Aim: The differential diagnosis between chondroma and grade I chondrosarcoma still represents a challenge. There are always cases in which a perfect diagnosis can’t be done for sure. This cases are defined in literature with different synonyms such as: borderline chondrosarcoma, grade 0 chondrosarcoma, atypical enchondroma or in situ chondrosarcoma. Enchondroma are benign lesions that do not require a surgical treatment. Low grade chondrosarcoma is a malignant tumour that can recur and also if in a low percentage of cases can metastasize. Methods: The Authors reviewed 22 cases of chondrosarcoma of the limbs for clinical, radiographycal and histological features. Results: Pain was present in 80% of cases of low grade chondrosarcoma, while was absent in enchondroma. Radiographic analysis was not significative. Bone scan was often positive in low grade chondrosarcoma as in enchondroma. Histology demonstrated a permeative pattern in chondrosarcoma with infiltration of the bone trabeculae. Conclusions: Only the complete evaluation of the patient resulted in a correct diagnosis. Follow-up of patients confirmed our findings

Purpose. Extraskeletal chondrosarcoma is a rare tumor with an indolent course and high propensity for local recurrence and metastasis. This tumor most commonly presents in the proximal extremities of middle-aged males, and is commonly asymptomatic. Although slow growing, these tumors have a significant risk of eventual relapse and metastases, especially to the lung. There are no clinical trials that investigated the best treatment options for this tumor given its very low incidence. The aim of this study is to present the surgical and clinical results of extraskeletal chondrosarcoma, which is a rare tumor. Methods. In our clinic, the information of 13 patients who were diagnosed with extra-skeletal chondrosarcoma between 2006 and 2018 were retrospectively reviewed. Demographic information, tumor size, surgical treatments, chemotherapy and radiotherapy status, follow-up times, recurrence and metastases of the patients were recorded. Results. This study included 13 patients with an average age of 53.6 ± 15 (range, 28 to 73) years diagnosed with extraskelatal chondrosarcoma. In 8 of the patients, the tumor was located in the lower limbs and it was observed that the thigh was located mostly (46.2%). The mean follow-up period of the patients was 52.8 ± 19.9 (range, 24 to 96) months. All patients underwent extensive resection and only one patient had a positive surgical margin. In the follow-up, 5 (38.5%) of the patients developed recurrence, while 6 patients had lung metastasis (46.2%) and 53.8% (7 patients) of the patients exitus. The mean tumor size was 10.4 ± 3.2 (range, 5 to 17) cm. The median survival time of the patients in the study was 61 (50.5–71.4) months. The 5-year survival rate is 51.8%. There was no significant difference between survival times according to age, gender, side, limb location, postoperative RT, recurrence and presence of lung metastasis (log rank tests p > 0.05). The cut off value for exitus obtained by ROC analysis of tumor size was determined as 11 cm (fig 1). Accordingly, the survival time of patients with 11 cm and above tumor size was observed to be statistically significantly shorter. Conclusion. Consequently, ECM is a rare soft tissue sarcoma with high local recurrence and metastasis capacity. Therefore, close follow-up is recommended. The first option should be extensive resection. Studies with large patient series on the prognostic factors of the future ECM are needed. For any figures or tables, please contact the authors directly

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

Chondrosarcoma of the spine is a rare condition. In a 45-year follow-up study in one institution, only 21 cases were recorded. We report on three cases, one a secondary chondrosarcoma and two primary tumours. Two presented with hyper-calcaemic renal calculi following massive bone destruction, and one, which we observed for four years, was a repeat chondrosarcoma. Adjuvant therapy has no role in treatment of these tumours, for which the only curative measure is radical surgery. We performed anteroposterior resection and spinal reconstruction on all three patients. CT scan shows that all three have been tumour-free for the past 30 months. All three are neurologically intact. Because of pulmonary complications, two have required extended hospitalisation. In the literature, a five-year survival rate is reported in 50% of cases. Good surgical planning and execution are vital in dealing with chondrosarcoma of the spine

