Aims

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.

Aims

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.

This article provides an overview of the role of genomics in sarcomas and describes how new methods of analysis and comparative screening have provided the potential to progress understanding and treatment of sarcoma. This article reviews genomic techniques, the evolution of the use of genomics in cancer, the current state of genomic analysis, and also provides an overview of the medical, social and economic implications of recent genomic advances.

We undertook a retrospective review of 33 patients who underwent total femoral endoprosthetic replacement as limb salvage following excision of a malignant bone tumour. In 22 patients this was performed as a primary procedure following total femoral resection for malignant disease. Revision to a total femoral replacement was required in 11 patients following failed segmental endoprosthetic or allograft reconstruction. There were 33 patients with primary malignant tumours, and three had metastatic lesions. The mean age of the patients was 31 years (5 to 68). The mean follow-up was 4.2 years (9 months to 16.4 years). At five years the survival of the implants was 100%, with removal as the endpoint and 56% where the endpoint was another surgical intervention. At five years the patient survival was 32%. Complications included dislocation of the hip in six patients (18%), local recurrence in three (9%), peri-prosthetic fracture in two and infection in one. One patient subsequently developed pulmonary metastases. There were no cases of aseptic loosening or amputation. Four patients required a change of bushings. The mean Musculoskeletal Tumour Society functional outcome score was 67%, the mean Harris Hip Score was 70, and the mean Oxford Knee Score was 34.

Total femoral endoprosthetic replacement can provide good functional outcome without compromising patient survival, and in selected cases provides an effective alternative to amputation.

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.

Aims

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 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.

We retrospectively studied 14 patients with proximal and diaphyseal tumours and disappearing bone (Gorham’s) disease of the humerus treated with wide resection and reconstruction using an allograft-resurfacing composite (ARC). There were ten women and four men, with a mean age of 35 years (8 to 69). At a mean follow-up of 25 months (10 to 89), two patients had a fracture of the allograft. In one of these it was revised with a similar ARC and in the other with an intercalary prosthesis. A further patient had an infection and a fracture of the allograft that was revised with a megaprosthesis. In all patients with an ARC, healing of the ARC-host bone interface was observed. One patient had failure of the locking mechanism of the total elbow replacement. The mean post-operative Musculoskeletal Tumor Society score for the upper extremity was 77% (46.7% to 86.7%), which represents good and excellent results; one patient had a poor result (46.7%).

In the short term ARC effectively relieves pain and restores shoulder function in patients with wide resection of the proximal humerus. Fracture and infection remain significant complications.

The use of allografts for the treatment of bone tumours in children is limited by nonunion and the difficulty of finding a suitable graft. Furthermore, appositional growth can’t be expected of an allograft.

We used an overlapping allograft in 11 children, with a mean age of ten years (4 to 15), with a mean follow-up of 24.1 months (20 to 33). There were five intercalary and six intra-articular resections, and the tumours were in the femur in six cases and the humerus in five. Rates of union, times to union, remodelling patterns and allograft-associated complications were evaluated.

No allograft was removed due to a complication. Of the 16 junctional sites, 15 (94%) showed union at a mean of 3.1 months (2 to 5). Remodelling between host and allograft was seen at 14 junctions at a mean of five months (4 to 7). The mean Musculoskeletal Tumor Society score was 26.5 of 30 (88.3%). One case of nonunion and another with screw protrusion required re-operation.

Overlapping allografts have the potential to shorten time to union, decrease rates of nonunion and have positive appositional growth effect.

Several techniques have been described to reconstruct a mobile wrist joint after resection of the distal radius for tumour. We reviewed our experience of using an osteo-articular allograft to do this in 17 patients with a mean follow-up of 58.9 months (28 to 119).

The mean range of movement at the wrist was 56° flexion, 58° extension, 84° supination and 80° pronation. The mean ISOLS-MSTS score was 86% (63% to 97%) and the mean patient-rated wrist evaluation score was 16.5 (3 to 34). There was no local recurrence or distant metastases. The procedure failed in one patient with a fracture of the graft and an arthrodesis was finally required. Union was achieved at the host-graft interface in all except two cases. No patient reported more than modest non-disabling pain and six reported no pain at all. Radiographs showed early degenerative changes at the radiocarpal joint in every patient.

A functional pain-free wrist can be restored with an osteo-articular allograft after resection of the distal radius for bone tumour, thereby avoiding the donor site morbidity associated with an autograft. These results may deteriorate with time.

We undertook a prospective study to evaluate the prognostic significance of the serum levels of vascular endothelial growth factor (VEGF) in predicting the survival of patients with osteosarcoma. The levels were measured by an enzyme-linked immunosorbent assay in 15 patients with osteosarcoma before commencing treatment. The patients were divided into two groups, with a high or a low serum VEGF level, and the incidence of metastases and overall survival rate were compared.

No significant relationship was observed between the serum VEGF levels and gender, age, the size of the tumour or the response to pre-operative chemotherapy. Patients with a serum VEGF > 1000 pg/ml had significantly worse survival than those with a level < 1000 pg/ml (p = 0.002). The serum VEGF level may be useful in predicting the prognosis for survival in patients with osteosarcoma.

