While a centralized system for the care of patients with a sarcoma has been advocated for decades, regional variations in survival remain unclear. The aim of this study was to investigate regional variations in survival and the impact of national policies in patients with a soft-tissue sarcoma (STS) in the UK. The study included 1,775 patients with a STS who were referred to a tertiary sarcoma centre. The geographical variations in survival were evaluated according to the periods before and after the issue of guidance by the National Institute for Health and Care Excellence (NICE) in 2006 and the relevant evolution of regional management.Aims
Methods
Limb salvage for pelvic sarcomas involving the acetabulum is a major surgical challenge. There remains no consensus about what is the optimum type of reconstruction after resection of the tumour. The aim of this study was to evaluate the surgical outcomes in these patients according to the methods of periacetabular reconstruction. The study involved a consecutive series of 122 patients with a periacetabular bone sarcoma who underwent limb-salvage surgery involving a custom-made prosthesis in 65 (53%), an ice-cream cone prosthesis in 21 (17%), an extracorporeal irradiated autograft in 18 (15%), and nonskeletal reconstruction in 18 (15%).Aims
Methods
Urgent referral to a specialist centre for patients with a soft-tissue sarcoma (STS) has been recommended by the National Institute for Health and Care Excellence (NICE) in the UK since 2006. However, the impact of this recommendation on the prognosis for these patients remains unclear. We aimed to determine the impact of the NICE guidelines on the disease-specific survival (DSS) of patients with an STS. A total of 2,427 patients with an STS referred to a supraregional centre in the ten-year periods before (n = 1,386) and after (n = 1,041) the issue of the NICE guidelines were evaluated.Aims
Methods
The primary aim of this study was to determine the effect of
the duration of symptoms (DOS) prior to diagnosis on the overall
survival in patients with a primary bone sarcoma. In a retrospective analysis of a sarcoma database at a single
institution between 1990 and 2014, we identified 1446 patients with
non-metastatic and 346 with metastatic bone sarcoma. Low-grade types
of tumour were excluded. Our data included the demographics of the
patients, the characteristics of the tumour, and the survival outcome
of patients. Cox proportional hazards analysis and Kaplan–Meier
survival analysis were performed, and the survivorship of the non-metastatic
and metastatic cohorts were compared.Aims
Patients and Methods
The purpose of this study was to describe the effect of histological
grade on disease-specific survival in patients with chondrosarcoma. A total of 343 patients with a chondrosarcoma were included.
The histological grade was assessed on the initial biopsy and on
the resection specimen. Where the histology showed a mixed grade,
the highest grade was taken as the definitive grade. When only small
focal areas showed higher grade, the final grade was considered
as both.Aims
Patients and Methods
The use of a noninvasive growing endoprosthesis in the management
of primary bone tumours in children is well established. However,
the efficacy of such a prosthesis in those requiring a revision
procedure has yet to be established. The aim of this series was
to present our results using extendable prostheses for the revision
of previous endoprostheses. All patients who had a noninvasive growing endoprosthesis inserted
at the time of a revision procedure were identified from our database.
A total of 21 patients (seven female patients, 14 male) with a mean
age of 20.4 years (10 to 41) at the time of revision were included.
The indications for revision were mechanical failure, trauma or infection
with a residual leg-length discrepancy. The mean follow-up was 70
months (17 to 128). The mean shortening prior to revision was 44 mm
(10 to 100). Lengthening was performed in all but one patient with
a mean lengthening of 51 mm (5 to 140).Aims
Patients and Methods
The aim of this study was to analyse a group of patients with
non-metastatic Ewing’s sarcoma at presentation and identify prognostic
factors affecting the development of local recurrence, in order
to assess the role of radiotherapy. A retrospective review of all patients with a Ewing’s sarcoma
treated between 1980 and 2012 was carried out. Only those treated
with chemotherapy followed by surgery and/or radiotherapy were included.
Patients were grouped according to site (central or limb) for further
analysis of the prognostic factors.Aims
Patients and Methods
The aim of the study was to investigate the controversial issue
of whether the pelvic ring should be reconstructed following resection
of the sarcomas of the ilium. From our database, we identified 64 patients who underwent excision
of a tumour involving the ilium between 1976 and 2015. A total of
35 underwent complete resection, of whom 24 were reconstructed with
a non-vascularised fibula graft, and four with extracorporeal irradiation
and reimplantation. A total of 29 patients had a partial resection.
The mean follow-up was 9.2 years (1.1 to 25.6). Functional outcomes
were assessed using the Toronto Extremity Salvation Score (TESS)
at final follow-up. In all, 32 patients (50%) had a chondrosarcoma.Aims
Patients and Methods
The aim of this study was to identify any progression between
benign osteofibrous dysplasia (OFD), OFD-like adamantinoma and malignant
adamantinoma, and to investigate the rates of local recurrence,
metastases and survival, in order to develop treatment algorithms
for each. A single institution retrospective review of all patients presenting
with OFD, OFD-like adamantinoma and adamantinoma between 1973 and
2012 was undertaken. Complete data were available for 73 patients
(42 with OFD; ten with an OFD-like adamantinoma and 21 with an adamantinoma).