Twenty-three patients with scapular chondrosarcomas presented to our institution between 1989 and 2003. Twenty-two were treated surgically while one presented with metastases and was treated palliatively. Fourteen patients underwent partial scapulectomy and eight had a Tikhoff-Linberg procedure. There were no local recurrences and only two patients have suffered a systemic recurrence at mean follow-up of fifty-two months. Mean functional scores were: TESS – 88, MSTS 1987 – 27 and MSTS 1993 – 84. Overall, the oncologic and functional outcome for these patients was excellent. To examine the oncologic and functional outcome of patients treated for chondrosarcoma of the scapula. Rates of local recurrence and metastasis for adequately treated chondrosarcomas of the scapula were very low and patient function was quite good. Unlike previous reports in the literature, we found that scapular chondrosarcomas are highly amenable to limb salvage surgery and the oncologic and functional outcomes are excellent. Retrospective review of our prospectively collected database for all patients treated surgically at our institution for scapular chondrosarcoma between 1989 and 2003. Twenty-three patients presented with scapular chondrosarcoma, but one had spine metastases and was treated palliatively. Thus twenty-two patients were treated with limb salvage surgery. There were fourteen males and eight females. One patient presented as a local recurrence. Four tumors were grade one, sixteen grade two and two grade three. Eight were secondary to a primary benign primary tumor of bone. There were fourteen partial scapulectomies and eight Tikhoff-Linberg procedures. Surgical margins were positive in three cases. two patients received post-operative radiation and no patients received adjuvant chemotherapy. At last follow-up, twenty patients were alive with no evidence of disease (90.9%), one was alive with disease and one was dead of disease. There were two systemic recurrences and no local recurrences at an average follow-up of fifty-two months (range 12–113). Mean functional scores were: TESS – 88, MSTS – 1987 27 and MSTS 1993 – 84

Chondrosarcoma are rare malignant tumors. About the biological characteristics of chondrosarcoma is little-known [. 2. ]. Endothelin and its receptors are involved in regulating angiogenesis and metastatic dissemination [. 1. ]. The aim of this study is first to identify if chondrosarcoma are expressing endothelin-1 (ET-1) and the endothelin-receptors and thereupon to identify potential molecular markers for new target therapies. Another aim is to determine if endothelin is a prognostic factor in chondrosarcoma. 32 cases were investigated clinically and histopathologically. The expression of vascular endothelial growth factor (VEGF), Endothelin-1, Endothelin-Receptor-A (ETR-A) and Endothelin-Receptor-B (ETR-B) were determined. All data were analyzed by Fisher’s exact test (p< 0,05). All tumors show an expression of either ET-1, ETR-A or ETR-B. Chondrosarcomas with grade (G) I are mostly expressing less than 10-% ET-1 in cells, Chondrosarcomas G II are expressing in most cases between 10–50% and nearly all Chondrosarcoms G III more than 50%. In addition ET-1-expression is correlating with the histological grading. The patients also show a significant high metastatic dissemination probability at the time when tumor samples present more than 10%-storing ET-1-cells. The intensity of ET-1-expression is correlating with VEGF, which is the most important angiogenetic factor in tumors. Chondrosarcomas are expressing ET-1, ETR-A and ETR-B. ET-1 seems to play a role in the angiogenesis of chondrosarcoma. Increased expression of ET-1 is accompanied with a high probability of metastatic dissemination. Endothelin receptor antagonists, which are used for example in prostate and breast cancer, can represent a potential therapy for chondrosarcoma [. 1. ]. Experiments on animals and clinical studies are required

Chondrosarcomas are malignant hyaline cartilage tumours of bone. They are clinically resistant to conventional chemo- and radiotherapy and the underlying mechanism is poorly studied. Chemoresistance is a multifactorial process and the inaccessibility due to abundant hyaline cartilaginous matrix surrounding the cells, presence of multi-drug resistance pumps, and expression of anti-apoptotic proteins such as BCL2, have been suggested. Our aim was to study chemoresistance mechanisms in chondrosarcoma. We first studied the sensitivity of chondrosarcoma cell lines (SW1353, CH2879, JJ012, OUMS27) and 2 primary cultures for doxorubicin and cisplatin. We used a 3D pellet model of CH2879 to study doxorubicin incorporation. To investigate whether chondrosarcoma cells could be resensitised to chemotherapy we tested the BH3 mimetic ABT737 inhibiting anti-apoptotic BCL2 family proteins. Cell viability was assessed using a WST assay for mitochondrial activity. Dose response curves showed that chondrosarcoma cell lines and cultures are partially resistant to doxorubicin, while primary cultures were completely resistant to cisplatin. In 3D cell pellets, with morphology strongly resembling high grade chondrosarcoma, doxorubicin incorporation was confirmed. Chondrosarcoma cells responded to ABT737 with a >60% reduction in cell viability at high concentrations (25μM). Combination treatment allowing 2 days between ABT737 and chemotherapy addition led to a complete reduction of cell viability in all cell cultures. In conclusion, chondrosarcoma cell lines show a partial response to doxorubicin and less response to cisplatin. The incorporation of doxorubicin in the cells in a 3D pellet model indicates that resistance is not caused by inaccessibility of the cells for the drugs nor by multi-drug resistance pump activity. By combining BCL2 inhibition with Doxorubicin treatment, a complete reduction of cell viability was obtained. This suggests that BCL2 overexpression plays an important role in chemoresistance of chondrosarcoma, and turning on the apoptotic machinery by BCL 2 inhibition can render them chemosensitive

