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 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
Opinion remains divided as to whether the development
of pathological fracture affects the prognosis of patients with
an osteosarcoma of the extremities. We conducted a comprehensive systematic review and meta-analysis
of papers which reported the outcomes of osteosarcoma patients with
and without a pathological fracture. There were eight eligible papers
for final analysis which reported on 1713 patients, of whom 303
(17.7%) had a pathological fracture. The mean age for 1464 patients in
six studies was 23.2 years old (2 to 82). The mean follow-up for
1481 patients in seven studies was 90.1 months (6 to 240). The pooled estimates of local recurrence rates in osteosarcoma
patients with and without pathological fractures were 14.4% (8.7
to 20.0) The development of a pathological fracture is a negative prognostic
indicator in osteosarcoma and is associated with a reduced five-year
event-free survival and a possibly higher rate of local recurrence.
Our findings suggest that there is no absolute indication for amputation,
as similar rates of local recurrence can be achieved in patients
who are carefully selected for limb salvage. Cite this article:
This paper aims to provide evidence-based guidance for the general orthopaedic surgeon faced with the presentation of a potential soft tissue sarcoma in an extremity.
We retrospectively reviewed 71 histopathologically-confirmed bone and soft-tissue metastases of unknown origin at presentation. In order to identify the site of the primary tumour all 71 cases were examined with conventional procedures, including CT, serum tumour markers, a plain radiograph, ultrasound examination and endoscopic examinations, and 24 of the 71 cases underwent 2-deoxy-2-[F-18] fluoro-D-glucose positron emission tomography (FDG-PET). This detected multiple bone metastases in nine patients and the primary site in 12 of the 24 cases; conventional studies revealed 16 primary tumours. There was no significant difference in sensitivity between FDG-PET and conventional studies. The mean maximal standardised uptake value of the metastatic tumours was significantly higher than that of the primary tumours, which is likely to explain why FDG-PET did not provide better results. It was not superior to conventional procedures in the search for the primary site of bone and soft-tissue metastases; however, it seemed to be useful in the staging of malignancy.
We reviewed the outcome of 69 uncemented, custom-made,
distal femoral endoprosthetic replacements performed in 69 patients
between 1994 and 2006. There were 31 women and 38 men with a mean
age at implantation of 16.5 years (5 to 37). All procedures were
performed for primary malignant bone tumours of the distal femur.
At a mean follow-up of 124.2 months (4 to 212), 53 patients were
alive, with one patient lost to follow-up. All nine implants (13.0%)
were revised due to aseptic loosening at a mean of 52 months (8
to 91); three implants (4.3%) were revised due to fracture of the
shaft of the prosthesis and three patients (4.3%) had a peri-prosthetic
fracture. Bone remodelling associated with periosteal cortical thinning
adjacent to the uncemented intramedullary stem was seen in 24 patients
but this did not predispose to failure. All aseptically loose implants
in this series were diagnosed to be loose within the first five
years. The results from this study suggest that custom-made uncemented
distal femur replacements have a higher rate of aseptic loosening
compared to published results for this design when used with cemented
fixation. Loosening of uncemented replacements occurs early indicating
that initial fixation of the implant is crucial. Cite this article:
Between 1997 and 2007, 68 consecutive patients underwent replacement of the proximal humerus for tumour using a fixed-fulcrum massive endoprosthesis. Their mean age was 46 years (7 to 87). Ten patients were lost to follow-up and 16 patients died. The 42 surviving patients were assessed using the Musculoskeletal Tumor Society (MSTS) Score and the Toronto Extremity Salvage Score (TESS) at a mean follow-up of five years and 11 months (one year to ten years and nine months). The mean MSTS score was 72.3% (53.3% to 100%) and the mean TESS was 77.2% (58.6% to 100%). Four of 42 patients received a new constrained humeral liner to reduce the risk of dislocation. This subgroup had a mean MSTS score of 77.7% and a mean TESS of 80.0%. The dislocation rate for the original prosthesis was 25.9; none of the patients with the new liner had a dislocation at a mean of 14.5 months (12 to 18). Endoprosthetic replacement for tumours of the proximal humerus using this prosthesis is a reliable operation yielding good results without the documented problems of unconstrained prostheses. The performance of this prosthesis is expected to improve further with a new constrained humeral liner, which reduces the risk of dislocation.
