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Survival in patients who have Ewing sarcoma is correlated with postchemotherapy response (tumor necrosis). This treatment response has been categorized as the response rate, similar to what has been used in osteosarcoma. There is controversy regarding whether this is appropriate or whether it should be a dichotomy of complete versus incomplete response, given how important a complete response is for in overall survival of patients with Ewing sarcoma. The purpose of this study was to evaluate the impact that the amount of chemotherapy-induced necrosis has on (1) overall survival, (2) local recurrence-free survival, (3) metastasis-free survival, and (4) event-free survival in patients with Ewing sarcoma.In total, 427 patients who had Ewing sarcoma or tumors in the Ewing sarcoma family and received treatment with preoperative chemotherapy and surgery at 10 international institutions were included. Multivariate Cox proportional-hazards analyses were used to assess the associations between tumor necrosis and all four outcomes while controlling for clinical factors identified in bivariate analysis, including age, tumor volume, location, surgical margins, metastatic disease at presentation, and preoperative radiotherapy.Patients who had a complete (100%) tumor response to chemotherapy had increased overall survival (hazard ratio [HR], 0.26; 95% CI, 0.14-0.48; p .01), recurrence-free survival (HR, 0.40; 95% CI, 0.20-0.82; p = .01), metastasis-free survival (HR, 0.27; 95% CI, 0.15-0.46; p ≤ .01), and event-free survival (HR, 0.26; 95% CI, 0.16-0.41; p ≤ .01) compared with patients who had a partial (0%-99%) response.Complete tumor necrosis should be the index parameter to grade response to treatment as satisfactory in patients with Ewing sarcoma. Any viable tumor in these patients after neoadjuvant treatment should be of oncologic concern. These findings can affect the design of new clinical trials and the risk-stratified application of conventional or novel treatments.

Chondrosarcoma (CS) is the second most common primary malignant bone tumour and, in the absence of reliable chemotherapy and radiotherapy, is effectively a surgical disease. Overall disease specific survival (DSS) is affected by tumour grade, whilst resection margin contributes to local recurrence free survival (LRFS). The aim of this study was to investigate factors that affect the local and systemic prognoses for conventional central CSs arising from the proximal humerus. A multi-centre, retrospective study from three international collaborative sarcoma centres identified 110 patients between 1995 and 2020 undergoing treatment for a conventional central CS of the proximal humerus; 58 patients (53%) had a grade 1 tumour, 36 (33%) had a grade 2 tumour, and 16 patients (13%) had a grade 3 CS. The mean age of patients was 50 years (range 10–85). The incidence of local recurrence (LR) was 9/110 (8.2%), and the disease specific mortality was 6/110 (5.5%). The grade was a statistically significant factor for LRFS (p < 0.001). None of the grade 1 tumours developed LR. The DSS was affected by the grade (p < 0.001) but not by the LR (p = 0.4). Only one patient with a grade 2 tumour died from the disease. The proximal humeral grade 1 CS behaved as a benign tumour, having no cases of LR nor death due to disease. Grade 2 CSs of the proximal humerus behaved in a more indolent way when compared with comparable grade tumours elsewhere in the appendicular skeleton, being locally aggressive with a higher LR rate than grade 1 CSs but still having very low mortality and a high rate of DSS. The LR in grade 2 CSs did not affect the DSS; therefore, surgical management in proximal humeral grade 2 CSs should have a greater emphasis on preserving function whilst maintaining an adequate margin for resection. The proximal humeral grade 3 CS was, as elsewhere in the skeleton, an aggressive, high-grade tumour. Therefore, surgical management should include en bloc resection with clear margins to avoid LR.

