Intra-articular (IA) tumours around the knee are treated with extra-articular (EA) resection, which is associated with poor functional outcomes. We aim to evaluate the accuracy of MRI in predicting IA involvement around the knee. We identified 63 cases of high-grade sarcomas in or around the distal femur that underwent an EA resection from a prospectively maintained database (January 1996 to April 2020). Suspicion of IA disease was noted in 52 cases, six had IA pathological fracture, two had an effusion, two had prior surgical intervention (curettage/IA intervention), and one had an osseous metastasis in the proximal tibia. To ascertain validity, two musculoskeletal radiologists (R1, R2) reviewed the preoperative imaging (MRI) of 63 consecutive cases on two occasions six weeks apart. The radiological criteria for IA disease comprised evidence of tumour extension within the suprapatellar pouch, intercondylar notch, extension along medial/lateral retinaculum, and presence of IA fracture. The radiological predictions were then confirmed with the final histopathology of the resected specimens.Aims
Methods
Proximal femoral endoprosthetic replacements (PFEPRs) are the most common reconstruction option for osseous defects following primary and metastatic tumour resection. This study aimed to compare the rate of implant failure between PFEPRs with monopolar and bipolar hemiarthroplasties and acetabular arthroplasties, and determine the optimum articulation for revision PFEPRs. This is a retrospective review of 233 patients who underwent PFEPR. The mean age was 54.7 years (SD 18.2), and 99 (42.5%) were male. There were 90 patients with primary bone tumours (38.6%), 122 with metastatic bone disease (52.4%), and 21 with haematological malignancy (9.0%). A total of 128 patients had monopolar (54.9%), 74 had bipolar hemiarthroplasty heads (31.8%), and 31 underwent acetabular arthroplasty (13.3%).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
The aim of this study was to determine the risk of local recurrence and survival in patients with osteosarcoma based on the proximity of the tumour to the major vessels. A total of 226 patients with high-grade non-metastatic osteosarcoma in the limbs were investigated. Median age at diagnosis was 15 years (4 to 67) with the ratio of male to female patients being 1.5:1. The most common site of the tumour was the femur (n = 103) followed by tibia (n = 66). The vascular proximity was categorized based on the preoperative MRI after neoadjuvant chemotherapy into four types: type 1 > 5 mm; type 2 ≤ 5 mm, > 0 mm; type 3 attached; type 4 surrounded.Aims
Patients and Methods
The aim of this study was to evaluate the prosthesis characteristics and associated conditions that may modify the survival of total femoral endoprosthetic replacements (TFEPR). In all, 81 patients treated with TFEPR from 1976 to 2017 were retrospectively evaluated and failures were categorized according to the Henderson classification. There were 38 female patients (47%) and 43 male patients (53%) with a mean age at diagnosis of 43 years (12 to 86). The mean follow-up time was 10.3 years (0 to 31.7). A survival analysis was performed followed by univariate and multivariate Cox regression to identify independent implant survival factors.Aims
Patients and Methods
The aim of this study was to investigate the local recurrence rate at an extended follow-up in patients following navigated resection of primary pelvic and sacral tumours. This prospective cohort study comprised 23 consecutive patients (nine female, 14 male) who underwent resection of a primary pelvic or sacral tumour, using computer navigation, between 2010 and 2012. The mean age of the patients at the time of presentation was 51 years (10 to 77). The rates of local recurrence and mortality were calculated using the Kaplan–Meier method.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
Following the resection of an extensive amount of bone in the
treatment of a tumour, the residual segment may be insufficient
to accept a standard length intramedullary cemented stem. Short-stemmed
endoprostheses conceivably have an increased risk of aseptic loosening.
Extra-cortical plates have been added to minimise this risk by supplementing
fixation. The aim of this study was to investigate the survivorship
of short-stemmed endoprostheses and extra-cortical plates. The study involved 37 patients who underwent limb salvage surgery
for a primary neoplasm of bone between 1998 and 2013. Endoprosthetic
replacement involved the proximal humerus in nine, the proximal
femur in nine, the distal femur in 13 and the proximal tibia in
six patients. There were 12 primary (32%) and 25 revision procedures (68%).
