The aim of this study is to evaluate the functional and oncological outcome of extracorporeally irradiated autografts as a method of pelvic reconstruction after internal hemipelvectomy. The study included fifteen patients with primary malignant bone tumours of the pelvis. There were 10 males and 5 females with a mean age of 21.5 years (range, 8 to 46 years). Six patients had Ewing's sarcoma, six osteosarcoma, and three chondrosarcoma.Introduction
Methods
The diagnosis and subsequent management of sarcoma often requires multiple separate attendances to Hospital and often a prolonged inpatient stay post surgery. Previous research has focused on the physical and emotional aspects of a diagnosis of cancer, but little has been discussed regarding the financial costs incurred by patients and their families. 40 patients treated at our Unit were contacted and were questioned about the financial costs relating to their diagnosis and treatment. The questionnaire included distance travelled to the Unit, mode of transport, who patients were accompanied by, costs whilst at the Hospital e.g. car parking, days off work and impact on employment and career progression.Introduction
Methods
General Practitioners will be confronted with a sarcoma rarely in their working lives. Most will never see a bone sarcoma although most will see at least one soft tissue sarcoma. Guidelines designed to lead to earlier diagnosis of the most common cancers were introduced by NICE in 2000 and were updated in 2005, containing basic guidance on the earlier diagnosis of bone and soft tissue tumours. Referral criteria include: masses greater than 5cm in diameter, masses deep to fascia, masses which are fixed or immobile, masses which are painful, those which are increasing in size and recurrence after previous excision We examined 350 General Practitioner referrals over the past 3 years to our service and examined the correlation between the above criteria and the likelihood of malignancy.Introduction
Methods
Osteosarcomas represent a heterogeneous group of primary bone tumours that affect predominantly the long bones of patients in the first two decades of life. We aim to describe the secondary effects of a poor response (⋋90% necrosis) to chemotherapy on the effectivity of other treatment outcomes, local recurrence and survival rates. 182 cases of osteosarcoma with necrosis of less than 90% and no metastases at diagnosis have been seen at our institution over 24 years. There were 60 amputations. 122 patients underwent limb salvage, with 105 marginal margins and 17 contaminated. There was no difference in size or location between the two groups. In the 122 patients with LSS, 21 had adjuvant radiotherapy and 101 did not. In the entirety of patients with ⋋90% necrosis, survival was 64% at 2 years and 37% at 5 years. When LSS Marginal resections were compared with amputation there was a significant (P=0.006) difference in survival. LSS with a marginal margin had a 25% risk of LR. In these patients there was 25% survival, whereas the absence of a local recurrence, conferred a benefit of a 40% survival XRT was used in 21 of the 122 who underwent limb salvage. The decision to use XRT was made by the local oncologist at the treating unit. There was a 24% rate of recurrence in the XRT group and 25% with no XRT. These data demonstrated that patients who had a poor response to chemotherapy and underwent an amputation faired poorly when compared to patients with LSS. There is a selection bias in patients selected to undergo amputation. Additionally, patients who underwent amputation had a lower rate of local recurrence, but still had a poorer survival when compared to LSS.
Angiosarcomas are rare aggressive sarcomas of vascular endothelial origin. These tumours have the potential to be multicentric and are associated with high rates of local recurrence, which makes treatment challenging. The gold-standard is that these patients are managed in specialist centres by a multidisciplinary team. We present our experience of managing patients with angiosarcoma in the North of England Bone and Soft Tissue Tumour Service and a review of the literature. A prospectively collated electronic database was used to identify patients with angiosarcoma treated between 2000 and 2008, and an analysis performed of demographics, anatomical site, surgical excision and reconstruction, local disease recurrence and metastatic disease.Introduction
Methods
Simple bone cysts are relatively common in children. When they present to a musculoskeletal oncology unit, they are usually treated by a variety of methods that range from observations following fracture to surgically curettage. The outcome is usually good. Very little is written in the literature regarding adults with a cystic bone lesion. We present 10 consecutive skeletally mature patients who presented with a benign cystic bone lesion. The mean age at diagnosis was 38 years (20–60 years). Three patients has a cyst in the foot, three in the hip, two in the knee, one in the pelvis and the remainder in the shoulder. Full work up, including biopsy, was performed in all cases. All hip lesions required internal fixation for actual or impending fractures. The average follow up was 1.5 years. The surgical outcome was good in all cases. The histological features are similar in all cases. Cementum, as found in Unicameral Bone Cysts, is not seen. This may present a new entity that is not described in the literature.