Introduction: Within a study group of 102 consecutive patients diagnosed with chondrosarcoma of the femur, tibia or humerus, an association with previously treated breast cancer was noted. We researched this proposed relationship. Methods: We retrospectively reviewed the records of all patients diagnosed histologically with chondrosarcoma of the femur, tibia or humerus over a six-year period at a supra-regional bone tumour unit. We identified those patients who had previously been treated for breast cancer. Results: There were 58 female and 44 male patients. The study group contained six females (10%, mean age 53 years) who had previously been treated for breast cancer, a higher proportion than would be expected. They were referred following identification of a solitary area of increased activity on routine screening with isotope bone scan, presumed to be a solitary bony metastasis. Most (86%) of this breast carcinoma sub-group had developed low-grade bone chondrosarcoma (Trojani grade 0.5-I) and only one case (14%) had developed high-grade chondrosarcoma (Trojani grade II–III). Discussion: A suspicious long bone lesion on bone scan in a patient with a past medical history of breast cancer must, therefore, not be assumed to be a metastasis without further investigation; the possibility of a chondral lesion should be considered. It is important that patients receive a full multidisciplinary team investigation prior to treatment in order to obtain the correct tissue diagnosis, as the management of these conditions is often different. Our study suggests there may be a relationship between patients previously treated for breast cancer and the development of subsequent chondrosarcoma

The influence of advancements in imaging and chemotherapy on patient with dedifferentiated chondrosarcoma was determined. There were forty-two cases in which twenty-seven patients received adjuvant therapy. Median survival was eight months and five-year survival was 4.8%. There was no statistical difference (p=0.62) in survival between patients who did and did not receive chemotherapy, had wide versus radical resection, or had limb sparing versus sacrificing procedures. There were no statistically significant differences between patients treated prior to 1986 and those subsequently. Despite advances, dedifferentiated chondrosarcoma continues to carry a poor prognosis. The routine adjuvant chemotherapy in this population should be questioned. The long-term survival for patients that presented with dedifferentiated chondrosarcoma has historically been poor. A large clinical series has not been analyzed in the era of modern diagnostic and treatment modalities. The current study was performed to look at the influence of advancements in imaging and chemotherapy on patient outcome. A retrospective chart review of all cases of patients presenting with dedifferentiated chondrosarcoma at our institution from 1984–2000 was performed. This was done as an extension to a study published in 1986 prior to the era of modern chemotherapy. There were forty-two cases in twenty-five men and seventeen women of average age fifty-six (range twenty-four-eighty-three years). MSTS grades at presentation were IIA(5), IIB(27), and III(10). Three patients underwent biopsy only, nineteen had limb sacrificing surgery, and twenty had limb sparing procedures. Surgical margins were intralesional in three, marginal in two, wide in twenty, and radical in fourteen. Twenty-seven patients received adjuvant therapy (twenty-two chemotherapy only, two radiotherapy only, three combined therapy). Median survival was eight months and five-year survival was 4.8%. There was no statistical difference (p=0.62) in survival between patients who did and did not receive chemotherapy, had wide versus radical resection, or had limb sparing versus sacrificing procedures. There were no statistically significant differences between patients treated prior to 1986 and those subsequently. Despite advances in diagnostic modalities, surgical treatments, and adjuvant therapies, dedifferentiated chondrosarcoma continues to carry a poor prognosis. The routine use of current adjuvant chemotherapy and its inherent risks and benefits in this population should be questioned

