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
We evaluated the risk of late
Aims. Tenosynovial giant cell tumour (TGCT) is one of the most common soft-tissue tumours of the foot and ankle and can behave in a locally aggressive manner. Tumour control can be difficult, despite the various methods of treatment available. Since treatment guidelines are lacking, the aim of this study was to review the multidisciplinary management by presenting the largest series of TGCT of the foot and ankle to date from two specialized sarcoma centres. Methods. The Oxford Tumour Registry and the Leiden University Medical Centre Sarcoma Registry were retrospectively reviewed for patients with histologically proven foot and ankle TGCT diagnosed between January 2002 and August 2019. Results. A total of 84 patients were included. There were 39 men and 45 women with a mean age at primary treatment of 38.3 years (9 to 72). The median follow-up was 46.5 months (interquartile range (IQR) 21.3 to 82.3). Localized-type TGCT (n = 15) predominantly affected forefoot, whereas diffuse-type TGCT (Dt-TGCT) (n = 9) tended to panarticular involvement. TGCT was not included in the radiological differential diagnosis in 20% (n = 15/75). Most patients had open rather than arthroscopic surgery (76 vs 17). The highest recurrence rates were seen with Dt-TGCT (61%; n = 23/38), panarticular involvement (83%; n = 5/8), and after arthroscopy (47%; n = 8/17). Three (4%) fusions were carried out for osteochondral destruction by Dt-TGCT. There were 14 (16%) patients with Dt-TGCT who underwent systemic treatment, mostly in refractory cases (79%; n = 11). TGCT initially decreased or stabilized in 12 patients (86%), but progressed in five (36%) during follow-up; all five underwent subsequent surgery. Side effects were reported in 12 patients (86%). Conclusion. We recommend open surgical excision as the primary treatment for TGCT of the foot and ankle, particularly in patients with Dt-TGCT with extra-articular involvement. Severe osteochondral destruction may justify salvage procedures, although these are not often undertaken. Systemic treatment is indicated for unresectable or refractory cases. However, side effects are commonly experienced, and
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. 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.Aims
Methods
The aim of this study was to investigate the safety and efficacy of 3D-printed modular prostheses in patients who underwent joint-sparing limb salvage surgery (JSLSS) for malignant femoral diaphyseal bone tumours. We retrospectively reviewed 17 patients (13 males and four females) with femoral diaphyseal tumours who underwent JSLSS in our hospital.Aims
Methods
Venous tumour thrombus (VTT) is a rare finding in osteosarcoma. Despite the high rate of VTT in osteosarcoma of the pelvis, there are very few descriptions of VTT associated with extrapelvic primary osteosarcoma. We therefore sought to describe the prevalence and presenting features of VTT in osteosarcoma of both the pelvis and the limbs. Records from a single institution were retrospectively reviewed for 308 patients with osteosarcoma of the pelvis or limb treated between January 2000 and December 2022. Primary lesions were located in an upper limb (n = 40), lower limb (n = 198), or pelvis (n = 70). Preoperative imaging and operative reports were reviewed to identify patients with thrombi in proximity to their primary lesion. Imaging and histopathology were used to determine presence of tumour within the thrombus.Aims
Methods
Radiotherapy is a well-known local treatment for spinal metastases. However, in the presence of postoperative systemic therapy, the efficacy of radiotherapy on local control (LC) and overall survival (OS) in patients with spinal metastases remains unknown. This study aimed to evaluate the clinical outcomes of post-surgical radiotherapy for spinal metastatic non-small-cell lung cancer (NSCLC) patients, and to identify factors correlated with LC and OS. A retrospective, single-centre review was conducted of patients with spinal metastases from NSCLC who underwent surgery followed by systemic therapy at our institution from January 2018 to September 2022. Kaplan-Meier analysis and log-rank tests were used to compare the LC and OS between groups. Associated factors for LC and OS were assessed using Cox proportional hazards regression analysis.Aims
