately 110 (21.0%) 27 (22.5%) 8 (12.7%) 54 (25.4%) 21 (16.4%)
Mildly 241 (46.0%) 60 (50.0%) 22 (34.9%) 112 (52.6%) 47 (36.7%)
Not disabled 149 (28.4) 31 (25.8%) 30 (47.6%) 36 (16.9%) 52 (40.6%)
Table 3 Characteristics of survivors reporting disability and TESS and
QOL-CS scores below the 25th
Characteristic No.
disabled (%)
TESS score
below 25th
percentile (%)
QOL-CS score
below 25th
percentile (%)
Age/surgery group
p12/Amp 121 24.2 15.8 20.0
p12/LS 63 17.5 17.5 22.2
412/Amp 215 30.5 28.6 29.1
412/LS 129 22.7 28.1 25.8
Amputation status
Amputation 336 28.2 24.0 25.8
192 20.9 24.6 24.6
General health
457 27.7 21.9 21.4
Fair/poor health 49 61.2 53.1 69.4
Female 270 26.2 29.2 28.8
Male 258 24.9 19.1 21.8
No H.S.
21 57.1 52.4 47.6
H.S. graduation 235 27.4 23.9 23.5
239 21.1 21.1 25.3
Age at questionnaire completion
p30 years old 132 16.8 15.3 17.6
31 35 years old 145 22.9 22.8 22.9
36 39 years old 128 26.0 26.8 30.7
X40 years old 123 27.7 32.8 31.2
Function/QOL of survivors of paediatric bone tumours
R Nagarajan et al
British Journal of Cancer (2004) 91(11), 1858 1865& 2004 Cancer Research UK
(4) does limb-sparing surgery impart improved psychosocial/
quality of life outcomes? Regarding the first question, survival and
local recurrence rates between amputation and limb-sparing
surgery have not been found to be significantly different when
adequate margins are achieved and adjuvant chemotherapy is used
(Simon et al, 1986; Sluga et al, 1999; Bacci et al, 2000; Bielack et al,
2002). When considering short- and long-term complications,
several studies have shown that there are more complications
following limb-sparing surgery (Ruggieri et al, 1993; Rougraff et al,
1994; Lindner et al, 1999; Nagarajan et al, 2002). Of note, with new
techniques and materials being developed for limb-sparig sur-
geries, long-term outcomes need to be continually evaluated.
In contrast, the last two questions have not been extensively
studied, which is the basis for the current investigations. Overall, a
trend toward an improvement of function has been reported for
limb-sparing approaches. No differences in quality of life have thus
far been shown between amputation and limb-sparing operative
procedures. However, it is important to note that despite a number
of authors addressing these outcomes, the overall conclusions have
limitations because of the differing methodologic approaches and
assessment tools used, small sample sizes, short follow-up, and
limited study of adult survivors of childhood cancer (Nagarajan
et al, 2002). The most common instrument that has been used for
functional assessment has been the Musculoskeletal Tumour
Society survey, which relies on the subjective ratings given by
the administering clinician with no other objective measure of
function. This has been questioned as to whether this is an accurate
reflection of function (Marchese et al, 2004) and whether other
more global functional assessments with QOL measures (TESS and
QOL-CS) are needed to better represent the clinical status.