Implant survivorship was compared with matched controls. The amount
of bone that was resected was >
70% of its length and statistically
greater than the standard control group at each anatomical site.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
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
The aim of this study was to evaluate the prognostic
and therapeutic factors which influence the oncological outcome
of parosteal osteosarcoma. A total of 80 patients with a primary parosteal osteosarcoma
were included in this retrospective study. There were 51 females
and 29 males with a mean age of 29.9 years (11 to 78). The mean follow-up was 11.2 years (1 to 40). Overall survival
was 91.8% at five years and 87.8% at ten years. Local recurrence
occurred in 14 (17.5%) patients and was associated with intralesional
surgery and a large volume of tumour. On histological examination,
80% of the local recurrences were dedifferentiated high-grade tumours.
A total of 12 (14.8%) patients developed pulmonary metastases, of
whom half had either a dedifferentiated tumour or a local recurrence.
Female gender and young age were good prognostic factors. Local
recurrence was a poor prognostic factor for survival. Medullary
involvement or the use of chemotherapy had no impact on survival. The main goal in treating a parosteal osteosarcoma must be to
achieve a wide surgical margin, as inadequate margins are associated
with local recurrence. Local recurrence has a significant negative
effect on survival, as 80% of the local recurrences are high-grade
dedifferentiated tumours, and half of these patients develop metastases. The role of chemotherapy in the treatment of parosteal osteosarcoma
is not as obvious as it is in the treatment of conventional osteosarcoma.
The mainstay of treatment is wide local excision. Cite this article:
The purpose of this study was to evaluate the
long-term outcome of patients with a sacral chordoma and the surgical
management of locally recurrent disease. Between October 1990 and August 2013 we operated on 54 consecutive
patients with a sacral chordoma. There were 34 men and 20 women
with a mean age of 60 years (25 to 86). The mean maximum diameter
of the tumour was 9.3 cm (3 to 20). The mean follow-up was 7.8 years (2 months to 23.4 years). The
disease-specific survival was 82% at five years, 57% at ten years
and 45% at 15 years. The local recurrence-free survival was 49%
at five years, 37% at ten years and 20% at 15 years. Local recurrence
occurred in 30 patients (56%) at a mean of 3.8 years (3 months to
13 years) post-operatively. Survival after the treatment of recurrence was 89% at two years,
56% at five years and 19% at ten years. Of nine patients who had
complete resection of a recurrence, one died after 72 months and
eight remain disease-free. Incomplete resection of recurrent disease
resulted in a survival of 54% at two years and 36% at five years. For 12 patients with a local recurrence who were treated palliatively,
survival was 81% at two years and 31% at five years. A wide margin of resection gave the best chance of long-term
survival and complete resection of local recurrence the best chance
of control of disease. 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:
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:
Bone sarcomas are rare cancers and orthopaedic
surgeons come across them infrequently, sometimes unexpectedly during
surgical procedures. We investigated the outcomes of patients who
underwent a surgical procedure where sarcomas were found unexpectedly
and were subsequently referred to our unit for treatment. We identified
95 patients (44 intra-lesional excisions, 35 fracture fixations,
16 joint replacements) with mean age of 48 years (11 to 83); 60%
were males (n = 57). Local recurrence arose in 40% who underwent
limb salvage surgery Cite this article:
We reviewed the outcome of patients who had been
treated operatively for symptomatic peri-acetabular metastases and
present an algorithm to guide treatment. The records of 81 patients who had been treated operatively for
symptomatic peri-acetabular metastases between 1987 and 2010 were
identified. There were 27 men and 54 women with a mean age of 61
years (15 to 87). The diagnosis, size of lesion, degree of pelvic
continuity, type of reconstruction, World Health Organization performance
status, survival time, pain, mobility and complications including
implant failure were recorded in each case. The overall patient survivorship at five years was 5%. The longest
lived patient survived 16 years from the date of diagnosis. The
mean survival was 23 months (<
1 to 16 years) and the median
was 15 months. At follow-up 14 patients remained alive. Two cementoplasties
failed because of local disease progression. Three Harrington rods broke:
one patient needed a subsequent Girdlestone procedure. One ‘ice-cream
cone’ prosthesis dislocated and was subsequently revised without
further problems. We recommend the ‘ice-cream cone’ for pelvic discontinuity
and Harrington rod reconstruction for severe bone loss. Smaller
defects can be safely managed using standard revision hip 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.