The mean follow-up was 10.3 years (3 to 25) for OFD, 9.2 years (3.0
to 26.3) for OFD-like and 11.6 years (0.25 to 33) for adamantinoma.Aims
Patients and Methods
Due to the complex anatomy of the pelvis, limb-sparing resections
of pelvic tumours achieving adequate surgical margins, can often
be difficult. The advent of computer navigation has improved the
precision of resection of these lesions, though there is little
evidence comparing resection with or without the assistance of navigation. Our aim was to evaluate the efficacy of navigation-assisted surgery
for the resection of pelvic bone tumours involving the posterior
ilium and sacrum. Using our prospectively updated institutional database, we conducted
a retrospective case control study of 21 patients who underwent
resection of the posterior ilium and sacrum, for the treatment of
a primary sarcoma of bone, between 1987 and 2015. The resection
was performed with the assistance of navigation in nine patients
and without navigation in 12. We assessed the accuracy of navigation-assisted
surgery, as defined by the surgical margin and how this affects
the rate of local recurrence, the disease-free survival and the
effects on peri-and post-operative morbidity. Aims
Patients and Methods
Extendible endoprostheses have been available for more than 30
years and have become more sophisticated with time. The latest generation
is ‘non-invasive’ and can be lengthened with an external magnetic
force. Early results have shown a worryingly high rate of complications
such as infection. This study investigates the incidence of complications
and the need for further surgery in a cohort of patients with a
non-invasive growing endoprosthesis. Between 2003 and June 2014, 50 children (51 prostheses) had a
non-invasive growing prosthesis implanted for a primary bone sarcoma.
The minimum follow-up was 24 months for those who survived. Their
mean age was 10.4 years (6 to 14). The incidence of complications
and further surgery was documented.Aims
Patients and Methods
The purpose of this study was to review a large cohort of patients
and further assess the correlation between the histological response
to chemotherapy in patients with Ewing’s sarcoma with the overall
(OS) and event-free survival (EFS). All patients treated for Ewing’s sarcoma between 1980 and 2012
were reviewed. Of these, 293 patients without metastases at the
time of diagnosis and treated with chemotherapy and surgery were
included. Patients were grouped according to the percentage of necrosis
after chemotherapy: Group I: 0% to 50%, Group II: 51% to 99% and Group
III: 100%.Aims
Patients and Methods
The aim of this study was to establish what happens to patients
in the long term after endoprosthetic replacement for a primary
malignant tumour of bone. We conducted a retrospective analysis of a prospectively maintained
database to identify all patients who had undergone an endoprosthetic
replacement more than 25 years ago and who were still alive. Their
outcomes were investigated with reference to their complications and
need for further surgery. A total of 230 patients were identified.
Their mean age at diagnosis was 20.7 years (five to 62). The most
common diagnosis was osteosarcoma (132). The most common site was
the distal femur (102). Aims
Patients and Methods
Osteosarcoma of the pelvis is a particularly difficult tumour
to treat as it often presents late, may be of considerable size
and/or associated with metastases when it presents, and is frequently
chondroid in origin and resistant to chemotherapy. The aim of this study was to review our experience of managing
this group of patients and to identify features predictive of a
poor outcome. Between 1983 and 2014, 121 patients, (74 females and 47 males)
were treated at a single hospital: 74 (61.2%) patients had a primary
osteosarcoma and 47 (38.8%) had an osteosarcoma which was secondary
either to Paget’s disease (22; 18.2%) or to previous pelvic irradiation
(25; 20.7%). The mean age of those with a primary osteosarcoma was 29.3 years
(nine to 76) and their mean follow-up 2.9 years (0 to 29). The mean
age of those with a secondary sarcoma was 61.9 years (15 to 85)
and their mean follow-up was one year (0 to 14). A total of 22 patients with a primary sarcoma (52.4%) and 20
of those with a secondary sarcoma (47.6%) had metastases at the
time of presentation. Aims
Patients and Methods
Patients who have limb amputation for musculoskeletal
tumours are a rare group of cancer survivors. This was a prospective
cross-sectional survey of patients from five specialist centres
for sarcoma surgery in England. Physical function, pain and quality
of life (QOL) outcomes were collected after lower extremity amputation
for bone or soft-tissue tumours to evaluate the survivorship experience
and inform service provision. Of 250 patients, 105 (42%) responded between September 2012 and
June 2013. From these, completed questionnaires were received from
100 patients with a mean age of 53.6 years (19 to 91). In total
60 (62%) were male and 37 (38%) were female (three not specified).