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. A single-centre retrospective study, reporting the functional and oncological outcomes of six patients (4 males, 2 females - mean age of 53 (22 to 79)) who underwent a custom-made endoprosthetic replacement of the distal radius with arthrodesis at our institution, during 1999 - 2010. Five patients were diagnosed with primary bone sarcoma of the distal radius (4 GCTs, 1 osteosarcoma) and another had a metastatic lesion from a primary renal cell carcinoma. The diagnosis was confirmed by needle biopsy in all cases. We assessed the patients' functional outcomes using the Musculoskeletal Tumour Society scoring system (MSTS) and the Toronto Extremity Salvage Score (TESS).Introduction
Methods
Death within one year has been shown for some cancers to be an indicator of late presentation. They may have widespread disease by the time of diagnosis and many will have experienced delays in diagnosis. The aim of this study is to identify if this holds true for patients with bone and soft tissue sarcomas. A prospective sarcoma database was used to identify all patients with a newly diagnosed bone or soft tissue sarcoma and to compare those who had died within one year with those who had survived for at least that time.Background
Method
Primary soft tissue sarcomas of the extremities are uncommon. Many such lesions will present to specialists in other clinics such as specialist Hand or Foot and Ankle clinics. Many are of a small size at presentation and may appear to be alternate, more common pathology. We collected data from all those patients with acral soft tissues sarcomas and referred to the Oxford Sarcoma Service, Nuffield Orthopaedic Centre, Oxford over a thirteen year period from 1997 – 2010. Data were collected regarding the primary suspected diagnosis, the final diagnosis, the referral route and whether patients had undergone previous inadvertent excision.Introduction
Methods
Bony tumours of foot account for approximately 3% of osseous tumours. However, literature regarding calcaneal tumours comprises individual case reports, short case series or literature reviews, with last large case series in 1973. Literature on bony tumours of the talus is limited to case reports only. We retrospectively reviewed medical notes and imaging for all patients with calcaneal or talar tumours recorded in the Scottish Bone Tumour Registry since the 1940's. Demographics, presentation, investigation, histology, management and outcome were reviewed.Introduction
Methods
Myxofibrosarcoma is described by WHO as comprising of a spectrum of malignant fibroblastic lesions with variably myxoid stoma, pleomorphism and with a distinctively curvilinear vascular pattern. They are reported to be one of the commonest sarcomas of elderly patients. It has previously been reported to have a high rate of locally recurrent disease (50-60%). The aim of the study was to investigate the ROH series of tumours to determine prognostic factors for survival and local recurrence. Patients were identified from the ROH database who had been treated with a new presentation of myxofibrosarcoma. The size, grade and depth of the tumour at presentation was noted. Those patients who had suffered local or distal disease relapse or died were highlighted. Analysis was undertaken by Kaplan Meier survival curves for univariate and cox regression for multivariate analysis.Introduction
Methods
The National Institute of Clinical Excellence (NICE) published clinical guidelines in 2006 defining urgent referral criteria for soft tissue sarcoma to help improve the diagnostic accuracy and overall outcome. Despite these guidelines inadvertent excisions of soft tissue sarcomas continue to occur with alarming frequency potentially compromising patient outcomes. We reviewed the East Midlands Sarcoma Service experience of treating inadvertent excision of STSs and highlight the patient profile, referral pattern, subsequent management and oncological outcome associated with inadvertent resection.Background
Objective
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.
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. The distance (mm) and tissue has been regularly reported for 5 years, prior to this it was reported as wide, marginal or intra-lesional. It is known from previous studies that 90% of locally recurrent disease for osteosarcoma and Ewing's occurs within 2 years from surgery. Therefore all primary bone sarcomas of the pelvis and appendicular skeleton that underwent surgery between 1/1/2005 and 1/1/2008 were entered, giving at least 2 years follow up. The pathology records were reviewed and the margins and percentage tumour necrosis were recorded. Those patients who had suffered local or distal disease relapse or died were highlighted. Analysis was undertaken by Kaplan Meier survival curves with univariate and multivariate analysis.Introduction
Methods
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. 43 patients were analysed in the upper and lower limb fibromatosis database in which functional data was available pre-op and at a minimum of two years post-op. Any plantar, palmer, chest or abdominal lesion was excluded as were hormonal or chemotherapy treated patients.Introduction
Methods
To evaluate the rise in impedance during percutaneous radiofrequency thermo coagulation (PRFTC) of osteoid osteomas as a predictor of local recurrence. A prospective study of 23 patients (24 PRFTC procedures) with minimum of 2.25-year follow-up (average 3.3 years). Average age 19.6 years (range 4–44), sex ratio 15□:□8 (male□:□female), 16 non-diaphyseal, 7 diaphyseal.Objective
Design and Patients
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. Blood test results on all newly diagnosed patients with bone tumours from 2005 – 2010 were exported and abnormalities identified. This was matched to diagnosis, clinical features and prognosis.Introduction
Method
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. Data was collected prospectively on patients with a cartilage lesion of the phalanges. Typical enchondromas, grade 2 or 3 chondrosarcomas and patients with Ollier's disease were excluded.Introduction
Methods
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. In a referral region of ∼3m, we reviewed the referral patterns of suspected sarcoma by General Practitioners since 2005 in an effort to determine whether the published NICE guidelines had influenced an improvement in the diagnosis and management of malignant disease.Introduction
Methods
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. This data shows there is huge room for improvementConclusion
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. 20 patients with soft tissue sarcomas involving the sciatic nerve were studied treated between 1997 and 2010. Nineteen underwent surgery with extended epineurectomy of the sciatic nerve and planned marginal excision. All patients underwent staging and follow up at our Sarcoma Clinic with functional assessment and TESS evaluation.Introduction
Methods
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. All patients with newly diagnosed primary chondrosarcoma of bone and without metastases at the time of diagnosis were identified from a prospective database. Their outcome was investigated to assess whether the margin of excision affected outcome. 492 patients were included in the analysis with a mean age of 48. The mean tumour size was 11cm and 59% were male. The 10 year tumour specific survival was 85% for clear cell (N=7) and grade 1 (N=210), 60% for grade 2 (N=180), 47% for grade 3 (N=59) and 16% for dedifferentiated (N=36). The 10 year local recurrence free survival was 86% (clear cell), 73% (grade 1), 67% (grade 2), 36% (grade 3) and 56% dedifferentiated. Local recurrence was strongly related to older age (p=0.0065), grade (p⋋0.0001) and margins (p⋋0.0001). Patients who developed local recurrence had a 43% survival at 10 years compared with 76% for those who did not (p⋋0.0001). They also had a 49% risk of developing metastases compared to a 17% risk for those without local recurrence (p⋋0.0001). However most of these patients had metastases before developing LR(57%). 16 of 65 patients with local recurrence but no metastases, died due to local progression (usually pelvic or spinal tumours). There was however no relation of the margin of excision to either the risk of developing metastases or survival for any grade of tumour.Method
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). The retention of critical structures whilst resecting fibromatosis continues to be an appropriate management strategy, as local recurrence rates seem to be independent of margin status. Although not statistically significant, the use of XRT did tend towards reducing local recurrence.Conclusions
We analyzed the results of extracorporeal radiated (ECRT) autogenous tumour bone for reconstruction of diaphyseal defects after tumour resection at our institute. Sixteen diaphyseal bone tumours operated between March 2006 to March 2008 were reconstructed with ECRT bone after appropriate oncologic resection. These included 10 cases of Ewing's sarcoma, 5 of Osteosarcoma and 1 Adamantinoma. Nine involved femur, 5 tibia and 2 humerus. Suitable internal stabilisation (14 cases plate fixation, 2 intramedullary nails) was used after re-implanting ECRT bone. Average resection length was 18.9 cm (range, 11 to 30 cm).Introduction