Background: Establishing the proper diagnosis and rendering appropriate treatment of spinal primary bone tumors (SPBT) can result in definitive cures. Notably, malignant, or benign SPBT (i.e., with aggressive local behavior) generally require en bloc resection. Osteotomies of the vertebral body in more than 1 plane may avoid critical structures, preserve nerve functions, and reduce the volume of healthy bone resected. Here, our objective was to report how we planned and performed navigated multiplanar osteotomies for en bloc resection of 14 SPBT. Methods: We performed a retrospective analysis of 14 patients with malignant or locally aggressive benign SPBT operated on consecutively between 2014 and 2019 utilizing preoperative 3D planning/navigation. Tumors were resected in an en bloc fashion utilizing multiplanar osteotomies. Patients were followed for a minimum of 12 postoperative months. Results: Diagnoses included three benign but locally aggressive bone tumors (i.e., all osteoblastomas) and 11 primary sarcomas (i.e., six chordomas and five chondrosarcomas). Eleven tumors involved the sacrum and the other three, the thoracic spine. In 12 patients, the en bloc margins were classified as marginal (1 cm). Intraoperative navigation facilitated the performance of 40 osteotomies in 14 patients (median = 2.9, range = 2–6). Conclusion: Navigated multiplanar osteotomies increased the precision and safety of en bloc resections for 14 primary spinal bone tumors SPBT that included 11 malignant and three benign/locally aggressive lesions.

Soft tissue sarcomas in children under 2 years of age are infrequent. During 2007-2017, a total of 445 patients diagnosed with soft tissue sarcomas were treated at our institution, 6(0.5%) were under 2 years. We analysed clinical and oncologic outcomes in this select group. The mean age of diagnosis was 15 months. Four patients were male and 2 female. The mean follow-up time was 29 months. Fibrosarcoma (n = 4) was the most frequent diagnosis. Five patients were treated with limb salvage surgery, and the remaining one had to undergo amputation. All patients received adjuvant treatment with chemotherapy. The 24-month survival rate was 100 %. Two patients presented a local recurrence before 24-months followup. Surgical treatment associated with chemotherapy seems to be the best therapeutic option. Local recurrence rate after limb salvage surgery is high for this group of patients.Los sarcomas de partes blandas en menores de 2 años son infrecuentes. Durante 2007-2017, 445 pacientes con sarcomas de partes blandas fueron tratados en nuestra Institución; 6 (el 0,5 %) eran menores de 2 años. Se analizaron los resultados clínicos y oncológicos en este grupo. La edad media de diagnóstico fue 15 meses. Cuatro eran varones y 2, mujeres. El seguimiento promedio fue 29 meses. El fibrosarcoma (n = 4) fue la variedad más frecuente. Cinco fueron tratados con cirugía de conservación del miembro; al restante se le realizó amputación. Todos realizaron tratamiento adyuvante con quimioterapia. La supervivencia a 24 meses fue del 100 %. Dos pacientes presentaron recidiva local; ambos casos, antes de los 24 meses. El tratamiento quirúrgico asociado a la quimioterapia impresiona ser la mejor opción terapéutica. La proporción de recurrencia local es alta para este grupo de pacientes luego de la cirugía de conservación del miembro.

Osteoarticular allograft is one possible treatment in wide surgical resections with large defects. Performing best osteoarticular allograft selection is of great relevance for optimal exploitation of the bone databank, good surgery outcome and patient's recovery. Current approaches are, however, very time consuming hindering these points in practice. We present a validation study of a software able to perform automatic bone measurements used to automatically assess the distal femur sizes across a databank. 170 distal femur surfaces were reconstructed from CT data and measured manually using a size measure protocol taking into account the transepicondyler distance (A), anterior-posterior distance in medial condyle (B) and anterior-posterior distance in lateral condyle (C). Intra- and inter-observer studies were conducted and regarded as ground truth measurements. Manual and automatic measures were compared. For the automatic measurements, the correlation coefficients between observer one and automatic method, were of 0.99 for A measure and 0.96 for B and C measures. The average time needed to perform the measurements was of 16 h for both manual measurements, and of 3 min for the automatic method. Results demonstrate the high reliability and, most importantly, high repeatability of the proposed approach, and considerable speed-up on the planning.