Between 1966 and 2001, 1254 patients underwent excision of a bone tumour with endoprosthetic replacement. All patients who had radiotherapy were identified. Their clinical details were retrieved from their records.

A total of 63 patients (5%) had received adjunctive radiotherapy, 29 pre-operatively and 34 post-operatively. The mean post-operative Musculoskeletal Tumor Society scores of irradiated patients were significantly lower (log-rank test, p = 0.009). The infection rate in the group who had not been irradiated was 9.8% (117 of 1191), compared with 20.7% (6 of 29) in those who had pre-operative radiotherapy and 35.3% (12 of 34) in those who radiotherapy post-operatively. The infection-free survival rate at ten years was 85.5% for patients without radiotherapy, 74.1% for those who had pre-operative radiotherapy and 44.8% for those who had post-operative radiotherapy (log-rank test, p < 0.001). The ten-year limb salvage rate was 89% for those who did not have radiotherapy and 76% for those who did (log-rank test, p = 0.02).

Radiotherapy increased the risk of revision (log-rank test, p = 0.015). A total of ten amputations were necessary to control infection, of which nine were successful. Radiotherapy may be necessary for the treatment of a bone sarcoma but increases the risk of deep infection for which amputation may be the only solution.

Patients who have limb amputation for musculoskeletal tumours are a rare group of cancer survivors. This was a prospective cross-sectional survey of patients from five specialist centres for sarcoma surgery in England. Physical function, pain and quality of life (QOL) outcomes were collected after lower extremity amputation for bone or soft-tissue tumours to evaluate the survivorship experience and inform service provision.

Of 250 patients, 105 (42%) responded between September 2012 and June 2013. From these, completed questionnaires were received from 100 patients with a mean age of 53.6 years (19 to 91). In total 60 (62%) were male and 37 (38%) were female (three not specified). The diagnosis was primary bone sarcoma in 63 and soft-tissue tumour in 37. A total of 20 tumours were located in the hip or pelvis, 31 above the knee, 32 between the knee and ankle and 17 in the ankle or foot. In total 22 had hemipelvectomy, nine hip disarticulation, 35 transfemoral amputation, one knee disarticulation, 30 transtibial amputation, two toe amputations and one rotationplasty. The Toronto Extremity Salvage Score (TESS) differed by amputation level, with poorer scores at higher levels (p < 0.001). Many reported significant pain. In addition, TESS was negatively associated with increasing age, and pain interference scores. QOL for Cancer Survivors was significantly correlated with TESS (p < 0.001). This relationship appeared driven by pain interference scores.

This unprecedented national survey confirms amputation level is linked to physical function, but not QOL or pain measures. Pain and physical function significantly impact on QOL. These results are helpful in managing the expectations of patients about treatment and addressing their complex needs.

Cite this article: Bone Joint J 2015;97-B:1284–90.

We determined the efficacy of a devitalised autograft (n = 13) and allograft (n = 16) cortical strut bone graft combined with long-stem endoprosthetic reconstruction in the treatment of massive tumours of the lower limb. A total of 29 patients (18 men:11 women, mean age 20.1 years (12 to 45) with a ratio of length of resection to that of the whole prosthesis of > 50% were treated between May 2003 and May 2012. The mean follow-up was 47 months (15 to 132). The stem of the prosthesis was introduced through bone graft struts filled with cement, then cemented into the residual bone. Bone healing was achieved in 23 patients (86%). The mean Musculoskeletal Tumour Society functional score was 85% (57 to 97). The five-year survival rate of the endoprostheses was 81% (95% confidence intervals 67.3 to 92.3). The mean length of devitalised autografts and allografts was 8.6 cm (5 to 15), which increased the ratio of the the length of the stem of the prosthesis to that of the whole length of the prosthesis from a theoretical 35% to an actual 55%.

Cortical strut bone grafting and long-stem endoprosthetic reconstruction is an option for treating massive segmental defects following resection of a tumour in the lower limb. Patients can regain good function with a low incidence of aseptic loosening. The strut graft and the residual bone together serve as a satisfactory bony environment for a revision prosthesis, if required, once union is achieved.

Cite this article: Bone Joint J 2015;97-B:544–9.

The long term biological effects of wear products following total hip arthroplasty (THA) are unclear. However, the indications for THA are expanding, with increasingly younger patients undergoing the procedure.

This prospective, randomised study compared two groups of patients undergoing THA after being randomised to receive one of two different bearing surfaces: metal-on-polyethylene (MoP) n = 22 and metal-on-metal (MoM) n = 23. We investigated the relationship between three variables: bearing surface (MoP vs MoM), whole blood levels of chromium (Cr) and cobalt (Co) and chromosomal aberrations in peripheral lymphocyte pre-operatively and at one, two and five years post-surgery.

