Between 1986 and 2002, 42 patients with synchronous multifocal osteosarcoma were treated with two different protocols of neoadjuvant chemotherapy. When feasible, the primary and secondary tumours were excised as a combined procedure. After initial chemotherapy 26 patients were excluded from
We performed a systematic literature review to define features of patients, treatment, and biological behaviour of multicentric giant cell tumour (GCT) of bone. The search terms used in combination were “multicentric”, “giant cell tumour”, and “bone”. Exclusion criteria were: reports lacking data, with only an abstract; papers not reporting data on multicentric GCT; and papers on multicentric GCT associated with other diseases. Additionally, we report three patients treated under our care.Aims
Methods
Orthopaedic and reconstructive surgeons are faced with large defects after the resection of malignant tumours of the sacrum. Spinopelvic reconstruction is advocated for resections above the level of the S1 neural foramina or involving the sacroiliac joint. Fixation may be augmented with either free vascularized fibular flaps (FVFs) or allograft fibular struts (AFSs) in a cathedral style. However, there are no studies comparing these reconstructive techniques. We reviewed 44 patients (23 female, 21 male) with a mean age of 40 years (SD 17), who underwent en bloc sacrectomy for a malignant tumour of the sacrum with a reconstruction using a total (n = 20), subtotal (n = 2), or hemicathedral (n = 25) technique. The reconstructions were supplemented with a FVF in 25 patients (57%) and an AFS in 19 patients (43%). The mean length of the strut graft was 13 cm (SD 4). The mean follow-up was seven years (SD 5).Aims
Methods
Cell-free DNA (cfDNA) and circulating tumour DNA (ctDNA) are used for prognostication and monitoring in patients with carcinomas, but their utility is unclear in sarcomas. The objectives of this pilot study were to explore the prognostic significance of cfDNA and investigate whether tumour-specific alterations can be detected in the circulation of sarcoma patients. Matched tumour and blood were collected from 64 sarcoma patients (n = 70 samples) prior to resection of the primary tumour (n = 57) or disease recurrence (n = 7). DNA was isolated from plasma, quantified, and analyzed for cfDNA. A subset of cases (n = 6) underwent whole exome sequencing to identify tumour-specific alterations used to detect ctDNA using digital droplet polymerase chain reaction (ddPCR).Aims
Methods
We retrospectively reviewed 101 consecutive patients
with 114 femoral tumours treated by massive bone allograft at our
institution between 1986 and 2005. There were 49 females and 52
males with a mean age of 20 years (4 to 74). At a median follow-up
of 9.3 years (2 to 19.8), 36 reconstructions (31.5%) had failed.
The allograft itself failed in 27 reconstructions (24%). Mechanical complications such as delayed union, fracture and
failure of fixation were studied. The most adverse factor on the
outcome was the use of intramedullary nails, followed by post-operative
chemotherapy, resection length >
17 cm and age >
18 years at the
time of intervention. The
Tenosynovial giant cell tumour (TGCT) is one of the most common soft-tissue tumours of the foot and ankle and can behave in a locally aggressive manner. Tumour control can be difficult, despite the various methods of treatment available. Since treatment guidelines are lacking, the aim of this study was to review the multidisciplinary management by presenting the largest series of TGCT of the foot and ankle to date from two specialized sarcoma centres. The Oxford Tumour Registry and the Leiden University Medical Centre Sarcoma Registry were retrospectively reviewed for patients with histologically proven foot and ankle TGCT diagnosed between January 2002 and August 2019.Aims
Methods
Iliac wing (Type I) and iliosacral (Type I/IV) pelvic resections for a primary bone tumour create a large segmental defect in the pelvic ring. The management of this defect is controversial as the surgeon may choose to reconstruct it or not. When no reconstruction is undertaken, the residual ilium collapses back onto the remaining sacrum forming an iliosacral pseudarthrosis. The aim of this study was to evaluate the long-term oncological outcome, complications, and functional outcome after pelvic resection without reconstruction. Between 1989 and 2015, 32 patients underwent a Type I or Type I/IV pelvic resection without reconstruction for a primary bone tumour. There were 21 men and 11 women with a mean age of 35 years (15 to 85). The most common diagnosis was chondrosarcoma (50%, n = 16). Local recurrence-free, metastasis-free, and overall survival were assessed using the Kaplan-Meier method. Patient function was evaluated using the Musculoskeletal Tumour Society (MSTS) and Toronto Extremity Salvage Score (TESS).Aims
Methods
After intercalary resection of a bone tumour from the femur,
reconstruction with a vascularized fibular graft (VFG) and massive
allograft is considered a reliable method of treatment. However,
little is known about the long-term outcome of this procedure. The
aims of this study were to determine whether the morbidity of this
procedure was comparable to that of other reconstructive techniques,
if it was possible to achieve a satisfactory functional result, and
whether biological reconstruction with a VFG and massive allograft
could achieve a durable, long-lasting reconstruction. A total of 23 patients with a mean age of 16 years (five to 40)
who had undergone resection of an intercalary bone tumour of the
femur and reconstruction with a VFG and allograft were reviewed
clinically and radiologically. The mean follow-up was 141 months
(24 to 313). The mean length of the fibular graft was 18 cm (12 to
29). Full weight-bearing without a brace was allowed after a mean
of 13 months (seven to 26).Aims
Patients and Methods
Free vascularised fibular grafting has been used for the treatment
of large bony defects for more than 40 years. However, there is
little information about the risk factors for failure and whether
newer locking techniques of fixation improve the rates of union.
