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
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:
