José M. Casanova

Mesenchymal chondrosarcoma: prognostic factors and outcome in 113 patients. A European Musculoskeletal Oncology Society study.

BACKGROUND Mesenchymal chondrosarcoma (MCS) is a distinct, very rare sarcoma with little evidence supporting treatment recommendations. PATIENTS AND METHODS Specialist centres collaborated to report prognostic factors and outcome for 113 patients. RESULTS Median age was 30 years (range: 11-80), male/female ratio 1.1. Primary sites were extremities (40%), trunk (47%) and head and neck (13%), 41 arising primarily in soft tissue. Seventeen patients had metastases at diagnosis. Mean follow-up was 14.9 years (range: 1-34), median overall survival (OS) 17 years (95% confidence interval (CI): 10.3-28.6). Ninety-five of 96 patients with localised disease underwent surgery, 54 additionally received combination chemotherapy. Sixty-five of 95 patients are alive and 45 progression-free (5 local recurrence, 34 distant metastases, 11 combined). Median progression-free survival (PFS) and OS were 7 (95% CI: 3.03-10.96) and 20 (95% CI: 12.63-27.36) years respectively. Chemotherapy administration in patients with localised disease was associated with reduced risk of recurrence (P=0.046; hazard ratio (HR)=0.482 95% CI: 0.213-0.996) and death (P=0.004; HR=0.445 95% CI: 0.256-0.774). Clear resection margins predicted less frequent local recurrence (2% versus 27%; P=0.002). Primary site and origin did not influence survival. The absence of metastases at diagnosis was associated with a significantly better outcome (P<0.0001). Data on radiotherapy indications, dose and fractionation were insufficiently complete, to allow comment of its impact on outcomes. Median OS for patients with metastases at presentation was 3 years (95% CI: 0-4.25). CONCLUSIONS Prognosis in MCS varies considerably. Metastatic disease at diagnosis has the strongest impact on survival. Complete resection and adjuvant chemotherapy should be considered as standard of care for localised disease.

Extra-articular shoulder resections: outcomes of 54 patients

BACKGROUND The survival of patients with tumors around the shoulder treated with extra-articular resection, the rates of reconstructions-related complications, and the function of the shoulder cannot be estimated because of limited available data from mainly small published related series and case reports. METHODS We studied 54 patients with tumors around the shoulder treated with extra-articular shoulder resections and proximal humeral megaprosthetic reconstructions from 1985 to 2012. Mean tumor volume was 549 cm3, and the mean length of the proximal humeral resection was 110 mm. Mean follow-up was 7.8 years (range, 3-21 years). We evaluated the outcomes (survival, metastases, recurrences, and function) and the survival and complications of the reconstruction. RESULTS Survival of patients with malignant tumors was 47%, 38%, and 35%, at 5, 10, and 20 years, respectively. Rates for metastasis and local recurrence were 60% and 18.5%, respectively. Survival was significantly higher for patients without metastases at diagnosis, tumor volume <549 cm3, and type IV resections. Survival of reconstructions was 56% at 10 years and 48% 20 years. Overall, 19 patients (35.2%) experienced 30 complications (55.5%), the most common being soft tissue failures that required subsequent surgery without, however, implant removal. The mean Musculoskeletal Tumour Society score was 25 points, without any significant difference between the types of extra-articular resections. CONCLUSION Tumor stage and volume as well as type of resection are important predictors of survival of patients with malignant tumors around the shoulder. Survival of the reconstructions is satisfactory; nevertheless, the complication rate is high. The Musculoskeletal Tumour Society score is similar with respect to the type of resection.

Agreement between dual x-ray absorptiometers using pencil beam and fan beam: indicators of bone health and whole-body plus appendicular tissue composition in adult athletes.

