Abstract
Background: Skeletally immature children with a primary malignant
tumor in the distal end of the femur are candidates for limb-salvage surgery;
however, functional impairment due to subsequent limb-length discrepancy must
be considered. Our aim was to evaluate the long-term clinical outcome of limb
salvage in patients with a sarcoma of the distal end of the femur who were
eleven years old or less, focusing on limb-length discrepancy and
complications.
Methods: The cases of forty children were retrospectively reviewed
in a multicenter study based on the responses to a questionnaire. Twenty-eight
patients had had endoprosthetic reconstruction, and twelve had had biological
reconstruction. Functional evaluation was based on the Musculoskeletal Tumor
Society scoring system, with numerical values from 0 to 5 points assigned for
each of the following six categories: pain, function, emotional acceptance,
use of supports, walking ability, and gait. These values were added, and the
functional score was presented as a percentage of the maximum possible score.
Limb-length discrepancy was measured with orthoroentgenograms. Complications
and their treatment were analyzed. Patient survival and the survival of the
reconstructions were analyzed with use of the Kaplan-Meier method.
Results: Seven patients died and thirty-three remained alive, for a
survival rate of 82% at ten years postoperatively. For the surviving patients,
the mean follow-up periods (and standard deviations) were similar for the
twenty-two who had endoprosthetic reconstruction (13.2 ± 3.9 years) and
the eleven who had biological reconstruction (10.4 ± 4.4 years). All
patients had reached skeletal maturity. The mean final functional score was
74% ± 18% in the endoprosthetic reconstruction group and 68% ±
17% in the biological reconstruction group (p = 0.37). For the nineteen
patients who underwent limb-lengthening, the mean functional score increased
significantly from 65% ± 21% before the procedure to 81% ± 11%
after the lengthening (p = 0.0016). There were five early and twenty-eight
late complications. In the endoprosthetic reconstruction group, the most
frequent complications were deep infection and aseptic loosening. In the
biological reconstruction group, the most frequent complications were implant
breakage and nonunion. Revision surgeries were required in seventeen patients,
including five who had an amputation. The rate of survival of the
endoprosthetic reconstructions was 77% at five years and 51% at ten years
postoperatively, whereas the rate of survival of the biological
reconstructions was 46% at both five and ten years postoperatively.
Conclusions: Endoprosthetic or biological reconstructions as limb
salvage provided good functional outcome in skeletally immature children with
a malignant bone tumor of the distal aspect of the femur despite a high rate
of revisions and limb-lengthening procedures.
Level of Evidence: Therapeutic Level III. See
Instructions to Authors for a complete description of levels of evidence.
Limb-salvage surgery is a widely accepted alternative to amputation in
patients with a sarcoma affecting the distal aspect of the
femur1,2.
The success of limb salvage is the result of advances in the understanding of
the biology and staging of tumors, improvement in the reconstructive
techniques, and the development of effective adjuvant chemotherapy for the
primary tumors. The patients can be expected to have long-term survival, which
is due, in particular, to the advances in
chemotherapy3-6.
The distal end of the femur is the most common site for a primary malignant
bone tumor in
children6-8.
The distal femoral physis presumably contributes approximately 10 mm of growth
per year until skeletal maturity at approximately sixteen years of age in boys
and fourteen years of age in
girls9,10.
In skeletally immature patients, the eventual functional impairment that is
due to the subsequent growth inhibition and the discrepancy in the length of
the lower extremities must be considered in conjunction with limb-salvage
surgery of the distal end of the femur. Several techniques, such as extendable
endoprostheses8,11,12
or distraction
osteogenesis13,14,
have been applied to address this problem.
We performed a multi-institutional retrospective analysis based on
responses to a questionnaire. Our aim was to assess the long-term clinical
outcome of limb-salvage techniques for the treatment of sarcomas in the distal
end of the femur in children who were eleven years old or less. In particular,
we focused on problems related to limb-length discrepancy and
complications.
