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
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 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:
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.
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 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.
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.
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.
We report our experience of treating 17 patients with benign lesions of the proximal femur with non-vascularised, autologous fibular strut grafts, without osteosynthesis. The mean age of the patients at presentation was 16.5 years (5 to 33) and they were followed up for a mean of 2.9 years (0.4 to 19.5). Histological diagnoses included simple bone cyst, fibrous dysplasia, aneurysmal bone cysts and giant cell tumour. Local recurrence occurred in two patients (11.7%) and superficial wound infection, chronic hip pain and deep venous thrombosis occurred in three. Pathological fracture did not occur in any patient following the procedure. We conclude that non-vascularised fibular strut grafts are a safe and satisfactory method of treating benign lesions of the proximal femur.
We investigated whether our policy of routine re-excision of the tumour bed after an unplanned excision of a soft-tissue sarcoma was justified. Between April 1982 and December 2005, 2201 patients were referred to our hospital with the diagnosis of soft-tissue sarcoma, of whom 402 (18%) had undergone an unplanned excision elsewhere. A total of 363 (16.5%) were included in this study. Each patient was routinely restaged and the original histology was reviewed. Re-excision was undertaken in 316 (87%). We analysed the patient, tumour and treatment factors in relation to local control, metastasis and overall survival. Residual tumour was found in 188 patients (59%). There was thus no residual disease in 128 patients of whom 10% (13) went on to develop a local recurrence. In 149 patients (47%), the re-excision specimen contained residual tumour, but it had been widely excised. Local recurrence occurred in 30 of these patients (20%). In 39 patients (12%), residual tumour was present in a marginal resection specimen. Of these, 46% (18) developed a local recurrence. A final positive margin in a high-grade tumour had a 60% risk of local recurrence even with post-operative radiotherapy. Metastases developed in 24% (86). The overall survival was 77% at five years. Survival was related to the grade of the tumour and the finding of residual tumour at the time of re-excision. We concluded that our policy of routine re-excision after unplanned excision of soft-tissue sarcoma was justified in view of the high risk of finding residual tumour.
Pathological fractures due to metastasis with destruction of the acetabulum and central dislocation of the hip present a difficult surgical challenge. We describe a series using a single technique in which a stable and long-lasting reconstruction was obtained using standard primary hip replacement implants augmented by strong, fully-threaded steel rods with cement and steel mesh, where required. Between 1997 and 2006, 19 patients with a mean age of 66 years (48 to 83) were treated using a modified Harrington technique. Acetabular destruction was graded as Harrington class II in six cases and class III in 13. Reconstruction was achieved using three 6.5 mm rods inserted through a separate incision in the iliac crest followed by augmentation with cement and a conventional cemented Charnley or Exeter primary hip replacement. There were no peri-operative deaths. At the final follow-up (mean 25 months (5 to 110)) one rod had fractured and one construct required revision. Of the 18 patients who did not require revision, 13 had died. The mean time to death was 16 months (5 to 55). The mean follow-up of the five survivors was 31 months (18 to 47). There were no cases of dislocation, deep infection or injury to a nerve, the blood vessels or the bladder.
We have investigated whether improvements in design have altered the outcome for patients undergoing endoprosthetic replacement of the proximal tibia following resection of a tumour. Survival of the implant and ‘servicing’ procedures have been documented using a prospective database. A total of 194 patients underwent a proximal tibial replacement, with 95 having a fixed-hinge design and 99 a rotating-hinge with a hydroxyapatite collar; their median age was 21.5 years (10 to 74). At a mean follow-up of 14.7 years (5 to 29), 115 patients remain alive. The risk of revision for any reason in the fixed-hinge group was 32% at five years, 61% at ten years and 75% at 15 and 20 years, and in the rotating-hinge group 12% at five years, 25% at ten years and 30% at 15 years. Aseptic loosening was the most common reason for revision in the fixed-hinge knees, fracture of the implant in the early design of rotating hinges and infection in the current version. The risk of revision for aseptic loosening in the fixed-hinge knees was 46% at ten years. This was reduced to 3% in the rotating-hinge knee with a hydroxyapatite collar. The cemented, rotating hinge design currently offers the best chance of long-term survival of the prosthesis.