To estimate the risk of bone malignancy arising in premalignant conditions. There are quite a number of possible premalignant conditions with considerable uncertainty about the actual risk of a bone sarcoma developing. The incidence of these malignant conditions was identified from a prospective database containing 3000 primary bone sarcomas.Aim
Methods
The role of adjuvants in curettage for giant cell tumours (GCT) is still controversial. Our aim was to determine if adjuvant cementation lowers local recurrence (LR) rates for GCTs treated with curettage. Detailed curettage has been the principal treatment for GCT for the past 30 years. Cement was used from 1996 onwards for tumours where there was concern about structural stability. We investigated factors affecting LR and also the incidence of complications for treatment with or without cement.Introduction
Methods
To investigate the effectiveness of silver coated prostheses in preventing periprosthetic infection in a high-risk group. We have used silver coated prostheses in 48 endoprosthetic replacements in whom there would be a high expected risk of infection.Aim
Methods
It is generally accepted that there is a high rate of local recurrence following surgical excision of chordoma of the sacrum, even if the margins of excision appear clear. There is uncertainty as to whether the addition of postoperative radiotherapy may decrease the risk of local recurrence, particularly if there are close or involved margins. We aimed to determine the effect of conventional radiotherapy, in the post-operative setting, on the effect of local recurrence, metastases and patient survival in a multi-centre study. 57 patients were identified from the combined databases of the RNOH and ROH, who underwent surgical excision of a primary sacral chordoma and who had a minimum of three years follow-up. There were 17 women and 40 men, with a median age of 64 (25-81 range). Median tumour length was 10cm (2-20 range). 22 of the 57 patients died. Survival was 60% at 5 years and 45% at ten years. 28 of the 57 patients developed local recurrence (49%) and margins of excision did not affect the rates of local recurrence. Adjuvant radiotherapy was used in 9 patients, to with intra-lesional resections, five marginal and two wide. Without radiotherapy the local recurrence rate was 45% at five years. With radiotherapy, the local recurrence rate was 62.5% at five years. There were no statistically significant differences between the two.Methods
Results
The aim of this study is to evaluate the functional and oncological outcome of extracorporeally irradiated autografts as a method of pelvic reconstruction after internal hemipelvectomy. The study included fifteen patients with primary malignant bone tumours of the pelvis. There were 10 males and 5 females with a mean age of 21.5 years (range, 8 to 46 years). Six patients had Ewing's sarcoma, six osteosarcoma, and three chondrosarcoma.Introduction
Methods
Osteosarcomas represent a heterogeneous group of primary bone tumours that affect predominantly the long bones of patients in the first two decades of life. We aim to describe the secondary effects of a poor response (⋋90% necrosis) to chemotherapy on the effectivity of other treatment outcomes, local recurrence and survival rates. 182 cases of osteosarcoma with necrosis of less than 90% and no metastases at diagnosis have been seen at our institution over 24 years. There were 60 amputations. 122 patients underwent limb salvage, with 105 marginal margins and 17 contaminated. There was no difference in size or location between the two groups. In the 122 patients with LSS, 21 had adjuvant radiotherapy and 101 did not. In the entirety of patients with ⋋90% necrosis, survival was 64% at 2 years and 37% at 5 years. When LSS Marginal resections were compared with amputation there was a significant (P=0.006) difference in survival. LSS with a marginal margin had a 25% risk of LR. In these patients there was 25% survival, whereas the absence of a local recurrence, conferred a benefit of a 40% survival XRT was used in 21 of the 122 who underwent limb salvage. The decision to use XRT was made by the local oncologist at the treating unit. There was a 24% rate of recurrence in the XRT group and 25% with no XRT. These data demonstrated that patients who had a poor response to chemotherapy and underwent an amputation faired poorly when compared to patients with LSS. There is a selection bias in patients selected to undergo amputation. Additionally, patients who underwent amputation had a lower rate of local recurrence, but still had a poorer survival when compared to LSS.
