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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.

Resumen Objetivo Analizar la incidencia, factores pronosticos, indicacion de tratamiento quirurgico y supervivencia de los pacientes con criterios de oligometastasis oseas con los criterios de solitaria y normal osea tratados en centros expertos en la reseccion tumoral de lesiones oseas. Material y metodo Analisis retrospectivo de 414 pacientes afectos de metastasis oseas secundarias a carcinomas del Hospital Universitario La Paz y Hospital MD Anderson Cancer Center (Madrid), entre mayo de 2006 y mayo de 2019. Fueron excluidos las metastasis localizadas en pelvis y esqueleto axial, analizandose un total de 28 pacientes que cumplian con el criterio de metastasis solitaria u oligometastasis con criterio normal. La estimacion de la supervivencia del estudio se llevo a cabo siguiendo el metodo estadistico de Kaplan-Meier. Resultados La supervivencia de los pacientes siguiendo los criterios de oligometastasis (solitaria y normal) fue del 53%. El cancer de mama fue el mas prevalente y presento una supervivencia superior al 70%. La edad promedio de los pacientes fue de 58 anos. Discusion Los tratamientos sistemicos en el tratamiento del cancer han conseguido mejorar las curvas de supervivencia libre de enfermedad y nos lleva a reflexionar sobre el paradigma del tratamiento de las oligometastasis, planteando que el tratamiento deberia realizarse en centros expertos en la reseccion tumoral de lesiones oseas. Conclusiones La eleccion del tratamiento quirurgico de los pacientes afectos de oligometastasis en sentido estricto (solitaria) y normal deben ser evaluada por equipos multidisciplinarios, segun el pronostico del paciente, localizacion anatomica e histiotipo de la neoplasia. Nivel de evidencia 3.

BACKGROUND: Tumor surgeons use a variety of endoprosthetic designs for reconstruction after bone tumor resection. However, functional results and implant survival have not been evaluated for each design. Because the outcomes and failure modes (for example, implant breakage, loosening) may differ between prosthetic design types, it is important to examine the problems associated with different designs. Because of experiences in our practice, we became concerned about a surprisingly high frequency of device breakage with one particular design, and we wished to report on that experience. QUESTION/PURPOSES: (1) In a small series of patients, what proportion of a particular design (Zimmer® Segmental [Zimmer Inc, Warsaw, IN, USA]) of rotating-hinge endoprosthesis experienced implant breakage at short-term follow-up? (2) What patient symptoms were associated with this finding? (3) What is the function as assessed by Musculoskeletal Tumor Society (MSTS) score with the use of this implant before and after revision? METHODS: We treated 87 patients in our tertiary center from 1987 to 2014 who had sarcomas around the knee with wide resection and reconstruction with tumoral endoprosthesis; five patients were lost to follow-up. In all, 33 of the remanining 82 prostheses, treated from 1987 to 2006, were reconstructed with fixed-hinge designs. From 2006 to 2014, 49 patients were reconstructed with a knee endoprosthesis, and 48 of them had a rotating-hinge prosthesis. In our center, we mostly used four designs: 16 of 49 patients were reconstructed with GMRS(™) (Stryker Howmedica, Kalamazoo, MI, USA), seven received the LPS™ (DePuy Synthes, Warsaw, IN, USA), 20 of 49 had the METS (Stanmore, Hertfordshire, UK), and six of 49 received the Zimmer Segmental. The focus of this report is on the six patients with the Segmental. We retrospectively gathered clinical and radiologic data from these six patients’ records and we assessed radiographic images. We evaluated function with the MSTS score of the 49 patients. The median follow-up duration of the Segmental prosthesis reconstruction was 65 months (range 24 to 85). RESULTS: Three of the six patients had posterior instability and recurrent joint effusion on physical examination. Three patients who did not have hyperextension presented with restricted knee ROM. Six revision procedures were performed in three patients. The median MSTS score at 6 months for the Segmental® prosthesis was 15 of 30 (range 6 to 24). The score in the three patients who had posterior instability was 9 of 30 (range 6 to 15) and it improved to median 25 of 30 (range 19 to 30) 6 months after revision. The patients with the Segmental® prosthesis who did not undergo revision had a median MSTS score of 20 (range 16 to 24). CONCLUSIONS: The Zimmer Segmental rotating-hinge tumoral prosthesis underwent revision for implant breakage at short term in three of six patients after tumor resection and reconstruction of the knee. Bumper breakage was associated with posterior instability that was related to wear of the bushing blocking system. We are unaware of reports of these issues by other observers or in other prosthetic designs, but we feel larger registries should be created to see if this failure mechanism has been observed by others. If so, this design needs to be improved or the blocking system should be avoided. Level of Evidence Level IV, therapeutic study.

