Aims. Intra-articular . 90. Yttrium (. 90. Y) is an adjunct to surgical treatment by synovectomy for patients with diffuse-type tenosynovial giant-cell tumour (dtTGCT) of the knee, with variable success rates. Clinical information is, however, sparse and its value remains unclear. We investigated the long-term outcome of patients who underwent synovectomy with and without adjuvant treatment with . 90. Yttrium. Patients and Methods. All patients with dtTGCT of the knee who underwent synovectomy between 1991 and 2014 were included in the study. Group A patients underwent synovectomy and an intra-articular injection of . 90. Yttrium between six and eight weeks after surgery. Group B patients underwent surgery alone. Results. There were 34 patients in group A and 22 in group B. Recurrence of dtTGCT was identified by MRI, which was undertaken in patients with further symptoms. At a mean follow-up of 7.3 years (2.5 to 25.4), there was residual disease in 15 patients in group A and 11 in group B (p < 0.363). The mean Musculoskeletal Tumor Society (MSTS) score at final follow-up was 85% and 83%, respectively (p < 0.91). Conclusion. There were no significant differences in outcome between patients treated surgically for dtTGCT of the knee with or without an adjuvant intra-articular injection of . 90. Yttrium. We were unable to provide conclusive evidence of any benefits derived from the adjuvant treatment. Cite this article: Bone Joint J 2018;100-B:984–8

Between December 1995 and March 2003, 38 adult patients with intermediate or high-grade liposarcoma in a limb were treated by limb-sparing surgery and post-operative radiotherapy. The ten-year local recurrence-free survival was 83%, the ten-year metastasis-free survival 61%, the ten-year disease-free survival 51% and the ten-year overall survival 67%. Analysis of failure and success showed no association with the age of the patients, gender, the location of the primary tumour, the type of liposarcoma and the quality of resection.

Our results indicate that liposarcoma may recur even ten years after the end of definitive therapy and may spread to unexpected sites as for soft-tissue sarcoma.

Aims

The aims of this study were to evaluate the efficacy of preoperative denosumab in achieving prospectively decided intention of therapy in operable giant cell tumour of bone (GCTB) patients, and to document local recurrence-free survival (LRFS).

We investigated whether the presence of a pathological fracture increased the risk of local recurrence in patients with a giant cell tumour (GCT) of bone. We also assessed if curettage is still an appropriate form of treatment in the presence of a pathological fracture. We conducted a comprehensive review and meta-analysis of papers which reported outcomes in patients with a GCT with and without a pathological fracture at presentation. We computed the odds ratio (OR) of local recurrence in those with and without a pathological fracture.

We selected 19 eligible papers for final analysis. This included 3215 patients, of whom 580 (18.0%) had a pathological fracture. The pooled OR for local recurrence between patients with and without a pathological fracture was 1.05 (95% confidence interval (CI) 0.66 to 1.67, p = 0.854). Amongst the subgroup of patients who were treated with curettage, the pooled OR for local recurrence was 1.23 (95% CI 0.75 to 2.01, p = 0.417).

A post hoc sample size calculation showed adequate power for both comparisons.

There is no difference in local recurrence rates between patients who have a GCT of bone with and without a pathological fracture at the time of presentation. The presence of a pathological fracture should not preclude the decision to perform curettage as carefully selected patients who undergo curettage can have similar outcomes in terms of local recurrence to those without such a fracture.

Cite this article: Bone Joint J 2015;97-B:1566–71.

