Ewing sarcoma is a small round-cell sarcoma characterized by gene fusion involving EWSR1 (or another TET family protein like FUS) and an ETS family transcription factor. The estimated incidence of this rare bone tumor, which occurs most frequently in adolescents and young adults, is 0.3 per 100,000/year. Although only 25% of patients with Ewing sarcoma are diagnosed with metastatic disease, historical series show that this is a systemic disease. Patient management requires multimodal therapies-including intensive chemotherapy-in addition to local treatments (surgery and/or radiotherapy). In the recurrent/refractory disease setting, different approaches involving systemic treatments and local therapies are also recommended as well as patient inclusion in clinical trials whenever possible. Because of the complexity of Ewing sarcoma diagnosis and treatment, it should be carried out in specialized centers and treatment plans should be designed upfront by a multidisciplinary tumor board. These guidelines provide recommendations for diagnosis, staging, and multimodal treatment of Ewing sarcoma.

UNASSIGNED: Extra-articular hip resection may be necessary in cases of malignant tumour of the pelvic bone or of the proximal femur invading the hip joint. When the tumour is in the proximal femur, it is possible to resect the acetabulum en bloc by performing a periacetabular osteotomy, but this creates a discontinuity in the pelvic ring with difficult reconstruction and diminished function. Several techniques described recently seek to be as sparing as possible on the pelvic bone by preserving the posterior column or both columns in order to facilitate reconstruction and improve function. However, these still require complex reconstructions and can necessitate intra-pelvic dissection.
UNASSIGNED: We describe here an extra-articular hip resection technique for tumours of the proximal femur invading the joint, with maintenance of pelvic continuity by preserving both columns and the quadrilateral plate of the acetabulum, without intra-pelvic dissection, that can be performed on patients in whom the medial wall of the acetabulum is thick enough. Our preliminary assessments have included studies on dry bone and imaging analyses. The technique was first tested on a single cadaver pelvis (encompassing 2 hips) and subsequently performed on a patient with a pathological fracture of the femoral neck due to osteosarcoma secondary to Paget\'s disease.
UNASSIGNED: Further clinical applications are essential to evaluate the overall effectiveness, safety and impact on patient functionality of this experimental technique.

OBJECTIVE: Socioeconomic and racial disparities have been recognized as impacting the care of patients with cancer, however there are a lack of data examining the impact of these disparities on patients with bone sarcoma. The purpose of this study was to examine socioeconomic and racial disparities that impact the oncological outcomes of patients with bone sarcoma.
METHODS: We reviewed 4,739 patients diagnosed with primary bone sarcomas from the Surveillance, Epidemiology and End Results (SEER) registry between 2007 and 2015. We examined the impact of race and insurance status associated with the presence of metastatic disease at diagnosis, treatment outcome, and overall survival (OS).
RESULTS: Patients with Medicaid (odds ratio (OR) 1.41; 95% confidence interval (CI) 1.15 to 1.72) and uninsured patients (OR 1.90; 95% CI 1.26 to 2.86) had higher risks of metastatic disease at diagnosis compared to patients with health insurance. Compared to White patients, Black (OR 0.63, 95% CI 0.47 to 0.85) and Asian/Pacific Islander (OR 0.65, 95% CI 0.46 to 0.91) were less likely to undergo surgery. In addition, Black patients were less likely to receive chemotherapy (OR 0.67, 95% CI 0.49 to 0.91) compared to White patients. In patients with chondrosarcoma, those with Medicaid had worse OS compared to patients with insurance (hazard ratio (HR) 1.65, 95% CI 1.06 to 2.56).
CONCLUSIONS: In patients with a bone sarcoma, the cancer stage at diagnosis varied based on insurance status, and racial disparities were identified in treatment. Further studies are needed to identify modifiable factors which can mitigate socioeconomic and racial disparities found in patients with bone sarcomas. Cite this article: Bone Joint Res 2022;11(5):278-291.