The appearance of bone metastases (BM) in individuals with advanced solid cancers (breast, prostate, lung) often worsens their quality of life and prognosis. Although none have been fully effective, several strategies have been used to combat BM. Hence, the need for new data that could be useful for treating bone metastasis. To this end, we reviewed the recent literature on the subject. About patients with prostate cancer, treatments with PIP5K1α inhibitors have been found to inhibit tumor invasion and metastasis, and G protein-coupled receptor class C group 5 member A (GPRC5A) could be a future therapeutic target. Regarding patients with breast cancer, we found the following: Asperolide A could be another curative drug; targeting transforming growth factor-beta (TGFβ) and bone morphogenetic protein (BMP) signaling pathways, along with osteoclast activity, could be a favorable therapeutic approach in the preclusion of osteolytic bone destruction; TRAF6 inhibitors such as 6877002 appear promising; aiming the BMP4-SMAD7 signaling axis is an innovative therapeutic approach; there is favorable proof for the plausible therapeutic utilization of bone aiming immunostimulatory MOF (BT-isMOF) nanoparticles, and inhibition of IL4R and macrophages could have therapeutic benefits. For lung cancer, the function of LIGHT in osteolytic osseous illness instigated by metastatic non-small cell lung cancer should be highlighted.

To analyze the incidence and survival of patients with oligometastases (solitary and normal) when they are treated in centers that are experts in multidisciplinary approach to patients with sarcoma.Retrospective analysis of 414 patients with bone metastases secondary to carcinomas at Hospital Universitario La Paz and Hospital MD Anderson Cancer Center (Madrid) between May 2006 and May 2019. Metastases located in the pelvis and axial skeleton were excluded, analyzing a total of 28 patients who met the criterion for solitary metastases or oligometastases with normal criteria. The study survival estimate was carried out following the Kaplan-Meier statistical method.The survival of the patients following the oligometastases criteria (solitary and normal) was 53%. Breast cancer was the most prevalent and had a survival rate of more than 70%. The average age of the patients was 58 years old.Systemic treatments in cancer treatment have managed to improve disease-free survival curves and lead us to redirect on the paradigm for the treatment of oligometastases, stating that treatment should be carried out in the centers that are experts in the treatment of sarcomas.The choice of surgical treatment for patients with oligometastases in the strict sense (solitary) and normal should be evaluated by multidisciplinary teams according to the prognoses of the patient, anatomical location and histiotype of the neoplasm.