Methods
To identify factors leading to the success or failure of extracorporeal irradiation and re-implantation of bone (ECIR). Review of experience of this technique since 1996 documenting successes and failures.Aim
Method
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. Between1985 and 2005 thirty-eight consecutive proximal humeral reconstructions using either, allograft-prosthesis composite (n=10), osteoarticular allograft (n=13) or a modular tumour prosthesis (n=14), were performed in our clinic. Their mean follow-up was ten years (nine months to 25 years). Of these, twenty-seven were disease free at latest follow-up (Mean follow-up 16.8 years) and ten had died of disease (4.2 years). Complications and implant survival with revision surgery as end-point are presented for the total group of patients, functional scores for surviving patients only. The endoprosthetic group presented the smallest complication rate of 21%, compared to 40% in the allograftprosthesis- composite and 62% in the osteoarticular allograft group. Only one revision was performed in the endoprosthetic group, in a case of shoulder instability. Infection after revision (n=3), pseudoarthrosis (n=2), fracture of the allograft (n=3), and shoulder instability (n=4) were major complications of allograft use in general. Kaplan-Meier-analysis showed a significantly better implant survival for the endoprosthetic group (log-rank p=0.002). At final follow-up the MusculoSkeletal Tumour Society scores averaged at; 72% for the allograft-prosthetic-composite (n=7, mean follow-up 19 years), 76% for the osteoarticular allograft (n=3, 16 years), and 77% for the endoprosthetic reconstruction (n=10, 6 years) groups.Method
Results
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
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. Between 1977 and 2009, 80 male and 66 female patients aged 2 to 60 (mean, 18) years with Pelvic Ewing's Sarcomas were retrospectively reviewed from the Royal Orthopaedic Hospital Oncology Service Registry. Treatments included surgery, radiotherapy, chemotherapy, or any of them in combination. Event-free (from presentation to recurrence) and overall (from presentation to death/latest follow-up) survival rates were calculated using the Kaplan- Meier method. Influence of various factors (age at diagnosis, gender, tumour site, metastasis at presentation, surgery (and surgical margins), radiotherapy, and type of treatment on survival was assessed using SPSS 14.0 statistical software.Aim
Method
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. From January 2003 to December 2009, 243 patients (148 men and 95 women; age range, 20-87 years) with painful bone metastases were treated with N-2-butyl Cyanoacrylate. Overall, 309 embolizations were performed; 56 patients had more than one embolization. Embolizations were performed in the pelvis (168 procedures), in the spine (83 procedures), in the upper limb (13 procedures), in the lower limb (38 procedures) and in the thoracic cage (21 procedures). Primary cancer included urogenital, breast, gastrointestinal, thyroid, lung, musculoskeletal, skin, nerve and unknown origin. Renal cell carcinoma was the most commonly treated tumour. In all patients, selective embolization was performed by transfemoral catheterization.Aim
Material and Methods
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. We analysed 165 bone sarcomas by immunohistochemical staining of tissue microarrays for expression of the galectin-1 (GAL1) lectin and by Western Blot experiments.Aim and purpose
Method
We investigated low grade intramedullary chondrosarcomas to see if curettage and cementation remains a strong alternative treatment to local resection. 39 patients with biopsy proven low grade chondrosarcoma treated with curettage and cementation in our clinic between 1993-2009. 32 were females and 7 were males. Mean age was 44. Mean follow up was 40.5 months. Anatomical localizations were 16 proximal humerus and 16 proximal femur, 6 distal femur and 1 proximal tibia. All patients had plain X-ray, CT scan, Tc99 bone scan and MRI before open biopsy. 28 patients had frozen intraoperative biopsy. Histological diagnosis were grade I chondrosarcoma in all our patients. Curettage followed by high speed burr and cementation with high viscosity bone cement was applied without any internal fixation. Active physiotherapy began after 2 days of the operation and full weight bearing is permitted at about 2 weeks later.Aim
Methods
Improving the quality of clinical and radiologic differential diagnosis of intramedullary tumours of long bones. A database includes clinical and radiologic (X-ray, CT and MRI methods) signs of 106 patients with osteosarcoma (n = 44), chondrosarcoma (n = 31) and giant cell tumour (n = 31). Multivariate analysis of clinical and radiologic characteristics and developing informative set of criteria (decision rule) for the differential diagnosis of osteosarcoma, chondrosarcoma and giant cell tumour were provided with program «ASTA».Aim