The purpose of this study was to compare the rates of union of free
fibular grafts fixed with locking and traditional techniques, and
to quantify the risk factors for nonunion and failure. A retrospective review involved 134 consecutive procedures over
a period of 20 years. Of these, 25 were excluded leaving 109 patients
in the study. There were 66 men and 43 women, with a mean age of
33 years (5 to 78). Most (62) were performed for oncological indications,
and the most common site (52) was the lower limb. Rate of union
was estimated using the Kaplan-Meier method and risk factors for
nonunion were assessed using Cox regression. All patients were followed
up for at least one year.Aims
Patients and Methods
Few studies dealing with chondrosarcoma of the pelvis are currently
available. Different data about the overall survival and prognostic
factors have been published but without a detailed analysis of surgery-related complications.
We aimed to analyse the outcome of a series of pelvic chondrosarcomas
treated at a single institution, with particular attention to the
prognostic factors. Based on a competing risk model, our objective
was to identify risk factors for the development of complications. In a retrospective single-centre study, 58 chondrosarcomas (26
patients alive, 32 patients dead) of the pelvis were reviewed. The
mean follow-up was 13 years (one week to 23.1 years).Aims
Patients and Methods
Rarely, the extent of a malignant bone tumour
may necessitate resection of the complete humerus to achieve adequate
oncological clearance. We present our experience with reconstruction
in such cases using a total humeral endoprosthesis (THER) in 20
patients (12 male and eight female) with a mean age of 22 years
(6 to 59). We assessed the complications, the oncological and functional
outcomes and implant survival. Surgery was performed between June
2001 and October 2009. The diagnosis included osteosarcoma in nine,
Ewing’s sarcoma in eight and chondrosarcoma in three. One patient
was lost to follow-up. The mean follow-up was 41 months (10 to 120)
for all patients and 56 months (25 to 120) in survivors. There were
five local recurrences (26.3%) and 11 patients were alive at time
of last follow-up, with overall survival for all patients being
52% (95% confidence interval (CI) 23.8 to 74) at five years. The
mean Musculoskeletal Tumor Society score for the survivors was 22
(73%; 16 to 23). The implant survival was 95% (95% CI 69.5 to 99.3)
at five years. The use of a THER in the treatment of malignant tumours of bone
is oncologically safe; it gives consistent and predictable results
with low rates of complication.
The current indications for functional restoration
of extension of the knee following quadriceps resection or loss require
reappraisal. The contribution of pedicled and free functional muscle
transfer is likely to be over-emphasised in many studies, with good
functional outcomes predominantly reported only in the context of
cases with residual quadriceps function. In cases with total quadriceps
resection or loss, all forms of reconstruction perform poorly. Furthermore,
in smaller resections with loss of two or fewer components of the
quadriceps, minimal impairment of function occurs in the absence
of functional reconstruction, suggesting that functional restoration
may not be warranted. Thus there is a paradox in the current approach
to quadriceps reconstruction, in that small resections are likely
to be over-treated and large resections remain under-treated. This review suggests a shift is required in the approach and
rationale for reconstructing functional extension of the knee after
quadriceps resection or loss. A classification based on current
evidence is suggested that emphasises more clearly the indications
and rationale for functional transfers.