The diagnosis was primary bone sarcoma in 63 and soft-tissue tumour
in 37. A total of 20 tumours were located in the hip or pelvis,
31 above the knee, 32 between the knee and ankle and 17 in the ankle
or foot. In total 22 had hemipelvectomy, nine hip disarticulation,
35 transfemoral amputation, one knee disarticulation, 30 transtibial
amputation, two toe amputations and one rotationplasty. The Toronto
Extremity Salvage Score (TESS) differed by amputation level, with
poorer scores at higher levels (p <
0.001). Many reported significant
pain. In addition, TESS was negatively associated with increasing
age, and pain interference scores. QOL for Cancer Survivors was
significantly correlated with TESS (p <
0.001). This relationship appeared
driven by pain interference scores. This unprecedented national survey confirms amputation level
is linked to physical function, but not QOL or pain measures. Pain
and physical function significantly impact on QOL. These results
are helpful in managing the expectations of patients about treatment
and addressing their complex needs. Cite this article:
We conducted a case-control study to examine
the merit of silver-coated tumour prostheses. We reviewed 85 patients
with Agluna-treated (silver-coated) tumour implants treated between
2006 and 2011 and matched them with 85 control patients treated
between 2001 and 2011 with identical, but uncoated, tumour prostheses. In all, 106 men and 64 women with a mean age of 42.2 years (18.4
to 90.4) were included in the study. There were 50 primary reconstructions
(29.4%); 79 one-stage revisions (46.5%) and 41 two-stage revisions
for infection (24.1%). The overall post-operative infection rate of the silver-coated
group was 11.8% compared with 22.4% for the control group (p = 0.033,
chi-square test). A total of seven of the ten infected prostheses
in the silver-coated group were treated successfully with debridement,
antibiotics, and implant retention compared with only six of the
19 patients (31.6%) in the control group (p = 0.048, chi-square
test). Three patients in the silver-coated group (3.5%) and 13 controls
(15.3%) had chronic periprosthetic infection (p = 0.009, chi-square
test). The overall success rates in controlling infection by two-stage
revision in the silver-coated group was 85% (17/20) compared with
57.1% (12/21) in the control group (p = 0.05, chi-square test).
The Agluna-treated endoprostheses were associated with a lower rate
of early periprosthetic infection. These silver-treated implants
were particularly useful in two-stage revisions for infection and
in those patients with incidental positive cultures at the time
of implantation of the prosthesis. Debridement with antibiotic treatment and retention of the implant
appeared to be more successful with silver-coated implants. Cite this article:
A poor response to chemotherapy (≤ 90% necrosis)
for osteosarcomas leads to poorer survival and an increased risk of
local recurrence, particularly if there is a close margin of excision.
We evaluated whether amputation confers any survival benefit over
limb salvage surgery (LSS) with narrow margins in patients who respond
poorly to chemotherapy. We only analysed patients with an osteosarcoma of the limb, a
poor response to chemotherapy and close margins on LSS (marginal/intralesional)
or primary amputation: 360 patients (36 LSS (intralesional margins),
197 LSS (marginal margins) and 127 amputations) were included. Local
recurrence developed in 13 (36%) following LSS with intralesional
margins, and 39 (20%) following LSS with marginal margins. There
was no local recurrence in patients who underwent amputation. The
five-year survival for all patients was 41% (95% confidence interval
(CI) 35 to 46), but for those treated by LSS with marginal margins
was 46.2% (95% CI 38 to 53), 36.3% (95% CI 27 to 45) for those treated
by amputation, and 28% (95 CI 14 to 44) for those treated by LSS
with intralesional margins. Patients who had LSS and then developed
local recurrence as a first event had the same survival as those
who had primary amputation without local recurrence. Prophylactic
adjuvant radiotherapy was used in 40 patients but had no discernible
effect in preventing local recurrence. Although amputation offered better local control, it conferred
no clear survival benefit over LSS with marginal margins in these
patients with a poor overall prognosis. Cite this article:
The aim of this study was to evaluate the functional
and oncological outcome of extracorporeally irradiated autografts
used to reconstruct the pelvis after a P1/2 internal hemipelvectomy. The study included 18 patients with a primary malignant bone
tumour of the pelvis. There were 13 males and five females with
a mean age of 24.8 years (8 to 62). Of these, seven had an osteogenic
sarcoma, six a Ewing’s sarcoma, and five a chondrosarcoma. At a
mean follow-up of 51.6 months (4 to 185), nine patients had died
with metastatic disease while nine were free from disease. Local
recurrence occurred in three patients all of whom eventually died of
their disease. Deep infection occurred in three patients and required
removal of their graft in two while the third underwent a hindquarter
amputation for extensive flap necrosis. The mean Musculoskeletal Tumor Society functional score of the
16 patients who could be followed-up for at least 12 months was
77% (50 to 90). Those 15 patients who completed the Toronto Extremity
Salvage Score questionnaire had a mean score of 71% (53 to 85). Extracorporeal irradiation and re-implantation of bone is a valid
method of reconstruction after an internal hemipelvectomy. It has
an acceptable morbidity and a functional outcome that compares favourably
with other available reconstructive techniques. Cite this article:
The purpose of this study was to assess whether
the use of a joint-sparing technique such as curettage and grafting was
successful in eradicating giant cell tumours of the proximal femur,
or whether an alternative strategy was more appropriate. Between 1974 and 2012, 24 patients with a giant cell tumour of
the proximal femur were treated primarily at our hospital. Treatment
was either joint sparing or joint replacing. Joint-sparing treatment
was undertaken in ten patients by curettage with or without adjunctive
bone graft. Joint replacement was by total hip replacement in nine patients
and endoprosthetic replacement in five. All 11 patients who presented
with a pathological fracture were treated by replacement. Local recurrence occurred in five patients (21%): two were treated
by hip replacement, three by curettage and none with an endoprosthesis.
Of the ten patients treated initially by curettage, six had a successful
outcome without local recurrence and required no further surgery.
Three eventually needed a hip replacement for local recurrence and
one an endoprosthetic replacement for mechanical failure. Thus 18
patients had the affected joint replaced and only six (25%) retained
their native joint. Overall, 60% of patients without a pathological
fracture who were treated with curettage had a successful outcome. Cite this article:
We hypothesised that the use of computer navigation-assisted
surgery for pelvic and sacral tumours would reduce the risk of an
intralesional margin. We reviewed 31 patients (18 men and 13 women)
with a mean age of 52.9 years (13.5 to 77.2) in whom computer navigation-assisted
surgery had been carried out for a bone tumour of the pelvis or
sacrum. There were 23 primary malignant bone tumours, four metastatic
tumours and four locally advanced primary tumours of the rectum.
The registration error when using computer navigation was <
1 mm
in each case. There were no complications related to the navigation,
which allowed the preservation of sacral nerve roots (n = 13), resection
of otherwise inoperable disease (n = 4) and the avoidance of hindquarter
amputation (n = 3). The intralesional resection rate for primary
tumours of the pelvis and sacrum was 8.7% (n = 2): clear bone resection
margins were achieved in all cases. At a mean follow-up of 13.1
months (3 to 34) three patients (13%) had developed a local recurrence.
The mean time alive from diagnosis was 16.8 months (4 to 48). Computer navigation-assisted surgery is safe and has reduced
our intralesional resection rate for primary tumours of the pelvis
and sacrum. We recommend this technique as being worthy of further
consideration for this group of patients. Cite this article:
We evaluated the risk of late relapse and further
outcome in patients with soft-tissue sarcomas who were alive and event-free
more than five years after initial treatment. From our database
we identified 1912 patients with these pathologies treated between
1980 and 2006. Of these 1912 patients, 603 were alive and event-free
more than five years after initial treatment and we retrospectively
reviewed them. The mean age of this group was 48 years (4 to 94)
and 340 were men. The mean follow-up was 106 months (60 to 336).
Of the original cohort, 582 (97%) were alive at final follow-up.
The disease-specific survival was 96.4% (95% confidence interval
(CI) 94.4 to 98.3) at ten years and 92.9% (95% CI 89 to 96.8) at
15 years. The rate of late relapse was 6.3% (38 of 603). The ten-
and 15-year event-free rates were 93.2% (95% CI 90.8 to 95.7) and
86.1% (95% CI 80.2 to 92.1), respectively. Multivariate analysis
showed that tumour size and tumour grade remained independent predictors
of events. In spite of further treatment, 19 of the 38 patients
died of sarcoma. The three- and five-year survival rates after the
late relapse were 56.2% (95% CI 39.5 to 73.3) and 43.2% (95% CI
24.7 to 61.7), respectively, with a median survival time of 46 months.
Patients with soft-tissue sarcoma, especially if large, require
long-term follow-up, especially as they have moderate potential
to have their disease controlled. Cite this article:
The aim of this study was to determine whether
the level of circulating C-reactive protein (CRP) before treatment predicted
overall disease-specific survival and local tumour control in patients
with a sarcoma of bone. We retrospectively reviewed 318 patients who presented with a
primary sarcoma of bone between 2003 and 2010. Those who presented
with metastases and/or local recurrence were excluded. Elevated CRP levels were seen in 84 patients before treatment;
these patients had a poorer disease-specific survival (57% at five
years) than patients with a normal CRP (79% at five years) (p <
0.0001). They were also less likely to be free of recurrence (71%
at five years) than patients with a normal CRP (79% at five years)
(p = 0.04). Multivariate analysis showed the pre-operative CRP level
to be an independent predictor of survival and local control. Patients
with a Ewing’s sarcoma or chondrosarcoma who had an elevated CRP
before their treatment started had a significantly poorer disease-specific
survival than patients with a normal CRP (p = 0.02 and p <
0.0001, respectively).