Questionnaires
In 1998, questionnaires were sent to 101 institutions belonging to the
Japanese Musculoskeletal Oncology Group. The group consists of institutions
with a department of orthopaedic oncology. The questionnaires were completed
by orthopaedic surgeons on the basis of the data in the patients' charts.
Questionnaires were received from forty-one (41%) of the 101 institutions.
The questionnaires included the following basic parameters: the age and sex
of the patient, date of hospitalization, primary site of the lesion,
histological diagnosis, and surgical stage according to the system of Enneking
et al.15. Stage I
indicates a low-grade lesion; stage II, a high-grade lesion; and stage III, a
lesion with either regional or distant metastases. Also, questions were asked
with regard to treatment, including neoadjuvant and/or adjuvant chemotherapy,
radiation therapy, the date of surgery, the surgical
margin15, and the
type of surgery (endoprosthetic or biological reconstruction). Furthermore,
the recipients were asked to grade the functional assessment, using the
scoring system of the Musculoskeletal Tumor
Society16, and to
describe the limb-length discrepancy and limb-lengthening procedures,
complications, and the management of complications. The scoring system of the
Musculoskeletal Tumor Society is based on six categories: pain, function,
emotional acceptance, use of supports, ability to walk, and gait. Each
category was assigned a numerical value from 0 to 5 points, with 5 points
indicating the best function. The values for each category were added, and the
functional score was presented as a percentage of the maximum possible score
(30 points).
Finally, the status of the patient with regard to the disease at the time
of final follow-up was recorded as continuously disease-free, no evidence of
disease at present but recurrence or metastasis in the past, or died of
disease.
In order to extend the follow-up data, subsequent questionnaires were sent
in September 2001 and April 2004 to the institutions that had responded in
1998. Responses were received from thirty-four (83%) of the forty-one
institutions in 2001 and from all forty-one in 2004.
Selection of the Patients
Patients were eligible for this study if they met the following criteria:
(1) they were eleven years of age or less at the time of diagnosis, (2) they
had a primary malignant bone tumor in the metaphysis and/or the epiphysis of
the distal end of the femur, and (3) they had been selected to have
limb-salvage surgery. Patients who had amputation or rotationplasty were
excluded. Also, patients with a primary malignant bone tumor in the proximal
end or shaft of the femur were excluded. Thus, forty patients from
twenty-three institutions who were treated during the period from 1981 to 1997
met the eligibility criteria. The patients were divided into two groups
according to the primary operative procedure: endoprosthetic reconstruction
(twenty-eight patients) and biological reconstruction (twelve patients).
Since this was a multicenter retrospective study, no randomization protocol
for implementation of the surgical procedures was followed. The final decision
was made by the treating orthopaedic surgeons at each of the participating
institutions.
Complete informed consent was obtained from each patient and his or her
parents. Institutional review board approval was obtained from the
institutions of the primary investigators.
Characteristics of the Patients
Demographic data on the patients are provided in the Appendix. Thirty-nine
patients had osteosarcoma, and one had Ewing sarcoma. The surgical stage
according to the system of Enneking et
al.15 was stage
II-B in thirty-five patients and stage II-A in five patients. The surgical
margin was wide in all patients except for two who had radical margins and
three who had marginal margins. The twenty-eight patients who had
endoprosthetic reconstruction were managed with custom-made endoprostheses
(eighteen patients), extendable endoprostheses (seven patients), and modular
resection systems (three patients). Of the twelve patients who had biological
reconstruction, eight were managed with knee arthrodesis after resection and
interposition with autogenous bone grafts (four patients), allograft (two
patients), or a vascularized fibular graft (two patients), and four underwent
en bloc tumor resection with preservation of the epiphysis, followed by
reconstruction with distraction osteogenesis (three patients) or a
vascularized fibular graft (one patient).
All patients received neoadjuvant and adjuvant chemotherapy except one
(Case 17) with a parosteal osteosarcoma. In the one patient with a Ewing
sarcoma (Case 4), preoperative and postoperative radiation therapy was also
performed. One other patient (Case 40) received preoperative radiation therapy
to obtain adequate surgical margins since there was a pathological fracture
and the extraosseous tumor was adjacent to a neurovascular bundle.
Clinical Outcome
Postoperative function was assessed with the scoring system of the
Musculoskeletal Tumor
Society16. The
limb-length discrepancy was evaluated with use of orthoroentgenograms. A
discrepancy of =2 cm was considered substantial enough to require
correction
surgery17. Two
major procedures, either distraction osteogenesis or endoprosthetic expansion,
were used. In addition, the use of a shoe lift was noted. The limb-length
discrepancy at the time of the last follow-up was measured.
Complications related to surgery, such as infection, implant breakage,
aseptic loosening, dislocation, skin necrosis, fractures, and nonunion, were
evaluated. Local recurrences were also recorded. A complication was defined as
early when it occurred within six months after the primary surgery and as late
when it occurred more than six months after the primary surgery. The
management of the complications, such as revision surgery (removal of the
endoprosthesis, arthrodesis, or amputation), was also recorded. Furthermore,
complications related to the disease itself, such as metastases, and any
treatment of such complications, were recorded.
Survival of the Reconstructions
The procedure was considered to be a failure when revision surgery had to
be performed for any reason or when the patient needed an amputation. The
survival of the reconstructions was calculated, with use of the Kaplan-Meier
method, from the time of the initial surgery until the date when revision or
amputation had to be performed or until the time of the latest follow-up.
Furthermore, the limb salvage was considered successful whenever the patient
had a preserved limb at the time of final follow-up despite any complications
or revisions.
Survival of the Patients
At the time of final follow-up, the patients were categorized according to
their prognosis into one of three groups: those who were continuously
disease-free, those with no evidence of disease, and those who had dvied of
disease. We calculated the survival rates for all patients and for the
subgroups according to the reconstruction procedure.
Statistical Analyses
For comparisons among groups, analysis of variance and analysis of variance
factorial tests were used, except for categorical data for which chi-square
tests were applied. Simple and multiple regression analyses were performed.
The survival rate of the patients was estimated with the method of Kaplan and
Meier18. This
method was also used to estimate complicationfree rates and survival of the
reconstruction procedures. The Mantel-Cox log-rank test was used to compare
the survivorship curves. All analyses were performed with use of the StatView
software package for Macintosh (Abacus Concepts, Berkeley, California). A p
value of <0.05 for a type-I error was considered to be significant.
Oncological Results
Seven patients died from metastases (six had lung metastases, and one had
brain metastases) at a mean duration of follow-up (and standard deviation) of
2.8 ± 2.3 years (range, 0.8 to 6.3 years). Three patients had no
evidence of disease, and thirty were continuously disease-free. No local
recurrence was found in the total series of forty patients. The cumulative
rates of survival in the total series of patients were 85% (95% confidence
interval, 74% to 96%) at five years postoperatively and 82% (95% confidence
interval, 71% to 94%) at ten years postoperatively. Furthermore, we calculated
the cumulative rates of survival for each of the surgical subgroups. The
survival rates at five and ten years were 82% (95% confidence interval, 68% to
96%) and 78% (95% confidence interval, 63% to 94%), respectively, in the
endoprosthetic reconstruction group and 92% (95% confidence interval, 76% to
100%) at both five and ten years in the biological reconstruction group
(Fig. 1). There was no
significant difference between the groups (p = 0.3160).
For the subsequent analyses, the patients who had died of the disease were
excluded; thus, only the thirty-three surviving patients (twenty-two who had
endoprosthetic reconstruction and eleven who had biological reconstruction)
were included. The mean age of the patients was 10 ± 1 years (range,
six to eleven years) at the time of diagnosis and 22 ± 4 years (range,
fourteen to thirty years) at the time of the latest follow-up. All patients
were skeletally mature. There were thirteen male and twenty female patients
(ratio, 0.65). The mean duration of follow-up for the endoprosthetic
reconstruction group (13.2 ± 3.9 years; range, 5.9 to twenty years) was
similar to that of the biological reconstruction group (10.4 ± 4.4
years; range, six to 19.8 years) (p = 0.0760).
Metastases to the lungs occurred in two patients who were successfully
treated with resection of the metastases. Skip metastasis to the tibia
occurred in one patient, who then underwent an amputation.
Functional Assessment
The Musculoskeletal Tumor Society functional scores at the time of final
follow-up were available for all patients. The mean final functional score was
74% ± 18% in the endoprosthetic reconstruction group and 68% ±
17% in the biological reconstruction group (p = 0.3672). Furthermore, with the
numbers available, no differences in functional scores were detected with
regard to sex, surgical staging, or margins. The mean values for the six
functional categories in the endoprosthetic reconstruction group and the
biological reconstruction group were 4.7 ± 0.9 and 4.8 ± 0.4,
respectively, for pain; 3.3 ± 1.0 and 3.5 ± 1.4 for function;
3.2 ± 1.3 and 2.7 ± 1.7 for emotional acceptance; 4.0 ±
1.4 and 3.5 ± 1.5 for the use of supports; 3.8 ± 1.2 and 3.5
± 1.3 for gait; and 3.3 ± 1.3 and 2.3 ± 1.5 for the
ability to walk.
Limb-Length Discrepancy
Nineteen (58%) of the thirty-three living patients had a limb-lengthening
procedure. Of the remaining fourteen patients, eight preferred not to undergo
limb-lengthening, five needed an amputation, and one had a limb-length
discrepancy of <2 cm. Twelve patients who had a lengthening procedure were
in the endoprosthetic reconstruction group
(Fig. 2), and seven were in the
biological reconstruction group. Lengthening was performed with distraction
osteogenesis in thirteen patients, including one who received a vascularized
fibular graft, and endoprosthetic expansion was used in the remaining six
patients. One of the patients who had distraction osteogenesis also had an
endoprosthetic expansion. A total of sixteen procedures were performed in the
thirteen patients undergoing distraction osteogenesis, with a mean lengthening
of 9.3 ± 7.8 cm for each procedure. A total of eighteen procedures were
performed in the six patients who had endoprosthetic expansion, with a mean
lengthening of 1.5 ± 0.4 cm for each procedure. The mean interval from
the time of the initial surgery to the first lengthening procedure was 43
± 41 months in the distraction osteogenesis group and 37 ± 32
months in the endoprosthetic expansion group (p = 0.7265). At the time of the
final follow-up, a limb-length discrepancy of =2 cm was present in fifteen
of the patients who had lengthening and in eight others who had not had
lengthening. These twenty-three patients had a mean discrepancy of 5.5
± 3.3 cm (range, 2 to 15 cm). When limb-length discrepancy was assessed
on the basis of the type of lengthening procedure (distraction osteogenesis,
endoprosthetic expansion, or none), no significant differences were noted,
with the numbers available (Fig.
3).
Nine of the nineteen patients who had a lengthening procedure and six of
those who did not were treated with a shoe lift. The Musculoskeletal Tumor
Society functional scores were available for sixteen patients before the
limb-lengthening procedure. Importantly, the mean functional score increased
significantly to 81% ± 11% (range, 60% to 100%) after limb-lengthening
from 65% ± 21% (range, 23% to 100%) before the lengthening (p =
0.0016). Regression analysis showed a significant negative correlation between
functional score and a limb-length discrepancy of =2 cm (r = 0.53 and p =
0.0091) (Fig. 4).
Complications
Five early complications occurred in four patients, and twenty-eight late
complications occurred in twenty-two patients. In summary, one patient had
three complications, seven patients had two complications, and sixteen
patients had one complication each, while the remaining nine patients had no
complications. Early complications included skin necrosis, infection, vessel
occlusion, and peroneal nerve palsy, and late complications included
superficial and deep infection, implant breakage, aseptic loosening, fracture,
nonunion, and dislocation. Details of the complications are shown in
Table I. In the endoprosthetic
reconstruction group, the most frequent complication was deep infection (six
patients) followed by aseptic loosening (five patients), and implant breakage
and dislocation (two patients each). In the biological reconstruction group,
the most frequent complications were implant breakage and nonunion (three
patients each), followed by superficial infection, deep infection, and
fracture (two patients each).
A tendency toward higher functional scores was noted in the patients who
did not have complications (mean score, 77% ± 27%) compared with those
who did (mean score, 70% ± 13%); however, the difference did not reach
significance (p = 0.3596).
The cumulative event-free rate with regard to complications related to
surgery was calculated for the total series of patients. Complications had
occurred in 55% (95% confidence interval, 38% to 71%) of the patients at five
years, and the rate increased to 74% (95% confidence interval, 57% to 90%) at
ten years. Furthermore, the cumulative event-free rate in the two surgical
groups was determined (Fig. 5).
At five years, complications had occurred in 45% (95% confidence interval, 25%
to 66%) of the patients in the endoprosthetic reconstruction group and in 73%
(95% confidence interval, 46% to 99%) of those who had biological
reconstruction (p = 0.0472). At ten years, the complication rate had increased
to 69% (95% confidence interval, 47% to 91%) of the patients in the
endoprosthetic reconstruction group and to 86% (95% confidence interval, 63%
to 109%) of those in the biological reconstruction group (p = 0.0836).
Survival of Reconstruction Modes
With regard to the management of the complications, revision operations
were necessary in thirteen patients, and one of them eventually underwent
amputation. In addition, four other amputations were performed. The details of
the revisions and amputations for these patients are shown in the Appendix and
are summarized in relation to complications in
Table I. A total of seventeen
patients (52%) needed additional surgery because of complications of the
initial surgery.
The survival rate of the reconstructions in the two surgical groups was
estimated (Fig. 6). The
survival rate was 77% (95% confidence interval, 60% to 95%) at five years and
51% (95% confidence interval, 29% to 73%) at ten years for the endoprosthetic
reconstructions and 46% (95% confidence interval, 16% to 75%) at both five and
ten years for the biological reconstructions. Interestingly, during the first
six years, a tendency toward better survival was noted for the endoprosthetic
reconstructions, but it did not reach significance (p = 0.0646) and did not
persist during longer follow-up. The total success rate for limb salvage was
85%, with a success rate of 91% for the endoprosthetic reconstructions and 73%
for the biological reconstructions.
Chemotherapy has dramatically changed the cure rate of malignant bone
tumors5,19.
Since the introduction of effective neoadjuvant chemotherapy in the 1980s,
>80% of patients with osteosarcoma of the extremity have been considered
candidates for limb-salvage
surgery20. Previous
studies have found no difference in the long-term survival rate between
patients treated with amputation and those treated with limb-salvage of the
distal end of the
femur1,2.
In the present study, the ten-year cumulative survival rate was 82%. This
finding is comparable with that in the study by Schindler et
al.8, who reported
an overall survival rate of 78% in children after limb-salvage surgery of the
femur. Local recurrence was not observed in any patient in our series. This
finding was similar to that in the study by Schiller et
al.19, who
performed limb salvage of the distal end of the femur in five children who
were less than fourteen years old, and to that described by Wilkins and
Miller21 in a study
of thirty-one patients who were less than nineteen years old.
We studied forty children who were eleven years of age or less at the time
of diagnosis. The purpose of selecting patients of this age was to evaluate
the effect of growth for at least two years, as skeletal maturity is expected
by the age of sixteen years in boys and fourteen years in
girls9,10.
The children were selected to undergo limb salvage with one of two major
procedures, either endoprosthetic or biological reconstruction. Furthermore,
they had a relatively long duration of follow-up of six to twenty years. All
patients had reached skeletal maturity at the time of the last follow-up. To
our knowledge, this is the largest report of the long-term results of limb
salvage for the treatment of sarcomas of the distal end of the femur in
skeletally immature children.
Kenan and Lewis reported that limb-lengthening was necessary in twenty-one
patients who were twelve years old or less at the time of limb-salvage
surgery11. A
limb-length discrepancy of =2 cm has been considered substantial enough to
require corrective
surgery17.
Therapeutic options range widely from no treatment or the use of a shoe lift
to contralateral surgical shortening or a limb-lengthening
procedure14,22,23.
In the present study, limb-lengthening procedures were performed in nineteen
(58%) of the thirty-three surviving patients. Only one patient had a
discrepancy of <2 cm, five patients needed amputation, and the eight other
patients refused to undergo any lengthening procedure (six of them used a shoe
lift). Lengthening was achieved mainly with two procedures, distraction
osteogenesis and endoprosthetic expansion. In nearly half of these patients
(nine of the nineteen patients), an additional shoe lift was necessary, as a
considerable discrepancy remained. Importantly, we found that the functional
score was significantly higher following limb-lengthening. In addition, at the
time of the final follow-up, we found that, as the limb-length discrepancy
decreased, the functional score increased (see
Fig. 4). Even if this
relationship were significant, the functional score is clearly not only
dependent on limb-length discrepancy, as other factors that have not been
explored further in this study may contribute. However, we believe that
limb-length discrepancy is a rather important factor relating to a poor
functional outcome, and our findings support the concept that lengthening
procedures are required to obtain better function in these young patients.
The final functional scores in the endoprosthetic and biological
reconstructions were 74% and 68%, respectively, despite the frequent necessity
for additional operations, such as limb-lengthening procedures and revisions.
Function in the endoprosthetic group was comparable with that described in the
study by Schindler et al., in which the mean Musculoskeletal Tumor Society
functional score was 77% in twelve children with Stanmore custom-made
extendable distal femoral
prostheses8. Wilkins
and Miller21 found
mean functional scores of 73% in twenty-six patients with primary
limb-preservation surgery with reoperations and 79% in ten patients without
reoperations. With regard to the different categories of the Musculoskeletal
Tumor Society functional scoring system, pain received the highest score,
which indicated that the patients experienced little pain, whereas emotional
acceptance and the ability to walk received the lowest scores.
Deep infection was the most common complication, occurring in eight (24%)
of the thirty-three patients in our study. In the previous literature, the
rate of deep infection in the femur has ranged from 4% (one of twenty-four
patients) in the study by Cool et
al.24 to two of
five patients in the study by Schiller et
al.19. Comparable
results have been reported by other
investigators8,25,26.
Among the patients who had endoprosthetic reconstruction in our study, six had
a deep infection. Débridement and irrigation without removal of the
endoprosthesis was performed in two patients; staged reimplantation, in two
patients27; removal
of the endoprosthesis followed by
arthrodesis28, in
one patient; and
amputation8, in one
patient. Two of the patients in the biological reconstruction group had a deep
infection. One was treated with débridement alone, and the other
required amputation.
In the present study, five (23%) of twenty-two patients with endoprosthetic
replacement experienced aseptic loosening. Aseptic loosening has been reported
as a major problem, accounting for a complication rate of 13% (three of
twenty-four patients) in the series by Cool et
al.24 and up to six
of twelve patients in the study by Schindler et
al.8. Our results
are comparable with those in several other
reports19,24,29.
Unwin and Walker30
found that sixteen (15%) of 105 patients managed with a Stanmore custom-made
extendable endoprosthesis of the distal end of the femur had to have a
revision because of aseptic loosening. This rate is similar to that in our
study, in which revision surgery was required in three (14%) of twenty-two
patients.
Implant breakage was found in 15% (five) of our thirty-three patients. In
the endoprosthetic reconstruction group, two patients fell accidentally; one
had breakage of the femoral stem and the other had breakage of the femoral
ceramic component. Those types of prostheses are no longer used. In the
biological reconstruction group, three of four patients who had reconstruction
with a Küntscher nail had breakage. Breakage of the nail after
arthrodesis occurred in twenty (24%) of eighty-four patients in the series by
Donati et al.31 and
in four of thirty-nine patients in the series by Weiner et
al.32.