Aims. The existing clinical guidelines do not describe a clear indication for adjuvant radiotherapy (RT) in the treatment of superficial soft tissue sarcomas (STSs). We aimed to determine the efficacy of adjuvant RT for superficial STSs. Methods. We retrospectively studied 304 patients with superficial STS of the limbs and trunk who underwent surgical resection at a tertiary sarcoma centre. The efficacy of RT was investigated according to the tumour size and grade: group 1, ≤ 5 cm, low grade; group 2, ≤ 5cm, high grade; group 3, > 5 cm, low grade; group 4, > 5 cm, high grade. Results. The five- and ten-year local recurrence-free survival (LRFS) for all patients was 88% and 81%, respectively. While the efficacy of adjuvant RT was not proven in local control of all patients (five-year LRFS; RT+, 90% versus RT-, 83%; p = 0.074), the LRFS was significantly improved by adjuvant RT in group 2 (five-year LRFS; RT+, 96% versus RT-, 82%; p = 0.019), and group 4 (five-year LRFS; RT+, 87% versus RT-, 73%; p = 0.027). In groups 2 and 4, adjuvant RT significantly reduced the LR risk if the resection margin was clear but less than 5 mm; the LR rate was 7% with adjuvant RT compared with 26% with surgery alone (p = 0.003). There was no statistical relationship with the use of adjuvant RT and survival in every group. Conclusion. Adjuvant RT reduces the risk of local recurrence in patients with superficial high-grade STS regardless of tumour size, especially when resection margin is less than 5 mm. Cite this article: Bone Joint J 2020;102-B(8):1088–1094

Aims. To investigate the benefits of denosumab in combination with nerve-sparing surgery for treatment of sacral giant cell tumours (GCTs). Methods. This is a retrospective cohort study of patients with GCT who presented between January 2011 and July 2017. Intralesional curettage was performed and patients treated from 2015 to 2017 also received denosumab therapy. The patients were divided into three groups: Cohort 1: control group (n = 36); cohort 2: adjuvant denosumab group (n = 9); and cohort 3: neo- and adjuvant-denosumab group (n = 17). Results. There were 68 patients within the study period. Six patients were lost to follow-up. The mean follow-up was 47.7 months (SD 23.2). Preoperative denosumab was found to reduce intraoperative haemorrhage and was associated with shorter operating time for tumour volume > 200 cm. 3. A total of 17 patients (27.4%) developed local recurrence. The locoregional control rate was 77.8% (7/9) and 87.5% (14/16) respectively for cohorts 2 and 3, in comparison to 66.7% (24/36) of the control group. The recurrence-free survival (RFS) rate was significantly higher for adjuvant denosumab group versus those without adjuvant denosumab during the first two years: 100% vs 83.8% at one year and 95.0% vs 70.3% at two years. No significant difference was found for the three-year RFS rate. Conclusion. Preoperative denosumab therapy was found to reduce intraoperative haemorrhage and was associated with shorter operating times. Adjuvant denosumab was useful to prevent early recurrence during the first two years after surgery. Cite this article: Bone Joint J 2020;102-B(2):177–185

Aims. Adjuvant treatment after intralesional curettage for atypical cartilaginous tumours (ACTs) of long bones is widely accepted for extending surgical margins. However, evaluating the isolated effect of adjuvant treatment is difficult, and it is unclear whether not using such adjuvants provides poor oncological outcomes. Hence, we analyzed whether intralesional curettage without cryosurgery or chemical adjuvants provides poor oncological outcomes in patients with an ACT. Patients and Methods. A total of 24 patients (nine men, 15 women) (mean age 45 years; 18 to 62) were treated for ACTs of long bones and followed up for a median of 66 months (interquartile range 50 to 84). All patients were treated with extensive manual curettage and limited burring. Bone cement and grafts were used to fill bone defects in 16 and eight patients, respectively. No chemical adjuvants or cryosurgery were used. Results. No local recurrence was detectable on plain radiographs and MRI or CT images. At the last follow-up, there were no distant metastases or disease-specific deaths. No procedure-related complications or postoperative fractures developed. Conclusion. Intralesional curettage without cryosurgery or chemical adjuvants may provide excellent oncological outcomes for patients with ACTs of long bones, without the risk of complications related to adjuvant use. Our investigation suggests thorough curettage alone is a reasonable treatment option for ACT. However, we acknowledge the limited size of our investigation warrants a multicentre collaborative study to confirm our findings. Cite this article: Bone Joint J 2018;100-B:256–61