Tumor excision is the primary treatment of aggressive or recurrent benign bone tumors and malignant bone sarcomas. This requires a surgical resection with the potential for large residual osseous defects that could be reconstructed using fresh frozen allografts. Virtual bone banks enable the creation of databases allowing a 3D pre-surgery evaluation of such allgorafts, based on segmentation of DICOM-CT images. This study demonstrates the usefulness of patient specific 3D models for an accurate host-donor allograft match. We describe one way to select the best match according to size and shape. The results suggest that a robust and reliable technique has been established. Since it is difficult to plan an allograft on a distal femur deformed by the tumor, we propose to plan the surgery on the contralateral side. Our results support this limb symmetry hypothesis. The use of this measurement protocol enables accurate selection of allografts from a contralateral healthy femur 3D CT model achieving the best match possible considering the geometry of available allograft candidate femur specimens.

Even though massive osteoarticular allografts have been reported to restore function successfully after tumor resection, little has been published about this procedure in post-traumatic joint reconstruction. We report two pediatric patients who sustained a grade III open fracture of the distal femur and were treated with osteoarticular allografts as a salvage procedure. In each patient, a femoral condyle was inserted to treat the bone and cartilage defect, and a 4.5 dynamic compressive plate was applied to fix the graft to the host bone. There were no complications after the reconstructive procedure. At 2 and 10 years of follow-up, both patients showed a favorable functional and radiographic outcome using the scoring instruments of the Musculo-skeletal Tumor Society and the International Symposium on Limb Salvage.

ResumenIntroducción: El objetivo de éste trabajo fue evaluar la sobrevida, complicaciones y resultados funcionales en un grupo de pacientes tratados con un aloinjerto intercalar de tibia luego de la resección tumoral primaria.Material y métodos: Se evaluaron 26 tratados con aloinjertos intercalares de tibia luego de un defecto óseo secundario a una resección tumoral primaria. El seguimiento promedio fue de 6 años. La supervivencia del aloinjerto fue determinada por el método de Kaplan –Meier. La evaluación funcional fue realizada con la escala de la Musculoskeletal Tumor Society (MSTS). También fueron registradas las complicaciones.Resultados: La sobrevida global de las reconstrucciones fue del 84% (CI 95%: 90%-70%) a los 5 años y 79% a los 10 años (CI 95%: 95%-63%). El aloinjerto debió ser removido en 5 pacientes, en 3 casos debido a infección y en 2 casos por recurrencia local. Dos pacientes presentaron falta de consolidación diafisaria y 3 pacientes presentaron una fractura incompleta. En ninguno de estos pacientes el aloinjerto debió ser removido. El resultado funcional promedio fue de 27 puntos con un rango de 27 a 30 puntos. Esto corresponde a un miembro funcional con capacidad funcional aceptable y sin dolor limitante.Conclusiones: A pesar de la incidencia de complicaciones, éste análisis demostró una sobrevida aceptable con excelentes resultados funcionales. La reconstrucción con aloinjerto intercalar de tibia permite restaurar la anatomía de la pierna proporcionando buenos resultados clínicos y funcionales.

Techniques for the reconstruction of knee ligaments must restore the injured knee to a satisfactory level of performance. For this, a precise anatomic reconstruction is necessary. Many arthroscopic techniques for reconstruction of the posterior cruciate ligament (PCL) have been reported to restore the normal anatomy of the intact PCL using a double-bundle reconstruction with a Y-shaped tendon graft (2 femoral tunnel and 1 tibial tunnel). However, this procedure is sometimes difficult because the graft must be tightened in different grades of flexion to obtain complete strength of both bundles. We thought that double-bundle PCL reconstruction using double-double tunnels (2 femoral and 2 tibial tunnels) would allow an anatomic reconstruction, restoring better knee biomechanics, and probably improving patient outcome.