Endoprosthetic reconstruction following resection of 31 tumours of the proximal femur in 30 patients was performed using a Wagner SL femoral revision stem. The mean follow-up was 25.6 months (0.6 to 130.0). Of the 28 patients with a metastasis, 27 died within a mean follow-up period of 18.1 months (0.6 to 56.3) after the operation, and the remaining patient was excluded from the study 44.4 months post-operatively when the stem was removed. The two patients with primary bone tumours were still alive at the latest follow-up of 81.0 and 130.0 months, respectively. One stem only was removed for suspected low-grade infection 44.4 months post-operatively. The worst-case survival rate with removal of the stem for any cause and/or loss to follow-up was 80.0% (95% confidence interval 44.9 to 100) at 130.0 months. The mean Karnofsky index increased from 44.2% (20% to 70%) pre-operatively to 59.7% (0% to 100%) post-operatively, and the mean Merle d’Aubigné score improved from 4.5 (0 to 15) to 12.0 (0 to 18). The mean post-operative Musculoskeletal Tumour Society score was 62.4% (3.3% to 100%).

The Wagner SL femoral revision stem offers an alternative to special tumour prostheses for the treatment of primary and secondary tumours of the proximal femur. The mid-term results are very promising, but long-term experience is necessary.

Pigmented villonodular synovitis (PVNS) is a rare benign disease of the synovium of joints and tendon sheaths, which may be locally aggressive. We present 18 patients with diffuse-type PVNS of the foot and ankle followed for a mean of 5.1 years (2 to 11.8). There were seven men and 11 women, with a mean age of 42 years (18 to 73). A total of 13 patients underwent open or arthroscopic synovectomy, without post-operative radiotherapy. One had surgery at the referring unit before presentation with residual tibiotalar PVNS. The four patients who were managed non-operatively remain symptomatically controlled and under clinical and radiological surveillance. At final follow-up the mean Musculoskeletal Tumour Society score was 93.8% (95% confidence interval (CI) 85 to 100), the mean Toronto Extremity Salvage Score was 92 (95% CI 82 to 100) and the mean American Academy of Orthopaedic Surgeons foot and ankle score was 89 (95% CI 79 to 100). The lesion in the patient with residual PVNS resolved radiologically without further intervention six years after surgery. Targeted synovectomy without adjuvant radiotherapy can result in excellent outcomes, without recurrence. Asymptomatic patients can be successfully managed non-operatively. This is the first series to report clinical outcome scores for patients with diffuse-type PVNS of the foot and ankle.

Cite this article: Bone Joint J 2013;95-B:384–90.

The October 2012 Oncology Roundup³⁶⁰ looks at: the causes of primary bone tumours; adjuvant chemotherapy in the longer term; vascularised fibular grafts to salvage massive femoral allografts; a new look at old risks; reconstruction with excised irradiated bone; predicting chemosensitivity in osteosarcoma ; and chemotherapy, osteoporosis and the risk of fracture.

We report our early experience with the use of a non-invasive distal femoral expandable endoprosthesis in seven skeletally immature patients with osteosarcoma of the distal femur. The patients had a mean age of 12.1 years (9 to 15) at the time of surgery. The prosthesis was lengthened at appropriate intervals in outpatient clinics, without anaesthesia, using the principle of electromagnetic induction. The patients were functionally evaluated using the Musculoskeletal Tumour Society scoring system. The mean follow-up was 20.2 months (14 to 30). The prostheses were lengthened by a mean of 25 mm (4.25 to 55) and maintained a mean knee flexion of 110° (100° to 120°). The mean Musculoskeletal Tumour Society score was 68% (11 to 29). Complications developed in two patients; one developed a flexion deformity of 25° at the knee joint, which was subsequently overcome and one died of disseminated disease. The early results from patients treated with this device have been encouraging. The implant avoids multiple surgical procedures, general anaesthesia and assists in maintaining leg-length equality.

Surgery is considered to be the most effective treatment for cartilaginous tumours. In recent years, a trend has emerged for patients with low-grade tumours to be treated less invasively using curettage followed by various forms of adjuvant therapy. We investigated the potential for phenol to be used as an adjuvant. Using a human chondrosarcoma-derived cartilage-producing cell line OUMS-27 as an in vitro model we studied the cytotoxic effect of phenol and ethanol. Since ethanol is the standard substance used to rinse phenol out of a bone cavity, we included an assessment of ethanol to see whether this was an important secondary factor with respect to cell death. The latter was assessed by flow cytometry.

A cytotoxic effect was found for concentrations of phenol of 1.5% and of ethanol of 42.5%. These results may provide a clinical rationale for the use of both phenol and ethanol as adjuvant therapy after intralesional curettage in low-grade central chondrosarcoma and justify further investigation.

We examined osteochondral autografts, obtained at a mean of 19.5 months (3 to 48) following extracorporeal irradiation and re-implantation to replace bone defects after removal of tumours. The specimens were obtained from six patients (mean age 13.3 years (10 to 18)) and consisted of articular cartilage (five), subchondral bone (five), external callus (one) and tendon (one). The tumour cells in the grafts were eradicated by a single radiation dose of 60 Gy. In three cartilage specimens, viable chondrocytes were detected. The survival of chondrocytes was confirmed with S-100 protein staining. Three specimens from the subchondral region and a tendon displayed features of regeneration. Callus was seen at the junction between host and irradiated bone.

We investigated the use of hypoxia-inducible factor (HIF) proteins as prognostic markers in chondrosarcoma and the relationship of HIF to the biological characteristics of cartilage tumours. The expression of HIF-1α, HIF-2α, proliferating cell nuclear antigen (PCNA) and microvessel density (MVD) were measured immunohistochemically in 29 specimens of cartilage tumour.

There was no HIF-1α and HIF-2α staining in any of the nine benign cartilage tumours. In 20 specimens of chondrosarcoma, the rate of HIF-1α and HIF-2α expression was 40% and 25%, respectively. The tumour size (≥ 8 cm), histological grade (grade 2 and grade 3) surgical margin (marginal and intralesional) and HIF-1α expression (positive) correlated significantly with a shorter disease-free survival. There was a significant association between HIF-1α and the MVD and a strong trend towards a correlation between HIF-1α and the PCNA index or histological grade.

Our findings suggest that HIF-1α protein may be a useful objective marker in the assessment of the prognosis in chondrosarcoma, since it plays an important role in tumour angiogenesis and cell proliferation.

A giant cell tumour is a primary lesion of bone of intermediate severity. Its histogenesis is unclear. In a few cases pulmonary metastases have been described. Multiple skeletal metastases in the absence of sarcomatous change have been observed.

We present a case report of a 25-year-old woman with a recurrent giant cell tumour of the distal fibula. After a second recurrence and six years after the initial diagnosis, she rapidly developed multiple bony metastases. The outcome was fatal.

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.

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.

Primary giant-cell tumour of soft tissue arising in the ligamentum teres has not been previously described. We report a case of such a tumour in a 46-year-old woman. The lesion was only detected at the time of hip arthroscopy despite pre-operative MRI being performed. It was successfully excised arthroscopically with resolution of the symptoms.

Short intense electrical pulses transiently increase the permeability of the cell membrane, an effect known as electroporation. This can be combined with antiblastic drugs for ablation of tumours of the skin and subcutaneous tissue. The aim of this study was to test the efficacy of electroporation when applied to bone and to understand whether the presence of mineralised trabeculae would affect the capability of the electric field to porate the membrane of bone cells.

Different levels of electrical field were applied to the femoral bone of rabbits. The field distribution and modelling were simulated by computer. Specimens of bone from treated and control rabbits were obtained for histology, histomorphometry and biomechanical testing.

After seven days, the area of ablation had increased in line with the number of pulses and/or with the amplitude of the electrical field applied. The osteogenic activity in the ablated area had recovered by 30 days. Biomechanical testing showed structural integrity of the bone at both times.

Electroporation using the appropriate combination of voltage and pulses induced ablation of bone cells without affecting the recovery of osteogenic activity. It can be an effective treatment in bone and when used in combination with drugs, an option for the treatment of metastases.

Internal hemipelvectomy is a standard treatment for malignant tumours of the pelvis. Reconstruction using a non-vascularised fibular graft is relatively straightforward compared to other techniques. We describe the surgical and functional outcomes for a series of ten patients who underwent an internal hemipelvectomy (type I or I/IV) with reconstruction by a non-vascularised fibular graft between 1996 and 2009. A key prerequisite for this procedure was a preserved sciatic notch, confirmed pre-operatively on MRI.

Graft-host union was achieved in all patients with a single fibular graft, and in the lower graft where two grafts had been used. The mean time to union was 7.3 months (3 to 12). The upper graft did not unite in four of six cases where two grafts had been used. Seven patients were eventually able to walk without a stick. The mean post-operative Musculoskeletal Tumour Society score was 75.4% (16.7 to 96.7). There were no cases of deep post-operative infection. The mean pelvic shortening was 0.9 cm (0.2 to 3.4). Recurrent tumour occurred in three cases, and death from tumour-related disease occured in one.

Patients who need an internal hemipelvectomy will do well if their pelvic ring is reconstructed with a non-vascularised fibular graft. The complication rate is low, and they attain a good functional outcome.

Elastofibroma dorsi is an uncommon, benign, slow-growing soft-tissue tumour of uncertain aetiology. It classically presents as an ill-defined mass at the inferior pole of the scapula with symptoms which include swelling, discomfort, snapping, stiffness and occasionally pain.

We report the symptoms, function and outcome after treatment of 21 elastofibromas in 15 patients. All were diagnosed by MRI and early in the series four also underwent CT-guided biopsy to confirm the diagnosis. In all, 18 tumours were excised and three were observed. After excision, the mean visual analogue score for pain decreased from 4.6 (0 to 10) pre-operatively to 2.4 (0 to 8) post-operatively (p = 0.04). The mean shoulder function, at a mean follow-up of 4.2 years (3 months to 16 years), was 78.1% (30 to 100) using the Stanmore percentage of normal shoulder assessment scoring system. The mean range of forward flexion improved from 135° (70° to 180°) to 166° (100° to 180°) after excision (p = 0.005). In four patients a post-operative haematoma formed; one required evacuation. Three patients developed a post-operative seroma requiring needle aspiration and one developed a superficial infection which was treated with antibiotics.

Our findings support previous reports suggesting that a pre-operative tissue diagnosis is not necessary in most cases since the lesion can be confidently diagnosed by MRI, when interpreted in the light of appropriate clinical findings. Surgical excision in symptomatic patients, is helpful.

It has been suggested that elastofibroma is caused by a local tissue reaction and is not a true neoplastic process. A strong association has been noted between elastofibroma and repetitive use of the shoulder, which is supported by our findings.

We undertook a study of the anti-tumour effects of hyperthermia, delivered via magnetite cationic liposomes (MCLs), on local tumours and lung metastases in a mouse model of osteosarcoma. MCLs were injected into subcutaneous osteosarcomas (LM8) and subjected to an alternating magnetic field which induced a heating effect in MCLs. A control group of mice with tumours received MCLs but were not exposed to an AMF. A further group of mice with tumours were exposed to an AMF but had not been treated with MCLs. The distribution of MCLs and local and lung metastases was evaluated histologically. The weight and volume of local tumours and the number of lung metastases were determined. Expression of heat shock protein 70 was evaluated immunohistologically. Hyperthermia using MCLs effectively heated the targeted tumour to 45°C. The mean weight of the local tumour was significantly suppressed in the hyperthermia group (p = 0.013). The mice subjected to hyperthermia had significantly fewer lung metastases than the control mice (p = 0.005). Heat shock protein 70 was expressed in tumours treated with hyperthermia, but was not found in those tumours not exposed to hyperthermia.

The results demonstrate a significant effect of hyperthermia on local tumours and reduces their potential to metastasise to the lung.

Bone loss secondary to primary or metastatic lesions of the proximal humerus remains a challenging surgical problem. Options include preservation of the joint with stabilisation using internal fixation or resection of the tumour with prosthetic replacement. Resection of the proximal humerus often includes the greater tuberosity and adjacent diaphysis, which may result in poor function secondary to loss of the rotator cuff and/or deltoid function. Preservation of the joint with internal fixation may reduce the time in hospital and peri-operative morbidity compared with joint replacement, and result in a better functional outcome. We included 32 patients with pathological fractures of the proximal humerus in this study. Functional and radiological assessments were performed. At a mean follow-up of 17.6 months (8 to 61) there was no radiological evidence of failure of fixation. The mean revised musculoskeletal Tumour Society functional score was 94.6% (86% to 99%). There was recurrent tumour requiring further surgery in four patients (12.5%). Of the 22 patients who were employed prior to presentation all returned to work without restrictions.

The use of a locking plate combined with augmentation with cement extends the indications for salvage of the proximal humerus with good function in patients with pathological and impending pathological fractures.

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 describe a patient with insufficient bone regeneration of the tibia after bone transport over an intramedullary nail, in whom union was ultimately achieved after exchange nailing and intramedullary application of rh-bone morphogenetic protein-7 at the site of distraction.