Aims. Clear cell sarcoma (CCS) of soft-tissue is a rare melanocytic subtype of mesenchymal malignancy. The aim of this study was to investigate the clinical and therapeutic factors associated with increased survival, stratified by clinical stage, in order to determine the optimal treatment. Methods. The study was a retrospective analysis involving 117 patients with histologically confirmed CCS, between July 2016 and November 2017, who were enrolled in the Bone and Soft Tissue Tumour Registry in Japan. Results. The five- and ten-year survival rates were 41% (95% confidence interval (CI) 29 to 52) and 37% (95% CI 25 to 49), respectively. On multivariable analysis, the size of the tumour of > 10 cm (p = 0.006), lymph node metastasis at the time of diagnosis (p < 0.001), distant metastases at the time of diagnosis (p < 0.001), and no surgery for the primary tumour (p = 0.019) were independently associated with a poor survival. For N0M0 CCS (n = 68), the development of distant metastases was an independent prognostic factor for survival (early (< 12 months), hazard ratio (HR) 116.78 (95% CI 11.69 to 1,166.50); p < 0.001; late (> 12 months), HR 14.79 (95% CI 1.66 to 131.63); p = 0.016); neoadjuvant/adjuvant chemotherapy (p = 0.895) and/or radiotherapy (p = 0.216) were not significantly associated with survival. The five-year cumulative incidence of local recurrence was 19% (95% CI 8 to 35) and the size of the tumour was significantly associated with an increased rate of local recurrence (p = 0.012). For N1M0 CCS (n = 18), the risk of mortality was significantly lower in patients who underwent surgery for both the primary tumour and lymph node metastases (HR 0.03 (95% CI 0.00 to 0.56); p = 0.020). For M1 CCS (n = 31), excision of the primary tumour was independently associated with better survival (HR 0.26 (95% CI 0.09 to 0.76); p = 0.013). There was no significant difference in survival between the different types of systemic treatment (p = 0.523). Conclusion. Complete excision of the primary tumour and lymph nodes is associated with a better survival in patients with CCS. Systemic treatment appears to provide limited benefits, demonstrating a pressing need for novel systemic agents. Cite this article: Bone Joint J 2023;105-B(11):1216–1225

Aims. Due to its indolent clinical behaviour, the treatment paradigm of atypical cartilaginous tumours (ACTs) in the long bones is slowly shifting from intralesional resection (curettage) and local adjuvants, towards active surveillance through wait-and-scan follow-up. In this retrospective cohort study performed in a tertiary referral centre, we studied the natural behaviour of ACT lesions by active surveillance with MRI. Clinical symptoms were not considered in the surveillance programme. Methods. The aim of this study was to see whether active surveillance is safe regarding malignant degeneration and local progression. In total, 117 patients were evaluated with MRI assessing growth, cortical destruction, endosteal scalloping, periosteal reaction, relation to the cortex, and perilesional bone marrow oedema. Patients received up to six follow-up scans. Results. At the time of the first follow-up MRI, 8% of the lesions showed growth (n = 9), 86% remained stable (101), and 6% decreased in size (n = 7). During the third follow-up, with a mean follow-up time of 60 months (SD 23), 24 patients were scanned, of whom 13% had lesions that had grown and 13% lesions that had decreased in size. After 96 months (SD 37), at the sixth follow-up MRI, 100% of the lesions remained stable. None of the lesions showed malignant progression and although some lesions grew in size (mean 1 mm (SD 0.8)), no malignant progression occurred. Conclusion. We conclude that active surveillance with MRI is safe for ACTs in the long bones in the short- and mid-term follow-up. Cite this article: Bone Joint J 2024;106-B(1):86–92