The aim of this study was to analyse the gait
pattern, muscle force and functional outcome of patients who had undergone
replacement of the proximal tibia for tumour and alloplastic reconstruction
of the extensor mechanism using the patellar-loop technique. Between February 1998 and December 2009, we carried out wide
local excision of a primary sarcoma of the proximal tibia, proximal
tibial replacement and reconstruction of the extensor mechanism
using the patellar-loop technique in 18 patients. Of these, nine
were available for evaluation after a mean of 11.6 years (0.5 to
21.6). The strength of the knee extensors was measured using an
Isobex machine and gait analysis was undertaken in our gait assessment
laboratory. Functional outcome was assessed using the American Knee
Society (AKS) and Musculoskeletal Tumor Society (MSTS) scores. The gait pattern of the patients differed in ground contact time,
flexion heel strike, maximal flexion loading response and total
sagittal plane excursion. The mean maximum active flexion was 91°
(30° to 110°). The overall mean extensor lag was 1° (0° to 5°).
The mean extensor muscle strength was 25.8% (8.3% to 90.3%) of that
in the non-operated leg (p <
0.001). The mean functional scores
were 68.7% (43.4% to 83.3%) (MSTS) and 71.1 (30 to 90) (AKS functional
score). In summary, the results show that reconstruction of the extensor
mechanism using this technique gives good biomechanical and functional
results. The patients’ gait pattern is close to normal, except for
a somewhat stiff knee gait pattern. The strength of the extensor
mechanism is reduced, but sufficient for walking. 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:
The clinical utility of routine cross sectional imaging of the
abdomen and pelvis in the screening and surveillance of patients
with primary soft-tissue sarcoma of the extremities for metastatic
disease is controversial, based on its questionable yield paired
with concerns regarding the risks of radiation exposure, cost, and
morbidity resulting from false positive findings. Through retrospective review of 140 patients of all ages (mean
53 years; 2 to 88) diagnosed with soft-tissue sarcoma of the extremity
with a mean follow-up of 33 months (0 to 291), we sought to determine
the overall incidence of isolated abdominopelvic metastases, their
temporal relationship to chest involvement, the rate of false positives, and
to identify disparate rates of metastases based on sarcoma subtype.Objectives
Methods
The aim of this study was to assess the effect
of injecting genetically engineered chondrocytes expressing transforming
growth factor beta 1 (TGF-β1) into the knees of patients with osteoarthritis.
We assessed the resultant function, pain and quality of life. A total of 54 patients (20 men, 34 women) who had a mean age
of 58 years (50 to 66) were blinded and randomised (1:1) to receive
a single injection of the active treatment or a placebo. We assessed
post-treatment function, pain severity, physical function, quality
of life and the incidence of treatment-associated adverse events. Patients
were followed at four, 12 and 24 weeks after injection. At final follow-up the treatment group had a significantly greater
improvement in the mean International Knee Documentation Committee
score than the placebo group (16 points; -18 to 49, This technique may result in improved clinical outcomes, with
the aim of slowing the degenerative process, leading to improvements
in pain and function. However, imaging and direct observational
studies are needed to verify cartilage regeneration. Nevertheless,
this study provided a sufficient basis to proceed to further clinical testing. Cite this article:
Metal-on-metal resurfacing of the hip (MoMHR)
has enjoyed a resurgence in the last decade, but is now again in question
as a routine option for osteoarthritis of the hip. Proponents of
hip resurfacing suggest that its survival is superior to that of
conventional hip replacement (THR), and that hip resurfacing is
less invasive, is easier to revise than THR, and provides superior
functional outcomes. Our argument serves to illustrate that none
of these proposed advantages have been realised and new and unanticipated
serious complications, such as pseudotumors, have been associated
with the procedure. As such, we feel that the routine use of MoMHR
is not justified. Cite this article:
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.
