Assess the oncological and clinical outcomes associated with intralesional curettage, phenol and bone grafting of the lesions. A retrospective study was performed using data from the Leiden University Medical Center. Ninety patients with low grade central chondrosarcoma of long bones and small tubular bones were treated using phenol and ethanol as adjuvant therapy after intralesional curettage. Standard clinical follow-up contained regular visits to the orthopaedic department, physical examination and radiological follow-up with plain X-rays and dynamic Gadolineum-enhanced MRI scans.Aim
Method
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
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
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.
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. A retrospective study was performed between 2004 and 2010. 6 patients were diagnosed with aggressive local recurrence of appendicular GCT. All patients were treated for the primary tumour by surgical curettage and cryoablation followed by cementation or biological reconstruction. In 5 patients the tumour was located in the distal radius and in one in the first metacarpal bone. All recurrences were in the bone with large soft-tissue extension. After histological diagnosis – by CT core needle biopsy – the patients were treated by intravenous bisphosphonate, followed by clinical & radiological assessments.Aim
Method
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. The clinical records, image methods and the histology reports of 157 metaphyseal paediatric osteosarcomas were reviewed. The mean follow-up time was 102 months. Location, histological subtype, time from initial symptoms to start of treatment, major diameter, % of necrosis, physis crossed by the tumour or not, and outcome (recurrence, metastases and status) were recorded in a SPSS v15.0 file.Aim
Patients and methods
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. 50 patients underwent en-bloc resection, extracorporeal irradiation with 50 Gy, and re-implantation for limb salvage between August 1996 and June 2003. Case notes and patients were reviewed.Aim
Methods
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.
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. From January 1994 to June 2009 we have operated in our Centre 976 patients affected by STS of extremities and superficial trunk. They were 741 High grade STS (76%), and 235 Low grade STS (24%). The most represented histotype was Liposarcoma (239) followed by Leiomyosarcoma (150) and synovial sarcoma (94). Regarding tumour site, upper limb was involved in 255 cases, lower limb in 679, superficial trunk in 42; regarding tumor size, 323 where less than 5 cm, 386 where between 5 and 10 cm and 267 where more than 10 cm. Radiation therapy was utilized in 447 cases (46%): 83 patients had a low grade STS, 364 a high grade STS.Aim
Method
was to analyze infections after bone tumour surgery. 1463 patients treated from 1976 to 2007 were analized: 1036 with resection and prostheses in the lower limbs, 344 with resection and prostheses in the upper limbs, 83 with surgery for sacral tumours. Infections were analyzed for time of occurrence (“postoperative” in the first 4 weeks from surgery, “early” within 6 months, and “late” after 6 months), microbic agents, treatment, outcome.Aim
Method
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. We therefore created a questionnaire concerning of 20 questions asking for scope, completeness and accuracy of information found on osteosarcoma. Three independent observers tested the English version of Wikipedia as well as the patient version and the health professional version of the website of the National Cancer Institute. Answers (scores 0-3) were verified with authoritative resources and international guidelines.Aim
Method
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. Human fibrosarcom (HT1080) was transplanted to athymic mice. The photosensitizer aluminium phthalocyanine disulfonat (AlPcS2a), and bleomycin (BLM) was systemically administrated 48 hours and 30 minutes respectively prior to surgery and light exposure. After resection with intralesional margin the tumour bed was illuminated at 670 nm (15 J/cm2).Aim
Method
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. To investigate the oncogenic properties of SSX. Validate it as tumour target and identify lead compounds. RNAi system for conditional silencing of SSX expression, Protein analysis and Mass Spec, PCR arrays, tumour xenographs and drug library screening.Aim
Methods
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 Review of interim recruitment and randomization rates in a large randomized multinational trial for resectable osteosarcoma, EURAMOS-1, jointly performed by COG, COSS, EOI, and SSG, within ECT-EUROCORES. Randomization scheduled after preoperative chemotherapy and definitive surgery of the primary tumour.Aim
Method
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. The quality in terms of precise distance (mm) and tissue has been regularly reported by our pathologists for 5 years, prior to this it was reported as wide, marginal or intralesional. It is known from previous studies that 90% of locally recurrent disease for osteosarcoma and Ewings 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 into the study group, giving at least 2 years follow up. The pathology records were reviewed and the quality of 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
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.
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. The surgery consists of a two-stage procedure. At the primary surgery (S1), a titanium screw (the fixture) is inserted into the remaining diaphyseal bone. The fixture is 80 mm long with a diameter of 16–20 mm (+0.5 mm increments). The patient is hospitalized 5-7 days. At the secondary surgery (S2), six months later, an abutment is inserted into the fixture. The abutment has a hexagonal press-fitting into the fixture and is secured by an abutment screw. The patient remains hospitalized 10–12 days. Gradual increase of load and activity is initiated over a 6 month period.Aim
Method
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. Data on 62 patients who had been treated for SS from 1968 to 1999 were studied retrospectively in a multicenter study. The following parameters were examined for their potential prognostic value: age at diagnosis, sex, tumour site and size, histology, histological grade, fusion type (SYT-SSX1 vs. SYT-SSX2), and surgical margin status. Mean follow-up of living patients was 17.2 years, and of dead patients 7.7 years.Aim
Method
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. Retrospective study of 48 patients observed 1966- 2007 was undertaken: 36 patients were managed at our Institution while 12 cases were consultations and not included in this study. Clinico-pathologic features and details of treatment of all 36 patients were correlated with outcome. Updated follow-up was available in all patients.Aim
Method
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. In our files, 12 cases curetted and grafted, without radiotherapy addition developed sarcomas from 6 to 28 years from curettage (median 18). Age at first diagnosis (9 GCT, 1 benign fibrous histiocytoma, ABC and solitary bone cyst) ranged from 13 to 55 (median 30). For all cases radiographic and clinic documentation was available. Histology was available for 7 of the benign lesions and for all malignant lesions. The type of bone used to fill cavities was autoplastic in 4 cases, homoplastic in 2 cases, homoplastic and tricalciumphosphate/hydrossiapatite in 1 case, autoplastic and homoplastic in 1 cases, heteroplastic in 1 case. In 3 cases the origin was not reported. Secondary sarcomas, all high grade, were 8 OS, 3 MFH, and 1 fibrosarcoma.Aim
Methods and Results