Giant cell tumours (GCTs) of the small bones of the hands and feet are rare. Small case series have been published but there is no consensus about ideal treatment. We performed a systematic review, initially screening 775 titles, and included 12 papers comprising 91 patients with GCT of the small bones of the hands and feet. The rate of recurrence across these publications was found to be 72% (18 of 25) in those treated with isolated curettage, 13% (2 of 15) in those treated with curettage plus adjuvants, 15% (6 of 41) in those treated by resection and 10% (1 of 10) in those treated by amputation. We then retrospectively analysed 30 patients treated for GCT of the small bones of the hands and feet between 1987 and 2010 in five specialised centres. The primary treatment was curettage in six, curettage with adjuvants (phenol or liquid nitrogen with or without polymethylmethacrylate (PMMA)) in 18 and resection in six. We evaluated the rate of complications and recurrence as well as the factors that influenced their functional outcome. At a mean follow-up of 7.9 years (2 to 26) the rate of recurrence was 50% (n = 3) in those patients treated with isolated curettage, 22% (n = 4) in those treated with curettage plus adjuvants and 17% (n = 1) in those treated with resection (p = 0.404). The only complication was pain in one patient, which resolved after surgical removal of remnants of PMMA. We could not identify any individual factors associated with a higher rate of complications or recurrence. The mean post-operative Musculoskeletal Tumor Society scores were slightly higher after intra-lesional treatment including isolated curettage and curettage plus adjuvants (29 (20 to 30)) compared with resection (25 (15 to 30)) (p = 0.091). Repeated curettage with adjuvants eventually resulted in the cure for all patients and is therefore a reasonable treatment for both primary and recurrent GCT of the small bones of the hands and feet. Cite this article: Bone Joint J 2013;95-B:838–45

Introduction. Local recurrence of Giant cell tumours of bone (GCT) is considered the main complication of surgical treatment (50%). Intra-lesional curettage with adjuvants like phenol or polymethylmethacrylate (PMMA) is recommended as initial treatment, decreasing the risk of recurrence. However, risk factors for local recurrence in skeletal GCT have not yet been firmly established and a golden standard for treatment remains controversial. Aim of this study is identification of risk factors for recurrence in GCT, specifically after intra-lesional curettage with or without adjuvants. Methods. In a retrospective single-institution study 191 patients treated for GCT between 1964 and 2009 were included. Mean follow-up was 111 months (range 12-415). The recurrence-free survival and hazards for different treatment strategies and various patient and tumour characteristics were determined. Results. Overall risk of recurrence was 36.1% (n=66, 95% CI: 28.3-42.1). Recurrence rate after wide resection was 20%, after curettage with adjuvants 33% and after curettage alone 77%. Hogendoorn-grade III (Hazard Ratio: 5.7, p⋋0.001), localisation in axial skeleton (HR: 3.7, p⋋0,001), primary treatment in a non-specialised centre (HR: 2.8, p⋋0.001) and extension into soft tissue (HR: 2.0, p=0.02) were significant risk factors for local recurrence. Curettage with adjuvants proved superior to curettage alone (p⋋0.004 p=0.07, HR: 0.54), but the application of both PMMA and phenol did not present a significantly better outcome than PMMA alone (HR: 1.07, p=0.9). Discussion. Of all possible risk factors only soft tissue extension and localisation in distal radius significantly influenced the risk of local recurrence for all treatments. We found that high-grade tumours and localisation in the axial skeleton were additional risk factors for local recurrence after intra-lesional surgery. Although wide resection increases morbidity, it can be the therapy of choice in high risk patients. Intra-lesional therapy can be advised for low risk patients using curettage adjuvants and PMMA