Methods
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. 208 patients resected for a bone sarcoma and with prosthetic reconstruction (45 in the upper and 163 in the lower limb) were evaluated. Assessment of results was done using the Karnofsky Scale (K.S.). Patients were followed in the clinic and functional results assessed according to the Musculoskeletal Tumour Society (MSTS) system. Moreover the Toronto Extremity Salvage Score questionnaire (TESS) was mailed to 144 patients.Aim
Method
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. Electronic patient records were reviewed on all patients with STS between February 1963 and January 2007. 2445 patients had over 30 types of STS. 1639 (67%) had not received any treatment prior to presentation, however, 770 patients (32%) had undergone a previous attempted excision. Survival analyses were done using Kaplan Meier and Cox regression analyses, however, for prognostic factor analysis, only patients presenting without prior treatment were included.Introduction
Methods
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. Out of our prospective database, 87 patients with osteosarcoma (43 female, 44 male with an average age of 20.4 years) have been identified with complete documentation of peri-operative CRP-levels, a minimum two year follow-up and after exclusion of concomitant infection, smoking-history or cardio-vascular disease. Pre-operative CRP before tumour resection was correlated with clinical and pathological factors, overall survival and infection rates in an uni- and multi-variate statistical model with and without landmark analysis.Aim
Method
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. Clinico-radiographic evolution in a consecutive series of 113 children, that had implanted below age 15 (range 6-14) a distal femur megaprosthesis in the period 1984-2007, was analized. A modular implant was used in 97 cases with uncemented femoral stem (three different models along the period). The implant presented fixed-hinge joint in 78 cases while rotating-hinge knee was utilized in 19 cases. In 39 cases the fixed-hinge joint had a tibial component with a polished stem to allow the residual growth of proximal tibia; in two cases a mechanically extendable prosthesis was used. A custom-made noninvasive extendable prosthesis with cemented femoral stem and smooth uncemented tibial stem was used in 15 cases since 2002. Radiological and functional results were analysed and a statistical comparison of implant outcome according different stems was obtained.Aim
Method
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. In a series of 575 patients < 21 years of age with soft tissue sarcomas (STS) we investigated the association patterns between SI patient/tumour characteristics or disease outcome. The analysis was based on multivariate models (linear for association's patient/tumour characteristics and Cox's for survival). The SI ranged between one week and 60 months (median 2 months) and tended to be longer the older the patient (i.e. the interval was longer in adolescents than in children) and the larger tumour's size and for tumours located at the extremities and for “non rhabmomio sarcoma” STS (as opposed to rhabmomio sarcomas). A longer SI unfavourably influenced survival (p=0.002): for SI of 1, 12 and 24 months, the 5-year survival for rhabmomio sarcoma was 65%, 46% and 19% respectively. A different pattern of association between SI and survival emerged for different types of STS histology.Methods
Results
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. Retrospective review of a prospective database that was started in 1986 identifying the actual diagnosis. There were 5922 patients with a confirmed diagnosis over the 23 year time period of this study. 2205 (37%) were found to have a primary malignant bone sarcoma and 1309 (22%) had a benign bone tumour. 992 patients had a general orthopaedic condition (e.g. geode or a vascular necrosis) whilst 303 (5%) had a haematological malignancy and 289 (4.9%) infection. 533 patients (9%) had metastases. There was a similar pattern of frequency of all diagnoses except for metastatic disease and haematologic malignancy at different ages. The incidence of metastases increased from the age of 35 onwardsMethod
Results
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. A database of 64 MO patients surgically treated between 1980-2009 was established. For 24 patients full radiological (including MRI), surgical and pathological records were complete. This group contained 14 osteochondroma patients and 10 chondrosarcoma patients. Non-parametric tests and Kaplan-Meier survival analysis were used to establish a cartilage-cap thickness cut off point and a volume cut off point.Aim
Methods
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. Clinical data were collected from all females with previous breast cancer who developed angiosarcomas/lymphangiosarcomas on the thoracic wall/upper extremity between 1958 and 2008 in the Southern Swedish health care region.Background
Methods
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.
