Aims. Socioeconomic and racial disparities have been recognized as impacting the care of patients with cancer, however there are a lack of data examining the impact of these disparities on patients with bone sarcoma. The purpose of this study was to examine socioeconomic and racial disparities that impact the oncological outcomes of patients with bone sarcoma. Methods. We reviewed 4,739 patients diagnosed with primary bone sarcomas from the Surveillance, Epidemiology and End Results (SEER) registry between 2007 and 2015. We examined the impact of race and insurance status associated with the presence of metastatic disease at diagnosis, treatment outcome, and overall survival (OS). Results. Patients with Medicaid (odds ratio (OR) 1.41; 95% confidence interval (CI) 1.15 to 1.72) and uninsured patients (OR 1.90; 95% CI 1.26 to 2.86) had higher risks of metastatic disease at diagnosis compared to patients with health insurance. Compared to White patients, Black (OR 0.63, 95% CI 0.47 to 0.85) and Asian/Pacific Islander (OR 0.65, 95% CI 0.46 to 0.91) were less likely to undergo surgery. In addition, Black patients were less likely to receive chemotherapy (OR 0.67, 95% CI 0.49 to 0.91) compared to White patients. In patients with chondrosarcoma, those with Medicaid had worse OS compared to patients with insurance (hazard ratio (HR) 1.65, 95% CI 1.06 to 2.56). Conclusion. In patients with a bone sarcoma, the cancer stage at diagnosis varied based on insurance status, and racial disparities were identified in treatment. Further studies are needed to identify modifiable factors which can mitigate socioeconomic and racial disparities found in patients with bone sarcomas. Cite this article: Bone Joint Res 2022;11(5):278–291

Aims. Surgical limb sparing for knee-bearing paediatric bone sarcoma is considered to have a clinically significant influence on postoperative function due to complications and leg-length discrepancies. However, researchers have not fully evaluated the long-term postoperative functional outcomes. Therefore, in this study, we aimed to elucidate the risk factors and long-term functional prognosis associated with paediatric limb-sparing surgery. Methods. We reviewed 40 patients aged under 14 years who underwent limb-sparing surgery for knee bone sarcoma (15 cases in the proximal tibia and 25 in the distal femur) between January 2000 and December 2013, and were followed up for a minimum of five years. A total of 35 patients underwent reconstruction using artificial materials, and five underwent biological reconstruction. We evaluated the patients’ postoperative complications, survival rate of reconstruction material, and limb, limb function, and leg-length discrepancy at the final follow-up, as well as the risk factors for each. Results. Complications were observed in 55% (22/40) of patients. The limb survival and reconstruction material rates at five and ten years were 95% and 91%, and 88% and 66%, respectively. Infection was the only risk factor in both survivals (p < 0.001, p = 0.019). In the 35 patients with limb preservation, the median International Society of Limb Salvage (ISOLS) score at the final follow-up was 80 (47% to 97%). Younger age (p = 0.027) and complications (p = 0.005) were poor prognostic factors. A negative correlation was found between age and leg-length discrepancy (R = −0.426; p = 0.011). The ISOLS scores were significantly lower in patients with a leg-length discrepancy of more than 5 cm (p = 0.005). Conclusion. Young age and complications were linked to an unfavourable functional prognosis. Leg-length correction was insufficient, especially in very young children, resulting in decreased function of the affected limb. Limb-sparing surgery for these children remains a considerable challenge. Cite this article: Bone Jt Open 2024;5(10):868–878