OBJECTIVE The current study was aimed to examine intra-individual variation on indicators of bone health in addition to whole-body plus appendicular tissue measurements using two concurrent assessments based on pencil beam and fan beam dual energy X-ray absorptiometry (DXA) systems in adult athletes from several sports. METHOD Thirty-two male participants (27.6±10.1 years) were measured on anthropometry including multifrequency bioelectric impedance and air-displacement plethysmography. Bone mineral content (BMC), bone area, fat and lean soft tissue were derived using pencil beam (Lunar DPX-MD+) and fan beam (Lunar iDXA) absorptiometry. Bone mineral density (BMD) was obtained for the femoral neck, trochanter and triangle of ward. Finally, the right thigh was defined as a region of interest (ROI). Analyses comprised intra-class correlation (ICC), Effect size (d) from mean differences of repeated measurements, coefficient of variation (CV). RESULTS ICC were >0.900 for all measurements. Intra-individual differences were large for BMC (d=1,312; CV=2,7%), bone area (d=1,761; CV=2,7%), fat tissue (d=1,612; CV=11%) and all indicators of appendicular lean soft tissue (d=1,237-1687; CV=2,0-4,1%). A very large difference (d=4,014; CV=8.4%) was diagnosed for lean soft tissue of the ROI. CONCLUSION Although differences among concurrent instruments for BMC and bone area, the effect size of mean differences was negligible for BMD. Fat and lean soft tissue derived from DXA should be interpreted as reference values (not criterion) due to equipment-related variation, more apparently in the ROI values.

Neoadjuvant Trabectedin plus Radiotherapy in High-Grade Sarcoma of the Leg: A Case Report

Here, we present the case of a 78-year-old male patient with undifferentiated spindle cell sarcoma on the posteromedial surface of the right leg who experienced a long-lasting progression-free survival. Due to an underlying cardiac disease, the patient was not suitable for anthracyclines. In September 2015, he received first-line chemotherapy with trabectedin (Yondelis®) at the approved dosage and regimen – concomitant with external radiotherapy (RT). After the first 9 cycles of trabectedin plus RT given in the neoadjuvant setting, the patient underwent surgical resection. At that stage, we observed a very good pathological response with 80% of necrotic area. The patient resumed the therapy with trabectedin; however, approximately 5 months later, we observed a new nodular heterogeneous lesion with ill-defined margins in the right leg and suggestive of tumor relapse. Subsequently an above-the-knee amputation was performed, and the patient resumed his trabectedin therapy with the same dosage and regimen. In January 2018, almost 2 1/2 years after the start of trabectedin treatment and 30+ cycles of trabectedin, the patient is locoregionally and distant metastatically disease-free. Currently, the treatment with trabectedin is maintained without any significant serious toxicity. Future clinical trials are needed to gain additional insights into the role of trabectedin maintenance therapy until disease progression in the neoadjuvant setting and to identify predictive and prognostic criteria for response to trabectedin in patients with advanced sarcoma.

Total and regional bone mineral and tissue composition in female adolescent athletes: comparison between volleyball players and swimmers

BackgroundExploring the osteogenic effect of different bone-loading sports is particular relevant to understand the interaction between skeletal muscle and bone health during growth. This study aimed to compare total and regional bone and soft-tissue composition between female adolescent swimmers (n=20, 15.71±0.93 years) and volleyball players (n=26, 16.20±0.77 years).MethodsDietary intake was obtained using food frequency questionnaires. Body size was given by stature, sitting height, and body mass. Six skinfolds were measured. Bone mineral content (BMC) and density (BMD), lean soft tissue, and fat tissue were assessed using dual-energy X-ray absorptiometry. Pearson’s product moment correlation coefficients were calculated to examine the relationships among variables, by type of sport. Comparisons between swimmers and volleyball players were performed using student t-tests for independent samples and multivariate analysis of covariance (controlling for age, training history and body size).ResultsSwimmers (BMC: 2328±338 g) and volleyball players (BMC: 2656±470 g) exceeded respectively by 2.1 and 2.8 standard deviation scores the average of international standards for whole body BMC of healthy adolescents. Years of training in swimmers were positively related to the upper limbs BMC (r=+0.49, p<0.05). In volleyball players, years of training correlated significantly with lower limbs BMD (r=+0.43, p<0.05). After adjustments for potential confounders, moderate differences (ES-r=0.32) between swimmers and volleyball players were noted in BMD at the lower limbs (volleyball players: +0.098 g∙cm-2, +7.8%).ConclusionsYouth female athletes who participate in high-intensity weight-loading activities such as volleyball exhibit moderately higher levels of BMD at the lower limbs compared to non-loading sports such as swimming.