Children with lower extremely bone tumours have unique
characteristics with implications for functional and quality-of-life
outcomes. The emotional maturity of patients at the time of
diagnosis can influence their ability to accept the loss of limb due
to cancer (Kagan, 1976; Ettinger and Heiney, 1993; Felder-Puig
et al, 1998). Skeletal maturity is another important determinant of
functional and QOL outcomes in children with lower extremity
bone sarcomas because of its importance in determining the type
of local surgical control (amputation/rotationplasty/expanding
prosthesis) and the associated risk of complications. Those who
are skeletally immature and have substantial growth potential at
diagnosis are often treated with limb-sparing surgery, which
requires removal of a skeletal growth plate. Such patients often
Table 4 Odds ratio for considering themselves disabled (moderately, severely or completely disabled) or having a TESS or QOL-CS score below the
25th percentile
Univariate Multivariate
Subgroups Self-rating disable TESS score QOL-CS score Self-rating disable TESS score QOL-CS score
p12/Amp 1.1 (0.60 1.96) 0.5 (0.26 0.90)* 0.7 (0.40 1.31) 1.5 (0.77 3.10) 0.4 (0.19 0.92)* 1.1 (0.54 2.37)
p12/LS 0.7 (0.33 1.56) 0.5 (0.25 1.15) 0.8 (0.40 1.68) 0.9 (0.37 2.19) 0.6 (0.25 1.45) 0.9 (0.37 2.06)
412/Amp 1.5 (0.90 2.49) 1.0 (0.63 1.67) 1.2 (0.72 1.94) 1.1 (0.74 1.78) 0.9 (0.55 1.59) 1.2 (0.69 2.25)
412/LS 1.0 (Ref) 1.0 (Ref) 1.0 (Ref) 1.0 (Ref) 1.0 (Ref) 1.0 (Ref)
Poor health 5.4 (2.90 9.93)** 4.1 (2.27 7.59)** 8.3 (4.35 15.89)** 4.9 (2.49 9.76)** 2.8 (1.41 5.47)** 7.5 (3.69 15.24)**
Good health 1.0 (ref) 1.0 (ref) 1.0 (Ref) 1.0 (Ref) 1.0 (Ref) 1.0 (Ref)
Female 1.1 (0.72 1.59) 1.7 (1.17 2.63)* 1.5 (0.98 2.16) 1.1 (0.74 1.78) 1.7 (1.08 2.68)* 1.6 (1.05 2.59)*
Male 1.0 (Ref) 1.0 (Ref) 1.0 (Ref) 1.0 (Ref) 1.0 (Ref) 1.0 (Ref)
Current age (years)
p30 0.3 (0.19 0.60)** 0.4 (0.20 0.68)* 0.5 (0.26 0.85)* 0.3 (0.13 0.62)** 0.5 (0.22 1.06) 0.5 (0.22 1.03)
31 35 0.5 (0.29 0.84)* 0.6 (0.35 1.00) 0.7 (0.38 1.13) 0.5 (0.26 0.86)* 0.6 (0.33 1.12) 0.7 (0.38 1.34)
36 39 0.6 (0.34 1.0)* 0.7 (0.44 1.29) 1.0 (0.57 1.68) 0.5 (0.28 0.95)* 0.8 (0.42 1.39) 1.0 (0.54 1.79)
X40 1.0 (Ref) 1.0 (Ref) 1.0 (Ref) 1.0 (Ref) 1.0 (Ref) 1.0 (Ref)
No high school 5.0 (1.99 2.50)** 4.1 (12.65 10.24)** 2.7 (1.09 6.63)* 4.3 (1.52 12.24)** 4.2 (1.52 12.24)** 1.5 (0.51 4.36)
High school graduate 1.4 (0.92 2.15) 1.2 (0.76 1.81) 0.9 (0.60 1.38) 1.4 (0.88 2.19) 1.2 (0.77 1.96) 0.6 (0.53 1.36)
College graduate 1.0 (Ref) 1.0 (Ref) 1.0 (Ref) 1.0 (Ref) 1.0 (Ref) 1.0 (Ref)
Ewing’s sarcoma 0.7 (0.43 1.20) 0.9 (0.54 1.49) 0.8 (0.47 1.29)
Osteosarcoma 1.0 (Ref) 1.0 (Ref) 1.0 (Ref)
Pelvic site 1.3 (0.67 2.52) 1.6 (0.82 3.03) 2.0 (1.08 3.80)* 0.9 (0.25 3.56)
Limb site 1.0 (Ref) 1.0 (Ref) 1.0 (Ref) 1.0 (Ref)
Pelvic irradiation 1.3 (0.60 2.61) 1.6 (0.76 3.18) 2.4 (1.22 4.76)* 2.6 (0.63 10.87)
No pelvic irradiation 1.0 (Ref) 1.0 (Ref) 1.0 (Ref) 1.0 (Ref)
Limb irradiation 1.1 (0.58 1.94) 0.7 (0.42 1.33) 1.6 (0.82 3.10)
No limb irradiation 1.0 (Ref) 1.0 (Ref) 1.0 (Ref)
Ampution 1.5 (0.97 2.27) 1.0 (0.64 1.47) 1.1 (0.71 1.61)
Limb sparing surgery 1.0 (Ref) 1.0 (Ref) 1.0 (Ref)
p12 years old at surgery 0.7 (0.48 1.2) 0.5 (0.31 0.78)** 0.7 (0.44 1.04)
412 years old at surgery 1.0 (Ref) 1.0 (Ref) 1.0 (Ref)
*Po0.05, **Po0.01.