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UNASSIGNED: The main challenge in reconstruction after malignant bone tumour resection in young children remains how and when growth-plates can be preserved and which options remain if impossible.
UNASSIGNED: We describe different strategies to assure best possible long-term function for young children undergoing resection of malignant bone tumours.
UNASSIGNED: Different resources are available to treat children with malignant bones tumours: a) preoperative planning simulates scenarios for tumour resection and limb reconstruction, facilitating decision-making for surgical and reconstructive techniques in individual patients; b) allograft reconstruction offers bone-stock preservation for future needs. Most allografts are intact at long-term follow-up, but limb-length inequalities and corrective/revision surgery are common in young patients; c) free vascularized fibula can be used as stand-alone reconstruction, vascularized augmentation of structural allograft or devitalized autograft. Longitudinal growth and joint remodelling potential can be preserved, if transferred with vascularized proximal physis; d) epiphysiolysis before resection with continuous physeal distraction provides safe resection margins and maintains growth-plate and epiphysis; e) 3D printing may facilitate joint salvage by reconstruction with patient-specific instruments. Very short stems can be created for fixation in (epi-)metaphysis, preserving native joints; f) growing endoprosthesis can provide for remaining growth after resection of epi-metaphyseal tumours. At ten-year follow-up, limb survival was 89%, but multiple surgeries are often required; g) rotationplasty and amputation should be considered if limb salvage is impossible and/or would result in decreased function and quality of life.
UNASSIGNED: Several biological and technological reconstruction options must be merged and used to yield best outcomes when treating young children with malignant bone tumours.
UNASSIGNED: Level V Expert opinion.

UNASSIGNED: Although malignant bone tumours in children are infrequent, it is important to know how to properly diagnose and stage them, in order to establish an adequate treatment.
UNASSIGNED: We present a review of the diagnostic workflow of malignant bone tumours in children, including history and clinical examination, imaging, laboratory tests and biopsy techniques. Moreover, the two most commonly used staging systems are reviewed.
UNASSIGNED: History, clinical examination and laboratory tests are nonspecific for diagnosing malignant bone tumours in children. Radiographs remain the mainstay for initial diagnosis, with MRI the modality of choice for local assessment and staging. Fluorine-18 labelled fluoro-deoxy-glucose-positron emission tomography scans provide a noninvasive method to assess the aggressiveness of the tumour and to rule out metastasis and is replacing the use of the bone scintigraphy. Biopsy must be always performed under the direction of the surgeon who is to perform the surgical treatment and after all diagnostic evaluation has been done. Staging systems are useful to study the extent of the tumour and its prognosis. They are expected to evolve as we better understand new molecular and genetic findings.
UNASSIGNED: When a malignant bone tumour is suspected in a child, it is essential to make a correct diagnosis and referral to an experienced centre. Following an appropriate workflow for diagnosis and staging facilitates, prompt access to treatment improves outcomes.
UNASSIGNED: Level V Expert opinion.

Introduction The management of malignant bone tumors of lower extremities involves various modalities, which depend not only on local and systemic affection but are also affected by psychosocial factors. The purpose of this study was to evaluate functional and psychosocial outcomes in patients with non-salvageable lower limbs having malignant or aggressive benign bone tumors of distal thighs, who were treated with a technique called straightplasty. Material and methods We enrolled 20 patients of non-salvageable primary malignant or aggressive benign bone tumors around the knee. Out of these, 15 patients were followed and evaluated in view of functional and clinical outcomes having a minimum of 22 months of final follow-up. Results A total of 15 patients (8 males, 7 females) having a non-salvageable lower limb with a mean age of 20.53 years (range, 12 to 45 years), who were managed with straightplasty and followed for a mean duration of 31.73 months (range 22 to 72 months) were evaluated clinico-radiologically, and the functional outcomes were measured by Enneking\' s method. The surgical procedure is simple and better in terms of functional outcomes than other procedures described in the literature, while it is observed as psychosocially more acceptable in developing nations, especially in the Indian context. Most of the parameters are comparable to rotationplasty and above-knee amputation, whereas it is less technically demanding and satisfying due to the straight limb rather than the rotated leg in rotationplasty. Conclusion We recommend straightplasty as an alternative to rotationplasty or above-knee amputation in patients having malignant or aggressive benign tumors around the knee joint and where limb salvage procedures are not feasible.

In 1999, we developed a technique using frozen autografts-tumour-containing bone treated with liquid nitrogen-for the reconstruction of malignant bone tumours. The purpose of this study was to evaluate the functional and oncological outcomes of frozen autografts for intercalary reconstruction of malignant bones and soft tissue tumours.
This retrospective study was designed to assess 34 patients of mean age 35 (range, 6-79) years. The mean follow-up period was 62 (24-214) months. The median length of the frozen autografts was 138.4 ± 60.39 (50-290) mm.
Postsurgically, 20 patients remained disease-free, seven patients survived with no evidence of disease, five patients were alive with disease, and two patients died of disease. The five- and ten-year survival rates of the frozen autografts were 91.2% and the mean International Society of Limb Salvage score was 90%. Complete bony union was achieved in 97% of the patients. There were five cases of nonunion, six cases of fracture, two cases of deep infection and four cases of local recurrence.
Utilizing intercalary frozen autografts for patients with a nonosteolytic primary or secondary bone tumour without involvement of the subchondral bone is a good alternative treatment, because it is a straightforward biological technique and can provide excellent limb function.