Aims. Treatment of high-grade limb bone sarcoma that invades a joint requires en bloc extra-articular excision. MRI can demonstrate joint invasion but is frequently inconclusive, and its predictive value is unknown. We evaluated the diagnostic accuracy of direct and indirect radiological signs of intra-articular tumour extension and the performance characteristics of MRI findings of intra-articular tumour extension. Methods. We performed a retrospective case-control study of patients who underwent extra-articular excision for sarcoma of the knee, hip, or shoulder from 1 June 2000 to 1 November 2020. Radiologists blinded to the pathology results evaluated preoperative MRI for three direct signs of joint invasion (capsular disruption, cortical breach, cartilage invasion) and indirect signs (e.g. joint effusion, synovial thickening). The discriminatory ability of MRI to detect intra-articular tumour extension was determined by receiver operating characteristic analysis. Results. Overall, 49 patients underwent extra-articular excision. The area under the curve (AUC) ranged from 0.65 to 0.76 for direct signs of joint invasion, and was 0.83 for all three combined. In all, 26 patients had only one to two direct signs of invasion, representing an equivocal result. In these patients, the AUC was 0.63 for joint effusion and 0.85 for synovial thickening. When direct signs and synovial thickening were combined, the AUC was 0.89. Conclusion. MRI provides excellent discrimination for determining intra-articular tumour extension when multiple direct signs of invasion are present. When MRI results are equivocal, assessment of synovial thickening increases MRI’s discriminatory ability to predict intra-articular joint extension. These results should be interpreted in the context of the study’s limitations. The inclusion of only extra-articular excisions enriched the sample for true positive cases. Direct signs likely varied with tumour histology and location. A larger, prospective study of periarticular bone sarcomas with spatial correlation of histological and radiological findings is needed to validate these results before their adoption in clinical practice. Cite this article: Bone Jt Open 2024;5(10):944–952

Aim. To estimate the risk of bone malignancy arising in premalignant conditions. Methods. There are quite a number of possible premalignant conditions with considerable uncertainty about the actual risk of a bone sarcoma developing. The incidence of these malignant conditions was identified from a prospective database containing 3000 primary bone sarcomas. Results. 178 of the 3000 patients with newly diagnosed bone sarcomas had a pre-exiting condition which in all probability led to the sarcoma. These included 50 with previous radiotherapy treatment and 47 with Paget's disease. 31 patients developed malignancy in HME, 8 with neurofibromatosis and 7 each with Ollier's disease and retinoblastoma. There were 4 malignancies in patients with Mafucci's syndrome, 3 in patients with fibrous dysplasia, 3 in patients with synovial chondromatosis and 2 in patients with Rothmund-Thomson syndrome. Given that the incidence of bone sarcomas is 9/million population per year, our 3000 patients represent 333 million population years. When the incidence of a condition is known in the population this allows an estimation of the risk of malignancy compared with the normal population. Retinoblastoma for instance is known to arise in 1 in 16000 births. The 7 malignancies we saw thus represents a risk to individuals with retinoblastoma of 336/million/yr - a figure 37 times the risk of the normal population. Approximate figures of risk have been calculated for other entities. Conclusion. Data from a supra-regional register allows an approximate estimate of the increased risk of bone tumours in premalignant conditions

Navigation-assisted surgery has been reported to enhance resection accuracy in bone sarcoma surgery. Patient-specific instruments (PSIs) have been proposed as a simpler alternative with fewer setup facilities. We investigated the use of 3D surgical planning and PSI in realising computer planning of complex resections in bone sarcoma patients with regards to surgical accuracy, problems, and early clinical results. We retrospectively studied twelve patients with bone sarcoma treated surgically by PSIs with 3D planning. The procedure was planned using engineering software. The resection accuracy was accessed by comparing CT images of tumour specimens with the planned in seven patients. Mean age was 30.9 (9 – 64). Mean follow-up was 3.1 year (0.5 – 5.3). 31 planes of bone resections were successfully performed using the technique and were considered accurate. The mean time required for placing PSIs was 5.7 minutes (1 – 10) and performing bone osteotomies with the assistance of PSIs was 4.7 minutes (2 – 7). The mean maximum deviation error was 1.7mm (0.5 – 4.4). One PSI was broken during bone resection, and one patient needed re-resection using the same PSI. One pelvic patient died of local recurrence and lung metastases six months postoperatively. One patient developed a soft tissue local recurrence and lung metastasis at 20 months after surgery. The mean MSTS functional score was 27.9 (21 – 30). There were no complications related to 3D planning and PSIs. In selected patients, 3D surgical planning and PSIs replicate complex bone resections and reconstructions in bone sarcoma surgery. Comparative studies with conventional or navigation- assisted resections are required

