The aim was to identify when primary amputation was used as primary treatment and to describe outcomes in patients managed with modern chemotherapy. A detailed review of the electronic patient records was undertaken. Statistical analysis was performed with univariate analysis using Kaplan-Meier curves and Chi² testing, whilst multivariate analysis was performed using Cox regression analysis.

There were 354 osteosarcomas. 93 patients presented with metastases and 192 subsequently developed metastases at a mean of 46 months. Amputation was performed as the primary surgical treatment in 101 patients. Endoprosthetic reconstruction was used in 253 patients. Amputation was performed as a secondary procedure on 15 patients.

The 5 and 10 year survival data for all patients, including those with metastatic disease were 60% and 60% for amputation with good chemotherapy response (>89% necrosis), 65% and 63% for limb salvage and good response, 21% and 21% for amputation and poor response (⋋90% necrosis) and 51% and 30% for limb salvage with poor response.

Local recurrence occurred both with amputation (10.8%) or limb salvage (9%), with no significant differences between the two.

Univariate analysis demonstrated that the extent of response to chemotherapy induced necrosis significantly affected survival, whether the patient had an amputation or not.

Whether or not amputation or LSS was used in the surgical management of patients, local recurrence rates where similar between the two groups. Further assessment of chemotherapy-induced necrosis is a key factor in determining subsequent limb salvage or amputation management strategies.

Sixty eight consecutive patients underwent proximal humeral replacement with a fixed fulcrum massive endoprosthesis for tumour between 1997 and 2007. The mean age was 46 years (7-87). Ten patients were lost to follow up and 16 patients died. The 42 surviving patients were assessed using the Musculoskeletal Tumour Society (MSTS) Score and the Toronto Extremity Salvage Score (TESS). The mean MSTS score was 72.3 % and the mean TESS was 77.2 %.

Four of 42 patients received a new constrained humeral liner to reduce the risk of dislocation. This sub group of 4 patients had a mean MSTS score of 77.7 % and a mean TESS of 80.0%.

Endoprosthetic replacement for tumour of the proximal humerus using this prosthesis is a reliable operation yielding good functional results without the documented problems of unconstrained prostheses. The performance of this prosthesis is expected to improve further with the new constrained humeral liner.

Introduction

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.

Introduction

Fibromatosis is a disorder characterised by a spectrum of biological behaviour from relative indolence to aggressive local infiltration. With aimed to describe the pre and post-operative functional status of these patients managed with surgery and analyse the effect of radiotherapy on functional outcome.

Introduction

Primary bone tumours of the distal radius are rare, while it remains the third commonest site for primary lesions and recurrences of Giant Cell Tumours (GCT). The functional demands on the hand make reconstruction of the wrist joint following the excision of distal radius, particularly challenging.

Objective

To evaluate the rise in impedance during percutaneous radiofrequency thermo coagulation (PRFTC) of osteoid osteomas as a predictor of local recurrence.

Introduction

It is generally held that blood tests are not particularly helpful in establishing the diagnosis of bone tumours but may be useful in prognosis. We reviewed the results of blood tests taken at the time of diagnosis to establish the frequency of abnormalities in common blood tests and whether this was significant in staging or prognosis.

We analysed the outcome of patients with primary non-metastatic diaphyseal sarcomas who had en bloc resection with preservation of the adjoining joints and reconstruction with re-implantation of sterilised tumour bone after extracorporeal radiation (50 Gy). Between March 2005 and September 2009, 32 patients (16 Ewing’s sarcoma and 16 osteogenic sarcoma) with a mean age of 15 years (2 to 35) underwent this procedure. The femur was the most common site in 17 patients, followed by the tibia in 11, humerus in three and ulna in one. The mean resected length of bone was 19 cm (10 to 26). A total of 31 patients were available at a mean follow-up of 34 months (12 to 74). The mean time to union for all osteotomy sites was 7.3 months (3 to 28): metaphyseal osteotomy sites united quicker than diaphyseal osteotomy sites (5.8 months (3 to 10) versus 9.5 months (4 to 28)). There were three local recurrences, all in soft-tissue away from irradiated graft. At the time of final follow-up, 19 patients were free of disease, one was alive with disease and 11 had died of disease. The mean Musculoskeletal Tumor Society Score for 29 patients evaluated at the last follow-up was 26 (9 to 30).