Methods
Osteosarcoma is the most common primary bone malignancy among children and adolescents. We investigated whether benzamil, an amiloride analogue and sodium-calcium exchange blocker, may exhibit therapeutic potential for osteosarcoma in vitro. MG63 and U2OS cells were treated with benzamil for 24 hours. Cell viability was evaluated with the MTS/PMS assay, colony formation assay, and flow cytometry (forward/side scatter). Chromosome condensation, the terminal deoxynucleotidyl transferase dUTP nick end labelling (TUNEL) assay, cleavage of poly-ADP ribose polymerase (PARP) and caspase-7, and FITC annexin V/PI double staining were monitored as indicators of apoptosis. Intracellular calcium was detected by flow cytometry with Fluo-4 AM. The phosphorylation and activation of focal adhesion kinase (FAK) and signal transducer and activator of transcription 3 (STAT3) were measured by western blot. The expression levels of X-linked inhibitor of apoptosis protein (XIAP), B-cell lymphoma 2 (Bcl-2), B-cell lymphoma-extra large (Bcl-xL), SOD1, and SOD2 were also assessed by western blot. Mitochondrial status was assessed with tetramethylrhodamine, ethyl ester (TMRE), and intracellular adenosine triphosphate (ATP) was measured with BioTracker ATP-Red Live Cell Dye. Total cellular integrin levels were evaluated by western blot, and the expression of cell surface integrins was assessed using fluorescent-labelled antibodies and flow cytometry.Aims
Methods
Between 1986 and 2002, 42 patients with synchronous multifocal osteosarcoma were treated with two different protocols of neoadjuvant chemotherapy. When feasible, the primary and secondary tumours were excised as a combined procedure. After initial chemotherapy 26 patients were excluded from simultaneous excision of all their secondary bone lesions as their disease was too advanced. In 12 patients only isolated excision of the primary lesion was possible. For 16 patients simultaneous operations were conducted to excise the primary and secondary lesions. This involved two supplementary sites in 15 patients and four additional sites in one patient. Of these, 15 attained remission but 12
Aim. Ten years ago at the EMSOS 2000 meeting we have presented our experience concerning the non-surgical treatment of stage IIB osteosarcoma of extremities. The purpose of study was to evaluate long-term results and complications related to this non-standard and controversial treatment modality. Methods. Since 1988 definitive radiation gamma therapy (RT) in the median dose of 60 Gy was administrated to 36 patients who refused surgery after preoperative chemotherapy. Until 1999 induction consisted in 3-5 cycles of intra-arterial doxorubicin (DOX) 75 mg/m2 or cis-platin (CDDP) 120 mg/m2. Since 2000 induction comprised 4 cycles of DOX 90 mg/m2+CDDP 120 mg/m2 intra-arterially. Tumour response before local treatment was assessed non-invasively by means of X-ray, CT, MRI, angiography, 3-phase bone scans and alkaline phosphatase measurement. The end-points were overall (OS), metastases free (MFS) and local progression free (LPFS) survival at 10 years. Results. OS, MFS and LPFS by Kaplan-Meier method were 48±9%, 51±9%, and 45±11% respectively. The outcome was significantly related to response assessed before RT. In 12 good responders OS, MFS and LPFS were 74±13%, 91±9%, and 69±19% respectively compared with 29±11%, 27±11%, and 34±13% in 24 non-responders, p- value 0.03, 0.003, 0.009, respectively. In 14 patients 5 years survival after local progression and delayed surgery was 38±10%. Median survival time after local
We have previously reported cryoablation-assisted joint-sparing surgery for osteosarcoma with epiphyseal involvement. However, it is not clear whether this is a comparable alternative to conventional joint arthroplasty in terms of oncological and functional outcomes. A total of 22 patients who had localized osteosarcoma with epiphyseal involvement around the knee and underwent limb salvage surgery were allocated to joint preservation (JP) group and joint arthroplasty (JA) group. Subjects were followed with radiographs, Musculoskeletal Tumor Society (MSTS) score, and clinical evaluations at one, three, and five years postoperatively.Aims