The purpose of this paper is to present the rare occurrence of clear cell chondrosarcoma in a patient suffering from Niemann-Pick disease (NPD). NPD is a rare autosomal recessive inborn error of metabolism. It is a lysosomal storage disorder affecting children and adolescents often causing death in early childhood although in milder form patient may survive up to adulthood. Although chondrosarcoma is a relatively common malignant bone lesion, these lesions rarely affect patients suffering from Niemann-Pick disease. This article presents the case of a 50-year-old man with a chondrosarcoma in the olecranon of the left ulna who also suffered from milder form of Niemann-Pick disease. Radiography and MRI showed cortical disruption and an expansile lesion. Histologically, the tumour was classified as grade 2 clear cell chondrosarcoma with classical differentiation according to the American Joint Committee of Cancer pathologic staging system. Amputation of an above elbow type was performed to obtain clear surgical margins. A case report and discussion of this rare association of a patient suffering from milder form of NPD who developed a clear cell chondrosarcoma is presented here. NPD and clear cell chondrosarcoma are rare diseases and together their occurrence was not found reported in the literature. NPD patients rarely live to the age of 50 years. This raises a possibility that if Niemann-Pick patients survived long enough they might develop chondrosarcoma. Alternatively this could just be coincidental. More research is needed to clarify this possibility

Low grade chondrosarcoma is currently followed up with the same schedule as all other chondrosarcoma patients. When treated adequately, low grade chondrosarcoma appears to have a very benign follow up course. Patients and Methods:. A retrospective study of follow ups was done on fifty six patients treated for chondrosarcoma at the Pretoria musculoskeletal tumour unit from 1987–2009, evaluating the outcome and presence of local recurrence. The lesions were divided into four histological grades; atypical enchondroma (grade 0), low grade chondrosarcoma (grade 1), intermediate grade chondrosarcoma (grade 2), and high grade chondrosarcoma (grade3). Results:. Biopsy revealed forty-six patients with low grade chondrosarcoma (82%), nine cases of grade 2 chondrosarcoma (16%), and one patient with a metastatic mesenchymal chondrosarcoma. The femur and humerus were the commonest sites involved, while the axial skeleton was only affected in three cases, all of whom had more aggressive lesions. Forty-nine patients had no recurrence after a mean period of 57.5 months follow up. Forty- two of those had low grade lesions, and seven patients had grade 2 lesions. Two patients with low grade lesions were lost to follow up. Three patients involved in the study presented with local recurrence, but no metastases were detected radiographically. Two patients died, one with metastatic mesenchymal chondrosarcoma involving the scapula, and the other with a grade 2 lesion of the pelvis. Conclusion:. We believe that adequately treated low grade chondrosarcomas have a very low local recurrence rate. When faced with recurrence, it rarely presents with distant metastases. A revised schedule of visits at 3 and 6 months followed by yearly visits for five years appears to be adequate for timely detection and treatment of any local recurrence

Chondrosarcoma responds poorly to adjuvant therapy and therefore, new targeted therapy is required. Animal models have been utilised to test therapeutic candidates, however clinically relevant, orthotopic models are lacking. The aim of this study was to develop such a model. In vitro: two human chondrosarcoma cell lines, JJ012 and FS090, were compared with respect to proliferation, colony formation, invasion, MMP-2 and MMP-9 secretion, osteoclastogenesis, endothelial tube stimulation, and expression of the angiogenic factor VEGF, and the anti-angiogenic factor RECK on western blotting. In vivo: 20,000 cells (JJ012 or FS090) were injected either into the intramedullary canal of the mouse tibia (n=5 for each cell line), or into the tibial periosteum (n=5 for each cell line). Animals were measured, and x-rayed weekly. Once euthanised, tibias and lungs were preserved, embedded and sectioned to determine the presence of tumour and lung metastases. In vitro: compared with FS090, JJ012 demonstrated significantly higher proliferative capacity at both day two and day four (p=0.017, and p=0.01). JJ012 had a significantly greater ability to invade Matrigel with an average number of 812.5 invading cells, versus 140.8 FS090 cells (p=0.0005). JJ012 readily formed colonies in collagen I, while FS090 formed none. JJ012 conditioned medium stimulated endothelial tube formation and osteoclastogenesis with a greater potency than FS090 conditioned medium. In vivo: tumours formed in the intratibial and periosteal groups injected with JJ012, whilst no mice injected with FS090 cells developed discernable tumours on physical inspection, caliper measurement or histological section. Periosteal tumours grew to three times the non-injected limb size by seven weeks, whereas intratibial injected limbs required 10 weeks to achieve the same extent of tumour growth. All JJ012 periosteal tumours resulted in lung micrometastases, while only 2/4 JJ012 intratibial tumours demonstrated metastases. Lung metastases stained positive with Von Kossa and alizarin red stains, indicating a tendency for calcification, which is similar to metastases in the human disease. Sectioned tumour tissue demonstrated features of grade II-III chondrosarcoma. Similarities with the human disease were also noted on the X-ray, including endosteal scalloping, and cortical thickening. Both intratibial and periosteal JJ012 models replicate the site, morphology, and many behavioural characteristics of human chondrosarcoma. Local tumour invasion of bone and spontaneous lung metastasis offer valuable assessment tools to test the potential of novel agents for future chondrosarcoma therapy