Myxofibrosarcoma is described by WHO as comprising of a spectrum of malignant fibroblastic lesions with variably myxoid stoma, pleomorphism and with a distinctively curvilinear vascular pattern. They are reported to be one of the commonest sarcomas of elderly patients. It has previously been reported to have a high rate of locally recurrent disease (50-60%). The aim of the study was to investigate the ROH series of tumours to determine prognostic factors for survival and local recurrence. Patients were identified from the ROH database who had been treated with a new presentation of myxofibrosarcoma. The size, grade and depth of the tumour at presentation was noted. Those patients who had suffered local or distal disease relapse or died were highlighted. Analysis was undertaken by Kaplan Meier survival curves for univariate and cox regression for multivariate analysis.Introduction
Methods
The aim was to identify when primary amputation was used as primary treatment and to describe outcomes in patients managed with modern chemotherapy. A detailed review of the electronic patient records was undertaken. Statistical analysis was performed with univariate analysis using Kaplan-Meier curves and Chi2 testing, whilst multivariate analysis was performed using Cox regression analysis. There were 354 osteosarcomas. 93 patients presented with metastases and 192 subsequently developed metastases at a mean of 46 months. Amputation was performed as the primary surgical treatment in 101 patients. Endoprosthetic reconstruction was used in 253 patients. Amputation was performed as a secondary procedure on 15 patients. The 5 and 10 year survival data for all patients, including those with metastatic disease were 60% and 60% for amputation with good chemotherapy response (>89% necrosis), 65% and 63% for limb salvage and good response, 21% and 21% for amputation and poor response (⋋90% necrosis) and 51% and 30% for limb salvage with poor response. Local recurrence occurred both with amputation (10.8%) or limb salvage (9%), with no significant differences between the two. Univariate analysis demonstrated that the extent of response to chemotherapy induced necrosis significantly affected survival, whether the patient had an amputation or not. Whether or not amputation or LSS was used in the surgical management of patients, local recurrence rates where similar between the two groups. Further assessment of chemotherapy-induced necrosis is a key factor in determining subsequent limb salvage or amputation management strategies.
To look at the effect of width and tissue at surgical margins, together with the use of adjuvant therapy on locally recurrent disease and disease free survival. The distance (mm) and tissue has been regularly reported for 5 years, prior to this it was reported as wide, marginal or intra-lesional. It is known from previous studies that 90% of locally recurrent disease for osteosarcoma and Ewing's occurs within 2 years from surgery. Therefore all primary bone sarcomas of the pelvis and appendicular skeleton that underwent surgery between 1/1/2005 and 1/1/2008 were entered, giving at least 2 years follow up. The pathology records were reviewed and the margins and percentage tumour necrosis were recorded. Those patients who had suffered local or distal disease relapse or died were highlighted. Analysis was undertaken by Kaplan Meier survival curves with univariate and multivariate analysis.Introduction
Methods
Chondrosarcoma of bone is a surgical disease and excision with wide margins is the optimum treatment. Sometimes the size or location of the tumour at the time of diagnosis mean that only a marginal excision can be achieved. The effect of the margin of excision on outcome is investigated. All patients with newly diagnosed primary chondrosarcoma of bone and without metastases at the time of diagnosis were identified from a prospective database. Their outcome was investigated to assess whether the margin of excision affected outcome. 492 patients were included in the analysis with a mean age of 48. The mean tumour size was 11cm and 59% were male. The 10 year tumour specific survival was 85% for clear cell (N=7) and grade 1 (N=210), 60% for grade 2 (N=180), 47% for grade 3 (N=59) and 16% for dedifferentiated (N=36). The 10 year local recurrence free survival was 86% (clear cell), 73% (grade 1), 67% (grade 2), 36% (grade 3) and 56% dedifferentiated. Local recurrence was strongly related to older age (p=0.0065), grade (p⋋0.0001) and margins (p⋋0.0001). Patients who developed local recurrence had a 43% survival at 10 years compared with 76% for those who did not (p⋋0.0001). They also had a 49% risk of developing metastases compared to a 17% risk for those without local recurrence (p⋋0.0001). However most of these patients had metastases before developing LR(57%). 16 of 65 patients with local recurrence but no metastases, died due to local progression (usually pelvic or spinal tumours). There was however no relation of the margin of excision to either the risk of developing metastases or survival for any grade of tumour.Method
Results