Aim: To estimate the risk of bone malignancy arising in premalignant conditions. Methods: There are quite a number of possible premalignant conditions with considerable uncertainty about the actual risk of a bone sarcoma developing. The incidence of these malignant conditions was identified from a prospective database containing 3000 primary bone sarcomas. Results: 178 of the 3000 patients with newly diagnosed bone sarcomas had a pre-exiting condition which in all probability led to the sarcoma. These included 50 with previous radiotherapy treatment and 47 with Paget’s disease. 31 patients developed malignancy in HME, 8 with neurofibromatosis and 7 each with Ollier’s disease and retinoblastoma. There were 4 malignancies in patients with Mafucci’s syndrome, 3 in patients with fibrous dysplasia, 3 in patients with synovial chondromatosis and 2 in patients with Rothmund-Thomson syndrome. Given that the incidence of bone sarcomas is 9/million population per year, our 3000 patients represent 333 million population years. When the incidence of a condition is known in the population this allows an estimation of the risk of malignancy compared with the normal population. Retinoblastoma for instance is known to arise in 1 in 16000 births. The 7 malignancies we saw thus represents a risk to individuals with retinoblastoma of 336/million/yr – a figure 37 times the risk of the normal population. Approximate figures of risk have been calculated for other entities. Conclusion: Data from a supra-regional register allows an approximate estimate of the increased risk of bone tumours in premalignant conditions

Objectives: To investigate whether components of MSTS-87 (Pain, ROM, Strength, Stability, Deformity, Acceptance and Function) correlate with function as measured by TESS following endoprosthetic replacement (EPR) for patients with bone sarcoma. Methods: 255 patients with extremity bone sarcoma treated by resection & EPR were identified from a prospective database. From this group we investigated 111 patients with primary bone sarcoma with > 2 years follow up, evaluated by both MSTS-87 & TESS, no local recurrence, metastasis or major complication for at least 2 years prior to the follow-up. Upper extremity patients were excluded due to small numbers. We examined the influence of patient demographics and tumour characteristics on functional outcome scores. Correlation between MSTS-87 & TESS was performed using linear regression analysis. Results: Age, gender, tumour size, anatomical site, chemotherapy treatment and presence of pathological fracture did not significantly correlate with TESS. Linear regression analysis of MSTS-87 individual criteria and total score revealed that only pain, ROM and function helped explain the TESS score (p < 0.05) while strength, stability, deformity & acceptance had no significant effect on overall functional outcome. Conclusions: Of the seven MSTS-87 variables, only pain, ROM and function significantly correlate with overall functional outcome as measured by TESS following EPR for bone sarcoma. This suggests that patients with decreased strength, stability, deformity and acceptance as defined by MSTS-87 scores, may still adapt well with good overall functional outcomes

Purpose: Prosthetic arthroplasty is the most common method of reconstruction of segmental bone defects following resection of bone sarcomas about the knee. The purpose of this study was to determine the survivorship of the reconstructions in short- and long-term follow-up. Methods: A retrospective study was performed on all patients diagnosed with a bone sarcoma between 1984 and 1995 who were treated with a limb-sparing osteoarticular resection and rotating hinge prosthetic knee arthroplasty. Prosthetic survival was calculated with endpoints of analysis based on any event, any prosthesis-related event and aseptic loosening of the prosthesis, which led to prosthetic revision, removal or limb amputation. Results: A total of 154 reconstructions were performed involving the distal femur (n=111) and proximal tibia (n=43). The median resection length was 155 mm (105–250mm) for the distal femur and 117 mm (85–150 mm) for the proximal tibia. All implants were fixed with polymethylmethacrylate cement. Early complications (within one year postoperatively) developed in fewer than 2 % of patients. Aseptic loosening accounted for the majority of events resulting in prosthetic failures (distal femur = 17 [median failure 34 mos]; proximal tibia = 10 [median failure 100 mos]). Polyethylene bushing wear was observed in seven patients (median time to replacement = 156 months). Conclusion: The early outcome of prosthetic arthroplasty was extremely favorable supporting this method of reconstruction following excision of high-grade bone sarcomas about the knee. Long-term survival of these prostheses is suboptimal and can be anticipated to be poor for the proximal tibia. Aseptic loosening continues to be the primary cause of prosthetic failure about the knee

Aim. The use of megaprostheses for knee reconstruction after distal femur resection in young bone sarcoma patients has become popular since early ′80. The authors reviewed their experience with different distal femur megaprostheses in children. Method. 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. Results. At a 74 months follow-up (29-294), 72 patients are alive; but 43 of them (60%) had further surgery related to primary implant. Surgical revision rate was 88% (39% for mechanical failure) in long survivors treated before 1995 and 50% (14% with mechanical failure) in more recent cases (p< 0.05). Three long survivors progressed in time to total femur megaprostheses. Five out the 15 cases treated by custom made expandable prostheses were revised before completion of skeletal growth because of implant failure. Conclusion. In limb-salvage for bone sarcoma, megaprostheses are the preferred method to reconstruct distal femur in growing children, but a durable reconstruction is not easy to be achieved. The use of new devices specially addressed to younger patients deserves a serious scientific survey by musculoskeletal oncology community