Extracorporeal irradiation is an oncologically safe and inexpensive technique for limb salvage in diaphyseal sarcomas and has good functional results.

Fibromatosis represent a highly heterogeneous group of tumours in growth pattern, location and management. Our aim was to describe the demographics of the patient population who had undergone surgical resection and to identify predictors of local recurrence. Any lesion that was infiltrating the chest or abdominal cavity was excluded. Patients were also not included if they had a plantar or palmar lesions or had received hormonal or chemotherapy.

67 men and 88 women aged from 16 to 77 with a median age of 39 were analyzed. 121 patients had no prior resective operative intervention. 34 patients had undergone an attempted resection procedure at another unit of which 30 had locally recurred. 3 were located in the abdominal wall, 5 chest wall, 15 paraspinal, 56 lower and 76 upper limb. 40 patients did not receive XRT, 18 in the post-operative period and 97 in the pre-operative period. 67 operations produced margin negative resection, 85 were positive and 3 in which the margin status was unknown. Follow-up ranged from 1 day post op to 23.3 years. 23 patients had a local recurrence. Following subsequent re-resections, the total number of patients who were alive with evidence of disease was 16.

6 pts had deceased. 149 were alive with no evidence of disease. No factors were found to be statistically significant for predicting local recurrence, including the use of radiation (0.06) and margin status (0.81). Although radiation, given either pre or post-operatively did trend towards preventing local recurrence (HR 0.40; 95% CI 0.15 to 1.06; p = 0.06).

Introduction

Enchondromas located in the phalangeal bones may be more cellular than non-digital locations necessitating clinical and radiological correlation to determine diagnosis. Atypical enchondromas have increased cellularity and atypia relative to simple enchondromas but no evidence of permeation. Chondrosarcomas of the phalanges are thought to have a more indolent course than chondrosarcomas in other locations. The aim of the study was to determine the outcome of atypical enchondromas and grade 1 chondrosarcomas of the phalanges treated surgically.

Introduction

A GP may only encounter one sarcoma in their professional career. Early diagnosis and treatment will improve the outcomes of this rare and malignant disease. Guidelines designed to lead to earlier diagnosis of the most common cancers were introduced by NICE in 2000 and were updated in 2005. These advise and assist in the early referral of potential bone and soft tissue sarcoma. Prior to 2000 only ~10% of GP referrals were proven to be malignant.

Earlier diagnosis is one of the key aims in achieving improved outcomes for patients with cancer. In general, the earlier a tumour is diagnosed, the easier it will be to treat and the greater the chance of cure. We have investigated how tumour size at diagnosis and duration of symptoms, both of which may act as a proxy for delay in diagnosis have varied over a 25 year period and whether there is evidence of improvement.

Data were available for 2568 patients with primary bone sarcomas and 2366 with soft tissue sarcomas. The mean size at diagnosis was 10.7 cm for bone tumours and 9.9cm for soft tissue sarcomas. The size of bone sarcomas had not changed with the passage of time but there had been a slight decrease in the size of soft tissue sarcomas (10.3 cm before 2000 vs 9.6cm after 2000, p=0.03). The duration of symptoms reported by patients varied widely with a median of 16 weeks for bone sarcomas and 26 weeks for soft tissue sarcomas. The median duration of symptoms for bone sarcomas had actually increased since 2000 (16 weeks before to 20 weeks after 2000, p⋋0.01), whilst it remained unchanged for soft tissue sarcomas. Further analysis showed that females tended to present with smaller tumours than males and that slower growing tumours (eg. liposarcoma and chondrosarcoma) tended to be larger and have a longer duration of symptoms than other tumours. 15% of patients with a soft tissue sarcoma had undergone a previous inadvertent excision – and this % has not changed over 20 years. Younger patients had smaller soft tissue soft tissue sarcomas than older patients but there was little difference for bone sarcomas.