Deep soft tissue sarcomas are frequently in contact with bone. The therapeutic decision of a composite resection strategy may be challenging, which is usually based on clinical and radiological criteria. The aims of the study were to evaluate the overall frequency of bone and periosteal infiltration in these patients in whom composite resection was indicated, and evaluate the role of magnetic resonance imaging and bone scintigraphy in this scenario. Forty-nine patients with a composite surgical resection (soft tissue sarcoma and bone), treated at a single institution between 2006 and 2018, were retrospectively included. Presurgical planning of the resection limits was based on clinical and imaging findings (magnetic resonance imaging and bone scintigraphy). Magnetic resonance imaging was performed in all patients (100%) and bone scintigraphy in 41 (83.7% of the cases). According to magnetic resonance imaging results, patients were divided into two groups: Group A, in which the tumor is adjacent to the bone without evidence of infiltration (n = 24, 48,9%), and Group B, patients with evidence of bone involvement by magnetic resonance imaging (n = 25, 51,1%). BS showed a pathological deposit in 28 patients (68.3%). Histological analysis of the resection specimen was preceded to identify bone and periosteal infiltration. For the analysis of the diagnostic validity of imaging tests, histological diagnosis was considered as the gold standard in the evaluation of STS bone infiltration. Histological bone infiltration was identified in 49% of patients and isolated periosteal infiltration in 14.3%. In terms of diagnostic accuracy, magnetic resonance imaging and bone scintigraphy sensitivity values were 92% and 90%, and their specificity values were 91.7% and 52.4%, respectively. The incidence of bone and periosteal infiltration of soft tissue sarcomas in contact with bone is high. Presurgical bone assessment by MRI has proven to be a sensitive and specific tool in the diagnosis of bone infiltration. Due to its high negative predictive value, BS is a useful test to rule out it. In those cases, in which there is suspicion of bone infiltration not confirmed by MRI, new diagnostic protocols should be established in order to avoid inappropriate resections.

Low-grade myofibroblastic sarcoma (L-G MFS) is a rare tumour that commonly affects the soft tissues of the head and neck. Its location in bone is very rare and there are few cases published in the literature, which sometimes leads to a wrong initial diagnosis. The following report will give an overview of the clinical, radiological and histological findings in two patients with a very unusual bone sarcoma, which are more often found in the soft tissues. Treatment options and outcome will be discussed. On excision of any sarcoma tumor, surgeons should be aware of the potential risk for erroneous management of malignancy. If not, careless surgery may render the treatment protocol complicated and additional bed tumor resection with poor function and prognosis. Low-grade myofibroblastic sarcoma (L-G MFS) requires a wide resection to avoid local recurrence and distant metastases. We present two cases with an initial diagnosis of giant cell tumor and fibrous dysplasia respectively, which were diagnosed as low-grade bone myofibroblastic sarcoma after clinical, radiological y pathological studies.

The ideal treatment of pelvis bone metastases may be complex and involve multimodality treatment approaches to achieve optimal outcomes. The chapter describes the treatments indications of these patients. It is described from nonoperative treatments, through minimally invasive palliative procedures until reaching the surgical treatment, emphasizing types of pelvis resection and indications for surgical reconstruction of the acetabulum.

Background and objectives Ewing sarcoma is the second most common bone sarcoma of childhood. Ewing sarcomas of the scapula are rare, with little known about their characteristics and outcomes. In this study, we describe the demographic characteristics, tumor characteristics, and oncologic outcomes of patients with Ewing sarcoma of the scapula. Methods This is a retrospective case series of thirty-four patients treated at three urban hospitals between 1993 and 2014 for Ewing sarcomas affecting the scapula. Their demographic data, tumor characteristics, and oncologic outcomes are reported and contrasted with data on Ewing sarcoma described in the literature. Results Patients in our case series were 59% male. The average age at diagnosis was 16 years. 44% of patients had metastatic disease at presentation. 26% of patients had a tumor size >8 cm in largest dimension at diagnosis. 9 patients in our series had the t (11; 22) translocation present. Patients had a survival rate of 68% at five years. No patients had local recurrence of disease. Compared with findings reported in the literature concerning Ewing sarcoma affecting other locations, patients with Ewing sarcoma of the scapula were slightly older at time of diagnosis, had a lower percentage of tumors with size > 8 cm in largest dimension at presentation, and more commonly had metastatic disease at presentation. Patients in our cohort had a 5-year survival rate of 68%, which is higher than the rate of approximately 55% as reported in the general literature. Conclusions In this study, we describe a retrospective case series of thirty-four patients with Ewing sarcomas of the scapula. This is the largest case series to date of Ewing sarcoma affecting this location to our knowledge. These results will contribute to the understanding of the clinical profile and oncologic behavior of Ewing sarcomas affecting the scapula.