Methods
Cell-free DNA (cfDNA) and circulating tumour DNA (ctDNA) are used for prognostication and monitoring in patients with carcinomas, but their utility is unclear in sarcomas. The objectives of this pilot study were to explore the prognostic significance of cfDNA and investigate whether tumour-specific alterations can be detected in the circulation of sarcoma patients. Matched tumour and blood were collected from 64 sarcoma patients (n = 70 samples) prior to resection of the primary tumour (n = 57) or disease recurrence (n = 7). DNA was isolated from plasma, quantified, and analyzed for cfDNA. A subset of cases (n = 6) underwent whole exome sequencing to identify tumour-specific alterations used to detect ctDNA using digital droplet polymerase chain reaction (ddPCR).Aims
Methods
Bone metastasis ultimately occurs due to a complex multistep process, during which the interactions between cancer cells and bone microenvironment play important roles. Prior to colonization of the bone, cancer cells must succeed through a series of steps that will allow them to gain migratory and invasive properties; epithelial-to-mesenchymal transition (EMT) is known to be integral here. The aim of this study was to determine the effects of G protein subunit alpha Q (GNAQ) on the mechanisms underlying bone metastasis through EMT pathway. A total of 80 tissue samples from patients who were surgically treated during January 2012 to December 2014 were used in the present study. Comparative gene analysis revealed that the GNAQ was more frequently altered in metastatic bone lesions than in primary tumour sites in lung cancer patients. We investigated the effects of GNAQ on cell proliferation, migration, EMT, and stem cell transformation using lung cancer cells with GNAQ-knockdown. A xenograft mouse model tested the effect of GNAQ using micro-CT analyses and histological analyses.Aims
Methods
Introduction. Myxofibrosarcoma is described by WHO as comprising of a spectrum of malignant fibroblastic lesions with variably myxoid stoma, pleomorphism and with a distinctively curvilinear vascular pattern. They are reported to be one of the commonest sarcomas of elderly patients. It has previously been reported to have a high rate of locally recurrent disease (50-60%). The aim of the study was to investigate the ROH series of tumours to determine prognostic factors for survival and local recurrence. Methods. Patients were identified from the ROH database who had been treated with a new presentation of myxofibrosarcoma. The size, grade and depth of the tumour at presentation was noted. Those patients who had suffered local or distal disease
Aims Although chondrosarcomas (CSs) display true malignant features, including local recurrence (LR) and metastases, their behaviour in the hands and feet is thought to differ from that in other parts of the axial and appendicular skeleton by having a lower metastatic potential. The purpose of this study was to investigate the disease-specific and surgical factors that affect the local and systemic prognosis of CS of the hands and feet. A multicentre retrospective study was carried out at two tertiary sarcoma centres. A database search identified all patients with a CS treated between January 1995 and January 2018. There were 810 CSs of which 76 (9.4%) were located in the fingers, toes, metacarpals, and metatarsal bones. The median age of the study population was 55 years (36 to 68) with a median follow-up of 52 months (22 to 87) months. Overall, 70% of the tumours were in the hand (n = 54) and 30% in the foot (n = 22). Predictors for LR were margin (p = 0.011), anatomical location (p = 0.017), and method of surgical management (p = 0.003). Anatomical location (p = 0.026), histological grade between 1 and 3 (p = 0.004) or 2 and 3 (p = 0.016), and surgical management (p = 0.001) were significant factors for LR-free survival. Disease-specific survival was affected by histological grade (p < 0.001), but not by LR (p = 0.397).Methods
Results