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 31 patients- 19 female, 12 male, age from 8 yrs to 42 yrs, were treated with intravenous zoledronic acid. In 17 patients (fibrous dysplasia-10, nonossifying fibroma- 4, UBC- 3) zoledronic acid alone was used as a therapeutic agent. In 14 patients (ABC- 3, GCT- 11), it was used as an adjuvant agent after curettage. Four patients presented with pathological fracture. In all patients, 4 mg. zoledronic acid was given at 2 monthly intervals. In 12 adult patients, in addition oral bisphosphonates - alendronate was given weekly for at least 6 months.Aim
Method
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.
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. Clonogenic survival, cell cycle analysis, apoptosis and gammaH2AX phosphorylation as a marker of DNA double strand breaks (DSBs) were examined in two OS (KHOS-24OS, SAOS2) and two RMS (A-204, RD) cell lines treated with SAHA alone and SAHA plus XRT, respectively. Protein expression was investigated via immunoblot analysis, cell cycle analysis, measurement of apoptosis and gammaH2AX expression were performed using flow cytometry.Aim
Methods
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. All patients with limb osteosarcoma, no metastases, a poor response to chemotherapy and either a marginal excision or primary amputation were identified from a prospective database. This group were investigated in terms of overall survival and local control. There were 182 patients in this category of whom 60 had an amputation, 105 limb salvage with marginal margins and 17 with an intralesional margin. Local recurrence (LR) arose in 41% of those with an intralesional margin, 22% of those with a marginal margin and 13% of those with an amputation. Radiotherapy was used in 21 of the 122 patients and the risk of LR was the same as in those who did not have radiotherapy. Neither age nor sex of the patient, size or site of the tumour affected the risk of LR. The overall survival for this group was 42% at 10 years. The survival was best in those with marginal margins (38%) than those with an amputation (28%) and worst for those with an intralesional margin (20%). Survival was worst in those who did develop LR, but no worse than in those having amputationMethod
Results
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 A retrospective analysis of 650 patients who had a diagnosis of Ewing's sarcoma treated between 1975 and 2009 at a single institution was performed and 64 patients (10%) who had a local recurrence were identified and analysed. Fifteen patients had metastases at diagnosis.20 patients had chemotherapy and radiotherapy only while 44 had chemotherapy and surgery +/− post op radiotherapy. Thirteen patients who were suitable for post –operative radiotherapy could not receive the treatment due to various reasons like biological reconstruction. The estimated 5 years survival for the patients was 15%. The risk of local recurrence is higher if the tumour is located in the axial skeleton, treatment with chemotherapy and radiotherapy alone [location and size of the tumour precluding surgery]. The risk of local recurrence is higher if the tumour was in the fibula or radius. One out of three patients who have good response to chemotherapy still went on to develop a LR. The use of biological reconstruction and younger age group often resulted in deferral of post-operative radiotherapy. Location and type of treatment can predict LR. Surgery with clear margins and post-operative radiotherapy given when indicated may reduce the incidence of LR.Methods
Results