Background Reconstruction after internal hemipelvectomy resection likely provides better function than hindquarter amputation. However, many reconstruction methods have been used, complications with these approaches are common, and function often is poor; because of these issues, it seems important to investigate alternative implants and surgical techniques.

ResumenLa biopsia es una etapa importante en el diagnóstico de los tumores óseos y de partes blandas. No es un procedimiento sencillo. El objetivo de este artículo es ofrecer algunas sugerencias a los médicos que se encargan del cuidado de estos pacientes y evitar los riesgos inherentes de la biopsia. Las decisiones relacionadas con la indicación de una biopsia y la zona exacta que debe ser biopsiada puede ser la diferencia entre una biopsia eficaz y un desastre. Se debe planificar el proceso de la biopsia, tan cuidadosamente como la cirugía definitiva. Si el cirujano ortopédico o la institución no están convenientemente preparados para realizar los estudios diagnósticos correctos, la cirugía definitiva, ni los tratamientos adyuvantes, el paciente debería ser canalizado a un centro con experiencia en esta dolencia y con un equipo multidisciplinario antes de la realización de la biopsia.AbstractBiopsy is a significant step in the diagnosis of a bone or soft tissue tumor. It is not a simple procedure. The purpose of this article is to offer some suggestions to physicians who care for such patients, to assistance them in avoiding the hazards inherent in the biopsy procedure. Decisions concerning the indication for biopsy, the exact region of the lesion that has to have a biopsy and the anatomic approach and biopsy procedure can make the difference between an efficacious biopsy and a disaster. Plan the biopsy procedure as carefully as the definitive surgery. If the orthopaedic surgeon or the institution is not equipped to accomplish accurate diagnostic studies or definitive surgery and/or adjunctive treatment, the patient should be referred to center, which has experience of these types of neoplasms and equipped with multidisciplinary team prior to performance the biopsy.

The optimal surgical treatment of bone metastases may be complex and require multimodality treatment strategies to achieve optimal outcomes. We describe the surgical indications of these patients, mainly in the periacetabular zone.

Bone tumors are commonly located around the knee, in the distal femur and proximal tibia and less frequently in the proximal fibula and patella. Treatment of bone tumors depends on the histological grade, the size and location of the tumor and the characteristics of the patient. Generally, benign bone tumors are adequately treated by either an intralesional or a marginal resection. To reduce the risk of recurrence, the intralesional resection may be extended with mechanical, chemical, and thermal adjuncts. Primary bone sarcomas and some aggressive benign tumors require a wide resection. Limb salvage principles are largely employed if neurovascular structures can be preserved and a level of function maintained. After tumor resection with a wide margin, the defect left poses a reconstructive challenge. In this chapter, we will refer to bone tumors that most frequently arise around the knee and may affect adult population. First, we consider the basic characteristics of these tumors and then the surgical treatment, focusing in those techniques that preserve the joint.

A hemophilic pseudotumor is basically an encapsulated hematoma; it has become rarer over the years with better treatment modalities for bleeding disorders like factor replacement. Its prevention is paramount; this goal can be achieved by primary prophylaxis to avoid muscle hematomas and by adequate and long-term hematological treatment of muscle hematomas in case they appear. There are a number of therapeutic alternatives for this dangerous condition: surgical removal, percutaneous management, exeresis and filling of the dead cavity, irradiation, and embolization. The management of the patient with a hemophilic pseudotumor is complex and with a high rate of potential complications. Surgical excision is the treatment of choice but should only be carried out in major hemophilia centers by a multidisciplinary surgical team. The main postoperative complications are death, infection, fistulization, and pathological fractures (requiring even amputation of the affected limb).