Table 5 Odds ratio of disability, TESS score (below 25th percentile), or
QOL-CS score (below 25th percentile)
disabled OR (CI)
TESS score
QOL-CS score
Pelvis 0.9 (0.36 2.15) 1.3 (0.56 3.16) 1.3 (0.55 3.14)
0.5 (0.25 0.96)* 0.7 (0.39 1.42) 0.7 (0.38 1.44)
Distal femur 1.0 (Ref) 1.0 (Ref) 1.0 (Ref)
Amputation 1.3 (0.82 2.08) 0.8 (0.54 1.34) 1.0 (0.66 1.64)
Limb-sparing 1.0 (Ref) 1.0 (Ref) 1.0 (Ref)
Comparisons by site adjusted for general health status, amputation, education, gender
and age. Comparisons by surgical procedure adjusted for general health status,
gender, education and age. *Po0.05.
Function/QOL of survivors of paediatric bone tumours
R Nagarajan et al
British Journal of Cancer (2004) 91(11), 1858 1865 & 2004 Cancer Research UK
need multiple subsequent surgeries to accommodate growth of the
unaffected limb. Moreover, children have a substantial lifespan
ahead of them following successful treatment of their malignancy
and this may increase the potential risk of further reconstructive
procedures and complications.
With the establishment of the CCSS to facilitate the investigation
of late effects among long-term survivors of childhood cancer, we
were able to examine some of these key issues. The current study
represents the largest series of adult survivors of paediatric lower
extremity bone tumours thus far evaluated for function and QOL.
However, some limitations must be considered in the interpreta-
tion of the results of this study. These include the inability to
further classify the procedures that nonamputees (e.g. allograft vs.
endoprosthesis) and amputees (above the knee vs below the knee
amputation) received and the fact that this study provides
information regarding treatments performed between 1970 and
1986, which are likely quite different from today’s surgical
treatments. However, this study does provide an excellent
assessment of the outcomes of amputees, which would be difficult
to examine today given the infrequency with which amputations
are performed for paediatric lower extremity bone tumours. An
additional shortcoming of the study relates to the length of time
from diagnosis (median 21 years) to current assessment, which
precludes the examination of outcomes within 10 years of
diagnosis. It is in this time frame that differences between groups
may be substantial and may be masked or lost by longer follow-up.
Other issues involved the assessment of subsequent surgeries or
complications. Since the assessment of surgeries was restricted to
the initial treating institution and complications were not
specifically noted, the accurate enumeration of subsequent
surgeries and complications is not possible. Further limitation of
the study includes ascertainment bias since the cohort includes
only those who have agreed to participate in the CCSS. This may
underestimate deficits by excluding those who are having more
difficulty adjusting and are unable or unwilling to participate.
We found no major differences in function and quality of life
between those who had an amputation and those treated with
limb-sparing surgery. Additionally, our assessment of the large
population of patients who underwent amputation provides clear
indication that amputees do well long-term. Thus, when an
amputation is clinically indicated, patients, families and clincians
can assume that long-term amputees have no different function
and QOL compared to those without an amputation. One may
attribute the lack of reported differences between amputees and
nonamputees to ‘adjustment and accommodation’ to their current
condition, rather than having comparable physical ability such as
range of motion and strength. To determine actual physical ability,
via clinical examination, in a cohort as large as this and as far from
diagnosis would be very difficult to accomplish. Additionally, one
may argue that ‘actual’ physical ability is only a component of
overall perceived physical function, which is influenced by other
individualised factors including self-image, motivation and social
support (Neugebauer, 2000).