Our centre has used a specially designed custom-made endoprostheses with curved stems to reconstruct femoral defects in patients with residual short proximal femur after excision of primary bone sarcoma over the last 18 years. Two designs of endoprostheses with curved intramedullary stems were used: the rhinohorn stem type and the bifid stem type. We report the safety, survival and functional outcome of this form of reconstruction. Twenty six patients who had these special endoprosthesis reconstruction were studied. The median age was 16 years (range 7 to 60 years). Prostheses with rhino horn stems were used in 15 patients and bifid-stem in 1 1 patients. Twenty patients had the prostheses inserted as a primary procedure after excision of primary bone sarcoma, and in six patients the prostheses were inserted after revision surgery of failed distal femur endoprostheses. Seventeen patients (65%) were alive and free of disease at a median follow-up of 98 months (12 to 203 months) and nine patients had died of metastatic disease. Local recurrence developed in two patients (1 0%) out of the 20 patients. Surgical complications occurred in five patients (191/o). Deep infections occurred in two patients (8%) requiring revision surgery in one patient. Prosthetic failure, occurred in nine patients (35%). The cumulative survival of prostheses was 69% at five years and 43% at 10 years. Musculoskeletal Tumour Society mean functional score was 83% (53% to 97%). In conclusion, preservation of a short segment of the proximal femur and the use of endoprostheses with curved stems for reconstruction of the femur is technically possible. There is an increased risk of fracture of the prostheses decreasing the survival rate. Functional outcome of patients with this form of reconstruction is not significantly different from the functional outcome of patients who have proximal femur or total femur endoprosthetic reconstruction. This operation is particularly desirable in skeletally immature patients and allows normal development of the acetabulum

Objectives. To assess the affects of a delay in diagnosis on the survival rates of Bone Sarcoma (BS) using size and symptom duration as measures of delay. Methods. All patients diagnosed with a Primary BS from 1970 to 2005 were included. Demographic data concerning age, sex, diagnosis and tumour site were recorded. The data were collected retrospectively from a prospective database, with 2573 patients included. Symptom duration is defined as the length of time the patient was experiencing symptoms for until the date of diagnosis. Survival analysis was performed using Kaplan-Meier curves and Cox Regression was carried out to identify variables affecting outcome. Results. 2489 patients with Primary BS that matched out inclusion and exclusion criteria were identified. Osteosarcoma (40.4%), Chondrosarcoma (20.6%) and Ewing's Sarcoma (17.6%) were the most common diagnoses, with a median size of 10cm at presentation. Overall 5 year survival was 61.2%, extremity sited tumours carried a statistically significant better prognosis compared to axial tumours (5yr survival 63.2% vs. 53.3%) and patients with axial tumours had significantly longer symptom duration (Mann-Whitney U test p = 0.000 31 and 12 weeks). Overall analysis of all BS diagnoses showed lengthier duration of symptoms was associated with a better outcome (Hazard Ratio = 0.998, p = 0.000), which suggests that 1 extra week of symptoms confers an increased survival of 0.2%. Larger tumour size was associated with a poorer survival rate (p=0.000 to 3 sig. fig, Hazard Ratio 1.059 respectively) which suggests that for every 1cm increase in size there is a decreased survival of 5.9%. Conclusions. Although many factors have been associated with survival, the limited literature concerning symptom interval and outcome is varied. This large study has shown that regardless of diagnosis, overall, shorter symptom duration and a large tumour size are associated with a poorer prognosis