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. Method. We analysed 165 bone sarcomas by immunohistochemical staining of tissue microarrays for expression of the galectin-1 (GAL1) lectin and by Western Blot experiments. Results. We found that GAL1 was abundant in normal human osteoblasts from benign proliferations and in osteosarcomas, including chondroblastic osteosarcomas, but not in chondrosarcomas. There was a highly significant statistical difference in the percentage of stained cells (p<10-4) and in the staining intensity (p<10-3) of chondroblastic osteosarcomas compared to conventional chondrosarcomas. This discriminatory potential of GAL1 staining for osteosarcoma-derived tumours was confirmed by western blotting. We propose a diagnostic test for bone tumours that takes into account the optimal discriminative values for the percentage of cells stained and the intensity of staining. The positive and negative predictive values were 85.7% (trust interval of 63.7-97%) and 90% (trust interval of 80-95.9%), respectively, demonstrating the pertinence of the test. Conclusion. Altogether, our data indicate that GAL1 is a powerful diagnostic marker that distinguishes chondroblastic osteosarcomas from conventional chondrosarcomas

Purpose of the study: Secondary chondrosarcoma is rare (1% of malignant bone tumours). Most cases develop from solitary exostosis or concern an exostosis disease. Localisations predominate in the girdles. Management is difficult and no consensus has been reached. The purpose of this study was to present a series of 25 secondary chondrosarcomas in order to improve diagnostic and therapeutic management. Material and methods: This was a retrospective analysis of 25 cases of secondary chondrosarcoma (10 on solitary exostosis and 15 on exostosis disease) treated by one surgical team from 1970 to 2008. The epidemiological features, clinical signs, radiographic findings, type of treatment and outcome at last follow-up were analysed for the two groups. Results: Patients with secondary chondrosarcoma were 10 to 20 years young than those with primary chondrosarcoma. There were an equivalent number of men and women and the predominant sites involved flat bones in both groups. The radiographic signs of sarcomatous degeneration most widely observed included heterogeneous calcifications, irregular contours, and soft tissue invasion. Tumours were generally well differentiated. The rate of local recurrence after surgery was 15% at five years and 20% at ten years. Mortality was 2% at five years and 5% at ten years. Most of the deaths occurred after local recurrence. Metastases were identified in four patients after the initial resection. The rate of local recurrence was lower after wide surgical resection. Discussion: The real objective with secondary chondrosarcoma is to ensure a regular effective follow-up of these patients with a known risk of recurrence (exostosis disease) in order to recognise early signs of sarcomatous degeneration. One of the most reliable signs is recent development of unusual pain on a known exostosis. Education of at-risk patients is crucial and should enable early screening and detection. Conclusion: Secondary chondrosarcoma occurs 10 to 20 years earlier than primary sarcoma and generally involves the girdles. Outcome and management practices are similar to primary chondrosarcoma. The most important issue is to ensure good patient follow-up in order to enable early diagnosis in patients at risk