Introduction

Our Unit has been treating large volume soft tissue sarcomas involving the sciatic nerve with epineurectomy for over a decade. The aim of this study was to quantify the functional outcome of patients who were known to have sciatic nerve involvement pre-operatively and went on to have nerve preserving surgery utilising a planned marginal excision with epineurectomy.

Periarticular metastasis may be treated with endoprosthetic reconstruction. The extensive surgery required may not, however, be appropriate for all patients. Our aim was to establish if the outcome of locking plate fixation in selected patients with periarticular metastases.

Prospective data collection was performed. Twenty one patients underwent surgery for periarticular metastatic tumours. The median duration of follow-up for surviving patients was one year. There have been no cases of implant failure and no requirement for revision surgery. Pain relief was excellent or good in the majority of patients. Patients who had sustained a fracture prior to fixation had restoration of their WHO performance status. All patients had a dramatic improvement in their MSTS scores. The median pre-operative score was 15% (0%-37%) improving to a median score of 80% (75% -96%) post operatively.

Locking plates provide reliable fixation and excellent functional restoration in selected patients suffering from periarticular metastatic bone disease.

Chondrosarcoma of bone is a surgical disease and excision with wide margins is the optimum treatment. Sometimes the size or location of the tumour at the time of diagnosis mean that only a marginal excision can be achieved. The effect of the margin of excision on outcome is investigated.

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 simultaneous use of a vascularised fibular graft to protect the allograft from mechanical complications improved the outcome, but the use of intramedullary cementing was not as successful.

In order to improve the strength of the reconstruction and to advance the biology of host–graft integration, we suggest avoiding the use of intramedullary nails and titanium plates, but instead using stainless steel plates, as these gave better results. The use of a supplementary vascularised fibular graft should be strongly considered in adult patients with resection > 17 cm and in those who require post-operative chemotherapy.

Aim

To present selective arterial embolization with N-2-butyl Cyanoacrylate for the palliative and/or adjuvant treatment of painful bone metastases not primarily amenable to surgery.

Aim and purpose

The clinical management of osteosarcoma differs significantly from that of chondrosarcoma;

Therefore it is extremely important to diagnose these two types of bone tumour accurately. In the absence of a specific marker, differential diagnosis by histochemistry is sometimes impossible, especially between chondroblastic osteosarcoma and conventional chondrosarcoma. The aim of the study was to find an useful diagnostic marker, simple to use for distinguishes chondroblastic osteosarcoma from conventional chondrosarcoma.

Aim

We investigated low grade intramedullary chondrosarcomas to see if curettage and cementation remains a strong alternative treatment to local resection.

Introduction

We analyzed the results of extracorporeal radiated (ECRT) autogenous tumour bone for reconstruction of diaphyseal defects after tumour resection at our institute.

Aim

To identify factors leading to the success or failure of extracorporeal irradiation and re-implantation of bone (ECIR).

The purpose of this study was to compare the outcome, complications and survival of the three most commonly used surgical reconstructions of the proximal humerus in adult patients after trans-articular tumour resection.

In proximal humerus reconstructions, osteoarticular massive allograft (OA) allows a biologic reattachment of tendineous structures of the host providing stability and functional recovery. Allograft-prosthesis composites (APC) are more technically demanding, but functional advantages with new prosthetic design are expected with lower fracture and failure rate with time. We compared the outcome of patients treated with OA reconstruction (group A) and patients treated with an APC (group B) after humerus resection for bone tumours.