Introduction. The role of adjuvants in curettage for giant cell tumours (GCT) is still controversial. Our aim was to determine if adjuvant cementation lowers local recurrence (LR) rates for GCTs treated with curettage. Methods. Detailed curettage has been the principal treatment for GCT for the past 30 years. Cement was used from 1996 onwards for tumours where there was concern about structural stability. We investigated factors affecting LR and also the incidence of complications for treatment with or without cement. Results. From 1975 to 2008, 330 patients with GCT were treated primarily with curettage. Eighty-four (25%) received adjuvant treatment with acrylic bone cementation. Cement was only used in Campannacci grade 2 or 3 GCTs. LR for curettage was 30% compared with 14% for curettage plus cementation. (p = 0.001). LR was halved by the use of cement for both stage 2 and stage 3 tumours (Stage 2, 8% LR with cement, 21% without (p=0.02); Stage 3, 19% with cement, 48% without (p⋋0.001)). On multivariate analysis both stage and use of cement were independent significant factors in predicting LR. Site was not significant although the distal tibia and proximal humerus had lower risk of LR than other sites. Cement was however associated with a higher risk for subsequent joint replacement surgery. In patients without LR, 18% with cement needed a joint replacement compared to 2% without. In patients with LR, 75% with cement required a joint replacement, compared to 44% without. Discussion. Although adjuvant cementation seems to give better local control for curettage of GCT, it is associated with an increased need for subsequent joint replacement

Aim. To study the efficacy of Zoledronic acid in the treatment of benign osteolytic tumours or tumour like conditions of bone as a therapeutic or as an adjuvant agent. Method. 31 patients- 19 female, 12 male, age from 8 yrs to 42 yrs, were treated with intravenous zoledronic acid. In 17 patients (fibrous dysplasia-10, nonossifying fibroma- 4, UBC- 3) zoledronic acid alone was used as a therapeutic agent. In 14 patients (ABC- 3, GCT- 11), it was used as an adjuvant agent after curettage. Four patients presented with pathological fracture. In all patients, 4 mg. zoledronic acid was given at 2 monthly intervals. In 12 adult patients, in addition oral bisphosphonates - alendronate was given weekly for at least 6 months. Results. Patients were evaluated using visual analog pain scale and x-rays. At last follow-up (6-40 months), in 15 patients, treated with zoledronic acid alone, there was thickening of cortices and reduction in the size of the lesion. Pain score decreased from an average of 8 to 2. All four fractures healed. In 2 patients, there was progression in size of the lesion. In 14 patients, where it was used as an adjuvant agent, there was early thickening of bone cortices. There was no local recurrence in this group. There was no adverse reaction to the drug in any of the patient. Conclusion. Zoledronic acid is a third generation bisphosphonates and a proven anti-osteolytic agent. It has proved effective in the treatment of number of osteolytic conditions. Our study also suggests that Zoledronic acid not only help to stabilize these lesions but also resulted in pronounced healing in majority of the patients. It also reduced recurrence rate in aggressive benign bone tumours such as ABC or GCT when used as an adjuvant treatment

Aim. To present selective arterial embolization with N-2-butyl Cyanoacrylate for the palliative and/or adjuvant treatment of painful bone metastases not primarily amenable to surgery. Material and Methods. From January 2003 to December 2009, 243 patients (148 men and 95 women; age range, 20-87 years) with painful bone metastases were treated with N-2-butyl Cyanoacrylate. Overall, 309 embolizations were performed;. 56 patients had more than one embolization. Embolizations were performed in the pelvis (168 procedures), in the spine (83 procedures), in the upper limb (13 procedures), in the lower limb (38 procedures) and in the thoracic cage (21 procedures). Primary cancer included urogenital, breast, gastrointestinal, thyroid, lung, musculoskeletal, skin, nerve and unknown origin. Renal cell carcinoma was the most commonly treated tumour. In all patients, selective embolization was performed by transfemoral catheterization. Results. Complete or significant resolution of pain for more than 6 months was observed in 236 patients (97%), in 56 of them (23%) after repeated embolization; 12 patients had subsequent surgical treatment. Function was restored to normal in the patients with extremity tumours. In 21 cases, after angiography embolization was judged infeasible due to arteriovascular reasons or high risk. Skin and subcutaneous necrosis at the pelvis occurred in one patient after multiple embolizations for a metastatic paraganglioma. Conclusions. Selective embolization of bone metastases with N-2-butyl Cyanoacrylate is safe and effective for the palliative and/or adjuvant management of painful bone metastases. It can be used for surgically inaccessible or irresponsive to radiation therapy lesions, and as an adjuvant to surgical treatment