Females reported significantly lower function and QOL, but did
not report more disability. Survivors with a lower educational
attainment also appeared to have lower function and QOL scores
and a significantly increased likelihood of self-reported disability.
In the author’s prior study of psychosocial outcomes (Nagarajan
et al, 2003) in this group of survivors, female survivors were less
likely to be employed and those with a higher educational
attainment were more likely to ever been employed or married
or ever have insurance. Female gender-related deficiencies may be
related to differences in coping styles (Znajda et al, 1999) and
other gender-specific issues and the benefit of higher educational
attainment may suggest more available opportunities or better
social support. In the current study, those who were younger in age
at completion of the questionnaire were less likely to have lower
TESS and QOL-CS scores and significantly less likely to consider
themselves disabled. This is most likely due to issues related to
normal aging.
Further follow-up of this cohort, including reassessment to
observe any changes in scores and perceived disability over time, is
clearly warranted. It will also be important to prospectively
investigate post-1986 populations of paediatric lower extremity
bone tumour survivors in order to see how current surgical
techniques affect function and quality of life. These prospective
studies must encompass long-term assessments and integrate
uniform methods of recording complications associated with the
surgical procedures, as well as uniform collection of function and
QOL data. Such an approach will aid paediatric oncologists and
orthopaedic surgeons by providing insight into disease control,
functional outcomes, and quality of life. We hope that this research
study will help provide patients and families with needed
information on anticipated long-term outcomes and on how to
maximise function and quality of life as these paediatric patients
grow into adulthood.
This work was supported by National Institutes of Health Grants
U24-CA55727 and T32-CA09607, Bethesda, MD, USA; Children’s
Cancer Research Fund, Minneapolis, MN, USA; National Child-
hood Cancer Foundation Research Fellowship, Arcadia, CA, USA
and American Society of Clinical Oncology Young Investigator
Award, Alexandria, VA, USA.
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CCSS Institutions and Investigators
University of California, San Francisco, CA Arthur Ablin, MD*
University of Alabama, Birmingham, AL Roger Berkow, MD*
International Epidemiology Institute, Rockville, MD John Boice, ScD
University of Washington, Seattle, WA Norman Breslow, PhD
UT-Southwestern Medican Center at Dallas, TX George R, Buchanan, MD*, Kevin Oeffinger. MD
Cincinnati Children’s Hospital Medical Center Stella Davies, MD, PhD
Dana-Farber Cancer Institute, Boston, MA Lisa Diller, MD*, Holcombe Grier, MD
, Frederick Li MD
Texas Children’s Center, Houston, TX Zoann Dreyer, MD*
Children’s Hospital and Medical Center, Seattle, WA Debra Friedman, MD, MPH*, Thomas Pendergrass, MD
Reswell Park Cancer Institute, Buffalo, NY Daniel M Green, MD*
Hospital for Sick Children, Toronto, NY Mark Greenberg, MB ChB*
St. Louis Children ‘s Hospital, MO Robert Hayashi, MD*, Teresa Vietti, MD
St. Jude Children’s Research Hospital, Memphis, TN Melissa Hudson MD*
University of Michigan, Ann Arbor, MI Raymond Hutchinson MD*
Stanford University School of Medicine, Stanford, CA Michael P Link, MD*, Sarah S Donaldson, MD
Function/QOL of survivors of paediatric bone tumours
R Nagarajan et al
British Journal of Cancer (2004) 91(11), 1858 1865 & 2004 Cancer Research UK
Emory University, Atlanta, GA Lillian Meacham, MD*
Children’s Hospital of Philadelphia PA Anna Meadows, MD*
Children’s Hospital, Oklahoma City, OK John Mulvihill MD