Thirty six patients treated between 1996 and 2009 were included in the study (18 group A; 18 group B). The diagnosis was a malignant bone tumour in 28 cases, an aggressive benign bone tumour in 6 and a bone metastasis in 2. Three total humerus resections were performed (two in group A and one in group B). Three different type of prosthesis were used (conventional, reverse and resurfacing). In group A, at an average follow-up of 66 months (2-116), 16 patients were continuously disease free.

Ten patients (56%) had no complication; in 6 cases (33%) we observed a fracture of the graft and in 3 cases (17%) a pseudoarthrosis. In group B, at an average follow up of 44 months (3-164), 16 patients were continuously disease free.

Thirteen patients (72%) had no complication, in 2 cases (11%) stem loosening, in 2 cases (11%) dislocation, in 1 case pseudoarthrosis and in 1 case resorption and detachment of allograft's great tuberosity was observed. Functional outcome was evaluated using MSTS scoring system: a mean score of 24 (13-30) in group A and 23 (15-27) in group B was observed.

In our experience, proximal humerus APC showed functional results comparable to OA allograft with lower complication rate.

In conclusion, in biologic reconstruction of the proximal humerus, APC seems to provide a more reliable solution than OA allograft at long term

Aim

To determine the overall survival of patients with Pelvic Ewing's Sarcoma treated in our unit and to identify prognostic factors in pelvic primaries that could be used to select patients who would most likely benefit from high intensity treatment.

Aim

The use of megaprostheses for knee reconstruction after distal femur resection in young bone sarcoma patients has become popular since early ′80. The authors reviewed their experience with different distal femur megaprostheses in children.

Aim

Improving the quality of clinical and radiologic differential diagnosis of intramedullary tumours of long bones.

Aim

Preoperative serum CRP has been identified as an independent predictor in various malignancies. For osteosarcoma, however, the value of serological markers is unreliable. Aim of this study was to evaluate the prognostic power of preoperative CRP in patients with osteosarcoma.

Aim

Patients treated with limb salvage surgery for bone sarcomas of the extremities (upper and lower) may have physical disability as a result of treatment. Goal of this study was to evaluate the quality of life after treatment (chemotherapy and conservative surgery) at long term.

Introduction

The aim of this study was to investigate the results of a series of cases from a single institution with respect to local disease control and patient survival to determine prognostic factors.

The rising incidence of atraumatic fractures in patients either with Ewing's sarcoma or osteosarcoma years after chemotherapy revealed a growing population of childhood cancer survivors with a decreased bone mineral density (BMD) possibly due to a long-term effect of the chemotherapy. Therefore we started to screen our patients below 50y of age who were treated for bone malignancies between 1994 and 2009.

The first series of measurements included 15 patients – eight Ewing's sarcoma, three female and five male, with a mean age of 18y (±13SD), and seven osteosarcoma, two female and five male, with a mean age of 19y(±9SD). We screened the patients for deficits in their bone status using DEXA (dual-energy-x-ray-absorptiometry) to gain the T-and Z-Scores of the proximal femur and the lumbal spine. Additionally we took blood samples for endocrinological analysis and utilised a questionnaire to scan the patient's liefestyle. The mean time between diagnosis and investigation was 95months (±79SD) in Ewing's sarcoma and 105months (±54 SD) in osteosarcoma.

The results of the age and gender matched lumbal measurement (Z-Score) of the Ewing's sarcoma patients showed a reduction of the BMD in six cases (6/8), including three times osteopenia (3/8) and two times osteoporosis (2/8). The osteosarcoma patients presented a BMD-decline in four cases (4/7) with two times osteopenia (2/7) and one osteoporosis (1/7).