Nowadays 80% of patients with bone sarcomas can benefit from limb salvage. Their disease-free life expectancy is not jeopardised by conservative surgery as long as safe margins are obtained. For this reason, the oncological result relies on the accuracy of pre-operative and per-operative surgical measurements. Pre-operative evaluation of tumours is now quite accurate with digital margins (computed tomography, MNR, digital angiography). However, surgeons are still using centimeters or conventional radiographs with their own technical limitations for per-operative evaluation. A more accurate technique is needed. The system is composed of three components: 1) a color, graphic computer workstation with software to calculate and present the location of the surgical instrument on a three-dimensional, reconstructed bone image, 2) a complete set of hand-held instruments containing infrared emitters, 3) an infrared receiver linked to the work station. This measuring system enables determination of the position and incidence of a surgical instrument in real time during surgery, with an accuracy of less than one mm. The system requires four steps: 1) recording data with C.T., N.M.R. or angiography, 2) creating a three-dimensional image displayed on the computer screen for preoperative simulation of a virtual operation, 3) recording the very important anatomical points of the patient and optimal incidences of the surgical instruments, 4) preoperative location of surgical instruments and control of their location on bone. This system is very useful for resection of bone tumours when the conventional location is uncertain (innonimate bone, rib), when very sharp accuracy is needed to preserve the growth plate of the distal femur in young children, and to avoid medullary damage in a spinal tumour. The frameless stereotactic device is also very accurate in the reconstructive phase of limb salvage. After an internal hemipelvectomy, the device permits localisation of the acetabular prosthesis in the precise location before resection. In our practice, the accuracy of the video guiding system is always within two mm as compared to conventional measurements usually between one or two cm for long bones and three to five cm for innominate bone. The use of a video guidance system is very beneficial for limb salvage surgery for pelvic bone tumours

Nowadays 80% of patients with bone sarcomas can benefit from limb salvage. Their disease-free life expectancy is not jeopardised by conservative surgery as long as safe margins are obtained. For this reason, the oncological result relies on the accuracy of pre-operative and per-operative surgical measurements. Pre-operative evaluation of tumours is now quite accurate with digital margins (computed tomography, MNR, digital angiography). However, surgeons are still using centimeters or conventional radiographs with their own technical limitations for per-operative evaluation. A more accurate technique is needed. The system is composed of three components: 1) a color, graphic computer workstation with software to calculate and present the location of the surgical instrument on a three-dimensional, reconstructed bone image, 2) a complete set of hand-held instruments containing infrared emitters, 3) an infrared receiver linked to the work station. This measuring system enables determination of the position and incidence of a surgical instrument in real time during surgery, with an accuracy of less than one mm. The system requires four steps: 1) recording data with C.T., N.M.R. or angiography, 2) creating a three-dimensional image displayed on the computer screen for preoperative simulation of a virtual operation, 3) recording the very important anatomical points of the patient and optimal incidences of the surgical instruments, 4) preoperative location of surgical instruments and control of their location on bone. This system is very useful for resection of bone tumours when the conventional location is uncertain (innonimate bone, rib), when very sharp accuracy is needed to preserve the growth plate of the distal femur in young children, and to avoid medullary damage in a spinal tumour. The frameless stereotactic device is also very accurate in the reconstructive phase of limb salvage. After an internal hemipelvectomy, the device permits localisation of the acetabular prosthesis in the precise location before resection. In our practice, the accuracy of the video guiding system is always within two mm as compared to conventional measurements usually between one or two cm for long bones and three to five cm for innominate bone. The use of a video guidance system is very beneficial for limb salvage surgery for pelvic bone tumours

Aim. In patients with bone sarcoma, placing mega prostheses in the proximal tibia is associated with high rates of infection. In studies with small numbers of patients and short follow-up periods, silver-coated mega prostheses have been reported to lead to reduced infection rates. To the best of our knowledge, this study is the largest one that has compared the infection rates with titanium versus silver-coated mega prostheses in patients treated for sarcomas in the proximal tibia. Method. The infection rate in 98 patients with sarcoma or giant cell tumour in the proximal tibia who underwent placement of a titanium (n = 42) or silver-coated (n = 56) mega prosthesis. *. was assessed, along with the treatments administered for any infection. Results. As the primary end point of the study, the rates of infection were 16.7% in the titanium group and 8.9% in the silver group, resulting in 5-year prosthesis survival rates of 90% in the silver group and 84% in the titanium group. Overall, seven of 56 patients in the silver group (12.5%) developed periprosthetic infection. Two patients became infected after revision surgery due to mechanical failure of the prosthesis. In the titanium group, one patient developed a periprosthetic infection after revision surgery (which was carried out in 50% of patients) due to a mechanical prosthetic failure, leading to an overall infection rate of 19.0% (eight of 42). Overall, nine of 12 (75%) periprosthetic infections in the two groups occurred within the first 2 years postoperatively, if later revision surgery due to mechanical failure was not necessary. Whereas three of the eight patients in the titanium group (37.5%) ultimately had to undergo amputation due to infected proximal tibia replacement, these mutilating surgical procedures were necessary in the silver group in only one patient (14.3%). In the titanium group, two-stage revision surgery with a temporary antibiotic-impregnated cement spacer was ultimately successful in four of eight patients (50.0%), but this procedure was necessary in only one patient in the silver group (14.3%). Conclusions. The use of silver-coated prostheses reduced the infection rate in a relatively large and homogeneous group of patients. In addition, less aggressive treatment of infection was possible in the group with silver-coated prostheses