Children’s Hospital, Denver, CO Brain Greffe*, Lorrie Odom, MD
Children’s Health Care-Minneapolis, MN Maura O’Leary, MD*
Columbus Children’s Hospital. OH Amanda Termuhlen MD*, Frederick Ruymann, MD
, Stephen Qualman, MD
Children’s National Medical Center. Washington, DC Gregory Reaman, MD*, Roger Packer, MD
Children’s Hospital of Pittsburgh, PA A Kim Ritchey, MD*, Julie Blatt MD
University of Minnesota. Minneapolis. MN Leslie L. Robison PhD*
, Ann Mertens, PhD
, Joseph Neglia, MD, MPH
, Mark
Nesbit, MD
Children’s Hospital Los Angeles, CA Kathy Ruccione, RN, MPH*
Memorial Sloan-Kettering Cancer Center New York Charles Sklar, MD*
National Cancer Institute, Bethesda, MD Malcolm Smith, MD
, Peter Inskip, ScD
Mayo Clinic, Rochester, MN W. Anthony Smithson, MD*, Gerald Gilchrist, MD
UTMD Anderson Cancer Center, Houston, TX Louise Strong, MD*
, Marilyn Stovall, PhD
Riley Hospital for Children, Indianapolis IN Terry A, Vik, MD*
, Robert Weetman, MD
Fred Hutchinson Cancer Center, Seattle. WA Yutaka Yasui, PhD*
, John Potter, MD., PhD*
University of California-Los Angeles. CA Lonnie Zeltzer, MD*
*Institutional Principal Investigator
Former Institutional Principal Investigator
Member CCSS Steering Committee
Function/QOL of survivors of paediatric bone tumours
R Nagarajan et al
British Journal of Cancer (2004) 91(11), 1858 1865& 2004 Cancer Research UK
    • "Prior investigations have relied on self-report or clinicianbased observations to describe long-term functional outcomes and HRQOL in adult survivors of childhood extremity sarco- mas [6, 11, 30, 31]. In this study, we provide results of an extensive clinical assessment of long-term musculoskeletal sarcomas of the extremities among survivors evaluated decades following treatment. "
    [Show abstract] [Hide abstract] ABSTRACT: Purpose This study compared measured physical performance, health-related quality of life (HRQOL), and social role attainment between extremity sarcoma survivors and controls, and evaluated associations between disease and treatment exposures, health conditions, and performance measures. Methods Survivors of extremity sarcoma from the St. Jude Lifetime cohort and controls frequency matched by age-, sex-, and race completed physical performance testing and questionnaires. Survivors with Z-scores on outcome measures ≤ −2.0 SD (compared to controls) were categorized with severe impairment/limitation. Results Among 206 survivors (52.4 % male median age 36 years (range 19–65)), 37 % had low relative lean mass, 9.7 % had an ejection fraction <50 %, 51.5 % had diffusion capacity for carbon monoxide <75 %, 27.7 % had sensory and 25.2 % motor neuropathy, and 78.2 % had musculoskeletal complications. Severe impairments/limitations were present among ≥25 % of survivors on fitness, balance, and physical HRQOL measures, and among ≥15 % on strength and activity of daily living measures. Lower extremity tumor location (OR 8.23, 95 % CI 2.54–26.67, P value 0.0004) and amputation (OR 8.07, 95 % CI 3.06–21.27, P value <0.0001) were associated with poor fitness. Poor fitness was associated with increased odds of scoring <40 on the SF-36 physical component summary (OR 4.83, 95 % CI 1.95–11.99, P value 0.001) and role-physical subscale (OR 3.34, 95 % CI 1.33–8.43, P value 0.01). Survivors and controls had similar rates of marriage, independent living, employment, and college attendance. Conclusions Extremity sarcoma survivors experience high rates of physical impairment and report lower than expected physical HRQOL. However, they are as likely as peers to be married, live independently, be employed, and attend college. Implications for Cancer Survivors Follow-up for extremity sarcoma survivors should include assessment of need for further orthopedic care and rehabilitation to address cardiopulmonary and musculoskeletal health.