Chondrosarcoma is a malignant tumour and accounts for approximately 20% of bone sarcomas. The pelvis is one of the commonest sites. Chondrosarcoma of the pelvis lends itself to surgical excision and is relatively resistant to irradiation and chemotherapy. A long term survival analysis of this challenging condition is rarely reported in literature. We review and evaluate the oncological and functional results of all the patients operated at our centre and we analyse the survival analysis of these patients with special focus on the prognostic factors. Fifty-four consecutive patients with chondrosarcoma of the pelvis who were treated at the Royal National Orthopaedic Hospital, Stanmore, UK between 1987 and 2001 were included in the study. Demographic data, case notes, histopathological results and follow-up data were obtained and statistically analysed. There were 38 males and 16 females with a mean age of 48.4 years [18-77]. The chondrosarcomas were primary [n=38], secondary [n-7] or recurrences [n=9]. The anatomical sites in the pelvis were in the epicentre I [n=24], II [n=20] and III [n=10]. The surgical procedures performed were local resection [n=28], local resection and hip arthroplasty [n=6], hemipelvectomy (+endoprothesis) [n=16], hemipelvectomy [+fibular strut graf] [n=2] and hinquarter amputation [n=2]. The histological grade was Gr [n=27], Gr 2[n=20] and Gr 3 [n=7]. The complication rate was 24%:wound revision [9%], dislocation [8%] and infection [7%]. There was a 5, 10 and 15 year cumulative survival rate of 74%, 65% and 40%. The overall recurrence rate was 24%. The factors associated with a worse prognosis were high histologic tumour grade, increasing patient age, anatomical location in site I and III, primary surgery outside of tumour centre, inadequate surgical margins, and those treated by local extension. Aggressive surgical approach significantly improves the prognosis of the patients with chondrosarcoma of the pelvis

Purpose of the study: To analyze survival and prognostic factors in a series of patients treated for chondrosarcoma of the pelvis. Material and methods: The series included 106 patients (53 women and 53 men) treated for non-metastatic chondrosarcoma of the pelvis. Minimum follow-up was two years. Mean age at diagnosis was 44 years. Tumors were grade 1 (n=47), grade 2 (n=37), grade 3 (n=22). Conservative surgery was performed in 73 patients (resection with or without reconstruction) and interilio-abdominal disarticulation for 33. Results: Resection margins were sufficient for 34 patients (wide or radical resection), marginal for 35, and intratumoral or malignant for 37. Local recurrence was noted in 39 patients (37%). Prognostic factors affecting local recurrence were: quality of resection (p=0.03), grade (p=0.01). Overall survival at 5, 10 and 15 years were 72, 56, and 46% respectively. Survival was strongly correlated with grade (p=0.08) and survival after five years was also correlated with resection margins. Conclusions: In this series, tumor grade was the most important prognostic factor for patients with chondrosarcoma of the pelvis but achieving satisfactory resection with wide margins also has a significant effect on prognosis for local recurrence and long-term survival

Chondrosarcoma is treated with respect by oncology surgeons, given that it is relatively resistant to chemotherapy and radiotherapy. The aim was to study the outcomes of surgery for chondrosarcoma and determine the role of initial surgical margins and local recurrence on outcome. Electronic patient records were retrieved on all patients seen with chondrosarcoma of bone with a minimum of two years follow up. A total of 532 patients were seen with Chondrosarcoma between 1970 and 2006. Patients were excluded if they had initial treatment in another unit (20 patients), a subdiagnosis of dedifferentiated chondrosarcoma (due to very poor prognosis, 43 patients), metastases at presentation (30 patients), if they presented with disseminated metastases prior to local recurrence (12 patients) or were not offered surgery, leaving 402 patients in the study group. The mean age was 48 years old (range 6–89 years) with the most commonly sites of presentation being in the pelvis in 132 patients (29%), proximal femur in 81 patients (18%), distal femur in 40 patients (9%) and proximal humerus in 40 patients (9%). Grade at presentation was grade 1 in 44%, grade 2 in 44% and grade 3 in 12%. Surgical margins were radical in 3%, wide in 44%, marginal in 29%, planned incisional in 13% and unplanned incisional in 11%. Local recurrence occurred in 87 patients (22%). Local recurrence rates were significantly different for surgical margins on Fisher exact testing (p=0.003), which held true even when stratified by presenting grade of tumour. Surgery for local control was successful in 62% of cases. Complex relationships exist between surgical margins, local recurrence and survival. Long term survival is possible in 1/3 patients who have local recurrence in intermediate and high grade chondrosarcomas and therefore ever effort should be made to regain local control following local recurrence