Background: Bone sarcomas are the fourth most common cancer in individuals under 25 years. Limb salvage procedures are popular for the treatment of osteosarcomas as they have functional and physiological benefits over traditional amputative procedures. Objectives: To apply disease specific measures to a group of intra-articular knee osteosarcoma patients and to evaluate structural and treatment variables predictive of the functional outcome scores. Methods: Twenty patients (10., 10.) treated with tumour resection and endoprosthetic knee arthroplasty took part in the study. The Musculoskeletal Tumour Society (MSTS) rating scale and the Toronto Extremity Salvage Score (TESS) were used to assess impairment and disability along with a musculoskeletal eveluation. Results: Impairment was recorded as 83.33% and disability was recorded as 86%. Task difficulty was shown to increase for activities requiring large knee flexion angles, presumably due to increased patellofemoral forces. Correlations were observed between the MSTS score and (a) knee flexion range of motion, (b) muscle removal, and also between the TESS and (a) time from surgery, (b) quadriceps strength and (c) knee flexion range of motion. ANOVA revealed no significant differences in impairment (P = 0.962) or disability (P = 0.411) between the differing types of prostheses. Conclusions: Clinicians and therapists should emphases restoration of post surgical range of motion and strength in order to enhance functional recovery

Purpose: Malignant primary tumours of the spine require wide resection with preservation of the cord and radicular elements. The purpose of this work was to report our oncological results and complications after spinal surgery for this indication. Material and methods: Twenty-two patients, mean age 30 years (15–65) underwent surgery. The pathology diagnosis was made preoperatively. There were 16 high-grade tumours, Ewing (n=7), osteosarcoma (n=5), other (n=4), and six low-grade tumours, chondrosarcoma (n=5), osteosarcoma (n=1). Four patients experienced local recurrence after an insufficient initial resection and three required emergency laminectomy. Sagittal hemivertebrectomy was performed in 11 patients for pediculotransverse tumours and total vertebrectomy in 10 patients for corporeal tumours. Posterior fixation was not used in one patient (Ewing tumour) in order to preserve the Adamkiewitz artery. Results: Complete oncological resection was achieved in 14 patients. The surgical margins were in a malignant zone in 7. At mean 6-year follow-up, ten patients were surviving disease-free (4 Ewing, 4 osteosarcoma, 2 chondrosarcoma), and one was living with active disease (chondrosarcoma). Eleven patients died: metastasis (n=4), local recurrence (n=6), infarction 3 months after surgery (n=1). Among the seven patients with local recurrence,osteosarcoma (n=5),chondrosarcoma (n=2), three had local recurrence at initial management and only one was living at last follow-up (active chondrosarcoma). There were no neurological complications; there were four mechanical complications (nonunion) after total vertebrectomy which required four re-operations. Discussion: Survival rate in this series was 45% at six years, comparable with rates reported in the literature (40 – 50% at 5 years). Local recurrence was observed in 85% of patients whose surgical margins were in malignant tissue (67–100% in the literature). Among the four patients who had recurrent disease at the time of surgery, complete resection was possible in only one. This patient is living (Ewing sarcoma responding to adjuvant therapy). Incomplete surgery or a poor biopsy procedure aggravates the prognosis. Mechanical failure is observed after total vertebrectomy if anterior osteosynthesis is not associated with the posterior fixation. Conclusion: Wide surgical resection of primary bone sarcomas of the spine provides encouraging results when the initial operation is successful. Better local control of Ewing sarcoma can be explained by its sensitivity to adjuvant therapy. Reconstruction after total vertebrectomy required anterior and posterior fixation