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:
Pathological fractures in children can occur
as a result of a variety of conditions, ranging from metabolic diseases and
infection to tumours. Fractures through benign and malignant bone
tumours should be recognised and managed appropriately by the treating
orthopaedic surgeon. The most common benign bone tumours that cause pathological
fractures in children are unicameral bone cysts, aneurysmal bone
cysts, non-ossifying fibromas and fibrous dysplasia. Although pathological
fractures through a primary bone malignancy are rare, these should
be recognised quickly in order to achieve better outcomes. A thorough
history, physical examination and review of plain radiographs are
crucial to determine the cause and guide treatment. In most benign
cases the fracture will heal and the lesion can be addressed at
the time of the fracture, or after the fracture is healed. A step-wise
and multidisciplinary approach is necessary in caring for paediatric
patients with malignancies. Pathological fractures do not have to
be treated by amputation; these fractures can heal and limb salvage
can be performed when indicated.
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.
This paper describes the preliminary results of a proximal tibial endoprosthesis which spares the knee joint and enables retention of the natural articulation by replacing part of the tibial metaphysis and diaphysis. In eight patients who had a primary malignant bone tumour of the proximal tibia, the distal stem, which had a hydroxyapatite-coated collar to improve fixation, was cemented into the medullary canal. The proximal end had hydroxyapatite-coated extracortical plates which were secured to the remaining proximal tibial metaphysis using cortical screws. The mean age of the patients at operation was 28.9 years (8 to 43) and the mean follow-up was for 35 months (4 to 48). The mean Musculoskeletal Tumour Society score was 79% (57% to 90%), the mean Oxford Knee score was 40 points of 48 (36 to 46) and the mean knee flexion was 112° (100° to 120°). In one patient, revision to a below-knee amputation through the prosthesis was required because of recurrence of the tumour. Another patient sustained a periprosthetic fracture which healed with a painful malunion. This was revised to a further endoprosthesis which replaced the knee. In the remaining six patients the prosthesis allowed preservation of the knee joint with good function and no early evidence of loosening. Further follow-up is required to assess the longevity of these prostheses.
We have developed a hollow perforated cannulated screw. One or more of these was implanted percutaneously in 11 patients with an osteolytic metastasis in the femoral neck and multiple metastases elsewhere. They were supplemented by one or two additional standard 6.5 mm cannulated screws in nine patients. Polymethylmethacrylate bone cement was injected through the screw into the neck of the femur using small syringes, as in vertebroplasty. The mean amount of cement injected was 23.2 ml (17 to 30). Radiotherapy was started on the fourth post-operative day and chemotherapy, on average, was resumed a day later. Good structural stability and satisfactory relief from pain were achieved in all the patients. This technique may be useful in the palliation of metastases in the femoral neck.
We describe a consecutive series of five patients with bone or soft-tissue sarcomas of the elbow and intra-articular extension treated by complex soft tissue, allograft bone and prosthetic joint replacement after wide extra-articular
We present a retrospective study of patients suffering from a variety of benign tumours in whom external fixators were used to treat deformity and limb-length discrepancy, and for the reconstruction of bone defects. A total of 43 limbs in 31 patients (12 male and 19 female) with a mean age of 14 years (2 to 54) were treated. The diagnosis was Ollier’s disease in 12 limbs, fibrous dysplasia in 11, osteochondroma in eight, giant cell tumour in five, osteofibrous dysplasia in five and non-ossifying fibroma in two. The lesions were treated in the tibia in 19 limbs, in the femur in 16, and in the forearm in eight. The Ilizarov frame was used in 25 limbs, the Taylor Spatial Frame in seven, the Orthofix fixator in six, the Monotube in four and the Heidelberg fixator in one. The mean follow-up was 72 months (22 to 221). The mean external fixation period was 168 days (71 to 352). The mean external fixation index was 42 days/cm (22.2 to 102.0) in the 22 patients who required limb lengthening. The mean correction angle for those with angular deformity was 23° (7° to 45°). At final follow-up all patients had returned to normal activities. Four patients required a second operation for recurrent deformity of further limb lengthening. Local recurrence occurred in one patient, requiring further surgery.