Patients with a conventional osteosarcoma and a raised CRP were
at an increased risk of poorer local control. We recommend that CRP levels are measured routinely in patients
with a suspected sarcoma of bone as a further prognostic indicator
of survival. Cite this article:
A total of 157 hindquarter amputations were carried
out in our institution during the last 30 years. We have investigated
the reasons why this procedure is still required and the outcome.
This operation was used as treatment for 13% of all pelvic bone
sarcomas. It was curative in 140 and palliative in 17, usually to
relieve pain. There were 90 primary procedures (57%) with the remaining
67 following the failure of previous operations to control the disease
locally. The indication for amputation in primary disease was for
large tumours for which limb-salvage surgery was no longer feasible.
The peri-operative mortality was 1.3% (n = 2) and major complications
of wound healing or infection arose in 71 (45%) patients. The survival
at five years after hindquarter amputation with the intent to cure
was 45%, and at ten years 38%. Local recurrence occurred in 23 patients
(15%). Phantom pain was a significant problem, and only 20% used
their prosthesis regularly. Functional scores were a mean of 57%.
With careful patient selection the oncological results and functional outcomes
of hindquarter amputation justify its continued use. Cite this article:
The purpose of this study was to assess the outcome
of 15 patients (mean age 13.6 years (7 to 25)) with a primary sarcoma
of the tibial diaphysis who had undergone excision of the affected
segment that was then irradiated (90 Gy) and reimplanted with an
ipsilateral vascularised fibular graft within it. The mean follow-up was 57 months (22 to 99). The mean time to
full weight-bearing was 23 weeks (9 to 57) and to complete radiological
union 42.1 weeks (33 to 55). Of the 15 patients, seven required
a further operation, four to obtain skin cover. The mean Musculoskeletal
Society Tumor Society functional score at final follow-up was 27
out of 30 once union was complete. The functional results were comparable
with those of allograft reconstruction and had a similar rate of
complication. We believe this to be a satisfactory method of biological reconstruction
of the tibial diaphysis in selected patients.
Amputation was once widely practised for primary bone tumours of the limbs. Yet this situation has changed with limb salvage surgery becoming increasingly popular in the last 30 years. Many different techniques are now available. These include allografts, autografts, endoprostheses and allograft-prosthesis composites. This article reviews these methods, concentrating on the functional outcomes and complications that have been reported.
Since1986 we have monitored the actual diagnosis of all cases referred to our Unit with the diagnosis of ‘possible primary malignant bone tumour’. We have excluded all patients referred with a known diagnosis of either a benign condition or known to have bone metastases. In most cases the suspected diagnosis was based on X-rays alone, sometimes supported by further imaging. Retrospective review of a prospective database that was started in 1986 identifying the actual diagnosis. There were 5922 patients with a confirmed diagnosis over the 23 year time period of this study. 2205 (37%) were found to have a primary malignant bone sarcoma and 1309 (22%) had a benign bone tumour. 992 patients had a general orthopaedic condition (e.g. geode or a vascular necrosis) whilst 303 (5%) had a haematological malignancy and 289 (4.9%) infection. 533 patients (9%) had metastases. There was a similar pattern of frequency of all diagnoses except for metastatic disease and haematologic malignancy at different ages. The incidence of metastases increased from the age of 35 onwardsMethod
Results
We reviewed our initial seven-year experience
with a non-invasive extendible prosthesis in 34 children with primary bone
tumours. The distal femur was replaced in 25 cases, total femur
in five, proximal femur in one and proximal tibia in three. The
mean follow-up was 44 months (15 to 86) and 27 patients (79%) remain
alive. The prostheses were lengthened by an electromagnetic induction
mechanism in an outpatient setting and a mean extension of 32 mm
(4 to 80) was achieved without anaesthesia. There were lengthening
complications in two children: failed lengthening in one and the
formation of scar tissue in the other. Deep infection developed
in six patients (18%) and local recurrence in three. A total of
11 patients required further surgery to the leg. Amputation was
necessary in five patients (20%) and a two-stage revision in another.
There were no cases of loosening, but two patients had implant breakage
and required revision. The mean Musculoskeletal Tumor Society functional
score was 85% (60% to 100%) at last known follow-up. These early
results demonstrate that the non-invasive extendible prosthesis
allows successful lengthening without surgical intervention, but
the high incidence of infection is a cause for concern.
We retrospectively compared the outcome after
the treatment of giant cell tumours of bone either with curettage alone
or with adjuvant cementation. Between 1975 and 2008, 330 patients
with a giant cell tumour were treated primarily by intralesional
curettage, with 84 (25%) receiving adjuvant bone cement in the cavity.