    Article · Jun 2016
    • "Consistent with other studies (Eiser et al, 2001; Hudson et al, 2003; Zeltzer et al, 2008), we reported that survivors were severely limited in health status, in particular physical function and pain. Although previous studies have suggested that health status among amputees is generally similar to non-amputees (Eiser and Grimer, 1999; Nagarajan et al, 2004; Paul, 2008; Eiser, 2009; Nagarajan et al, 2009; Barrera et al, 2012), we found that osteosarcoma amputees reported the worst health status for all scales, with significantly higher limitations in physical function relative to osteosarcoma non-amputees and Ewing sarcoma (95% of which were non-amputees) survivors. Although we report here on appreciable proportions of the bone sarcoma survivors experiencing detrimental effects to their health, many of their social outcomes were favourable. "
    [Show abstract] [Hide abstract] ABSTRACT: With improved survival, more bone sarcoma survivors are approaching middle age making it crucial to investigate the late effects of their cancer and its treatment. We investigated the long-term risks of adverse outcomes among 5-year bone sarcoma survivors within the British Childhood Cancer Survivor Study. Cause-specific mortality and risk of subsequent primary neoplasms (SPNs) were investigated for 664 bone sarcoma survivors. Use of health services, health and marital status, alcohol and smoking habits, and educational qualifications were investigated for survivors who completed a questionnaire. Survivors were seven times more likely to experience all-cause mortality than expected, and there were substantial differences in risk depending on tumour type. Beyond 25 years follow-up the risk of dying from all-causes was comparable to the general population. This is in contrast to dying before 25 years where the risk was 12.7-fold that expected. Survivors were also four times more likely to develop a SPN than expected, where the excess was restricted to 5-24 years post diagnosis. Increased health-care usage and poor health status were also found. Nonetheless, for some psychosocial outcomes survivors were better off than expected. Up to 25 years after 5-year survival, bone sarcoma survivors are at substantial risk of death and SPNs, but this is greatly reduced thereafter. As 95% of all excess deaths before 25 years follow-up were due to recurrences and SPNs, increased monitoring of survivors could prevent mortality. Furthermore, bone and breast SPNs should be a particular concern. Since there are variations in the magnitude of excess risk depending on the specific adverse outcome under investigation and whether the survivors were initially diagnosed with osteosarcoma or Ewing sarcoma, risks need to be assessed in relation to these factors. These findings should provide useful evidence for risk stratification and updating clinical follow-up guidelines.British Journal of Cancer advance online publication, 19 May 2015; doi:10.1038/bjc.2015.159
    Full-text · Article · May 2015
    • "Significant differences within the domain school environment in this study are consistent with previous studies. Eiser and Morse (2001), Nagarajan et al. (2004) and Mokkink et al. (2007 reported a reduced school performance and high absence in adolescents with a malignant disease. The school attendance might be increased by use of 'the Web Chair' (or equivalent web-based learning platform), one especially for secondary schools developed mobile system that uses web based connection, which can be established between the school and the patient at home or in the hospital (Al-Rousan, 2005). "
    [Show abstract] [Hide abstract] ABSTRACT: Purpose Adolescents experience physical and psychosocial changes as part of their normal development. It can be hypothesized that they have lower scores on Quality of Life (QoL) and self-perception when additional changes occur due to cancer treatment. The purpose of our study was to assess self-perception and QoL of adolescents during or up to three months after adjuvant treatment for a primary malignant bone tumour. Methods Ten adolescent patients (median age of 15 years) were included. Every patient was matched with two healthy peers. Participants completed the dutch version of the Self Perception Profile of Adolescents (SPPA) to measure self-perception and the KIDSCREEN-52 questionnaire for QoL. For both instruments, normative data were available. Results Adolescents with a bone tumour had consistently lower scores on QoL as compared to healthy peers. Significantly on domains: physical well-being (P < 0.002), autonomy (P = 0.02), social support (P = 0.04) and school environment (P = 0.02). Scores on self-perception in this group were similar in both the study and control group. Conclusions Adolescents with a primary malignant bone tumour during or up to three months after adjuvant treatment had lower scores on QoL (KIDSCREEN-52), significantly on domains of physical well-being and social functioning. Unlike most other quality of life instruments, the KIDSCREEN-52 contains different areas of social functioning and has shown to be a useful instrument in our patient group. Scores on self-perception in this group were similar in both study and control group.
    Full-text · Article · Jun 2014
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