Our results demonstrated significantly higher mean cobalt and chromium (Co and Cr) blood levels in the MoM group at all follow-up points following surgery (p < 0.01), but there were no significant differences in the chromosomal aberration indices between MoM and MoP at two or five years (two years: p = 0.56, p = 0.08, p = 0.91, p = 0.51 and five years: p = 0.086, p = 0.73, p = 0.06, p = 0.34) for translocations, breaks, loss and gain of chromosomes respectively. Regression analysis showed a strong linear relationship between Cr levels and the total chromosomal aberration indices in the MoM group (R² = 0.90016), but this was not as strong for Co (R² = 0.68991). In the MoP group, the analysis revealed a poor relationship between Cr levels and the total chromosomal aberration indices (R² = 0.23908) but a slightly stronger relationship for Co (R² = 0.64292). Across both groups, Spearman’s correlation detected no overall association between Co and Cr levels and each of the studied chromosomal aberrations. There remains no clear indication which THA bearing couple is the most biocompatible, especially in young active patients. While THA continues to be very successful at alleviating pain and restoring function, the long-term biological implications of the procedure still require further scrutiny.

Cite this article: Bone Joint J 2015;97-B:1183–91.

We evaluated the construct validity of the Musculoskeletal Tumour Society rating scale (Enneking score) as a functional measure for patients with sarcoma involving the upper limb. We compared the Enneking score by examining the correlation between two patient-derived outcome measures, the Disability of the Arm, Shoulder, and Hand (DASH) questionnaire and the Medical Outcomes Study Short Form-36 (SF-36) as indicators of functional status in 40 patients with malignant or aggressive benign bone and soft-tissue tumours of the upper limb who had undergone surgical treatment.

The frequency distributions were similar among the three scoring systems. As for the validity, Spearman’s rank correlation coefficient of the Enneking score to the DASH questionnaire was −0.79 and that of the Enneking to the SF-36 subscales ranged from 0.38 to 0.60. Despite being a measure from the surgeon’s perspective, the Enneking score was shown to be a valid indicator of physical disability in patients with malignant or aggressive benign tumours of the upper limb and reflected their opinion.

Objective

Clinical studies of patients with bone sarcomas have been challenged by insufficient numbers at individual centres to draw valid conclusions. Our objective was to assess the feasibility of conducting a definitive multi-centre randomised controlled trial (RCT) to determine whether a five-day regimen of post-operative antibiotics, in comparison to a 24-hour regimen, decreases surgical site infections in patients undergoing endoprosthetic reconstruction for lower extremity primary bone tumours.

Vascularised fibular grafts (VFGs ) are a valuable surgical technique in limb salvage after resection of a tumour. The primary objective of this multicentre study was to assess the risk factors for failure and complications for using a VFG after resection of a tumour.

The study involved 74 consecutive patients (45 men and 29 women with mean age of 23 years (1 to 64) from four tertiary centres for orthopaedic oncology who underwent reconstruction using a VFG after resection of a tumour between 1996 and 2011. There were 52 primary and 22 secondary reconstructions. The mean follow-up was 77 months (10 to 195).

In all, 69 patients (93%) had successful limb salvage; all of these united and 65 (88%) showed hypertrophy of the graft. The mean time to union differed between those involving the upper (28 weeks; 12 to 96) and lower limbs (44 weeks; 12 to 250). Fracture occurred in 11 (15%), and nonunion in 14 (19%) patients.

In 35 patients (47%) at least one complication arose, with a greater proportion in lower limb reconstructions, non-bridging osteosynthesis, and in children. These complications resulted in revision surgery in 26 patients (35%).

VFG is a successful and durable technique for reconstruction of a defect in bone after resection of a tumour, but is accompanied by a significant risk of complications, that often require revision surgery. Union was not markedly influenced by the need for chemo- or radiotherapy, but should not be expected during chemotherapy. Therefore, restricted weight-bearing within this period is advocated.

Cite this article: Bone Joint J 2015;97-B:853–61.

Peri-acetabular tumour resections and their subsequent reconstruction are among the most challenging procedures in orthopaedic oncology. Despite the fact that a number of different pelvic endoprostheses have been introduced, rates of complication remain high and long-term results are mostly lacking.

In this retrospective study, we aimed to evaluate the outcome of reconstructing a peri-acetabular defect with a pedestal cup endoprosthesis after a type 2 or type 2/3 internal hemipelvectomy.

A total of 19 patients (11M:8F) with a mean age of 48 years (14 to 72) were included, most of whom had been treated for a primary bone tumour (n = 16) between 2003 and 2009. After a mean follow-up of 39 months (28 days to 8.7 years) seven patients had died. After a mean follow-up of 7.9 years (4.3 to 10.5), 12 patients were alive, of whom 11 were disease-free. Complications occurred in 15 patients. Three had recurrent dislocations and three experienced aseptic loosening. There were no mechanical failures. Infection occurred in nine patients, six of whom required removal of the prosthesis. Two patients underwent hindquarter amputation for local recurrence.

The implant survival rate at five years was 50% for all reasons, and 61% for non-oncological reasons. The mean Musculoskeletal Tumor Society score at final follow-up was 49% (13 to 87).

Based on these poor results, we advise caution if using the pedestal cup for reconstruction of a peri-acetabular tumour resection.

Cite this article: Bone Joint J 2014;96-B:1706–12.