To identify factors leading to the success or failure of extracorporeal irradiation and re-implantation of bone (ECIR). Review of experience of this technique since 1996 documenting successes and failures.Aim
Method
To determine the overall survival of patients with Pelvic Ewing's Sarcoma treated in our unit and to identify prognostic factors in pelvic primaries that could be used to select patients who would most likely benefit from high intensity treatment. Between 1977 and 2009, 80 male and 66 female patients aged 2 to 60 (mean, 18) years with Pelvic Ewing's Sarcomas were retrospectively reviewed from the Royal Orthopaedic Hospital Oncology Service Registry. Treatments included surgery, radiotherapy, chemotherapy, or any of them in combination. Event-free (from presentation to recurrence) and overall (from presentation to death/latest follow-up) survival rates were calculated using the Kaplan- Meier method. Influence of various factors (age at diagnosis, gender, tumour site, metastasis at presentation, surgery (and surgical margins), radiotherapy, and type of treatment on survival was assessed using SPSS 14.0 statistical software.Aim
Method
The aim of this study was to investigate the results of a series of cases from a single institution with respect to local disease control and patient survival to determine prognostic factors. Electronic patient records were reviewed on all patients with STS between February 1963 and January 2007. 2445 patients had over 30 types of STS. 1639 (67%) had not received any treatment prior to presentation, however, 770 patients (32%) had undergone a previous attempted excision. Survival analyses were done using Kaplan Meier and Cox regression analyses, however, for prognostic factor analysis, only patients presenting without prior treatment were included.Introduction
Methods
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
Neoadjuvant chemotherapy for osteosarcoma improves outcomes for the majority, but if the chemotherapy does not work then the dilemma often arises as to whether to do limb salvage with a marginal (or worse) margin of excision or to do an amputation. If limb salvage is carried out with a close margin, does post operative radiotherapy make any difference? This study aims to address these questions. All patients with limb osteosarcoma, no metastases, a poor response to chemotherapy and either a marginal excision or primary amputation were identified from a prospective database. This group were investigated in terms of overall survival and local control. There were 182 patients in this category of whom 60 had an amputation, 105 limb salvage with marginal margins and 17 with an intralesional margin. Local recurrence (LR) arose in 41% of those with an intralesional margin, 22% of those with a marginal margin and 13% of those with an amputation. Radiotherapy was used in 21 of the 122 patients and the risk of LR was the same as in those who did not have radiotherapy. Neither age nor sex of the patient, size or site of the tumour affected the risk of LR. The overall survival for this group was 42% at 10 years. The survival was best in those with marginal margins (38%) than those with an amputation (28%) and worst for those with an intralesional margin (20%). Survival was worst in those who did develop LR, but no worse than in those having amputationMethod
Results
The outcome for patients with Ewing's sarcoma recurrence is poor. Local recurrences occur in 8%-25%of these patients. The aim of the study was to analyze the patients who had a local recurrence to identify factors predicting the local recurrence and if it could be prevented A retrospective analysis of 650 patients who had a diagnosis of Ewing's sarcoma treated between 1975 and 2009 at a single institution was performed and 64 patients (10%) who had a local recurrence were identified and analysed. Fifteen patients had metastases at diagnosis.20 patients had chemotherapy and radiotherapy only while 44 had chemotherapy and surgery +/− post op radiotherapy. Thirteen patients who were suitable for post –operative radiotherapy could not receive the treatment due to various reasons like biological reconstruction. The estimated 5 years survival for the patients was 15%. The risk of local recurrence is higher if the tumour is located in the axial skeleton, treatment with chemotherapy and radiotherapy alone [location and size of the tumour precluding surgery]. The risk of local recurrence is higher if the tumour was in the fibula or radius. One out of three patients who have good response to chemotherapy still went on to develop a LR. The use of biological reconstruction and younger age group often resulted in deferral of post-operative radiotherapy. Location and type of treatment can predict LR. Surgery with clear margins and post-operative radiotherapy given when indicated may reduce the incidence of LR.Methods
Results