EUROpean Bone Over 40 Sarcoma Study (EURO-B.O.S.S.) is the first prospective multicenter international study for patients 41–65 year old with high-grade bone sarcoma. Patients with HG Osteosarcoma (OS), HG sarcoma NOS (S), Fibrosarcoma, MFH, Leiomyosarcoma, Dedifferentiated Chondrosarcoma (DCh) were included. Chemotherapy: Combinations of cisplatin/doxorubicin (CDP 100mg/m2/ADM 60mg/m2), ifosfamide/CDP(IFO 6g/m2/CDP 100mg/m2) and IFO/ADM (IFO 6g/m2/ADM 60mg/m2) were repeated three times (9 cycles). Surgery was planned after 3 cycles. Methotrexate (8g/m2) was postoperatively added in poor responders. Immediate surgery was allowed and 9 cycles with CDP, ADM, IFO were postoperatively given. At December 2007, 140 patients were registered (median age 51 years). OS (51%), S (16%), and DCh (11%) were the more frequent histotypes. Synchronous metastases in 30 (21%) patients, central location of tumor in 45(32%).Surgical complete remission (SCR) was achieved in 84% of patients, (localized 91%, meta-static 37%) without difference among the histology groups. One surgical-related and one chemotherapy-related death were reported. Grade4 WBC and PLT incidence was 55% and 17%.Renal toxicity and peripheral neurotoxicity were reported in 16% and 20% of patients. With a median follow-up of 25 months (4–68) 3 year OS was 58% (95%CI 48–68%) [7% (95%CI 0–19%) without SCR]. In patients with SCR, 3-year OS and EFS were 46% (95%CI 9–83%) and 0% in case of synchronous metastases and 69% (95%CI58–80%) and 45% (95%CI33–57%) for localized patients; 50% (95%CI 29–71%) and 40% (95%CI 20–59%) for patients with central tumor, 73% (95%CI61–85%) and 44% (95%CI31–57%) for those with extremity tumor; 68% (95%CI 52–83%) and 46% (95%CI 32–54%) for OS, 64% (95%CI 42–85%) and 48% (95%CI 25–71%) for S, 48% (95%CI 13–82%) and 27% (95%CI 1–54%) for DCh. The protocol is feasible, but the chemotherapy-related toxicity is remarkable. Surgical complete remission is the main factor influencing survival. Central location and synchronous metastases are negative prognostic factors, but 50% 3-year OS can be achieved with aggressive local and systemic treatment. Osteosarcoma and high-grade sarcoma NOS benefit from chemotherapy more than patients with dedifferentiated chondrosarcoma

Abstract

From January 2003 a long term follow-up project started for adult patients treated in our Centre for cancer in pediatric age, to evaluate late effects of therapy. For all patients a personalized follow-up was scheduled (time, function-tests, etc).

We analyzed 24 cases of bone tumors: 14 osteosarcoma (OS) and 10 Ewing’s sarcoma (ES). Median age at diagnosis was 13 years (range 11–18) for OS patients, 11.6 years (range 6–18) for ES; 50% males in both groups. All patients were treated according current CNR/ISG-protocols: all OS cases underwent surgery; in 5/10 ES patients local treatment was surgery, in 5/10 radiotherapy; 7/24 received hematopoietic stem cells transplantation (HSCT).

Median age at evaluation is respectively 26.5 years (range 18.7–34) and 23.5 (range 21.6–32); median follow-up is 13 years (range 6–22) and 13.7 (range 6.7–22.3).

Cardiovascular function is normal in all OS cases; 3/10 ES patients developed asymptomatic ejection fraction reduction, currently not treated.

One OS patient underwent bilateral thoracotomy and HSCT for multiple metastases at diagnosis and had a mild lung function alteration. One OS patient developed mild chronic kidney disease, one ES nephrolithiasis. Liver function is normal in all cases.

Height velocity and final height are normal in 10/14 OS and 9/10 ES patients; in remaining 5/24 no growth hormone secretion deficit was found.

One OS patient developed primitive hypothyroidism and one OS benign thyroid nodule with partial thyroidectomy; one patient multifocal papillary thyroid carcinoma with total thyroidectomy at 11 years from diagnosis of ES.

Spermatogenesis deficit is a common find (5/7 OS and 5/5 ES male patients); one female treated with HSCT and radiotherapy for ES pelvic relapse has primitive hypogonadism. No other hypothalamo-hypophyseal-adrenocortical system hormones deficit was found.

We reported no significant neuropsychological alterations nor employment problems: 20/24 patients have a job, 4/24 are students. Three OS females have children.