The local recurrence rate for curettage alone was 29.7% (73 of 246)
compared with 14.3% (12 of 84) for curettage and cementation (p
= 0.001). On multivariate analysis both the stage of disease and
use of cement were independent significant factors associated with
local recurrence. The use of cement was associated with a higher
risk of the subsequent need for joint replacement. In patients without
local recurrence, 18.1% (13 of 72) of those with cement needed a
subsequent joint replacement compared to 2.3% (4 of 173) of those
without cement (p = 0.001). In patients who developed local recurrence,
75.0% (9 of 12) of those with previous cementation required a joint
replacement, compared with 45.2% (33 of 73) of those without cement
(p = 0.044).
We aimed to identify the incidence, outcome and prognostic factors associated with spindle cell sarcomas of bone (SCSB). We studied 196 patients with a primary non-metastatic tumour treated with the intent to cure. The results were compared with those of osteosarcoma patients treated at our hospital during the same period. The overall incidence of SCSB was 7.8% of all patients with a primary bone sarcoma. The five- and ten-year survival rates were 67.0% and 60.0%, respectively, which were better than those of patients with osteosarcoma treated over the same period. All histological subtypes had similar outcomes. On univariate analysis, factors that were significantly associated with decreased survival were age >
40 years, size >
8 cm, the presence of a pathological fracture, amputation, involved margins and a poor response to pre-operative chemotherapy. Multivariate analyses showed that age >
65 years, amputation and involved margins were all statistically significant prognostic factors. Involved margins and poor response to pre-operative chemotherapy were associated with an increased risk of local recurrence. SCSB has a better prognosis than osteosarcoma when matched for age. Most prognostic factors for osteosarcoma also seem to apply to SCSB. Patients with SCSB should be treated in the same way as patients of the same age with osteosarcoma.
We investigated the eventual diagnosis in patients referred to a tertiary centre with a possible diagnosis of a primary bone malignancy. We reviewed our database from between 1986 and 2010, during which time 5922 patients referred with a suspicious bone lesion had a confirmed diagnosis. This included bone sarcoma in 2205 patients (37%), benign bone tumour in 1309 (22%), orthopaedic conditions in 992 (17%), metastatic disease in 533 (9%), infection in 289 (5%) and haematological disease in 303 (5%). There was a similar frequency of all diagnoses at different ages except for metastatic disease. Only 0.6% of patients (17 of 2913) under the age of 35 years had metastatic disease compared with 17.1% (516 of 3009) of those over 35 years (p <
0.0001). Of the 17 patients under 35 years with metastatic disease, only four presented with an isolated lesion, had no past history of cancer and were systematically well. Patients under the age of 35 years should have suitable focal imaging (plain radiography, CT or MRI) and simple systemic studies (blood tests and chest radiography). Reduction of the time to biopsy can be achieved by avoiding an unnecessary investigation for a primary tumour to rule out metastatic disease.
Endoprosthetic replacement of the pelvis is one of the most challenging types of limb-salvage surgery, with a high rate of complications. In an attempt to reduce this and build greater versatility into the reconstruction process, a new type of pelvic endoprosthesis was developed in 2003, based on the old McKee-Farrar prosthesis. This study reviews the outcomes in 27 patients who had an ice-cream cone pelvic prosthesis inserted at two different specialist bone tumour centres in the United Kingdom over the past six years. The indications for treatment included primary bone tumours in 19 patients and metastatic disease in two, and six implants were inserted following failure of a previous pelvic reconstruction. Most of the patients had a P2+P3 resection as classified by Enneking, and most had resection of the ilium above the sciatic notch. The mean age of the patients at operation was 49 years (13 to 81). Complications occurred in ten patients (37.0%), of which dislocation was the most common, affecting four patients (14.8%). A total of three patients (11.1%) developed a deep infection around the prosthesis but all were successfully controlled by early intervention and two patients (7.4%) developed a local recurrence, at the same time as widespread metastases appeared. In one patient the prosthesis was removed for severe pain. This method of treatment is still associated with high morbidity, but early results are promising. Complications are diminishing with increasing experience.
We report a case of spontaneous physeal growth arrest of the distal femur in a nine-year-old child with Ewing’s sarcoma of the proximal femur treated with chemotherapy and endoprosthetic replacement. Owing to the extent of disuse osteoporosis at the time of surgery, the entire intramedullary canal up to the distal femoral physis was filled with cement. Three years later, the femur remained at its pre-operative length of 19 cm. Pre-operative calculations of further growth failed to account for the growth arrest, and the initial expandable growing prosthesis inserted has been revised to a longer one in order to address the leg-length discrepancy. To our knowledge, this is the only reported case of distal femoral physeal growth arrest following cemented endoprosthetic replacement of the proximal femur.
The aim of this study was to identify whether there was any difference in patient, tumour, treatment or outcome characteristics between patients with skeletal or extra-skeletal Ewing’s sarcoma. We identified 300 patients with new primary Ewing’s sarcoma diagnosed between 1980 and 2005 from the centres’ local database. There were 253 (84%) with skeletal and 47 (16%) with extra-skeletal Ewing’s sarcomas. Although patients with skeletal Ewing’s were younger (mean age 16.8 years) than those with extra-skeletal Ewing’s sarcoma (mean age 27.5 years), there was little difference between the groups in terms of tumour stage or treatment. Nearly all the patients were treated with chemotherapy and most had surgery. There was no difference in the overall survival of patients with skeletal (64%) and extra-skeletal Ewing’s sarcoma (61%) (p = 0.85), and this was also the case when both groups were split by whether they had metastases or not. This large series has shown that the oncological outcomes of Ewing’s sarcoma are related to tumour characteristics and patient age, and not determined by whether they arise in bone or soft tissue.
Of 3000 patients diagnosed with primary malignant bone tumours and treated at our unit over the past 25 years, 234 (7.8%) were considered to be spindle cell sarcomas of bone (ie not osteosarcoma, chondrosarcoma, Ewing’s, chordoma or adamantinoma). We have analyzed their management and outcomes. The diagnosis of these cases varied with fluctuations in the popularity of conditions such as MFH, fibrosarcoma and leiomyosarcoma with the passage of time. Treatment was with chemotherapy and surgery whenever possible. 36 patients had metastases at diagnosis and 17 had palliative treatment only because of age or infirmity. The most common site was the femur followed by the tibia, pelvis and humerus. The mean age was 45 and the mean tumour size 10.2cm at diagnosis. 25% of patients presented with a pathological fracture. Chemotherapy was used in 70% of patients the most common regime being cisplatin and doxorubicin. 35% of patients having neoadjuvant chemotherapy had a good (>
90% necrosis) response. The amputation rate was 22% and was higher in patients presenting with a fracture and in older patients not having chemotherapy. With a mean follow up of 8 years the overall survival was 64% at 5 yrs and 58% at 10 yrs. Adverse prognostic factors included the need for amputation, older age and poor response to chemotherapy as well as a pathological fracture at presentation. The few patients with angiosarcoma fared badly but there was no difference in outcomes between patients with other diagnoses. We conclude that patients with spindle cell sarcomas should be treated similarly to patients with osteosarcoma and can expect comparable outcomes. The histological diagnosis does not appear to predict behaviour.
The two week wait has been established as a potential means of diagnosing malignant tumors earlier and thus hopefully leading to improvements in outcome. There remains controversy as to whether these clinic achieve this end or whether they just speed up treatment of patients already diagnosed (eg by imaging)
Endoprosthetic replacement of the pelvis is one of the most challenging types of limb salvage surgery with a high rate of complications. In order to try and decrease the high risk of complications and to allow greater versatility in the reconstruction options, a new concept of pelvic endoprosthesis was developed in 2003. Since then 20 of these ice cream cone pelvic prostheses have been inserted at our centre incorporating antibiotic laden cement around the prosthesis to minimize infection risk.
Low grade central osteosarcoma is a rare intramedullary bone producing tumour. It accounts for only 1–2% of all osteosarcomas. Due to the indolent nature of low grade central osteosarcoma, achieving a correct and prompt diagnosis is the real challenge both from imaging and histology, particularly as it may resemble a benign condition, i.e. Fibrous Dysplasia. We have reviewed 15 cases of low grade central osteosarcoma with long term follow-up (2 to 22 years) to identify problems in diagnosis and treatment and to assess outcome. There were 7 females and 8 males with a mean age of 37 yrs (range 11 to 72 years); 13 cases arose in the lower limb (8 femur, 4 tibia, 1 os calcis), 1 in the pelvis and 1 in the upper limb. The average duration of symptoms prior to presentation was over 2yrs. A primary diagnosis of low grade central osteosarcoma was achieved for only 6 cases (4 open and 2 needle biopsies), in the other 9 the primary diagnoses were GCT, cystic lesion or fibrous lesion (both benign and malignant) and all of them had undergone treatment (usually curettage with or without bone grafting for this). Definitive treatment was with surgery attempting to obtain wide margins. Marginal excision was associated with local recurrence in three cases but there were no local recurrences in patients who had a wide excision, even in those with prior treatment. Only one patient has died following the development of multiple metastases after 9 years. The survival rate is 90% at 15 years. We present this study to show the difficulties in diagnosing this rare type of osteosarcoma and to highlight the importance of wide surgical margins to obtain local control.
LR arose in 11 patients (17.5%) at a mean of 38 months and was related to surgical margins. There were no LR in the 26 wide resections, but the risk of LR was 20% in those with a marginal and 46% in those with an intralesional excision. LR appeared as a higher grade than the primary tumor in 3 patients (27%). 9 (14.3%) patients developed metastases at a median of 22 months (6 – 123). Five patients developed both LR and metastases. Seven patients died from the tumor, 2 patients are alive with metastatic disease. None of the patients with a low grade tumor and wide or marginal margins died of tumor, but 2 patients (18%) operated intralesional died of tumor.