In the present study, revision surgery other than limb-lengthening was
necessary in seventeen (52%) of thirty-three patients; five of them eventually
needed amputation. In previous studies, revision rates have ranged from a
minimum of 21% (five of twenty-four patients in the series by Cool et
al.24) to 100% (all
twelve patients in the study by Schindler et
al.8). Comparable
results have been reported by other
investigators19,24,26,30,33.
After long-term follow-up in our series of patients, the survival rates for
endoprosthetic and biological reconstructions were similar. The survival rate
for endoprosthetic reconstruction was 77% at five years and 51% at ten years,
whereas the survival rate for biological reconstruction was 46% at both five
and ten years. Unwin and Walker reported that 105 patients with distal femoral
extendable prostheses had a probability of survival of 80% at four
years30. However,
long-term results were not reported in that study. Our long-term results are
comparable with those reported in studies that included all age-groups, such
as that by Kawai et al., who found a survival rate for endoprosthetic
reconstruction of 67% at five years and 48% at ten years in thirty-two
patients33.
There are few studies on the survival rate of biological reconstructions.
In a report on eighty-four patients who had knee arthrodesis with use of a
temporary spacer, Donati et
al.31 found that
the overall rate of implant survival was only 5% at twelve years, which was
low compared with our findings. It should be noted that the rate of mechanical
complications, such as implant breakage, bending, and migration, in that study
was very high (39%; thirty-three patients). Those complications occurred soon
after the initial surgery. Also, wound complications occurred in twenty-six
patients. Alman et
al.26 reported on
eight young patients with a tumor of the femur who were managed with knee
arthrodesis or an intercalary reconstruction of the femur; all except one had
failure of the reconstruction and needed a revision.
The use of arthrodesis has decreased after the introduction of expandable
endoprostheses26.
However, an arthrodesis may still be considered in patients with a large tumor
when an extensive resection of bone or soft tissue, such as complete resection
of the quadriceps muscle, is expected; when there is a lack of bone source; or
when there is a poor prognosis for the
patient31.
Furthermore, tumor resection with preservation of the epiphysis followed by
reconstruction with distraction osteogenesis is the only current technique
that has the advantage of preserving the natural joint, and thus it might be
considered for patients with a tumor of <15 cm in length and with at least
0.5 cm of the epiphysis remaining after removal of the
tumor14.
The rate of amputation in our study (five patients; 15%) is comparable with
that in previous
reports8,26.
In our series, the causes leading to the amputations varied. In the previous
literature, the major causes of amputation were infection or local
recurrence8,30,31.
In order to improve the success rate of limb-salvage surgery, every effort
should be made to avoid these two complications.
One limitation of this study is the relatively small number of patients in
each of the surgical reconstruction groups, and therefore any firm conclusion
about differences across the groups should be made with caution. The study
design represents another limitation, since this was a multicenter
retrospective study and no randomization protocol for the implementation of
surgical procedures was followed. Thus, tumor-related factors; the preferences
of the institution, the surgeon, or the patient; or other unexplored factors
might have biased the decision with regard to the type of surgery
performed.
We concluded that, even if additional revision and limb-lengthening
procedures are required, endoprosthetic or biological reconstructions provide
good functional outcome in skeletally immature children with a malignant bone
tumor of the distal end of the femur. However, the patients and their families
must be carefully selected with an emphasis on motivation and acceptance,
since rather frequent and substantial treatment during follow-up is inevitably
required. In the future, improvements in the surgical techniques and equipment
are needed to diminish the number of complications related to the initial
limb-salvage surgery.
A table presenting demographic data on the patients in the study is
available with the electronic versions of this article, on our web site at
(go to
the article citation and click on "Supplementary Material") and on
our quarterly CD-ROM (call our subscription department, at 781-449-9780, to
order the CD-ROM). ?
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