We report our experience of using a computer
navigation system to aid resection of malignant musculoskeletal tumours
of the pelvis and limbs and, where appropriate, their subsequent
reconstruction. We also highlight circumstances in which navigation
should be used with caution. We resected a musculoskeletal tumour from 18 patients (15 male,
three female, mean age of 30 years (13 to 75) using commercially
available computer navigation software (Orthomap 3D) and assessed
its impact on the accuracy of our surgery. Of nine pelvic tumours,
three had a biological reconstruction with extracorporeal irradiation,
four underwent endoprosthetic replacement (EPR) and two required
no bony reconstruction. There were eight tumours of the bones of
the limbs. Four diaphyseal tumours underwent biological reconstruction.
Two patients with a sarcoma of the proximal femur and two with a
sarcoma of the proximal humerus underwent extra-articular resection
and, where appropriate, EPR. One soft-tissue sarcoma of the adductor
compartment which involved the femur was resected and reconstructed
using an EPR. Computer navigation was used to aid reconstruction
in eight patients. Histological examination of the resected specimens revealed tumour-free
margins in all patients. Post-operative radiographs and CT showed
that the resection and reconstruction had been carried out as planned
in all patients where navigation was used. In two patients, computer
navigation had to be abandoned and the operation was completed under
CT and radiological control. The use of computer navigation in musculoskeletal oncology allows
accurate identification of the local anatomy and can define the
extent of the tumour and proposed resection margins. Furthermore,
it helps in reconstruction of limb length, rotation and overall
alignment after resection of an appendicular tumour. Cite this article:
The December 2014 Oncology Roundup360 looks at: metaphyseal and diaphyseal osteosarcoma subtly different beasts; sports and endoprosthetic reconstruction of the knee; is curettage without tissue diagnosis sensible in cartilaginous tumours?; autoclaved autograft in bone tumour reconstruction; vascularised graft a step too far in bone defects?; interdigitated neoadjuvant chemoradiotherapy in high-grade sarcoma; predicting life expectancy in patients with painful metastasis; and osteolytic lesions of the hands and feet.
Custom-made intercalary endoprostheses may be used for the reconstruction of diaphyseal defects following the resection of bone tumours. The aim of this study was to determine the survival of intercalary endoprostheses with a lap joint design, and to evaluate the clinical results, complications and functional outcome. We retrospectively reviewed six consecutive patients, three of whom underwent limb salvage with intercalary endoprostheses of the tibia, two of the femur, and one of the humerus. Their mean age was 42 years (28 to 64). The mean follow-up was 21.6 months (9 to 58). The humeral prosthesis required revision at 14 months owing to aseptic loosening. There were no implant-related failures. Musculoskeletal Tumour Society functional outcome scores indicated that patients achieved 90% of premorbid function. Custom intercalary endoprostheses result in reconstructions comparable with, if not better than, those of allografts. Using this design of implant reduces the incidence of early complications and difficulties experienced with previous versions.
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.
In this case study, we describe the clinical
presentation and treatment of 36 patients with periosteal chondrosarcoma
collected over a 59-year period by the archive of the Netherlands
Committee on Bone Tumours. The demographics, clinical presentation,
radiological features, treatment and follow-up are presented with
the size, location, the histological grading of the tumour and the
survival. We found a slight predominance of men (61%), and a predilection
for the distal femur (33%) and proximal humerus (33%). The metaphysis
was the most common site (47%) and the most common presentation
was with pain (44%). Half the tumours were classified histologically
as grade 1. Pulmonary metastases were reported in one patient after
an intra-lesional resection. A second patient died from local recurrence
and possible pulmonary and skin metastases after an incomplete resection. It is clearly important to make the diagnosis appropriately because
an incomplete resection may result in local recurrence and metastatic
spread. Staging for metastatic disease is recommended in grade II
or III lesions. These patients should be managed with a contrast-enhanced MRI
of the tumour and histological confirmation by biopsy, followed
by Cite this article: