Several methods have been used for proximal humeral reconstruction following tumour resection. None of these modalities allow the patient to regain his normal shoulder range of motion. Moreover, every modality has its advantages and disadvantages.

The aim of this study was to compare the functional outcome of 2 reconstructive modalities that we are using in our institution for proximal humeral reconstruction; endoprosthesis and shoulder arthrodesis using a vascularised autograft This study included 48 patients diagnosed with malignant or benign aggressive tumours that required resection of their proximal humerus. They were divided into 2 groups according to the method of reconstruction. Group 1 included 22 patients with an average age of 20 years were reconstructed by shoulder arthrodesis using a free vascularised fibular graft (6) or a pedicled scapular crest graft (16). Group 2 included 26 patients with an average age of 26 years were reconstructed with an endoprosthesis.

In group 1 the average follow up period was 88 months (range 12 to 184 months). The average functional outcome (according to the MSTS scoring system) was 25 points (range 19 – 28). The average abduction and forward flexion range of motion (scapulothoracic) was 40 degrees (range 20 -60). Complications included failed fixation (2), non union (1), infection (1) and temporary radial nerve palsy (2). In group 2 the average follow up period was 36 months (range 12 – 110). The average functional outcome was 24 points (range 20 – 27). The average abduction and forward flexion range of motion was 40 (range 30 –70). Complications included sublaxation (2), loosening (1) and infection (1).

Reconstruction of the proximal humerus by arthrodesis or endoprosthesis yield similar functional outcome. Although endoprosthesis is a much more expensive modality, it does not provide any superior functional outcome over shoulder arthrodesis.

Aim

The aim of this study was to assess the financial implications of managing skeletal metastases in a tertiary hospital and explore its impact on the provision of acute care trauma services.

Besides conventional chondrosarcoma, several rare chondrosarcoma subtypes are described, comprising about 15% of all chondrosarcomas. Clear cell chondrosarcoma (CCS) is a low-grade malignant tumour, often recurring after curettage, and showing overall survival of about 85%. Mesenchymal chondrosarcoma (MCS) is a highly malignant tumour occurring in bone and soft tissue of relatively young patients. The tumour shows differentiated cartilage mixed with undifferentiated small round cells. It often metastasises and shows a 5-year overall survival of 55%. Dedifferentiated chondrosarcoma (DDCS) is a tumour containing a high-grade non-cartilaginous sarcoma (DD), and a usually low-grade malignant cartilage-forming tumour (WD).

The prognosis is poor. The lack of efficacious treatment of these rare tumours emphasises the need to learn more about their characteristics and to unravel potential targets for therapy.

We constructed tissue microarrays (TMAs) with 2mm cores of 45 DDCS (WD and DD), 24 CCS, and 25 MCS, in triplicate.

Using immunohistochemistry, we investigated protein expression of estrogen-signaling molecules, growth plate-signaling molecules, and other molecules which might be potential targets for therapy. In addition, we gathered genomic information using Agilent 44K oligo arrays.

30% of the WD components were positive for Cox-2. Almost all others were negative. For Bcl2, 88% of the small cells and 32% of the cartilage in MCS were positive. In CCS, WD, and DD 48%, 4%, and 12% were positive, respectively. We demonstrated the presence of ESR1 and aromatase protein in the majority of tumours in all subtypes. Using array CGH, we observed similar aberrations in the two components of DDCS, with additional aberrations in the DD.

Celecoxib treatment is not recommended, as most of the tumours are negative for Cox-2. However, the presence of ESR1 and aromatase support a possible effect of anti-estrogen treatment in all subtypes, and application of Bcl2 inhibitors might chemosensitise MCS.

Aim

Assess the oncological and clinical outcomes associated with intralesional curettage, phenol and bone grafting of the lesions.

Aim

Giant cell tumour (GCT) of bone is a benign but locally aggressive tumour. Although topical adjuvants have been used in the past, local recurrence following intralesional excision of GCT of bone continues to remain a problem. The use of bisphosphonates as an anti-osteoclastic agent in the management of osteolytic bone metastases is well accepted. Therefore our study aims to retrospectively demonstrate whether the administration of bisphosphonate as an adjuvant can control aggressive local recurrence of GCT and prevent wide resections of bones or amputations.

Aim

To determine whether delayed diagnosis (lapse from initial symptoms to the beginning of treatment) has influence on the possibilities of crossing the physis by the tumour, and/or on the outcome in pediatric patients with high grade metaphyseal osteosarcoma.

Aim

To review the first 50 cases, looking at survivorship of the irradiated autograft, complications and functional outcomes in a wide range of bony malignancies and anatomical locations.

Bone and Soft Tissue Sarcomas represent approximately 1% of all malignant tumours. Delays in diagnosis are frequent and the average size of Sarcomas at diagnosis has averaged 10cm for many years. In 1999 guidance was produced by NICE with the aim of leading to the earlier diagnosis of common cancers – including Sarcomas. We have attempted to analyze whether this guidance has had any impact on either the size of the tumours at diagnosis or the symptom duration prior to diagnosis experienced by the patients.

Data for patients referred to the Royal Orthopaedic Hospital in Birmingham between 1992 and 2007 with Bone Sarcomas (n=1592) and Soft Tissue Sarcomas (n=2004) were analysed to determine the effect of the guidance. For Bone Sarcomas the mean size of the tumours decreased from 11.2cm prior to the guidance to 10.7cm after the guidance but the change was not statistically significant (p=0.09). The mean duration of symptoms increased from 18 to 21.2 weeks (p=0.01). For Soft Tissue Sarcomas, mean size fell from 10.8cm to 9.5cm (p<0.001), however the duration of symptoms actually increased from 27.3 to 32.1 weeks (p=0.01). Statistical modelling using restricted cubic splines confirmed these trends in the data.

These results show that whilst there may have been a slight improvement in the size at diagnosis of Soft Tissue Sarcomas, overall most patients still experience a long delay between the onset of symptoms and diagnosis and commencement of treatment. It is difficult to conclude that the early diagnosis guidance produced in 1999 has had a significant effect on the basis of this study. Strategies to improve awareness of the symptoms and clinical features of Bone and Soft Tissue Sarcomas are still urgently required.

Aim

While the association of surgery and radiation therapy in high grade Soft Tissue Sarcoma (STS) of extremities is considered the “golden standard”, there is not international agreement regarding type, timing, overall dose of radiation, and size, site and histology of tumours to be irradiated. A similar consideration is about low grade STS. The aim of our paper is critically reconsider our experience, trough a retrospective analysis of 15 years experience. This in order to propose a perspective protocol of treatment of high and low grade STS, in order to minimize the late complication rate.

Aim

was to analyze infections after bone tumour surgery.

Aim

Accurate and reliable patient information plays a crucial role in the multidisciplinary treatment of malignancies helping to ensure compliance of the patients and their relatives with often long-lasting and stressful treatment. The English version of the online encyclopaedia Wikipedia has been recently reported to be the prominent source of online health information. However, there is little information concerning the quality of information found in Wikipedia.

Aim

Local recurrence after surgery of soft tissue sarcomas is dependent on surgical margins. Wide margins require large resections which may lead to impaired function or loss of limb. In some cases it may be technical impossible or even ethical unacceptable to achieve ideal surgical margins. Standard adjuvant treatment in such cases is ionising radiation, which may cause severe toxic side effects.

PCI is a unique procedure for site-specific delivery of several types of membrane impermeable molecules. The technology is based on the photochemical induced cytosolic release of endocytosed macromolecules from endosomes and lysosomes into the cytosol. PCI has in this study been evaluated as an adjuvant to the surgical resection of sarcoma.

SSX was initially identified as a melanoma associated tumour antigen MEL2 and in the SS18/SSX fusion gene of synovial sarcoma. It consists of a family of nine, highly homologous X chromosome genes, being SSX1, SSX2 and SSX4 the most commonly expressed in tumours. In normal tissue, SSX expression is restricted to germ cells, trophoblasts, and mesenchymal stem cells. In malignant cells, SSX expression is over-represented in sarcomas. SSX expression is epigenetically regulated by methylation and histone deacetylation.

Aim

The percentage of adolescents and young adults with sarcoma enrolled in multicenter clinical trials is reportedly much lower than that of younger children. We intended to determine if this remained true despite the availability of a study open to patients up to the age of 40 years

Introduction

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.

Expandable prostheses were designed to allow progressive growth after tumour resection in children. The aim of this study was to report the late results of the non-invasive growing prostheses designed by A Soubeyran (Phenix prosthesis or Wright Repiphysis).

From 1994 to January 2006, 27 children aged 4 to 12 (mean 8.5), underwent a resection of the knee for a bone tumour, with reconstruction by a non invasive expandable prosthesis. There were 16 boys and 11 girls. The tumours were 25 osteosarcomas and 2 Ewing tumours. All patients received pre and post-operative chemotherapy. There were 18 distal femur, 7 proximal tibia, and 2 femur + tibia resections.

There were different successive designs based on the same electro-magnetic growing mechanism using a pre bent spring, released by eating in an induction coil.

After, 7.2 years mean follow-up (4 months to 15 years), 20 patients had no evidence of disease and 7 were deceased. Two with a local recurrence were amputated. Mean lengthening was 5.1 centimeters (0 to 8), after 3 to 11 lengthening procedures. Mean limb-length discrepancy was 1.8 cm. Two patients had a secondary infection. Eleven had a revision for arthrofibrosis. All surviving patients were revised to a conventional hinged prosthesis. The mean MSTS functional score of the definitive prosthesis was 82% (63 to 96%).

Theses prostheses showed many mechanical complications as loosening, fracture of the growing mechanism, and arthrofibrosis. The positive outcome was the possibility to perform a progressive lengthening, without surgery limiting the risk of infection. Theses prostheses should be considered as temporary until reconstruction with a conventional hinged prosthesis. Patients with multiple revisions had a tendency to show less favourable late functional results than with primary implanted hinged prosthesis.

Aim

The first osseointegrated transfemoral amputation prosthesis operation was performed in Gothenburg in 1990. The aim is improving quality of life for patients who cannot use conventional socket prosthesis. In 1999 the prospective OPRA-study (Osseointegrated Prosthesis for Rehabiliation of Amputees) was initiated with standardized surgery, equipment and rehabilitation program.

Aim

Synovial sarcoma (SS) is a malignant soft tissue sarcoma with a poor prognosis because of late local recurrence and distant metastases. To our knowledge, no studies have minimum follow-up of 10 years that evaluate long-term outcomes for survivors.

Aim

Purpose of this study was to review a single Institution experience and results of management of extraskeletal osteosarcoma (OGS), with emphasis on the role of combined treatment consisting of surgery and adjuvant chemotherapy.

Chondrosarcomas are malignant hyaline cartilage tumours of bone. They are clinically resistant to conventional chemo- and radiotherapy and the underlying mechanism is poorly studied. Chemoresistance is a multifactorial process and the inaccessibility due to abundant hyaline cartilaginous matrix surrounding the cells, presence of multi-drug resistance pumps, and expression of anti-apoptotic proteins such as BCL2, have been suggested. Our aim was to study chemoresistance mechanisms in chondrosarcoma.

We first studied the sensitivity of chondrosarcoma cell lines (SW1353, CH2879, JJ012, OUMS27) and 2 primary cultures for doxorubicin and cisplatin. We used a 3D pellet model of CH2879 to study doxorubicin incorporation. To investigate whether chondrosarcoma cells could be resensitised to chemotherapy we tested the BH3 mimetic ABT737 inhibiting anti-apoptotic BCL2 family proteins. Cell viability was assessed using a WST assay for mitochondrial activity.

Dose response curves showed that chondrosarcoma cell lines and cultures are partially resistant to doxorubicin, while primary cultures were completely resistant to cisplatin. In 3D cell pellets, with morphology strongly resembling high grade chondrosarcoma, doxorubicin incorporation was confirmed. Chondrosarcoma cells responded to ABT737 with a >60% reduction in cell viability at high concentrations (25μM). Combination treatment allowing 2 days between ABT737 and chemotherapy addition led to a complete reduction of cell viability in all cell cultures.

In conclusion, chondrosarcoma cell lines show a partial response to doxorubicin and less response to cisplatin. The incorporation of doxorubicin in the cells in a 3D pellet model indicates that resistance is not caused by inaccessibility of the cells for the drugs nor by multi-drug resistance pump activity. By combining BCL2 inhibition with Doxorubicin treatment, a complete reduction of cell viability was obtained. This suggests that BCL2 overexpression plays an important role in chemoresistance of chondrosarcoma, and turning on the apoptotic machinery by BCL 2 inhibition can render them chemosensitive.

Background

Cryosurgery is a well established modality in the treatment of benign aggressive and low grade malignant tumours. In this setting it allows for intra-lesional resection and preservation of function without compromising oncological outcome. Here we present the outcome of 87 patients treated with cryosurgery for low-grade chondrosarcoma of bone.

Aim

Bumps and lumps of the hand are a common cause for consultation in general practice. However not all of these lesions are of true neoplastic nature and malignant tumours are a rarity in this location.

Aim

To present our experience and results of percutaneus sclerotherapy of aneurysmal bone cysts (ABC).

Aim

The recent discovery of small non-coding RNAs, so-called micro RNAs (miRNAs), has provided new insights into cancer diagnosis. Several studies have shown that profiles of miRNA expression differ between normal tissue and tumour tissue and vary among tumour types. To exploit this difference, we evaluated the feasibility of using muscle-specific miRNAs (miR-1, 133a, 133b, 206) as biomarkers of rhabdomyosarcoma (RMS).

Retroperitoneal sarcomas (RS) are rare tumours that may reach considerable size at diagnosis and should optimally be treated by a specialized multidisciplinary team. In Sweden, we have since 10 years enforced referral of RS to sarcoma centers, but have experienced a considerable delay, which provides the basis for this study on delays in RS diagnosis and treatment.

We identified 33 patients treated for RS at the Southern Sweden Sarcoma Center (covering a population of 1.5 million), Lund University Hospital between 2003-2009. Data, including onset of symptoms, time to diagnosis and time to treatment were recorded from clinical files. Patient's delay was defined as the time from onset of symptoms to the first visit to a doctor, which could be a general practitioner or a specialist. Doctor's delay was defined as the time from the first visit to a doctor to the start of treatment, which was in most cases surgery.

In total, 30 patients were referred to the sarcoma centre for treatment. Complete data are available from 25 patients (13 men) with a median age of 62 (20-86) years. Median patient's delay was 15 days (0-9 months) and median doctor's delay was 97 days (0-40 months). Median doctor's delay was indeed somewhat longer (52 days) at the sarcoma centre than at the local hospitals (38 days). Some of the longest delays were caused by primary erroneous diagnosis (16 and 40 months) and comorbidity (4, 8 and 19 months) that required other medical procedures before surgery.

Though almost all patients with RS in Southern Sweden are referred to a sarcoma centre for treatment, delays are considerable for many patients with doctor's delays outnumbering patient's delays. Our findings demonstrate that centralisation per se is not sufficient to treat RS, but that optimized diagnostics and clinical management is needed also at sarcoma centers.

Aim

Grade is the most important predictor of the biological behaviour of soft tissue sarcomas. Assigning a pathologic grade is always a difficult task as discordance rate is 30-40% even among experienced sarcoma pathologists. Many of these tumours are heterogeneously large and only small fractions are sampled for biopsy. This emphasizes the need for an objective and accurate assessment of histology. Our aim is to evaluate the role of Choline as a tumour marker in (i) differentiating benign from malignant soft tissue tumour, (ii) to distinguish recurrent/residual tumours using in-vivo MR spectroscopy.

Aim

The aim of this study was to analyze our results using a modular endoprosthetic replacement system (MUTARS) for bone tumours of the proximal humerus.

Aim

The saddle prosthesis was originally developed for reconstruction of large acetabular defects in hip revision arthroplasty. Later on the saddle prosthesis was also used for hip reconstruction after resection of peri-acetabular tumours. In case of patient survival a long-term good hip function is required of the saddle prosthesis. The goal of this study is the measurement of long-term clinical results of saddle prosthesis after reconstruction of peri-acetabular tumours.

Aim

To report late development of sarcomas on sites of previously curetted and grafted benign tumours. Rare cases of development of sarcomas in sites of previous benign lesions are documented, and the development is generally considered secondary to progression of benign lesions, even without radiotherapy.

Introduction

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.

Aim

We present the greatest study of patients with proximal fibula resection. Moreover we describe a new classification system for tumour resection of the proximal fibula independent of the tumour dignity.

Aim

Grade is the most important predictor of the biological behaviour of soft tissue sarcomas. Assigning a pathologic grade is always a difficult task as discordance rate is 30-40% even among experienced sarcoma pathologists. Many of these tumours are heterogeneously large and only small fractions are sampled for biopsy. This emphasizes the need for an objective and accurate assessment of histology. Our aim is to evaluate the role of Choline as a tumour marker in (i) differentiating benign from malignant soft tissue tumour, (ii) to distinguish recurrent/residual tumours using in-vivo MR spectroscopy.

Background

Bone lesions in Ewing's sarcoma (ES/PNET) have been traditionally diagnosed with bone Scan. PET-scan is emerging as a promising investigative modality for detection of metastatic lesions. In this prospective study, we compare the utility of both to detect the metastatic sites.

There is currently no standard follow up protocol for patients who have been diagnosed with and treated for high-grade osteosarcoma. We therefore investigated the possibility of creating a risk based follow-up protocol for patients with primary osteosarcomas.

313 patients diagnosed with primary osteosarcomas were studied. The identified risk factors for local recurrence included poor necrosis, inadequate margins and high risk tumour site in the bone. The risk factors for metastases were poor necrosis, inadequate margins, extra-compartmental stage and tumour size ≥5cm.

The risk of local recurrence and/or metastases within three years of diagnosis increases as the number of risk factors increase. Patients were grouped according to their number of risk factors. The cumulative risk of metastases for patients with 0, 1, 2, 3 and 4 risk factors is 0%, 12%, 21%, 54% and 60% respectively (p=<0.0001). Risk of local recurrence for patients with 0, 1, 2 and 3 risk factors is 5%, 14%, 25% and 20% respectively (p=0.0025).

Our investigation shows that by grouping patients together according to their number of identified risk factors, it is possible to identify groups of patients that are most at risk. This information can be used to design an evidence based follow up protocol which would have important implications for clinical practice.

Introduction

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.

Aim

To evaluate outcome and complications of knee arthrodesis with a modular prosthetic system (MUTARS(r) Implantcast), as primary and revision implants in musculoskeletal oncology.

Aim

To compare the functional outcome of proximal femoral reconstruction using endoprosthetic replacement and hip arthrodesis using a vascularised fibular graft

Background

It is common practice nowadays to treat patients with metastatic epidural spinal cord compression (MESCC) surgically. Extend and type of surgery should be in proper relation to the expected survival time of the patient. It is still difficult to predict patient's survival time and different scoring systems are used. Reliable prediction of survival is mandatory, in that way adjustable surgical treatment can be established.

Aim

Assess symptoms and diagnostic problems of chest wall chondrosarcoma and factors related to long doctor's delay.

Aim

Local treatment of Ewing sarcoma of the hip bones and sacrum remains one of the most difficult tasks in the treatment of bone sarcomas. We investigated the difference between size, local treatment and overall survival in Ewing sarcoma of the sacrum and hip bones.

Aim

To compare two consecutive treatment programs in metastatic osteosarcoma at presentation.

Aim

Ten years ago at the EMSOS 2000 meeting we have presented our experience concerning the non-surgical treatment of stage IIB osteosarcoma of extremities. The purpose of study was to evaluate long-term results and complications related to this non-standard and controversial treatment modality.