In the proximal femur six Ewing's sarcoma (6/8) and six osteosarcoma patients (6/7) showed a BMD-decrease including three osteopenic (3/8) and one osteoporotic (1/8) Ewing's sarcoma and four osteopenic osteosarcoma (4/7). We found two cases of pathologic fractures (2/15), one Ewing's sarcoma 29 months after diagnosis with a fracture of the distal femur and the proximal Tibia (1/8) and one osteosarcoma with a fractured distal femur after 72 months (1/7).

As presented in our case series osteoporosis after chemotherapy is an underestimated long-term effect of the chemotherapeutic treatment. In our series BMD-reduction seems to be independent of tumour-type and chemotherapeutic agent like MTX.

Early diagnosis is assumed to improve patient survival, but whether symptom interval (SI i.e. the period between the onset of the first symptoms signs of the disease and its definitive diagnosis) has significant impact on outcome or not remain unclear.

Since1986 we have monitored the actual diagnosis of all cases referred to our Unit with the diagnosis of ‘possible primary malignant bone tumour’. We have excluded all patients referred with a known diagnosis of either a benign condition or known to have bone metastases. In most cases the suspected diagnosis was based on X-rays alone, sometimes supported by further imaging.

Aim

Multiple (hereditary) osteochondroma (MO) is a rare autosomal dominant disease. Previous reports show that the risk of a malignant degeneration varies between 5-25%, but these are often combined with data on other cartilaginous diseases. The aim of this study was to establish clinical and radiological parameters that could identify a group of MO patients who are at risk for peripheral chondrosarcoma.

Background

Angiosarcoma is a rare complication of breast cancer treatment. In order to define predictors, clinical presentation, and outcome, we characterized a population-based 50-year cohort of angiosarcomas after breast cancer.

CT guided percutaneous radiofrequency thermocoagulation is the treatment of choice for osteoid osteomas. Good results with a low complication rate have been shown for spinal lesions. When lesions are within close proximity to neurological structures or if patients have radicular symptoms surgery rather than radiofrequency ablation has been advocated. We present our experience of radiofrequency ablation of spinal osteoid osteomas which are less than 5mm from neurological structures, including those causing radicular symptoms.

Data was collected prospectively on all patients with a spinal osteoid osteoma within 5mm of nerve roots or the spinal cord as measured on CT scanning.

There were nine patients, five female and four male with a mean age of 15 years. Four tumours were located in the thoracic spine, three in the lumbar spine and two in the cervical spine. The mean distance to the nearest neurological structure was 3mm. Radicular symptoms were present in two patients. The mean number of probe positions used was two. Lesions were heated to 90 degrees for 5 minutes for each probe position. There were two cases of recurrence, both treated successfully with one further procedure each. There were no cases of neurological injury. The two patients with radicular symptoms had full resolution of their symptoms. At a mean follow up of 2 years following treatment all patients are asymptomatic.

Radiofrequency ablation can be safely performed to treat osteoid osteomas located within 5mm of neurological structures and has a low rate of recurrence.

Aim

To study the efficacy of Zoledronic acid in the treatment of benign osteolytic tumours or tumour like conditions of bone as a therapeutic or as an adjuvant agent

Prognostic stratification of patients with non-metastatic osteosarcoma may improve the clinical management and the design of clinical trials.

Data from 773 patients [median age 15 years (3-40)] treated at our Institute from 1983 to 2000 with high-dose methotrexate, cisplatin, doxorubicin and ifosfamide (neoadjuvant chemotherapy) were analyzed. After multivariate analysis including age, site, tumour volume (cut-off 200 mL), serum LDH and Alkaline Phosphatase (SAP), histology (osteoblastic and chondroblastic vs others), high LDH and SAP, osteoblastic and chondroblastic histotypes resulted independent prognostic factors of DFS.

Patients were grouped according to a score from 0 (absence) to 3 (one to 3 adverse factors). The scoring system was implemented by the addition of PgP expression and grade of chemotherapy-induced necrosis.