Introduction. To look at the effect of width and tissue at surgical margins, together with the use of adjuvant therapy on locally recurrent disease and disease free survival. Methods. The distance (mm) and tissue has been regularly reported for 5 years, prior to this it was reported as wide, marginal or intra-lesional. It is known from previous studies that 90% of locally recurrent disease for osteosarcoma and Ewing's occurs within 2 years from surgery. Therefore all primary bone sarcomas of the pelvis and appendicular skeleton that underwent surgery between 1/1/2005 and 1/1/2008 were entered, giving at least 2 years follow up. The pathology records were reviewed and the margins and percentage tumour necrosis were recorded. Those patients who had suffered local or distal disease
Aim. To evaluate outcome and complications of knee arthrodesis with a modular prosthetic system (MUTARS(r) Implantcast), as primary and revision implants in musculoskeletal oncology. Method. Between 1975 and 2009, 24 prostheses were used for knee arthrodesis. Nineteen in oncologic cases: 6 osteosarcomas, chondrosarcoma, synovial sarcoma and metastatic carcinoma 3 each, 2 pigmented villonodular synovitis (PVNS), malignant fibrous hystiocitoma and giant cell tumour 1 each. Patients were grouped into: A) primary implants, B) revision implants. Group A included 9 patients: 8 arthrodeses after extra-articular resection with major soft tissue removal, 1 after primary resection following multiple excisions of locally recurrent PVNS. Group B included 15 patients: 12 arthrodeses for infection (5 infected TKAs, 7 infected megaprostheses), 2 for failures of temporary arthodesis with Kuntscher nail and cement, 1 for recurrent chondrosarcoma in previous arthrodesis. Results. Oncologic outcome ata mean follow-up of 6 years (ranging 1 to 26), showed 13 NED (68.4%), 2 NED after treatment of
From the European Osteosarcoma Intergroup study 202 patients were assessed with respect to their surgical treatment. Although treated in three different centres the survival of the three groups was identical (57% at five years). Two of the centres had rates of limb salvage of 85% and 83%, respectively, while the third had a rate of 49%. The corresponding risks of local recurrence were 13.3%, 6.8% and 2.5%, with all local recurrences arising in limbs with attempted limb salvage. Local recurrence was closely related to the adequacy of the margins of excision and to the chemotherapeutic response. Patients who had undergone limb-salvage surgery and who developed local recurrence still survival at five years). Of patients who
Our aim was to develop and validate nomograms that would predict the cumulative incidence of sarcoma-specific death (CISSD) and disease progression (CIDP) in patients with localized high-grade primary central and dedifferentiated chondrosarcoma. The study population consisted of 391 patients from two international sarcoma centres (development cohort) who had undergone definitive surgery for a localized high-grade (histological grade II or III) conventional primary central chondrosarcoma or dedifferentiated chondrosarcoma. Disease progression captured the first event of either metastasis or local recurrence. An independent cohort of 221 patients from three additional hospitals was used for external validation. Two nomograms were internally and externally validated for discrimination (c-index) and calibration plot.Aims
Methods
Introduction. Recently a great deal of interest has emerged in new techniques for resection of bone tumours, such as the use of computer guided surgery, joint sparing prostheses and epiphysiolysis. However, all the techniques may require narrower margins at resection than the traditional Enneking wide margins. The aim of the study was to look at the effect of width and tissue at surgical margins, together with the use of adjuvant therapy on locally recurrent disease and disease free survival. Methods. The quality in terms of precise distance (mm) and tissue has been regularly reported by our pathologists for 5 years, prior to this it was reported as wide, marginal or intralesional. It is known from previous studies that 90% of locally recurrent disease for osteosarcoma and Ewings occurs within 2 years from surgery. Therefore all primary bone sarcomas of the pelvis and appendicular skeleton that underwent surgery between 1/1/2005 and 1/1/2008 were entered into the study group, giving at least 2 years follow up. The pathology records were reviewed and the quality of the margins and percentage tumour necrosis were recorded. Those patients who had suffered local or distal disease