Recently a great deal of interest has emerged in new techniques for resection of bone tumours, such as the use of computer guided surgery, joint sparing prostheses and epiphysiolysis. However, all the techniques may require narrower margins at resection than the traditional Enneking wide margins. The aim of the study was to look at the effect of width and tissue at surgical margins, together with the use of adjuvant therapy on locally recurrent disease and disease free survival. The quality in terms of precise distance (mm) and tissue has been regularly reported by our pathologists for 5 years, prior to this it was reported as wide, marginal or intralesional. It is known from previous studies that 90% of locally recurrent disease for osteosarcoma and Ewings occurs within 2 years from surgery. Therefore all primary bone sarcomas of the pelvis and appendicular skeleton that underwent surgery between 1/1/2005 and 1/1/2008 were entered into the study group, giving at least 2 years follow up. The pathology records were reviewed and the quality of the margins and percentage tumour necrosis were recorded. Those patients who had suffered local or distal disease relapse or died were highlighted. Analysis was undertaken by Kaplan Meier survival curves with univariate and multivariate analysis.Introduction
Methods
The pelvis has always been a difficult area for surgeons, with high complication rates from surgery and the perception of poor oncological outcomes. The aim of the study was to look at the surgical and oncological outcomes of pelvic tumours treated at our centre. From the 3100 primary bone tumours seen at the ROH. Information was retrieved on 539 patients seen with a primary bone tumour of the pelvis. The demographic details, oncological and surgical outcomes were reviewed.Introduction
Methods
Bone tumours rarely involve the joint surface as cartilage is thought to be a good barrier to tumour spread. When the tumour does cross the surface the surgeon is faced with the dilemma of whether to amputate the limb, resect it without reconstruction or reconstruct with an implant. This paper aims to investigate the oncological and functional outcomes of patients undergoing an extra-articular resection and reconstruction with an endoprosthesis. 3100 patients have been seen in ROH with primary bone tumours. Patients were identified who had an extra-articular resection considered pre-operatively and the notes and imaging was reviewed. This group was subdivided into a group who did have an extra-articular resection (EAR) and those who either had an amputation or traditional through joint resection. The outcomes of the three groups (group 1 = no joint involvement, group 2 = EAR considered but not done and group 3 = EAR) were then compared in terms of oncological outcome, surgical margins and complications.Introduction
Method
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.
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 have analysed the pattern of symptoms in patients presenting with synovial sarcoma to identify factors which led to long delays in diagnosis. In 35 children, the early symptoms and the results of clinical and radiological investigation were reviewed, along with the presumed diagnoses. The duration of symptoms was separated into patient delay and doctor delay. Only half of the patients had one or more of the four clinical findings suggestive of sarcoma according to the guidance of the National Institute for Clinical Excellence at the onset of symptoms. Of the 33 children for whom data were available, 16 (48.5%) presented with a painless mass and in ten (30.3%) no mass was identified. Seven (21.2%) had an unexplained joint contracture. Many had been extensively investigated unsuccessfully. The mean duration of symptoms was 98 weeks (2 to 364), the mean patient delay was 43 weeks (0 to 156) and the mean doctor delay was 50 weeks (0 to 362). The mean number of doctors seen before referral was three (1 to 6) and for 15 patients the diagnosis was obtained after unplanned excision. Tumours around the knee and elbow were associated with a longer duration of symptoms and longer doctor delay compared with those at other sites. Delays did not improve significantly over the period of our study of 21 years, and we were unable to show that delay in diagnosis led to a worse prognosis. Our findings highlight the variety of symptoms associated with synovial sarcoma and encourage greater awareness of this tumour as a potential diagnosis in childhood.
We undertook this retrospective study to determine the rate of recurrence and functional outcome after intralesional curettage for chondroblastoma of bone. The factors associated with aggressive behaviour of the tumour were also analysed. We reviewed 53 patients with histologically-proven chondroblastoma who were treated by intralesional curettage in our unit between 1974 and 2000. They were followed up for at least two years to a maximum of 27 years. Seven (13.2%) had a histologically-proven local recurrence. Three underwent a second intralesional curettage and had no further recurrence. Two had endoprosthetic replacement of the proximal humerus and two underwent below-knee amputation after aggressive local recurrence. One patient had the rare malignant metastatic chondroblastoma and eventually died. The mean Musculoskeletal Tumour Society functional score of the survivors was 94.2%. We conclude that meticulous intralesional curettage alone can achieve low rates of local recurrence and excellent long-term function.