A score of 0, 1, 2, 3 was given to 14%, 38%, 32% and 16% of patients respectively.10-year DFS was 80% (95%CI 72-89) for score of 0, 58% (95%CI 52-64) for 1, 53% (95%CI 46-59) for 2 and 40% (95%CI 32-50) for 3 (p= 0.001). PgP expression (168 patients) identified patients with 100% probability of DFS (score of 0 and negative PgP) and patients with 18% (95%CI 52-64) DFS (score of 3 and positive PgP).

Good (GR) and poor responder (PR) patients had the same probability of DFS in case of score of 0 [GR82% (95% CI 72-91), PR79% (95% CI 65-93)] and score of 3 [GR43% (95% CI 32-55) PR36% (95% CI 21-51)]. Different probability of DFS in case of score of 1 [GR64% (95% CI 57-72) PR47% (95% CI 36-59)] and score of 2 [GR63% (95% CI 55-71) PR36% (95% CI 21-51)].

It is possible to stratify outcomes of patients with non metastatic osteosarcoma of the extremity by means of a simple score based on easily available clinical parameters. This scoring system is worth to be validated on larger series.

Aim

In osteosarcoma, local control of the tumour is absolutely critical otherwise the chances of long term survival are <10% and may effectively approach zero. Radiotherapy is used in case of non-resectable tumours. Histone deacetylase inhibitors (HDACIs) can enhance the sensitivity of cells to photon radiation (XRT) by altering numerous molecular pathways. Therefore, we investigated the effect of the pan-HDAC inhibitor suberoylanilide hydroxamic acid (SAHA) on radiation response in osteosarcoma (OS) and rhabdomyosarcoma (RMS) cell lines.

Neoadjuvant chemotherapy for osteosarcoma improves outcomes for the majority, but if the chemotherapy does not work then the dilemma often arises as to whether to do limb salvage with a marginal (or worse) margin of excision or to do an amputation. If limb salvage is carried out with a close margin, does post operative radiotherapy make any difference? This study aims to address these questions.

For many surgeons amputation is the usual treatment in sarcoma of the foot. The aim of our study was to report the functional and oncologic results of treatment in 54 sarcomas of the foot to assess if conservative treatment was acceptable

We retrospectively reviewed the records of 54 patients with sarcomas of the foot, aged 6 to 50 (mean 17), 30 females and 26 males. At time of referral, 18 had a local recurrence of a previous inadequate treatment. There were 27 soft tissue sarcomas (STS: 10 synovial sarcomas, 6 rhadomyosarcomas, 1 liposarcomas and 10 others) and 27 bone tumours (16 Ewing's, 8 chondrosarcomas, 3 osteosarcomas). Toes tumours were excluded, 18 tumours involved the metatarsal, 12 the plantar soft tissues, 11 the calcaneum, 3 the talus, 2 the midtarsal bones.

Surgery consisted in 19 resection without reconstruction, 21 resections with bone reconstruction, 9 partial amputations of the foot, and 6 trans tibial amputations.

In 34 cases surgical margins were adequate (R0), in 13 patients resection was inadequate (9 R1 and 4 R2). In 7 cases the margins were not assessed.

After a 5.5 years average follow-up (3m to 17y), 31 patients had no evidence of disease, 8 were in second remission, 4 had an evolutive disease and 11 were deceased. The mean MSTS score was 26/30 (31 cases).

In conclusion, a conservative treatment is feasible in metatarsal bones with skin coverage by flap if necessary. In STS adequate margins are difficult to achieve with a high rate of local recurrence. In calcaneus and talus, a conservative treatment is possible in tumours limited to bone after good response to chemotherapy. In other cases conservative treatment is debatable because amputation gives excellent functional results.

The outcome for patients with Ewing's sarcoma recurrence is poor. Local recurrences occur in 8%-25%of these patients. The aim of the study was to analyze the patients who had a local recurrence to identify factors predicting the local recurrence and if it could be prevented

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.

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

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

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.