Although bone and soft-tissue sarcomas are rare, early diagnosis and prompt referral to a specialised unit offers the best chance of a successful outcome both in terms of survival and surgical resection. This paper highlights the clinical and radiological features that might suggest the possibility of a bone or soft-tissue sarcoma and suggests a succinct management pathway for establishing whether a suspicious bone or soft-tissue lesion could be malignant.
Ewing’s sarcoma principally arises in bone but can also present as a soft tissue tumour. Very few studies have assessed the outcomes of extra-skeletal Ewing’s sarcomas. This study compares the oncological outcomes of the two forms of Ewing’s sarcomas to see if there is any difference in prognostic factors. 198 patients with primary, non metastatic Ewing’s sarcoma diagnosed between 1980 and 2005 were identified from our database. There were 118 males and 80 females with a median age of 15 years. The three most common sites of diagnosis were the femur (24%), pelvis (15%) and tibia (13%). There were 169(85%) bony Ewing’s and 29 (15%) extra-skeletal Ewing’s sarcomas. All patients received chemotherapy. 86% of the patients had surgery for local control but 28(14%) patients had radiotherapy. The overall survival at five years was 89% and was related to the age of patient (92% <
16years p=0.005), size (p=0.03) and site of tumour (p=0.004) as well as the response to chemotherapy. There was no difference in the overall survival of patients with bony Ewing’s (90%) and extra-skeletal Ewing’s (85%) (p=0.85). There was a 10% risk of local recurrence at 5 years with site of tumour (p=0.01) and surgical excision (p=0.05) being significant prognostic factors. The risk of local recurrence was also not related to the type of Ewing’s sarcoma. This large series has shown that the oncological outcomes of Ewing’s sarcoma is related to tumour characteristics, patient age and treatment factors and not determined by the tissue component.
Of the 7242 patients with soft tissue lumps, 476 had a past history of malignancy. Of these patients, only 12% actually had a soft tissue metastasis while 28% had a benign diagnosis, 55% a soft tissue sarcoma and 5% other malignancy.
We identified eight patients of 2900 with a primary malignant bone tumour who had coexisting neurofibromatosis type 1. This was a much higher incidence than would be expected by chance. The patients had a mean age of 22.4 years (9 to 54): five were male. Two patients subsequently developed a second bone sarcoma, one of which was radiation induced. Four of the primary tumours were osteosarcomas, four were spindle-cell sarcomas and one a Ewing’s sarcoma. All the patients were treated with chemotherapy and surgery: six of the eight appear to be cured. This study suggests a possible relationship between neurofibromatosis type 1 and the development of a bone sarcoma, the increased risk being estimated at eight times that of the normal population. We recommend that further research into this possible link should be considered.
In our database of 7935 patients referred for investigation of a soft-tissue mass, only 100 were found to have a soft-tissue metastasis (1.3%). Our aim was to define the clinical features of such patients and to identify the site of their primary tumour. The most common presentation was a painful lump, deep to the fascia, ranging between 2 cm and 35 cm (mean 8.3 cm) with 78% of the lumps located deep to the fascia. The mean age of the patients at presentation was 64 years (22 to 84) and there were almost equal numbers of men and women. Of 53 patients with a history of malignancy, 52 had metastases from the same primary (lung in 12, melanoma in ten, kidney in nine, gastrointestinal track in four, breast in five, bladder in four, and others in eight). The other 47 had no history of malignancy and the metastasis was the first presentation. The primary sites in these cases were the lung in 19, gastro-intestinal track in four, kidney in two, melanoma in nine, other in three, and unknown (despite investigations) in ten. There was no correlation between the site of the metastases and the primary tumour. Of the 7935 patients, 516 had a history of malignancy. Of these, only 10% had a soft-tissue metastasis, 29% had a benign diagnosis, 55% a soft-tissue sarcoma and 6% another malignancy. Patients with soft-tissue metastases have similar clinical features to those with soft-tissue sarcomas and should be considered for assessment at appropriate diagnostic centres for patients with suspicious soft-tissue lumps.
We undertook a cemental unipolar proximal femoral endoprosthetic replacement in 131 patients with a mean age of 50 years (2 to 84). Primary malignant tumours were present in 54 patients and 67 had metastatic disease. In addition, eight patients had either lymphoma or myeloma and two had non-oncological disorders. The mean follow-up was 27 months (0 to 180). An acetabular revision was required later in 14 patients, 12 of whom had been under the age of 21 years at the time of insertion of their original prosthesis. The risk of acetabular revision in patients over 21 years of age was 8% at five years compared with 36% in those aged under 21 years. All the unipolar hips in this younger age group required revision within 11 years of the initial operation. We conclude that unipolar replacement should not be used in younger patients and should be avoided in patients with a life expectancy of more than five years.