Dedifferentiated chondrosarcoma is a rare but highly malignant manifestation that can occasionally arise in patients with cartilage tumours. There remains uncertainty as to the best treatment for this condition and in particular whether chemotherapy may have a role in improving prognosis. Members of EMSOS were invited to contribute data on patients, tumours, treatment and outcomes of patients with dedifferentiated chondrosarcoma. Eight centres contributed data on 317 patients from 7 countries. The mean age was 59 (range 15 to 89) and the most common site was the femur (46%) followed by the pelvis (28%). 25% of patients presented with a pathological fracture and the most common high grade component identified was MFH. 23% had metastases at diagnosis and these patients had a median survival of 5 months. 30% of patients received chemotherapy, with 47% under 60 having chemotherapy compared with 10% over 60. One third of this group had neoadjuvant chemotherapy and the rest had adjuvant reatment. 88% had surgery with limb salvage in 80% of this group. The overall survival was 38% at 2 years and 24% at 5 years but in patients without metastases at diagnosis these figures were 44% and 28% respectively. Poor prognostic factors for survival were: metastases at diagnosis, amputation or no operation, local recurrence, age over 60 and pathological fracture at presentation. We were unable to identify any group in whom chemotherapy appeared to have a survival benefit. Dedifferentiated chondrosarcoma carries a dismal prognosis. Although 30% of patients received chemotherapy in this study we were not able to prove that it improved survival. Early diagnosis and complete surgical excision still offer the best prognosis for this condition

Introduction: Chondrosarcoma is the second most common primary malignant bone tumour. Distinguishing between grades is not necessarily straightforward and may alter the management of the disease. We evaluated the correlation between the pre-operative needle biopsy and excision biopsy histological grading of chondrosarcoma of the femur, tibia and humerus. Methods: A consecutive retrospective series of 100 patients with a histological diagnosis of chondrosarcoma made at a supra-regional bone tumour unit was reviewed. Twenty-one patients were excluded because 20 had only excision biopsy, due to radiological confidence in the diagnosis, and one had only the pre-operative biopsy on record, thus this series included 79 available cases. The remaining patients underwent a pre-operative needle biopsy. Results: In 11 instances, there was a discrepancy in histological grade. Therefore, there was an 86% (68 out of 79) accuracy rate for pre-operative histological grading of chondrosarcoma, based on needle biopsy. However, the accuracy of the diagnostic biopsy to distinguish low-grade from high-grade was 90% (71 out of 79). Discussion: From this series we conclude that accurate image-guided biopsy is a very useful adjunct in determining histological grade of chondrosarcoma and the subsequent treatment plan. At present, a multidisciplinary approach, comprising experienced Orthopaedic Surgeons, Radiologists and Pathologists offers the most reliable means of accurately diagnosing and grading chondrosarcoma of long bones

Purpose: The purpose of this work was to analyse survival and prognostic factors in a series of patients treated for pelvic chondrosarcoma. Material and methods: This series included 67 patients (27 women and 40 men) treated between 1971 and 1996 for pelvic chondrosarcoma. Mean age at diagnosis was 45 years (range 18–78). Forty percent of the tumours were grade I. The most frequent localisation was the iliac bone. Conservative surgery was performed in 45 patients. The only surgical treatment possible in 22 patients was an inter-ilioabdominal disarticulation. Resection margins were adequate for only 19 patients (wide resection). Marginal resection was noted in 14 patients with intra-tumour resection in 17. Results: Overall 5- and 8-year survival was 65% and 58% respectively. Local recurrence rate was 40%, occurring a mean 27 months after initial surgery. Statistical analysis did not reveal any correlation between tumour size, tumour grade, type of surgery, resection margin, and local recurrence. Results were nevertheless less favourable in case of inadequate surgical margins. Tumour grade, tumour size, patient age, gender, and quality of resection did not have a significant effect on overall survival. Local recurrence was the only negative factor predictive of survival (p< 0.05). Discussion: Development of local recurrence appears to be the most important negative predictive factor in patients with pelvic chondrosarcoma. In this localisation, satisfactory resection margins are often difficult to achieve. Most authors propose inter-ilioabdominal disarticulation as a last resort procedure. The question of the indication for more aggressive initial surgery to obtain more radical resection margins remains open