Aim. 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. Method. 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). Results. PCI was found to induce longer delay in tumour growth than photodynamic therapy (PDT). In combination with surgery little was achieved with respect to tumour growth delay by adding the photosensitizer and light (photodynamic therapy, PDT) while PCI induced synergistic effect. Conclusion. The results indicate that PCI targets the viable peripheral zone of the tumour where PDT is apparently less effective. PCI with BLM seems promising as an adjuvant treatment after inadequate resection of sarcomas. The PCI technology is currently explored in a Phase I/II trial in University Hospital (London, UK)

Introduction. Angiosarcomas are rare aggressive sarcomas of vascular endothelial origin. These tumours have the potential to be multicentric and are associated with high rates of local recurrence, which makes treatment challenging. The gold-standard is that these patients are managed in specialist centres by a multidisciplinary team. We present our experience of managing patients with angiosarcoma in the North of England Bone and Soft Tissue Tumour Service and a review of the literature. Methods. A prospectively collated electronic database was used to identify patients with angiosarcoma treated between 2000 and 2008, and an analysis performed of demographics, anatomical site, surgical excision and reconstruction, local disease recurrence and metastatic disease. Results. Fifteen patients (ten female, ?ve male, mean age 71 years) were identi?ed. Eight patients developed tumours in a previously irradiated area, after a mean of 11 years. Six patients had metastatic disease at presentation. Fourteen patients underwent wide surgical excision of the tumour, of which nine required defect reconstruction(?ve free latissimus dorsi ?aps, two free anterolateral thigh ?aps, two pedicled latissimus dorsi ?aps). One patient was treated with chemotherapy only. Five of 14 patients received adjuvant radiotherapy, and one received adjuvant chemotherapy. Two out of 14 patients developed local recurrence. Eight patients developed metastases, the majority of which were pulmonary. Estimated ?ve-year survival was calculated as 33% in our patient cohort. Conclusions. Angiosarcomas are aggressive, difficult to treat tumours, which can occur secondary to a multitude of causes. Clinical suspicion, biopsy and early diagnosis are essential to allow optimum treatment, which currently consists of radical surgery, together with adjuvant radiotherapy and chemotherapy

Aims. The aim of this paper was to investigate the prognostic factors for local recurrence in patients with pathological fracture through giant cell tumours of bone (GCTB). Patients and Methods. A total of 107 patients presenting with fractures through GCTB treated at our institution (Royal Orthopaedic Hospital, Birmingham, United Kingdom) between 1995 and 2016 were retrospectively studied. Of these patients, 57 were female (53%) and 50 were male (47%).The mean age at diagnosis was 33 years (14 to 86). A univariate analysis was performed, followed by multivariate analysis to identify risk factors based on the treatment and clinical characteristics. Results. The initial surgical treatment was curettage with or without adjuvants in 55 patients (51%), en bloc resection with or without reconstruction in 45 patients (42%), and neoadjuvant denosumab, followed by resection (n = 3, 3%) or curettage (n = 4, 4%). The choice of treatment depended on tumour location, Campanacci tumour staging, intra-articular involvement, and fracture displacement. Neoadjuvant denosumab was used only in fractures through Campanacci stage 3 tumours. Local recurrence occurred in 28 patients (25%). Surgery more than six weeks after the fracture did not affect the risk of recurrence in any of the groups. In Campanacci stage 3 tumours not treated with denosumab, en bloc resection had lower local recurrences (13%), compared with curettage (39%). In tumours classified as Campanacci 2, intralesional curettage and en bloc resections had similar recurrence rates (21% and 24%, respectively). After univariate analysis, the type of surgical intervention, location, and the use of denosumab were independent factors predicting local recurrence. Further surgery was required 33% more often after intralesional curettage in comparison with resections (mean 1.59, 0 to 5 vs 1.06, 0 to 3 operations). All patients treated with denosumab followed by intralesional curettage developed local recurrence. Conclusion. In patients with pathological fractures through GCTB not treated with denosumab, en bloc resection offers lower risks of local recurrence in tumours classified as Campanacci stage 3. Curettage or resections are both similar options in terms of the risk of local recurrence for tumours classified as Campanacci stage 2. The benefits of denosumab followed by intralesional curettage in these patients still remains unclear

Aim. To compare two consecutive treatment programs in metastatic osteosarcoma at presentation. Methods. Between 1987 and 1999 treatment of stage IIIB osteosarcoma included induction chemotherapy (CHT) which consisted in 3-5 cycles of intra-arterial doxorubicin (DOX) 75 mg/m2 or cis-platin (CDDP) 120 mg/m2, surgery or definitive local radiation therapy (RT) for primary and adjuvant CHT with DOX, CDDP or HDMTX. Thirty nine patients were included in this protocol. Since 2000 a more intensive induction CHT protocol was adopted. It consisted in 4 cycles of DOX 90 mg/m2 +CDDP 120 mg/m2 (24 patients) or DOX 60 mg/m2 +CDDP 120 mg/m2+Ifosfamide (IFO) 6 g/m2 (7 patients). Adjuvant chemotherapy included DOX, CDDP, IFO or VP-16. Residual lung deposits were removed at the end of adjuvant treatment. Results. In the old protocol surgical removal of primary after preoperative CHT was performed only in 24% of cases. There was only one limb-salvage procedure. Due to early disease progression 19 patients (51%) received induction CHT only. Ten patients (25%) received CHT and RT. In the last series the distribution according to treatment was as follows: CHT + surgery – 61% (limb-salvage procedures in 14 cases (74%)); CHT + RT – 23%; CHT only – 16%. Overall survival was 18±7% and 7 ±5% at 5 and 3 years respectively in the old protocol compared with 42±10% and 21±10% in the recent series, p=0.004. Median survival increased from 11 to 27 months. Conclusions. More aggressive CHT and systematic removal of residual lung metastases significantly increased the rate of limb-salvage procedures and improved survival. Overall results in metastatic osteosarcoma at presentation remain unsatisfactory

Aims

Giant cell tumour of bone (GCTB) is a locally aggressive lesion that is difficult to treat as salvaging the joint can be associated with a high rate of local recurrence (LR). We evaluated the risk factors for tumour relapse after treatment of a GCTB of the limbs.

Aim. 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. Method. 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. Results. There were 21 males and 15 females, mean age was 53.6+/−19.3 years (range 14-84 yrs); 23 patients (63.9%) presented with localised disease while distant metastases were detected in 13 patients (36.1%). Primary tumour involved the extremities in 25 patients (69.4%) and the girdles or trunk in 11 (30.6%). Surgery consisted of a limb-salvage procedure in 25 cases (69.4%) and amputation in 9 (25%) while 2 patients refused surgery (5.6%). Multiagent chemotherapy was administered in 19 patients (52.8%). At mean follow-up of 5.8 years, 23 patients had died of disease, expected 5 and 10 year overall survival rates were 41% and 31%. Tumour volume (p=0.01) and age at presentation (p=0.07) were the most important predictors of survival. Disease-specific survival rate was 43.5% for patients with localised disease and 23% in patients with metastasis; difference did not reach statistical significance. Addition of adjuvant chemotherapy to local management was associated with improved disease-free survival in patients with localised disease (60% vs 31%, p=0.09). Conclusion. Extraskeletal OGS was associated with overall worse prognosis than skeletal OGS despite multimodal management. Incidence of metastasis at diagnosis (36.1%) was higher than usually observed in skeletal OGS. Tumour volume and age were significant factors affecting the outcome while adjuvant chemotherapy showed a trend towards improved survival in patients with localised disease